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Sample records for optic nerve hypoplasia

  1. Optic nerve hypoplasia

    Directory of Open Access Journals (Sweden)

    Savleen Kaur

    2013-01-01

    Full Text Available Optic nerve hypoplasia (ONH is a congenital anomaly of the optic disc that might result in moderate to severe vision loss in children. With a vast number of cases now being reported, the rarity of ONH is obviously now refuted. The major aspects of ophthalmic evaluation of an infant with possible ONH are visual assessment, fundus examination, and visual electrophysiology. Characteristically, the disc is small, there is a peripapillary double-ring sign, vascular tortuosity, and thinning of the nerve fiber layer. A patient with ONH should be assessed for presence of neurologic, radiologic, and endocrine associations. There may be maternal associations like premature births, fetal alcohol syndrome, maternal diabetes. Systemic associations in the child include endocrine abnormalities, developmental delay, cerebral palsy, and seizures. Besides the hypoplastic optic nerve and chiasm, neuroimaging shows abnormalities in ventricles or white- or gray-matter development, septo-optic dysplasia, hydrocephalus, and corpus callosum abnormalities. There is a greater incidence of clinical neurologic abnormalities in patients with bilateral ONH (65% than patients with unilateral ONH. We present a review on the available literature on the same to urge caution in our clinical practice when dealing with patients with ONH. Fundus photography, ocular coherence tomography, visual field testing, color vision evaluation, neuroimaging, endocrinology consultation with or without genetic testing are helpful in the diagnosis and management of ONH. (Method of search: MEDLINE, PUBMED.

  2. Schizencephaly with optic nerve hypoplasia simulating septo-optic dysplasia and other syndromes

    International Nuclear Information System (INIS)

    Osborn, R.E.; Byrd, S.E.

    1988-01-01

    Three patients with fused-lip schizencephaly with optic nerve hypoplasia were evaluated radiographically. The computed tomographic and magnetic resonance findings demonstrated unilateral or bilateral gray matter lined clefts in the cerebral hemisphere which allowed differentiation from the clinical diagnosis of septo-optic dysplasia in each case. Schizencephaly has also been mistakenly labelled as part of a syndrome known as the syndrome of absence of the septum pellucidum with porencephalies and as heterotopias with absence of the septum pellucidum. 15 refs.; 3 figs

  3. Reduced ventral cingulum integrity and increased behavioral problems in children with isolated optic nerve hypoplasia and mild to moderate or no visual impairment.

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    Webb, Emma A; O'Reilly, Michelle A; Clayden, Jonathan D; Seunarine, Kiran K; Dale, Naomi; Salt, Alison; Clark, Chris A; Dattani, Mehul T

    2013-01-01

    To assess the prevalence of behavioral problems in children with isolated optic nerve hypoplasia, mild to moderate or no visual impairment, and no developmental delay. To identify white matter abnormalities that may provide neural correlates for any behavioral abnormalities identified. Eleven children with isolated optic nerve hypoplasia (mean age 5.9 years) underwent behavioral assessment and brain diffusion tensor imaging, Twenty four controls with isolated short stature (mean age 6.4 years) underwent MRI, 11 of whom also completed behavioral assessments. Fractional anisotropy images were processed using tract-based spatial statistics. Partial correlation between ventral cingulum, corpus callosum and optic radiation fractional anisotropy, and child behavioral checklist scores (controlled for age at scan and sex) was performed. Children with optic nerve hypoplasia had significantly higher scores on the child behavioral checklist (pchildren with optic nerve hypoplasia. Right ventral cingulum fractional anisotropy correlated with total and externalising child behavioral checklist scores (r = -0.52, pchildren with optic nerve hypoplasia and mild to moderate or no visual impairment require behavioral assessment to determine the presence of clinically significant behavioral problems. Reduced structural integrity of the ventral cingulum correlated with behavioral scores, suggesting that these white matter abnormalities may be clinically significant. The presence of reduced fractional anisotropy in the optic radiations of children with mild to moderate or no visual impairment raises questions as to the pathogenesis of these changes which will need to be addressed by future studies.

  4. Reduced ventral cingulum integrity and increased behavioral problems in children with isolated optic nerve hypoplasia and mild to moderate or no visual impairment.

    Directory of Open Access Journals (Sweden)

    Emma A Webb

    Full Text Available OBJECTIVES: To assess the prevalence of behavioral problems in children with isolated optic nerve hypoplasia, mild to moderate or no visual impairment, and no developmental delay. To identify white matter abnormalities that may provide neural correlates for any behavioral abnormalities identified. PATIENTS AND METHODS: Eleven children with isolated optic nerve hypoplasia (mean age 5.9 years underwent behavioral assessment and brain diffusion tensor imaging, Twenty four controls with isolated short stature (mean age 6.4 years underwent MRI, 11 of whom also completed behavioral assessments. Fractional anisotropy images were processed using tract-based spatial statistics. Partial correlation between ventral cingulum, corpus callosum and optic radiation fractional anisotropy, and child behavioral checklist scores (controlled for age at scan and sex was performed. RESULTS: Children with optic nerve hypoplasia had significantly higher scores on the child behavioral checklist (p<0.05 than controls (4 had scores in the clinically significant range. Ventral cingulum, corpus callosum and optic radiation fractional anisotropy were significantly reduced in children with optic nerve hypoplasia. Right ventral cingulum fractional anisotropy correlated with total and externalising child behavioral checklist scores (r = -0.52, p<0.02, r = -0.46, p<0.049 respectively. There were no significant correlations between left ventral cingulum, corpus callosum or optic radiation fractional anisotropy and behavioral scores. CONCLUSIONS: Our findings suggest that children with optic nerve hypoplasia and mild to moderate or no visual impairment require behavioral assessment to determine the presence of clinically significant behavioral problems. Reduced structural integrity of the ventral cingulum correlated with behavioral scores, suggesting that these white matter abnormalities may be clinically significant. The presence of reduced fractional anisotropy in the optic

  5. Recessive Mutations in SLC38A8 Cause Foveal Hypoplasia and Optic Nerve Misrouting without Albinism

    NARCIS (Netherlands)

    Poulter, James A.; Al-Araimi, Musallam; Conte, Ivan; van Genderen, Maria M.; Sheridan, Eamonn; Carr, Ian M.; Parry, David A.; Shires, Mike; Carrella, Sabrina; Bradbury, John; Khan, Kamron; Lakeman, Phillis; Sergouniotis, Panagiotis I.; Webster, Andrew R.; Moore, Anthony T.; Pal, Bishwanath; Mohamed, Moin D.; Venkataramana, Anandula; Ramprasad, Vedam; Shetty, Rohit; Saktivel, Murugan; Kumaramanickavel, Govindasamy; Tan, Alex; Mackey, David A.; Hewitt, Alex W.; Banfi, Sandro; Ali, Manir; Inglehearn, Chris F.; Toomes, Carmel

    2013-01-01

    Foveal hypoplasia and optic nerve misrouting are developmental defects of the visual pathway and only co-occur in connection with albinism; to date, they have only been associated with defects in the melanin-biosynthesis pathway. Here, we report that these defects can occur independently of albinism

  6. Optical Coherence Tomography in Optic Nerve Hypoplasia: Correlation With Optic Disc Diameter, Nerve Fiber Layer Thickness, and Visual Function.

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    Kelly, John P; Baran, Francine; Phillips, James O; Weiss, Avery H

    2017-12-15

    The correlation between optic disc diameters (DDs) with average retinal nerve fiber layer thickness (RNFLT) and visual function in children with optic nerve hypoplasia (ONH) having nystagmus is unknown. Data were obtained from a retrospective review of 28 children (mean age: 9.4 years; ±5.1). Optic DD was defined as the maximal horizontal opening of Bruch membrane with spectral optical coherence tomography combined with a confocal laser ophthalmoscope. Average RNFLT was obtained from circumpapillary b-scans. RNFLT was also remeasured at eccentricities that were proportionate with DD to rule out potential sampling artifacts. Visual function was assessed by visual acuity at last follow-up and by visual evoked potentials (VEP) in 11 patients. The eye with the larger DD, which had better visual acuity, was analyzed to exclude potential effects of amblyopia. DD was correlated with average RNFLT (r = 0.61), visual acuity (r = 0.32), and VEPs (r = 0.66). The relationship between RNFLT and DD was as follows: average RNFLT (μm) = 0.074 * DD (μm) - 18.8. RNFLT also correlated with the ratio of horizontal optic DD to macula-disc-margin distance (DD:DM; r = 0.59). RNFLT measured at eccentricities proportionate with DD showed progressive decrease in thickness only for DDs <1,100 μm. All patients with DD <1,000 μm had subnormal visual acuity, whereas those with DD <1,200 μm had subnormal VEPs. DD correlates with average RNFLT and with visual function in children with ONH. Using OCT imaging, DD can be obtained in children with nystagmus and provides objective information.

  7. Reliability of magnetic resonance imaging for the detection of hypopituitarism in children with optic nerve hypoplasia.

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    Ramakrishnaiah, Raghu H; Shelton, Julie B; Glasier, Charles M; Phillips, Paul H

    2014-01-01

    It is essential to identify hypopituitarism in children with optic nerve hypoplasia (ONH) because they are at risk for developmental delay, seizures, or death. The purpose of this study is to determine the reliability of neurohypophyseal abnormalities on magnetic resonance imaging (MRI) for the detection of hypopituitarism in children with ONH. Cross-sectional study. One hundred one children with clinical ONH who underwent MRI of the brain and orbits and a detailed pediatric endocrinologic evaluation. Magnetic resonance imaging studies were performed on 1.5-Tesla scanners. The imaging protocol included sagittal T1-weighted images, axial fast fluid-attenuated inversion-recovery/T2-weighted images, and diffusion-weighted images of the brain. Orbital imaging included fat-saturated axial and coronal images and high-resolution axial T2-weighted images. The MRI studies were reviewed by 2 pediatric neuroradiologists for optic nerve hypoplasia, absent or ectopic posterior pituitary, absent pituitary infundibulum, absent septum pellucidum, migration anomalies, and hemispheric injury. Medical records were reviewed for clinical examination findings and endocrinologic status. All patients underwent a clinical evaluation by a pediatric endocrinologist and a standardized panel of serologic testing that included serum insulin-like growth factor-1, insulin-like growth factor binding protein-3, prolactin, cortisol, adrenocorticotropic hormone, thyroid-stimulating hormone, and free thyroxine levels. Radiologists were masked to patients' endocrinologic status and funduscopic findings. Sensitivity and specificity of MRI findings for the detection of hypopituitarism. Neurohypophyseal abnormalities, including absent pituitary infundibulum, ectopic posterior pituitary bright spot, and absent posterior pituitary bright spot, occurred in 33 children. Magnetic resonance imaging disclosed neurohypophyseal abnormalities in 27 of the 28 children with hypopituitarism (sensitivity, 96%). A

  8. Children with optic nerve hypoplasia face a high risk of neurodevelopmental disorders.

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    Dahl, Sara; Wickström, Ronny; Ek, Ulla; Teär Fahnehjelm, Kristina

    2018-03-01

    Optic nerve hypoplasia (ONH) is a congenital ocular malformation that has been associated with neurodevelopmental disorders, but the prevalence in unilateral disease and less severe visual impairment is unknown. We studied intellectual disability and autism spectrum disorders (ASDs) in patients with ONH. This was a population-based cross-sectional cohort study of 65 patients (33 female) with ONH below 20 years of age, living in Stockholm in December 2009, with data analysed in January 2016. Of these 35 were bilateral and 30 were unilateral. Neurodevelopmental disorders were diagnosed or confirmed by neurological assessments, the Five to Fifteen parent questionnaire and reviewing previous neuropsychological investigations or conducting neuropsychological tests. Bilateral ONH patients had lower mean full scale intelligence quotient scores than unilateral patients (84.4 and 99.4, respectively, p = 0.049). We assessed intellectual disability in 55 eligible patients, and it was more common in patients with bilateral ONH (18 of 32, 56%) than unilateral ONH (two of 23, 9%, p neurodevelopmental disorders, especially intellectual disability. The risk was lower in unilateral ONH, but the levels of neurodevelopmental disorders warrant screening of both groups. ©2017 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

  9. Optical coherence tomography detection of characteristic retinal nerve fiber layer thinning in nasal hypoplasia of the optic disc.

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    Haruta, M; Kodama, R; Yamakawa, R

    2017-12-01

    PurposeTo determine the clinical usefulness of optical coherence tomography (OCT) for detecting thinning of the retinal nerve fiber layer (RNFL) in eyes with nasal hypoplasia of the optic discs (NHOD).Patients and methodsThe medical records of five patients (eight eyes) with NHOD were reviewed. The ratio of the disc-macula distance to the disc diameter (DM/DD) and the disc ovality ratio of the minimal to maximal DD were assessed using fundus photographs. The RNFL thicknesses of the temporal, superior, nasal, and inferior quadrants were evaluated using OCT quadrant maps.ResultsAll eight eyes had temporal visual field defects that respected the vertical meridians that needed to be differentiated from those related to chiasmal compression. The mean DM/DD ratio was 3.1 and the mean disc ovality ratio was 0.81. The mean RNFL thicknesses of the temporal, superior, nasal, and inferior quadrants were 90.3, 103.1, 34.8, and 112.8 microns, respectively.ConclusionSmall optic discs and tilted discs might be associated with NHOD. Measurement of the RNFL thickness around the optic disc using OCT scans clearly visualized the characteristic RNFL thinning of the nasal quadrants corresponding to the temporal sector visual field defects in eyes with NHOD. OCT confirmed the presence of NHOD and might differentiate eyes with NHOD from those with chiasmal compression.

  10. Prenatal ethanol exposure in mice phenocopies Cdon mutation by impeding Shh function in the etiology of optic nerve hypoplasia

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    Benjamin M. Kahn

    2017-01-01

    Full Text Available Septo-optic dysplasia (SOD is a congenital disorder characterized by optic nerve, pituitary and midline brain malformations. The clinical presentation of SOD is highly variable with a poorly understood etiology. The majority of SOD cases are sporadic, but in rare instances inherited mutations have been identified in a small number of transcription factors, some of which regulate the expression of Sonic hedgehog (Shh during mouse forebrain development. SOD is also associated with young maternal age, suggesting that environmental factors, including alcohol consumption at early stages of pregnancy, might increase the risk of developing this condition. Here, we address the hypothesis that SOD is a multifactorial disorder stemming from interactions between mutations in Shh pathway genes and prenatal ethanol exposure. Mouse embryos with mutations in the Shh co-receptor, Cdon, were treated in utero with ethanol or saline at embryonic day 8 (E8.0 and evaluated for optic nerve hypoplasia (ONH, a prominent feature of SOD. We show that both Cdon−/− mutation and prenatal ethanol exposure independently cause ONH through a similar pathogenic mechanism that involves selective inhibition of Shh signaling in retinal progenitor cells, resulting in their premature cell-cycle arrest, precocious differentiation and failure to properly extend axons to the optic nerve. The ONH phenotype was not exacerbated in Cdon−/− embryos treated with ethanol, suggesting that an intact Shh signaling pathway is required for ethanol to exert its teratogenic effects. These results support a model whereby mutations in Cdon and prenatal ethanol exposure increase SOD risk through spatiotemporal perturbations in Shh signaling activity.

  11. Optic nerve size evaluated by magnetic resonance imaging in children with optic nerve hypoplasia, multiple pituitary hormone deficiency, isolated growth hormone deficiency, and idiopathic short stature.

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    Birkebaek, Niels Holtum; Patel, Leena; Wright, Neville Bryce; Grigg, John Russell; Sinha, Smeeta; Hall, Catherine Margaret; Price, David Anthony; Lloyd, Ian Christopher; Clayton, Peter Ellis

    2004-10-01

    To objectively define criteria for intracranial optic nerve (ON) size in ON hypoplasia (ONH) on magnetic resonance imaging (MRI) scans. Intracranial ON sizes from MRI were compared between 46 children with ONH diagnosed by ophthalmoscopy (group 1, isolated ONH, 8 children; and group 2, ONH associated with abnormalities of the hypothalamic-pituitary axis and septum pellucidum, 38 children) and children with multiple pituitary hormone deficiency (group 3, multiple pituitary hormone deficiency, 14 children), isolated growth hormone deficiency (group 4, isolated growth hormone deficiency, 15 children), and idiopathic short stature (group 5, idiopathic short stature, 10 children). Intracranial ON size was determined by the cross-sectional area, calculated as [pi x (1/2) height x (1/2) width]. Groups 1 and 2 had lower intracranial ON size than did groups 3, 4, and 5 (P imaging of the ONs with cross-sectional area short child more than 12 months of age, with or without hypothalamic-pituitary axis abnormalities, confirms the clinical diagnosis of ONH.

  12. Recessive mutations in SLC38A8 cause foveal hypoplasia and optic nerve misrouting without albinism.

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    Poulter, James A; Al-Araimi, Musallam; Conte, Ivan; van Genderen, Maria M; Sheridan, Eamonn; Carr, Ian M; Parry, David A; Shires, Mike; Carrella, Sabrina; Bradbury, John; Khan, Kamron; Lakeman, Phillis; Sergouniotis, Panagiotis I; Webster, Andrew R; Moore, Anthony T; Pal, Bishwanath; Mohamed, Moin D; Venkataramana, Anandula; Ramprasad, Vedam; Shetty, Rohit; Saktivel, Murugan; Kumaramanickavel, Govindasamy; Tan, Alex; Mackey, David A; Hewitt, Alex W; Banfi, Sandro; Ali, Manir; Inglehearn, Chris F; Toomes, Carmel

    2013-12-05

    Foveal hypoplasia and optic nerve misrouting are developmental defects of the visual pathway and only co-occur in connection with albinism; to date, they have only been associated with defects in the melanin-biosynthesis pathway. Here, we report that these defects can occur independently of albinism in people with recessive mutations in the putative glutamine transporter gene SLC38A8. Nine different mutations were identified in seven Asian and European families. Using morpholino-mediated ablation of Slc38a8 in medaka fish, we confirmed that pigmentation is unaffected by loss of SLC38A8. Furthermore, by undertaking an association study with SNPs at the SLC38A8 locus, we showed that common variants within this gene modestly affect foveal thickness in the general population. This study reveals a melanin-independent component underpinning the development of the visual pathway that requires a functional role for SLC38A8. Copyright © 2013 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  13. ALDH1A3 loss of function causes bilateral anophthalmia/microphthalmia and hypoplasia of the optic nerve and optic chiasm.

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    Yahyavi, Mani; Abouzeid, Hana; Gawdat, Ghada; de Preux, Anne-Sophie; Xiao, Tong; Bardakjian, Tanya; Schneider, Adele; Choi, Alex; Jorgenson, Eric; Baier, Herwig; El Sada, Mohamad; Schorderet, Daniel F; Slavotinek, Anne M

    2013-08-15

    The major active retinoid, all-trans retinoic acid, has long been recognized as critical for the development of several organs, including the eye. Mutations in STRA6, the gene encoding the cellular receptor for vitamin A, in patients with Matthew-Wood syndrome and anophthalmia/microphthalmia (A/M), have previously demonstrated the importance of retinol metabolism in human eye disease. We used homozygosity mapping combined with next-generation sequencing to interrogate patients with anophthalmia and microphthalmia for new causative genes. We used whole-exome and whole-genome sequencing to study a family with two affected brothers with bilateral A/M and a simplex case with bilateral anophthalmia and hypoplasia of the optic nerve and optic chiasm. Analysis of novel sequence variants revealed homozygosity for two nonsense mutations in ALDH1A3, c.568A>G, predicting p.Lys190*, in the familial cases, and c.1165A>T, predicting p.Lys389*, in the simplex case. Both mutations predict nonsense-mediated decay and complete loss of function. We performed antisense morpholino (MO) studies in Danio rerio to characterize the developmental effects of loss of Aldh1a3 function. MO-injected larvae showed a significant reduction in eye size, and aberrant axonal projections to the tectum were noted. We conclude that ALDH1A3 loss of function causes anophthalmia and aberrant eye development in humans and in animal model systems.

  14. Serum prolactin concentrations in relation to hypopituitarism and obesity in children with optic nerve hypoplasia.

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    Vedin, Amy M; Garcia-Filion, Pamela; Fink, Cassandra; Borchert, Mark; Geffner, Mitchell E

    2012-01-01

    The majority of children with optic nerve hypoplasia (ONH) develop hypopituitarism and many also become obese. These associated conditions are a major cause of morbidity and are possibly due to hypothalamic dysfunction. Because mild hyperprolactinemia often occurs in subjects with disorders of the hypothalamus, we examined whether hyperprolactinemia was present in children with ONH during the first 3 years of life and whether it was a marker for hypopituitarism and/or obesity. Data were retrospectively analyzed from a registry study of children with ONH. The initial serum prolactin was obtained prior to age 36 months (n = 125) and compared with pituitary function and body mass index at age 5. 72% of subjects had an elevated initial serum prolactin and 60% had hypopituitarism. An elevated initial prolactin was associated with hypopituitarism (OR 2.58; 95% CI 1.16, 5.73), specifically with growth hormone deficiency (OR 2.77; 95% CI 1.21, 6.34). 31% of subjects had a body mass index ≥ 85th percentile, but this did not correlate with initial hyperprolactinemia. Early hyperprolactinemia correlates with the presence of hypopituitarism in children with ONH, but it is not a reliable prognosticator of hypopituitarism. Additionally, hyperprolactinemia does not predict future weight excess. Copyright © 2012 S. Karger AG, Basel.

  15. Septo-optic dysplasia with pachygyria

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    Abhay A Lune

    2014-01-01

    Full Text Available Optic nerve hypoplasia can be associated with other central nervous system malformations. When associated with the absence of the septum pellucidum or thinning of corpus callosum and pituitary hypoplasia, it is termed septo-optic dysplasia. Pachygyria is a rare congenital disorder characterized by a defect in migration of cerebral neurons resulting in failure of cortical gyri to develop.We report a case of a young learning-disabled male presenting with decreased vision, nystagmus, esotropia,, and seizures since childhood. Fundoscopy revealed bilateral optic nerve hypoplasia. Growth hormone levels were reduced. Magnetic resonance imaging revealed thinned optic nerves, optic chiasma and pituitary infundibulum, absent septum pellucidum, box-shaped ventricles and right frontal cortical thickening with few and broad gyri.This case report highlights a rare case of septo-optic dysplasia with pachygyria. It also emphasizes that every patient with optic nerve hypoplasia should have an endocrinal and neurological evaluation and neuro-radiographic imaging for associated abnormalities.

  16. Comparison of Heidelberg Retina Tomograph with disc-macula distance to disc diameter ratio in diagnosing optic nerve hypoplasia.

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    Pang, Yi; Frantz, Kelly A

    2016-05-01

    To evaluate whether Heidelberg Retinal Tomograph (HRT) is a valid test for diagnosing congenital optic nerve hypoplasia (CONH) compared to the ratio of the distance between the centre of the optic disc and the centre of the macula and the mean optic disc diameter (DM:DD ratio). Furthermore, to determine the optimal cut-off value of HRT disc area to differentiate a hypoplastic disc from a normal optic disc. A total of 33 subjects with CONH (4-67 years old) and 160 normal subjects (5-65 years old) were recruited and underwent comprehensive eye examinations, fundus photography and HRT. Receiver operating characteristic curves for DM:DD ratio and HRT disc area were constructed based on data from the 46 CONH eyes and 160 control eyes. Mean (±S.D.) HRT disc area was 1.94 (±0.54) mm(2) for the control eyes and 0.84 (±0.35) mm(2) for the CONH eyes (p < 0.0001). The area under the curve (AUC) for DM:DD ratio was 0.83 (95% confidence interval: 0.76-0.90). The AUC for HRT disc area was 0.96 (95% confidence interval: 0.94-0.99). A statistically significant difference was found between AUC for HRT disc area and that for DM:DD ratio (p = 0.0004). The optimal cut-off value for HRT disc area was 1.42 mm(2) with 95% sensitivity and 85% specificity. The optimal cut-off value for DM:DD ratio was 3.20 with 78% sensitivity and 78% specificity. Both HRT and the DM:DD ratio are valid tests to aid diagnosis of CONH. HRT is superior to DM:DD ratio in diagnosing CONH with higher sensitivity and specificity. We suggest the optimal cut-off value for HRT disc area as 1.42 mm(2) in order to discriminate a hypoplastic disc from a normal optic disc. © 2016 The Authors Ophthalmic & Physiological Optics © 2016 The College of Optometrists.

  17. Improved imaging of cochlear nerve hypoplasia using a 3-Tesla variable flip-angle turbo spin-echo sequence and a 7-cm surface coil.

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    Giesemann, Anja M; Raab, Peter; Lyutenski, Stefan; Dettmer, Sabine; Bültmann, Eva; Frömke, Cornelia; Lenarz, Thomas; Lanfermann, Heinrich; Goetz, Friedrich

    2014-03-01

    Magnetic resonance imaging of the temporal bone has an important role in decision making with regard to cochlea implantation, especially in children with cochlear nerve deficiency. The purpose of this study was to evaluate the usefulness of the combination of an advanced high-resolution T2-weighted sequence with a surface coil in a 3-Tesla magnetic resonance imaging scanner in cases of suspected cochlear nerve aplasia. Prospective study. Seven patients with cochlear nerve hypoplasia or aplasia were prospectively examined using a high-resolution three-dimensional variable flip-angle turbo spin-echo sequence using a surface coil, and the images were compared with the same sequence in standard resolution using a standard head coil. Three neuroradiologists evaluated the magnetic resonance images independently, rating the visibility of the nerves in diagnosing hypoplasia or aplasia. Eight ears in seven patients with hypoplasia or aplasia of the cochlear nerve were examined. The average age was 2.7 years (range, 9 months-5 years). Seven ears had accompanying malformations. The inter-rater reliability in diagnosing hypoplasia or aplasia was greater using the high-resolution three-dimensional variable flip-angle turbo spin-echo sequence (fixed-marginal kappa: 0.64) than with the same sequence in lower resolution (fixed-marginal kappa: 0.06). Examining cases of suspected cochlear nerve aplasia using the high-resolution three-dimensional variable flip-angle turbo spin-echo sequence in combination with a surface coil shows significant improvement over standard methods. © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

  18. Optic nerve oxygenation

    DEFF Research Database (Denmark)

    Stefánsson, Einar; Pedersen, Daniella Bach; Jensen, Peter Koch

    2005-01-01

    The oxygen tension of the optic nerve is regulated by the intraocular pressure and systemic blood pressure, the resistance in the blood vessels and oxygen consumption of the tissue. The oxygen tension is autoregulated and moderate changes in intraocular pressure or blood pressure do not affect...... the optic nerve oxygen tension. If the intraocular pressure is increased above 40 mmHg or the ocular perfusion pressure decreased below 50 mmHg the autoregulation is overwhelmed and the optic nerve becomes hypoxic. A disturbance in oxidative metabolism in the cytochromes of the optic nerve can be seen...... at similar levels of perfusion pressure. The levels of perfusion pressure that lead to optic nerve hypoxia in the laboratory correspond remarkably well to the levels that increase the risk of glaucomatous optic nerve atrophy in human glaucoma patients. The risk for progressive optic nerve atrophy in human...

  19. Optic Nerve Pit

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    ... Conditions Frequently Asked Questions Español Condiciones Chinese Conditions Optic Nerve Pit What is optic nerve pit? An optic nerve pit is a ... may be seen in both eyes. How is optic pit diagnosed? If the pit is not affecting ...

  20. [Glaucoma and optic nerve drusen: Limitations of optic nerve head OCT].

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    Poli, M; Colange, J; Goutagny, B; Sellem, E

    2017-09-01

    Optic nerve head drusen are congenital calcium deposits located in the prelaminar section of the optic nerve head. Their association with visual field defects has been classically described, but the diagnosis of glaucoma is not easy in these cases of altered optic nerve head anatomy. We describe the case of a 67-year-old man with optic nerve head drusen complicated by glaucoma, which was confirmed by visual field and OCT examination of the peripapillary retinal nerve fiber layer (RNFL), but the measurement of the minimum distance between the Bruch membrane opening and the internal limiting membrane (minimum rim width, BMO-MRW) by OCT was normal. OCT of the BMO-MRW is a new diagnostic tool for glaucoma. Superficial optic nerve head drusen, which are found between the internal limiting membrane and the Bruch's membrane opening, overestimate the value of this parameter. BMO-MRW measurement is not adapted to cases of optic nerve head drusen and can cause false-negative results for this parameter, and the diagnosis of glaucoma in this case should be based on other parameters such as the presence of a fascicular defect in the retinal nerve fibers, RNFL or macular ganglion cell complex thinning, as well as visual field data. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  1. Optic Nerve Disorders

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    The optic nerve is a bundle of more than 1 million nerve fibers that carry visual messages. You have one connecting ... retina) to your brain. Damage to an optic nerve can cause vision loss. The type of vision ...

  2. Tumors of the optic nerve

    DEFF Research Database (Denmark)

    Lindegaard, Jens; Heegaard, Steffen

    2009-01-01

    A variety of lesions may involve the optic nerve. Mainly, these lesions are inflammatory or vascular lesions that rarely necessitate surgery but may induce significant visual morbidity. Orbital tumors may induce proptosis, visual loss, relative afferent pupillary defect, disc edema and optic...... atrophy, but less than one-tenth of these tumors are confined to the optic nerve or its sheaths. No signs or symptoms are pathognomonic for tumors of the optic nerve. The tumors of the optic nerve may originate from the optic nerve itself (primary tumors) as a proliferation of cells normally present...... in the nerve (e.g., astrocytes and meningothelial cells). The optic nerve may also be invaded from tumors originating elsewhere (secondary tumors), invading the nerve from adjacent structures (e.g., choroidal melanoma and retinoblastoma) or from distant sites (e.g., lymphocytic infiltration and distant...

  3. Optic Nerve Imaging

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    ... News About Us Donate In This Section Optic Nerve Imaging email Send this article to a friend ... measurements of nerve fiber damage (or loss). The Nerve Fiber Analyzer (GDx) uses laser light to measure ...

  4. Optic nerve invasion of uveal melanoma

    DEFF Research Database (Denmark)

    Lindegaard, Jens; Isager, Peter; Prause, Jan Ulrik

    2007-01-01

    in Denmark between 1942 and 2001 were reviewed (n=157). Histopathological characteristics and depth of optic nerve invasion were recorded. The material was compared with a control material from the same period consisting of 85 cases randomly drawn from all choroidal/ciliary body melanomas without optic nerve...... juxtapapillary tumors invading the optic nerve because of simple proximity to the nerve. A neurotropic subtype invades the optic nerve and retina in a diffuse fashion unrelated to tumor size or location. Udgivelsesdato: 2007-Jan...

  5. Magnetic resonance imaging of optic nerve

    International Nuclear Information System (INIS)

    Gala, Foram

    2015-01-01

    Optic nerves are the second pair of cranial nerves and are unique as they represent an extension of the central nervous system. Apart from clinical and ophthalmoscopic evaluation, imaging, especially magnetic resonance imaging (MRI), plays an important role in the complete evaluation of optic nerve and the entire visual pathway. In this pictorial essay, the authors describe segmental anatomy of the optic nerve and review the imaging findings of various conditions affecting the optic nerves. MRI allows excellent depiction of the intricate anatomy of optic nerves due to its excellent soft tissue contrast without exposure to ionizing radiation, better delineation of the entire visual pathway, and accurate evaluation of associated intracranial pathologies

  6. Regeneration of Optic Nerve

    Directory of Open Access Journals (Sweden)

    Kwok-Fai So

    2011-05-01

    Full Text Available The optic nerve is part of the central nervous system (CNS and has a structure similar to other CNS tracts. The axons that form the optic nerve originate in the ganglion cell layer of the retina and extend through the optic tract. As a tissue, the optic nerve has the same organization as the white matter of the brain in regard to its glia. There are three types of glial cells: Oligodendrocytes, astrocytes, and microglia. Little structural and functional regeneration of the CNS takes place spontaneously following injury in adult mammals. In contrast, the ability of the mammalian peripheral nervous system (PNS to regenerate axons after injury is well documented. A number of factors are involved in the lack of CNS regeneration, including: (i the response of neuronal cell bodies against the damage; (ii myelin-mediated inhibition by oligodendrocytes; (iii glial scarring, by astrocytes; (iv macrophage infiltration; and (v insufficient trophic factor support. The fundamental difference in the regenerative capacity between CNS and PNS neuronal cell bodies has been the subject of intensive research. In the CNS the target normally conveys a retrograde trophic signal to the cell body. CNS neurons die because of trophic deprivation. Damage to the optic nerve disconnects the neuronal cell body from its target-derived trophic peptides, leading to the death of retinal ganglion cells. Furthermore, the axontomized neurons become less responsive to the peptide trophic signals they do receive. On the other hand, adult PNS neurons are intrinsically responsive to neurotrophic factors and do not lose trophic responsiveness after axotomy. In this talk different strategies to promote optic-nerve regeneration in adult mammals are reviewed. Much work is still needed to resolve many issues. This is a very important area of neuroregeneration and neuroprotection, as currently there is no cure after traumatic optic nerve injury or retinal disease such as glaucoma, which

  7. Retrobulbar diameter of optic nerve in glaucoma

    Directory of Open Access Journals (Sweden)

    Stefanović Ivan

    2009-01-01

    Full Text Available Introduction. The ultrasound diagnostics of the optic nerve includes the analysis of the optic nerve disc (PNO and measuring of its retrobulbar diameter. With B-scan, by Schraeder's method, it is possible to measure very precisely the optic nerve, the pial diameter, the normal values for the pial diameter being 2.8-4.1 mm. In glaucoma, the disease that is most frequently associated with higher intraocular pressure, there comes the destruction of nerve fibres, which can be visualized as the excavation of the optic nerve disc. Objective. In this paper, we were interested in finding whether in glaucoma, and in what phase of the disease, the optic nerve starts growing thinner. Aware of many forms of this very complex disease, we were interested in knowing if the visualization of excavation on the optic nerve disc is related to diminishing of the pial diameter of the retrobulbar nerve part. Methods. There were treated the patients who had already had the diagnosis of glaucoma and the visualized excavation of the optic disc of various dimensions. Echographically, there was measured the thickness of the retrobulbar part of the optic nerve and the finding compared in relation to the excavation of the optic disc. Results. In all eyes with glaucoma, a normal size of the retrobulbar part of the optic nerve was measured, ranging from 3.01 to 3.91 mm with the median of 3.36 mm. Also, by testing the correlation between the thickness of the optic nerve and the excavation of the PNO, by Pearson test, we found that there was no correlation between these two parameters (r=0.109; p>0.05. Conclusion. In the patients with glaucoma, the retrobulbar part of the optic nerve is not thinner (it has normal values, even not in the cases with a totally excavated optic disc. There is no connection between the size of the PNO excavation and the thickness of the retrobulbar part of the optic nerve.

  8. Size of the optic nerve in computed tomography

    International Nuclear Information System (INIS)

    Asanagi, Kaoru; Shigemori, Hiroichi; Sunabori, Shozo; Nakamura, Yasuhisa.

    1980-01-01

    Recently, the measurement of optic nerve diameter from CT images has become of great interest. For measuring the optic nerve diameter, the method of Neuro-Ocular index is advocated by Magadure, 1978. But it is very difficult to support this method, because no relationship exists between the ocular diameter and the optic nerve diameter. In order to measure the optic nerve diameter directly by CT image, we examined several Window Level and Window Width settings and print out tables. Results are as follows, 1) Width 400 and all Level settings, all optic nerves appear thick. 2) Width 100, 75, 50 and Level 0 settings show optic nerves thin. 3) Optic nerve looks thick by Width of 100, 75, 50 and Level of -50 settings. 4) By the Level set of CT value of optic nerve in each case and Width set 75 or 50, optic images show nearly the correct diameter. 5) The midpoint of CT value of optic nerve obtained from print out tables are 8 to -22 and the the average is -10. (author)

  9. Isolated optic nerve pseudotumour

    International Nuclear Information System (INIS)

    Patankar, T.; Prasad, S.; Krishnan, A.; Laxminarayan, R.

    2000-01-01

    Isolated optic nerve involvement by the idiopathic inflammatory process is a rare finding and very few reports are available. Here a case of an isolated optic nerve inflammatory pseudotumour presenting with gradually progressive unilateral loss of vision is described. It showed dramatic response to a trial of steroids and its differential diagnoses are discussed. Copyright (1999) Blackwell Science Pty Ltd

  10. Transnasal Endoscopic Optic Nerve Decompression in Post Traumatic Optic Neuropathy.

    Science.gov (United States)

    Gupta, Devang; Gadodia, Monica

    2018-03-01

    To quantify the successful outcome in patients following optic nerve decompression in post traumatic unilateral optic neuropathy in form of improvement in visual acuity. A prospective study was carried out over a period of 5 years (January 2011 to June 2016) at civil hospital Ahmedabad. Total 20 patients were selected with optic neuropathy including patients with direct and indirect trauma to unilateral optic nerve, not responding to conservative management, leading to optic neuropathy and subsequent impairment in vision and blindness. Decompression was done via Transnasal-Ethmo-sphenoidal route and outcome was assessed in form of post-operative visual acuity improvement at 1 month, 6 months and 1 year follow up. After surgical decompression complete recovery of visual acuity was achieved in 16 (80%) patients and partial recovery in 4 (20%). Endoscopic transnasal approach is beneficial in traumatic optic neuropathy not responding to steroid therapy and can prevent permanent disability if earlier intervention is done prior to irreversible damage to the nerve. Endoscopic optic nerve surgery can decompress the traumatic and oedematous optic nerve with proper exposure of orbital apex and optic canal without any major intracranial, intraorbital and transnasal complications.

  11. Relationship Between Optic Nerve Appearance and Retinal Nerve Fiber Layer Thickness as Explored with Spectral Domain Optical Coherence Tomography

    Science.gov (United States)

    Aleman, Tomas S.; Huang, Jiayan; Garrity, Sean T.; Carter, Stuart B.; Aleman, Wendy D.; Ying, Gui-shuang; Tamhankar, Madhura A.

    2014-01-01

    Purpose To study the relationship between the appearance of the optic nerve and the retinal nerve fiber layer (RNFL) thickness determined by spectral domain optical coherence tomography (OCT). Methods Records from patients with spectral domain-OCT imaging in a neuro-ophthalmology practice were reviewed. Eyes with glaucoma/glaucoma suspicion, macular/optic nerve edema, pseudophakia, and with refractive errors > 6D were excluded. Optic nerve appearance by slit lamp biomicroscopy was related to the RNFL thickness by spectral domain-OCT and to visual field results. Results Ninety-one patients (176 eyes; mean age: 49 ± 15 years) were included. Eighty-three eyes (47%) showed optic nerve pallor; 89 eyes (50.6%) showed RNFL thinning (sectoral or average peripapillary). Average peripapillary RNFL thickness in eyes with pallor (mean ± SD = 76 ± 17 μm) was thinner compared to eyes without pallor (91 ± 14 μm, P < 0.001). Optic nerve pallor predicted RNFL thinning with a sensitivity of 69% and a specificity of 75%. Optic nerve appearance predicted RNFL thinning (with a sensitivity and specificity of 81%) when RNFL had thinned by ∼ 40%. Most patients with pallor had RNFL thinning with (66%) or without (25%) visual field loss; the remainder had normal RNFL and fields (5%) or with visual field abnormalities (4%). Conclusions Optic nerve pallor as a predictor of RNFL thinning showed fair sensitivity and specificity, although it is optimally sensitive/specific only when substantial RNFL loss has occurred. Translational Relevance Finding an acceptable relationship between the optic nerve appearance by ophthalmoscopy and spectral domain-OCT RNFL measures will help the clinician's interpretation of the information provided by this technology, which is gaining momentum in neuro-ophthalmic research. PMID:25374773

  12. Optic nerve oxygen tension

    DEFF Research Database (Denmark)

    la Cour, M; Kiilgaard, Jens Folke; Eysteinsson, T

    2000-01-01

    To investigate the influence of acute changes in intraocular pressure on the oxygen tension in the vicinity of the optic nerve head under control conditions and after intravenous administration of 500 mg of the carbonic anhydrase inhibitor dorzolamide.......To investigate the influence of acute changes in intraocular pressure on the oxygen tension in the vicinity of the optic nerve head under control conditions and after intravenous administration of 500 mg of the carbonic anhydrase inhibitor dorzolamide....

  13. High-resolution CT of lesions of the optic nerve

    International Nuclear Information System (INIS)

    Peyster, R.G.; Hoover, E.D.; Hershey, B.L.; Haskin, M.E.

    1983-01-01

    The optic nerves are well demonstrated by high-resolution computed tomography. Involvement of the optic nerve by optic gliomas and optic nerve sheath meningiomas is well known. However, nonneoplastic processes such as increased intracranial pressure, optic neuritis, Grave ophthalmopathy, and orbital pseudotumor may also alter the appearance of the optic nerve/sheath on computed tomography. Certain clinical and computed tomographic features permit distinction of these nonneoplastic tumefactions from tumors

  14. Can preoperative MR imaging predict optic nerve invasion of retinoblastoma?

    International Nuclear Information System (INIS)

    Song, Kyoung Doo; Eo, Hong; Kim, Ji Hye; Yoo, So-Young; Jeon, Tae Yeon

    2012-01-01

    Purpose: To evaluate the accuracy of pre-operative MRI for the detection of optic nerve invasion in retinoblastoma. Materials and methods: Institutional review board approval and informed consent were waived for this retrospective study. A total of 41 patients were included. Inclusion criteria were histologically proven retinoblastoma, availability of diagnostic-quality preoperative MR images acquired during the 4 weeks before surgery, unilateral retinoblastoma, and normal-sized optic nerve. Two radiologists retrospectively reviewed the MR images independently. Five imaging findings (diffuse mild optic nerve enhancement, focal strong optic nerve enhancement, optic sheath enhancement, tumor location, and tumor size) were evaluated against optic nerve invasion of retinoblastoma. The predictive performance of all MR imaging findings for optic nerve invasion was also evaluated by the receiver operating characteristic curve analysis. Results: Optic nerve invasion was histopathologically confirmed in 24% of study population (10/41). The differences in diffuse mild enhancement, focal strong enhancement, optic sheath enhancement, and tumor location between patients with optic nerve invasion and patients without optic nerve invasion were not significant. Tumor sizes were 16.1 mm (SD: 2.2 mm) and 14.9 mm (SD: 3.6 mm) in patients with and without optic nerve involvement, respectively (P = 0.444). P-Values from binary logistic regression indicated that all five imaging findings were not significant predictors of tumor invasion of optic nerve. The AUC values of all MR imaging findings for the prediction of optic nerve invasion were 0.689 (95% confidence interval: 0.499–0.879) and 0.653 (95% confidence interval: 0.445–0.861) for observer 1 and observer 2, respectively. Conclusion: Findings of MRI in patients with normal-sized optic nerves have limited usefulness in preoperatively predicting the presence of optic nerve invasion in retinoblastoma.

  15. Can preoperative MR imaging predict optic nerve invasion of retinoblastoma?

    Energy Technology Data Exchange (ETDEWEB)

    Song, Kyoung Doo, E-mail: kdsong0308@gmail.com [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-Dong, Kangnam-Ku, Seoul 135-710 (Korea, Republic of); Eo, Hong, E-mail: rtombow@gmail.com [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-Dong, Kangnam-Ku, Seoul 135-710 (Korea, Republic of); Kim, Ji Hye, E-mail: jhkate.kim@samsung.com [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-Dong, Kangnam-Ku, Seoul 135-710 (Korea, Republic of); Yoo, So-Young, E-mail: sy1131.yoo@samsung.com [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-Dong, Kangnam-Ku, Seoul 135-710 (Korea, Republic of); Jeon, Tae Yeon, E-mail: hathor97.jeon@samsung.com [Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Ilwon-Dong, Kangnam-Ku, Seoul 135-710 (Korea, Republic of)

    2012-12-15

    Purpose: To evaluate the accuracy of pre-operative MRI for the detection of optic nerve invasion in retinoblastoma. Materials and methods: Institutional review board approval and informed consent were waived for this retrospective study. A total of 41 patients were included. Inclusion criteria were histologically proven retinoblastoma, availability of diagnostic-quality preoperative MR images acquired during the 4 weeks before surgery, unilateral retinoblastoma, and normal-sized optic nerve. Two radiologists retrospectively reviewed the MR images independently. Five imaging findings (diffuse mild optic nerve enhancement, focal strong optic nerve enhancement, optic sheath enhancement, tumor location, and tumor size) were evaluated against optic nerve invasion of retinoblastoma. The predictive performance of all MR imaging findings for optic nerve invasion was also evaluated by the receiver operating characteristic curve analysis. Results: Optic nerve invasion was histopathologically confirmed in 24% of study population (10/41). The differences in diffuse mild enhancement, focal strong enhancement, optic sheath enhancement, and tumor location between patients with optic nerve invasion and patients without optic nerve invasion were not significant. Tumor sizes were 16.1 mm (SD: 2.2 mm) and 14.9 mm (SD: 3.6 mm) in patients with and without optic nerve involvement, respectively (P = 0.444). P-Values from binary logistic regression indicated that all five imaging findings were not significant predictors of tumor invasion of optic nerve. The AUC values of all MR imaging findings for the prediction of optic nerve invasion were 0.689 (95% confidence interval: 0.499–0.879) and 0.653 (95% confidence interval: 0.445–0.861) for observer 1 and observer 2, respectively. Conclusion: Findings of MRI in patients with normal-sized optic nerves have limited usefulness in preoperatively predicting the presence of optic nerve invasion in retinoblastoma.

  16. Cell proliferation and apoptosis in optic nerve and brain integration centers of adult trout Oncorhynchus mykiss after optic nerve injury

    Science.gov (United States)

    Pushchina, Evgeniya V.; Shukla, Sachin; Varaksin, Anatoly A.; Obukhov, Dmitry K.

    2016-01-01

    Fishes have remarkable ability to effectively rebuild the structure of nerve cells and nerve fibers after central nervous system injury. However, the underlying mechanism is poorly understood. In order to address this issue, we investigated the proliferation and apoptosis of cells in contralateral and ipsilateral optic nerves, after stab wound injury to the eye of an adult trout Oncorhynchus mykiss. Heterogenous population of proliferating cells was investigated at 1 week after injury. TUNEL labeling gave a qualitative and quantitative assessment of apoptosis in the cells of optic nerve of trout 2 days after injury. After optic nerve injury, apoptotic response was investigated, and mass patterns of cell migration were found. The maximal concentration of apoptotic bodies was detected in the areas of mass clumps of cells. It is probably indicative of massive cell death in the area of high phagocytic activity of macrophages/microglia. At 1 week after optic nerve injury, we observed nerve cell proliferation in the trout brain integration centers: the cerebellum and the optic tectum. In the optic tectum, proliferating cell nuclear antigen (PCNA)-immunopositive radial glia-like cells were identified. Proliferative activity of nerve cells was detected in the dorsal proliferative (matrix) area of the cerebellum and in parenchymal cells of the molecular and granular layers whereas local clusters of undifferentiated cells which formed neurogenic niches were observed in both the optic tectum and cerebellum after optic nerve injury. In vitro analysis of brain cells of trout showed that suspension cells compared with monolayer cells retain higher proliferative activity, as evidenced by PCNA immunolabeling. Phase contrast observation showed mitosis in individual cells and the formation of neurospheres which gradually increased during 1–4 days of culture. The present findings suggest that trout can be used as a novel model for studying neuronal regeneration. PMID:27212918

  17. Farris-Tang retractor in optic nerve sheath decompression surgery.

    Science.gov (United States)

    Spiegel, Jennifer A; Sokol, Jason A; Whittaker, Thomas J; Bernard, Benjamin; Farris, Bradley K

    2016-01-01

    Our purpose is to introduce the use of the Farris-Tang retractor in optic nerve sheath decompression surgery. The procedure of optic nerve sheath fenestration was reviewed at our tertiary care teaching hospital, including the use of the Farris-Tang retractor. Pseudotumor cerebri is a syndrome of increased intracranial pressure without a clear cause. Surgical treatment can be effective in cases in which medical therapy has failed and disc swelling with visual field loss progresses. Optic nerve sheath decompression surgery (ONDS) involves cutting slits or windows in the optic nerve sheath to allow cerebrospinal fluid to escape, reducing the pressure around the optic nerve. We introduce the Farris-Tang retractor, a retractor that allows for excellent visualization of the optic nerve sheath during this surgery, facilitating the fenestration of the sheath and visualization of the subsequent cerebrospinal fluid egress. Utilizing a medial conjunctival approach, the Farris-Tang retractor allows for easy retraction of the medial orbital tissue and reduces the incidence of orbital fat protrusion through Tenon's capsule. The Farris-Tang retractor allows safe, easy, and effective access to the optic nerve with good visualization in optic nerve sheath decompression surgery. This, in turn, allows for greater surgical efficiency and positive patient outcomes.

  18. Cranial nerve injury after Le Fort I osteotomy.

    Science.gov (United States)

    Kim, J-W; Chin, B-R; Park, H-S; Lee, S-H; Kwon, T-G

    2011-03-01

    A Le Fort I osteotomy is widely used to correct dentofacial deformity because it is a safe and reliable surgical method. Although rare, various complications have been reported in relation to pterygomaxillary separation. Cranial nerve damage is one of the serious complications that can occur after Le Fort I osteotomy. In this report, a 19-year-old man with unilateral cleft lip and palate underwent surgery to correct maxillary hypoplasia, asymmetry and mandibular prognathism. After the Le Fort I maxillary osteotomy, the patient showed multiple cranial nerve damage; an impairment of outward movement of the eye (abducens nerve), decreased vision (optic nerve), and paraesthesia of the frontal and upper cheek area (ophthalmic and maxillary nerve). The damage to the cranial nerve was related to an unexpected sphenoid bone fracture and subsequent trauma in the cavernous sinus during the pterygomaxillary osteotomy. Copyright © 2010 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

  19. Optic Nerve Sheath Mechanics in VIIP Syndrome

    Science.gov (United States)

    Raykin, Julia; Forte, Taylor E.; Wang, Roy; Feola, Andrew; Samuels, Brian; Myers, Jerry; Nelson, Emily; Gleason, Rudy; Ethier, C. Ross

    2016-01-01

    Visual Impairment Intracranial Pressure (VIIP) syndrome is a major concern in current space medicine research. While the exact pathology of VIIP is not yet known, it is hypothesized that the microgravity-induced cephalad fluid shift increases intracranial pressure (ICP) and drives remodeling of the optic nerve sheath. To investigate this possibility, we are culturing optic nerve sheath dura mater samples under different pressures and investigating changes in tissue composition. To interpret results from this work, it is essential to first understand the biomechanical response of the optic nerve sheath dura mater to loading. Here, we investigated the effects of mechanical loading on the porcine optic nerve sheath.Porcine optic nerves (number: 6) were obtained immediately after death from a local abattoir. The optic nerve sheath (dura mater) was isolated from the optic nerve proper, leaving a hollow cylinder of connective tissue that was used for biomechanical characterization. We developed a custom mechanical testing system that allowed for unconfined lengthening, twisting, and circumferential distension of the dura mater during inflation and under fixed axial loading. To determine the effects of variations in ICP, the sample was inflated (0-60 millimeters Hg) and circumferential distension was simultaneously recorded. These tests were performed under variable axial loads (0.6 grams - 5.6 grams at increments of 1 gram) by attaching different weights to one end of the dura mater. Results and Conclusions: The samples demonstrated nonlinear behavior, similar to other soft connective tissue (Figure 1). Large increases in diameter were observed at lower transmural pressures (approximately 0 to 5 millimeters Hg), whereas only small diameter changes were observed at higher pressures. Particularly interesting was the existence of a cross-over point at a pressure of approximately 11 millimeters Hg. At this pressure, the same diameter is obtained for all axial loads applied

  20. On the CT-diagnosis of optic nerve lesions. Differential diagnostic criteria

    International Nuclear Information System (INIS)

    Unsoeld, R.

    1982-01-01

    Computed tomograms of 166 optic nerve lesions were analyzed: 97 were mainly orbital and 69 mainly intracranial. The criteria were clinical course, size, density and delineation of the optic nerve shadow, orbital and cerebral soft tissue abnormalities, and bony changes in the optic canal. Characteristic CT features are described of individual disease entities such as optic gliomas, optic nerve sheath meningiomas, neoplastic and inflammatory infiltrations. The differential diagnostic importance of individual CT criteria is evaluated and discussed. Simultaneous visualization of orbital and intracranial soft tissue changes as well as bony changes in the optic canal allow the location and identification of the majority of optic nerve lesions based on the criteria mentioned above, and optic nerve tumors can be differentiated. In 9 patients with optic neuritis due to clinically proven encephalitis and in 17 patients with total optic atrophy, no changes in the size of the optic nerve could be found. CT evaluation of the intraorbital portion of the optic nerve requires special examination techniques. Oblique computer reformations through the optic canal provide excellent visualization of bony changes in the optic canal. The exclusion of intracranial causes of optic nerve lesions requires intravenous injection of contrast material. (orig.) [de

  1. Effect of diabetic retinopathy and panretinal photocoagulation on retinal nerve fiber layer and optic nerve appearance.

    Science.gov (United States)

    Lim, Michele C; Tanimoto, Suzana A; Furlani, Bruno A; Lum, Brent; Pinto, Luciano M; Eliason, David; Prata, Tiago S; Brandt, James D; Morse, Lawrence S; Park, Susanna S; Melo, Luiz A S

    2009-07-01

    To determine if panretinal photocoagulation (PRP) alters retinal nerve fiber layer (RNFL) thickness and optic nerve appearance. Patients with diabetes who did and did not undergo PRP and nondiabetic control subjects were enrolled in a prospective study. Participants underwent optical coherence tomography of the peripapillary retina and optic nerve. Stereoscopic optic nerve photographs were graded in a masked fashion. Ninety-four eyes of 48 healthy individuals, 89 eyes of 55 diabetic patients who did not undergo PRP, and 37 eyes of 24 subjects with diabetes who underwent PRP were included in this study. Eyes that had been treated with PRP had thinner peripapillary RNFL compared with the other groups; this was statistically significantly different in the inferior (P = .004) and nasal (P = .003) regions. Optic nerve cupping did not increase with severity of disease classification, but the proportion of optic nerves graded as suspicious for glaucoma or as having nonglaucomatous optic neuropathy did (P = .008). These grading categories were associated with thinner RNFL measurements. Diabetic eyes that have been treated with PRP have thinner RNFL than nondiabetic eyes. Optic nerves in eyes treated with PRP are more likely to be graded as abnormal, but their appearance is not necessarily glaucomatous and may be related to thinning of the RNFL.

  2. Neurophysiological intraoperative monitoring during an optic nerve schwannoma removal.

    Science.gov (United States)

    San-Juan, Daniel; Escanio Cortés, Manuel; Tena-Suck, Martha; Orozco Garduño, Adolfo Josué; López Pizano, Jesús Alejandro; Villanueva Domínguez, Jonathan; Fernández Gónzalez-Aragón, Maricarmen; Gómez-Amador, Juan Luis

    2017-10-01

    This paper reports the case of a patient with optic nerve schwannoma and the first use of neurophysiological intraoperative monitoring of visual evoked potentials during the removal of such tumor with no postoperative visual damage. Schwannomas are benign neoplasms of the peripheral nervous system arising from the neural crest-derived Schwann cells, these tumors are rarely located in the optic nerve and the treatment consists on surgical removal leading to high risk of damage to the visual pathway. Case report of a thirty-year-old woman with an optic nerve schwannoma. The patient underwent surgery for tumor removal on the left optic nerve through a left orbitozygomatic approach with intraoperative monitoring of left II and III cranial nerves. We used Nicolet Endeavour CR IOM (Carefusion, Middleton WI, USA) to performed visual evoked potentials stimulating binocularly with LED flash goggles with the patient´s eyes closed and direct epidural optic nerve stimulation delivering rostral to the tumor a rectangular current pulse. At follow up examinations 7 months later, the left eye visual acuity was 20/60; Ishihara score was 8/8 in both eyes; the right eye photomotor reflex was normal and left eye was mydriatic and arreflectic; optokinetic reflex and ocular conjugate movements were normal. In this case, the epidural direct electrical stimulation of optic nerve provided stable waveforms during optic nerve schwannoma resection without visual loss.

  3. Optic Nerve Avulsion after Blunt Trauma

    Directory of Open Access Journals (Sweden)

    Hacı Halil Karabulut

    2014-05-01

    Full Text Available Optic nerve avulsion is an uncommon presentation of ocular trauma with a poor prognosis. It can be seen as complete or partial form due to the form of trauma. We assessed the complete optic nerve avulsion in a 16-year-old female patient complaining of loss of vision in her left eye after a traffic accident. (Turk J Ophthalmol 2014; 44: 249-51

  4. Imaging of the optic nerve

    Energy Technology Data Exchange (ETDEWEB)

    Becker, Minerva [Head and Neck and Maxillofacial Radiology, Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, CH - 1211 Geneva 14 (Switzerland)], E-mail: minerva.becker@hcuge.ch; Masterson, Karen [Head and Neck and Maxillofacial Radiology, Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, CH - 1211 Geneva 14 (Switzerland); Delavelle, Jacqueline [Neuroradiology, Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, CH - 1211 Geneva 14 (Switzerland); Viallon, Magalie [Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, CH - 1211 Geneva 14 (Switzerland); Vargas, Maria-Isabel [Neuroradiology, Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, CH - 1211 Geneva 14 (Switzerland); Becker, Christoph D. [Department of Radiology, Geneva University Hospital, Rue Gabrielle-Perret-Gentil 4, CH - 1211 Geneva 14 (Switzerland)

    2010-05-15

    This article provides an overview of the imaging findings of diseases affecting the optic nerve with special emphasis on clinical-radiological correlation and on the latest technical developments in MR imaging and CT. The review deals with congenital malformations, tumors, toxic/nutritional and degenerative entities, inflammatory and infectious diseases, compressive neuropathy, vascular conditions and trauma involving the optic nerve from its ocular segment to the chiasm. The implications of imaging findings on patient management and outcome and the importance of performing high-resolution tailored examinations adapted to the clinical situation are discussed.

  5. Imaging of the optic nerve

    International Nuclear Information System (INIS)

    Becker, Minerva; Masterson, Karen; Delavelle, Jacqueline; Viallon, Magalie; Vargas, Maria-Isabel; Becker, Christoph D.

    2010-01-01

    This article provides an overview of the imaging findings of diseases affecting the optic nerve with special emphasis on clinical-radiological correlation and on the latest technical developments in MR imaging and CT. The review deals with congenital malformations, tumors, toxic/nutritional and degenerative entities, inflammatory and infectious diseases, compressive neuropathy, vascular conditions and trauma involving the optic nerve from its ocular segment to the chiasm. The implications of imaging findings on patient management and outcome and the importance of performing high-resolution tailored examinations adapted to the clinical situation are discussed.

  6. Establishing a cat model of acute optic nerve injury

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    BACKGROUND: In order to investigate the progress in optic nerve injury and the following regeneration and repair, many kinds of animal models of optic nerve injury have been established, such as models of acute and chronic ocular hypertension, compression, amputating wound, ischemia reperfusion or hypoxia,intravitreal injection of excitatory amino acids, etc. However, most of these models are established by squeezing intraorbital optic nerve, and suitable for ophthalmology, and there are fewer models suitable for the acute cranial contusion in neurosurgery.OBJECTIVE: To observe the changes of optic nerve after acute injury, and the characteristics of methods for establishing model of acute optic nerve injury in cats.DESIGN: A complete randomized grouping and controlled animal trial.SETTING: Department of Neurosurgery, General Hospital of Ji'nan Military Area Command of Chinese PLA.MATERIALS: Twenty-eight healthy adult cats, common degree, either sex, weighing 2.0 - 3.5 kg, were provided by the animal experimental center of Fudan University. The cats were randomly divided into control group (n =3) and model group (n =25), and 5 cats in the model group were observed at 6 hours and 1,3, 7 and 14 days after injury respectively. JX-2000 biological signal processing system (Department of Physiology, Second Military Medical University of Chinese PLA, Shanghai); Inverted phase contrast microscope (Olympus); Axioplan 2 imaging microgram analytical system (Labsystems).METHODS: The experiments were carried out in the Department of Neurosurgery, General Hospital of Jinan Military Area Command of Chinese PLA from June 2004 to June 2005. The cats in the model groups were made into models of acute optic nerve injury: The cats were anesthetized, then the limbs were fixed in a lateral recumbent position. Pterion approach in human was imitated, the operative incision was made along the line between lateral canthus and tragus, and it could be seen deep along the skull base that white

  7. Optic Nerve Assessment Using 7-Tesla Magnetic Resonance Imaging.

    Science.gov (United States)

    Singh, Arun D; Platt, Sean M; Lystad, Lisa; Lowe, Mark; Oh, Sehong; Jones, Stephen E; Alzahrani, Yahya; Plesec, Thomas

    2016-04-01

    The purpose of this study was to correlate high-resolution magnetic resonance imaging (MRI) and histologic findings in a case of juxtapapillary choroidal melanoma with clinical evidence of optic nerve invasion. With institutional review board approval, an enucleated globe with choroidal melanoma and optic nerve invasion was imaged using a 7-tesla MRI followed by histopathologic evaluation. Optical coherence tomography, B-scan ultrasonography, and 1.5-tesla MRI of the orbit (1-mm sections) could not detect optic disc invasion. Ex vivo, 7-tesla MRI detected optic nerve invasion, which correlated with histopathologic features. Our case demonstrates the potential to document the existence of optic nerve invasion in the presence of an intraocular tumor, a feature that has a major bearing on decision making, particularly for consideration of enucleation.

  8. Peri-optic nerve infiltration during leukaemic relapse: MRI diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Madani, A.; Christophe, C. [Department of Imaging, Hopital Universitaire des Enfants Reine Fabiola, Brussels (Belgium); Ferster, A.; Dan, B. [Department of Paediatrics, Hopital Universitaire des Enfants Reine Fabiola, Brussels (Belgium)

    2000-01-01

    Background. A 10-year-old boy with a history of acute lymphoblastic leukaemia (ALL), but without previous evidence of central nervous system involvement, presented with seizures 3 years after complete remission. Materials and methods. MRI showed bilateral enlargement of the optic nerves despite normal ophthalmological examination. Results. Only the third cerebrospinal fluid examination showed 2 % blasts without concomitant bone-marrow infiltration. Enlargement of the optic nerves was consistent with bilateral leukaemic peri-optic nerve infiltration. The appearances returned to normal after chemotherapy. Conclusion. The optic nerves are a potential site of relapse in patients with systemic and meningeal ALL, even in the absence of ophthalmological signs. (orig.)

  9. MRI of the optic nerve in benign intracranial hypertension

    International Nuclear Information System (INIS)

    Gass, A.; Barker, G.J.; Riordan-Eva, P.; MacManus, D.; Sanders, M.; Tofts, P.S.; McDonald, W.I.; Moseley, I.F.; Miller, D.H.

    1996-01-01

    We investigated the MRI appearance of the optic nerve and its cerebrospinal-fluid-containing sheath in 17 patients with benign intracranial hypertension (BIH) and 15 normal controls. Using phased-array local coils, 3-mm coronal T2-weighted fat-suppressed fast spin-echo images were obtained with an in-plane resolution of < 0.39 mm. The optic nerve and its sheath were clearly differentiated. An enlarged, elongated subarachnoid space around the optic nerve was demonstrated in patients with BIH. High-resolution MRI of the optic nerve offers additional information which may be of value for diagnosis and in planning and monitoring treatment. (orig.). With 5 figs

  10. Glaucoma severity affects diffusion tensor imaging (DTI) parameters of the optic nerve and optic radiation

    Energy Technology Data Exchange (ETDEWEB)

    Sidek, S. [Department of Biomedical Imaging, University Malaya, Research Imaging Centre, Faculty of Medicine, University Malaya (Malaysia); Medical Imaging Unit, Faculty of Medicine, Universiti Teknologi MARA, Selangor (Malaysia); Ramli, N. [Department of Biomedical Imaging, University Malaya, Research Imaging Centre, Faculty of Medicine, University Malaya (Malaysia); Rahmat, K., E-mail: katt_xr2000@yahoo.com [Department of Biomedical Imaging, University Malaya, Research Imaging Centre, Faculty of Medicine, University Malaya (Malaysia); Ramli, N.M.; Abdulrahman, F. [Department of Ophthalmology, Faculty of Medicine, University Malaya, Kuala Lumpur (Malaysia); Tan, L.K. [Department of Biomedical Imaging, University Malaya, Research Imaging Centre, Faculty of Medicine, University Malaya (Malaysia)

    2014-08-15

    Objectives: To evaluate whether MR diffusion tensor imaging (DTI) of the optic nerve and optic radiation in glaucoma patients provides parameters to discriminate between mild and severe glaucoma and to determine whether DTI derived indices correlate with retinal nerve fibre layer (RNFL) thickness. Methods: 3-Tesla DTI was performed on 90 subjects (30 normal, 30 mild glaucoma and 30 severe glaucoma subjects) and the FA and MD of the optic nerve and optic radiation were measured. The categorisation into mild and severe glaucoma was done using the Hodapp–Parrish–Anderson (HPA) classification. RNFL thickness was also assessed on all subjects using OCT. Receiver operating characteristic (ROC) analysis and Spearman's correlation coefficient was carried out. Results: FA and MD values in the optic nerve and optic radiation decreased and increased respectively as the disease progressed. FA at the optic nerve had the highest sensitivity (87%) and specificity (80%). FA values displayed the strongest correlation with RNFL thickness in the optic nerve (r = 0.684, p ≤ 0.001) while MD at the optic radiation showed the weakest correlation with RNFL thickness (r = −0.360, p ≤ 0.001). Conclusions: The high sensitivity and specificity of DTI-derived FA values in the optic nerve and the strong correlation between DTI-FA and RNFL thickness suggest that these parameters could serve as indicators of disease severity.

  11. Glaucoma severity affects diffusion tensor imaging (DTI) parameters of the optic nerve and optic radiation

    International Nuclear Information System (INIS)

    Sidek, S.; Ramli, N.; Rahmat, K.; Ramli, N.M.; Abdulrahman, F.; Tan, L.K.

    2014-01-01

    Objectives: To evaluate whether MR diffusion tensor imaging (DTI) of the optic nerve and optic radiation in glaucoma patients provides parameters to discriminate between mild and severe glaucoma and to determine whether DTI derived indices correlate with retinal nerve fibre layer (RNFL) thickness. Methods: 3-Tesla DTI was performed on 90 subjects (30 normal, 30 mild glaucoma and 30 severe glaucoma subjects) and the FA and MD of the optic nerve and optic radiation were measured. The categorisation into mild and severe glaucoma was done using the Hodapp–Parrish–Anderson (HPA) classification. RNFL thickness was also assessed on all subjects using OCT. Receiver operating characteristic (ROC) analysis and Spearman's correlation coefficient was carried out. Results: FA and MD values in the optic nerve and optic radiation decreased and increased respectively as the disease progressed. FA at the optic nerve had the highest sensitivity (87%) and specificity (80%). FA values displayed the strongest correlation with RNFL thickness in the optic nerve (r = 0.684, p ≤ 0.001) while MD at the optic radiation showed the weakest correlation with RNFL thickness (r = −0.360, p ≤ 0.001). Conclusions: The high sensitivity and specificity of DTI-derived FA values in the optic nerve and the strong correlation between DTI-FA and RNFL thickness suggest that these parameters could serve as indicators of disease severity

  12. Magnetic resonance imaging in optic nerve lesions with multiple sclerosis

    International Nuclear Information System (INIS)

    Kojima, Shigeyuki; Hirayama, Keizo; Kakisu, Yonetsugu; Adachi, Emiko

    1990-01-01

    Magnetic resonance imaging (MRI) of the optic nerve was performed in 10 patients with multiple sclerosis (MS) using short inversion time inversion recovery (STIR) pulse sequences, and the results were compared with the visual evoked potentials (VEP). The 10 patients had optic neuritis in the chronic or remitting phase together with additional symptoms or signs allowing a diagnosis of clinically definite or probable MS. Sixteen optic nerves were clinically affected and 4 were unaffected. MRI was performed using a 0.5 tesla supeconducting unit, and multiple continuous 5 mm coronal and axial STIR images were obtained. A lesion was judged to be present if a focal or diffuse area of increased signal intensity was detectd in the optic nerve. In VEP, a delay in peak latency or no P 100 component was judged to be abnormal. With regard to the clinically affected optic nerves, MRI revealed a region of increased signal intensity in 14/16 (88%) and the VEP was abnormal in 16/16 (100%). In the clinically unaffected optic nerves, MRI revealed an increased signal intensity in 2/4 (50%). One of these nerves had an abnormal VEP and the other had a VEP latency at the upper limit of normal. The VEP was abnormal in 1/4 (25%). In the clinically affected optic nerves, the degree of loss of visual acuity was not associated with the longitudinal extent of the lesions shown by MRI. The mean length was 17.5 mm in optic nerves with a slight disturbance of visual acuity and 15.0 mm in nerves with severe visual loss. MRI using STIR pulse sequences was found to be almost as sensitive as VEP in detecting both clinically affected and unaffected optic nerve lesions in patients with MS, and was useful in visualizing the location or size of the lesions. (author)

  13. Optic nerve pH and PO2

    DEFF Research Database (Denmark)

    Pedersen, Daniella B; Stefánsson, Einar; Kiilgaard, Jens Folke

    2006-01-01

    Earlier studies have demonstrated that carbonic anhydrase inhibitors (CAIs) increase optic nerve oxygen tension (ONPO(2)) in pigs. We hypothesized that the mechanism of this effect was either a CO(2) increase or a pH decrease in tissue and blood. To test this hypothesis we investigated and compared...... how optic nerve pH (ONpH) and ONPO(2) are affected by: (1) carbonic anhydrase inhibition; (2) respiratory acidosis, and (3) metabolic acidosis. We measured ONpH with a glass pH electrode and ONPO(2) with a polarographic oxygen electrode. One of the electrodes was placed in the vitreous cavity 0.5 mm...... over the optic nerve in the eyes of domestic pigs....

  14. High resolution ultrasound and magnetic resonance imaging of the optic nerve and the optic nerve sheath: anatomic correlation and clinical importance.

    Science.gov (United States)

    Steinborn, M; Fiegler, J; Kraus, V; Denne, C; Hapfelmeier, A; Wurzinger, L; Hahn, H

    2011-12-01

    We performed a cadaver study to evaluate the accuracy of measurements of the optic nerve and the optic nerve sheath for high resolution US (HRUS) and magnetic resonance imaging (MRI). Five Thiel-fixated cadaver specimens of the optic nerve were examined with HRUS and MRI. Measurements of the optic nerve and the ONSD were performed before and after the filling of the optic nerve sheath with saline solution. Statistical analysis included the calculation of the agreement of measurements and the evaluation of the intraobserver and interobserver variation. Overall a good correlation of measurement values between HRUS and MRI can be found (mean difference: 0.02-0.97 mm). The repeatability coefficient (RC) and concordance correlation coefficient (CCC) values were good to excellent for most acquisitions (RC 0.2-1.11 mm; CCC 0.684-0.949). The highest variation of measurement values was found for transbulbar sonography (RC 0.58-1.83 mm; CCC 0.615/0.608). If decisive anatomic structures are clearly depicted and the measuring points are set correctly, there is a good correlation between HRUS and MRI measurements of the optic nerve and the ONSD even on transbulbar sonography. As most of the standard and cut-off values that have been published for ultrasound are significantly lower than the results obtained with MRI, a reevaluation of sonographic ONSD measurement with correlation to MRI is necessary. © Georg Thieme Verlag KG Stuttgart · New York.

  15. Microgravity-Driven Optic Nerve/Sheath Biomechanics Simulations

    Science.gov (United States)

    Ethier, C. R.; Feola, A.; Myers, J. G.; Nelson, E.; Raykin, J.; Samuels, B.

    2016-01-01

    Visual Impairment and Intracranial Pressure (VIIP) syndrome is a concern for long-duration space flight. Current thinking suggests that the ocular changes observed in VIIP syndrome are related to cephalad fluid shifts resulting in altered fluid pressures [1]. In particular, we hypothesize that increased intracranial pressure (ICP) drives connective tissue remodeling of the posterior eye and optic nerve sheath (ONS). We describe here finite element (FE) modeling designed to understand how altered pressures, particularly altered ICP, affect the tissues of the posterior eye and optic nerve sheath (ONS) in VIIP. METHODS: Additional description of the modeling methodology is provided in the companion IWS abstract by Feola et al. In brief, a geometric model of the posterior eye and optic nerve, including the ONS, was created and the effects of fluid pressures on tissue deformations were simulated. We considered three ICP scenarios: an elevated ICP assumed to occur in chronic microgravity, and ICP in the upright and supine positions on earth. Within each scenario we used Latin hypercube sampling (LHS) to consider a range of ICPs, ONH tissue mechanical properties, intraocular pressures (IOPs) and mean arterial pressures (MAPs). The outcome measures were biomechanical strains in the lamina cribrosa, optic nerve and retina; here we focus on peak values of these strains, since elevated strain alters cell phenotype and induce tissue remodeling. In 3D, the strain field can be decomposed into three orthogonal components, denoted as first, second and third principal strains. RESULTS AND CONCLUSIONS: For baseline material properties, increasing ICP from 0 to 20 mmHg significantly changed strains within the posterior eye and ONS (Fig. 1), indicating that elevated ICP affects ocular tissue biomechanics. Notably, strains in the lamina cribrosa and retina became less extreme as ICP increased; however, within the optic nerve, the occurrence of such extreme strains greatly increased as

  16. Age-related ultrastructural and monoamine oxidase changes in the rat optic nerve.

    Science.gov (United States)

    Taurone, S; Ripandelli, G; Minni, A; Lattanzi, R; Miglietta, S; Pepe, N; Fumagalli, L; Micera, A; Pastore, F S; Artico, M

    2016-01-01

    The aim of this paper is to study the morphology and the distribution of the monoamine oxidase enzymatic system in the optic nerve of 4 month-old Wistar (young) and 28 month-old Wistar (old) rats. The optic nerve was harvested from 20 young and old rats. The segment of optic nerve was divided longitudinally into two pieces, each 0.1 mm in length. The first piece was used for transmission electron microscopy. The second piece was stained with histochemical reaction for monoamine oxidase. The agerelated changes in the optic nerve of rats include micro-anatomical details, ultrastructure and monoamine oxidase histochemical staining. A strong decrease of the thin nerve fibers and a swelling of the thick ones can be observed in optic nerve fibers of old rats. Increased monoamine oxidase histochemical staining of the optic nerve of aged rats is well demonstrated. The increase of meningeal shealth and the decrease of thin nerve fibers of the optic nerve in old rats are well documented. Morphological, ultrastructural and histochemical changes observed in optic nerve fibers of the old rats show a close relation with aging.

  17. Congenital Unilateral Hypoplasia of Depressor Anguli Oris

    Directory of Open Access Journals (Sweden)

    Seckin O. Ulualp

    2012-01-01

    Full Text Available Objectives. Asymmetric facial appearance may originate from abnormalities of facial musculature or facial innervation. We describe clinical features of congenital hypoplasia of depressor anguli oris muscle in a child. Material and Methods. Chart of a 10-month-old female referred to a tertiary care pediatric hospital for assessment of facial paralysis was reviewed. Data included relevant history and physical examination, diagnostic work up, and management. Results. The child presented with asymmetric movement of lower lip since birth. Asymmetry of lower lip was more pronounced when she smiled and cried. Rest of the face movement was symmetric. On examination, the face appeared symmetric at rest. The child had inward deviation of right lower lip when she smiled. Facial nerve function, as determined by frowning/forehead, wrinkling, eye closure, nasolabial fold depth, and tearing, was symmetric. Magnetic resonance imaging of the temporal bones and internal auditory canals were within normal limits. Echocardiogram did not show cardiac abnormality. Auditory brainstem response showed no abnormality. Conclusions. Congenital hypoplasia of depressor anguli oris is a rare anomaly that causes asymmetric crying face. Pediatricians and otolaryngologists need to be cognizant of cardiac, head and neck, and central nervous system anomalies associated with congenital unilateral hypoplasia of depressor anguli oris.

  18. Anatomical relationship between the optic nerve and posterior paranasal sinuses on ostiomeatal unit CT

    International Nuclear Information System (INIS)

    Cho, June Il; Kim, Hong In; Seol, Hae Young; Lee, Nam Joon; Kim, Jung Hyuk; Cha, In Ho

    1997-01-01

    To determine the anatomic variations that can lead to optic nerve damage during the sugical treatment of posterior paranasal sinus lesions two hundred optic nerves of 100 persons were examined using ostiomeatal unit CT(OMU CT). The anatomical features of this nerve and posterior paranasal sinuses were classified into four types:the optic nerve adjacent to the sphenoid sinus without indentation of the sinus wall (type 1);the optic nerve adjacent to the sphenoid sinus, causing indentation of the sinus wall (type 2);the optic nerve passing through the sphenoid sinus (type 3);and the optic nerve adjacent to the sphenoid sinus and posterior ethmoid sinus (type 4). Bony dehiscence around the optic nerve and pneumatization of the anterior clinoid process were also evaluated. The anatomical classification of the optic nerve and posterior paranasal sinuses was as follows:type 1, 1326(66%); type 2, 60(30%); type 3, 6(3%), and type 4, 2(1%). Bony dehiscence around the optic nerve had developed in 58 cases (29%) and pneumatization of the anterior clinoid process in 13(6.5%). These conditions were most common in type 3 optic nerve, and second most common in type 2. The 2 and 3 optic nerves, bony dehiscence around the optic nerve and pneumatization of the anterior clinoid process are the anatomic variations that can lead to optic nerve damage during the surgical treatment of posterior paranasal sinus lesions. To prevent optic nerve damage, these factors should be carefully evaluated by OMU CT

  19. Pathology of the vestibulocochlear nerve

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    De Foer, Bert [Department of Radiology, Sint-Augustinus Hospital, Oosterveldlaan 24, 2610 Wilrijk (Belgium)], E-mail: bert.defoer@GZA.be; Kenis, Christoph [Department of Radiology, Sint-Augustinus Hospital, Oosterveldlaan 24, 2610 Wilrijk (Belgium)], E-mail: christophkenis@hotmail.com; Van Melkebeke, Deborah [Department of Neurology, Sint-Augustinus Hospital, Oosterveldlaan 24, 2610 Wilrijk (Belgium)], E-mail: Deborah.vanmelkebeke@Ugent.be; Vercruysse, Jean-Philippe [University Department of ENT, Sint-Augustinus Hospital, Oosterveldlaan 24, 2610 Wilrijk (Belgium)], E-mail: jphver@yahoo.com; Somers, Thomas [University Department of ENT, Sint-Augustinus Hospital, Oosterveldlaan 24, 2610 Wilrijk (Belgium)], E-mail: Thomas.somers@GZA.be; Pouillon, Marc [Department of Radiology, Sint-Augustinus Hospital, Oosterveldlaan 24, 2610 Wilrijk (Belgium)], E-mail: marc.pouillon@GZA.be; Offeciers, Erwin [University Department of ENT, Sint-Augustinus Hospital, Oosterveldlaan 24, 2610 Wilrijk (Belgium)], E-mail: Erwin.offeciers@GZA.be; Casselman, Jan W. [Department of Radiology, AZ Sint-Jan AV Hospital, Ruddershove 10, Bruges (Belgium); Consultant Radiologist, Sint-Augustinus Hospital, Oosterveldlaan 24, 2610 Wilrijk (Belgium); Academic Consultent, University of Ghent (Belgium)], E-mail: jan.casselman@azbrugge.be

    2010-05-15

    There is a large scala of pathology affecting the vestibulocochlear nerve. Magnetic resonance imaging is the method of choice for the investigation of pathology of the vestibulocochlear nerve. Congenital pathology mainly consists of agenesis or hypoplasia of the vestibulocochlear nerve. Tumoral pathology affecting the vestibulocochlear nerve is most frequently located in the internal auditory canal or cerebellopontine angle. Schwannoma of the vestibulocochlear nerve is the most frequently found tumoral lesion followed by meningeoma, arachnoid cyst and epidermoid cyst. The most frequently encountered pathologies as well as some more rare entities are discussed in this chapter.

  20. Pathology of the vestibulocochlear nerve

    International Nuclear Information System (INIS)

    De Foer, Bert; Kenis, Christoph; Van Melkebeke, Deborah; Vercruysse, Jean-Philippe; Somers, Thomas; Pouillon, Marc; Offeciers, Erwin; Casselman, Jan W.

    2010-01-01

    There is a large scala of pathology affecting the vestibulocochlear nerve. Magnetic resonance imaging is the method of choice for the investigation of pathology of the vestibulocochlear nerve. Congenital pathology mainly consists of agenesis or hypoplasia of the vestibulocochlear nerve. Tumoral pathology affecting the vestibulocochlear nerve is most frequently located in the internal auditory canal or cerebellopontine angle. Schwannoma of the vestibulocochlear nerve is the most frequently found tumoral lesion followed by meningeoma, arachnoid cyst and epidermoid cyst. The most frequently encountered pathologies as well as some more rare entities are discussed in this chapter.

  1. Acute optic nerve sheath fenestration with the free-electron laser

    Science.gov (United States)

    Shen, Jin-Hui; Casagrande, Vivien A.; Joos, Karen M.; Shetlar, Debra J.; Robinson, Richard D.; Head, William S.; Mavity-Hudson, Julia A.; Nunnally, Amy H.

    1999-06-01

    Purpose: To determine if the free electron laser (FEL) energy can be delivered to a small space to perform optic nerve sheath fenestration with minimal acute nerve damage. Methods: A 530 mm hollow waveguide probe was designed. Optic nerve sheath fenestration (1.0 mm diameter) was performed in 8 rabbits using either the FEL (4 eyes, 6.45mm, 10 Hz, 2 mJ) or a knife (4 eyes). Within 2 hours following surgery, the animals were perfused with aldehyde fixative. The integrity of the optic nerve and glial response at the site of fenestration were evaluated on tissue selections with H&E, and antibodies to S100β or GFAP. Results: Surgery using the FEL probe was found to be technically superior to the knife. The glial reaction was limited to a zone adjacent to the fenestration and was similar in both the FEL and knife incisions. Conclusions: The FEL appears capable of efficiently performing an optic nerve sheath fenestration in a small space with minimal acute damage. Both the FEL and knife incisions result in a rapid glial response at the site of fenestration even when optic nerve integrity is not compromised.

  2. Supraorbital keyhole surgery for optic nerve decompression and dura repair.

    Science.gov (United States)

    Chen, Yuan-Hao; Lin, Shinn-Zong; Chiang, Yung-Hsiao; Ju, Da-Tong; Liu, Ming-Ying; Chen, Guann-Juh

    2004-07-01

    Supraorbital keyhole surgery is a limited surgical procedure with reduced traumatic manipulation of tissue and entailing little time in the opening and closing of wounds. We utilized the approach to treat head injury patients complicated with optic nerve compression and cerebrospinal fluid leakage (CSF). Eleven cases of basal skull fracture complicated with either optic nerve compression and/or CSF leakage were surgically treated at our department from February 1995 to June 1999. Six cases had primary optic nerve compression, four had CSF leakage and one case involved both injuries. Supraorbital craniotomy was carried out using a keyhole-sized burr hole plus a small craniotomy. The size of craniotomy approximated 2 x 3 cm2. The optic nerve was decompressed via removal of the optic canal roof and anterior clinoid process with high-speed drills. The defect of dura was repaired with two pieces of tensa fascia lata that were attached on both sides of the torn dural defect with tissue glue. Seven cases with optic nerve injury included five cases of total blindness and two cases of light perception before operation. Vision improved in four cases. The CSF leakage was stopped successfully in all four cases without complication. As optic nerve compression and CSF leakage are skull base lesions, the supraorbital keyhole surgery constitutes a suitable approach. The supraorbital keyhole surgery allows for an anterior approach to the skull base. This approach also allows the treatment of both CSF leakage and optic nerve compression. Our results indicate that supraorbital keyhole operation is a safe and effective method for preserving or improving vision and attenuating CSF leakage following injury.

  3. Optic nerve oxygen tension

    DEFF Research Database (Denmark)

    Kiilgaard, Jens Folke; Pedersen, D B; Eysteinsson, T

    2004-01-01

    The authors have previously reported that carbonic anhydrase inhibitors such as acetazolamide and dorzolamide raise optic nerve oxygen tension (ONPO(2)) in pigs. The purpose of the present study was to investigate whether timolol, which belongs to another group of glaucoma drugs called beta...

  4. A rare case of bilateral optic nerve sheath meningioma

    Directory of Open Access Journals (Sweden)

    Somen Misra

    2014-01-01

    Full Text Available A 60-year-old female presented with gradual, painless, progressive diminution of vision, and progressive proptosis of left eye since 7 years. Ophthalmological examination revealed mild proptosis and total optic atrophy in the left eye. Magnetic resonance imaging (MRI and computed tomography (CT brain with orbit showed bilateral optic nerve sheath meningioma (ONSM involving the intracranial, intracanalicular, intraorbital part of the optic nerve extending up to optic chiasma and left cavernous sinus.

  5. Optical stimulation of peripheral nerves in vivo

    Science.gov (United States)

    Wells, Jonathon D.

    This dissertation documents the emergence and validation of a new clinical tool that bridges the fields of biomedical optics and neuroscience. The research herein describes an innovative method for direct neurostimulation with pulsed infrared laser light. Safety and effectiveness of this technique are first demonstrated through functional stimulation of the rat sciatic nerve in vivo. The Holmium:YAG laser (lambda = 2.12 mum) is shown to operate at an optimal wavelength for peripheral nerve stimulation with advantages over standard electrical neural stimulation; including contact-free stimulation, high spatial selectivity, and lack of a stimulation artifact. The underlying biophysical mechanism responsible for transient optical nerve stimulation appears to be a small, absorption driven thermal gradient sustained at the axonal layer of nerve. Results explicitly prove that low frequency optical stimulation can reliably stimulate without resulting in tissue thermal damage. Based on the positive results from animal studies, these optimal laser parameters were utilized to move this research into the clinic with a combined safety and efficacy study in human subjects undergoing selective dorsal rhizotomy. The clinical Holmium:YAG laser was used to effectively stimulate human dorsal spinal roots and elicit functional muscle responses recorded during surgery without evidence of nerve damage. Overall these results predict that this technology can be a valuable clinical tool in various neurosurgical applications.

  6. Optic Nerve Head and Retinal Nerve Fiber Layer Differences Between Caribbean Black and African American Patients as Measured by Spectral Domain OCT.

    Science.gov (United States)

    Rao, Rohini; Dhrami-Gavazi, Elona; Al-Aswad, Lama; Ciarleglio, Adam; Cioffi, George A; Blumberg, Dana M

    2015-01-01

    There are well-established differences in optic nerve morphology between patients of African and European descent. Spectral domain optical coherence tomography (OCT) scanning has demonstrated these differences with respect to optic disc area (DA), average cup-disc ratio, cup volume, and nerve fiber layer thickness. However, the term "African descent" describes a heterogenous group with considerable variability. This study evaluates differences in optic nerve and retinal nerve fiber layer (RNFL) parameters as measured by Cirrus HD-OCT between Caribbean black and African American patients. A total of 25 African American subjects and 25 Caribbean black subjects with normal ocular examinations were consecutively recruited to this study. All patients received imaging of the optic nerve and nerve fiber layer with Cirrus HD-OCT. Optic nerve and RNFL parameters were evaluated for statistically significant differences using a t test. A mixed effect model for correlated data was then created to adjust outcome variables for (1) repeated measures and (2) optic nerve size. Two one-sided t tests were then utilized to determine equivalence. After adjustment for DA, RNFL thickness, cup volume, DA, inferior nerve fiber layer, and vertical cup-disc ratio demonstrated statistically significant equivalence between the 2 groups (P value fiber layer quadrant was significantly different between the 2 groups and may merit further investigation. Findings of this study suggest that optic nerve and RNFL morphology is markedly similar between Caribbean blacks and African Americans once adjusted for optic nerve size but cannot be considered equivalent in all measures, particularly in the superior nerve fiber layer.

  7. Assessment of nerve ultrastructure by fibre-optic confocal microscopy.

    Science.gov (United States)

    Cushway, T R; Lanzetta, M; Cox, G; Trickett, R; Owen, E R

    1996-01-01

    Fibre-optic technology combined with confocality produces a microscope capable of optical thin sectioning. In this original study, tibial nerves have been stained in a rat model with a vital dye, 4-(4-diethylaminostyryl)-N-methylpyridinium iodide, and analysed by fibre-optic confocal microscopy to produce detailed images of nerve ultrastructure. Schwann cells, nodes of Ranvier and longitudinal myelinated sheaths enclosing axons were clearly visible. Single axons appeared as brightly staining longitudinal structures. This allowed easy tracing of multiple signal axons within the nerve tissue. An accurate measurement of internodal lengths was easily accomplished. This technique is comparable to current histological techniques, but does not require biopsy, thin sectioning or tissue fixing. This study offers a standard for further in vivo microscopy, including the possibility of monitoring the progression of nerve regeneration following microsurgical neurorraphy.

  8. Radiotherapeutic management of optic nerve gliomas in children

    International Nuclear Information System (INIS)

    Danoff, B.F.; Kramer, S.; Thompson, N.

    1980-01-01

    Optic nerve gliomas represent one to five percent of all intracranial tumors in children. The management of these tumors remains controversial. From 1956 to 1977, 18 children with optic nerve gliomas were treated at Thomas Jefferson University Hospital using external beam radiotherapy. All children presented with decreased visual acuity and five of eighteen were blind in one eye. No patient was found to have involvement of a single optic nerve. in eight patients, the chiasm was involved, in ten patients, tumor had extended to the frontal lobes and/or hypothalamus. Initial surgical management included biopsy only in seven patients, inspection of tumor in two patients and partial excision in seven patients. Two patients were treated with radiotherapy based on radiological findings. A tumor dose of 5000 to 6000 rad was given in 5.5 to 6.5 weeks. Stabilization of visual impairment or improvement in vision was noted in 78 percent of patients who were evaluable. The ten year survival was 73 percent. Radiological evidence of tumor regression will be presented. It is our impression that radiotherapy is indicated in the treatment of children with optic nerve gliomas who have poor prognostic signs

  9. Cerebellar Hypoplasia

    Science.gov (United States)

    ... hypoplasia is a feature of a number of congenital (present at birth) malformation syndromes, such as Walker-Warburg syndrome (a form ... hypoplasia is a feature of a number of congenital (present at birth) malformation syndromes, such as Walker-Warburg syndrome (a form ...

  10. Evolution of optic nerve photography for glaucoma screening: a review.

    Science.gov (United States)

    Myers, Jonathan S; Fudemberg, Scott J; Lee, Daniel

    2018-03-01

    Visual evaluation of the optic nerve has been one of the earliest and most widely used methods to evaluate patients for glaucoma. Photography has proven very useful for documentation of the nerve's appearance at a given time, allowing more detailed scrutiny then, and later comparison for change. Photography serves as the basis for real-time or non-simultaneous review in telemedicine and screening events allowing fundus and optic nerve evaluation by experts elsewhere. Expert evaluation of disc photographs has shown diagnostic performance similar to other methods of optic nerve evaluation for glaucoma. Newer technology has made optic nerve photography simpler, cheaper and more portable creating opportunities for broader utilization in screening in underserved populations by non-physicians. Recent investigations suggest that non-physicians or software algorithms for disc photograph evaluation have promise to allow more screening to be done with fewer experts. © 2017 Royal Australian and New Zealand College of Ophthalmologists.

  11. Astrocytes as gate-keepers in optic nerve regeneration--a mini-review.

    Science.gov (United States)

    García, Dana M; Koke, Joseph R

    2009-02-01

    Animals that develop without extra-embryonic membranes (anamniotes--fish, amphibians) have impressive regenerative capacity, even to the extent of replacing entire limbs. In contrast, animals that develop within extra-embryonic membranes (amniotes--reptiles, birds, mammals) have limited capacity for regeneration as adults, particularly in the central nervous system (CNS). Much is known about the process of nerve development in fish and mammals and about regeneration after lesions in the CNS in fish and mammals. Because the retina of the eye and optic nerve are functionally part of the brain and are accessible in fish, frogs, and mice, optic nerve lesion and regeneration (ONR) has been extensively used as a model system for study of CNS nerve regeneration. When the optic nerve of a mouse is severed, the axons leading into the brain degenerate. Initially, the cut end of the axons on the proximal, eye-side of the injury sprout neurites which begin to grow into the lesion. Simultaneously, astrocytes of the optic nerve become activated to initiate wound repair as a first step in reestablishing the structural integrity of the optic nerve. This activation appears to initiate a cascade of molecular signals resulting in apoptotic cell death of the retinal ganglion cells axons of which make up the neural component of the optic nerve; regeneration fails and the injury is permanent. Evidence specifically implicating astrocytes comes from studies showing selective poisoning of astrocytes at the optic nerve lesion, along with activation of a gene whose product blocks apoptosis in retinal ganglion cells, creates conditions favorable to neurites sprouting from the cut proximal stump, growing through the lesion and into the distal portion of the injured nerve, eventually reaching appropriate targets in the brain. In anamniotes, astrocytes ostensibly present no such obstacle since optic nerve regeneration occurs without intervention; however, no systematic study of glial involvement

  12. [Gradient-index (GRIN) endoscopic examinations from the inner structures of the optic nerve meninges].

    Science.gov (United States)

    Sens, Frank Michael; Killer, Hanspeter Esriel; Meyer, Peter

    2003-03-01

    Due to the excellent image quality and the small outer diameter of the GRIN-(gradient index) endoscope tips we were able to examine the subdural and the subarachnoidal space of the optic nerve meninges by endoscopy. This examination was performed to obtain more information about the inner structure of the optic nerve meninges. In this post-mortem study 7 optic nerves were examined from the chiasm to the globe by GRIN endoscopy (Volpi, Schlieren, Switzerland), with an outer diameter of 0.89 mm, integrated optic of 0.5 mm diameter and an integrated fluid channel of 0.2 mm diameter. In all cases the endoscopic examination of the optic nerve meninges was technically easy to perform. It was possible to study the inner surface of the nerve sheaths and the nerve sheath spaces in close-up. We found horizontal and vertical cords on the inner surface of the dura mater, which could tighten by movements of the optic nerve. With a gradient-index (GRIN) endoscope we obtained new information about the inner structure of the optic nerve meninges. New theories about the changes of the optic nerve meninges during movements of the optic nerve may evolve from this study. Further studies with this new method should be encouraged.

  13. The optic nerve head in glaucoma

    Directory of Open Access Journals (Sweden)

    Rupert RA Bourne

    2006-09-01

    Full Text Available ll types of glaucoma involve glaucomatous optic neuropathy. The key to detection and management of glaucoma is understanding how to examine the optic nerve head (ONH. This pictorial glossary addresses the following issues: how to examine the ONH; normal characteristics of the ONH; characteristics of a glaucomatous ONH; how to tell if the glaucomatous optic neuropathy is getting worse;‘pitfalls and pearls’.

  14. High Spatial Resolution Imaging Mass Spectrometry of Human Optic Nerve Lipids and Proteins

    Science.gov (United States)

    Anderson, David M. G.; Spraggins, Jeffrey M.; Rose, Kristie L.; Schey, Kevin L.

    2015-06-01

    The human optic nerve carries signals from the retina to the visual cortex of the brain. Each optic nerve is comprised of approximately one million nerve fibers that are organized into bundles of 800-1200 fibers surrounded by connective tissue and supportive glial cells. Damage to the optic nerve contributes to a number of blinding diseases including: glaucoma, neuromyelitis optica, optic neuritis, and neurofibromatosis; however, the molecular mechanisms of optic nerve damage and death are incompletely understood. Herein we present high spatial resolution MALDI imaging mass spectrometry (IMS) analysis of lipids and proteins to define the molecular anatomy of the human optic nerve. The localization of a number of lipids was observed in discrete anatomical regions corresponding to myelinated and unmyelinated nerve regions as well as to supporting connective tissue, glial cells, and blood vessels. A protein fragment from vimentin, a known intermediate filament marker for astrocytes, was observed surrounding nerved fiber bundles in the lamina cribrosa region. S100B was also found in supporting glial cell regions in the prelaminar region, and the hemoglobin alpha subunit was observed in blood vessel areas. The molecular anatomy of the optic nerve defined by MALDI IMS provides a firm foundation to study biochemical changes in blinding human diseases.

  15. Characterizing Intraorbital Optic Nerve Changes on Diffusion Tensor Imaging in Thyroid Eye Disease Before Dysthyroid Optic Neuropathy.

    Science.gov (United States)

    Lee, Hwa; Lee, Young Hen; Suh, Sang-Il; Jeong, Eun-Kee; Baek, Sehyun; Seo, Hyung Suk

    The aim of this study was to determine whether the optic nerve is affected by thyroid eye disease (TED) before the development of dysthyroid optic neuropathy with diffusion-tensor imaging (DTI). Twenty TED patients and 20 controls were included. The mean, axial, and radial diffusivities and fractional anisotropy (FA) value were measured at the optic nerves in DTI. Extraocular muscle diameters were measured on computed tomography. The diffusivities and FA of the optic nerves were compared between TED and controls and between active and inactive stages of TED. The correlations between these DTI parameters and the clinical features were determined. The mean, axial, and radial diffusivities were lower in TED compared with the controls (P optic nerve before dysthyroid optic neuropathy in TED. The FA, in particular, reflected TED activity and severity.

  16. An Optic Nerve Crush Injury Murine Model to Study Retinal Ganglion Cell Survival

    Science.gov (United States)

    Tang, Zhongshu; Zhang, Shuihua; Lee, Chunsik; Kumar, Anil; Arjunan, Pachiappan; Li, Yang; Zhang, Fan; Li, Xuri

    2011-01-01

    Injury to the optic nerve can lead to axonal degeneration, followed by a gradual death of retinal ganglion cells (RGCs), which results in irreversible vision loss. Examples of such diseases in human include traumatic optic neuropathy and optic nerve degeneration in glaucoma. It is characterized by typical changes in the optic nerve head, progressive optic nerve degeneration, and loss of retinal ganglion cells, if uncontrolled, leading to vision loss and blindness. The optic nerve crush (ONC) injury mouse model is an important experimental disease model for traumatic optic neuropathy, glaucoma, etc. In this model, the crush injury to the optic nerve leads to gradual retinal ganglion cells apoptosis. This disease model can be used to study the general processes and mechanisms of neuronal death and survival, which is essential for the development of therapeutic measures. In addition, pharmacological and molecular approaches can be used in this model to identify and test potential therapeutic reagents to treat different types of optic neuropathy. Here, we provide a step by step demonstration of (I) Baseline retrograde labeling of retinal ganglion cells (RGCs) at day 1, (II) Optic nerve crush injury at day 4, (III) Harvest the retinae and analyze RGC survival at day 11, and (IV) Representative result. PMID:21540827

  17. Optic Nerve Hemangioblastoma: A Case Report

    Directory of Open Access Journals (Sweden)

    Holly Zywicke

    2012-01-01

    Full Text Available Hemangioblastomas are World Health Organization (WHO grade I tumors of uncertain histologic origin. These central nervous system tumors are most often found in the posterior fossa, brainstem, and spinal cord. There are fewer than 20 reported cases of optic nerve hemangioblastomas in the literature. We present a patient with visual decline found to have a mass arising from within the posterior orbital canal that grossly involved the optic nerve sheath. Neuropathologic evaluation showed hemangioblastoma. Although not a common tumor in this location, consideration of hemangioblastoma in the differential diagnosis is important as they can have a more aggressive course than other tumors of this region and have a detrimental effect on visual prognosis.

  18. Ischial hypoplasia, tibial hypoplasia and facial abnormalities: a new syndrome?

    International Nuclear Information System (INIS)

    Nishimura, G.; Haga, Yoshihiko; Aoki, Katsuhiko; Hasegawa, Tomoko

    1998-01-01

    A child with facial abnormalities, short stature and a variety of skeletal alterations is reported. The facial abnormalities comprised low-set ears, short nose with a long philtrum, micrognathia and cleft palate. The skeletal alterations included ischial hypoplasia, malformations of the cervical spine, hypoplasia of the lesser trochanters, tibial hypoplasia with bowing of the lower legs, tibio-fibular diastasis with malformed distal tibial epiphyses, clubfeet and brachymesophalangy. The constellation of clinical and radiological findings in the present patient do not fit any known malformation syndrome. (orig.)

  19. Ischial hypoplasia, tibial hypoplasia and facial abnormalities: a new syndrome?

    Energy Technology Data Exchange (ETDEWEB)

    Nishimura, G. [Department of Radiology, Dokkyo University School of Medicine (Japan); Haga, Yoshihiko [Department of Orthopaedics, Shizuoka Children`s Hospital, Shizuoka (Japan); Aoki, Katsuhiko [Department of Radiology, Shizuoka Children`s Hospital, Shizuoka (Japan); Hasegawa, Tomoko [Division of Clinical Genetics, Shizuoka Children`s Hospital, Shizuoka (Japan)

    1998-12-01

    A child with facial abnormalities, short stature and a variety of skeletal alterations is reported. The facial abnormalities comprised low-set ears, short nose with a long philtrum, micrognathia and cleft palate. The skeletal alterations included ischial hypoplasia, malformations of the cervical spine, hypoplasia of the lesser trochanters, tibial hypoplasia with bowing of the lower legs, tibio-fibular diastasis with malformed distal tibial epiphyses, clubfeet and brachymesophalangy. The constellation of clinical and radiological findings in the present patient do not fit any known malformation syndrome. (orig.) With 4 figs., 8 refs.

  20. Optic Nerve Injury in a Patient with Chronic Allergic Conjunctivitis

    Directory of Open Access Journals (Sweden)

    Ribhi Hazin

    2014-01-01

    Full Text Available Manipulation of the optic nerve can lead to irreversible vision changes. We present a patient with a past medical history of skin allergy and allergic conjunctivitis (AC who presented with insidious unexplained unilateral vision loss. Physical exam revealed significant blepharospasm, mild lid edema, bulbar conjunctival hyperemia, afferent pupillary defect, and slight papillary hypertrophy. Slit lamp examination demonstrated superior and inferior conjunctival scarring as well as superior corneal scarring but no signs of external trauma or neurological damage were noted. Conjunctival cultures and cytologic evaluation demonstrated significant eosinophilic infiltration. Subsequent ophthalmoscopic examination revealed optic nerve atrophy. Upon further questioning, the patient admitted to vigorous itching of the affected eye for many months. Given the presenting symptoms, history, and negative ophthalmological workup, it was determined that the optic nerve atrophy was likely secondary to digital pressure from vigorous itching. Although AC can be a significant source of decreased vision via corneal ulceration, no reported cases have ever described AC-induced vision loss of this degree from vigorous itching and chronic pressure leading to optic nerve damage. Despite being self-limiting in nature, allergic conjunctivitis should be properly managed as extreme cases can result in mechanical compression of the optic nerve and compromise vision.

  1. Morphometric analysis of the fascicular organisation of the optic nerve

    Directory of Open Access Journals (Sweden)

    Radunović Miroslav

    2015-01-01

    Full Text Available Background/Aim. The optic nerve is anatomically observed in four segments: intrabulbar, orbital, canalicular, and cranial. According to the literature, the surface of the transversal cut of the nerve is different through it. The aim of this study was to evaluate the fascicular organisation of the optic nerve, throughout its three segments from the eye. Methods. Five pairs of optic nerves, obtained from the autopsies were examined. Using Heidenhain's (azan staining, the cuts were prepared for microscopy. Morphometric analysis was performed using the stereological methods for morphometric cytology - the Weible’s testing system M42. The following measures were established: the surface of the transverse cut of the nerve, the entire surface of fasciculi, the entire surface of connective tissue and blood vessels, the number of fasciculi, the surface of a single fasciculus. Results. The surface of the transverse cut of the nerve was found to grow from the orbital to the cranial segment, as well as the entire surface of fasciculi. While their number is significantly lower in the cranial segment, the number of fasciculi varied slightly between the orbital and the canalicular segment. The surface of a single fasciculus grows from the bulb to the chiasma. There is probable a cause to believe that this may be due to fusion of the “small” fasciculi in the orbitocranial direction. Conclusion. There are significant differences among the examined parameters of the different parts of the optic nerve.

  2. Optical stimulation of the facial nerve: a surgical tool?

    Science.gov (United States)

    Richter, Claus-Peter; Teudt, Ingo Ulrik; Nevel, Adam E.; Izzo, Agnella D.; Walsh, Joseph T., Jr.

    2008-02-01

    One sequela of skull base surgery is the iatrogenic damage to cranial nerves. Devices that stimulate nerves with electric current can assist in the nerve identification. Contemporary devices have two main limitations: (1) the physical contact of the stimulating electrode and (2) the spread of the current through the tissue. In contrast to electrical stimulation, pulsed infrared optical radiation can be used to safely and selectively stimulate neural tissue. Stimulation and screening of the nerve is possible without making physical contact. The gerbil facial nerve was irradiated with 250-μs-long pulses of 2.12 μm radiation delivered via a 600-μm-diameter optical fiber at a repetition rate of 2 Hz. Muscle action potentials were recorded with intradermal electrodes. Nerve samples were examined for possible tissue damage. Eight facial nerves were stimulated with radiant exposures between 0.71-1.77 J/cm2, resulting in compound muscle action potentials (CmAPs) that were simultaneously measured at the m. orbicularis oculi, m. levator nasolabialis, and m. orbicularis oris. Resulting CmAP amplitudes were 0.3-0.4 mV, 0.15-1.4 mV and 0.3-2.3 mV, respectively, depending on the radial location of the optical fiber and the radiant exposure. Individual nerve branches were also stimulated, resulting in CmAP amplitudes between 0.2 and 1.6 mV. Histology revealed tissue damage at radiant exposures of 2.2 J/cm2, but no apparent damage at radiant exposures of 2.0 J/cm2.

  3. Parallel changes in structural and functional measures of optic nerve myelination after optic neuritis.

    Directory of Open Access Journals (Sweden)

    Anneke van der Walt

    Full Text Available Visual evoked potential (VEP latency prolongation and optic nerve lesion length after acute optic neuritis (ON corresponds to the degree of demyelination, while subsequent recovery of latency may represent optic nerve remyelination. We aimed to investigate the relationship between multifocal VEP (mfVEP latency and optic nerve lesion length after acute ON.Thirty acute ON patients were studied at 1, 3, 6 and 12 months using mfVEP and at 1 and 12 months with optic nerve MRI. LogMAR and low contrast visual acuity were documented. By one month, the mfVEP amplitude had recovered sufficiently for latency to be measured in 23 (76.7% patients with seven patients having no recordable mfVEP in more than 66% of segments in at least one test. Only data from these 23 patients was analysed further.Both latency and lesion length showed significant recovery during the follow-up period. Lesion length and mfVEP latency were highly correlated at 1 (r = 0.94, p = <0.0001 and 12 months (r = 0.75, p < 0.001. Both measures demonstrated a similar trend of recovery. Speed of latency recovery was faster in the early follow-up period while lesion length shortening remained relatively constant. At 1 month, latency delay was worse by 1.76 ms for additional 1mm of lesion length while at 12 months, 1mm of lesion length accounted for 1.94 ms of latency delay.A strong association between two putative measures of demyelination in early and chronic ON was found. Parallel recovery of both measures could reflect optic nerve remyelination.

  4. Optic nerve pH and PO2

    DEFF Research Database (Denmark)

    Pedersen, Daniella B; Stefánsson, Einar; Kiilgaard, Jens Folke

    2006-01-01

    Earlier studies have demonstrated that carbonic anhydrase inhibitors (CAIs) increase optic nerve oxygen tension (ONPO(2)) in pigs. We hypothesized that the mechanism of this effect was either a CO(2) increase or a pH decrease in tissue and blood. To test this hypothesis we investigated and compared...... how optic nerve pH (ONpH) and ONPO(2) are affected by: (1) carbonic anhydrase inhibition; (2) respiratory acidosis, and (3) metabolic acidosis. We measured ONpH with a glass pH electrode and ONPO(2) with a polarographic oxygen electrode. One of the electrodes was placed in the vitreous cavity 0.5 mm...

  5. Optic Nerve Sheath Meningocele: A Case Report

    Science.gov (United States)

    Halimi, E.; Wavreille, O.; Rosenberg, R.; Bouacha, I.; Lejeune, J.-P.; Defoort-Dhellemmes, S.

    2013-01-01

    Abstract Isolated optic nerve sheath meningocele is a rare affection defined as the cystic enlargement of the optic nerve sheath filled with cerebrospinal fluid. We report the case of a 39-year-old woman presenting with bilateral meningocele uncovered during a routine examination for headache complaints. A 5-year follow-up validated the lesion’s clinical and imaging stability. Magnetic resonance imaging (MRI) is an essential tool in the diagnosis of this pathology, alongside characteristic symptoms indicating that the meningocele might have progressively expanded into the orbit. In this case we present a therapeutic approach based on pathophysiological hypotheses and review of the literature. PMID:28163760

  6. Multiple dental anomalies accompany unilateral disturbances in abducens and facial nerves: A case report

    Directory of Open Access Journals (Sweden)

    Elham Talatahari

    2016-01-01

    Full Text Available This article describes the oral rehabilitation of an 8-year-old girl with extensively affected primary and permanent dentition. This report is unique in which distinct dental anomalies including enamel hypoplasia, irregular dentin formation, taurodontism, hpodontia and dens in dente accompany unilateral disturbance of abducens and facial nerves which control the lateral eye movement, and facial expression, respectively.   Keywords: enamel hypoplasia; irregular dentin formation; taurodontism; hypodontia; dens in dente; abducens and facial nerves;

  7. Computed tomography of the optic nerve with special reference to the attenuation values

    International Nuclear Information System (INIS)

    Mimura, Osamu; Shimooku, Masashi; Sakamoto, Kiyoshi; Sakamoto, Taeko

    1980-01-01

    The value of computed tomography (CT) for ophthalmology needs no longer to be emphasized. Everything, however, has its limitations. In this article, the limitations of CT for the diagnosis of the optic nerve diameter were studied in an orbit-optic nerve model. The difference of 15 Hounsfield units between two scans which demonstrated the optic nerve having almost the same diameter, as a result of the partial volume effect, was obtained. This suggested that the attenuation values of the optic nerve are markedly influenced by the partial volume effect, and that they require further investigation for clinical use. (author)

  8. Genetics Home Reference: pontocerebellar hypoplasia

    Science.gov (United States)

    ... PCH1 also have very weak muscle tone (hypotonia), joint deformities called contractures, vision impairment, and breathing and ... InfoSearch: Pontocerebellar hypoplasia type 6 MalaCards: pontocerebellar hypoplasia Neuromuscular Disease Center, Washington University, St. Louis: Pontocerebellar Hypoplasia ...

  9. Size of the intracranial optic nerve and optic tract in neonates at term-equivalent age at magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Oyama, Jun; Mori, Kouichi [Tsuchiura Kyodo General Hospital, Department of Radiology, Tsuchiura, Ibaraki (Japan); Imamura, Masatoshi [Tsuchiura Kyodo General Hospital, Department of Neonatology, Tsuchiura, Ibaraki (Japan); Mizushima, Yukiko [Tsuchiura Kyodo General Hospital, Department of Ophthalmology, Tsuchiura, Ibaraki (Japan); Tateishi, Ukihide [Tokyo Medical and Dental University, Departments of Diagnostic Radiology and Nuclear Medicine, Tokyo (Japan)

    2016-04-15

    The expected MRI-based dimensions of the intracranial optic nerve and optic tract in neonates are unknown. To evaluate the sizes of the intracranial optic nerve and optic tract in neonates at term-equivalent age using MRI. We retrospectively analyzed brain MRI examinations in 62 infants (28 boys) without intracranial abnormalities. The images were obtained in infants at term-equivalent age with a 1.5-tesla MRI scanner. We measured the widths and heights of the intracranial optic nerve and optic tract and calculated the cross-sectional areas using the formula for an ellipse. The means ± standard deviation of the width, height and cross-sectional area of the intracranial optic nerve were 2.7 ± 0.2 mm, 1.7 ± 0.2 mm and 3.5 ± 0.5 mm{sup 2}, respectively. The width, height and cross-sectional area of the optic tract were 1.5 ± 0.1 mm, 1.6 ± 0.1 mm and 2.0 ± 0.2 mm{sup 2}, respectively. Using univariate and multivariate analyses, we found that postmenstrual age showed independent intermediate positive correlations with the width (r = 0.48, P < 0.01) and cross-sectional area (r = 0.40, P < 0.01) of the intracranial optic nerve. The lower bounds of the 95% prediction intervals for the width and cross-sectional area of the intracranial optic nerve were 0.07 x (postmenstrual age in weeks) - 0.46 mm, and 0.17 x (postmenstrual age in weeks) - 4.0 mm{sup 2}, respectively. We identified the sizes of the intracranial optic nerve and optic tract in neonates at term-equivalent age. The postmenstrual age at MRI independently positively correlated with the sizes. (orig.)

  10. Targeted Metabolomics Reveals Early Dominant Optic Atrophy Signature in Optic Nerves of Opa1delTTAG/+ Mice.

    Science.gov (United States)

    Chao de la Barca, Juan Manuel; Simard, Gilles; Sarzi, Emmanuelle; Chaumette, Tanguy; Rousseau, Guillaume; Chupin, Stéphanie; Gadras, Cédric; Tessier, Lydie; Ferré, Marc; Chevrollier, Arnaud; Desquiret-Dumas, Valérie; Gueguen, Naïg; Leruez, Stéphanie; Verny, Christophe; Miléa, Dan; Bonneau, Dominique; Amati-Bonneau, Patrizia; Procaccio, Vincent; Hamel, Christian; Lenaers, Guy; Reynier, Pascal; Prunier-Mirebeau, Delphine

    2017-02-01

    Dominant optic atrophy (MIM No. 165500) is a blinding condition related to mutations in OPA1, a gene encoding a large GTPase involved in mitochondrial inner membrane dynamics. Although several mouse models mimicking the disease have been developed, the pathophysiological mechanisms responsible for retinal ganglion cell degeneration remain poorly understood. Using a targeted metabolomic approach, we measured the concentrations of 188 metabolites in nine tissues, that is, brain, three types of skeletal muscle, heart, liver, retina, optic nerve, and plasma in symptomatic 11-month-old Opa1delTTAG/+ mice. Significant metabolic signatures were found only in the optic nerve and plasma of female mice. The optic nerve signature was characterized by altered concentrations of phospholipids, amino acids, acylcarnitines, and carnosine, whereas the plasma signature showed decreased concentrations of amino acids and sarcosine associated with increased concentrations of several phospholipids. In contrast, the investigation of 3-month-old presymptomatic Opa1delTTAG/+ mice showed no specific plasma signature but revealed a significant optic nerve signature in both sexes, although with a sex effect. The Opa1delTTAG/+ versus wild-type optic nerve signature was characterized by the decreased concentrations of 10 sphingomyelins and 10 lysophosphatidylcholines, suggestive of myelin sheath alteration, and by alteration in the concentrations of metabolites involved in neuroprotection, such as dimethylarginine, carnitine, spermine, spermidine, carnosine, and glutamate, suggesting a concomitant axonal metabolic dysfunction. Our comprehensive metabolomic investigations revealed in symptomatic as well as in presymptomatic Opa1delTTAG/+ mice, a specific sensitiveness of the optic nerve to Opa1 insufficiency, opening new routes for protective therapeutic strategies.

  11. Inflammatory Papillitis in Uveitis: Response to Treatment and Use of Optic Nerve Optical Coherence Tomography for Monitoring.

    Science.gov (United States)

    Cho, Heeyoon; Pillai, Parvathy; Nicholson, Laura; Sobrin, Lucia

    2016-01-01

    To describe the clinical course of uveitis-associated inflammatory papillitis and evaluate the utility and reproducibility of optic nerve spectral domain optical coherence tomography (SD-OCT). Data on 22 eyes of 14 patients with uveitis-related papillitis and optic nerve imaging were reviewed. SD-OCT measure reproducibility was determined and parameters were compared in active vs. inactive uveitis. Papillitis resolution lagged behind uveitis resolution in three patients. For SD-OCT measures, the intraclass correlation coefficients were 99.1-100% and 86.9-100% for intraobserver and interobserver reproducibility, respectively. All SD-OCT optic nerve measures except inferior and nasal peripapillary retinal thicknesses were significantly higher in active vs. inactive uveitis after correction for multiple hypotheses testing. Mean optic nerve central thickness decreased from 545.1 to 362.9 µm (p = 0.01). Resolution of inflammatory papillitis can lag behind resolution of uveitis. SD-OCT assessment of papillitis is reproducible and correlates with presence vs. resolution of uveitis.

  12. A comparison of optic nerve dosimetry in craniospinal radiotherapy planned and treated with conventional and intensity modulated techniques

    International Nuclear Information System (INIS)

    Rene, Nicholas J.; Brodeur, Marylene; Parker, William; Roberge, David; Freeman, Carolyn

    2010-01-01

    Background and purpose: Some CNS tumours present leptomeningeal dissemination. Craniospinal radiotherapy is complex and recurrences may occur at sites of target volume underdosage. IMRT, being highly conformal to the target, could theoretically underdose the optic nerves if they are not specifically targeted leading to optic nerve recurrences. We analyzed optic nerve dosimetry when they are not specifically targeted. Materials and methods: We designed 3D-conformal and tomotherapy plans for our last five patients treated to the craniospinal axis, not including the optic nerves in the target volume. We analyzed the dose delivered to the optic nerves, to the anterior and posterior half of the optic nerves, and to a theoretical optic nerve-PTV. Results: The dose delivered to the optic nerves was similar for both plans in all patients (V95% close to 100%) except one in whom tomotherapy considerably underdosed the anterior optic nerves. The dose to the optic nerve-PTV was lower with tomotherapy in all patients. Conclusion: Despite not intentionally targeting the optic nerves, the dose to the optic nerves with IMRT was similar to 3D-conformal plans in most cases but left no margin for setup error. In individual cases the anterior half of the optic nerves could be significantly underdosed.

  13. Bilateral Traumatic Globe Luxation with Optic Nerve Transection

    Directory of Open Access Journals (Sweden)

    Levent Tok

    2014-12-01

    Full Text Available Purpose: The purpose of this study was to document clinical findings and management of a patient with bilateral globe luxation and optic nerve transection. Materials and Methods: A 25-year-old female patient was admitted to the emergency department with bilateral traumatic globe luxation following a motor vehicle accident. Results: Visual acuity testing showed no light perception. The right pupil was dilated and bilaterally did not react to light. The globes were bilaterally intact. A computed tomography scan revealed Le Fort type II fractures, bilateral optic nerve transection and disruption of all extraocular muscles. The globes of the patient were bilaterally reduced into the orbit. However, the patient developed phthisis bulbi in the right eye at month 3. Conclusion: Globe luxation presents a dramatic clinical picture, and may lead to the development of severe complications due to the concomitance of complete optic nerve dissection and multiple traumas. Even if the luxated globe is repositioned into the orbit, there is still an increased risk of the development of phthisis due to ischemia.

  14. Bilateral Traumatic Globe Luxation with Optic Nerve Transection

    Science.gov (United States)

    Tok, Levent; Tok, Ozlem Yalcin; Argun, Tugba Cakmak; Yilmaz, Omer; Gunes, Alime; Unlu, Elif Nisa; Sezer, Sezgin; Ibisoglu, Seda; Argun, Mehmet

    2014-01-01

    Purpose The purpose of this study was to document clinical findings and management of a patient with bilateral globe luxation and optic nerve transection. Materials and Methods A 25-year-old female patient was admitted to the emergency department with bilateral traumatic globe luxation following a motor vehicle accident. Results Visual acuity testing showed no light perception. The right pupil was dilated and bilaterally did not react to light. The globes were bilaterally intact. A computed tomography scan revealed Le Fort type II fractures, bilateral optic nerve transection and disruption of all extraocular muscles. The globes of the patient were bilaterally reduced into the orbit. However, the patient developed phthisis bulbi in the right eye at month 3. Conclusion Globe luxation presents a dramatic clinical picture, and may lead to the development of severe complications due to the concomitance of complete optic nerve dissection and multiple traumas. Even if the luxated globe is repositioned into the orbit, there is still an increased risk of the development of phthisis due to ischemia. PMID:25606034

  15. Retinal Nerve Fiber Layer Converges More Convexly on Normal Smaller Optic Nerve Head.

    Science.gov (United States)

    Jung, Kyoung In; Shin, Jeong Ah; Park, Hae-Young Lopilly; Park, Chan Kee

    2015-08-01

    To investigate retinal nerve fiber layer (RNFL) configuration in the optic nerve head (ONH) and peripapillary area according to disc size and to determine whether it explains cup discrepancy among eyes with different disc sizes. Horizontal and vertical RNFL curvature and mean thickness were measured using confocal scanning laser ophthalmoscopy (Heidelberg Retina Tomograph) in 63 normal subjects grouped by disc size. Average and quadrant RNFL thickness, disc size, average cup-to-disc ratio (CDR), and convergence angle at the optic disc were also measured using Cirrus HD-optical coherence tomography. The relationships between disc size and RNFL curvature, thickness, angle at optic disc, and CDR were evaluated. RNFL curvature and convergence angle reflects convexity "on" and "into" the optic disc, respectively. CDR was smaller for small discs and was positively correlated with disc size (Poptic disc were positively correlated with disc size (POptic disc area was negatively correlated with mean RNFL thickness at the optic disc margin measured by HRT (P=0.002), but not in the peripapillary area by optical coherence tomography. Using imaging techniques, we demonstrated that the shape of the RNFLs converging "on" and entering "into" the optic disc was more convex for small optic discs compared with large discs. A low CDR for small discs could be mediated by these RNFL profiles at the ONH, which may guide the clinical evaluation of glaucomatous ONH damage.

  16. Bilateral optical nerve atrophy secondary to lateral occipital lobe infarction.

    Science.gov (United States)

    Mao, Junfeng; Wei, Shihui

    2013-06-01

    To report a phenomenon of optical nerve atrophy secondary to lateral occipital lobe infarction. Two successive patients with unilateral occipital lobe infarction who experienced bilateral optical nerve atrophy during the follow-up underwent cranial imaging, fundus photography, and campimetry. Each patient was diagnosed with occipital lobe infarction by cranial MRI. During the follow-up, a bilateral optic atrophy was revealed, and campimetry showed a right homonymous hemianopia of both eyes with concomitant macular division. Bilateral optic atrophy was related to occipital lobe infarction, and a possible explanation for the atrophy was transneuronal degeneration caused by occipital lobe infarction.

  17. Endoscopic optic nerve decompression for nontraumatic compressive optic neuropathy

    Directory of Open Access Journals (Sweden)

    Cheng-long REN

    2015-11-01

    Full Text Available Objective To describe the preliminary experience with endoscopic optic nerve decompression (EOND for nontraumatic compressive optic neuropathies (NCONs. Methods The clinical data of 10 patients, male 5 and female 5, with a mean age of 44.3±5.1 years, who underwent EOND for visual loss (n=5 or visual deterioration (n=5 due to tumor compression in General Hospital of Armed Police Forces of China in the period from April 2013 to April 2014 were analyzed retrospectively. Preoperative and 6-month-postoperative clinical and imaging data of these patients were reviewed and analyzed. Results Among 5 patients who lost light perception (including 2 patients with bilateral optic nerve compression before operation, 4 of them showed visual improvement to different degrees on the 7th day after operation (with improvement of bilateral visual acuity. The other 5 patients with visual impairment before operation recovered their visual acuity to different extent after the operation. All of the patients had no obvious post-operative complications. Conclusion EOND is a safe, effective, and minimally invasive surgical technique affording recovery of visual function to NCON patients. DOI: 10.11855/j.issn.0577-7402.2015.11.12

  18. Optic nerve magnetisation transfer ratio after acute optic neuritis predicts axonal and visual outcomes.

    Directory of Open Access Journals (Sweden)

    Yejun Wang

    Full Text Available Magnetisation transfer ratio (MTR can reveal the degree of proton exchange between free water and macromolecules and was suggested to be pathological informative. We aimed to investigate changes in optic nerve MTR over 12 months following acute optic neuritis (ON and to determine whether MTR measurements can predict clinical and paraclinical outcomes at 6 and 12 months. Thirty-seven patients with acute ON were studied within 2 weeks of presentation and at 1, 3, 6 and 12 months. Assessments included optic nerve MTR, retinal nerve fibre layer (RNFL thickness, multifocal visual evoked potential (mfVEP amplitude and latency and high (100% and low (2.5% contrast letter acuity. Eleven healthy controls were scanned twice four weeks apart for comparison with patients. Patient unaffected optic nerve MTR did not significantly differ from controls at any time-point. Compared to the unaffected nerve, affected optic nerve MTR was significantly reduced at 3 months (mean percentage interocular difference = -9.24%, p = 0.01, 6 months (mean = -12.48%, p<0.0001 and 12 months (mean = -7.61%, p = 0.003. Greater reduction in MTR at 3 months in patients was associated with subsequent loss of high contrast letter acuity at 6 (ρ = 0.60, p = 0.0003 and 12 (ρ = 0.44, p = 0.009 months, low contrast letter acuity at 6 (ρ = 0.35, p = 0.047 months, and RNFL thinning at 12 (ρ = 0.35, p = 0.044 months. Stratification of individual patient MTR time courses based on flux over 12 months (stable, putative remyelination and putative degeneration predicted RNFL thinning at 12 months (F(2,32 = 3.59, p = 0.02. In conclusion, these findings indicate that MTR flux after acute ON is predictive of axonal degeneration and visual disability outcomes.

  19. Determining degree of optic nerve edema from color fundus photography

    Science.gov (United States)

    Agne, Jason; Wang, Jui-Kai; Kardon, Randy H.; Garvin, Mona K.

    2015-03-01

    Swelling of the optic nerve head (ONH) is subjectively assessed by clinicians using the Frisén scale. It is believed that a direct measurement of the ONH volume would serve as a better representation of the swelling. However, a direct measurement requires optic nerve imaging with spectral domain optical coherence tomography (SD-OCT) and 3D segmentation of the resulting images, which is not always available during clinical evaluation. Furthermore, telemedical imaging of the eye at remote locations is more feasible with non-mydriatic fundus cameras which are less costly than OCT imagers. Therefore, there is a critical need to develop a more quantitative analysis of optic nerve swelling on a continuous scale, similar to SD-OCT. Here, we select features from more commonly available 2D fundus images and use them to predict ONH volume. Twenty-six features were extracted from each of 48 color fundus images. The features include attributes of the blood vessels, optic nerve head, and peripapillary retina areas. These features were used in a regression analysis to predict ONH volume, as computed by a segmentation of the SD-OCT image. The results of the regression analysis yielded a mean square error of 2.43 mm3 and a correlation coefficient between computed and predicted volumes of R = 0:771, which suggests that ONH volume may be predicted from fundus features alone.

  20. CT evaluation of optic nerve compression in thyroid eye disease

    International Nuclear Information System (INIS)

    Barrett, L.; Giatt, H.J.; Burde, R.M.; Gado, M.

    1986-01-01

    In thyroid eye disease, visual loss due to optic nerve compression by enlarged muscles near the orbital apex requires prompt surgical decompression and must be differentiated from visual loss due to other mechanisms. Seventy-two high-resolution orbital CT scans of patients with thyroid eye disease were analyzed. From a coronal reconstruction, an easily measured ''apical index'' was determined. Average apical indices for orbits without optic neuropathy (41.0%) and with optic neuropathy (70.2%) were significantly different (P < .001). With the aid of the apical index, CT findings can be used to predict which patients with thyroid eye disease have optic nerve compression

  1. Diabetes mellitus and optic atrophy in two siblings: a report on a new association and a review of the literature.

    Science.gov (United States)

    Khardori, R; Stephens, J W; Page, O C; Dow, R S

    1983-01-01

    Two siblings with diabetes mellitus and optic atrophy (Wolfram syndrome) are described. As often noted, they also had atonic urinary bladders. Only one of the siblings had some impairment of hearing. Other findings not previously reported that appeared in each subject were esophageal dysphagia and vertigo. An autopsy in one revealed brain stem hypoplasia and thinning and flattening of the optic nerves with atrophy of the lateral geniculate bodies.

  2. Proteomics Analyses of Human Optic Nerve Head Astrocytes Following Biomechanical Strain*

    OpenAIRE

    Rogers, Ronan S.; Dharsee, Moyez; Ackloo, Suzanne; Sivak, Jeremy M.; Flanagan, John G.

    2011-01-01

    We investigate the role of glial cell activation in the human optic nerve caused by raised intraocular pressure, and their potential role in the development of glaucomatous optic neuropathy. To do this we present a proteomics study of the response of cultured, optic nerve head astrocytes to biomechanical strain, the magnitude and mode of strain based on previously published quantitative models. In this case, astrocytes were subjected to 3 and 12% stretches for either 2 h or 24 h. Proteomic me...

  3. Retinal nerve fiber layer thickness is associated with lesion length in acute optic neuritis

    DEFF Research Database (Denmark)

    Kallenbach, K; Simonsen, Helle Juhl; Sander, B

    2010-01-01

    included 41 patients with unilateral optic neuritis and 19 healthy volunteers. All patients were evaluated and examined within 28 days of onset of symptoms. The peripapillary retinal nerve fiber layer thickness (RNFLT), an objective quantitative measure of optic nerve head edema, was measured by optical...... coherence tomography and the length and location of the inflammatory optic nerve lesion were evaluated using MRI. RESULTS: Ophthalmoscopically, 34% of the patients had papillitis. The retinal nerve fiber layer in affected eyes (mean 123.1 microm) was higher during the acute phase than that of fellow eyes......BACKGROUND: Acute optic neuritis occurs with and without papillitis. The presence of papillitis has previously been thought to imply an anterior location of the neuritis, but imaging studies seeking to test this hypothesis have been inconclusive. METHODS: This prospective observational cohort study...

  4. Optic nerve histopathology in a case of Wolfram Syndrome

    DEFF Research Database (Denmark)

    Ross-Cisneros, Fred N; Pan, Billy X; Silva, Ruwan A

    2013-01-01

    Mitochondrial dysfunction in Wolfram Syndrome (WS) is controversial and optic neuropathy, a cardinal clinical manifestation, is poorly characterized. We here describe the histopathological features in postmortem retinas and optic nerves (ONs) from one patient with WS, testing the hypothesis...

  5. Retinal Nerve Fiber Layer Protrusion Associated with Tilted Optic Discs.

    Science.gov (United States)

    Chiang, Jaclyn; Yapp, Michael; Ly, Angelica; Hennessy, Michael P; Kalloniatis, Michael; Zangerl, Barbara

    2018-03-01

    This study resulted in the identification of an optic nerve head (ONH) feature associated with tilted optic discs, which might potentially contribute to ONH pathologies. Knowledge of such findings will enhance clinical insights and drive future opportunities to understand disease processes related to tilted optic discs. The aim of this study was to identify novel retinal nerve fiber layer (RNFL) anomalies by evaluating tilted optic discs using optical coherence tomography. An observed retinal nerve fiber protrusion was further investigated for association with other morphological or functional parameters. A retrospective review of 400 randomly selected adult patients with ONH examinations was conducted in a referral-only, diagnostic imaging center. After excluding other ONH pathologies, 215 patients were enrolled and evaluated for optic disc tilt and/or torsion. Gross anatomical ONH features, including size and rim or parapapillary region elevation, were assessed with stereoscopic fundus photography. Optical coherence tomography provided detailed morphological information of individual retinal layers. Statistical analysis was applied to identify significant changes between individual patient cohorts. A dome-shaped hyperreflective RNFL bulge, protruding into the neurosensory retina at the optic disc margins, was identified in 17 eyes with tilted optic discs. Available follow-up data were inconclusive regarding natural changes with this ONH feature. This RNFL herniation was significantly correlated with smaller than average optic disc size (P = .005), congenital disc tilt (P optic discs, which has not previously been assessed as an independent ONH structure. The feature is predominantly related to congenital crowded, small optic discs and variable between patients. This study is an important first step to elucidate diagnostic capabilities of tilted disc morphological changes and understanding associated functional deficits.

  6. Assessment of Optic Nerve Impairment in Patients with Neuromyelitis Optica by MR Diffusion Tensor Imaging.

    Directory of Open Access Journals (Sweden)

    Zhiye Chen

    Full Text Available Diffusion tensor imaging (DTI has been used for the evaluation of the white matter integrity. In this study, we evaluated optic nerve impairment in patients with neuromyelitis optica (NMO using DTI.Optic nerve DTI were performed on 28 NMO patients and 38 normal controls. Fractional anisotropy (FA values were measured in the anterior, middle, and posterior parts of the intraorbital optic nerve segment. For the posterior intraorbital optic nerve, FA values of BI (0.20±0.07, MI (0.24±0.16, and NA (0.25±0.14 decreased significantly compared with that of NC (0.43±0.07 (P<0.05, and ROC analysis demonstrated that the area under the curve (AUC measurements for BI vs. NC, MI vs. NC, NA vs. NC, and NMO (including BI, MI, and NA vs. NC were 0.99, 0.93, 0.88, and 0.96, respectively. The corresponding diagnostic sensitivities of ROC analysis were 100%, 80%, 80%, and 91%; and the specificities were 93%, 97%, 91%, and 93%.Decreased FA value in the intraorbital optic nerve, especially in the posterior part of the nerve, was demonstrated as a characteristic MR feature for NMO-related optic nerve impairment.

  7. Optical signature of nerve tissue-Exploratory ex vivo study comparing optical, histological, and molecular characteristics of different adipose and nerve tissues.

    Science.gov (United States)

    Balthasar, Andrea J R; Bydlon, Torre M; Ippel, Hans; van der Voort, Marjolein; Hendriks, Benno H W; Lucassen, Gerald W; van Geffen, Geert-Jan; van Kleef, Maarten; van Dijk, Paul; Lataster, Arno

    2018-05-14

    During several anesthesiological procedures, needles are inserted through the skin of a patient to target nerves. In most cases, the needle traverses several tissues-skin, subcutaneous adipose tissue, muscles, nerves, and blood vessels-to reach the target nerve. A clear identification of the target nerve can improve the success of the nerve block and reduce the rate of complications. This may be accomplished with diffuse reflectance spectroscopy (DRS) which can provide a quantitative measure of the tissue composition. The goal of the current study was to further explore the morphological, biological, chemical, and optical characteristics of the tissues encountered during needle insertion to improve future DRS classification algorithms. To compare characteristics of nerve tissue (sciatic nerve) and adipose tissues, the following techniques were used: histology, DRS, absorption spectrophotometry, high-resolution magic-angle spinning nuclear magnetic resonance (HR-MAS NMR) spectroscopy, and solution 2D 13 C- 1 H heteronuclear single-quantum coherence spectroscopy. Tissues from five human freshly frozen cadavers were examined. Histology clearly highlights a higher density of cellular nuclei, collagen, and cytoplasm in fascicular nerve tissue (IFAS). IFAS showed lower absorption of light around 1200 nm and 1750 nm, higher absorption around 1500 nm and 2000 nm, and a shift in the peak observed around 1000 nm. DRS measurements showed a higher water percentage and collagen concentration in IFAS and a lower fat percentage compared to all other tissues. The scattering parameter (b) was highest in IFAS. The HR-MAS NMR data showed three extra chemical peak shifts in IFAS tissue. Collagen, water, and cellular nuclei concentration are clearly different between nerve fascicular tissue and other adipose tissue and explain some of the differences observed in the optical absorption, DRS, and HR-NMR spectra of these tissues. Some differences observed between fascicular

  8. Blunt Facial Trauma Causing Isolated Optic Nerve Hematoma

    Directory of Open Access Journals (Sweden)

    R. Parab

    2013-01-01

    Full Text Available Traumatic optic neuropathy is an uncommon, yet serious, result of facial trauma. The authors present a novel case of a 59-year-old gentleman who presented with an isolated blunt traumatic left optic nerve hematoma causing vision loss. There were no other injuries or fractures to report. This case highlights the importance of early recognition of this rare injury and reviews the current literature and management of traumatic optic neuropathy.

  9. Neuromyelitis optica IgG in the cerebrospinal fluid induces astrocytopathy in optic nerve

    DEFF Research Database (Denmark)

    Soelberg, Kerstin; Lillevang, Søren Thue; Mørch, Marlene

    (anti-CD59a). A total of five mice received AQP4-IgG + C + anti-CD59a, four mice received normal-IgG + C + anti-CD59a, four mice received AQP4-IgG+ C and one normal-IgG + C. Mice were killed four days later. The optic nerves were isolated and fixed in paraformaldehyde. Paraffin embedded optic nerves...... was coincident with deposition of complement. Histopathological lesions were markedly enhanced with extensive/long-segment astrocytopathy of optic nerve and optic chiasm involvement in AQP4- IgG+ C + anti-CD59a treated mice. Such pathology was not seen in mice receiving normal human IgG, C and anti-CD59a...

  10. The MRI appearance of the optic nerve sheath following fenestration for benign intracranial hypertension

    Energy Technology Data Exchange (ETDEWEB)

    Sallomi, D.; Taylor, H.; Hibbert, J.; Sanders, M.D.; Spalton, D.J.; Tonge, K. [Guys and St. Thomas` Hospitals, London (United Kingdom)

    1998-09-01

    Optic nerve fenestration is carried out in cases of severe benign intracranial hypertension. This study aimed to monitor the optic nerve sheath appearances and orbital changes that occur following this procedure. The eight patients were all female with an average age of 37.3 years and a range of 20-58 years. The duration of symptoms was 2-6 years. Symptoms included headaches, diplopia and visual obscurations. Examination revealed severe papilledema. All investigations, including MRI, biochemical and immunological tests, were negative. Patients had fenestration of a 2 mm x 3 mm segment of the medial aspect of the optic nerve sheath. Imaging was obtained with a 1 T MRI machine using a head coil. Coronal, axial and sagittal 3 mm contiguous sections using STIR sequences with TR 4900 ms, IT 150 ms and TE 60 ms were obtained. Five patients showed clinical improvement. The post-operative MRI findings in four of these included a decreased volume of cerebrospinal fluid (CSF) around the optic nerve sheaths and a localized collection of fluid within the orbit. There were no MRI changes in the three patients with no clinical improvement. Decreased CSF volume around the optic nerve and a fluid collection within the orbit may indicate a favorable outcome in optic nerve fenestration. (orig.) With 3 figs., 12 refs.

  11. Atypical clinical presentation and long-term survival in a patient with optic nerve medulloepithelioma: a case report

    Directory of Open Access Journals (Sweden)

    Pastora-Salvador Natalia

    2012-05-01

    Full Text Available Abstract Introduction Medulloepithelioma is a rare congenital tumor of the primitive medullary neuroepithelium. A significant proportion of patients with medulloepithelioma arising from the optic nerve die from intracranial spread or cerebral metastasis. Because it has no known distinct clinical features and because of its low frequency, this tumor presents within the first two to six years of life and is usually misdiagnosed clinically as a different type of optic nerve tumor. Here, we describe a new and atypical case of medulloepithelioma of the optic nerve in a 12-year-old boy. To the best of our knowledge, he is the oldest reported patient to present with this disease and, now as an adult, has the longest documented period of disease-free survival. Case presentation A 12-year-old Caucasian boy with headache and unilateral amaurosis was referred for a presumed optic nerve glioma to our hospital. A computed tomography scan showed optic nerve enlargement, and fundoscopy showed a whitish mass at the optic disc. Our patient had been followed at his local hospital for four years for an 'optic disc cyst' with no change or progression. He experienced mild progressive visual impairment during that period. He was admitted for resection, and a histopathological analysis revealed a medulloepithelioma of the optic nerve. Supplemental orbital radiotherapy was performed. He remained disease-free for 25 years. Conclusions Medulloepithelioma of the optic nerve can clinically mimic more common pediatric tumors, such as optic glioma, meningioma, or retinoblastoma. Thus, medulloepithelioma should be included in the differential diagnoses of pediatric optic nerve lesions. Fundoscopy in these patients may provide relevant information for diagnosis. Anterior optic nerve medulloepitheliomas may behave differently from and have a better prognosis than medulloepitheliomas that have a more posterior location. Our case report illustrates that long-term survival can be

  12. Quantitative relations between the eyeball, the optic nerve, and the optic canal important for intracranial pressure monitoring.

    Science.gov (United States)

    Vaiman, Michael; Gottlieb, Paul; Bekerman, Inessa

    2014-08-17

    To find correlations between diameters of the optic nerve sheath (ONSD), the eyeball, and the optic canal that might be important for intracranial pressure monitoring. In a prospective cohort study, the CT data of consecutive 400 adults (18+) with healthy eyes and optic nerves and absence of neurological diseases were collected and analyzed. When the CT scans were obtained, the diameters of the optic nerve sheath, the eyeball, and the optic canal were measured and statistically analyzed. The data obtained from the left and from the right eyeballs and optic nerves were compared. The correlation analysis was performed within these variables, with the gender, and the age. In healthy persons, the ONSD varies from 3.65 mm to 5.17 mm in different locations within the intraorbital space with no significant difference between sexes and age groups. There is a strong correlation between the eyeball transverse diameter (ETD) and ONSD that can be presented as ONSD/ETD index. In healthy subjects, the ONSD/ETD index equals 0.19. The calculation of an index when ONSD is divided by the ETD of the eyeball presents precise normative database for ONSD intracranial pressure measurement technique. When the ONSD is measured for intracranial pressure monitoring, the most stable results can be obtained if the diameter is measured 10 mm from the globe. These data might serve as a normative database at emergency departments and in general neurological practice.

  13. Evolution of optic nerve and retina alterations in a child with indirect traumatic neuropathy as assessed by optical coherence tomography

    Directory of Open Access Journals (Sweden)

    Julia Dutra Rossetto

    Full Text Available ABSTRACT Herein, we describe the case of a 4-year-old child with indirect traumatic optic neuropathy and serial changes of the optic nerve head and retinal nerve fiber layer (RNFL documented using optical coherence tomography (OCT. Visual acuity improved despite progressive RNFL thinning and optic disc pallor. We concluded that OCT may be useful for monitoring axonal loss but may not predict the final visual outcome.

  14. Detection of optic nerve atrophy following a single episode of unilateral optic neuritis by MRI using a fat-saturated short-echo fast FLAIR sequence

    International Nuclear Information System (INIS)

    Hickman, S.J.; Brex, P.A.; Silver, N.C.; Barker, G.J.; Miller, D.H.; Brierley, C.M.H.; Compston, D.A.S.; Scolding, N.J.; Moseley, I.F.; Plant, G.T.

    2001-01-01

    We describe an MRI technique for quantifying optic nerve atrophy resulting from a single episode of unilateral optic neuritis. We imaged 17 patients, with a median time since onset of optic neuritis of 21 months (range 3-81 months), using a coronal-oblique fat-saturated short-echo fast fluid-attenuated inversion-recovery (sTE fFLAIR) sequence. The mean cross-sectional area of the intraorbital portion of the optic nerves was calculated by a blinded observer from five consecutive 3 mm slices from the orbital apex forwards using a semiautomated contouring technique and compared with data from 16 controls. The mean optic nerve area was 11.2mm 2 in the affected eye of the patients, 12.9mm 2 in the contralateral eye (P = 0.006 compared to the affected eye) and 12.8mm 2 in controls (P = 0.03 compared to the affected eyes). There was a significant negative correlation between disease duration and the size of the affected optic nerve (r = -0.59, P = 0.012). The measurement coefficient of variation was 4.8 %. The sTE fFLAIR sequence enables measurement of optic nerve area with sufficient reproducibility to show optic nerve atrophy following a single episode of unilateral optic neuritis. The correlation of increasing optic nerve atrophy with disease duration would be consistent with ongoing axonal loss in a persistently demyelinated lesion, or Wallerian degeneration following axonal damage during the acute inflammatory phase. (orig.)

  15. Retinal nerve fiber and optic disc morphology using spectral-domain optical coherence tomography in scleroderma patients.

    Science.gov (United States)

    Sahin-Atik, Sevinc; Koc, Feray; Akin-Sari, Sirin; Ozmen, Mustafa

    2017-05-11

    To evaluate the optic nerve head parameters and peripapillary retinal nerve fiber layer using spectral-domain optical coherence tomography (SD-OCT) in a systemic sclerosis (SSc) cohort and age-matched controls to determine whether SSc patients have an increased risk of normal-tension glaucoma (NTG). We examined 30 patients (3 male, 27 female) with SSc and 28 age- and sex-matched controls. Retinal nerve fiber and optic disc morphology were evaluated using Cirrus SD-OCT. Optic disc morphology measurements including disc area, rim area, average and vertical cup/disc (C/D) ratio, and cup volume were not significantly different between the study groups. The average and 4-quadrant retinal nerve fiber layer (RNFL) measurements of the C/D >0.3 subgroups were not significantly different in the patients and controls. These values were also similar for the C/D >0.5 subgroups except that the average inferior quadrant RNFL thickness of the right eyes in the patient subgroup was significantly thinner than in the control subgroup (p<0.05). Our SSc cohort had relatively shorter disease duration but increased prevalence of early glaucomatous damage signs. Our findings indicate that SSc is a risk factor for developing normal-tension glaucoma. Further studies combined with visual field evaluation are necessary to identify the long-term glaucomatous effects of SSc.

  16. Peripapillary retinal nerve fiber layer and optic nerve head characteristics in eyes with situs inversus of the optic disc.

    Science.gov (United States)

    Kang, Sunah; Jin, Sunyoung; Roh, Kyu Hwa; Hwang, Young Hoon

    2015-01-01

    This study was performed to investigate the peripapillary retinal nerve fiber layer (RNFL) and optic nerve head (ONH) characteristics, as determined using a spectral-domain optical coherence tomography (OCT), in eyes with situs inversus of the optic disc. The peripapillary RNFL and the ONH were assessed in 12 eyes belonging to 6 subjects with situs inversus of the optic disc (situs inversus group) and 24 eyes in 12 age-matched, sex-matched, and refractive error-matched healthy subjects (control group) by using OCT. The average, quadrant, and clock-hour RNFL thicknesses (clock-hour 9 on the scan represented the temporal side of the optic disc in both eyes), the superior/inferior RNFL peak locations, and ONH characteristics such as disc area, rim area, cup-to-disc ratio, vertical cup-to-disc ratio, and cup volume were obtained. The differences in RNFL and ONH characteristics between the 2 groups were analyzed. The situs inversus group had a thicker RNFL in the clock-hour sectors 3 and 4, a thinner RNFL in the clock-hour sectors 7, 8, and 11, and more nasally located superior and inferior RNFL peak locations than the control group (P≤0.001). The situs inversus group had a smaller cup-to-disc area ratio, smaller vertical cup-to-disc ratio, and a lesser cup volume than the control group (Poptic disc showed different peripapillary RNFL and ONH characteristics from those without this abnormality. These findings should be considered when assessing eyes with situs inversus of the optic disc.

  17. Focal dermal hypoplasia without focal dermal hypoplasia

    NARCIS (Netherlands)

    Contreras-Capetillo, Silvina N.; Lombardi, Maria Paola; Pinto-Escalante, Doris; Hennekam, Raoul C.

    2014-01-01

    Focal dermal hypoplasia (FDH; Goltz-Gorlin syndrome) is an X-linked dominant disorder affecting mainly tissues of ectodermal and mesodermal origin. The phenotype is characterized by hypoplastic linear skin lesions, eye malformations, hair and teeth anomalies, and multiple limbs malformations. The

  18. Dural ectasia of the optic nerve sheath: is it always benign?

    Directory of Open Access Journals (Sweden)

    Berker Bakbak

    2009-11-01

    Full Text Available Berker Bakbak1, Hava Dönmez2, Tülay Kansu3, Hayyam Kiratli41Hacettepe University Institute of Neurological Sciences and Psychiatry, Neuro-ophthalmology Unit, Ankara, Turkey; 2Diskapi Yildirim Beyazid Education and Research Hospital Neurology Clinic, Ankara, Turkey; 3Hacettepe University Medical Faculty, Department of Neurology, Neuro-Ophthalmology Unit, Ankara, Turkey; 4Hacettepe University Medical Faculty, Department of Ophthalmology, Ocular Oncology Unit, Ankara, TurkeyAbstract: A 36-year-old woman with a 3-month history of progressive visual loss had papilledema, dilatation of the optic nerve sheaths and normal cerebrospinal fluid pressures. She was diagnosed as dural ectasia of the optic nerve sheaths and surgical decompression was performed. In this case report, severe visual loss is described as a serious complication of this rare disease and the importance of early surgical intervention is emphasized.Keywords: optic nerve, dural ectasia, meningocele

  19. Detection of optic nerve lesions in optic neuritis using frequency-selective fat-saturation sequences

    International Nuclear Information System (INIS)

    Miller, D.H.; MacManus, D.G.; Bartlett, P.A.; Kapoor, R.; Morrissey, S.P.; Moseley, I.F.

    1993-01-01

    MRI was performed on seven patients with acute optic neuritis, using two sequences which suppress the signal from orbital fat: frequency-selective fat-saturation and inversion recovery with a short inversion time. Lesions were seen on both sequences in all the symptomatic optic nerves studied. (orig.)

  20. Human umbilical cord blood-derived stem cells and brain-derived neurotrophic factor protect injured optic nerve: viscoelasticity characterization

    Directory of Open Access Journals (Sweden)

    Xue-man Lv

    2016-01-01

    Full Text Available The optic nerve is a viscoelastic solid-like biomaterial. Its normal stress relaxation and creep properties enable the nerve to resist constant strain and protect it from injury. We hypothesized that stress relaxation and creep properties of the optic nerve change after injury. More-over, human brain-derived neurotrophic factor or umbilical cord blood-derived stem cells may restore these changes to normal. To validate this hypothesis, a rabbit model of optic nerve injury was established using a clamp approach. At 7 days after injury, the vitreous body re-ceived a one-time injection of 50 µg human brain-derived neurotrophic factor or 1 × 106 human umbilical cord blood-derived stem cells. At 30 days after injury, stress relaxation and creep properties of the optic nerve that received treatment had recovered greatly, with patho-logical changes in the injured optic nerve also noticeably improved. These results suggest that human brain-derived neurotrophic factor or umbilical cord blood-derived stem cell intervention promotes viscoelasticity recovery of injured optic nerves, and thereby contributes to nerve recovery.

  1. Comparison of human expert and computer-automated systems using magnitude-squared coherence (MSC) and bootstrap distribution statistics for the interpretation of pattern electroretinograms (PERGs) in infants with optic nerve hypoplasia (ONH).

    Science.gov (United States)

    Fisher, Anthony C; McCulloch, Daphne L; Borchert, Mark S; Garcia-Filion, Pamela; Fink, Cassandra; Eleuteri, Antonio; Simpson, David M

    2015-08-01

    Pattern electroretinograms (PERGs) have inherently low signal-to-noise ratios and can be difficult to detect when degraded by pathology or noise. We compare an objective system for automated PERG analysis with expert human interpretation in children with optic nerve hypoplasia (ONH) with PERGs ranging from clear to undetectable. PERGs were recorded uniocularly with chloral hydrate sedation in children with ONH (aged 3.5-35 months). Stimuli were reversing checks of four sizes focused using an optical system incorporating the cycloplegic refraction. Forty PERG records were analysed; 20 selected at random and 20 from eyes with good vision (fellow eyes or eyes with mild ONH) from over 300 records. Two experts identified P50 and N95 of the PERGs after manually deleting trials with movement artefact, slow-wave EEG (4-8 Hz) or other noise from raw data for 150 check reversals. The automated system first identified present/not-present responses using a magnitude-squared coherence criterion and then, for responses confirmed as present, estimated the P50 and N95 cardinal positions as the turning points in local third-order polynomials fitted in the -3 dB bandwidth [0.25 … 45] Hz. Confidence limits were estimated from bootstrap re-sampling with replacement. The automated system uses an interactive Internet-available webpage tool (see http://clinengnhs.liv.ac.uk/esp_perg_1.htm). The automated system detected 28 PERG signals above the noise level (p ≤ 0.05 for H0). Good subjective quality ratings were indicative of significant PERGs; however, poor subjective quality did not necessarily predict non-significant signals. P50 and N95 implicit times showed good agreement between the two experts and between experts and the automated system. For the N95 amplitude measured to P50, the experts differed by an average of 13% consistent with differing interpretations of peaks within noise, while the automated amplitude measure was highly correlated with the expert measures but was

  2. Optic Nerve Atrophy Due to Long-Standing Compression by Planum Sphenoidale Meningioma.

    Science.gov (United States)

    Di Somma, Alberto; Kaen, Ariel Matias; Cárdenas Ruiz-Valdepeñas, Eugenio; Cavallo, Luigi Maria

    2018-05-01

    In this study we report an uncommon endoscopic endonasal image of an atrophic optic nerve as seen after surgical removal of a suprasellar meningioma. The peculiarity of this case is the long-lasting underestimated ocular symptomatology of the patient who reported a 15-year history of impairment of vision on her left eye. A 51-year-old woman was admitted to our hospital complaining of a 15-year history of impairment of vision on her left eye. After making serendipitously the diagnosis of a suprasellar mass, we performed endoscopic endonasal surgery. The tumor was reached from below and removed safely, without manipulation of the optic pathways. At the end of tumor removal, the impressive left optic nerve atrophy due to enduring local tumor compression was visualized. To the best of our knowledge, no endoscopic endonasal image with such features has been provided in the pertinent literature. Possibly, this contribution will help identify damaged optic nerves during endoscopic endonasal surgery. Copyright © 2018 Elsevier Inc. All rights reserved.

  3. Integrated Model of the Eye/Optic Nerve Head Biomechanical Environment

    Science.gov (United States)

    Ethier, C. R.; Feola, A.; Myers, J. G.; Nelson, E.; Raykin, J.; Samuels, B.

    2017-01-01

    Visual Impairment and Intracranial Pressure (VIIP) syndrome is a concern for long-duration space flight. Previously, it has been suggested that ocular changes observed in VIIP syndrome are related to the cephalad fluid shift that results in altered fluid pressures [1]. We are investigating the impact of changes in intracranial pressure (ICP) using a combination of numerical models, which simulate the effects of various environment conditions, including finite element (FE) models of the posterior eye. The specific interest is to understand how altered pressures due to gravitational changes affect the biomechanical environment of tissues of the posterior eye and optic nerve sheath. METHODS: Additional description of the numerical modeling is provided in the IWS abstract by Nelson et al. In brief, to simulate the effects of a cephalad fluid shift on the cardiovascular and ocular systems, we utilized a lumped-parameter compartment model of these systems. The outputs of this lumped-parameter model then inform boundary conditions (pressures) for a finite element model of the optic nerve head (Figure 1). As an example, we show here a simulation of postural change from supine to 15 degree head-down tilt (HDT), with primary outcomes being the predicted change in strains at the optic nerve head (ONH) region, specifically in the lamina cribrosa (LC), retrolaminar optic nerve, and prelaminar neural tissue (PLNT). The strain field can be decomposed into three orthogonal components, denoted as the first, second and third principal strains. We compare the peak tensile (first principal) and compressive (third principal) strains, since elevated strain alters cell phenotype and induces tissue remodeling. RESULTS AND CONCLUSIONS: Our lumped-parameter model predicted an IOP increase of c. 7 mmHg after 21 minutes of 15 degree HDT, which agreed with previous reports of IOP in HDT [1]. The corresponding FEM simulations predicted a relative increase in the magnitudes of the peak tensile

  4. Bilateral vision loss in a captive cheetah (Acinonyx jubatus).

    Science.gov (United States)

    Walser-Reinhardt, Ladina; Wernick, Morena B; Hatt, Jean-Michel; Spiess, Bernhard M

    2010-09-01

    The following case report describes a 1-year-old female cheetah (Acinonyx jubatus) with bilateral blindness and unresponsive pupils. For comparison, a second healthy 2.5-year-old male cheetah without visual deficits was also examined. Clinical examination of both animals included biomicroscopy, indirect ophthalmoscopy, tonometry, and electroretinography. The young female cheetah showed no menace response, no direct or indirect pupillary light reflex, and no dazzle reflex in either eye. Fundus lesions, as detected by indirect ophthalmoscopy, are described for the female animal. In both eyes, the fundus color was green/turquoise/yellow with multiple hyperpigmented linear lesions in the tapetal area around the optic nerve. The optic nerve head was dark gray and about half the normal size suggesting bilateral optic nerve hypoplasia and retinal dysplasia or differentially optic nerve atrophy and chorioretinal scarring. The ERG had low amplitudes in the right eye but appeared normal in the left eye compared with the male cheetah. Blood levels did not suggest current taurine deficiency. This is addressed to some degree in the discussion. Bilateral optic nerve hypoplasia or optic nerve atrophy is a rare anomaly in cats and has not yet been described in a cheetah.

  5. MR images of optic nerve compression by the intracranial carotid artery. Including the patients with normal tension glaucoma

    International Nuclear Information System (INIS)

    Kurokawa, Hiroaki; Kin, Kiyonori; Arichi, Miwa; Ogata, Nahoko; Shimizu, Ken; Akai, Mikio; Ikeda, Koshi; Sawada, Satoshi; Matsumura, Miyo

    2003-01-01

    Twenty-one eyes of 12 patients with MRI-defined optic nerve compression by the intracranial carotid artery were examined to investigate whether the visual field defects result from optic nerve compression or other causes. In 4 affected eyes with 2 patients, we could not distinguish whether the visual field defects were due to optic nerve compression or normal-tension glaucoma. These patients had evidence of glaucoma-like cupping of the optic disc and visual field defects. Nine affected eyes with 7 patients were diagnosed as having compressive optic neuropathy due to unilateral optic nerve compression associated with visual field defects or non-glaucomatous visual field defects. Four of 9 affected eyes were associated with optic disc cupping of various degrees. We suggest that the glaucoma-like visual field defects and optic disc cupping may result from a compressive lesion of the anterior visual pathway. Frequently, this feature caused confusion in the differential diagnosis between optic nerve compression by carotid artery and normal-tension glaucoma. (author)

  6. Specific changes in rapidly transported proteins during regeneration of the goldfish optic nerve

    International Nuclear Information System (INIS)

    Benowitz, L.I.; Shashoua, V.E.; Yoon, M.G.

    1981-01-01

    Double labeling methods were used to identify changes in the complement of proteins synthesized in the retinal ganglion cells and transported down the optic nerve during the process of axonal regeneration. Eight to 62 days after goldfish underwent a unilateral optic nerve crush, one eye was labeled with [3H]-, the other with [14C]proline. Control and regenerating optic nerves were dissected out and homogenized together after 5 hr, a time which allowed us to examine selectively membrane-bound components which migrate in the rapid phase of axoplasmic transport. Proteins from the two sides were so-purified and separated by sodium dodecyl sulfate-polyacrylamide gel electrophoresis. Analysis of the 3H and 14C incorporation patterns along the gels revealed a radical shift away from the normal labeling spectrum during regeneration, with selective changes in labeling at particular molecular weights varying over a 3-fold range. Eight days after crushing the optic nerve, the greatest increases in labeling were seen for material with apparent molecular weights of 24,000 to 27,000, 44,000, and 210,000 daltons. These peaks declined thereafter, and on days 29 to 39, the most prominent increases were at 110,000 to 140,000 daltons. These studies indicate a continuously changing pattern in the synthesis and/or degradation of proteins that are rapidly transported down the optic nerve during regeneration and point to molecular species potential significance in the establishment of the visual map upon the brain

  7. Optic pathway glioma associated with orbital rhabdomyosarcoma and bilateral optic nerve sheath dural ectasia in a child with neurofibromatosis-1

    International Nuclear Information System (INIS)

    Nikas, Ioannis; Theofanopoulou, Maria; Lampropoulou, Penelope; Hadjigeorgi, Christiana; Pourtsidis, Apostolos; Kosmidis, Helen

    2006-01-01

    Neurofibromatosis-1 (NF-1) is a multisystem disorder presenting with a variety of clinical and imaging manifestations. Neural and non-neural tumours, and unusual benign miscellaneous conditions, separately or combined, are encountered in variable locations. We present a 21/2-year-old boy with NF-1 who demonstrated coexisting optic pathway glioma with involvement of the chiasm and optic nerve, orbital alveolar rhabdomyosarcoma and bilateral optic nerve sheath dural ectasia. (orig.)

  8. Optic nerve injury demonstrated by MRI with STIR sequences

    International Nuclear Information System (INIS)

    Takehara, S.; Tanaka, T.; Uemura, K.; Shinohara, Y.; Yamamoto, T.; Tokuyama, T.; Satoh, A.

    1994-01-01

    We studied nine patients with optic nerve injury associated with closed head trauma by magnetic resonance imaging (MRI) with short inversion time inversion recovery (STIR) sequences on 11 occasions from 4 days to 14 years after the injury: three studies were within 17 days and eight over 4 months to 14 years. MRI revealed abnormal high signal in 10 of the 11 injured nerves. MRI 4 days after the injury showed no abnormality. (orig.)

  9. Ciliary body melanoma with optic nerve invasion.

    Science.gov (United States)

    al-Haddab, S; Hidayat, A; Tabbara, K F

    1990-01-01

    A case of melanoma of the ciliary body is presented. Initially the patient was diagnosed and treated for uveitis, but following CT scanning and ultrasound a tumour was detected and the eye enucleated. Histopathologically it was found that the tumour had invaded the optic nerve head, apparently via Cloquet's canal. Images PMID:2310725

  10. Experience on treatment of acute head injury combined with optic nerve damage

    International Nuclear Information System (INIS)

    Gao Heng; Feng Dongxia; Ma Yuanpin; Chen Jinqing

    2000-01-01

    Objective: To investigate the therapeutic principle for the management of acute head injury combined with optic nerve damage. Method: the clinical data of treatment and prognosis from 24 patients, in which 15 received operative and 9 conservative measures were collected and analyzed. Results: In 15 operated cases, the vision of 10 cases including one with blindness before operation was improved obviously, while those of other 5 did not get any improvement. In 9 conservatively treated cases, the vision was improved in 4 cases, deteriorated in 4 case and no change in 1 case with blindness after injury. Conclusion: One the optic nerve damage has been manifested by clinical or radiological evidences in acute head injury patients, despite it was primary or secondary reason, surgical optic nerve bone canal decompression should be done as soon as possible

  11. Ultrastructure of the extracellular matrix of bovine dura mater, optic nerve sheath and sclera.

    Science.gov (United States)

    Raspanti, M; Marchini, M; Della Pasqua, V; Strocchi, R; Ruggeri, A

    1992-10-01

    The sclera, the outermost sheath of the optic nerve and the dura mater have been investigated histologically and ultrastructurally. Although these tissues appear very similar under the light microscope, being dense connective tissues mainly composed of collagen bundles and a limited amount of cells and elastic fibres, they exhibit subtle differences on electron microscopy. In the dura and sclera collagen appears in the form of large, nonuniform fibrils, similar to those commonly found in tendons, while in the optic nerve sheath the fibrils appear smaller and uniform, similar to those commonly observed in reticular tissues, vessel walls and skin. Freeze-fracture also reveals these fibrils to have different subfibrillar architectures, straight or helical, which correspond to 2 distinct forms of collagen fibril previously described (Raspanti et al. 1989). The other extracellular matrix components also vary with the particular collagen fibril structure. Despite their common embryological derivation, the dura mater, optic nerve sheath and sclera exhibit diversification of their extracellular matrix consistent with the mechanical loads to which these tissues are subjected. Our observations indicate that the outermost sheath of the optic nerve resembles the epineurium of peripheral nerves rather than the dura to which it is commonly likened.

  12. Vermian aplasia and hypoplasia

    International Nuclear Information System (INIS)

    Fitz, C.R.

    1990-01-01

    This paper reports vermian aplasia or hypoplasia unrelated to Dandy-Walker complex or Joubert syndrome. Vermian hypoplasia is most commonly associated with the malformations of Dandy-Walker cyst or variant. There have been few reports of familial vermian anomaly (Joubert syndrome) with episodes of polypnea and apnea. Five patients with vermian hypoplasia or absence were prospectively discovered among 1,130 children undergoing brain MR imaging. Ages ranged from 2 mo to 15 y at the time of discovery: The abnormalities were poorly defined in three patients who had previously undergone CT. There was no common link in these patients with a variable number of other systemic, brain, or facial abnormalities. No unifying symptoms were identified. Two patients had the MR imaging appearance of Joubert syndrome, but none had the cyclical respiratory symptoms usually associated with this syndrome

  13. Optic nerve sheath diameter: A novel way to monitor the brain

    Directory of Open Access Journals (Sweden)

    Seelora Sahu

    2017-01-01

    Full Text Available Measurement and monitoring of intracranial pressure is pivotal in management of brain injured patients. As a rapid and easily done bed side measurement, ultrasonography of the optic nerve sheath diameter presents itself as a possible replacement of the conventional invasive methods of intracranial pressure management. In this review we go through the evolution of optic nerve sheath diameter measurement as a novel marker of predicting raised intracranial pressure, the modalities by which it can be measured as well as its correlation with the invasive methods of intracranial pressure monitoring.

  14. Sodium channels in axons and glial cells of the optic nerve of Necturus maculosa.

    Science.gov (United States)

    Tang, C M; Strichartz, G R; Orkand, R K

    1979-11-01

    Experiments investigating both the binding of radioactively labelled saxitoxin (STX) and the electrophysiological response to drugs that increase the sodium permeability of excitable membranes were conducted in an effort to detect sodium channels in glial cells of the optic nerve of Necturus maculosa, the mudpuppy. Glial cells in nerves from chronically enucleated animals, which lack optic nerve axons, show no saturable uptake of STX whereas a saturable uptake is clearly present in normal optic nerves. The normal nerve is depolarized by aconitine, batrachotoxin, and veratridine (10(-6)-10(-5) M), whereas the all-glial preparation is only depolarized by veratridine and at concentrations greater than 10(-3) M. Unlike the depolarization caused by veratridine in normal nerves, the response in the all-glial tissue is not blocked by tetrodotoxin nor enhanced by scorpion venom (Leiurus quinquestriatus). In glial cells of the normal nerve, where axons are also present, the addition of 10(-5) M veratridine does lead to a transient depolarization; however, it is much briefer than the axonal response to veratridine in this same tissue. This glial response to veratridine could be caused by the efflux of K+ from the drug-depolarized axons, and is similar to the glial response to extracellular K+ accumulation resulting from action potentials in the axon.

  15. Genetics Home Reference: focal dermal hypoplasia

    Science.gov (United States)

    ... in people with focal dermal hypoplasia is an omphalocele , which is an opening in the wall of ... Dermal Hypoplasia MedlinePlus Encyclopedia: Ectodermal dysplasia MedlinePlus Encyclopedia: Omphalocele General Information from MedlinePlus (5 links) Diagnostic Tests ...

  16. Primary optic nerve sheath meningioma

    Energy Technology Data Exchange (ETDEWEB)

    Jeremic, Branislav [International Atomic Energy Agency, Vienna (Austria); Pitz, Susanne (eds.) [University Eye Hospital, Mainz (Germany)

    2008-07-01

    Optic nerve sheath meningioma (ONSM) is a rare tumour. Cases are usually separated into primary ONSM, which arises either intraorbitally or, less commonly, intracanalicularly, and secondary ONSM, which arises intracranially and subsequently invades the optic canal and orbit. This is the first book to cover all important aspects of the diagnosis and treatment of primary ONSM. After a general introduction, individual chapters discuss the clinical presentation, clinical examination and diagnosis, imaging, and histology. Treatment options are then addressed in detail, with special emphasis on external beam radiation therapy, and in particular stereotactic fractionated radiation therapy. The latter has recently produced consistently good results and is now considered the emerging treatment of choice for the vast majority of patients with primary ONSM. This well-illustrated book will prove invaluable to all practitioners who encounter primary ONSM in their clinical work. (orig.)

  17. Computed tomography in orbital fractures and optic nerve trauma

    International Nuclear Information System (INIS)

    Dietrich, U.; Sievers, K.; Feldges, A.; Nau, H.E.

    1990-01-01

    Ten patients with orbital fractures and optic nerve trauma are reported. Fractures of the optic canal could be demonstrated by computed tomography in six cases and fractures of the orbital apex in another three cases. Surgical decompression of the optic canal was performed in seven cases. Computed tomography enhanced decision for surgery in cases of intraorbital haematoma with exophthalmus and narrowing of the canal by bony fragments, especially in those patients presenting with incomplete or progressive visual disturbance. (orig.) [de

  18. Optic nerve head biomechanics in aging and disease.

    Science.gov (United States)

    Downs, J Crawford

    2015-04-01

    This nontechnical review is focused upon educating the reader on optic nerve head biomechanics in both aging and disease along two main themes: what is known about how mechanical forces and the resulting deformations are distributed in the posterior pole and ONH (biomechanics) and what is known about how the living system responds to those deformations (mechanobiology). We focus on how ONH responds to IOP elevations as a structural system, insofar as the acute mechanical response of the lamina cribrosa is confounded with the responses of the peripapillary sclera, prelaminar neural tissues, and retrolaminar optic nerve. We discuss the biomechanical basis for IOP-driven changes in connective tissues, blood flow, and cellular responses. We use glaucoma as the primary framework to present the important aspects of ONH biomechanics in aging and disease, as ONH biomechanics, aging, and the posterior pole extracellular matrix (ECM) are thought to be centrally involved in glaucoma susceptibility, onset and progression. Copyright © 2015 Elsevier Ltd. All rights reserved.

  19. Optical coherence tomography of the prostate nerves

    Science.gov (United States)

    Chitchian, Shahab

    Preservation of the cavernous nerves during prostate cancer surgery is critical in preserving a man's ability to have spontaneous erections following surgery. These microscopic nerves course along the surface of the prostate within a few millimeters of the prostate capsule, and they vary in size and location from one patient to another, making preservation of the nerves difficult during dissection and removal of a cancerous prostate gland. These observations may explain in part the wide variability in reported sexual potency rates (9--86%) following prostate cancer surgery. Any technology capable of providing improved identification, imaging, and visualization of the cavernous nerves during prostate cancer surgery would be of great assistance in improving sexual function after surgery, and result in direct patient benefit. Optical coherence tomography (OCT) is a noninvasive optical imaging technique capable of performing high-resolution cross-sectional in vivo and in situ imaging of microstructures in biological tissues. OCT imaging of the cavernous nerves in the rat and human prostate has recently been demonstrated. However, improvements in the OCT system and the quality of the images for identification of the cavernous nerves is necessary before clinical use. The following chapters describe complementary approaches to improving identification and imaging of the cavernous nerves during OCT of the prostate gland. After the introduction to OCT imaging of the prostate gland, the optimal wavelength for deep imaging of the prostate is studied in Chapter 2. An oblique-incidence single point measurement technique using a normal-detector scanning system was implemented to determine the absorption and reduced scattering coefficients, mua and m's , of fresh canine prostate tissue, ex vivo, from the diffuse reflectance profile of near-IR light as a function of source-detector distance. The effective attenuation coefficient, mueff, and the Optical Penetration Depth (OPD) were

  20. Isolated optic nerve gliomas: a multicenter historical cohort study.

    Science.gov (United States)

    Shofty, Ben; Ben-Sira, Liat; Kesler, Anat; Jallo, George; Groves, Mari L; Iyer, Rajiv R; Lassaletta, Alvaro; Tabori, Uri; Bouffet, Eric; Thomale, Ulrich-Wilhelm; Hernáiz Driever, Pablo; Constantini, Shlomi

    2017-12-01

    OBJECTIVE Isolated optic nerve gliomas (IONGs) constitute a rare subgroup of optic pathway gliomas (OPGs). Due to the rarity of this condition and the difficulty in differentiating IONGs from other types of OPGs in most clinical series, little is known about these tumors. Currently, due to lack of evidence, they are managed the same as any other OPG. METHODS The authors conducted a multicenter retrospective cohort study aimed at determining the natural history of IONGs. Included were patients with clear-cut glioma of the optic nerve without posterior (chiasmatic/hypothalamic) involvement. At least 1 year of follow-up, 2 MRI studies, and 2 neuro-ophthalmological examinations were required for inclusion. RESULTS Thirty-six patients with 39 tumors were included in this study. Age at diagnosis ranged between 6 months and 16 years (average 6 years). The mean follow-up time was 5.6 years. Twenty-five patients had neurofibromatosis Type 1. During the follow-up period, 59% of the tumors progressed, 23% remained stable, and 18% (all with neurofibromatosis Type 1) displayed some degree of spontaneous regression. Fifty-one percent of the patients presented with visual decline, of whom 90% experienced further deterioration. Nine patients were treated with chemotherapy, 5 of whom improved visually. Ten patients underwent operation, and no local or distal recurrence was noted. CONCLUSIONS Isolated optic nerve gliomas are highly dynamic tumors. Radiological progression and visual deterioration occur in greater percentages than in the general population of patients with OPGs. Response to chemotherapy may be better in this group, and its use should be considered early in the course of the disease.

  1. In vivo phosphorylation of axonal proteins in goldfish optic nerve during regeneration

    Energy Technology Data Exchange (ETDEWEB)

    Larrivee, D.C.; Grafstein, B.

    1987-01-01

    In vivo phosphorylation of axonal proteins was investigated in normal and regenerating optic nerves of goldfish by two-dimensional gel electrophoresis. By 6-24 h after intraocular injection of H/sub 3/(32)PO/sub 4/, approximately 20 optic nerve proteins ranging in size from 19 to 180 kilodaltons and in pI from 4.4 to 6.8 were seen to have incorporated radiolabel. Five of these proteins showed a robust increase in incorporation of phosphate during regeneration. Among the latter was an acidic (pI 4.5) 45-kilodalton protein, which has previously been shown to be conveyed by fast axonal transport and to increase dramatically in its rate of synthesis during regeneration of goldfish optic axons.

  2. Tooth enamel hypoplasia in PHACE syndrome.

    Science.gov (United States)

    Chiu, Yvonne E; Siegel, Dawn H; Drolet, Beth A; Hodgson, Brian D

    2014-01-01

    Individuals with PHACE syndrome (posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, sternal cleft, and supraumbilical raphe syndrome) have reported dental abnormalities to their healthcare providers and in online forums, but dental involvement has not been comprehensively studied. A study was conducted at the third PHACE Family Conference, held in Milwaukee, Wisconsin, in July 2012. A pediatric dentist examined subjects at enrollment. Eighteen subjects were enrolled. The median age was 4.2 years (range 9 mos-9 yrs; 14 girls, 4 boys). Eleven of 18 patients had intraoral hemangiomas and five of these (50%) had hypomature enamel hypoplasia. None of the seven patients without intraoral hemangiomas had enamel hypoplasia. No other dental abnormalities were seen. Enamel hypoplasia may be a feature of PHACE syndrome when an intraoral hemangioma is present. Enamel hypoplasia increases the risk of caries, and clinicians should refer children with PHACE syndrome to a pediatric dentist by 1 year of age. © 2014 Wiley Periodicals, Inc.

  3. Quantitative assessment of optic nerve head pallor

    International Nuclear Information System (INIS)

    Vilser, W; Seifert, B U; Riemer, T; Nagel, E; Weisensee, J; Hammer, M

    2008-01-01

    Ischaemia, loss of neural tissue, glial cell activation and tissue remodelling are symptoms of anterior ischaemic as well as glaucomatous optic neuropathy leading to pallor of the optic nerve head. Here, we describe a simple method for the pallor measurement using a fundus camera equipped with a colour CCD camera and a special dual bandpass filter. The reproducibility of the determined mean pallor value was 11.7% (coefficient of variation for repeated measurements in the same subject); the variation over six healthy subjects was 14.8%. A significant difference between the mean pallor of an atrophic disc and that of the contralateral eye of the same individual was found. However, even the clinically unaffected eye showed a significantly increased pallor compared to the mean of the healthy control group. Thus, optic disc pallor measurement, as described here, may be helpful in the early detection and follow-up of optic neuropathy

  4. Human umbilical cord blood stem cells and brain-derived neurotrophic factor for optic nerve injury: a biomechanical evaluation

    Directory of Open Access Journals (Sweden)

    Zhong-jun Zhang

    2015-01-01

    Full Text Available Treatment for optic nerve injury by brain-derived neurotrophic factor or the transplantation of human umbilical cord blood stem cells has gained progress, but analysis by biomechanical indicators is rare. Rabbit models of optic nerve injury were established by a clamp. At 7 days after injury, the vitreous body received a one-time injection of 50 μg brain-derived neurotrophic factor or 1 × 10 6 human umbilical cord blood stem cells. After 30 days, the maximum load, maximum stress, maximum strain, elastic limit load, elastic limit stress, and elastic limit strain had clearly improved in rabbit models of optical nerve injury after treatment with brain-derived neurotrophic factor or human umbilical cord blood stem cells. The damage to the ultrastructure of the optic nerve had also been reduced. These findings suggest that human umbilical cord blood stem cells and brain-derived neurotrophic factor effectively repair the injured optical nerve, improve biomechanical properties, and contribute to the recovery after injury.

  5. Optic nerve oxygen tension in pigs and the effect of carbonic anhydrase inhibitors

    DEFF Research Database (Denmark)

    Stefánsson, E; Jensen, P K; Eysteinsson, T

    1999-01-01

    To evaluate how the oxygen tension of the optic nerve (ONP(O)2) is affected by the administration of the carbonic anhydrase inhibitors dorzolamide and acetazolamide and by alterations in oxygen and carbon dioxide in the breathing mixture.......To evaluate how the oxygen tension of the optic nerve (ONP(O)2) is affected by the administration of the carbonic anhydrase inhibitors dorzolamide and acetazolamide and by alterations in oxygen and carbon dioxide in the breathing mixture....

  6. Case Report: Bilateral iris, choroid, optic nerve colobomas and ...

    African Journals Online (AJOL)

    Background: Baraitser–Winter syndrome (BRWS) is a malformation syndrome, characterized by facial dysmorphism, ocular colobomata, pachygyria, and intellectual defects. Case report: A 3.5 year old female child with BRWS has bilateral congenital ptosis, microcornea, iris, choroid, and optic nerve coloboma, retinal ...

  7. Congenital pits of the optic nerve head and retinochoroidal colobomas.

    Science.gov (United States)

    Brown, G C; Augsburger, J J

    1980-07-01

    A 45-year-old man presented with bilateral retinochoroidal colobomas associated with an inferonasal congenital pit of the optic nerve head in each eye. Different theories on the origin of such pits are discussed. This case supports the theory that some congenital optic pits occur as a result of failure of closure of the superior end of the embryonic fissure.

  8. Developmental abnormalities of the optic nerve head in mouse fetuses caused by simultaneous irradiation of x-rays and ultrasound

    International Nuclear Information System (INIS)

    Shirai, Shoichiro; Yuguchi, Shuji; Majima, Akio.

    1981-01-01

    Eye abnormalities in mouse fetuses caused by irradiation of X-rays alone, or simultaneous irradiation of X-rays and ultrasound on day 7 of gestation were histologically studied on day 18 of gestation. Developmental abnormalities of the optic nerve head were examined in the present experiment, and the following results were obtained: 1. Developmental abnormalities of the optic nerve head associated with developmental abnormalities of the vitreous were detected in 4 fetuses (5 eyes). In all cases, excessive mesenchymal tissue of components of the primary vitreous was found from the optic nerve head to the vitreous cavity. It was impossible to distinguish between the neuroectodermal tissue of Bergmeister's papilla and the mesodermal tissue of components of the primary vitreous. 2. In 3 fetuses (4 eyes), the fetal fissure involving the optic nerve head was open. At the peripapillary region, the inner layer of the optic cup was everted and hyperplastic. The inner neuroblastic layer of the everted portion contacted the outer coat of the eyeball, directly. In these cases, the optic nerve entrance was very wide. 3. The relation between the congenital optic nerve head anomalies encountered clinically and those observed experimentally in the mouse fetuses was discussed. It was considered that the pathogenesis of congenital optic nerve head anomalies consists of the malformation of the primitive epithelial papilla, the faulty closure of the proximal end of the fetal fissure, the anomalies of Bergmeister's papilla, the anomalies of the hyaloid system, or the abnormal differentiation and growth of the neuroectodermal cells of the optic cup. (author)

  9. Retinal glutamate transporter changes in experimental glaucoma and after optic nerve transection in the rat.

    Science.gov (United States)

    Martin, Keith R G; Levkovitch-Verbin, Hana; Valenta, Danielle; Baumrind, Lisa; Pease, Mary Ellen; Quigley, Harry A

    2002-07-01

    High levels of glutamate can be toxic to retinal ganglion cells. Effective buffering of extracellular glutamate by retinal glutamate transporters is therefore important. This study was conducted to investigate whether glutamate transporter changes occur with two models of optic nerve injury in the rat. Glaucoma was induced in one eye of 35 adult Wistar rats by translimbal diode laser treatment to the trabecular meshwork. Twenty-five more rats underwent unilateral optic nerve transection. Two glutamate transporters, GLAST (EAAT-1) and GLT-1 (EAAT-2), were studied by immunohistochemistry and quantitative Western blot analysis. Treated and control eyes were compared 3 days and 1, 4, and 6 weeks after injury. Optic nerve damage was assessed semiquantitatively in epoxy-embedded optic nerve cross sections. Trabecular laser treatment resulted in moderate intraocular pressure (IOP) elevation in all animals. After 1 to 6 weeks of experimental glaucoma, all treated eyes had significant optic nerve damage. Glutamate transporter changes were not detected by immunohistochemistry. Western blot analysis demonstrated significantly reduced GLT-1 in glaucomatous eyes compared with control eyes at 3 days (29.3% +/- 6.7%, P = 0.01), 1 week (55.5% +/- 13.6%, P = 0.02), 4 weeks (27.2% +/- 10.1%, P = 0.05), and 6 weeks (38.1% +/- 7.9%, P = 0.01; mean reduction +/- SEM, paired t-tests, n = 5 animals per group, four duplicate Western blot analyses per eye). The magnitude of the reduction in GLT-1 correlated significantly with mean IOP in the glaucomatous eye (r(2) = 0.31, P = 0.01, linear regression). GLAST was significantly reduced (33.8% +/- 8.1%, mean +/- SEM) after 4 weeks of elevated IOP (P = 0.01, paired t-test, n = 5 animals per group). In contrast to glaucoma, optic nerve transection resulted in an increase in GLT-1 compared with the control eye (P = 0.01, paired t-test, n = 15 animals). There was no significant change in GLAST after transection. GLT-1 and GLAST were significantly

  10. Anatomic relationship of the optic nerve channel with sphenoid al sinus: a computed tomography study

    International Nuclear Information System (INIS)

    Dias, Paulo Cesar J.; Albernaz, Pedro Luiz M.; Yamashida, Helio K.

    2004-01-01

    The sphenoid sinus, out of the facial sinuses, is certainly the most neglected as far as diagnosis is concerned. The surgical approach requires a detailed anatomical knowledge, considering the serious complications resulting from injuries of vital structures adjacent to this region. Aim: the objective of our research is to evaluate the anatomic relationship of the optic nerve with the sphenoid sinus making use of the computed tomography. Study design: series report. Material and method: the authors present a retrospective analysis of 203 computed tomographies of facial sinus belonging to individuals of both sexes aged 14 and over. The examinations were evaluated observing the course of the optic nerve, obtained through the degree of its projection on the wall of the sphenoid sinus. The method used was the modified classification of Delano, evaluating the absence of bone reduction (dehiscence) of the optic nerve in the sphenoid sinus. We analysed the degree of pneumatization of the sphenoid sinus, using Hammer's classification adapted by Guerrero, apart from the pneumatization of the anterior clinoidal process and pterigoid and the presence of the Onodi cell. Results: most of the patients (78.96%) presented their optic nerve with a type 1 course. Type 2 was observed in 16.83% of the patients, type 3 in 3.47% and type 4 in 0.74%. The presence of dehiscence of the optic nerve in the wall of the sphenoid sinus was evidenced in 21.29% of the cases. Related to pneumatization, we noticed that the pre-selar type was observed in 6.44%, the post-selar type appeared in 39.11%, the selar type appeared in 54.45%, and the apneumatized type was not observed in any of the cases. The pneumatization of the anterior clinoidal process was verified in 10.64% of the cases, while the pterigoid process was apparent in 21.29% of the cases. The Onodi cell (sphenoetmoidal) was found in 7.92% of the cases. Conclusion: the presence of dehiscence of the optic nerve is related with the degree of

  11. High-resolution imaging of retinal nerve fiber bundles in glaucoma using adaptive optics scanning laser ophthalmoscopy.

    Science.gov (United States)

    Takayama, Kohei; Ooto, Sotaro; Hangai, Masanori; Ueda-Arakawa, Naoko; Yoshida, Sachiko; Akagi, Tadamichi; Ikeda, Hanako Ohashi; Nonaka, Atsushi; Hanebuchi, Masaaki; Inoue, Takashi; Yoshimura, Nagahisa

    2013-05-01

    To detect pathologic changes in retinal nerve fiber bundles in glaucomatous eyes seen on images obtained by adaptive optics (AO) scanning laser ophthalmoscopy (AO SLO). Prospective cross-sectional study. Twenty-eight eyes of 28 patients with open-angle glaucoma and 21 normal eyes of 21 volunteer subjects underwent a full ophthalmologic examination, visual field testing using a Humphrey Field Analyzer, fundus photography, red-free SLO imaging, spectral-domain optical coherence tomography, and imaging with an original prototype AO SLO system. The AO SLO images showed many hyperreflective bundles suggesting nerve fiber bundles. In glaucomatous eyes, the nerve fiber bundles were narrower than in normal eyes, and the nerve fiber layer thickness was correlated with the nerve fiber bundle widths on AO SLO (P fiber layer defect area on fundus photography, the nerve fiber bundles on AO SLO were narrower compared with those in normal eyes (P optic disc, the nerve fiber bundle width was significantly lower, even in areas without nerve fiber layer defect, in eyes with glaucomatous eyes compared with normal eyes (P = .026). The mean deviations of each cluster in visual field testing were correlated with the corresponding nerve fiber bundle widths (P = .017). AO SLO images showed reduced nerve fiber bundle widths both in clinically normal and abnormal areas of glaucomatous eyes, and these abnormalities were associated with visual field defects, suggesting that AO SLO may be useful for detecting early nerve fiber bundle abnormalities associated with loss of visual function. Copyright © 2013 Elsevier Inc. All rights reserved.

  12. Significant correlations between optic nerve head microcirculation and visual field defects and nerve fiber layer loss in glaucoma patients with myopic glaucomatous disk

    Directory of Open Access Journals (Sweden)

    Yokoyama Y

    2011-12-01

    Full Text Available Yu Yokoyama, Naoko Aizawa, Naoki Chiba, Kazuko Omodaka, Masahiko Nakamura, Takaaki Otomo, Shunji Yokokura, Nobuo Fuse, Toru NakazawaDepartment of Ophthalmology, Tohoku University Graduate School of Medicine, Sendai, JapanBackground: Eyes with glaucoma are characterized by optic neuropathy with visual field defects in the areas corresponding to the optic disk damage. The exact cause for the glaucomatous optic neuropathy has not been determined. Myopia has been shown to be a risk factor for glaucoma. The purpose of this study was to determine whether a significant correlation existed between the microcirculation of the optic disk and the visual field defects and the retinal nerve fiber layer thickness (RNFLT in glaucoma patients with myopic optic disks.Methods: Sixty eyes of 60 patients with myopic disks were studied; 36 eyes with glaucoma (men:women = 19:17 and 24 eyes with no ocular diseases (men:women = 14:10. The mean deviation (MD determined by the Humphrey field analyzer, and the peripapillary RNFLT determined by the Stratus-OCT were compared between the two groups. The ocular circulation was determined by laser speckle flowgraphy (LSFG, and the mean blur rate (MBR was compared between the two groups. The correlations between the RNFLT and MBR of the corresponding areas of the optic disk and between MD and MBR of the optic disk in the glaucoma group were determined by simple regression analyses.Results: The average MBR for the entire optic disk was significantly lower in the glaucoma group than that in the control group. The differences of the MBR for the tissue in the superior, inferior, and temporal quadrants of the optic disk between the two groups were significant. The MBR for the entire optic disk was significantly correlated with the MD (r = 0.58, P = 0.0002 and the average RNFLT (r = 0.53, P = 0.0008. The tissue MBR of the optic disk was significantly correlated with the RNFLT in the superior, inferior, and temporal quadrants

  13. CT diagnosis of optic nerve lesions in children

    International Nuclear Information System (INIS)

    Havlova, M.; Kvicala, V.; Nevsimalova, S.; Otradovec, J.

    1985-01-01

    Computer tomography (CT) was used to examine 17 children with lesions of the optic nerve. The procedure is described of the examination of the orbit and the sellar area. CT gave a very good image of expansive processes in both areas and of associated lesions of the CNS insofar as they were based on anatomical or pathological changes. (author)

  14. MR assessment of fetal pulmonary hypoplasia

    Energy Technology Data Exchange (ETDEWEB)

    Kuwashima, Shigeko; Kohno, Atsushi; Saiki, Natoru; Iimura, Fumitoshi; Kohno, Tatsuo; Hashimoto, Teisuke; Fujioka, Mutsuhisa [Dokkyo Univ. School of Medicine, Mibu, Tochigi (Japan)

    2000-08-01

    The purpose of this study is to evaluate pulmonary hypoplasia of the fetus using MRI. The subjects consisted of 36 fetuses (18 to 40 weeks' gestation). All fetuses or mothers had major anomalies diagnosed on fetal ultrasonography. MR imaging was performed with a 1.5-T magnet and HASTE (half-Fourier acquisition single-shot turbo spin-echo) sequence. MR images were evaluated with special attention to the intensity of the lung. A diagnosis of pulmonary hypoplasia was based on the clinical, surgical, and autopsy findings. All fetuses with normal pulmonary development showed high intensity in the lung, while all fetuses with pulmonary hypoplasia showed a low intensity in the lung, obscured pulmonary vessels and a small thorax. There was a close correlation between the lung intensity and pulmonary growth. MR assessment of lung intensity may facilitate the diagnosis of pulmonary hypoplasia, particularly after 26 weeks' gestation. Some of the normally developing lung showed a low intensity from 20 to 24 weeks of gestational age. The change to normal lung intensity may occur during this period. (author)

  15. Biomechanics of the Optic Nerve Sheath in VIIP Syndrome

    Science.gov (United States)

    Ethier, C. Ross; Raykin, Julia; Gleason, Rudy; Mulugeta, Lealem; Myers, Jerry; Nelson, Emily; Samuels, Brian C.

    2014-01-01

    Long-duration space flight carries the risk of developing Visual Impairment and Intracranial Pressure (VIIP) syndrome, a spectrum of ophthalmic changes including posterior globe flattening, choroidal folds, distension of the optic nerve sheath (ONS), optic nerve kinking and potentially permanent degradation of visual function. The slow onset of VIIP, its chronic nature, and certain clinical features strongly suggest that biomechanical factors acting on the ONS play a role in VIIP. Here we measure several relevant ONS properties needed to model VIIP biomechanics. The ONS (meninges) of fresh porcine eyes (n7) was reflected, the nerve proper was truncated near the sclera, and the meninges were repositioned to create a hollow cylinder of meningeal connective tissue attached to the posterior sclera. The distal end was cannulated, sealed, and pressure clamped (mimicking cerebrospinal fluid [CSF] pressure), while the eye was also cannulated for independent control of intraocular pressure (IOP). The meninges were inflated (CSF pressure cycling 7-50 mmHg) while ONS outer diameter was imaged. In another set of experiments (n4), fluid permeation rate across the meninges was recorded by observing the drainage of an elevated fluid reservoir (30 mmHg) connected to the meninges. The ONS showed behavior typical of soft tissues: viscoelasticity, with hysteresis in early preconditioning cycles and repeatable behavior after 4 cycles, and nonlinear stiffening, particularly at CSF pressures 15 mmHg (Figure). Tangent moduli measured from the loading curve were 372 101, 1199 358, and 2050 379 kPa (mean SEM) at CSF pressures of 7, 15 and 30 mmHg, respectively. Flow rate measurements through the intact meninges at 30mmHg gave a permeability of 1.34 0.46 lmincm2mmHg (mean SEM). The ONS is a tough, strain-stiffening connective tissue that is surprisingly permeable. The latter observation suggests that there could be significant CSF drainage through the ONS into the orbit, likely important

  16. Optic Nerve Sheath Mechanics and Permeability in VIIP Syndrome

    Science.gov (United States)

    Raykin, Julia; Best, Lauren; Gleason, Rudy; Mulugeta, Lealem; Myers, Jerry; Nelson, Emily; Samuels, Brian C.; Ethier, C. R.

    2014-01-01

    Long-duration space flight carries the risk of developing Visual Impairment and Intracranial Pressure (VIIP) syndrome, a spectrum of ophthalmic changes including posterior globe flattening, choroidal folds, distension of the optic nerve sheath (ONS), optic nerve kinking and potentially permanent degradation of visual function. The slow onset of VIIP, its chronic nature, and certain clinical features strongly suggest that biomechanical factors acting on the ONS play a role in VIIP. Here we measure several relevant ONS properties needed to model VIIP biomechanics. The ONS (meninges) of fresh porcine eyes (n7) was reflected, the nerve proper was truncated near the sclera, and the meninges were repositioned to create a hollow cylinder of meningeal connective tissue attached to the posterior sclera. The distal end was cannulated, sealed, and pressure clamped (mimicking cerebrospinal fluid [CSF] pressure), while the eye was also cannulated for independent control of intraocular pressure (IOP). The meninges were inflated (CSF pressure cycling 7-50 mmHg) while ONS outer diameter was imaged. In another set of experiments (n4), fluid permeation rate across the meninges was recorded by observing the drainage of an elevated fluid reservoir (30 mmHg) connected to the meninges. The ONS showed behavior typical of soft tissues: viscoelasticity, with hysteresis in early preconditioning cycles and repeatable behavior after 4 cycles, and nonlinear stiffening, particularly at CSF pressures 15 mmHg (Figure). Tangent moduli measured from the loading curve were 372 101, 1199 358, and 2050 379 kPa (mean SEM) at CSF pressures of 7, 15 and 30 mmHg, respectively. Flow rate measurements through the intact meninges at 30mmHg gave a permeability of 1.34 0.46 lmincm2mmHg (mean SEM). The ONS is a tough, strain-stiffening connective tissue that is surprisingly permeable. The latter observation suggests that there could be significant CSF drainage through the ONS into the orbit, likely important

  17. Nerve Fiber Flux Analysis Using Wide-Field Swept-Source Optical Coherence Tomography.

    Science.gov (United States)

    Tan, Ou; Liu, Liang; Liu, Li; Huang, David

    2018-02-01

    To devise a method to quantify nerve fibers over their arcuate courses over an extended peripapillary area using optical coherence tomography (OCT). Participants were imaged with 8 × 8-mm volumetric OCT scans centered at the optic disc. A new quantity, nerve fiber flux (NFF), represents the cross-sectional area transected perpendicular to the nerve fibers. The peripapillary area was divided into 64 tracks with equal flux. An iterative algorithm traced the trajectory of the tracks assuming that the relative distribution of the NFF was conserved with compensation for fiber connections to ganglion cells on the macular side. Average trajectory was averaged from normal eyes and use to calculate the NFF maps for glaucomatous eyes. The NFF maps were divided into eight sectors that correspond to visual field regions. There were 24 healthy and 10 glaucomatous eyes enrolled. The algorithm converged on similar patterns of NFL tracks for all healthy eyes. In glaucomatous eyes, NFF correlated with visual field sensitivity in the arcuate sectors (Spearman ρ = 0.53-0.62). Focal nerve fiber loss in glaucomatous eyes appeared as uniform tracks of NFF defects that followed the expected arcuate fiber trajectory. Using an algorithm based on the conservation of flux, we derived nerve fiber trajectories in the peripapillary area. The NFF map is useful for the visualization of focal defects and quantification of sector nerve fiber loss from wide-area volumetric OCT scans. NFF provides a cumulative measure of volumetric loss along nerve fiber tracks and could improve the detection of focal glaucoma damage.

  18. Expansions of the neurovascular scleral canal and contained optic nerve occur early in the hypertonic saline rat experimental glaucoma model.

    Science.gov (United States)

    Pazos, Marta; Yang, Hongli; Gardiner, Stuart K; Cepurna, William O; Johnson, Elaine C; Morrison, John C; Burgoyne, Claude F

    2016-04-01

    To characterize early optic nerve head (ONH) structural change in rat experimental glaucoma (EG). Unilateral intraocular pressure (IOP) elevation was induced in Brown Norway rats by hypertonic saline injection into the episcleral veins and animals were sacrificed 4 weeks later by perfusion fixation. Optic nerve cross-sections were graded from 1 (normal) to 5 (extensive injury) by 5 masked observers. ONHs with peripapillary retina and sclera were embedded, serial sectioned, 3-D reconstructed, delineated, and quantified. Overall and animal-specific EG versus Control eye ONH parameter differences were assessed globally and regionally by linear mixed effect models with significance criteria adjusted for multiple comparisons. Expansions of the optic nerve and surrounding anterior scleral canal opening achieved statistical significance overall (p < 0.0022), and in 7 of 8 EG eyes (p < 0.005). In at least 5 EG eyes, significant expansions (p < 0.005) in Bruch's membrane opening (BMO) (range 3-10%), the anterior and posterior scleral canal openings (8-21% and 5-21%, respectively), and the optic nerve at the anterior and posterior scleral canal openings (11-30% and 8-41%, respectively) were detected. Optic nerve expansion was greatest within the superior and inferior quadrants. Optic nerve expansion at the posterior scleral canal opening was significantly correlated to optic nerve damage (R = 0.768, p = 0.042). In the rat ONH, the optic nerve and surrounding BMO and neurovascular scleral canal expand early in their response to chronic experimental IOP elevation. These findings provide phenotypic landmarks and imaging targets for detecting the development of experimental glaucomatous optic neuropathy in the rat eye. Copyright © 2015 Elsevier Ltd. All rights reserved.

  19. Foveal hemorrhage in an eye with foveal hypoplasia associated with albinism

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    Masuda N

    2014-09-01

    Full Text Available Naonori Masuda, Taiji Hasegawa, Mariko Yamashita, Nahoko Ogata Department of Ophthalmology, Nara Medical University, Nara, Japan Abstract: Oculocutaneous albinism is a group of congenital disorders caused by alterations of melanin biosynthesis. We report our findings in a patient with oculocutaneous albinism who presented with foveal hypoplasia and a foveal hemorrhage. A 48-year-old man noted a dark spot in the middle of the visual field of his right eye. He had depigmented skin, white hair, white eyebrows, and white cilia. He also had horizontal nystagmus and depigmented irides. His best-corrected visual acuity was 2/100 with -14.0 diopters in the right eye and 3/100 with -5.0 diopters in the left eye. Ophthalmoscopy showed diffuse depigmentation in both eyes and a foveal hemorrhage in the right eye. Optical coherence tomography showed the absence of a foveal pit in both eyes and a subretinal hyperreflective lesion corresponding to the foveal hemorrhage in the right eye. Fluorescein angiography showed that the retinal and choroidal vessels were relatively hypofluorescent because of the lack of a blocking effect of the pigments in the retinal pigment epithelium. Fluorescein angiography and indocyanine green angiography did not show any evidence of choroidal neovascularization in either eye. The foveal hemorrhage in the right eye spontaneously regressed and finally resolved at 3 months after onset. At the final examination, the patient reported that his vision had recovered. A foveal hemorrhage is a rare condition in an eye with foveal hypoplasia associated with albinism. The hemorrhage may be related to high myopia and also to the hypoplasia of the fovea associated with albinism. Keywords: albinism, foveal hemorrhage, foveal hypoplasia, simple hemorrhage

  20. Incidence of enhancement of the optic nerve/sheath complex in fat-suppression orbit MRI

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Ho Kyu; Yoon, Kwon Ha; Choi, Choong Gon; Suh, Dae Chul [Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea, Republic of)

    1995-04-15

    To elucidate the incidence of Gd-DTPA enhancement of the optic nerve/sheath complex (ONC) in patients with various ophthalmopathies using fat-suppression MRI. Orbit MRI with fat-suppression technique (ChemSat) was performed in 58 patients with normal and various orbital lesions. The fat-suppression MR was done with and without Gd-DTPA injection in all cases. MR findings were reviewed retrospectively in a blind fashion with respect to presence or absence of contrast enhancement of the ONC. Contrast enhancement of the ONC was seen in 86% (6/7) of cavernous sinus lesions, 80% (8/10) of intraconal lesions excluding the ONC, 57% (16/28) of ONC lesions, 38% (3/8) of ocular lesions, and 2% (1/55) of normal orbits. The ONC enhancement was the most common in optic nerve/sheath tumors (10/10), and pseudotumors (6/6), cavernous sinus dural arteriovenous malformations (3/3) and cavernous sinus thrombosis (2/2), and less frequently seen in optic neuritis (3/14). Enhancement of the ONC may be seen in lesions of the cavernous sinus and orbit other than optic nerve/sheath lesion.

  1. Study of optic nerve in patients with neuromyelitis optica using diffusion tensor imaging

    International Nuclear Information System (INIS)

    Chen Zhiye; Zhu Lijun; Lou Xin; Li Jinfeng; Yang Yang; Ma Lin

    2012-01-01

    Objective: To explore the diagnostic value of optic diffusion tensor imaging (DTI) in detecting the impairment of optic nerve in neuromyelitis optica (NMO) patients. Methods: Conventional MRI and optic DTI were performed in 28 NMO patients and 38 normal controls (NC). Fractional anisotropy (FA) values were measured in the anterior part, middle part and posterior part of intraorbital segment of optic nerve. The patients were classified into 3 groups based on the impairment of vision and visual evoked potential (VEP): monocular impairment (MI) group, 10 eyes; biocular impairment (BI) group, 36 eyes; and normal-appearing (NA) group, 10 eyes. All patients were performed with the evaluation of expanded disability status scale (EDSS). One-way analysis of variance (ANOVA), receiver operating characteristic (ROC) curve, and Spearman correlation analysis were performed among the subgroups of NMO and normal controls. Results: There was significantly statistical difference between the four groups (F=43.54, P<0.01). Decreased FA values were demonstrated in the MI group (0.29 ±0.08), BI group (0.27 ±0.08), and NA group (0.35 ±0.13) compared with NC (0.45 ±0.07) (P<0.01). FA value in BI group was significantly lower than that of NA group (P<0.01). Area under curve by ROC analysis in NC vs MI, NC vs BI, NC vs NA, and NC vs NMO was 0.92, 0.95, 0.74, and 0.91, respectively. The diagnostic sensitivity of ROC was 80%, 86%, 50%, and 79%, respectively. The diagnostic specificity of ROC was 95% for the each compared groups. FA value showed no correlation with EDSS for each NMO groups, and showed negative correlation with disease duration for BI group (r=-0.371, P<0.05). Conclusions: Various degrees of optic nerve injuries, indicated by decreased FA value,are present in NMO patients, and optic DTI may be a simple and effective tool for the quantitative evaluation of optic nerve in NMO patients. (authors)

  2. Cholinergic Potentiation of Restoration of Visual Function after Optic Nerve Damage in Rats

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    Mira Chamoun

    2017-01-01

    Full Text Available Enhancing cortical plasticity and brain connectivity may improve residual vision following a visual impairment. Since acetylcholine plays an important role in attention and neuronal plasticity, we explored whether potentiation of the cholinergic transmission has an effect on the visual function restoration. To this end, we evaluated for 4 weeks the effect of the acetylcholinesterase inhibitor donepezil on brightness discrimination, visually evoked potentials, and visual cortex reactivity after a bilateral and partial optic nerve crush in adult rats. Donepezil administration enhanced brightness discrimination capacity after optic nerve crush compared to nontreated animals. The visually evoked activation of the primary visual cortex was not restored, as measured by evoked potentials, but the cortical neuronal activity measured by thallium autometallography was not significantly affected four weeks after the optic nerve crush. Altogether, the results suggest a role of the cholinergic system in postlesion cortical plasticity. This finding agrees with the view that restoration of visual function may involve mechanisms beyond the area of primary damage and opens a new perspective for improving visual rehabilitation in humans.

  3. Peripapillary Choroidal Neovascularization Associated with Optic Nerve Head Drusen Treated with Anti-VEGF Agents

    Directory of Open Access Journals (Sweden)

    Norman A. Saffra

    2015-02-01

    Full Text Available Optic nerve head drusen can be associated with peripapillary choroidal neovascularization, in both the pediatric and adult population. These membranes can involve the macula, causing significant visual loss. Herein, we present a case that required treatment with an anti-VEGF agent. The patient failed to respond to the initial agent, but subsequently responded to a change of agent. Adult patients with macular degeneration involving peripapillary choroidal neovascularization associated with optic nerve head drusen may require individualized treatment plans.

  4. Trans-eyebrow supraorbital approach in large suprasellar craniopharyngioma surgery in adults: analysis of optic nerve length and extent of tumor resection. Original article.

    Science.gov (United States)

    Prat, Ricardo; Galeano, Inma; Evangelista, Rocío; Pancucci, Giovanni; Guarín, Juliana; Ayuso, Angel; Misra, Mukesh

    2017-05-01

    One of the main drawbacks in the surgery of large craniopharyngiomas is the presence of a prefixed optic chiasm. Our main objective in this study is to compare the predictive value of the optic nerve length and optic chiasm location on large craniopharyngiomas' extent of resection. We retrospectively studied 21 consecutive patients with large craniopharyngiomas who underwent tumor resection through the trans-eyebrow supraorbital approach. Clinical and radiological findings on preoperative MRI were recorded, including the optic chiasm location classified as prefixed, postfixed or normal. We registered the optic nerve length measured intraoperatively prior to tumor removal and confirmed the measurements on preoperative MRI. Using a linear regression model, we calculated a prediction formula of the percentage of the extent of resection as a function of optic nerve length. On preoperative MRI, 15 patients were considered to have an optic chiasm in a normal location, 3 cases had a prefixed chiasm, and the remaining 3 had a postfixed chiasm. In the group with normal optic chiasm location, a wide range of percentage of extent of resection was observed (75-100%). The percentage of extent of resection of large craniopharyngiomas was observed to be dependent on the optic nerve length in a linear regression model (p < 0.0001). According to this model in the normal optic chiasm location group, we obtained an 87% resection in 9-mm optic nerve length patients, a 90.5% resection in 10-mm optic nerve length patients and 100% resection in 11-mm optic nerve length patients. Optic chiasm location provides useful information to predict the percentage of resection in both prefixed and postfixed chiasm patients but not in the normal optic chiasm location group. Optic nerve length was proven to provide a more accurate way to predict the percentage of resection than the optic chiasm location in the normal optic chiasm location group.

  5. The utility of three-dimensional optical projection tomography in nerve injection injury imaging

    Czech Academy of Sciences Publication Activity Database

    Cvetko, E.; Čapek, Martin; Damjanovska, M.; Reina, M. A.; Eržen, I.; Stopar-Pintarič, T.

    2015-01-01

    Roč. 70, č. 8 (2015), s. 939-947 ISSN 0003-2409 R&D Projects: GA ČR(CZ) GA13-12412S; GA MŠk(CZ) LH13028 Institutional support: RVO:67985823 Keywords : optical projection tomography * 3D nerve visualization * nerve disruption Subject RIV: EA - Cell Biology Impact factor: 3.794, year: 2015

  6. Optic nerve head pit-associated maculopathy

    International Nuclear Information System (INIS)

    Beltrán Sainz, Raisa Ivis; Hernández Baguer, Raisa; Gonzales Díaz, Rafael Ernesto; Hernández Martínez, Rocío; Galindo Reimond, Kenia

    2016-01-01

    A literature review was made to expand knowledge on the optic nerve head pit-related maculopathy, its physiopathology and main manifestations, clinical and angiographic diagnosis, optical coherence tomography, and to describe some of the treatments recently used worldwide. The current therapeutic tendencies were taken into account. The fundamental sources of information were scientific articles from journals in PubMED and Cochrane databases as well as basic texts which dealt with this topic in the last five years through Google search engine. Despite the rare occurrence of the disease, its management may be difficult particularly in macular effect cases. This paper confirmed that most of the studies used small samples, were retrospective, non-comparative and non-randomized. However, some therapeutic modalities were found, which have been used for years and also descriptions of new techniques that require further research. There is no consensus on the ideal treatment protocol for this disease. (author)

  7. [Effectiveness of magnetotherapy in optic nerve atrophy. A preliminary study].

    Science.gov (United States)

    Zobina, L V; Orlovskaia, L S; Sokov, S L; Sabaeva, G F; Kondé, L A; Iakovlev, A A

    1990-01-01

    Magnetotherapy effects on visual functions (vision acuity and field), on retinal bioelectric activity, on conductive vision system, and on intraocular circulation were studied in 88 patients (160 eyes) with optic nerve atrophy. A Soviet Polyus-1 low-frequency magnetotherapy apparatus was employed with magnetic induction of about 10 mT, exposure 7-10 min, 10-15 sessions per course. Vision acuity of patients with its low (below 0.04 diopters) values improved in 50 percent of cases. The number of patients with vision acuity of 0.2 diopters has increased from 46 before treatment to 75. Magnetotherapy improved ocular hemodynamics in patients with optic nerve atrophy, it reduced the time of stimulation conduction along the vision routes and stimulated the retinal ganglia cells. The maximal effect was achieved after 10 magnetotherapy sessions. A repeated course carried out in 6-8 months promoted a stabilization of the process.

  8. Three-dimensional imaging of the optic nerve using magnetic resonance angiography. Application to anterior communicating artery aneurysm and craniopharingioma

    International Nuclear Information System (INIS)

    Okuyama, Tohru; Fukuyama, Atsushi; Fukuyama, Koichi; Ikeno, Kunihiro; Araki, Hiroyuki; Okada, Kinya; Sohma, Noriko

    2005-01-01

    The purpose of this investigation was to analyze three-dimensional images of the optic nerve obtained by magnetic resonance angiography (MRA) in cases of anterior communicating artery aneurysm and craniopharingioma. Four ruptured anterior communicating artery aneurysms, five non-ruptured anterior communicating artery aneurysms and two craniopharingiomas were examined. The images were taken using MR/i Hispeed Plus 1.5 T Infinity version, and analyzed by Advantage Work station AW4.1. The routine MR imaging parameters are shown in Table. The imaging time was about 10 minutes. Analysis was made by reformation of images parallel to the optic nerve obtained from the original MRA images. The optic nerve and brain tumor were traced with paintbrush from one sheet to another of the reformed images after subtraction of the blood vessels around the anterior communicating artery in these reformed images, and then three-dimensional images were constructed. Three-dimensional images of the blood vessels were reconstructed from MIP (maximum intensity projection) images using the threshold method. The optic nerve and anterior communicating arterial aneurysm or brain tumor were both observed in the overlapped 3D-SSD (shaded surface display) images. The analysis time was about 15 minutes. Three-dimensional images of the optic nerve and anterior communicating artery aneurysm or brain tumor were able to be made in all cases. As a preoperative investigation for anterior communicating artery aneurysm or suprasellar brain tumor, we considered that three-dimensional imaging of the optic nerve is useful in the operative approach because the optic nerve acts as a merkmal for the anterior communicating aneurysm or brain tumor. (author)

  9. Is the term "fasciculus opticus cerebralis" more justifiable than the term "optic nerve"?

    Science.gov (United States)

    Vojniković, Bojo; Bajek, Snjezana; Bajek, Goran; Strenja-Linić, Ines; Grubesić, Aron

    2013-04-01

    The terminology of the optic nerve had already been changed three times, since 1895 until 1955 when the term "nervus opticus" was introduced in the "Terminologia Anatomica". Following our study we claim that, from the aspect of phylogenetic evolution of binocular vision development as well as optical embryogenesis where opticus is evidently presented as a product of diencephalic structures, the addition of the term "nervus" to opticus is not adequate and justified. From the clinical aspect the term "nervus opticus" is also inadequate, both as a "nerve" that has no functional regenerative properties, unlike other cranial nerves, as well as from a pedagogical and didactical aspect of educating future physicians. We suggest that the term "Fasciculus Opticus Cerebralis" should be used as it much better explains the origin as well as its affiliation to the central nervous system.

  10. Proteomics analyses of human optic nerve head astrocytes following biomechanical strain.

    Science.gov (United States)

    Rogers, Ronan S; Dharsee, Moyez; Ackloo, Suzanne; Sivak, Jeremy M; Flanagan, John G

    2012-02-01

    We investigate the role of glial cell activation in the human optic nerve caused by raised intraocular pressure, and their potential role in the development of glaucomatous optic neuropathy. To do this we present a proteomics study of the response of cultured, optic nerve head astrocytes to biomechanical strain, the magnitude and mode of strain based on previously published quantitative models. In this case, astrocytes were subjected to 3 and 12% stretches for either 2 h or 24 h. Proteomic methods included nano-liquid chromatography, tandem mass spectrometry, and iTRAQ labeling. Using controls for both stretch and time, a six-plex iTRAQ liquid chromatography- tandem MS (LC/MS/MS) experiment yielded 573 proteins discovered at a 95% confidence limit. The pathways included transforming growth factor β1, tumor necrosis factor, caspase 3, and tumor protein p53, which have all been implicated in the activation of astrocytes and are believed to play a role in the development of glaucomatous optic neuropathy. Confirmation of the iTRAQ analysis was performed by Western blotting of various proteins of interest including ANXA 4, GOLGA2, and αB-Crystallin.

  11. Proteomics Analyses of Human Optic Nerve Head Astrocytes Following Biomechanical Strain*

    Science.gov (United States)

    Rogers, Ronan S.; Dharsee, Moyez; Ackloo, Suzanne; Sivak, Jeremy M.; Flanagan, John G.

    2012-01-01

    We investigate the role of glial cell activation in the human optic nerve caused by raised intraocular pressure, and their potential role in the development of glaucomatous optic neuropathy. To do this we present a proteomics study of the response of cultured, optic nerve head astrocytes to biomechanical strain, the magnitude and mode of strain based on previously published quantitative models. In this case, astrocytes were subjected to 3 and 12% stretches for either 2 h or 24 h. Proteomic methods included nano-liquid chromatography, tandem mass spectrometry, and iTRAQ labeling. Using controls for both stretch and time, a six-plex iTRAQ liquid chromatography- tandem MS (LC/MS/MS) experiment yielded 573 proteins discovered at a 95% confidence limit. The pathways included transforming growth factor β1, tumor necrosis factor, caspase 3, and tumor protein p53, which have all been implicated in the activation of astrocytes and are believed to play a role in the development of glaucomatous optic neuropathy. Confirmation of the iTRAQ analysis was performed by Western blotting of various proteins of interest including ANXA 4, GOLGA2, and αB-Crystallin. PMID:22126795

  12. Transgenic inhibition of astroglial NF-κB protects from optic nerve damage and retinal ganglion cell loss in experimental optic neuritis

    Directory of Open Access Journals (Sweden)

    Brambilla Roberta

    2012-09-01

    Full Text Available Abstract Background Optic neuritis is an acute, demyelinating neuropathy of the optic nerve often representing the first appreciable symptom of multiple sclerosis. Wallerian degeneration of irreversibly damaged optic nerve axons leads to death of retinal ganglion cells, which is the cause of permanent visual impairment. Although the specific mechanisms responsible for triggering these events are unknown, it has been suggested that a key pathological factor is the activation of immune-inflammatory processes secondary to leukocyte infiltration. However, to date, there is no conclusive evidence to support such a causal role for infiltrating peripheral immune cells in the etiopathology of optic neuritis. Methods To dissect the contribution of the peripheral immune-inflammatory response versus the CNS-specific inflammatory response in the development of optic neuritis, we analyzed optic nerve and retinal ganglion cells pathology in wild-type and GFAP-IκBα-dn transgenic mice, where NF-κB is selectively inactivated in astrocytes, following induction of EAE. Results We found that, in wild-type mice, axonal demyelination in the optic nerve occurred as early as 8 days post induction of EAE, prior to the earliest signs of leukocyte infiltration (20 days post induction. On the contrary, GFAP-IκBα-dn mice were significantly protected and showed a nearly complete prevention of axonal demyelination, as well as a drastic attenuation in retinal ganglion cell death. This correlated with a decrease in the expression of pro-inflammatory cytokines, chemokines, adhesion molecules, as well as a prevention of NAD(PH oxidase subunit upregulation. Conclusions Our results provide evidence that astrocytes, not infiltrating immune cells, play a key role in the development of optic neuritis and that astrocyte-mediated neurotoxicity is dependent on activation of a transcriptional program regulated by NF-κB. Hence, interventions targeting the NF-κB transcription

  13. Changes of Radial Diffusivity and Fractional Anisotopy in the Optic Nerve and Optic Radiation of Glaucoma Patients

    Directory of Open Access Journals (Sweden)

    Tobias Engelhorn

    2012-01-01

    Full Text Available Purpose of this study was to evaluate with diffusion-tensor imaging (DTI changes of radial diffusivity (RD and fractional anisotropy (FA in the optic nerve (ON and optic radiation (OR in glaucoma and to determine whether changes in RD and FA correlate with disease severity. Therefore, glaucoma patients and controls were examined using 3T. Regions of interest were positioned on RD and FA maps, and mean values were calculated for ON and OR and correlated with optic nerve atrophy and reduced spatial-temporal contrast sensitivity (STCS of the retina. We found, that RD in glaucoma patients was significantly higher in the ON (0.74 ± 0.21 versus 0.58 ± 0.17⋅10−3 mm2 s−1; P0.77. In conclusion, DTI at 3 Tesla allows robust RD and FA measurements in the ON and OR. Hereby, the extent of RD increase and FA decrease in glaucoma correlate with established ophthalmological examinations.

  14. Trabeculectomy and optic nerve head topography

    Directory of Open Access Journals (Sweden)

    A. Paranhos Jr.

    2006-01-01

    Full Text Available The objective of the present study was to evaluate changes in optic nerve head parameters, measured by confocal laser tomography, before and after trabeculectomy in order to identify outcome measures for the management of glaucoma. The optic nerve head of 22 eyes (22 patients was analyzed by confocal laser tomography with the Heidelberg retinal tomogram (HRT before and after trabeculectomy. The median time between the first HRT and surgery was 4.6 months (mean: 7.7 ± 8.3 and the median time between surgery and the second HRT was 10.8 months (mean: 12.0 ± 6.8. The patients were divided into two groups, i.e., those with the highest (group A and lowest (group B intraocular pressure (IOP change after surgery. Differences in the 12 standard topographic parameters before and after surgery for each group were evaluated by the Wilcoxon signed rank test and the differences in these parameters between the two groups were compared by the Mann-Whitney rank sum test. Multiple regression analysis was used to evaluate the influence of the change in IOP (deltaIOP and deltaIOP% and the changes in the other parameters. There were significant differences in the HRT measures before and after surgery in group A only for cup volume. In group B, no parameter was statistically different. The changes in group A were not significantly different than those in group B for any parameter (P > 0.004, Bonferroni correction for multiple comparisons. deltaIOP and deltaIOP% had a statistically significant effect on delta cup disk area, delta cup volume and delta mean cup depth. Changes in cup shape size were influenced significantly only by deltaIOP. Some optic disc parameters measured by HRT presented a significant improvement after filtering surgery, depending on the amount of IOP reduction. Long-term studies are needed to determine the usefulness of these findings as outcome measures in the management of glaucoma.

  15. Time-Dependent Nerve Growth Factor Signaling Changes in the Rat Retina During Optic Nerve Crush-Induced Degeneration of Retinal Ganglion Cells

    Directory of Open Access Journals (Sweden)

    Louise A. Mesentier-Louro

    2017-01-01

    Full Text Available Nerve growth factor (NGF is suggested to be neuroprotective after nerve injury; however, retinal ganglion cells (RGC degenerate following optic-nerve crush (ONC, even in the presence of increased levels of endogenous NGF. To further investigate this apparently paradoxical condition, a time-course study was performed to evaluate the effects of unilateral ONC on NGF expression and signaling in the adult retina. Visually evoked potential and immunofluorescence staining were used to assess axonal damage and RGC loss. The levels of NGF, proNGF, p75NTR, TrkA and GFAP and the activation of several intracellular pathways were analyzed at 1, 3, 7 and 14 days after crush (dac by ELISA/Western Blot and PathScan intracellular signaling array. The progressive RGC loss and nerve impairment featured an early and sustained activation of apoptotic pathways; and GFAP and p75NTR enhancement. In contrast, ONC-induced reduction of TrkA, and increased proNGF were observed only at 7 and 14 dac. We propose that proNGF and p75NTR contribute to exacerbate retinal degeneration by further stimulating apoptosis during the second week after injury, and thus hamper the neuroprotective effect of the endogenous NGF. These findings might aid in identifying effective treatment windows for NGF-based strategies to counteract retinal and/or optic-nerve degeneration.

  16. Time-Dependent Nerve Growth Factor Signaling Changes in the Rat Retina During Optic Nerve Crush-Induced Degeneration of Retinal Ganglion Cells.

    Science.gov (United States)

    Mesentier-Louro, Louise A; De Nicolò, Sara; Rosso, Pamela; De Vitis, Luigi A; Castoldi, Valerio; Leocani, Letizia; Mendez-Otero, Rosalia; Santiago, Marcelo F; Tirassa, Paola; Rama, Paolo; Lambiase, Alessandro

    2017-01-05

    Nerve growth factor (NGF) is suggested to be neuroprotective after nerve injury; however, retinal ganglion cells (RGC) degenerate following optic-nerve crush (ONC), even in the presence of increased levels of endogenous NGF. To further investigate this apparently paradoxical condition, a time-course study was performed to evaluate the effects of unilateral ONC on NGF expression and signaling in the adult retina. Visually evoked potential and immunofluorescence staining were used to assess axonal damage and RGC loss. The levels of NGF, proNGF, p75 NTR , TrkA and GFAP and the activation of several intracellular pathways were analyzed at 1, 3, 7 and 14 days after crush (dac) by ELISA/Western Blot and PathScan intracellular signaling array. The progressive RGC loss and nerve impairment featured an early and sustained activation of apoptotic pathways; and GFAP and p75 NTR enhancement. In contrast, ONC-induced reduction of TrkA, and increased proNGF were observed only at 7 and 14 dac. We propose that proNGF and p75 NTR contribute to exacerbate retinal degeneration by further stimulating apoptosis during the second week after injury, and thus hamper the neuroprotective effect of the endogenous NGF. These findings might aid in identifying effective treatment windows for NGF-based strategies to counteract retinal and/or optic-nerve degeneration.

  17. Congenital aplasia of the optic chiasm and esophageal atresia: a case report

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    Madonia Maurizio

    2011-08-01

    Full Text Available Abstract Introduction The complete absence of the chiasm (chiasmal aplasia is a rare clinical condition. Hypoplasia of the optic nerve and congenital nystagmus are almost invariably associated characteristics. Microphthalmos or anophthalmos are common features in chiasmal aplasia, while central nervous system abnormalities are less frequent. Esophageal atresia can be isolated or syndromic. In syndromic cases, it is frequently associated with cardiac, limb, renal or vertebral malformations and anal atresia. More rarely, esophageal atresia can be part of anophthalmia-esophageal-genital syndrome, which comprises anophthalmia or microphthalmia, genital abnormalities, vertebral defects and cerebral malformations. Here, a previously unreported case of chiasmal aplasia presenting without microphthalmos and associated with esophageal atresia is described. Case presentation Aplasia of the optic chiasm was identified in a Caucasian Italian 8-month-old boy with esophageal atresia. An ultrasound examination carried out at 21 weeks' gestation revealed polyhydramnios. Intrauterine growth retardation, esophageal atresia and a small atrial-septal defect were subsequently detected at 28 weeks' gestation. Repair of the esophageal atresia was carried out shortly after birth. A jejunostomy was carried out at four months to facilitate enteral feeding. The child was subsequently noted to be visually inattentive and to be neurodevelopmentally delayed. Magnetic resonance imaging revealed chiasmal aplasia. No other midline brain defects were found. His karyotype was normal. Conclusion If achiasmia is a spectrum, our patient seems to depict the most severe form, since he appears to have an extremely severe visual impairment. This is in contrast to most of the cases described in the literature, where patients maintain good--or at least useful-- visual function. To the best of our knowledge, the association of optic nerve hypoplasia, complete chiasmal aplasia, esophageal

  18. Sox10 Expression in Goldfish Retina and Optic Nerve Head in Controls and after the Application of Two Different Lesion Paradigms.

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    Marta Parrilla

    Full Text Available The mammalian central nervous system (CNS is unable to regenerate. In contrast, the CNS of fish, including the visual system, is able to regenerate after damage. Moreover, the fish visual system grows continuously throughout the life of the animal, and it is therefore an excellent model to analyze processes of myelination and re-myelination after an injury. Here we analyze Sox10+ oligodendrocytes in the goldfish retina and optic nerve in controls and after two kinds of injuries: cryolesion of the peripheral growing zone and crushing of the optic nerve. We also analyze changes in a major component of myelin, myelin basic protein (MBP, as a marker for myelinated axons. Our results show that Sox10+ oligodendrocytes are located in the retinal nerve fiber layer and along the whole length of the optic nerve. MBP was found to occupy a similar location, although its loose appearance in the retina differed from the highly organized MBP+ axon bundles in the optic nerve. After optic nerve crushing, the number of Sox10+ cells decreased in the crushed area and in the optic nerve head. Consistent with this, myelination was highly reduced in both areas. In contrast, after cryolesion we did not find changes in the Sox10+ population, although we did detect some MBP- degenerating areas. We show that these modifications in Sox10+ oligodendrocytes are consistent with their role in oligodendrocyte identity, maintenance and survival, and we propose the optic nerve head as an excellent area for research aimed at better understanding of de- and remyelination processes.

  19. Bilateral optic nerve swelling after thyroidectomy followed by a course of radioiodine therapy

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    Ioyleva E.E.

    2018-03-01

    Full Text Available The most common cancer of the endocrine system is thyroid cancer, representing 1.0–1.5 % all newly diagnosed cases of cancer. According to the cancer society of Russia, the thyroid cancer in children is much rarer than in adults. Thyroid cancer in children and adolescents is characterized by an adverse clinical course and a high risk of developing metastases in the lymph nodes. The main method of treatment for pediatric thyroid cancer is total thyroidectomy with central neck lymph node dissection followed by radioactive iodine therapy. In foreign and domestic literature, complications of the organ of vision, namely, changes of the optic disc, after surgical treatment for thyroid cancer are poorly understood. The risk of transient hypocalcemia and hypothyroidism increases after thyroidectomy. In the literature, there are two reported cases of the optic nerve swelling combined with hypoparathyroidism and hypocalcemia. While hypocalcemia intracranial hypertension and swelling of the optic nerves are often recorded. In this article, the authors present their own clinical observation of a 13-year-old patient after thyroidectomy and radioactive iodine therapy with detailed analysis of the clinical data and study results. According to the survey of the patient, bilateral swelling of the optic disc was revealed, which could occur due to hypothyroid state. Objective: to identify the cause of the development of bilateral optic nerve swelling in a patient after thyroidectomy and treatment course with radioactive iodine.

  20. Extracting structural features of rat sciatic nerve using polarization-sensitive spectral domain optical coherence tomography

    NARCIS (Netherlands)

    Islam, M.S.; Oliveira, M.C.; Wang, Y.; Henry, F.P.; Randolph, M.A.; Park, B. H.; de Boer, J.F.

    2012-01-01

    We present spectral domain polarization-sensitive optical coherence tomography (SD PS-OCT) imaging of peripheral nerves. Structural and polarization-sensitive OCT imaging of uninjured rat sciatic nerves was evaluated both qualitatively and quantitatively. OCT and its functional extension, PS-OCT,

  1. Immune mapping of the peripheral part of the visual analyzer and optic nerve

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    V. G. Likhvantseva

    2014-10-01

    Full Text Available Aim. To perform immune mapping of the peripheral part of visual analyzer and optic nerve in order to identify potential antigenic targets of autoimmune attack. Methods. Eyes enucleated for terminal painful glaucoma (n = 30 were studied. Immunohistochemistry (IHC was performed on paraffin-embedded sections of isolated retina and optic nerve using a broad panel of antibodies, i.e., monoclonal murine anti-MBP (myelin basic protein antibodies, polyclonal rabbit anti-alpha fodrin antibodies, monoclonal murine anti-NSE2 (neuron-specific enolase antibodies, monoclonal murine anti-GFAP (glial fibrillary acidic protein, and polyclonal rabbit anti-S100 antibodies. IHC reaction was visualized using Mouse and Rabbit Specific HRP / AEC Detection IHC Kit. IHC reaction without primary antibodies included was a negative control. IHC reaction was considered as follows: negative — no specific cellular staining or less than 10 % of cells are stained; mild — 10‑30 % of cells are stained (+; moderate — 30‑75 % of cells are stained (++; marked — more than 75 % of cells are stained (+++; overexpression — 100 % of cells intensively express markers. Additionally, staining intensity was considered as mild (+1, moderate (+2, strong (+3 and intense (+4.Results. Immune mapping with a broad panel of monoclonal antibodies identified ocular structures which were stained with IHC markers. Retina was stained with almost all markers of neural differentiation (i.e., antibodies against NSE, GFAP, S100, and α-fodrin excepting anti-MBP autoantibodies. IHC reaction intensity in retinal layers and structures varied and depended on markers. Moderate (2+ staining with antibodies against MBP, NSE, GFAP, and S100 and marked (3+ staining with antibodies against alpha-fodrin was detected in the cytoplasm of optic nerve glia.Conclusion. Complete labelling of retina structures was performed. As a result, IHC profiles of retinal neurons, optic nerve axons

  2. Immune mapping of the peripheral part of the visual analyzer and optic nerve

    Directory of Open Access Journals (Sweden)

    V. G. Likhvantseva

    2014-01-01

    Full Text Available Aim. To perform immune mapping of the peripheral part of visual analyzer and optic nerve in order to identify potential antigenic targets of autoimmune attack. Methods. Eyes enucleated for terminal painful glaucoma (n = 30 were studied. Immunohistochemistry (IHC was performed on paraffin-embedded sections of isolated retina and optic nerve using a broad panel of antibodies, i.e., monoclonal murine anti-MBP (myelin basic protein antibodies, polyclonal rabbit anti-alpha fodrin antibodies, monoclonal murine anti-NSE2 (neuron-specific enolase antibodies, monoclonal murine anti-GFAP (glial fibrillary acidic protein, and polyclonal rabbit anti-S100 antibodies. IHC reaction was visualized using Mouse and Rabbit Specific HRP / AEC Detection IHC Kit. IHC reaction without primary antibodies included was a negative control. IHC reaction was considered as follows: negative — no specific cellular staining or less than 10 % of cells are stained; mild — 10‑30 % of cells are stained (+; moderate — 30‑75 % of cells are stained (++; marked — more than 75 % of cells are stained (+++; overexpression — 100 % of cells intensively express markers. Additionally, staining intensity was considered as mild (+1, moderate (+2, strong (+3 and intense (+4.Results. Immune mapping with a broad panel of monoclonal antibodies identified ocular structures which were stained with IHC markers. Retina was stained with almost all markers of neural differentiation (i.e., antibodies against NSE, GFAP, S100, and α-fodrin excepting anti-MBP autoantibodies. IHC reaction intensity in retinal layers and structures varied and depended on markers. Moderate (2+ staining with antibodies against MBP, NSE, GFAP, and S100 and marked (3+ staining with antibodies against alpha-fodrin was detected in the cytoplasm of optic nerve glia.Conclusion. Complete labelling of retina structures was performed. As a result, IHC profiles of retinal neurons, optic nerve axons

  3. [Traumatic lesion of the optic nerve head by flying fish: a case report].

    Science.gov (United States)

    Martin, M; Orgül, S; Robertson, A; Flammer, J

    2004-05-01

    Traumatic lesion to the optic nerve often leads to severe and persistent functional loss. A male patient was transferred to our hospital from the University Eye Clinic of Guadeloupe 5 days after ocular injury caused by a flying fish. Visual function was light perception. The anterior part of the eye and retina were unremarkable. A computer tomography disclosed a fracture of the sphenoid sinus, with a little bone fragment (DD: foreign body) located close to the optic nerve. Therapy had been started with Aminopenicillin combined with clavulan acid (Augmentin) i. v., 500 ml methylprednisolone (Solumedrol) i. v., lysine-acetyl salicylate (Aspegic) and topical application of dexamethasone combined with neomycin/polymyxin B (Maxitrol). We continued this therapy and intensified it by adding nimodipine (Nimotop) 30 1-1-1 and acetazolamide retard (Diamox sustet) 1-0-1. Unfortunately visual function did not recover under therapy. Traumatic lesions of the optic nerve head, especially when due to axial or tangential forces, can lead to severe and irreversible functional loss. Severe traumatic lesions, even bone fractures induced by flying fish are not a seldom encounter in the Caribbean Sea.

  4. [Gene Therapy for Inherited RETINAL AND OPTIC NERVE Disorders: Current Knowledge].

    Science.gov (United States)

    Ďuďáková, Ľ; Kousal, B; Kolářová, H; Hlavatá, L; Lišková, P

    The aim of this review is to provide a comprehensive summary of current gene therapy clinical trials for monogenic and optic nerve disorders.The number of genes for which gene-based therapies are being developed is growing. At the time of writing this review gene-based clinical trials have been registered for Leber congenital amaurosis 2 (LCA2), retinitis pigmentosa 38, Usher syndrome 1B, Stargardt disease, choroideremia, achromatopsia, Leber hereditary optic neuropathy (LHON) and X-linked retinoschisis. Apart from RPE65 gene therapy for LCA2 and MT-ND4 for LHON which has reached phase III, all other trials are in investigation phase I and II, i.e. testing the efficacy and safety.Because of the relatively easy accessibility of the retina and its ease of visualization which allows monitoring of efficacy, gene-based therapies for inherited retinal disorders represent a very promising treatment option. With the development of novel therapeutic approaches, the importance of establishing not only clinical but also molecular genetic diagnosis is obvious.Key words: gene therapy, monogenic retinal diseases, optic nerve atrophy, mitochondrial disease.

  5. Positive effects of bFGF modified rat amniotic epithelial cells transplantation on transected rat optic nerve.

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    Jia-Xin Xie

    Full Text Available Effective therapy for visual loss caused by optic nerve injury or diseases has not been achieved even though the optic nerve has the regeneration potential after injury. This study was designed to modify amniotic epithelial cells (AECs with basic fibroblast growth factor (bFGF gene, preliminarily investigating its effect on transected optic nerve.A human bFGF gene segment was delivered into rat AECs (AECs/hbFGF by lentiviral vector, and the gene expression was examined by RT-PCR and ELISA. The AECs/hbFGF and untransfected rat AECs were transplanted into the transected site of the rat optic nerve. At 28 days post transplantation, the survival and migration of the transplanted cells was observed by tracking labeled cells; meanwhile retinal ganglion cells (RGCs were observed and counted by employing biotin dextran amine (BDA and Nissl staining. Furthermore, the expression of growth associated protein 43 (GAP-43 within the injury site was examined with immunohistochemical staining.The AECs/hbFGF was proven to express bFGF gene and secrete bFGF peptide. Both AECs/hbFGF and AECs could survive and migrate after transplantation. RGCs counting implicated that RGCs numbers of the cell transplantation groups were significantly higher than that of the control group, and the AECs/hbFGF group was significantly higher than that of the AECs group. Moreover GAP-43 integral optical density value in the control group was significantly lower than that of the cell transplantation groups, and the value in the AECs/hbFGF group was significantly higher than that of the AECs group.AECs modified with bFGF could reduce RGCs loss and promote expression of GAP-43 in the rat optic nerve transected model, facilitating the process of neural restoration following injury.

  6. Neuronal apoptosis and neurofilament protein expression in the lateral geniculate body of cats following acute optic nerve injuries

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    BACKGROUND: The visual pathway have 6 parts, involving optic nerve, optic chiasm, optic tract, lateral geniculate body, optic radiation and cortical striatum area. Corresponding changes may be found in these 6 parts following optic nerve injury. At present, studies mainly focus on optic nerve and retina, but studies on lateral geniculate body are few.OBJECTIVE: To prepare models of acute optic nerve injury for observing the changes of neurons in lateral geniculate body, expression of neurofilament protein at different time after injury and cell apoptosis under the optical microscope, and for investigating the changes of neurons in lateral geniculate body following acute optic nerve injury.DESIGN: Completely randomized grouping design, controlled animal experiment.SETTING: Department of Neurosurgery, General Hospital of Ji'nan Military Area Command of Chinese PLA.MATERIALS: Twenty-eight adult healthy cats of either gender and common grade, weighing from 2.0 to 3.5 kg, were provided by the Animal Experimental Center of Fudan University. The involved cats were divided into 2 groups according to table of random digit: normal control group (n =3) and model group (n =25). Injury 6 hours, 1, 3, 7 and 14 days five time points were set in model group for later observation, 5 cats at each time point. TUNEL kit (Bohringer-Mannheim company)and NF200& Mr 68 000 mouse monoclonal antibody (NeoMarkers Company) were used in this experiment.METHODS: This experiment was carried out in the Department of Neurosurgery, General Hospital of Ji'nan Military Area Command of Chinese PLA between June 2004 and June 2005. ① The cats of model group were developed into cat models of acute intracranial optic nerve injury as follows: The anesthetized cats were placed in lateral position. By imitating operation to human, pterion approach was used. An incision was made at the joint line between outer canthus and tragus, and deepened along cranial base until white optic nerve via optic nerve pore

  7. Cranial nerve threshold for thermal injury induced by MRI-guided high-intensity focused ultrasound (MRgHIFU): preliminary results on an optic nerve model.

    Science.gov (United States)

    Harnof, Sagi; Zibly, Zion; Cohen, Zvi; Shaw, Andrew; Schlaff, Cody; Kassel, Neal F

    2013-04-01

    Future clinical applications of magnetic resonance imaging-guided high-intensity focused ultrasound (MRgHIFU) are moving toward the management of different intracranial pathologies. We sought to validate the production, safety, and efficacy of thermal injury to cranial nerves generated by MRgHIFU. In this study, five female domestic pigs underwent a standard bifrontal craniectomy under general anesthesia. Treatment was then given using an MRgHIFU system to induce hyperthermic ablative sonication (6 to 10 s; 50 to 2000 J.) Histological analyses were done to confirm nerve damage; temperature measured on the optic nerve was approximately 53.4°C (range: 39°C to 70°C.) Histology demonstrated a clear definition between a necrotic, transitional zone, and normal tissue. MRgHIFU induces targeted thermal injury to nervous tissue within a specific threshold of 50°C to 60°C with the tissue near the sonication center yielding the greatest effect; adjacent tissue showed minimal changes. Additional studies utilizing this technology are required to further establish accurate threshold parameters for optic nerve thermo-ablation.

  8. Retinal, optic nerve and chiasmal function following radiation therapy demonstrated by visual evoked response testing

    International Nuclear Information System (INIS)

    Soni, A.B.; Constine, L.S.; Smith, D.; Palisca, M.; Ojomo, K.; Muhs, A.

    1997-01-01

    Purpose: To evaluate the tolerance of the retina, optic nerve, and optic chiasm to radiation doses conventionally used to treat patients with primary brain or pituitary tumors and to explore the character of detectable radiation effects. Visual evoked response (VER) testing is a noninvasive and sensitive method for identifying radiation injury to the visual system due to alterations in small vessel or myelin integrity. Such evaluations may increase our understanding of the threshold for and the pathogenesis of radiation injury. Materials and Methods: Twenty-four patients irradiated for brain or pituitary tumors between 1972 and 1996 had VER testing. Patients were included in study if the retina, optic nerves or chiasm were in the radiation (RT) field. At the time of RT patients ranged in age from 1.5 to 55 years (median 33). Mean doses were as follows: right retina, 29 Gy (range 10 - 60 Gy); left retina, 29.5 Gy (range 10 - 60 Gy); right optic nerve, 42.9 Gy (range 10 - 60 Gy); left optic nerve, 42.6 Gy (range 10 - 60 Gy); and optic chiasm, 48.2 Gy (range 10 - 65 Gy). Daily fractionation ranged from 1.5 to 1.8 Gy. Pattern VER testing distinguishes compressive or ischemic effects of tumor on the visual system from radiation retinopathy or optic neuropathy on the basis of the conduction amplitude and delay pattern. Prechiasm, chiasm, and postchiasm injuries are distinguishable by analyzing VER changes. Four evoked responses were obtained for each eye, each representing the average of 100 stimulus reversals. Results: VER was normal in 11 patients and abnormal in 13 patients. Only 2 patients (8%) had VER evidence of radiation injury to the visual system, one of whom had visual compromise. The other 11 abnormal patients had characteristic VER changes attributable to tumor or surgical damage. There was no significant difference in the radiation doses given to any subgroup. The one patient with radiation retinopathy had received 55-60 Gy to the posterior globe. Ten years

  9. Moebius syndrome with macular hyperpigmentation, skeletal anomalies, and hypoplasia of pectoralis major muscle in an Egyptian child

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    Rabah M. Shawky

    2015-07-01

    Full Text Available We report a 4 month old female infant, 3rd in order of birth of the first cousin consanguineous parents. The patient has congenital right facial nerve palsy, with asymmetry of facial expression during crying and difficulty in swallowing. Associated anomalies include abnormal facial features, bilateral finger anomalies, bilateral talipes equinovarus, kyphoscoliosis, hypotonia, high frequency hearing loss. Bilateral macular hyperpigmentation was detected in our patient on fundus examination which was not reported previously in Moebius syndrome cases. In addition there is hypoplasia of the right pectoralis major muscle.

  10. [The neuroprotective effect of erigeron breviscapus (vant) hand-mazz on retinal ganglion cells after optic nerve crush injury].

    Science.gov (United States)

    Jiang, Bing; Jiang, You-qin

    2003-08-01

    To investigate whether a Chinese herbal medicine, erigeron breviscapus (vant) hand-mazz (EBHM), can protect the retinal ganglion cells (RGC) damaged by calibrated optic nerve crush injury. Forty-two Sprague-Dawley rats were randomly divided into two groups. Calibrated optic nerve crush injury model was induced in the right eyes by a special designed optic nerve clip. The left eyes served as a control. All 42 rats were randomly divided into 2 groups. Group A consisted of the rats with calibrated optic nerve crush injury and group B consisted of rats with calibrated optic nerve crush injury treated with EBHM. In group B, EBHM solution was given once after the crush injury. According to the time interval between the optic nerve crush and the sacrifice, both groups A and B were further divided into three subgroups (day 4, day 14 and day 21). Therefore, there were 7 rats in each subgroup. Three days before sacrifice, 3% fast blue was injected into superior colliculi bilaterally. The eyes were enucleated after the rat was sacrificed, and flat mounts of the retina from both eyes were prepared on a slide and observed under a fluorescence microscope. Four photos with 400 x magnification were taken from each of the four quadrants of the retina 1 mm away from the optic disc. The labeled RGC were counted by a computerized image analyzer. The labeled RGC rate was used for statistical analysis (the labeled RGC rate = number of RGC in injured eye/control eye x 100%). In group A, the labeled RGC rate was (77.79 +/- 7.11)%, (63.76 +/- 3.79)% and (54.66 +/- 4.75)% on day 4, day 14 and day 21, respectively. In group B, the labeled RGC rate was (80.13 +/- 12.03)%, (78.17 +/- 9.19)% and (83.59 +/- 12.61)% on day 4, day 14 and day 21, respectively. In group B, which was treated with EBHM after injury, the labeled RGC rate was significantly higher than that of group A on day 14 and day 21. In the experimental optic nerve crush model in rats, EBHM therapy can increase the survival rate of

  11. Structure-function relationships with spectral-domain optical coherence tomography retinal nerve fiber layer and optic nerve head measurements.

    Science.gov (United States)

    Pollet-Villard, Frédéric; Chiquet, Christophe; Romanet, Jean-Paul; Noel, Christian; Aptel, Florent

    2014-05-02

    To evaluate the regional structure-function relationship between visual field sensitivity and retinal nerve fiber layer (RNFL) thickness and optic nerve head (ONH) measurements using spectral-domain optical coherence tomography (SD-OCT). Prospective cross-sectional study conducted on patients with glaucoma, suspected glaucoma, and healthy subjects. Eyes were tested on Cirrus OCT and standard achromatic perimetry. RNFL thickness of 12 peripapillary 30° sectors, neuroretinal rim thickness extracted from 36 neuroretinal rim scans, and Bruch membrane opening minimum rim width (BMO-MRW)-a recently defined parameter-extracted from 36 neuroretinal rim scans were obtained. Correlations between peripapillary RNFL thickness, neuroretinal rim thickness, all six sectors of BMO-MRW, and visual field sensitivity in the six corresponding areas were evaluated using logarithmic regression analysis. Receiver operating curve areas were calculated for each RNFL, ONH, and macular ganglion cell analysis parameter. We included 142 eyes of 142 subjects. The correlations (r(2)) between RNFL thickness, Cirrus-based neuroretinal rim thickness, BMO-MRW and visual field sensitivity ranged from 0.07 to 0.60, 0.15 to 0.49, and 0.24 to 0.66, respectively. The structure-function correlations were stronger with BMO-MRW than with Cirrus-based neuroretinal rim thickness. The largest areas under the receiver operating curve were seen for rim area (0.926 [95% confidence interval 0.875, 0.977]; P function relationship was significantly stronger with BMO-MRW than other ONH SD-OCT parameters. The best diagnostic capabilities were seen with rim area and average RNFL.

  12. Effect of chronic administration of sildenafil citrate (Viagra) on the histology of the retina and optic nerve of adult male rat.

    Science.gov (United States)

    Eltony, Sohair A; Abdelhameed, Sally Y

    2017-04-01

    Abnormal vision has been reported by 3% of patients treated with sildenafil citrate (Viagra). Although many men use Viagra for an extended period for treatment of erectile dysfunction, the implications of the long term-daily use of it on the retina and optic nerve are unclear. To investigate the effect of chronic daily use of sildenafil citrate in a dose equivalent to men preferred therapeutic dose on the histology of the retina and optic nerve of adult male rat. Eighteen adult male Wistar rats were equally divided into three groups. Group I: control. Group II: treated with sildenafil citrate orally (10mg/kg/day) for 8 weeks. Group III (withdrawal): treated as group II and then left for 4 weeks without treatment. Specimens from the retina and optic nerve were processed for light and electron microscopy. In sildenafil citrate treated group, the retina and optic nerve revealed vacuolations and congested blood capillaries with apoptotic endothelial and pericytic cells, and thickened basal lamina. Caspase-3 (apoptotic marker) and CD31 (endothelial marker) expression increased. Glial cells revealed morphological changes: Müller cells lost their processes, activated microglia, astrocytic clasmatodendrosis, degenerated oligodendrocytes surrounded by disintegrated myelin sheathes of the optic nerve fibers. The retina and optic nerve of the withdrawal group revealed less vacuolations and congestion, and partial recovery of the glial cells. Chronic treatment with sildenafil citrate (Viagra) caused toxic effect on the structure of the retina and optic nerve of the rat. Partial recovery was observed after drug withdrawal. Copyright © 2017 Elsevier Ltd. All rights reserved.

  13. Structural changes of the macula and optic nerve head in the remaining eyes after enucleation for retinoblastoma: an optical coherence tomography study.

    Science.gov (United States)

    Said, Azza Mohamed Ahmed; Elbayomi, Ahmed Mohamed; Shaat, Ashraf Abdelsalam Kandeel

    2017-12-16

    To describe objectively the possible structural changes of the macula and optic nerve head in the free eyes of unilateral cured retinoblastoma patients and, also after enucleation using spectral domain optical coherence tomography. A cross sectional study involving 60 patients subdivided into three groups; 15 unilateral RB patients in whom enucleation was indicated as a sole treatment performed earlier in life [(study group (I)], 15 unilateral RB patients who had completely regressed disease with a preserved eye [(study group (II)] and 30 age and sex matched healthy controls. The remaining and free eyes in study groups and right eyes of control group had full ophthalmological examination, static automated perimetry and optical coherence tomography of the macula and optic nerve head. In study group (II); a significant thinning of total macula, central fovea, ganglion cell layer (GCL), ganglion cell complex (GCC), and some sectors of outer nuclear layer (P- values ≤0.05) was found with no significant difference in peripapillary nerve fiber layer (pRNFL) thickness and optic nerve head parameters compared to the control group and the study group (I). A significantly thickened total macula, GCL, GCC, and pRNFL in study group (I) compared to study group (II). Thickened pRNFL was significantly correlated to standard automated perimetry pattern deviations. No significant difference was found between study group (I) and control group. Retinoblastoma eyes characterized by thinning of central fovea, GCL, GCC compared to the control group. After unilateral enucleation, increased GCC and pRNFL thicknesses were detected compared to retinoblastoma group.

  14. Diagnostic capability of optic nerve head rim width and retinal nerve fiber thickness in open-angle glaucoma.

    Science.gov (United States)

    Di Staso, Silvio; Agnifili, Luca; Di Staso, Federico; Climastone, Hilary; Ciancaglini, Marco; Scuderi, Gian Luca

    2018-03-01

    This study was performed to test the diagnostic capability of the minimum rim width compared to peripapillary retinal nerve fiber layer thickness in patients with glaucoma. A case control, observer masked study, was conducted. Minimum rim width and retinal nerve fiber layer thickness were assessed using the patient-specific axis traced between fovea-to-Bruch's membrane opening center axis. For both minimum rim width and retinal nerve fiber layer thickness, the regionalization in six sectors (nasal, superior-nasal, superior-temporal, temporal, inferior-temporal, and inferior-nasal) was analyzed. Eyes with at least one sector with value below the 5% or 1% normative limit of the optical coherence tomography normative database were classified as glaucomatous. The area under the receiver operator characteristic curve, the accuracy, sensitivity, specificity, and predictive positive and negative values were calculated for both minimum rim width and retinal nerve fiber layer thickness. A total of 118 eyes of 118 Caucasian subjects (80 eyes with open-angle glaucoma and 38 control eyes) were enrolled in the study. Accuracy, sensitivity, and specificity were 79.7%, 77.5%, and 84.2%, respectively, for minimum rim width and 84.7%, 82.5%, and 89.5% for retinal nerve fiber layer thickness. The positive predictive values were 0.91% and 0.94% for minimum rim width and retinal nerve fiber layer thickness, respectively, whereas the negative predictive values were 0.64% and 0.70%. The area under the receiver operator characteristic curve was 0.892 for minimum rim width and 0.938 for retinal nerve fiber layer thickness. Our results indicated that the sector analysis based on Bruch's membrane opening and fovea to disk alignment is able to detect glaucomatous defects, and that Bruch's membrane opening minimum rim width and retinal nerve fiber layer thickness showed equivalent diagnostic ability.

  15. Fast axonal transport of 3H-leucin-labelled proteins in the unhurt and isolated optical nerve of rats

    International Nuclear Information System (INIS)

    Wagner, H.E.

    1981-01-01

    The distribution of radioactivity of amino acid molecules incorporated in protein after injection of 3 H-Leucin into the right bulb was investigated and determined along optical nerve after 1, 2, and 4 h. A slightly increased radioactivity at the point of entrance of the optical nerves into the optical duct was found. A slightly reduced axon diameter was discussed as a possible cause. The radioactivity brought into the optical nerve via the vascular system was determined by measuring the contralateral optical nerve. In relation to the axonally transported activity, it was low. The speed of the fast axonal transport is 168 mm/d. If the processes ruling the amino acids in the perikaryon are taken into consideration, the transport speed is 240 mm/d. The application of the protein synthesis prohibitor, Cycloheximide, 5 minutes after the injection of Leucinin completely prevented the appearance of axonally transported labelled proteins. When cycloheximide was administered 2 h after Leucin, a significantly loner radioactivity than in the nerve could be determined after another 2 h; i.e. the incorporation of Leucin was not completed yet after 2 h. The profile of active compounds was the same as in the control group. In other experiments, the axonal transport of labelled proteins in isolated optical nerve fibres was tested. If the separation was carried out 2 h after the injection of Leucin an extreme reduction in activity could be determined after 1 or 2 h. The continued distribution of activity after cycloheximide treatment and removal of perikarya in comparison with the control indicate the continuation of the transport, also after separation of the axon from the perikaryon. This means that, during the time of the experiment, the mechanism of the fast axonal transport functions independently of the perikaryon. (orig./MG) [de

  16. Enamel hypoplasia in the middle pleistocene hominids from Atapuerca (Spain).

    Science.gov (United States)

    Bermúdez de Castro, J M; Pérez, P J

    1995-03-01

    The prevalence and chronology of enamel hypoplasias were studied in a hominid dental sample from the Sima de los Huesos (SH) Middle Pleistocene site at the Sierra de Atapuerca (Burgos, northern Spain). A total of 89 permanent maxillary teeth, 143 permanent mandibular teeth, and one deciduous lower canine, belonging to a minimum of 29 individuals, were examined. Excluding the antimeres (16 maxillary and 37 mandibular cases) from the sample, the prevalence of hypoplasias in the permanent dentition is 12.8% (23/179), whereas the deciduous tooth also showed an enamel defect. No statistically significant differences were found between both arcades and between the anterior and postcanine teeth for the prevalence of hypoplasias. In both the maxilla and the mandible the highest frequency of enamel hypoplasias was recorded in the canines. Only one tooth (a permanent upper canine) showed two different enamel defects, and most of the hypoplasias were expressed as faint linear horizontal defects. Taking into account the limitations that the incompleteness of virtually all permanent dentitions imposes, we have estimated that the frequency by individual in the SH hominid sample was not greater than 40%. Most of the hypoplasias occurred between birth and 7 years (N = 18, X = 3.5, SD = 1.3). Both the prevalence and severity of the hypoplasias of the SH hominid sample are significantly less than those of a large Neandertal sample. Furthermore, prehistoric hunter-gatherers and historic agricultural and industrial populations exhibit a prevalence of hypoplasias generally higher than that of the SH hominids. Implications for the survival strategies and life quality of the SH hominids are also discussed.

  17. Optic nerve infiltration by acute lymphoblastic leukemia: MRI contribution

    Energy Technology Data Exchange (ETDEWEB)

    Soares, Maria de Fatima; Braga, Flavio Tulio [Federal University of Sao Paulo, Department of Diagnostic Imaging, Paulista School of Medicine, Sao Paulo (Brazil); Rocha, Antonio Jose da [Santa Casa de Misericordia de Sao Paulo, Servico de Diagnostico por Imagem, Sao Paulo (Brazil); Lederman, Henrique Manoel [Federal University of Sao Paulo, Division of Diagnostic Imaging in Pediatrics, Department of Diagnostic Imaging, Sao Paulo (Brazil)

    2005-08-01

    We describe the clinical presentation and imaging features of a patient with acute lymphoblastic leukemia (ALL) that was complicated by optic nerve infiltration. The clinical and diagnostic characteristics of this complication must be recognized so that optimal therapy can be started to prevent blindness. MR imaging is useful in early detection and should be performed in any leukemic patient with ocular complaints, even during remission. (orig.)

  18. Optic nerve infiltration by acute lymphoblastic leukemia: MRI contribution

    International Nuclear Information System (INIS)

    Soares, Maria de Fatima; Braga, Flavio Tulio; Rocha, Antonio Jose da; Lederman, Henrique Manoel

    2005-01-01

    We describe the clinical presentation and imaging features of a patient with acute lymphoblastic leukemia (ALL) that was complicated by optic nerve infiltration. The clinical and diagnostic characteristics of this complication must be recognized so that optimal therapy can be started to prevent blindness. MR imaging is useful in early detection and should be performed in any leukemic patient with ocular complaints, even during remission. (orig.)

  19. The radiological features of Goltz syndrome: Focal dermal hypoplasia

    International Nuclear Information System (INIS)

    Boothyrod, A.E.; Hall, C.M.

    1988-01-01

    Two female infants with Goltz syndrome (focal dermal hypoplasia) were recently investigated for severe feeding problems and failure to thrive. Both demonstrated severe skeletal malformations and marked gastrooesophageal reflux with laxity of the hiatus. One child (case 1) exhibited nasal regurgitation during feeding. Interestingly, both children had undergone surgery; Case 1 or a right parasagittal abdominal hernia associated with focal dermal hypoplasia of the abdominal wall and Case 2 for an exomphalos also associated with dermal hypoplasia. This observation suggests more widespread mesodermal abnormality. (orig./GDG)

  20. Brain stem hypoplasia associated with Cri-du-Chat syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Jin Ho; Lee, Ha Young; Lim, Myung Kwan; Kim, Mi Young; Kang, Young Hye; Lee, Kyung Hee; Cho, Soon Gu [Dept. of Radiology, Inha University Hospital, Inha University School of Medicine, Incheon (Korea, Republic of)

    2013-12-15

    Cri-du-Chat syndrome, also called the 5p-syndrome, is a rare genetic abnormality, and only few cases have been reported on its brain MRI findings. We describe the magnetic resonance imaging findings of a 1-year-old girl with Cri-du-Chat syndrome who showed brain stem hypoplasia, particularly in the pons, with normal cerebellum and diffuse hypoplasia of the cerebral hemispheres. We suggest that Cri-du-Chat syndrome chould be suspected in children with brain stem hypoplasia, particularly for those with high-pitched cries.

  1. Unilateral Glenoid Hypoplasia: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Ashish Suryawanshi

    2011-01-01

    Full Text Available Glenoid hypoplasia is a relatively rare alteration that in most cases involves the pectoral girdle in a bilateral and symmetrical manner. In general, glenoid hypoplasia is associated with skeletal changes such as hypoplasia of the humeral head or changes in the morphology of the acromion and of the coracoid. We describe a rare case of unilateral glenoid hypoplasia without instability and not involving humeral head. The patient was managed effectively with nonoperative measures that featured specific rehabilitation exercises for the shoulder.

  2. Characterization of Abnormal Optic Nerve Head Morphology in Albinism Using Optical Coherence Tomography.

    Science.gov (United States)

    Mohammad, Sarim; Gottlob, Irene; Sheth, Viral; Pilat, Anastasia; Lee, Helena; Pollheimer, Ellen; Proudlock, Frank Anthony

    2015-07-01

    To characterize abnormalities in three-dimensional optic nerve head (ONH) morphology in people with albinism (PWA) using spectral-domain optical coherence tomography (SD-OCT) and to determine whether ONH abnormalities relate to other retinal and clinical abnormalities. Spectral-domain OCT was used to obtain three-dimensional images from 56 PWA and 60 age- and sex-matched control subjects. B-scans were corrected for nystagmus-associated motion artefacts. Disc, cup, and rim ONH dimensions and peripapillary retinal nerve fiber layer (ppRNFL) thickness were calculated using Copernicus and ImageJ software. Median disc areas were similar in PWA (median = 1.65 mm2) and controls (1.71 mm2, P = 0.128), although discs were significantly elongated horizontally in PWA (P < 0.001). In contrast, median optic cup area in PWA (0.088 mm2) was 23.7% of that in controls (0.373 mm2, P < 0.001), with 39.4% of eyes in PWA not demonstrating a measurable optic cup. This led to significantly smaller cup to disc ratios in PWA (P < 0.001). Median rim volume in PWA (0.273 mm3) was 136.6% of that in controls (0.200 mm3). The ppRNFL was significantly thinner in PWA compared with controls (P < 0.001), especially in the temporal quadrant. In PWA, ppRNFL thickness was correlated to ganglion cell thickness at the central fovea (P = 0.007). Several ONH abnormalities, such as cup to disc ratio, were related to higher refractive errors in PWA. In PWA, ocular maldevelopment is not just limited to the retina but also involves the ONH. Reduced ppRNFL thickness is consistent with previous reports of reduced ganglion cell numbers in PWA. The thicker rim volumes may be a result of incomplete maturation of the ONH.

  3. The association of sphenoidal encephalocele and right anophthalmia with septo-optic dysplasia: a case report.

    Science.gov (United States)

    Erol, Fatih Serhat; Ucler, Necati; Kaplan, Metin; Yilmaz, Ilhan

    2012-01-01

    Septo-optic dysplasia (SOD) is an extremely rare congenital anomaly, characterized with optic nerve hypoplasia and absence of septum pellucidum and/or pituitary dysfunction. In addition to classical findings of SOD, we report for the first time an 11-year-old boy, with encephalocele extending to the right sphenoidal sinus, right anophthalmia and normal pituitary functions. Despite all the major anomalies, the patient's presenting symptoms were very few and during the 11-year period the SDO had caused no complaints in our case. These findings show that the SOD course may be fairly benign. No neurological problem was encountered in the patient's follow-up, except headache. We believe that SOD should be kept in mind because of its rarity and the severity of its combined pathologies.

  4. P3-7: On Prototyping a Visual Prosthesis System with Artificial Retina and Optic Nerve Based on Arrayed Microfibers

    Directory of Open Access Journals (Sweden)

    Jian Hong Chen

    2012-10-01

    Full Text Available The traditional visual prosthesis system combines both a camera and a microelectrode array implanted on the visual neural network including retina, optic nerve, and visual cortex. Here, we introduce a new visual prosthesis system in which an artificial retina and optic nerve are demonstrated. The prototype of optic nerve for image transmission is comprised of arrayed PMMA microfibers with both ends connected with two planes, one functioned as retina for light reception and another attached to visual cortex. The microfibers are drawn from the thin film prepared by PMMA/chlorobenzene solution. Each micro fiber serves as an optical waveguide for the delivery of a single image pixel. It is demonstrated that with proper imaging optics, arrayed micro fibers could be lit as discrete light spots in accordance with the input image. Each micro fiber is expected to function as a stimulation unit for optical neural modulation in a visual prosthesis system.

  5. MR findings in pontocerebellar hypoplasia

    Energy Technology Data Exchange (ETDEWEB)

    Uhl, M.; Pawlik, H.; Laubenberger, J.; Langer, M. [Department of Radiology, Division of Paediatric Radiology, University Hospital of Freiburg, Freiburg (Germany); Darge, K. [Department of Paediatric Radiology, Children`s Hospital of the University of Heidelberg, Heidelberg (Germany); Baborie, A. [Department of Neuropathology, Neurozentrum, University of Freiburg, Freiburg (Germany); Korinthenberg, R. [Department of Neuropaediatrics, Children`s Hospital, University of Freiburg, Freiburg (Germany)

    1998-07-01

    We present four cases with combined hypoplasia of the cerebellum and the ventral pons - pontocerebellar hypoplasia (PCH). PCH represents an autosomal recessive neurodegenerative disorder with fetal onset. The disease is rare, with less than 20 cases having been reported. The main findings of PCH and the inclusion criteria for our cases can be summarised as progressive microcephaly from birth, pontocerebellar hypoplasia documented by MRI and marked chorea, which may change, later in childhood, to more dystonic patterns. The cerebral cortex becomes progressively atrophic. Motor and mental development are delayed, and epilepsy, mainly tonic-clonic seizures, is frequent. The MRI features in all of our cases were: (1) Hypoplastic cerebellum situated close to the tentorium. The hypoplastic cerebellum has a reduced number of folia, in contrast to the normal number of thin folia in simple cerebellar atrophy. (2) The cerebellar hemispheres are reduced to bean-like or wing-like structures. The cerebellar hemispheres appear to `float` in the posterior fossa. (3) Markedly hypoplastic ventral pons. (4) Slight atrophy of the supratentorial gyral pattern. (5) Dilated cerebromedullary cistern and fourth ventricle. (6) Delayed myelination of the white matter. (7) No significant disorganisation of brain architecture and no severe corpus callosum defect. (orig.) With 3 figs., 2 tabs., 13 refs.

  6. MR findings in pontocerebellar hypoplasia

    International Nuclear Information System (INIS)

    Uhl, M.; Pawlik, H.; Laubenberger, J.; Langer, M.; Darge, K.; Baborie, A.; Korinthenberg, R.

    1998-01-01

    We present four cases with combined hypoplasia of the cerebellum and the ventral pons - pontocerebellar hypoplasia (PCH). PCH represents an autosomal recessive neurodegenerative disorder with fetal onset. The disease is rare, with less than 20 cases having been reported. The main findings of PCH and the inclusion criteria for our cases can be summarised as progressive microcephaly from birth, pontocerebellar hypoplasia documented by MRI and marked chorea, which may change, later in childhood, to more dystonic patterns. The cerebral cortex becomes progressively atrophic. Motor and mental development are delayed, and epilepsy, mainly tonic-clonic seizures, is frequent. The MRI features in all of our cases were: (1) Hypoplastic cerebellum situated close to the tentorium. The hypoplastic cerebellum has a reduced number of folia, in contrast to the normal number of thin folia in simple cerebellar atrophy. (2) The cerebellar hemispheres are reduced to bean-like or wing-like structures. The cerebellar hemispheres appear to 'float' in the posterior fossa. (3) Markedly hypoplastic ventral pons. (4) Slight atrophy of the supratentorial gyral pattern. (5) Dilated cerebromedullary cistern and fourth ventricle. (6) Delayed myelination of the white matter. (7) No significant disorganisation of brain architecture and no severe corpus callosum defect. (orig.)

  7. Optical coherence tomography evaluation of retinal nerve fiber layer in longitudinally extensive transverse myelitis

    Directory of Open Access Journals (Sweden)

    Frederico C. Moura

    2011-02-01

    Full Text Available OBJECTIVE: To compare optical coherence tomography (OCT measurements on the retinal nerve fiber layer (RNFL of healthy controls and patients with longitudinally extensive transverse myelitis (LETM without previous optic neuritis. METHOD: Twenty-six eyes from 26 patients with LETM and 26 control eyes were subjected to automated perimetry and OCT for comparison of RNFL measurements. RESULTS: The mean deviation values from perimetry were significantly lower in patients with LETM than in controls (p<0.0001. RNFL measurements in the nasal quadrant and in the 3-o'clock segment were significantly smaller in LETM eyes than in controls. (p=0.04 and p=0.006, respectively. No significantly differences in other RNFL measurements were found. CONCLUSION: Patients with LETM may present localized RNFL loss, particularly on the nasal side of the optic disc, associated with slight visual field defects, even in the absence of previous episodes of optic neuritis. These findings emphasize the fact that patients with LETM may experience attacks of subclinical optic nerve damage.

  8. Patulous Subarachnoid Space of the Optic Nerve Associated with X-Linked Hypophosphatemic Rickets.

    Science.gov (United States)

    Galvez-Ruiz, Alberto; Chaudhry, Imtiaz

    2013-01-01

    Although the deficiency forms are the most common manifestations of rickets, there are other forms of rickets that are resistant to vitamin D. Of these, the most common is X-linked hypophosphatemic rickets. Rickets represents a group of multiple cranial bone disorders-craniosynostosis and the presence of Chari I malformation being the most notable-that explain the increase in intracranial pressure. We present a 4-year-old patient with an unusual association of X-linked hypophosphataemic rickets, bilateral proptosis, and prominent bilateral widening of the optic nerve sheaths. Although the association between intracranial hypertension and rickets is known, to the best of our knowledge, such a prominent distention of the subarachnoid space of the optic nerve without papilloedema has not been previously described.

  9. Foveal hemorrhage in an eye with foveal hypoplasia associated with albinism.

    Science.gov (United States)

    Masuda, Naonori; Hasegawa, Taiji; Yamashita, Mariko; Ogata, Nahoko

    2014-01-01

    Oculocutaneous albinism is a group of congenital disorders caused by alterations of melanin biosynthesis. We report our findings in a patient with oculocutaneous albinism who presented with foveal hypoplasia and a foveal hemorrhage. A 48-year-old man noted a dark spot in the middle of the visual field of his right eye. He had depigmented skin, white hair, white eyebrows, and white cilia. He also had horizontal nystagmus and depigmented irides. His best-corrected visual acuity was 2/100 with -14.0 diopters in the right eye and 3/100 with -5.0 diopters in the left eye. Ophthalmoscopy showed diffuse depigmentation in both eyes and a foveal hemorrhage in the right eye. Optical coherence tomography showed the absence of a foveal pit in both eyes and a subretinal hyperreflective lesion corresponding to the foveal hemorrhage in the right eye. Fluorescein angiography showed that the retinal and choroidal vessels were relatively hypofluorescent because of the lack of a blocking effect of the pigments in the retinal pigment epithelium. Fluorescein angiography and indocyanine green angiography did not show any evidence of choroidal neovascularization in either eye. The foveal hemorrhage in the right eye spontaneously regressed and finally resolved at 3 months after onset. At the final examination, the patient reported that his vision had recovered. A foveal hemorrhage is a rare condition in an eye with foveal hypoplasia associated with albinism. The hemorrhage may be related to high myopia and also to the hypoplasia of the fovea associated with albinism.

  10. [Measuring contrast sensitivity using visual acuity tests in retinal and optic nerve diseases].

    Science.gov (United States)

    Sommer, E; Marré, E; Mierdel, P

    1990-01-01

    The luminance contrast needed to discern various test types was measured with monochromatic and achromatic light to detect discrete functional deficiencies of the retina and optic nerve in cases of normal visual acuity. Landolt rings corresponding to visual acuity levels from 0.04 to 1.0 were used as test types. A significant increase in the necessary minimum contrast was detectable with blue test light on large Landolt rings in patients with diabetic retinopathy, ocular hypertension and glaucoma and with green or yellow test light on medium-sized and small Landolt rings in patients with central serous chorioidopathy and optic atrophy. The additional contrast needed to reach the maximum visual acuity amounts to 14-100% compared with normal visual acuity, depending on the color of the test light and the diagnosis. The amount of contrast needed is greatest in retinal diseases, and it is therefore possible to a certain extent to distinguish these from diseases of the optic nerve.

  11. [Progression of nerve fiber layer defects in retrobulbar optic neuritis by the macular ganglion cell complex].

    Science.gov (United States)

    Hong, D; Bosc, C; Chiambaretta, F

    2017-11-01

    Recent studies with SD OCT had shown early axonal damage to the macular ganglion cell complex (which consists of the three innermost layers of the retina: Inner Plexiform Layer [IPL], Ganglion Cell Layer [GCL], Retinal Nerve Fibre layer [RNFL]) in optic nerve pathology. Retrobulbar optic neuritis (RBON), occurring frequently in demyelinating diseases, leads to atrophy of the optic nerve fibers at the level of the ganglion cell axons, previously described in the literature. The goal of this study is to evaluate the progression of optic nerve fiber defects and macular ganglion cell complex defects with the SPECTRALIS OCT via a reproducible method by calculating a mean thickness in each quadrant after an episode of retrobulbar optic neuritis. This is a prospective monocentric observational study including 8 patients at the Clermont-Ferrand university medical center. All patients underwent ocular examination with macular and disc OCT analysis and a Goldmann visual field at the time of inclusion (onset or recurrence of RBON), at 3 months and at 6 months. Patients were 40-years-old on average at the time of inclusion. After 6 months of follow-up, there was progression of the atrophy of the macular ganglion cell complex in the affected eye on (11.5% or 11μm) predominantly inferonasally (13.9% or 16μm) and superonasally (12.9% or 14μm) while the other eye remained stable. The decrease in thickness occurred mainly in the most internal 3 layers of the retina. On average, the loss in thickness of the peripapillary RNFL was predominantly inferotemporal (24.9% or 39μm) and superotemporal (21.8% or 28μm). In 3 months of progression, the loss of optic nerve fibers is already seen on macular and disc OCT after an episode of RBON, especially in inferior quadrants in spite of the improvement in the Goldmann visual field and visual acuity. Segmentation by quadrant was used here to compare the progression of the defect by region compared to the fovea in a global and reproducible

  12. Retinal and Optic Nerve Degeneration in Patients with Multiple Sclerosis Followed up for 5 Years.

    Science.gov (United States)

    Garcia-Martin, Elena; Ara, Jose R; Martin, Jesus; Almarcegui, Carmen; Dolz, Isabel; Vilades, Elisa; Gil-Arribas, Laura; Fernandez, Francisco J; Polo, Vicente; Larrosa, Jose M; Pablo, Luis E; Satue, Maria

    2017-05-01

    To quantify retinal nerve fiber layer (RNFL) changes in patients with multiple sclerosis (MS) and healthy controls with a 5-year follow-up and to analyze correlations between disability progression and RNFL degeneration. Observational and longitudinal study. One hundred patients with relapsing-remitting MS and 50 healthy controls. All participants underwent a complete ophthalmic and electrophysiologic exploration and were re-evaluated annually for 5 years. Visual acuity (Snellen chart), color vision (Ishihara pseudoisochromatic plates), visual field examination, optical coherence tomography (OCT), scanning laser polarimetry (SLP), and visual evoked potentials. Expanded Disability Status Scale (EDSS) scores, disease duration, treatments, prior optic neuritis episodes, and quality of life (QOL; based on the 54-item Multiple Sclerosis Quality of Life Scale score). Optical coherence tomography (OCT) revealed changes in all RNFL thicknesses in both groups. In the MS group, changes were detected in average thickness and in the mean deviation using the GDx-VCC nerve fiber analyzer (Laser Diagnostic Technologies, San Diego, CA) and in the P100 latency of visual evoked potentials; no changes were detected in visual acuity, color vision, or visual fields. Optical coherence tomography showed greater differences in the inferior and temporal RNFL thicknesses in both groups. In MS patients only, OCT revealed a moderate correlation between the increase in EDSS and temporal and superior RNFL thinning. Temporal RNFL thinning based on OCT results was correlated moderately with decreased QOL. Multiple sclerosis patients exhibit a progressive axonal loss in the optic nerve fiber layer. Retinal nerve fiber layer thinning based on OCT results is a useful marker for assessing MS progression and correlates with increased disability and reduced QOL. Copyright © 2017 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

  13. Septo-optic dysplasia: case report Displasia septo-óptica: relato de caso

    Directory of Open Access Journals (Sweden)

    Paulo de Tarso P. Pierre Filho

    2004-08-01

    Full Text Available The clinical triad of septo-optic dysplasia (SOD comprises the absence of the septum pellucidum, congenital optic nerve dysplasia, and multiple endocrine disorders. When any two of these factors are present, the condition is defined as an incomplete form of SOD. The authors report the case of an incomplete form of SOD in a 9-year-old boy with low vision and nystagmus present from birth. The bilateral ophthalmoscopic examination revealed small papillae with double contour images. Magnetic resonance imaging showed hypoplasia of the optic nerve bilaterally, chiasm and absence of the septum pellucidum.A tríade clínica da displasia septo-óptica (DSO compreende a ausência de septo pelúcido, displasia congênita do nervo óptico e múltiplos defeitos endócrinos. Uma forma incompleta de DSO tem sido reconhecida com dois dos três elementos. Os autores relatam um caso de displasia septo-óptica na forma incompleta em um garoto de 9 anos com baixa visão e nistagmo presentes desde o nascimento. O exame oftalmoscópico revelou papila pequena com imagem em duplo contorno bilateralmente. Ressonância magnética mostrou hipoplasia dos nervos ópticos, quiasma e ausência do septo pelúcido.

  14. Protection by an oral disubstituted hydroxylamine derivative against loss of retinal ganglion cell differentiation following optic nerve crush.

    Science.gov (United States)

    Lindsey, James D; Duong-Polk, Karen X; Dai, Yi; Nguyen, Duy H; Leung, Christopher K; Weinreb, Robert N

    2013-01-01

    Thy-1 is a cell surface protein that is expressed during the differentiation of retinal ganglion cells (RGCs). Optic nerve injury induces progressive loss in the number of RGCs expressing Thy-1. The rate of this loss is fastest during the first week after optic nerve injury and slower in subsequent weeks. This study was undertaken to determine whether oral treatment with a water-soluble N-hydroxy-2,2,6,6-tetramethylpiperidine derivative (OT-440) protects against loss of Thy-1 promoter activation following optic nerve crush and whether this effect targets the earlier quick phase or the later slow phase. The retina of mice expressing cyan fluorescent protein under control of the Thy-1 promoter (Thy1-CFP mice) was imaged using a blue-light confocal scanning laser ophthalmoscope (bCSLO). These mice then received oral OT-440 prepared in cream cheese or dissolved in water, or plain vehicle, for two weeks and were imaged again prior to unilateral optic nerve crush. Treatments and weekly imaging continued for four more weeks. Fluorescent neurons were counted in the same defined retinal areas imaged at each time point in a masked fashion. When the counts at each time point were directly compared, the numbers of fluorescent cells at each time point were greater in the animals that received OT-440 in cream cheese by 8%, 27%, 52% and 60% than in corresponding control animals at 1, 2, 3 and 4 weeks after optic nerve crush. Similar results were obtained when the vehicle was water. Rate analysis indicated the protective effect of OT-440 was greatest during the first two weeks and was maintained in the second two weeks after crush for both the cream cheese vehicle study and water vehicle study. Because most of the fluorescent cells detected by bCSLO are RGCs, these findings suggest that oral OT-440 can either protect against or delay early degenerative responses occurring in RGCs following optic nerve injury.

  15. Protection by an oral disubstituted hydroxylamine derivative against loss of retinal ganglion cell differentiation following optic nerve crush.

    Directory of Open Access Journals (Sweden)

    James D Lindsey

    Full Text Available Thy-1 is a cell surface protein that is expressed during the differentiation of retinal ganglion cells (RGCs. Optic nerve injury induces progressive loss in the number of RGCs expressing Thy-1. The rate of this loss is fastest during the first week after optic nerve injury and slower in subsequent weeks. This study was undertaken to determine whether oral treatment with a water-soluble N-hydroxy-2,2,6,6-tetramethylpiperidine derivative (OT-440 protects against loss of Thy-1 promoter activation following optic nerve crush and whether this effect targets the earlier quick phase or the later slow phase. The retina of mice expressing cyan fluorescent protein under control of the Thy-1 promoter (Thy1-CFP mice was imaged using a blue-light confocal scanning laser ophthalmoscope (bCSLO. These mice then received oral OT-440 prepared in cream cheese or dissolved in water, or plain vehicle, for two weeks and were imaged again prior to unilateral optic nerve crush. Treatments and weekly imaging continued for four more weeks. Fluorescent neurons were counted in the same defined retinal areas imaged at each time point in a masked fashion. When the counts at each time point were directly compared, the numbers of fluorescent cells at each time point were greater in the animals that received OT-440 in cream cheese by 8%, 27%, 52% and 60% than in corresponding control animals at 1, 2, 3 and 4 weeks after optic nerve crush. Similar results were obtained when the vehicle was water. Rate analysis indicated the protective effect of OT-440 was greatest during the first two weeks and was maintained in the second two weeks after crush for both the cream cheese vehicle study and water vehicle study. Because most of the fluorescent cells detected by bCSLO are RGCs, these findings suggest that oral OT-440 can either protect against or delay early degenerative responses occurring in RGCs following optic nerve injury.

  16. Septooptic dysplasia : a case report

    International Nuclear Information System (INIS)

    Kim, Jung Hae; Choi, Dae Seob; Lee, Chang Wook; Kim, Soon; Kim, Seung Hyeon; Lee, Sung Woo; Ha, Jung Ho; Sakong, Jung Kyu; Lee, Hyeon Kyeong

    2001-01-01

    Septooptic dysplasia is a rare anterior midline anomaly considered to be a mild form of lobar holoprosencephaly. We describe a case with unilateral optic nerve hypoplasia and the absence of a septum pellucidum

  17. Imaging of optic nerve head pore structure with motion corrected deeply penetrating OCT using tracking SLO

    NARCIS (Netherlands)

    Vienola, Kari V.; Braaf, Boy; Sheehy, Christy K.; Yang, Qiang; Tiruveedhula, Pavan; de Boer, Johannes F.; Roorda, Austin

    2013-01-01

    Purpose To remove the eye motion and stabilize the optical frequency domain imaging (OFDI) system for obtaining high quality images of the optic nerve head (ONH) and the pore structure of the lamina cribrosa. Methods An optical coherence tomography (OCT) instrument was combined with an active eye

  18. Silicone oil migration along the optic nerve after intraocular tamponade.

    Science.gov (United States)

    Gargallo Vaamonde, Á; Ibáñez Muñoz, D; Salceda Artola, J; Garatea Aznar, P; Zalazar, R; Yanguas Barea, N

    2016-11-01

    We present a case of silicone oil migration trough the optic nerve in a diabetic patient with retinal detachment and review the etiologic mechanism and clinical implications. Intracranial silicone oil migration is an uncommon complication associated with silicone oil tamponade. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  19. Clinical Features and Management of Cartilage-Hair Hypoplasia: A Narrative Review

    Directory of Open Access Journals (Sweden)

    Kobra Shiasi Arani

    2015-01-01

    Full Text Available Context: Cartilage-hair hypoplasia is a rare hereditary cause of short stature. The aim of this study was to familiarize physicians with this rare but important disease. Evidence Acquisition: This article is a narrative review of the scientific literature to inform about clinical features and management of Cartilage-hair hypoplasia. A systematic search identified 127 papers include original and review articles and case reports. Results: Cartilage-Hair Hypoplasia characterized by short-limb dwarfism associated with metaphyseal chondrodysplasia. The inheritance is autosomal recessive. Other findings include hair hypoplasia, anemia, immunodeficiency, propensity to infections, gastrointestinal disorders (Hirschsprung disease, anal stenosis, esophageal atresia and malabsorption, defective spermatogenesis, increased risk of malignancies and higher rate of mortality. Immunodeficiency in cartilage-hair hypoplasia may be an isolated B-cell or isolated T-cell immunodeficiency or combined B and T-cell immunodeficiency; however, severe combined immunodeficiency is rare. There is no known treatment for hair hypoplasia. Growth hormone was used with conflicting results for short stature in children with Cartilage-hair hypoplasia. Skeletal problems must be managed with physiotherapy and appropriate orthopedic interventions. Hirschsprung disease, anal stenosis and esophageal atresia should be surgically corrected. Patients with severe hypoplastic anemia require repeated transfusions. Bone marrow transplantation may be required for patients with severe combined immunodeficiency or severe persistent hypoplastic anemia. Treatment with G-CSF is useful for neutropenia. Patients should be monitored closely for developing malignancy such as skin neoplasms, lymphomas and leukemias. Conclusions: Cartilage-hair hypoplasia is an important hereditary disease with different medical aspects. The high rate of consanguineous marriages in Iran necessitates considering CHH in any

  20. Dominant inheritance of retinal ganglion cell resistance to optic nerve crush in mice

    Directory of Open Access Journals (Sweden)

    Schlamp Cassandra L

    2007-03-01

    Full Text Available Abstract Background Several neurodegenerative diseases are influenced by complex genetics that affect an individual's susceptibility, disease severity, and rate of progression. One such disease is glaucoma, a chronic neurodegenerative condition of the eye that targets and stimulates apoptosis of CNS neurons called retinal ganglion cells. Since ganglion cell death is intrinsic, it is reasonable that the genes that control this process may contribute to the complex genetics that affect ganglion cell susceptibility to disease. To determine if genetic background influences susceptibility to optic nerve damage, leading to ganglion cell death, we performed optic nerve crush on 15 different inbred lines of mice and measured ganglion cell loss. Resistant and susceptible strains were used in a reciprocal breeding strategy to examine the inheritance pattern of the resistance phenotype. Because earlier studies had implicated Bax as a susceptibility allele for ganglion cell death in the chronic neurodegenerative disease glaucoma, we conducted allelic segregation analysis and mRNA quantification to assess this gene as a candidate for the cell death phenotype. Results Inbred lines showed varying levels of susceptibility to optic nerve crush. DBA/2J mice were most resistant and BALB/cByJ mice were most susceptible. F1 mice from these lines inherited the DBA/2J phenotype, while N2 backcross mice exhibited the BALB/cByJ phenotype. F2 mice exhibited an intermediate phenotype. A Wright Formula calculation suggested as few as 2 dominant loci were linked to the resistance phenotype, which was corroborated by a Punnett Square analysis of the distribution of the mean phenotype in each cross. The levels of latent Bax mRNA were the same in both lines, and Bax alleles did not segregate with phenotype in N2 and F2 mice. Conclusion Inbred mice show different levels of resistance to optic nerve crush. The resistance phenotype is heritable in a dominant fashion involving

  1. The effect of the menstrual cycle on optic nerve head analysis in healthy women.

    Science.gov (United States)

    Akar, Munire Erman; Taskin, Omur; Yucel, Iclal; Akar, Yusuf

    2004-12-01

    To determine the effect of the menstrual cycle on optic nerve head topographic analysis in normally menstruating, healthy women. The study included single eyes selected randomly from each of 52 healthy women with regular menstrual cycles. All subjects underwent a complete ocular examination. Optic nerve head topographic analyses were performed using a confocal scanning laser ophthalmoscope, the Heidelberg Retinal Tomograph II (HRT II, software version 1.6). The analyses were repeated three times during the menstrual cycle: in the follicular phase (days 7-10 of the cycle), at ovulation, and in the late luteal phase (days 1-3 before menstrual bleeding). Serum oestradiol, progesterone and luteinizing hormone levels were measured at each menstrual phase. Fourteen subjects were excluded from the study. The mean age of the subjects (n = 38) was 25.6 +/- 3.7 years (range 21-34 years). Blood oestradiol levels were significantly lower in the late luteal phase (35.8 pg/ml) (p cup : disc ratio, cup : disc area ratio and the cup area were significantly higher during the luteal phase (p menstrual cycle in healthy women significantly alter neuroretinal rim area and cup variables of the optic nerve head. These findings should be taken into consideration in the clinical follow-up of young women with glaucoma.

  2. Influence of corneal power on circumpapillary retinal nerve fiber layer and optic nerve head measurements by spectral-domain optical coherence tomography

    Directory of Open Access Journals (Sweden)

    Kazunori Hirasawa

    2017-09-01

    Full Text Available AIM: To evaluate the influence of corneal power on circumpapillary retinal nerve fiber layer (cpRNFL and optic nerve head (ONH measurements by spectral-domain optical coherence tomography (SD-OCT. METHODS: Twenty-five eyes of 25 healthy participants (mean age 23.6±3.6y were imaged by SD-OCT using horizontal raster scans. Disposable soft contact lenses of different powers (from −11 to +5 diopters including 0 diopter were worn to induce 2-diopter changes in corneal power. Differences in the cpRNFL and ONH measurements per diopter of change in corneal power were analyzed. RESULTS: As corneal power increased by 1 diopter, total and quadrant cpRNFL thicknesses, except for the nasal sector, decreased by −0.19 to −0.32 μm (P<0.01. Furthermore, the disc, cup, and rim areas decreased by −0.017, −0.007, and −0.015 mm2, respectively (P<0.001; the cup and rim volumes decreased by −0.0013 and −0.006 mm3, respectively (P<0.01; and the vertical and horizontal disc diameters decreased by −0.006 and −0.007 mm, respectively (P<0.001. CONCLUSION: For more precise OCT imaging, the ocular magnification should be corrected by considering both the axial length and corneal power. However, the effect of corneal power changes on cpRNFL thickness and ONH topography are small when compare with those of the axial length.

  3. Surgical decompression in endocrine orbitopathy. Visual evoked potential evaluation and effect on the optic nerve.

    Science.gov (United States)

    Clauser, Luigi C; Tieghi, Riccardo; Galie', Manlio; Franco, Filippo; Carinci, Francesco

    2012-10-01

    Endocrine orbitopathy (EO) represents the most frequent and important extrathyroidal stigma of Graves disease. This chronic autoimmune condition involves the orbital contents, including extraocular muscles, periorbital connective-fatty tissue and lacrimal gland. The increase of fat tissue and the enlargement of extraocular muscles within the bony confines of the orbit leads to proptosis, and in the most severe cases optic neuropathy, caused by compression and stretching of the optic nerve. The congestion and the pressure of the enlarged muscles, constrict the nerve and can lead to reduced sight or loss of vision with the so called "orbital apex syndrome". Generally surgical treatment of EO, based on fat and/or orbital wall expansion, is possible and effective in improving exophthalmos and diplopia. Since there are limited reports focussing on optic neuropathy recovery after fat and/or orbital walls decompression the Authors decided to perform a retrospective analysis on a series of patients affected by EO. The study population was composed of 10 patients affected by EO and presenting to the Unit of Cranio Maxillofacial Surgery, Center for Craniofacial Deformities & Orbital Surgery St. Anna Hospital and University, Ferrara, Italy, for evaluation and treatment. A complete Visual Evoked Potentials (VEP) evaluation was performed. There were seven women and three men with a median age of 55 years. Optic nerve VEP amplitude and latency were recorded as normal or pathological. Abnormal results were scored as moderate, mild and severe. Differences in VEP pre and post-operatively were recorded as present or absent (i.e. VEP Delta). Pearson chi square test was applied. There were 20 operated orbits. The first VEP evaluation was performed 3.2 months before surgery and post-operative VEP control was done after a mean of 18.7 months. Fat decompression was performed in all cases and eight patients had also bony decompression. VEP amplitude and latency were affected in 10 and 15

  4. Prevalence of Split Nerve Fiber Layer Bundles in Healthy People Imaged with Spectral Domain Optical Coherence Tomography

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    Sirel Gür Güngör

    2016-12-01

    Full Text Available Objectives: The presence of retinal nerve fiber layer (RNFL split bundles was recently described in normal eyes scanned using scanning laser polarimetry and by histologic studies. Split bundles may resemble RNFL loss in healthy eyes. The aim of our study was to determine the prevalence of nerve fiber layer split bundles in healthy people. Materials and Methods: We imaged 718 eyes of 359 healthy persons with the spectral domain optical coherence tomography in this cross-sectional study. All eyes had intraocular pressure of 21 mmHg or less, normal appearance of the optic nerve head, and normal visual fields (Humphrey Field Analyzer 24-2 full threshold program. In our study, a bundle was defined as ‘split’ when there is localized defect not resembling a wedge defect in the RNFL deviation map with a symmetrically divided RNFL appearance on the RNFL thickness map. The classification was performed by two independent observers who used an identical set of reference examples to standardize the classification. Results: Inter-observer consensus was reached in all cases. Bilateral superior split bundles were seen in 19 cases (5.29% and unilateral superior split was observed in 15 cases (4.16%. In 325 cases (90.52% there was no split bundle. Conclusion: Split nerve fiber layer bundles, in contrast to single nerve fiber layer bundles, are not common findings in healthy eyes. In eyes with normal optic disc appearance, especially when a superior RNFL defect is observed in RNFL deviation map, the RNLF thickness map and graphs should also be examined for split nerve fiber layer bundles.

  5. Computed tomographic features of the bony canal of the cochlear nerve in pediatric patients with unilateral sensorineural hearing loss

    International Nuclear Information System (INIS)

    Kono, Tatsuo

    2008-01-01

    The aim of this study was to evaluate the diameters of the various bony canals of the inner ear in patients with sensorineural hearing loss (SNHL) and establish criteria for detecting hypoplasia of the bony canal of the cochlear nerve. Measurements obtained in 118 patients without inner ear malformations among 160 patients with unilateral SNHL were analyzed. The diameters of the internal auditory canal and the bony canals of the cochlear, vestibular, and facial nerves were measured on transverse or coronal computed tomographic images. Mean values (±standard deviation (SD)) were compared between the affected and unaffected ears, and statistical analysis was done. The diameter of the bony canal of the cochlear nerve was significantly smaller in affected ears than in unaffected ears (P<0.01). The affected ears could be divided into groups with (72 ears) and without (46 ears) bony canal stenosis. Most (60%) of the patients with unilateral SNHL showed a significant difference in the diameters of the bony canals of the cochlear nerve between the affected and unaffected sides; moreover, the mean value was significantly smaller in affected ears. The diameter of <1.7 mm on transverse images or <1.8 mm on coronal images suggests hypoplasia. (author)

  6. Quantitative comparison of disc rim color in optic nerve atrophy of compressive optic neuropathy and glaucomatous optic neuropathy.

    Science.gov (United States)

    Nakano, Eri; Hata, Masayuki; Oishi, Akio; Miyamoto, Kazuaki; Uji, Akihito; Fujimoto, Masahiro; Miyata, Manabu; Yoshimura, Nagahisa

    2016-08-01

    The purpose was to investigate an objective and quantitative method to estimate the redness of the optic disc neuroretinal rim, and to determine the usefulness of this method to differentiate compressive optic neuropathy (CON) from glaucomatous optic neuropathy (GON). In our study there were 126 eyes: 40 with CON, 40 with normal tension glaucoma (NTG), and 46 normal eyes (NOR). Digital color fundus photographs were assessed for the redness of disc rim color using ImageJ software. We separately measured the intensity of red, green, and blue pixels from RGB images. Three disc color indices (DCIs), which indicate the redness intensity, were calculated through existing formulas. All three DCIs of CON were significantly smaller than those of NOR (P  -6 dB), in which the extent of retinal nerve fiber layer thinning is comparable, the DCIs of mild CON were significantly smaller than those of mild NTG (P optic disc color was useful in differentiating early-stage CON from GON and NOR.

  7. Indomethacin lowers optic nerve oxygen tension and reduces the effect of carbonic anhydrase inhibition and carbon dioxide breathing

    DEFF Research Database (Denmark)

    Pedersen, D B; Eysteinsson, T; Stefánsson, E

    2004-01-01

    Prostaglandins are important in blood flow regulation. Carbon dioxide (CO(2)) breathing and carbonic anhydrase inhibition increase the oxygen tension in the retina and optic nerve. To study the mechanism of this effect and the role of cyclo-oxygenase in the regulation of optic nerve oxygen tension...... (ONPO(2)), the authors investigated how indomethacin affects ONPO(2) and the ONPO(2) increases caused by CO(2) breathing and carbonic anhydrase inhibition in the pig....

  8. Association between optic nerve blood flow and objective examinations in glaucoma patients with generalized enlargement disc type

    Directory of Open Access Journals (Sweden)

    Nakazawa T

    2011-10-01

    Full Text Available Naoki Chiba, Kazuko Omodaka, Yu Yokoyama, Naoko Aizawa, Satoru Tsuda, Masayuki Yasuda, Takaaki Otomo, Shunji Yokokura, Nobuo Fuse, Toru Nakazawa Department of Ophthalmology, Tohoku University Graduate School of Medicine, Sendai, Japan Background: The purpose of this study was to investigate the correlations between microcirculation in the optic disc, average peripapillary retinal nerve fiber layer thickness cupping parameters, and visual field defects in glaucoma patients with the generalized enlargement disc type. Methods: A total of 38 eyes from 38 glaucoma patients with the generalized enlargement disc type were included. The microcirculation of the optic nerve head was examined with laser speckle flow graphy, and the mean blur rate in all areas, in vessel area, and in tissue area were calculated using the laser speckle flow graphy analyzer software. Average peripapillary retinal nerve fiber layer thickness was measured using Stratus optical coherence tomography, and cupping parameters were accessed using the Heidelberg retina tomograph. The mean deviation in the Humphrey field analyzer (30-2 SITA standard was analyzed. The correlation between these parameters was evaluated using the Spearman rank correlation coefficient. Results: The correlation coefficient of mean blur rate in all optic disc area to the average peripapillary retinal nerve fiber layer thickness, vertical C/D, and mean deviation were r = 0.7546 (P < 0.0001, r = –0.6208 (P < 0.0001, and r = 0.6010 (P = 0.0001, respectively. The mean blur rate in tissue area of the optic disc showed r = 0.7305 (P < 0.0001, r = –0.6438 (P < 0.0001, and r = 0.6338 (P < 0.0001. Conclusion: We found that the mean blur rate in the optic disc was significantly correlated with the average peripapillary retinal nerve fiber layer thickness, vertical C/D, and mean deviation in patients with the generalized enlargement disc type of glaucoma. In particular, the mean blur rate in tissue area was more

  9. Compliance between clinical and genetic diagnosis of choroidal hypoplasia in 103 Norwegian Border Collie puppies.

    Science.gov (United States)

    Grosås, Siv; Lingaas, Frode; Prestrud, Kristin Wear; Ropstad, Ernst-Otto

    2017-11-07

    To describe the frequency of the nonhomologous end-joining factor 1 (NHEJ1) mutation and the compliance between clinical and genetic diagnosis of choroidal hypoplasia (CH) in a group of Norwegian Border Collies. Border collie puppies in the age from 5 to 8 weeks. Puppies included in the study had a complete ophthalmological examination. All findings were recorded, and an ECVO scheme form was issued for each puppy. DNA samples were achieved from buccal swabs. Genetic typing was performed for the 7.8-kb deletion in the gene encoding NHEJ1. Dogs with none, one, or two copies of the mutated allele were classified as free, carriers, and affected, respectively. 103 Border Collie puppies from 16 litters, 52 females and 51 males, were included in the study. Ages ranged from 5.1 to 8.9 weeks. One puppy had clinical findings consistent with CH and optic nerve coloboma compatible with the diagnosis Collie Eye Anomaly (CEA). Findings on ophthalmological examination of the remaining puppies were within normal limits. On genetic testing, 85 puppies were clear of the mutation in the NHEJ1 gene, 17 puppies were carriers, and one puppy was genetically affected. A good compliance between the clinical diagnosis and the genetic test results was found in all of the puppies examined. The allele frequency of the mutation was 6.3%. © 2017 American College of Veterinary Ophthalmologists.

  10. Transsynaptic neuronal degeneration of optic nerves associated with bilateral occipital lesions

    Directory of Open Access Journals (Sweden)

    Sachdev Mahipal

    1990-01-01

    Full Text Available A case is reported of a 9-year old male who presented with abnormal behaviour and progressive diminution of vision. Pupils were middilated in both eyes but the pupillary reflexes were preserved. Fundus examination revealed a bilateral optic atrophy and radiological investigations showed a bilateral occipital calcification. We hereby document a case of retrograde transsynaptic neuronal degeneration of the visual system secondary to bilateral occipital lesions. Transsynapptic neuronal degeneration of optic nerves consequent to occipital lobe lesions is a rare phenomenon. Experimentally occipital lobe ablation in non-human primates has been shown to result in optic atrophy. Herein, we document a case of retrograde transsynaptic neuronal degeneration of the visual system secondary to bilateral occipital lesions.

  11. Change of Retinal Nerve Layer Thickness in Non-Arteritic Anterior Ischemic Optic Neuropathy Revealed by Fourier Domain Optical Coherence Tomography.

    Science.gov (United States)

    Han, Mei; Zhao, Chen; Han, Quan-Hong; Xie, Shiyong; Li, Yan

    2016-08-01

    To examine the changes of non-arteritic anterior ischemic optic neuropathy (NAION) by serial morphometry using Fourier domain optical coherence tomography (FD-OCT). Retrospective study in patients with newly diagnosed NAION (n=33, all unilateral) and controls (n=75 unilateral NAION patients with full contralateral eye vision) who underwent FD-OCT of the optic disk, optic nerve head (ONH), and macula within 1 week of onset and again 1, 3, 6, and 12 months later. The patients showed no improvement in vision during follow-up. Within 1 week of onset, all NAION eyes exhibited severe ONH fiber crowding and peripapillary retinal nerve fiber layer (RNFL) edema. Four had subretinal fluid accumulation and 12 had posterior vitreous detachment (PVD) at the optic disc surface. Ganglion cell complex (GCC) and RNFL thicknesses were reduced at 1 and 3 months (p < 0.05), with no deterioration thereafter. Initial RNFL/GCC contraction magnitude in the superior hemisphere correlated with the severity of inferior visual field deficits. NAION progression is characterized by an initial phase of accelerated RNFL and GCC deterioration. These results reveal that the kinetic change of neural retina in NAION and may have implication on the time window for treatment of NAION. FD-OCT is useful in the evaluation of NAION.

  12. Probabilistic Modeling of Intracranial Pressure Effects on Optic Nerve Biomechanics

    Science.gov (United States)

    Ethier, C. R.; Feola, Andrew J.; Raykin, Julia; Myers, Jerry G.; Nelson, Emily S.; Samuels, Brian C.

    2016-01-01

    Altered intracranial pressure (ICP) is involved/implicated in several ocular conditions: papilledema, glaucoma and Visual Impairment and Intracranial Pressure (VIIP) syndrome. The biomechanical effects of altered ICP on optic nerve head (ONH) tissues in these conditions are uncertain but likely important. We have quantified ICP-induced deformations of ONH tissues, using finite element (FE) and probabilistic modeling (Latin Hypercube Simulations (LHS)) to consider a range of tissue properties and relevant pressures.

  13. Comparison of the Deep Optic Nerve Head Structure between Normal-Tension Glaucoma and Nonarteritic Anterior Ischemic Optic Neuropathy.

    Science.gov (United States)

    Lee, Eun Ji; Choi, Yun Jeong; Kim, Tae-Woo; Hwang, Jeong-Min

    2016-01-01

    To compare the deep optic nerve head (ONH) structure between normal-tension glaucoma (NTG) and nonarteritic anterior ischemic optic neuropathy (NAION) and also in healthy subjects as a control using enhanced depth imaging (EDI) spectral-domain optical coherence tomography (SD-OCT). This prospective cross-sectional study included 21 NAION patients who had been diagnosed as NAION at least 6 months prior to study entry, and 42 NTG patients and 42 healthy controls who were matched with NAION patients in terms of age, intraocular pressure (IOP), and optic disc area. The retinal nerve fiber layer (RNFL) thickness in the affected sector was also matched between NAION and NTG patients. The ONH was imaged using SD-OCT with the EDI technique. The anterior lamina cribrosa surface depth (LCD) and average prelaminar tissue (PT) thickness were measured in a sector of interest in each eye and compared among the three groups. In the sector-matched comparison, LCD was largest in NTG patients, followed by NAION patients, while PT was thinner in NTG patients than in NAION patients (all P < 0.001). NAION patients had a comparable LCD and a thinner PT relative to normal controls (P = 0.170 and < 0.001, respectively). The deep ONH configuration is strikingly different between NTG and NAION. The differing features provide comparative insight into the pathophysiology of the two diseases, and may be useful for differential diagnosis.

  14. Dural Ectasia of the Optic Nerve and Unilateral Proptosis-Two Rare Comorbidities Associated with Idiopathic Intracranial Hypertension

    Directory of Open Access Journals (Sweden)

    Serkan Dağdelen

    2013-08-01

    Full Text Available A 24-year-old woman with a recent history of visual abnormality (obscurations had bilateral optic disc swellings, dilatation of the optic nerve sheaths, monocular proptosis, and increased cerebrospinal fluid pressures. She was diagnosed as idiopathic intracranial hypertension (IIH. IIH can present with both papilledema and dural ectasia although the latter is usually seen as an isolated entity. Various conditions also have been implicated in IIH. However, apart from female sex, recent weight gain and obesity, there are no proven associations. In this case report, we present a case of IHH associated with two different pathologies: dural ectasia of the optic nerve sheaths and monocular proptosis. (Turk J Ophthalmol 2013; 43: 297-300

  15. Retinal nerve fiber layer thickness map determined from optical coherence tomography images

    NARCIS (Netherlands)

    Mujat, M.; Chan, R. C.; Cense, B.; Park, B.H.; Joo, C.; Akkin, T.; Chen, TC; de Boer, JF

    2005-01-01

    We introduce a method to determine the retinal nerve fiber layer (RNFL) thickness in OCT images based on anisotropic noise suppression and deformable splines. Spectral-Domain Optical Coherence Tomography (SDOCT) data was acquired at 29 kHz A-line rate with a depth resolution of 2.6 mum and a depth

  16. Improvement of visual acuity and VEP after optic nerve contusion by NGF and its safety analysis

    Directory of Open Access Journals (Sweden)

    Ming Zhao

    2018-02-01

    Full Text Available AIM:To investigate the effect of neuropathic factor(NGFon visual acuity and visual evoked potential(VEPin patients with optic nerve contusion. METHODS:Totally 78 patients(78 eyeswith optic nerve contusion were selected. From January 2013 to June 2016, 39 cases(39 eyeswere divided into observation group and control group respectively according to the random number table method. Prednisone, vitamins and mecobalamin tablets treatment were given to both groups, based on that, the observation group was given NGF treatment, continuous treatment of 2 courses(21d for a course of treatment. RESULTS: There was no significant difference in visual field defect and visual field sensitivity between the observation group and the control group before treatment(P>0.05. After treatment, the visual field defect degree of the observation group was smaller, the visual field sensitivity was better than that of the control group(PP>0.05. After treatment, the P100 wave latency of the observation group was significantly shorter than that of the control group(PPPCONCLUSION: NGF treatment for optic nerve contusion can significantly improve the patient's visual acuity, VEP indicators, reduce visual field defects, improve visual field sensitivity.

  17. Optic nerve sheath meningioma treated with radiation conformal therapy. Clinical case report with long follow up

    International Nuclear Information System (INIS)

    Zomosa R, Gustavo; Cruz T, Sebastian; Miranda G, Gonzalo; Harbst S, Hans

    2016-01-01

    Optic nerve sheath meningiomas (ONSM) are rare tumors of the anterior visual pathway. Without treatment, tumor growth leads to progressive loss of visual acuity and blindness due to optic nerve compression. Case report: Patient, female, 42 years without other morbility , begins in 1992 with decreased visual acuity of the left eye, magnetic resonance imaging (MRI) showed enlargement of the left optic nerve sheath, suggestive of ONSM. On that occasion, orbit exploration failed, so it was decided to follow up with annual clinical and imaging controls. About ten years later, begins with progressive deterioration of visual acuity and visual field , with ptosis and ocular motor palsy of the left eye, confirmed with neuro-ophthalmological examinations. MRI shows tumor progression. A new surgical approach was discarded by the risk of visual worsening. A conformal radiotherapy was performed with a fractionated 54 Gy dose. Today, at age 65, after 24 years of follow up,13 post radiation therapy. clinical and radiological stability of ONSM is confirmed. Discussion: Conformal radiotherapy has been shown as an effective therapy, with fewer complications and better outcomes in the preservation of visual function in the long term follow up Radio-fluoro guided surgery in high grade gliomas

  18. Amelogenesis Imperfect, Enamel Hypoplasia and Fluorosis Dental - Literature Review

    Directory of Open Access Journals (Sweden)

    Flávia Magnani Bevilacqua

    2015-12-01

    Full Text Available The developmental disorders of enamel are abnormalities of structure which can affect both dentitions. These abnormalities include amelogenesis imperfecta, enamel hypoplasia and dental fluorosis. The amelogenesis imperfecta is a hereditary change and enamel hypoplasia is a quantitative defect of enamel that occurs as a result of systemic problems, local and also inherited factors, or even the combination of them. Dental fluorosis is a hypoplasia caused by the chronic ingestion of fluoride during odontogenesis. All these anomalies have similar clinical characteristics, and it is necessary to be careful in their assessment. It is extremely important to know these abnormalities to establish a differential diagnosis and, consequently, a treatment plan, which can be set for each situation. Therefore, the purpose of this study was to review the literature regarding these three anomalies: amelogenesis imperfecta, enamel hypoplasia and dental fluorosis. It was concluded that to establish the differential diagnosis of these abnormalities as well as a proper treatment plan, it is indispensable the professional knowledge associated with the clinical examination. The examination has to consist of medical history and physical examination, and in some cases, x-ray examination.

  19. Optic neuritis

    Science.gov (United States)

    Retro-bulbar neuritis; Multiple sclerosis - optic neuritis; Optic nerve - optic neuritis ... The exact cause of optic neuritis is unknown. The optic nerve carries visual information from your eye to the brain. The nerve can swell when ...

  20. Biliary atresia and cerebellar hypoplasia in polysplenia syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Vanderdood, Kurt; Op de Beeck, Bart; Desprechins, Brigitte; Osteaux, Michel [Department of Radiology, Free University Brussels, AZ-VUB, Laarbeeklaan 101, 1090 Brussels (Belgium)

    2003-09-01

    We report a 3.5-month-old boy with polysplenia syndrome who demonstrated hemiazygos continuation of the inferior vena cava, extrahepatic biliary atresia, multiple splenunculi, bowel malrotation, and the rare finding of brainstem and cerebellar hypoplasia. A possible pathogenesis for cerebellar hypoplasia in this syndrome is suggested after review of the literature. The importance of seeking associated anomalies in biliary atresia, which may be possible indicators of polysplenia syndrome, is stressed since these patients need appropriate management when surgery is considered. (orig.)

  1. Long-term retinal nerve fiber layer changes following nonarteritic anterior ischemic optic neuropathy

    Directory of Open Access Journals (Sweden)

    Dotan G

    2013-04-01

    Full Text Available Gad Dotan,1 Michaella Goldstein,1 Anat Kesler,1 Barry Skarf21Department of Ophthalmology, Tel Aviv Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; 2Eye Care Services, Henry Ford Hospital, Detroit, MI, USABackground: In cases of nonarteritic anterior ischemic optic neuropathy (NAION, retinal nerve fiber layer (RNFL thickness changes have been described during the first 12 months following the acute event. The purpose of this study was to report on the long-term RNFL changes in these eyes beyond the first year following onset of NAION.Methods: Fourteen eyes of 13 patients with NAION were analyzed in this retrospective observational case series study. Uninvolved eyes served as controls. All patients underwent a complete neuro-ophthalmological examination and repeat measurements of peripapillary RNFL thickness using Stratus optical coherence tomography.Results: On optical coherence tomography scan performed on average 6 months following onset of NAION, the mean global RNFL thickness (59.8 ± 11.8 μm was significantly thinner (P < 0.001 compared with uninvolved eyes (95.1 ± 13.9 μm. In a second optical coherence tomography scan performed on average 13 (range 12–23 months later, the mean global RNFL thickness (58.9 ± 6.5 μm was not significantly different (P = 0.702 from the first scan.Conclusion: There appears to be no further RNFL loss beyond the first 6 months following an acute event of NAION.Keywords: optical coherence tomography, retinal nerve fiber layer, nonartertic anterior ischemic optic neuropathy

  2. Internal Carotid Artery Hypoplasia: Role of Color-Coded Carotid Duplex Sonography.

    Science.gov (United States)

    Chen, Pei-Ya; Liu, Hung-Yu; Lim, Kun-Eng; Lin, Shinn-Kuang

    2015-10-01

    The purpose of this study was to determine the role of color-coded carotid duplex sonography for diagnosis of internal carotid artery hypoplasia. We retrospectively reviewed 25,000 color-coded carotid duplex sonograms in our neurosonographic database to establish more diagnostic criteria for internal carotid artery hypoplasia. A definitive diagnosis of internal carotid artery hypoplasia was made in 9 patients. Diagnostic findings on color-coded carotid duplex imaging include a long segmental small-caliber lumen (52% diameter) with markedly decreased flow (13% flow volume) in the affected internal carotid artery relative to the contralateral side but without intraluminal lesions. Indirect findings included markedly increased total flow volume (an increase of 133%) in both vertebral arteries, antegrade ipsilateral ophthalmic arterial flow, and a reduced vessel diameter with increased flow resistance in the ipsilateral common carotid artery. Ten patients with distal internal carotid artery dissection showed a similar color-coded duplex pattern, but the reductions in the internal and common carotid artery diameters and increase in collateral flow from the vertebral artery were less prominent than those in hypoplasia. The ipsilateral ophthalmic arterial flow was retrograde in 40% of patients with distal internal carotid artery dissection. In addition, thin-section axial and sagittal computed tomograms of the skull base could show the small diameter of the carotid canal in internal carotid artery hypoplasia and help distinguish hypoplasia from distal internal carotid artery dissection. Color-coded carotid duplex sonography provides important clues for establishing a diagnosis of internal carotid artery hypoplasia. A hypoplastic carotid canal can be shown by thin-section axial and sagittal skull base computed tomography to confirm the final diagnosis. © 2015 by the American Institute of Ultrasound in Medicine.

  3. Premise and Prediction – How Optic Nerve Head Biomechanics Underlies the Susceptibility and Clinical Behavior of the Aged Optic Nerve Head

    Science.gov (United States)

    Burgoyne, Claude F.; Downs, J. Crawford

    2009-01-01

    We propose that age-related alterations in optic nerve head (ONH) biomechanics underlie the clinical behavior and increased susceptibility of the aged ONH to glaucomatous damage. The literature which suggests that the aged ONH is more susceptible to glaucomatous damage at all levels of intraocular pressure is reviewed. The relevant biomechanics of the aged ONH are discussed and a biomechanical explanation for why, on average, the stiffened peripapillary scleral and lamina cribrosa connective tissues of the aged eye should lead to a shallow (senile sclerotic) form of cupping is proposed. A logic for why age-related axon loss and the optic neuropathy of glaucoma in the aged eye may overlap is discussed. Finally, we argue for a need to characterize all forms of clinical cupping into prelaminar and laminar components so as to add precision to the discussion of clinical cupping which does not currently exist. Such characterization may lead to the early detection of ONH axonal and connective tissue pathology in ocular hypertension and eventually aid in the assessment of etiology in all forms of optic neuropathy including those that may be purely age-related. PMID:18552618

  4. Scanning laser topography and scanning laser polarimetry: comparing both imaging methods at same distances from the optic nerve head.

    Science.gov (United States)

    Kremmer, Stephan; Keienburg, Marcus; Anastassiou, Gerasimos; Schallenberg, Maurice; Steuhl, Klaus-Peter; Selbach, J Michael

    2012-01-01

    To compare the performance of scanning laser topography (SLT) and scanning laser polarimetry (SLP) on the rim of the optic nerve head and its surrounding area and thereby to evaluate whether these imaging technologies are influenced by other factors beyond the thickness of the retinal nerve fiber layer (RNFL). A total of 154 eyes from 5 different groups were examined: young healthy subjects (YNorm), old healthy subjects (ONorm), patients with normal tension glaucoma (NTG), patients with open-angle glaucoma and early glaucomatous damage (OAGE) and patients with open-angle glaucoma and advanced glaucomatous damage (OAGA). SLT and SLP measurements were taken. Four concentric circles were superimposed on each of the images: the first one measuring at the rim of the optic nerve head (1.0 ONHD), the next measuring at 1.25 optic nerve head diameters (ONHD), at 1.5 ONHD and at 1.75 ONHD. The aligned images were analyzed using GDx/NFA software. Both methods showed peaks of RNFL thickness in the superior and inferior segments of the ONH. The maximum thickness, registered by the SLT device was at the ONH rim where the SLP device tended to measure the lowest values. SLT measurements at the ONH were influenced by other tissues besides the RNFL like blood vessels and glial tissues. SLT and SLP were most strongly correlated at distances of 1.25 and 1.5 ONHD. While both imaging technologies are valuable tools in detecting glaucoma, measurements at the ONH rim should be interpreted critically since both methods might provide misleading results. For the assessment of the retinal nerve fiber layer we would like to recommend for both imaging technologies, SLT and SLP, measurements in 1.25 and 1.5 ONHD distance of the rim of the optic nerve head.

  5. Comparison of the Deep Optic Nerve Head Structure between Normal-Tension Glaucoma and Nonarteritic Anterior Ischemic Optic Neuropathy.

    Directory of Open Access Journals (Sweden)

    Eun Ji Lee

    Full Text Available To compare the deep optic nerve head (ONH structure between normal-tension glaucoma (NTG and nonarteritic anterior ischemic optic neuropathy (NAION and also in healthy subjects as a control using enhanced depth imaging (EDI spectral-domain optical coherence tomography (SD-OCT.This prospective cross-sectional study included 21 NAION patients who had been diagnosed as NAION at least 6 months prior to study entry, and 42 NTG patients and 42 healthy controls who were matched with NAION patients in terms of age, intraocular pressure (IOP, and optic disc area. The retinal nerve fiber layer (RNFL thickness in the affected sector was also matched between NAION and NTG patients. The ONH was imaged using SD-OCT with the EDI technique. The anterior lamina cribrosa surface depth (LCD and average prelaminar tissue (PT thickness were measured in a sector of interest in each eye and compared among the three groups.In the sector-matched comparison, LCD was largest in NTG patients, followed by NAION patients, while PT was thinner in NTG patients than in NAION patients (all P < 0.001. NAION patients had a comparable LCD and a thinner PT relative to normal controls (P = 0.170 and < 0.001, respectively.The deep ONH configuration is strikingly different between NTG and NAION. The differing features provide comparative insight into the pathophysiology of the two diseases, and may be useful for differential diagnosis.

  6. Comparative study of the retinal nerve fibre layer thickness performed with optical coherence tomography and GDx scanning laser polarimetry in patients with primary open-angle glaucoma.

    Science.gov (United States)

    Wasyluk, Jaromir T; Jankowska-Lech, Irmina; Terelak-Borys, Barbara; Grabska-Liberek, Iwona

    2012-03-01

    We compared the parameters of retinal nerve fibre layer in patients with advanced glaucoma with the use of different OCT (Optical Coherence Tomograph) devices in relation to analogical measurements performed with GDx VCC (Nerve Fiber Analyzer with Variable Corneal Compensation) scanning laser polarimetry. Study subjects had advanced primary open-angle glaucoma, previously treated conservatively, diagnosed and confirmed by additional examinations (visual field, ophthalmoscopy of optic nerve, gonioscopy), A total of 10 patients were enrolled (9 women and 1 man), aged 18-70 years of age. Nineteen eyes with advanced glaucomatous neuropathy were examined. 1) Performing a threshold perimetry Octopus, G2 strategy and ophthalmoscopy of optic nerve to confirm the presence of advanced primary open-angle glaucoma; 2) performing a GDx VCC scanning laser polarimetry of retinal nerve fibre layer; 3) measuring the retinal nerve fibre layer thickness with 3 different optical coherence tomographs. The parameters of the retinal nerve fibre layer thickness are highly correlated between the GDx and OCT Stratus and 3D OCT-1000 devices in mean retinal nerve fibre layer thickness, retinal nerve fibre layer thickness in the upper sector, and correlation of NFI (GDx) with mean retinal nerve fibre layer thickness in OCT examinations. Absolute values of the retinal nerve fibre layer thickness (measured in µm) differ significantly between GDx and all OCT devices. Examination with OCT devices is a sensitive diagnostic method of glaucoma, with good correlation with the results of GDx scanning laser polarimetry of the patients.

  7. Effect of the menstrual cycle on the optic nerve head in diabetes: analysis by confocal scanning laser ophthalmoscopy.

    Science.gov (United States)

    Akar, Munire Erman; Yucel, Iclal; Erdem, Uzeyir; Taskin, Omur; Ozel, Alper; Akar, Yusuf

    2005-04-01

    The purpose of this study was to examine and compare menstrual-cycle-dependent topographic changes in the optic nerve head of normally menstruating women with different grades of type 2 diabetes mellitus. We studied the right eyes of 123 normally menstruating women (36 with severe nonproliferative diabetic retinopathy [NPDR], 42 with mild NPDR and 45 healthy subjects). All subjects underwent a complete ocular examination at baseline. At 4 hormonally distinct phases of the menstrual cycle (early follicular, late follicular, mid-luteal and late luteal), we analysed the topography of the optic nerve head, using a confocal scanning laser ophthalmoscope, and measured the serum levels of estradiol, progesterone and luteinizing hormone. We excluded from analysis the data for 8 patients with severe NPDR, 10 patients with mild NPDR and 15 control subjects who were lost to follow-up examinations during the menstrual cycle. The mean age and optic disc area did not differ significantly among the 3 groups. The duration of diabetes was significantly longer in the patients with severe NPDR than in those with mild NPDR (p cup-shape measure, linear cup/disc ratio, cup/disc area ratio and cup area in the late luteal phase compared with the other phases of the menstrual cycle (p menstrual cycle. Severe NPDR is associated with significant topographic changes in the rim and cup of the optic nerve head during the menstrual cycle. This must be considered in the evaluation of women with both diabetes and glaucoma. The normal fluctuations in serum sex hormone levels during the menstrual cycle of diabetic women seem to affect the optic nerve head more when the disease is advanced.

  8. Isolated unilateral pulmonary artery hypoplasia with accompanying pulmonary parenchymal findings on CT: A case report

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    Park, Surin; Cha, Yoon Ki; Kim, Jeung Sook; Kwon, Jae Hyun; Jeong, Yun Jeong [Dongguk University Ilsan Hospital, Dongguk University College of Medicine, Goyang (Korea, Republic of); Kim, Seon Jeong [Dept. of Radiology, Myongji Hospital, Goyang (Korea, Republic of)

    2017-05-15

    Unilateral pulmonary artery hypoplasia or agenesis without congenital cardiovascular anomalies is rare in adults. We report a case of a 36-year-old man with isolated left unilateral pulmonary artery hypoplasia with recurrent hemoptysis. On computed tomography (CT), the left pulmonary artery showed hypoplasia with multiple collateral vessels seen in the mediastinum and the left hemithorax. Also, parenchymal bands and peripheral linear opacities were seen in the affected lung, which were probably due to chronic infarction induced by unilateral pulmonary artery hypoplasia. There are only a few reports focusing on the radiologic findings in the pulmonary parenchyma induced by unilateral pulmonary artery hypoplasia, such as parenchymal bands and peripheral linear opacities. Therefore we report this case, which focused on the CT findings in the pulmonary parenchyma due to isolated unilateral pulmonary artery hypoplasia.

  9. Isolated unilateral pulmonary artery hypoplasia with accompanying pulmonary parenchymal findings on CT: A case report

    International Nuclear Information System (INIS)

    Park, Surin; Cha, Yoon Ki; Kim, Jeung Sook; Kwon, Jae Hyun; Jeong, Yun Jeong; Kim, Seon Jeong

    2017-01-01

    Unilateral pulmonary artery hypoplasia or agenesis without congenital cardiovascular anomalies is rare in adults. We report a case of a 36-year-old man with isolated left unilateral pulmonary artery hypoplasia with recurrent hemoptysis. On computed tomography (CT), the left pulmonary artery showed hypoplasia with multiple collateral vessels seen in the mediastinum and the left hemithorax. Also, parenchymal bands and peripheral linear opacities were seen in the affected lung, which were probably due to chronic infarction induced by unilateral pulmonary artery hypoplasia. There are only a few reports focusing on the radiologic findings in the pulmonary parenchyma induced by unilateral pulmonary artery hypoplasia, such as parenchymal bands and peripheral linear opacities. Therefore we report this case, which focused on the CT findings in the pulmonary parenchyma due to isolated unilateral pulmonary artery hypoplasia

  10. Primary radiotherapy in progressive optic nerve sheath meningiomas: a long-term follow-up study

    NARCIS (Netherlands)

    Saeed, P.; Blank, L.; Selva, D.; Wolbers, J.G.; Nowak, P.J.C.M.; Geskus, R.B.; Weis, E.; Mourits, M.P.; Rootman, J.

    2010-01-01

    Background/aims To report the outcome of primary radiotherapy in patients with progressive optic nerve sheath meningioma (ONSM). Methods The clinical records of all patients were reviewed in a retrospective, observational, multicentre study. Results Thirty-four consecutive patients were included.

  11. Evolution of an Astrocytic Hamartoma of the Optic Nerve Head in a Patient with Retinitis Pigmentosa - Photographic Documentation over 2 Years of Follow-Up.

    Science.gov (United States)

    Loukianou, Eleni; Kisma, Nacima; Pal, Bishwanathan

    2011-02-02

    To report photographically the evolution of an astrocytic hamartoma of the left optic nerve head over a 2-year follow-up in a patient with retinitis pigmentosa. A 14-year-old boy was seen in the medical retina clinic with a 3-year history of night blindness. Best corrected visual acuity was 6/18 in both eyes. Colour vision was normal in both eyes and confrontation fields showed peripheral constriction. Fundus examination revealed bone spicule pigmentary changes at the retinal mid periphery typical of retinitis pigmentosa and superficial globules at the margins of both optic nerve heads. Electrodiagnostic tests confirmed moderately severe rod cone dystrophy with macular involvement bilaterally. Two years later, the ocular examination was unchanged except for the appearance of the optic nerve head lesion in the left eye. There was an increase in the size of the lesion which was diagnosed as an astrocytic hamartoma. Further investigations were recommended to exclude neurofibromatosis and tuberous sclerosis. Astrocytic hamartomas of the optic nerve head and optic nerve head drusen have both been described in patients with retinitis pigmentosa. They can be a diagnostic dilemma although drusen are more common (10%). To differentiate these two entities it is very important to document any growth during the follow-up period which is suggestive of astrocytic hamartoma rather than optic disc drusen.

  12. Circulatory CNP Rescues Craniofacial Hypoplasia in Achondroplasia.

    Science.gov (United States)

    Yamanaka, S; Nakao, Kazumasa; Koyama, N; Isobe, Y; Ueda, Y; Kanai, Y; Kondo, E; Fujii, T; Miura, M; Yasoda, A; Nakao, Kazuwa; Bessho, K

    2017-12-01

    Achondroplasia is the most common genetic form of human dwarfism, characterized by midfacial hypoplasia resulting in occlusal abnormality and foramen magnum stenosis, leading to serious neurologic complications and hydrocephalus. Currently, surgery is the only way to manage jaw deformity, neurologic complications, and hydrocephalus in patients with achondroplasia. We previously showed that C-type natriuretic peptide (CNP) is a potent stimulator of endochondral bone growth of long bones and vertebrae and is also a potent stimulator in the craniofacial region, which is crucial for midfacial skeletogenesis. In this study, we analyzed craniofacial morphology in a mouse model of achondroplasia, in which fibroblast growth factor receptor 3 (FGFR3) is specifically activated in cartilage ( Fgfr3 ach mice), and investigated the mechanisms of jaw deformities caused by this mutation. Furthermore, we analyzed the effect of CNP on the maxillofacial area in these animals. Fgfr3 ach mice exhibited midfacial hypoplasia, especially in the sagittal direction, caused by impaired endochondral ossification in craniofacial cartilage and by premature closure of the spheno-occipital synchondrosis, an important growth center in craniomaxillofacial skeletogenesis. We crossed Fgfr3 ach mice with transgenic mice in which CNP is expressed in the liver under the control of the human serum amyloid-P component promoter, resulting in elevated levels of circulatory CNP ( Fgfr3 ach /SAP-Nppc-Tg mice). In the progeny, midfacial hypoplasia in the sagittal direction observed in Fgfr3 ach mice was improved significantly by restoring the thickness of synchondrosis and promoting proliferation of chondrocytes in the craniofacial cartilage. In addition, the foramen magnum stenosis observed in Fgfr3 ach mice was significantly ameliorated in Fgfr3 ach /SAP-Nppc-Tg mice due to enhanced endochondral bone growth of the anterior intraoccipital synchondrosis. These results clearly demonstrate the therapeutic

  13. Method for imaging pulmonary arterial hypoplasia

    International Nuclear Information System (INIS)

    Triantafillou, M.

    2000-01-01

    Full text: Pulmonary hypoplasia represents an incomplete development of the lung, resulting in the reduction of distended lung volume. This is associated with small or absent number of airway divisions, alveoli, arteries and veins. Unilateral pulmonary Hypoplasia is often asymptomatic and may be demonstrated as a hypodense lung on a chest X-ray. Computer Tomography (CT) scanning would show anatomical detail and proximal vessels. Magnetic Resonance Imaging (MRI) will show no more detail than which the CT scan has already demonstrated. It is, also, difficult to visualise collateral vessels from systemic and/or bronchial vessels on both these modalities. Pulmonary Angiography would give the definitive answer, but it is time consuming and has significant risks associated with the procedure. There are high costs associated with these modalities. Nuclear Medicine Ventilation/Perfusion (V/Q) scan performed on these patients would demonstrate diminished ventilation due to reduced lung volume and absence of perfusion to the hypoplastic lung. To date, we have performed V/Q lung scan on two children in our department. Both cases demonstrate diminished ventilation with no perfusion to the hypoplastic lung. Though the gold standard is Pulmonary Angiography, V/Q scanning is cost effective, less time consuming and a non invasive procedure that can be performed as an outpatient. It is accurate as it demonstrates absent lung perfusion, confirming the patient has pulmonary arterial hypoplasia. Copyright (2000) The Australian and New Zealand Society of Nuclear Medicine Inc

  14. Analysis of macular and nerve fiber layer thickness in multiple sclerosis patients according to severity level and optic neuritis episodes.

    Science.gov (United States)

    Soler García, A; Padilla Parrado, F; Figueroa-Ortiz, L C; González Gómez, A; García-Ben, A; García-Ben, E; García-Campos, J M

    2016-01-01

    Quantitative assessment of macular and nerve fibre layer thickness in multiple sclerosis patients with regard to expanded disability status scale (EDSS) and presence or absence of previous optic neuritis episodes. We recruited 62 patients with multiple sclerosis (53 relapsing-remitting and 9 secondary progressive) and 12 disease-free controls. All patients underwent an ophthalmological examination, including quantitative analysis of the nerve fibre layer and macular thickness using optical coherence tomography. Patients were classified according to EDSS as A (lower than 1.5), B (between 1.5 and 3.5), and C (above 3.5). Mean nerve fibre layer thickness in control, A, B, and C groups was 103.35±12.62, 99.04±14.35, 93.59±15.41, and 87.36±18.75μm respectively, with statistically significant differences (P<.05). In patients with no history of optic neuritis, history of episodes in the last 3 to 6 months, or history longer than 6 months, mean nerve fibre layer thickness was 99.25±13.71, 93.92±13.30 and 80.07±15.91μm respectively; differences were significant (P<.05). Mean macular thickness in control, A, B, and C groups was 220.01±12.07, 217.78±20.02, 217.68±20.77, and 219.04±24.26μm respectively. Differences were not statistically significant. The mean retinal nerve fibre layer thickness in multiple sclerosis patients is related to the EDSS level. Patients with previous optic neuritis episodes have a thinner retinal nerve fibre layer than patients with no history of these episodes. Mean macular thickness is not correlated to EDSS level. Copyright © 2014 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  15. Intracranial pressure-induced optic nerve sheath response as a predictive biomarker for optic disc edema in astronauts.

    Science.gov (United States)

    Wostyn, Peter; De Deyn, Peter Paul

    2017-11-01

    A significant proportion of the astronauts who spend extended periods in microgravity develop ophthalmic abnormalities. Understanding this syndrome, called visual impairment and intracranial pressure (VIIP), has become a high priority for National Aeronautics and Space Administration, especially in view of future long-duration missions (e.g., Mars missions). Moreover, to ensure selection of astronaut candidates who will be able to complete long-duration missions with low risk of the VIIP syndrome, it is imperative to identify biomarkers for VIIP risk prediction. Here, we hypothesize that the optic nerve sheath response to alterations in intracranial pressure may be a potential predictive biomarker for optic disc edema in astronauts. If confirmed, this biomarker could be used for preflight identification of astronauts at risk for developing VIIP-associated optic disc edema.

  16. ULTRASOUND AND COMPUTED TOMOGRAPHIC DIAGNOSIS OF OPTIC NERVE TUMORS

    Directory of Open Access Journals (Sweden)

    S. V. Saakyan

    2012-01-01

    Full Text Available A comprehensive examination was made in 93 patients, including 18 children, with tumors of the optic nerve (ON. Duplex ultrasound scanning was performed in 39 patients, of them there were 11 patients with ON gliomas and 28 with ON meningiomas. The specific computed tomographic and echographic signs of ON glioma and meningiomas were detected. The studies have shown that duplex ultrasound scanning and structural computed tomography of orbital sockets are highly informative complementary imaging procedures for ON tumors, which permits one to make their correct diagnosis, to specify surgical volume, and to plan adequate treatment.

  17. Prospective Retinal and Optic Nerve Vitrectomy Evaluation (PROVE study: findings at 3 months

    Directory of Open Access Journals (Sweden)

    Reddy RK

    2013-09-01

    Full Text Available Rahul K Reddy,1 Maziar Lalezary,1 Stephen J Kim,1 Jeffrey A Kammer,1 Rachel W Kuchtey,1 Edward F Cherney,1 Franco M Recchia,2 Karen M Joos,1 Anita Agarwal,1 Janice C Law11Department of Ophthalmology, Vanderbilt University School of Medicine, Nashville, TN, USA; 2Tennessee Retina, PC, Nashville, TN, USABackground: The purpose of this paper is to report the 3-month findings of the Prospective Retinal and Optic Nerve Vitrectomy Evaluation (PROVE study.Methods: Eighty eyes of 40 participants undergoing vitrectomy were enrolled. Participants underwent baseline evaluation of the study (surgical and fellow (control eye that included: intraocular pressure, central corneal thickness, gonioscopy, cup-to-disc ratio measurement, color fundus and optic disc photography, automated perimetry, and optical coherence tomography of the macula and optic nerve. Evaluation was repeated at 3 months. Main outcome measures were changes in macula and retinal nerve fiber layer (RNFL thickness and intraocular pressure.Results: All participants completed follow-up. Mean cup-to-disc ratio of study and fellow eyes at baseline was 0.43 ± 0.2 and 0.46 ± 0.2, respectively, and 13% of participants had undiagnosed narrow angles. There was no significant change in intraocular pressure, cup-to-disc ratio, or pattern standard deviation in study eyes compared with baseline or fellow eyes at 3 months. Vision improved in all study eyes at 3 months compared with baseline (P = 0.013, but remained significantly worse than fellow eyes (P < 0.001. Central subfield and temporal peripapillary RNFL thickness were significantly greater in eyes with epiretinal membrane (P < 0.05, and resolution after surgery correlated with visual improvement (P < 0.05.Conclusion: The 3-month results do not indicate any increased risk for open-angle glaucoma but suggest that a relatively high percentage of eyes may be at risk of angle closure glaucoma. Temporal RNFL thickness and central subfield were increased

  18. A joint estimation detection of Glaucoma progression in 3D spectral domain optical coherence tomography optic nerve head images

    Science.gov (United States)

    Belghith, Akram; Bowd, Christopher; Weinreb, Robert N.; Zangwill, Linda M.

    2014-03-01

    Glaucoma is an ocular disease characterized by distinctive changes in the optic nerve head (ONH) and visual field. Glaucoma can strike without symptoms and causes blindness if it remains without treatment. Therefore, early disease detection is important so that treatment can be initiated and blindness prevented. In this context, important advances in technology for non-invasive imaging of the eye have been made providing quantitative tools to measure structural changes in ONH topography, an essential element for glaucoma detection and monitoring. 3D spectral domain optical coherence tomography (SD-OCT), an optical imaging technique, has been commonly used to discriminate glaucomatous from healthy subjects. In this paper, we present a new framework for detection of glaucoma progression using 3D SD-OCT images. In contrast to previous works that the retinal nerve fiber layer (RNFL) thickness measurement provided by commercially available spectral-domain optical coherence tomograph, we consider the whole 3D volume for change detection. To integrate a priori knowledge and in particular the spatial voxel dependency in the change detection map, we propose the use of the Markov Random Field to handle a such dependency. To accommodate the presence of false positive detection, the estimated change detection map is then used to classify a 3D SDOCT image into the "non-progressing" and "progressing" glaucoma classes, based on a fuzzy logic classifier. We compared the diagnostic performance of the proposed framework to existing methods of progression detection.

  19. Regional disc change in segmental hypoplasia of the lumbosacral vertebral bodies: MR findings

    International Nuclear Information System (INIS)

    Kim, Sung Kyu; Lee Seung Ro; Moon, Won Jin; Park, Dong Woo; Hahm, Chang Kok

    2000-01-01

    To classify types of vertebral hypoplasia and to investigate the prevalence and patterns of associated disc degeneration. Defining vertebral hypoplasia as occurring when the AP diameter of a lower vertebral body is smaller than that of an upper ones, we retrospectively reviewed the MR images obtained in 34 cases of this condition involving young adults. Two major types and two subtypes, a total of four different entities were classified as follows; type I: hypoplasia involving a single vertebral body; type II: hypoplasia involving serial lower segmental vertebral bodies; subtype a: hypoplastic body located anteriorly along the anterior spinal line; subtype b: hypoplastic body located posteriorly along the posterior spinal line. We also investigated each type of vertebral hypoplasia and patterns of associated disc changes. Three different types were observed. In type IIa (n=3D29), posterior disc occurred in 8/29 cases, diffuse degeneration in 21/29 patients, and posterior disc herniation in all. All type Ia cases (3/3) showed diffuse disc degeneration at both upper and lower disc levels, with posterior disc herniation, while both type IIb cases (2/2) showed diffuse disc degeneration, with bidirectional disc herniation. By identifying the exact patterns of vertebral hypoplasia, we were able to predict which portion of the disc was likely to degenerate. (author)

  20. Novel Combinatory Approaches to Repair Visual System after Optic Nerve Damage

    Science.gov (United States)

    2014-09-01

    Novel Combinatory Approaches to Repair Visual System After Optic Nerve Damage 5a. CONTRACT NUMBER 5b. GRANT NUMBER 5c. PROGRAM ELEMENT NUMBER 6...biological functions such as metabolism, growth, cell cycle, and survival ( Cardone et al., 1998; Diehl, Cheng, Roussel, & Sherr, 1998; Hers, Vincent...of injured adult sensory neurons. The Journal of Neuroscience, 21, 7161–7170. Cardone , M. H., Roy, N., Stennicke, H. R., Salvesen, G. S., Franke, T. F

  1. The Connective Tissue Components of Optic Nerve Head Cupping in Monkey Experimental Glaucoma Part 1: Global Change

    Science.gov (United States)

    Yang, Hongli; Ren, Ruojin; Lockwood, Howard; Williams, Galen; Libertiaux, Vincent; Downs, Crawford; Gardiner, Stuart K.; Burgoyne, Claude F.

    2015-01-01

    Purpose To characterize optic nerve head (ONH) connective tissue change within 21 monkey experimental glaucoma (EG) eyes, so as to identify its principal components. Methods Animals were imaged three to five times at baseline then every 2 weeks following chronic unilateral IOP elevation, and euthanized early through end-stage confocal scanning laser tomographic change. Optic nerve heads were serial-sectioned, three-dimensionally (3D) reconstructed, delineated, and quantified. Overall EG versus control eye differences were assessed by general estimating equations (GEE). Significant, animal-specific, EG eye change was required to exceed the maximum physiologic intereye differences in six healthy animals. Results Overall EG eye change was significant (P connective tissue components of ONH “cupping” in monkey EG which serve as targets for longitudinally staging and phenotyping ONH connective tissue alteration within all forms of monkey and human optic neuropathy. PMID:26641545

  2. Measurement of amino acid levels in the vitreous humor of rats after chronic intraocular pressure elevation or optic nerve transection.

    Science.gov (United States)

    Levkovitch-Verbin, Hana; Martin, Keith R G; Quigley, Harry A; Baumrind, Lisa A; Pease, Mary Ellen; Valenta, Danielle

    2002-10-01

    To investigate whether the levels of free amino acids and protein in the vitreous of rat eyes are altered with chronic intraocular pressure (IOP) elevation or after optic nerve transection. The concentrations of 20 amino acids in the vitreous humor were measured by high-performance liquid chromatography in both eyes of 41 rats with unilateral IOP elevation induced by translimbal photocoagulation. Eyes were studied 1 day and 1, 2, 4, and 9 weeks after initial IOP elevation. The same amino acids were measured in 41 rats 1 day and 2, 4, and 9 weeks after unilateral transection of the orbital optic nerve. The intravitreal protein level was assayed in additional 22 rats with IOP elevation and 12 rats after nerve transection. Two masked observers evaluated the amount of optic nerve damage with a semiquantitative, light-microscopic technique. In rats with experimental glaucoma, amino acid concentrations were unchanged 1 day after treatment. At 1 week, 4 of 20 amino acids (aspartate, proline, alanine, and lysine) were higher than in control eyes ( 0.05). Vitreous protein level was significantly higher in glaucomatous eyes than their paired controls at 1 day ( 0.01).

  3. ImagePAD, a novel counting application for the Apple iPad, used to quantify axons in the mouse optic nerve.

    Science.gov (United States)

    Templeton, Justin P; Struebing, Felix L; Lemmon, Andrew; Geisert, Eldon E

    2014-11-01

    The present article introduces a new and easy to use counting application for the Apple iPad. The application "ImagePAD" takes advantage of the advanced user interface features offered by the Apple iOS platform, simplifying the rather tedious task of quantifying features in anatomical studies. For example, the image under analysis can be easily panned and zoomed using iOS-supported multi-touch gestures without losing the spatial context of the counting task, which is extremely important for ensuring count accuracy. This application allows one to quantify up to 5 different types of objects in a single field and output the data in a tab-delimited format for subsequent analysis. We describe two examples of the use of the application: quantifying axons in the optic nerve of the C57BL/6J mouse and determining the percentage of cells labeled with NeuN or ChAT in the retinal ganglion cell layer. For the optic nerve, contiguous images at 60× magnification were taken and transferred onto an Apple iPad. Axons were counted by tapping on the touch-sensitive screen using ImagePAD. Nine optic nerves were sampled and the number of axons in the nerves ranged from 38,872 axons to 50,196 axons with an average of 44,846 axons per nerve (SD = 3980 axons). Copyright © 2014 Elsevier Ltd. All rights reserved.

  4. Stem Cell Ophthalmology Treatment Study (SCOTS) for retinal and optic nerve diseases: a case report of improvement in relapsing auto-immune optic neuropathy.

    Science.gov (United States)

    Weiss, Jeffrey N; Levy, Steven; Benes, Susan C

    2015-09-01

    We present the results from a patient with relapsing optic neuropathy treated within the Stem Cell Ophthalmology Treatment Study (SCOTS). SCOTS is an Institutional Review Board approved clinical trial and has become the largest ophthalmology stem cell study registered at the National Institutes of Health to date (www.clinicaltrials.gov Identifier NCT 01920867). SCOTS utilizes autologous bone marrow-derived stem cells (BMSCs) for treatment of retinal and optic nerve diseases. Pre-treatment and post-treatment comprehensive eye exams of a 54 year old female patient were performed both at the Florida Study Center, USA and at The Eye Center of Columbus, USA. As a consequence of a relapsing optic neuritis, the patient's previously normal visual acuity decreased to between 20/350 and 20/400 in the right eye and to 20/70 in the left eye. Significant visual field loss developed bilaterally. The patient underwent a right eye vitrectomy with injection of BMSCs into the optic nerve of the right eyeand retrobulbar, subtenon and intravitreal injection of BMSCs in the left eye. At 15 months after SCOTS treatment, the patient's visual acuity had improved to 20/150 in the right eye and 20/20 in the left eye. Bilateral visual fields improved markedly. Both macular thickness and fast retinal nerve fiber layer thickness were maximally improved at 3 and 6 months after SCOTS treatment. The patient also reduced her mycophenylate dose from 1,500 mg per day to 500 mg per day and required no steroid pulse therapy during the 15-month follow up.

  5. Evolution of an Astrocytic Hamartoma of the Optic Nerve Head in a Patient with Retinitis Pigmentosa – Photographic Documentation over 2 Years of Follow-Up

    Science.gov (United States)

    Loukianou, Eleni; Kisma, Nacima; Pal, Bishwanathan

    2011-01-01

    Aim To report photographically the evolution of an astrocytic hamartoma of the left optic nerve head over a 2-year follow-up in a patient with retinitis pigmentosa. Methods A 14-year-old boy was seen in the medical retina clinic with a 3-year history of night blindness. Best corrected visual acuity was 6/18 in both eyes. Colour vision was normal in both eyes and confrontation fields showed peripheral constriction. Fundus examination revealed bone spicule pigmentary changes at the retinal mid periphery typical of retinitis pigmentosa and superficial globules at the margins of both optic nerve heads. Electrodiagnostic tests confirmed moderately severe rod cone dystrophy with macular involvement bilaterally. Results Two years later, the ocular examination was unchanged except for the appearance of the optic nerve head lesion in the left eye. There was an increase in the size of the lesion which was diagnosed as an astrocytic hamartoma. Further investigations were recommended to exclude neurofibromatosis and tuberous sclerosis. Conclusion Astrocytic hamartomas of the optic nerve head and optic nerve head drusen have both been described in patients with retinitis pigmentosa. They can be a diagnostic dilemma although drusen are more common (10%). To differentiate these two entities it is very important to document any growth during the follow-up period which is suggestive of astrocytic hamartoma rather than optic disc drusen. PMID:21347192

  6. Evolution of an Astrocytic Hamartoma of the Optic Nerve Head in a Patient with Retinitis Pigmentosa – Photographic Documentation over 2 Years of Follow-Up

    Directory of Open Access Journals (Sweden)

    Eleni Loukianou

    2011-02-01

    Full Text Available Aim: To report photographically the evolution of an astrocytic hamartoma of the left optic nerve head over a 2-year follow-up in a patient with retinitis pigmentosa. Methods: A 14-year-old boy was seen in the medical retina clinic with a 3-year history of night blindness. Best corrected visual acuity was 6/18 in both eyes. Colour vision was normal in both eyes and confrontation fields showed peripheral constriction. Fundus examination revealed bone spicule pigmentary changes at the retinal mid periphery typical of retinitis pigmentosa and superficial globules at the margins of both optic nerve heads. Electrodiagnostic tests confirmed moderately severe rod cone dystrophy with macular involvement bilaterally. Results: Two years later, the ocular examination was unchanged except for the appearance of the optic nerve head lesion in the left eye. There was an increase in the size of the lesion which was diagnosed as an astrocytic hamartoma. Further investigations were recommended to exclude neurofibromatosis and tuberous sclerosis. Conclusion: Astrocytic hamartomas of the optic nerve head and optic nerve head drusen have both been described in patients with retinitis pigmentosa. They can be a diagnostic dilemma although drusen are more common (10%. To differentiate these two entities it is very important to document any growth during the follow-up period which is suggestive of astrocytic hamartoma rather than optic disc drusen.

  7. Engrafted human induced pluripotent stem cell-derived anterior specified neural progenitors protect the rat crushed optic nerve.

    Directory of Open Access Journals (Sweden)

    Leila Satarian

    Full Text Available BACKGROUND: Degeneration of retinal ganglion cells (RGCs is a common occurrence in several eye diseases. This study examined the functional improvement and protection of host RGCs in addition to the survival, integration and neuronal differentiation capabilities of anterior specified neural progenitors (NPs following intravitreal transplantation. METHODOLOGY/PRINCIPAL FINDINGS: NPs were produced under defined conditions from human induced pluripotent stem cells (hiPSCs and transplanted into rats whose optic nerves have been crushed (ONC. hiPSCs were induced to differentiate into anterior specified NPs by the use of Noggin and retinoic acid. The hiPSC-NPs were labeled by green fluorescent protein or a fluorescent tracer 1,1' -dioctadecyl-3,3,3',3'-tetramethylindocarbocyanine perchlorate (DiI and injected two days after induction of ONC in hooded rats. Functional analysis according to visual evoked potential recordings showed significant amplitude recovery in animals transplanted with hiPSC-NPs. Retrograde labeling by an intra-collicular DiI injection showed significantly higher numbers of RGCs and spared axons in ONC rats treated with hiPSC-NPs or their conditioned medium (CM. The analysis of CM of hiPSC-NPs showed the secretion of ciliary neurotrophic factor, basic fibroblast growth factor, and insulin-like growth factor. Optic nerve of cell transplanted groups also had increased GAP43 immunoreactivity and myelin staining by FluoroMyelin™ which imply for protection of axons and myelin. At 60 days post-transplantation hiPSC-NPs were integrated into the ganglion cell layer of the retina and expressed neuronal markers. CONCLUSIONS/SIGNIFICANCE: The transplantation of anterior specified NPs may improve optic nerve injury through neuroprotection and differentiation into neuronal lineages. These NPs possibly provide a promising new therapeutic approach for traumatic optic nerve injuries and loss of RGCs caused by other diseases.

  8. Simultaneous central retinal artery occlusion and optic nerve vasculitis in Crohn disease

    Directory of Open Access Journals (Sweden)

    Razek Georges Coussa

    2017-04-01

    Conclusions and importance: To our knowledge, this is the first case of unilateral CRAO and bilateral optic nerve occlusive vasculitis in Crohn disease, which should be considered as an etiology of retinal vascular occlusive disorders especially in young patients. It is important for ophthalmologists to be aware of the ophthalmic risks associated with Crohn disease as aggressive treatment with systemic steroids and immunosuppressive agents is often needed.

  9. Optic disc and peripapillary retinal nerve fiber layer characteristics associated with glaucomatous optic disc in young myopia.

    Science.gov (United States)

    Lee, Jong Eun; Sung, Kyung Rim; Park, Ji Min; Yoon, Joo Young; Kang, Sung Yong; Park, Sung Bae; Koo, Hyung Jin

    2017-03-01

    To explore optic disc and peripapillary retinal nerve fiber layer (RNFL) features associated with glaucomatous optic disc (GOD) in young myopia. Presence of GOD, optic disc tilt, and disc torsion were determined using fundus photographs. If the measured disc tilt ratio was >1.3, the optic disc was classified as tilted. Optic disc torsion was defined as a >15° deviation in the long axis of the optic disc from the vertical meridian. The average and four quadrants RNFL thicknesses were assessed using spectral domain optical coherence tomography (SD-OCT). Logistic regression analyses were performed to identify factors associated with the presence of GOD. Nine hundred and sixty myopic subjects were recruited from four refractive surgery clinic databases. The mean age was 26.6 ± 5.7 years and spherical equivalent (SE) was -5.5 ± 2.5 diopters. Among 960 eyes, 26 (2.7%) received GOD group classification. Among 934 normal eyes, 290 (31.0%) had titled optic discs. Eighteen eyes (69.2%) in the GOD group had tilted optic discs. When compared to normal eyes, the GOD group had significantly higher tilt ratios (1.4 ± 0.2 vs. 1.2 ± 0.1, p Optic disc tilt was found in approximately one-third of young myopic eyes and was independently associated with the presence of GOD.

  10. Goldenhar Syndrome with Dextrocardia and Right Pulmonary Hypoplasia: An Unusual Association

    Directory of Open Access Journals (Sweden)

    Nagendra Chaudhary

    2017-01-01

    Full Text Available Goldenhar syndrome (GS, a rare condition, occurring due to defect in development of first and second branchial arches, is characterized by a combination of various anomalies involving face, eyes, ears, vertebrae, heart, and lungs. The etiology of GS is not fully known, although various hypotheses have been proposed along with its genetic association and many other causes. Facial asymmetry and hypoplasia of the mandible are characteristic features of GS along with microtia and preauricular appendages and pits. Dextrocardia or pulmonary hypoplasia in GS has previously been reported separately. We report a 7-year-old female child of GS with combination of anomalies, dextrocardia, and pulmonary hypoplasia, which is a rare association.

  11. Surgical Management of Pilocytic Astrocytoma of the Optic Nerve: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Ifeoluwa Apanisile

    2017-01-01

    Full Text Available Optic nerve astrocytomas (ONAs are frequent types of optic nerve gliomas (ONGs, which can affect the visual pathway. An 18-year-old male patient was admitted to our department with right-sided intraorbital/retrobulbar swelling, which progressively grew over several months. Clinical examination showed right-sided diplopia, mydriasis, low visual acuity (0.4, exophthalmus (3 cm, epiphora, and severe retrobulbar pain. There was a family history of high-grade (IV astrocytomas in which two of the family members died due to the disease. Preoperative MRI scan revealed a soft tissue mass around the retrobulbar area of the right eye with intact orbital bony walls. Surgery was performed whereby it was dissected freely from the muscles and was separated from the optic nerve and the globe. Histopathologic analysis confirmed a benign astrocytoma. The follow-up examination revealed no recurrent or residual tumor. A systemic review of the literature indicates that early diagnosis and experienced multidisciplinary management are required in case of unilateral, resectable forms of ONAs with no distant metastasis, in order to provide a long-time survival of patients. Surgical intervention of unilateral ONAs is a relatively safe procedure, allowing complete or partial tumor removal with minimal morbidity and low recurrence rate.

  12. Genetics Home Reference: Leydig cell hypoplasia

    Science.gov (United States)

    ... normal male sexual development before birth and during puberty. In Leydig cell hypoplasia , affected individuals with a typical male chromosomal pattern (46,XY) may have a range of genital abnormalities. Affected males may have a small penis (micropenis), the opening of the urethra on the ...

  13. Spectral Domain Optical Coherence Tomography in Glaucoma: Qualitative and Quantitative Analysis of the Optic Nerve Head and Retinal Nerve Fiber Layer (An AOS Thesis)

    Science.gov (United States)

    Chen, Teresa C.

    2009-01-01

    Purpose: To demonstrate that video-rate spectral domain optical coherence tomography (SDOCT) can qualitatively and quantitatively evaluate optic nerve head (ONH) and retinal nerve fiber layer (RNFL) glaucomatous structural changes. To correlate quantitative SDOCT parameters with disc photography and visual fields. Methods: SDOCT images from 4 glaucoma eyes (4 patients) with varying stages of open-angle glaucoma (ie, early, moderate, late) were qualitatively contrasted with 2 age-matched normal eyes (2 patients). Of 61 other consecutive patients recruited in an institutional setting, 53 eyes (33 patients) met inclusion/exclusion criteria for quantitative studies. Images were obtained using two experimental SDOCT systems, one utilizing a superluminescent diode and the other a titanium:sapphire laser source, with axial resolutions of about 6 μm and 3 μm, respectively. Results: Classic glaucomatous ONH and RNFL structural changes were seen in SDOCT images. An SDOCT reference plane 139 μm above the retinal pigment epithelium yielded cup-disc ratios that best correlated with masked physician disc photography cup-disc ratio assessments. The minimum distance band, a novel SDOCT neuroretinal rim parameter, showed good correlation with physician cup-disc ratio assessments, visual field mean deviation, and pattern standard deviation (P values range, .0003–.024). RNFL and retinal thickness maps correlated well with disc photography and visual field testing. Conclusions: To our knowledge, this thesis presents the first comprehensive qualitative and quantitative evaluation of SDOCT images of the ONH and RNFL in glaucoma. This pilot study provides basis for developing more automated quantitative SDOCT-specific glaucoma algorithms needed for future prospective multicenter national trials. PMID:20126502

  14. Glenoid hypoplasia: a report of 2 patients.

    Science.gov (United States)

    Lynch, Christopher J; Taylor, John A M; Buchberger, Dale J

    2008-06-01

    This article discusses the imaging findings, clinical findings, and conservative chiropractic management of 2 patients with glenoid hypoplasia. Conventional radiographs of both patients revealed a hypoplastic glenoid bilaterally. Notch-like defects along with signs of degenerative disease were evident within the lower portion of the glenoid rims bilaterally in 1 patient and in the left glenoid rim of the other patient. Magnetic resonance imaging revealed a degenerative cyst or cortical defect in one patient along the anterior humeral head. The second patient showed a small slightly lobulated cystic region just posterior to the glenoid rim, consistent with the appearance of a synovial or ganglion cyst. Computed tomography with 3-dimensional reconstruction in 1 patient confirmed the presence of large posterior and superior osteophytes arising from the significantly hypoplastic glenoid. These images also revealed a slight posterior subluxation of the humeral head, widening of the anterior glenohumeral joint space, and retroversion of the glenoid. Treatment consisted of manual joint manipulation, soft tissue therapies, and therapeutic exercise for both patients. Both patients experienced improvements in symptoms, function, and physical examination findings. Glenoid hypoplasia is a developmental anomaly of the scapular neck which is predominantly bilateral and symmetric. Cross-sectional imaging studies should be considered in patients with symptoms that fail to improve over time. Conservative chiropractic care may be effective in managing symptoms in patients with glenoid hypoplasia.

  15. ImagePAD, a Novel Counting Application for the Apple iPad®, Used to Quantify Axons in the Mouse Optic Nerve

    Science.gov (United States)

    Templeton, Justin P.; Struebing, Felix L.; Lemmon, Andrew; Geisert, Eldon E.

    2014-01-01

    The present article introduces a new and easy to use counting application for the Apple iPad. The application “ImagePAD” takes advantage of the advanced user interface features offered by the Apple iOS® platform, simplifying the rather tedious task of quantifying features in anatomical studies. For example, the image under analysis can be easily panned and zoomed using iOS-supported multi-touch gestures without losing the spatial context of the counting task, which is extremely important for ensuring count accuracy. This application allows one to quantify up to 5 different types of objects in a single field and output the data in a tab-delimited format for subsequent analysis. We describe two examples of the use of the application: quantifying axons in the optic nerve of the C57BL/6J mouse and determining the percentage of cells labeled with NeuN or ChAT in the retinal ganglion cell layer. For the optic nerve, contiguous images at 60× magnification were taken and transferred onto an Apple iPad®. Axons were counted by tapping on the touch-sensitive screen using ImagePAD. Nine optic nerves were sampled and the number of axons in the nerves ranged from 38872 axons to 50196 axons with an average of 44846 axons per nerve (SD = 3980 axons). PMID:25281829

  16. Pregnancy Outcome in Cartilage-Hair Hypoplasia, a Rare Form of Dwarfism

    Directory of Open Access Journals (Sweden)

    Harshithaa Thavarajah

    2017-01-01

    Full Text Available Background. This case report discusses the pregnancy outcome of a patient with cartilage-hair hypoplasia, a rare form of dwarfism, and multiple previous orthopedic surgeries. Literature on pregnancy outcomes in patients with cartilage-hair hypoplasia is limited. Case. A 32-year-old patient with cartilage-hair hypoplasia presented at 12 weeks’ gestation to the high-risk obstetrics clinic for care. Preterm labor resulted in cesarean delivery at 34 weeks’ gestation with general anesthetic. Breastfeeding was stopped at 6 weeks due to neonatal complications. Conclusion. Pregnancy and delivery were uncomplicated. A multidisciplinary approach allowed for effective management during pregnancy and postnatal care. This is the first known documented case of prenatal care, delivery, and breastfeeding in a woman with this rare disorder.

  17. Imaging retinal nerve fiber bundles using optical coherence tomography with adaptive optics.

    Science.gov (United States)

    Kocaoglu, Omer P; Cense, Barry; Jonnal, Ravi S; Wang, Qiang; Lee, Sangyeol; Gao, Weihua; Miller, Donald T

    2011-08-15

    Early detection of axonal tissue loss in retinal nerve fiber layer (RNFL) is critical for effective treatment and management of diseases such as glaucoma. This study aims to evaluate the capability of ultrahigh-resolution optical coherence tomography with adaptive optics (UHR-AO-OCT) for imaging the RNFL axonal bundles (RNFBs) with 3×3×3μm(3) resolution in the eye. We used a research-grade UHR-AO-OCT system to acquire 3°×3° volumes in four normal subjects and one subject with an arcuate retinal nerve fiber layer defect (n=5; 29-62years). Cross section (B-scans) and en face (C-scan) slices extracted from the volumes were used to assess visibility and size distribution of individual RNFBs. In one subject, we reimaged the same RNFBs twice over a 7month interval and compared bundle width and thickness between the two imaging sessions. Lastly we compared images of an arcuate RNFL defect acquired with UHR-AO-OCT and commercial OCT (Heidelberg Spectralis). Individual RNFBs were distinguishable in all subjects at 3° retinal eccentricity in both cross-sectional and en face views (width: 30-50μm, thickness: 10-15μm). At 6° retinal eccentricity, RNFBs were distinguishable in three of the five subjects in both views (width: 30-45μm, thickness: 20-40μm). Width and thickness RNFB measurements taken 7months apart were strongly correlated (p<0.0005). Mean difference and standard deviation of the differences between the two measurement sessions were -0.1±4.0μm (width) and 0.3±1.5μm (thickness). UHR-AO-OCT outperformed commercial OCT in terms of clarity of the microscopic retina. To our knowledge, these are the first measurements of RNFB cross section reported in the living human eye. Copyright © 2011 Elsevier Ltd. All rights reserved.

  18. Evaluation of a combined index of optic nerve structure and function for glaucoma diagnosis

    Science.gov (United States)

    2011-01-01

    Background The definitive diagnosis of glaucoma is currently based on congruent damage to both optic nerve structure and function. Given widespread quantitative assessment of both structure (imaging) and function (automated perimetry) in glaucoma, it should be possible to combine these quantitative data to diagnose disease. We have therefore defined and tested a new approach to glaucoma diagnosis by combining imaging and visual field data, using the anatomical organization of retinal ganglion cells. Methods Data from 1499 eyes of glaucoma suspects and 895 eyes with glaucoma were identified at a single glaucoma center. Each underwent Heidelberg Retinal Tomograph (HRT) imaging and standard automated perimetry. A new measure combining these two tests, the structure function index (SFI), was defined in 3 steps: 1) calculate the probability that each visual field point is abnormal, 2) calculate the probability of abnormality for each of the six HRT optic disc sectors, and 3) combine those probabilities with the probability that a field point and disc sector are linked by ganglion cell anatomy. The SFI was compared to the HRT and visual field using receiver operating characteristic (ROC) analysis. Results The SFI produced an area under the ROC curve (0.78) that was similar to that for both visual field mean deviation (0.78) and pattern standard deviation (0.80) and larger than that for a normalized measure of HRT rim area (0.66). The cases classified as glaucoma by the various tests were significantly non-overlapping. Based on the distribution of test values in the population with mild disease, the SFI may be better able to stratify this group while still clearly identifying those with severe disease. Conclusions The SFI reflects the traditional clinical diagnosis of glaucoma by combining optic nerve structure and function. In doing so, it identifies a different subset of patients than either visual field testing or optic nerve head imaging alone. Analysis of prospective

  19. Equating spatial summation in visual field testing reveals greater loss in optic nerve disease.

    Science.gov (United States)

    Kalloniatis, Michael; Khuu, Sieu K

    2016-07-01

    To test the hypothesis that visual field assessment in ocular disease measured with target stimuli within or close to complete spatial summation results in larger threshold elevation compared to when measured with the standard Goldmann III target size. The hypothesis predicts a greater loss will be identified in ocular disease. Additionally, we sought to develop a theoretical framework that would allow comparisons of thresholds with disease progression when using different Goldmann targets. The Humphrey Field Analyser (HFA) 30-2 grid was used in 13 patients with early/established optic nerve disease using the current Goldmann III target size or a combination of the three smallest stimuli (target size I, II and III). We used data from control subjects at each of the visual field locations for the different target sizes to establish the number of failed points (events) for the patients with optic nerve disease, as well as global indices for mean deviation (MD) and pattern standard deviation (PSD). The 30-2 visual field testing using alternate target size stimuli showed that all 13 patients displayed more defects (events) compared to the standard Goldmann III target size. The median increase for events was seven additional failed points: (range 1-26). The global indices also increased when the new testing approach was used (MD -3.47 to -6.25 dB and PSD 4.32 to 6.63 dB). Spatial summation mapping showed an increase in critical area (Ac) in disease and overall increase in thresholds when smaller target stimuli were used. When compared to the current Goldmann III paradigm, the use of alternate sized targets within the 30-2 testing protocol revealed a greater loss in patients with optic nerve disease for both event analysis and global indices (MD and PSD). We therefore provide evidence in a clinical setting that target size is important in visual field testing. © 2016 The Authors Ophthalmic & Physiological Optics © 2016 The College of Optometrists.

  20. Cerebrospinal Fluid Pressure: Revisiting Factors Influencing Optic Nerve Head Biomechanics

    Science.gov (United States)

    Hua, Yi; Voorhees, Andrew P.; Sigal, Ian A.

    2018-01-01

    Purpose To model the sensitivity of the optic nerve head (ONH) biomechanical environment to acute variations in IOP, cerebrospinal fluid pressure (CSFP), and central retinal artery blood pressure (BP). Methods We extended a previously published numerical model of the ONH to include 24 factors representing tissue anatomy and mechanical properties, all three pressures, and constraints on the optic nerve (CON). A total of 8340 models were studied to predict factor influences on 98 responses in a two-step process: a fractional factorial screening analysis to identify the 16 most influential factors, followed by a response surface methodology to predict factor effects in detail. Results The six most influential factors were, in order: IOP, CON, moduli of the sclera, lamina cribrosa (LC) and dura, and CSFP. IOP and CSFP affected different aspects of ONH biomechanics. The strongest influence of CSFP, more than twice that of IOP, was on the rotation of the peripapillary sclera. CSFP had similar influence on LC stretch and compression to moduli of sclera and LC. On some ONHs, CSFP caused large retrolamina deformations and subarachnoid expansion. CON had a strong influence on LC displacement. BP overall influence was 633 times smaller than that of IOP. Conclusions Models predict that IOP and CSFP are the top and sixth most influential factors on ONH biomechanics. Different IOP and CSFP effects suggest that translaminar pressure difference may not be a good parameter to predict biomechanics-related glaucomatous neuropathy. CON may drastically affect the responses relating to gross ONH geometry and should be determined experimentally. PMID:29332130

  1. Pontocerebellar hypoplasia associated with respiratory-chain defects

    NARCIS (Netherlands)

    de Koning, T. J.; de Vries, L. S.; Groenendaal, F.; Ruitenbeek, W.; Jansen, G. H.; Poll-The, B. T.; Barth, P. G.

    1999-01-01

    Pontocerebellar hypoplasias are congenital disorders of brain morphogenesis which include such diverse etiologies as carbohydrate-deficient glycoprotein syndrome type 1, cerebromuscular dystrophies (Walker-Warburg syndrome, Fukuyama syndrome, muscle-eye-brain disease) and at least two types of

  2. Usefulness of optic nerve ultrasound to predict clinical progression in multiple sclerosis.

    Science.gov (United States)

    Pérez Sánchez, S; Eichau Madueño, S; Rus Hidalgo, M; Domínguez Mayoral, A M; Vilches-Arenas, A; Navarro Mascarell, G; Izquierdo, G

    2018-03-21

    Progressive neuronal and axonal loss are considered the main causes of disability in patients with multiple sclerosis (MS). The disease frequently involves the visual system; the accessibility of the system for several functional and structural tests has made it a model for the in vivo study of MS pathogenesis. Orbital ultrasound is a non-invasive technique that enables various structures of the orbit, including the optic nerve, to be evaluated in real time. We conducted an observational, ambispective study of MS patients. Disease progression data were collected. Orbital ultrasound was performed on all patients, with power set according to the 'as low as reasonably achievable' (ALARA) principle. Optical coherence tomography (OCT) data were also collected for those patients who underwent the procedure. Statistical analysis was conducted using SPSS version 22.0. Disease progression was significantly correlated with ultrasound findings (P=.041 for the right eye and P=.037 for the left eye) and with Expanded Disability Status Scale (EDSS) score at the end of the follow-up period (P=.07 for the right eye and P=.043 for the left eye). No statistically significant differences were found with relation to relapses or other clinical variables. Ultrasound measurement of optic nerve diameter constitutes a useful, predictive factor for the evaluation of patients with MS. Smaller diameters are associated with poor clinical progression and greater disability (measured by EDSS). Copyright © 2018 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  3. Anatomical Variations of Carotid Artery and Optic Nerve in Sphenoid Sinus Using Computerized Tomographic Imaging

    Directory of Open Access Journals (Sweden)

    Nikakhlagh

    2014-12-01

    Full Text Available Background Sphenoid sinus is surrounded by many vital vascular and nervous structures. In more than 20% of patients with chronic sinusitis, involvement of sphenoid sinus has been observed. Besides, sphenoid sinus is an appropriate route to access anterior and middle cranial fossa in surgery. Therefore, it is important to have an adequate knowledge about the contents of sphenoid sinus and its proximity for nasal endoscopy, sinus surgeries and neurosurgeries. Objectives The aim of this study was to study sphenoid sinus proximity with carotid artery and the optic nerve using computerized tomographic imaging. Materials and Methods In this prospective study, computerized tomographic images of sphenoid sinus of patients referred to Imam Khomeini and Apadana hospitals were studied. The images were studied regarding any bulging, as well as not having a bone covering in sphenoid sinus regarding internal carotid artery and optic nerve. Furthermore, unilateralness or bilateralness of their relationships was studied. Results Among 468 coronal and axial CT scan images of sphenoid sinus, 365 (78% showed post-sellar pneumatization and 103 (22% pre-sellar pneumatization. Regarding existence of internal septa, 346 (74% cases showed multiple septation, and the remaining images were reported to have a single septum. According to the reports of CT scan images, the existence of bulging as a result of internal carotid artery and uncovered artery were 4.22% and 5.8% in the right sinus, 4.9% and 5.4% in the left sinus, and 4.34% and 4.6% in both sinuses, respectively. According to the reports of CT scan images, existence of bulging as a result of optic nerve and uncovered nerve were 5.7% and 4.3% in the right sinus, 6% and 5.4% in the left sinus, and 12% and 3.2% in both sinuses, respectively. Conclusions Due to variability of sphenoid sinus pneumatization and the separator blade of the two sinus cavities, careful attention is required during sinus surgery to avoid

  4. Multifocal visual evoked potentials for quantifying optic nerve dysfunction in patients with optic disc drusen

    DEFF Research Database (Denmark)

    Malmqvist, Lasse; de Santiago, Luis; Boquete, Luciano

    2017-01-01

    and 22 control subjects were examined. Mean amplitude, mean inner ring (IR) amplitude (0.87-5.67° of visual field) and mean outer ring amplitude (5.68-24° of visual field) were calculated using signal-to-noise ratio (SNR) and peak-to-peak analysis. Monocular latency was calculated using second peak......PURPOSE: To explore the applicability of multifocal visual evoked potentials (mfVEPs) for research and clinical diagnosis in patients with optic disc drusen (ODD). This is the first assessment of mfVEP amplitude in patients with ODD. METHODS: MfVEP amplitude and latency from 33 patients with ODD......, full eye and IR. In the control group, SNR intersubject variability was 17.6% and second peak latency intersubject variability was 2.8%. CONCLUSION: Decreased mfVEP amplitude in patients with ODD suggests a direct mechanical compression of the optic nerve axons. Our results suggest that mfVEP amplitude...

  5. [Local involvement of the optic nerve by acute lymphoblastic leukemia].

    Science.gov (United States)

    Bernardczyk-Meller, Jadwiga; Stefańska, Katarzyna

    2005-01-01

    The leucemias quite commonly involve the eyes and adnexa. In some cases it causes visual complants. Both, the anterior chamber of the eye and the posterior portion of the globe may sites of acute or chronic leukemia and leucemic relapse. We report an unique case of a 14 years old leucemic patient who suffered visual loss and papilloedema, due to a unilateral local involvement within optic nerve, during second relapse of acute lymphocytic leuemia. In spite of typical treatment of main disease, the boy had died. The authors present typical ophthalmic features of the leucemia, too.

  6. A Deep Learning Approach to Digitally Stain Optical Coherence Tomography Images of the Optic Nerve Head.

    Science.gov (United States)

    Devalla, Sripad Krishna; Chin, Khai Sing; Mari, Jean-Martial; Tun, Tin A; Strouthidis, Nicholas G; Aung, Tin; Thiéry, Alexandre H; Girard, Michaël J A

    2018-01-01

    To develop a deep learning approach to digitally stain optical coherence tomography (OCT) images of the optic nerve head (ONH). A horizontal B-scan was acquired through the center of the ONH using OCT (Spectralis) for one eye of each of 100 subjects (40 healthy and 60 glaucoma). All images were enhanced using adaptive compensation. A custom deep learning network was then designed and trained with the compensated images to digitally stain (i.e., highlight) six tissue layers of the ONH. The accuracy of our algorithm was assessed (against manual segmentations) using the dice coefficient, sensitivity, specificity, intersection over union (IU), and accuracy. We studied the effect of compensation, number of training images, and performance comparison between glaucoma and healthy subjects. For images it had not yet assessed, our algorithm was able to digitally stain the retinal nerve fiber layer + prelamina, the RPE, all other retinal layers, the choroid, and the peripapillary sclera and lamina cribrosa. For all tissues, the dice coefficient, sensitivity, specificity, IU, and accuracy (mean) were 0.84 ± 0.03, 0.92 ± 0.03, 0.99 ± 0.00, 0.89 ± 0.03, and 0.94 ± 0.02, respectively. Our algorithm performed significantly better when compensated images were used for training (P deep learning algorithm can simultaneously stain the neural and connective tissues of the ONH, offering a framework to automatically measure multiple key structural parameters of the ONH that may be critical to improve glaucoma management.

  7. Hydrostatic Pressure–Induced Release of Stored Calcium in Cultured Rat Optic Nerve Head Astrocytes

    Science.gov (United States)

    Mandal, Amritlal; Delamere, Nicholas A.

    2010-01-01

    Purpose. Elevated intraocular pressure is associated with glaucomatous optic nerve damage. Other investigators have shown functional changes in optic nerve head astrocytes subjected to elevated hydrostatic pressure (HP) for 1 to 5 days. Recently, the authors reported ERK1/2, p90RSK and NHE1 phosphorylation after 2 hours. Here they examine calcium responses at the onset of HP to determine what precedes ERK1/2 phosphorylation. Methods. Cytoplasmic calcium concentration ([Ca2+]i) was measured in cultured rat optic nerve astrocytes loaded with fura-2. The cells were placed in a closed imaging chamber and subjected to an HP increase of 15 mm Hg. Protein phosphorylation was detected by Western blot analysis. Results. The increase of HP caused an immediate slow increase in [Ca2+]i. The response persisted in calcium-free solution and when nickel chloride (4 mM) was added to suppress channel-mediated calcium entry. Previous depletion of the ER calcium stores by cyclopiazonic acid abolished the HP-induced calcium level increase. The HP-induced increase persisted in cells exposed to xestospongin C, an inhibitor of IP3R-mediated calcium release. In contrast, ryanodine receptor (RyR) antagonist ruthenium red (10 μM) or dantrolene (25 μM) inhibited the HP-induced calcium increase. The HP-induced calcium increase was abolished when ryanodine-sensitive calcium stores were pre-depleted with caffeine (3 mM). HP caused ERK1/2 phosphorylation. The magnitude of the ERK1/2 phosphorylation response was reduced by ruthenium red and dantrolene. Conclusions. Increasing HP causes calcium release from a ryanodine-sensitive cytoplasmic store and subsequent ERK1/2 activation. Calcium store release appears to be a required early step in the initial astrocyte response to an HP increase. PMID:20071675

  8. Phenotype analysis of early risk factors from electronic medical records improves image-derived diagnostic classifiers for optic nerve pathology

    Science.gov (United States)

    Chaganti, Shikha; Nabar, Kunal P.; Nelson, Katrina M.; Mawn, Louise A.; Landman, Bennett A.

    2017-03-01

    We examine imaging and electronic medical records (EMR) of 588 subjects over five major disease groups that affect optic nerve function. An objective evaluation of the role of imaging and EMR data in diagnosis of these conditions would improve understanding of these diseases and help in early intervention. We developed an automated image processing pipeline that identifies the orbital structures within the human eyes from computed tomography (CT) scans, calculates structural size, and performs volume measurements. We customized the EMR-based phenome-wide association study (PheWAS) to derive diagnostic EMR phenotypes that occur at least two years prior to the onset of the conditions of interest from a separate cohort of 28,411 ophthalmology patients. We used random forest classifiers to evaluate the predictive power of image-derived markers, EMR phenotypes, and clinical visual assessments in identifying disease cohorts from a control group of 763 patients without optic nerve disease. Image-derived markers showed more predictive power than clinical visual assessments or EMR phenotypes. However, the addition of EMR phenotypes to the imaging markers improves the classification accuracy against controls: the AUC improved from 0.67 to 0.88 for glaucoma, 0.73 to 0.78 for intrinsic optic nerve disease, 0.72 to 0.76 for optic nerve edema, 0.72 to 0.77 for orbital inflammation, and 0.81 to 0.85 for thyroid eye disease. This study illustrates the importance of diagnostic context for interpretation of image-derived markers and the proposed PheWAS technique provides a flexible approach for learning salient features of patient history and incorporating these data into traditional machine learning analyses.

  9. Trans-sinusal maxillary distraction for correction of midfacial hypoplasia: long-term clinical results.

    NARCIS (Netherlands)

    Nadjmi, N.; Schutyser, F.A.C.; Erum, R. van

    2006-01-01

    Maxillary distraction osteogenesis is indicated in severe angle class III malocclusions, and severe maxillary hypoplasia among some cleft patients and other craniofacial deformities. Twenty patients, aged 8-48 years (mean 17.8+/-10.5 SD) with maxillary and midfacial hypoplasia were treated. The

  10. Optic Nerve Head and Retinal Nerve Fiber Layer Analysis in Ocular Hypertension and Early-Stage Glucoma Using Spectral-Domain Optical Coherence Tomography Copernicus

    Directory of Open Access Journals (Sweden)

    Nilgün Solmaz

    2014-01-01

    Full Text Available Objectives: Evaluation of structural alterations of the optic nerve head (ONH and the retinal nerve fiber layer (RNFL in patients with ocular hypertension (OHT and early-stage glaucoma and assessment of the discriminatory diagnostic performance of spectral-domain optical coherence tomography (SD-OCT Copernicus (Optopol Technology S.A.. Materials and Methods: This study included 59 eyes of a total of 59 patients, 29 of whom were diagnosed with OHT (Group 1 and 30 with early-stage glaucoma (Group 2. The differentiation of early-stage glaucoma and OHT was carried out on the basis of standard achromatic visual field test results. Analysis of the ONH and RNFL thickness of all cases was made using SD-OCT. Group 1 and Group 2 were compared with respect to the ONH parameters and RNFL thickness. The diagnostic sensitivity of the OCT parameters was evaluated by the area under the receiver operating characteristics curves (AUC. Results: The average, superior, inferior, and nasal RNFL thicknesses in early-stage glaucoma cases were approximately 10% (12-14 µm less compared to the OHT eyes, with differences being highly significant (p≤0.001. However, there was no statistically significant difference in the temporal RNFL thicknesses. The most sensitive parameter in the early diagnosis of glaucoma was average RNFL thickness corresponding to AUC: 0.852, followed by AUC: 0.816 and AUC: 0.773 values in superior and inferior RNFL thickness, respectively. In localized RNFL defects, the highest sensitivity corresponded to superior and superonasal quadrants (ACU: 0.805 and ACU: 0.781, respectively. There were not any statistically significant differences between the ONH morphological parameters of the two groups. Conclusion: RNFL analysis obtained using SD-OCT Copernicus is able to discriminate early-stage glaucoma eyes from those with OHT. However, ONH morphological parameters do not have the same diagnostic sensitivity. Turk J Ophthalmol 2014; 44: 35-41

  11. [Effectiveness of sub-Tenon's capsule implantation of collage sponge in the treatment of glaucomatous atrophy of the optic nerve].

    Science.gov (United States)

    Svirin, A V; Khou Sian'zhu; Eliseeva, T O

    2003-01-01

    The surgery of "subtenon implantation of collagen sponge" is offered; its essence is in implanting a fragment of hemostatic collagen sponge for the purpose of revasculizing and improving the trophism of the retina and optic nerve. A total of 96 patients (105 eyes) with glaucomatous atrophy of the optic nerve were operated on. The reliable results of visometry as well as of kinetic and static perimetry and of rheography were obtained. The efficiency of the surgery (without any additional conservative therapy) ranged from 63.4 to 78.6% according to various examination methods. The visual functions improved within 6 to 12 months after surgery.

  12. Pulmonary Hypoplasia Caused by Fetal Ascites in Congenital Cytomegalovirus Infection Despite Fetal Therapy

    Directory of Open Access Journals (Sweden)

    Kazumichi Fujioka

    2017-11-01

    Full Text Available We report two cases of pulmonary hypoplasia due to fetal ascites in symptomatic congenital cytomegalovirus (CMV infections despite fetal therapy. The patients died soon after birth. The pathogenesis of pulmonary hypoplasia in our cases might be thoracic compression due to massive fetal ascites as a result of liver insufficiency. Despite aggressive fetal treatment, including multiple immunoglobulin administration, which was supposed to diminish the pathogenic effects of CMV either by neutralization or immunomodulatory effects, the fetal ascites was uncontrollable. To prevent development of pulmonary hypoplasia in symptomatic congenital CMV infections, further fetal intervention to reduce ascites should be considered.

  13. Genetics Home Reference: VLDLR-associated cerebellar hypoplasia

    Science.gov (United States)

    ... hypoplasia include moderate to profound intellectual disability, impaired speech (dysarthria) or a lack of speech, and eyes that do not look in the ... the United States. This condition has also been reported in families from Iran and Turkey. Related Information ...

  14. Developmental impairment of compound action potential in the optic nerve of myelin mutant taiep rats.

    Science.gov (United States)

    Roncagliolo, Manuel; Schlageter, Carol; León, Claudia; Couve, Eduardo; Bonansco, Christian; Eguibar, José R

    2006-01-05

    The taiep rat is a myelin mutant with an initial hypomyelination, followed by a progressive demyelination of the CNS. The neurological correlates start with tremor, followed by ataxia, immobility episodes, epilepsy and paralysis. The optic nerve, an easily-isolable central tract fully myelinated by oligodendrocytes, is a suitable preparation to evaluate the developmental impairment of central myelin. We examined the ontogenic development of optic nerve compound action potentials (CAP) throughout the first 6 months of life of control and taiep rats. Control optic nerves (ON) develop CAPs characterized by three waves. Along the first month, the CAPs of taiep rats showed a delayed maturation, with lower amplitudes and longer latencies than controls; at P30, the conduction velocity has only a third of the normal value. Later, as demyelination proceeds, the conduction velocity of taiep ONs begins to decrease and CAPs undergo a gradual temporal dispersion. CAPs of control and taiep showed differences in their pharmacological sensitivity to TEA and 4-AP, two voltage dependent K+ channel-blockers. As compared with TEA, 4-AP induced a significant increase of the amplitudes and a remarkable broadening of CAPs. After P20, unlike controls, the greater sensitivity to 4-AP exhibited by taiep ONs correlates with the detachment and retraction of paranodal loops suggesting that potassium conductances could regulate the excitability as demyelination of CNS axons progresses. It is concluded that the taiep rat, a long-lived mutant, provides a useful model to study the consequences of partial demyelination and the mechanisms by which glial cells regulate the molecular organization and excitability of axonal membranes during development and disease.

  15. Comparison of optic disc morphology of optic nerve atrophy between compressive optic neuropathy and glaucomatous optic neuropathy.

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    Masayuki Hata

    Full Text Available To compare the optic nerve head (ONH structure between compressive optic neuropathy (CON and glaucomatous optic neuropathy (GON, and to determine whether selected ONH quantitative parameters effectively discriminate between GON and CON, especially CON cases presenting with a glaucoma-like disc.We prospectively assessed 34 patients with CON, 34 age-matched patients with moderate or severe GON, and 34 age-matched healthy control subjects. The quantitative parameters of ONH structure were compared using the Heidelberg Retina Tomograph 2 (HRT2 and Spectralis optical coherence tomography with an enhanced depth imaging method.The mean and maximum cup depths of CON were significantly smaller than those with GON (P < 0.001 and P < 0.001, respectively. The distance between Bruch's membrane opening and anterior surface of the lamina cribrosa (BMO-anterior LC of CON was also significantly smaller than that of glaucoma but was similar to that of the healthy group (P < 0.001 and P = 0.47, respectively. Based on Moorfields regression analysis of the glaucoma classification of HRT2, 15 eyes with CON were classified with a glaucoma-like disc. The cup/disc area ratio did not differ between cases of CON with a glaucoma-like disc and cases of GON (P = 0.16, but the BMO-anterior LC and mean and maximum cup depths of CON cases with a glaucoma-like disc were smaller than those in GON (P = 0.005, P = 0.003, and P = 0.001, respectively.Measurements of the cup depths and the LC depth had good ability to differentiate between CON with a glaucoma-like disc and glaucoma. There was no laminar remodeling detected by laminar surface position in the patients with CON compared to those with GON.

  16. Microsurgical training model for residents to approach to the orbit and the optic nerve in fresh cadaveric sheep cranium

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    M Emre Altunrende

    2014-01-01

    Full Text Available Background: Neurosurgery and ophthalmology residents need many years to improve microsurgical skills. Laboratory training models are very important for developing surgical skills before clinical application of microsurgery. A simple simulation model is needed for residents to learn how to handle microsurgical instruments and to perform safe dissection of intracranial or intraorbital nerves, vessels, and other structures. Materials and Methods: The simulation material consists of a one-year-old fresh cadaveric sheep cranium. Two parts (Part 1 and Part 2 were designed to approach structures of the orbit. Part 1 consisted of a 2-step approach to dissect intraorbital structures, and Part 2 consisted of a 3-step approach to dissect the optic nerve intracranially. Results: The model simulates standard microsurgical techniques using a variety of approaches to structures in and around the orbit and the optic nerve. Conclusions: This laboratory training model enables trainees to gain experience with an operating microscope, microsurgical instruments and orbital structures.

  17. Relevance of CT and MRI in retinoblastoma for the diagnosis of postlaminar invasion with normal-size optic nerve: a retrospective study of 150 patients with histological comparison

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    Brisse, Herve J.; Guesmi, Myriam; Neuenschwander, Sylvia; Aerts, Isabelle; Sastre-Garau, Xavier; Savignoni, Alexia; Asselain, Bernard; Bours, Daniele; Lumbroso-Le Rouic, Livia; Desjardins, Laurence; Doz, Francois

    2007-01-01

    Detection of optic nerve invasion is mandatory in children primarily enucleated for retinoblastoma to ensure a free resection margin. To assess the accuracy of CT and MRI for the detection of postlaminar invasion in normal-size nerves. A total of 150 patients enucleated for retinoblastoma were included. Imaging data (119 CT and 46 MRI) were retrospectively reviewed and compared with histological findings. Abnormal contrast enhancement of the optic nerve was used as diagnostic criterion for invasion. The associations between postlaminar invasion and several indirect signs were also assessed. Statistical analysis was performed with the Kruskal-Wallis and Fisher exact tests. Postlaminar invasion on histology was observed in 8% (12/150). The sensitivity, specificity, accuracy and negative and positive predictive values were 60%, 95%, 91%, 95% and 60% for MRI, and 0%, 100%, 94% and 94% (PPV not assessable) for CT, respectively. Tumour diameter was the only indirect radiological sign significantly associated with postlaminar optic nerve invasion (P=0.002). Our results suggest that MRI is more relevant than CT for preoperative detection of optic nerve invasion in patients with retinoblastoma. Tumour diameter is the only indirect sign significantly associated with postlaminar invasion. (orig.)

  18. Relevance of CT and MRI in retinoblastoma for the diagnosis of postlaminar invasion with normal-size optic nerve: a retrospective study of 150 patients with histological comparison

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    Brisse, Herve J.; Guesmi, Myriam; Neuenschwander, Sylvia [Institute Curie, Department of Radiology, Paris (France); Aerts, Isabelle [Institute Curie, Department of Paediatric Oncology, Paris (France); Sastre-Garau, Xavier [Institute Curie, Department of Pathology, Paris (France); Savignoni, Alexia; Asselain, Bernard; Bours, Daniele [Institute Curie, Department of Biostatistics, Paris (France); Lumbroso-Le Rouic, Livia; Desjardins, Laurence [Institute Curie, Department of Ocular Oncology, Paris (France); Doz, Francois [Institute Curie, Department of Paediatric Oncology, Paris (France); Faculty of Medicine, Department of Paediatrics, Paris (France)

    2007-07-15

    Detection of optic nerve invasion is mandatory in children primarily enucleated for retinoblastoma to ensure a free resection margin. To assess the accuracy of CT and MRI for the detection of postlaminar invasion in normal-size nerves. A total of 150 patients enucleated for retinoblastoma were included. Imaging data (119 CT and 46 MRI) were retrospectively reviewed and compared with histological findings. Abnormal contrast enhancement of the optic nerve was used as diagnostic criterion for invasion. The associations between postlaminar invasion and several indirect signs were also assessed. Statistical analysis was performed with the Kruskal-Wallis and Fisher exact tests. Postlaminar invasion on histology was observed in 8% (12/150). The sensitivity, specificity, accuracy and negative and positive predictive values were 60%, 95%, 91%, 95% and 60% for MRI, and 0%, 100%, 94% and 94% (PPV not assessable) for CT, respectively. Tumour diameter was the only indirect radiological sign significantly associated with postlaminar optic nerve invasion (P=0.002). Our results suggest that MRI is more relevant than CT for preoperative detection of optic nerve invasion in patients with retinoblastoma. Tumour diameter is the only indirect sign significantly associated with postlaminar invasion. (orig.)

  19. Transdermal optogenetic peripheral nerve stimulation

    Science.gov (United States)

    Maimon, Benjamin E.; Zorzos, Anthony N.; Bendell, Rhys; Harding, Alexander; Fahmi, Mina; Srinivasan, Shriya; Calvaresi, Peter; Herr, Hugh M.

    2017-06-01

    Objective: A fundamental limitation in both the scientific utility and clinical translation of peripheral nerve optogenetic technologies is the optical inaccessibility of the target nerve due to the significant scattering and absorption of light in biological tissues. To date, illuminating deep nerve targets has required implantable optical sources, including fiber-optic and LED-based systems, both of which have significant drawbacks. Approach: Here we report an alternative approach involving transdermal illumination. Utilizing an intramuscular injection of ultra-high concentration AAV6-hSyn-ChR2-EYFP in rats. Main results: We demonstrate transdermal stimulation of motor nerves at 4.4 mm and 1.9 mm depth with an incident laser power of 160 mW and 10 mW, respectively. Furthermore, we employ this technique to accurately control ankle position by modulating laser power or position on the skin surface. Significance: These results have the potential to enable future scientific optogenetic studies of pathologies implicated in the peripheral nervous system for awake, freely-moving animals, as well as a basis for future clinical studies.

  20. Malnutrition and myelin structure: an X-ray scattering study of rat sciatic and optic nerves

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    Vargas, V.; Vargas, R.; Marquez, G.; Vonasek, E.; Mateu, L.; Luzzati, V.; Borges, J.

    2000-01-01

    Taking advantage of the fast and accurate X-ray scattering techniques recently developed in our laboratory, we tackled the study of the structural alterations induced in myelin by malnutrition. Our work was performed on sciatic and optic nerves dissected from rats fed with either a normal or a low-protein caloric diet, as a function of age (from birth to 60 days). By way of electrophysiological controls we also measured (on the sciatic nerves) the height and velocity of the compound action potential. Malnutrition was found to decrease the amount of myelin and to impair the packing order of the membranes in the sheaths. (orig.)

  1. Time course Analysis of Gene expression patterns in ZebrafIsh Eye during Optic Nerve Regeneration

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    Amy T. Mccurley

    2010-01-01

    Full Text Available It is well-established that neurons in the adult mammalian central nervous system (CNS are terminally differentiated and, if injured, will be unable to regenerate their connections. In contrast to mammals, zebrafish and other teleosts display a robust neuroregenerative response. Following optic nerve crush (ONX, retinal ganglion cells (RGC regrow their axons to synapse with topographically correct targets in the optic tectum, such that vision is restored in ~21 days. What accounts for these differences between teleostean and mammalian responses to neural injury is not fully understood. A time course analysis of global gene expression patterns in the zebrafish eye after ONX can help to elucidate cellular and molecular mechanisms that contribute to a successful neuroregeneration. To define different phases of regeneration after ONX, alpha tubulin 1 ( tuba1 and growth-associated protein 43 ( gap43 , markers previously shown to correspond to morphophological events, were measured by real time quantitative PCR (qPCR. Microarray analysis was then performed at defined intervals (6 hours, 1, 4, 12, and 21 days post-ONX and compared to SHAM. Results show that optic nerve damage induces multiple, phase-related transcriptional programs, with the maximum number of genes changed and highest fold-change occurring at 4 days. Several functional groups affected by optic nerve regeneration, including cell adhesion, apoptosis, cell cycle, energy metabolism, ion channel activity, and calcium signaling, were identified. Utilizing the whole eye allowed us to identify signaling contributions from the vitreous, immune and glial cells as well as the neural cells of the retina. Comparisons between our dataset and transcriptional profiles from other models of regeneration in zebrafish retina, heart and fin revealed a subset of commonly regulated transcripts, indicating shared mechanisms in different regenerating tissues. Knowledge of gene expression patterns in all

  2. Anterior ischemic optic neuropathy in association with optic nervehead drusen

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    Bharathi Megur

    2014-01-01

    Full Text Available Optic nerve head drusen (ONHD are incidental ophthalmologic finding in the optic nerve. Patients with ONHD are often asymptomatic, but sometimes present with transient visual obscuration′s (TVO, the reported incidence of which is 8.6%. Optic nerve head drusen are of two types: Superficial; visible and deep. The deep-buried drusen mimic papilledema. Because of the varied presentation deep-buried drusen pose a diagnostic challenge to the ophthalmologists. In young patients, they are mistaken for papilledema as it is clinically difficult to detect a buried drusen in the optic nerve head, but are seen on the surface with aging as the retinal nerve fiber layer thins out. They are observed as pale yellow lesions more often located towards the poles. Clinical examination aided with diagnostic tests like computed tomography (CT orbits and ultrasound B scan can help establish the diagnosis. Herein, we report a rare case of optic nerve head drusen in a young lady, who presented with loss of vision and clinical evaluation and investigations suggested ONHD with anterior ischemic optic neuropathy.

  3. Quantification of retinal nerve fiber layer thickness using spectral domain optical coherence tomography in normal Indian population

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    Tarannum Mansoori

    2012-01-01

    Full Text Available The purpose of this study was to measure peripapillary retinal nerve fiber layer thickness (RNFLT using spectral domain optical coherence tomography (SD-OCT in normal Indian eyes, for which, 210 normal volunteers were recruited. One eye of each subject underwent RNFL scanning at 3.4 mm circle diameter around optic nerve using SD OCT. The data were analyzed to determine RNFLT in the sample population and its variation with age and gender. The average peripapillary RNFLT was 114.03 ± 9.59 μm. There was no effect of gender on RNFLT parameters. Age had significant negative correlation with average (P = 0.005, superior (P = 0.04, temporal (P = 0.049, and nasal quadrants (P = 0.01 RNFLT. Inferior quadrant RNFLT also had a negative correlation with age, but it was not statistically significant (P = 0.15.

  4. Comparison of optic area measurement using fundus photography and optical coherence tomography between optic nerve head drusen and control subjects.

    Science.gov (United States)

    Flores-Rodríguez, Patricia; Gili, Pablo; Martín-Ríos, María Dolores; Grifol-Clar, Eulalia

    2013-03-01

    To compare optic disc area measurement between optic nerve head drusen (ONHD) and control subjects using fundus photography, time-domain optical coherence tomography (TD-OCT) and spectral-domain optical coherence tomography (SD-OCT). We also made a comparison between each of the three techniques. We performed our study on 66 eyes (66 patients) with ONHD and 70 healthy control subjects (70 controls) with colour ocular fundus photography at 20º (Zeiss FF 450 IR plus), TD-OCT (Stratus OCT) with the Fast Optic Disc protocol and SD-OCT (Cirrus OCT) with the Optic Disc Cube 200 × 200 protocol for measurement of the optic disc area. The measurements were made by two observers and in each measurement a correction of the image magnification factor was performed. Measurement comparison using the Student's t-test/Mann-Whitney U test, the intraclass correlation coefficient, Pearson/Spearman rank correlation coefficient and the Bland-Altman plot was performed in the statistical analysis. Mean and standard deviation (SD) of the optic disc area in ONHD and in controls was 2.38 (0.54) mm(2) and 2.54 (0.42) mm(2), respectively with fundus photography; 2.01 (0.56) mm(2) and 1.66 (0.37) mm(2), respectively with TD-OCT, and 2.03 (0.49) mm(2) and 1.75 (0.38) mm(2), respectively with SD-OCT. In ONHD and controls, repeatability of optic disc area measurement was excellent with fundus photography and optical coherence tomography (TD-OCT and SD-OCT), but with a low degree of agreement between both techniques. Optic disc area measurement is smaller in ONHD compared to healthy subjects with fundus photography, unlike time-domain and spectral-domain optical coherence tomography in which the reverse is true. Both techniques offer good repeatability, but a low degree of correlation and agreement, which means that optic disc area measurement is not interchangeable or comparable between techniques. Ophthalmic & Physiological Optics © 2013 The College of Optometrists.

  5. Somatostatin Receptor SPECT/CT using 99mTc Labeled HYNIC-TOC Aids in Diagnosis of Primary Optic Nerve Sheath Meningioma.

    Science.gov (United States)

    Chandra, Piyush; Purandare, Nilendu; Shah, Sneha; Agrawal, Archi; Rangarajan, Venkatesh

    2017-01-01

    Primary optic nerve sheath meningiomas (ONSM) are rare, benign and slow growing tumor involving the intra-orbital/intra-canalicular segment of the optic nerve. Untreated, they can potentially lead to visual deterioration. Magnetic resonance (MR) is the gold standard imaging modality for diagnosing the entity. Often, a clinical dilemma exists to narrow the differential diagnosis of an enhancing intra-orbital mass on MR. Molecular imaging provides a high degree of precision in diagnosing meningioma in view of relatively high levels of somatostatin receptor expression by these tumors. The following case demonstrates the potential clinical utility of somatostatin receptor SPECT using 99m Tc- labeled HYNIC-TOC in clinical diagnosis of ONSM.

  6. Digital versus film stereo-photography for assessment of the optic nerve head in glaucoma and glaucoma suspect patients.

    Science.gov (United States)

    Hasanreisoglu, Murat; Priel, Ethan; Naveh, Lili; Lusky, Moshe; Weinberger, Dov; Benjamini, Yoav; Gaton, Dan D

    2013-03-01

    One of the leading methods for optic nerve head assessment in glaucoma remains stereoscopic photography. This study compared conventional film and digital stereoscopy in the quantitative and qualitative assessment of the optic nerve head in glaucoma and glaucoma suspect patients. Fifty patients with glaucoma or suspected glaucoma underwent stereoscopic photography of the optic nerve head with a 35-mm color slide film and a digital camera. Photographs/images were presented in random order to 3 glaucoma specialists for independent analysis using a standardized assessment form. Findings for the following parameters were compared among assessors and between techniques: cup/disc (C/D) ratio, state of the optic rim, presence of peripapillary atrophy and appearance of the retinal nerve fiber layer, blood vessels, and lamina cribrosa. The film-based and image-based diagnoses (glaucoma yes/no) were compared as well. Despite high level of agreement across graders using the same method for the horizontal and vertical C/D ratio, (intraclass correlations 0.80 to 0.83), the agreement across graders was much lower for the other parameters using the same method. Similarly the agreement between the findings of the same grader using either method was high for horizontal and vertical C/D ratio, but low for the other parameters. The latter differences were reflected in the disagreement regarding the final diagnosis: The diagnoses differed by technique for each grader in 18% to 46% of eyes, resulting in 38.5% of eyes diagnosed with glaucoma by film photography that "lost" their diagnosis on the digital images, whereas 18.7% of eyes diagnosed as nonglaucomatous by film photography were considered to have glaucoma on the digital images. Although there is consistency between 35-mm film stereoscopy and digital stereoscopy in determining the cup/disc (C/D) ratio, in all other parameters large differences exist, leading to differences in diagnosis. Differences in capturing images between

  7. The Impact of Ocular Pressures, Material Properties and Geometry on Optic Nerve Head Deformation

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    Feola, Andrew J.; Myers, Jerry G.; Raykin, Julia; Nelson, Emily S.; Samuels, Brian C.; Ethier C. Ross

    2017-01-01

    Alteration in intracranial pressure (ICP) has been associated with various diseases that cause visual impairment, including glaucoma, idiopathic intracranial hypertension and Visual Impairment and Intracranial Pressure (VIIP) syndrome. However, how changes in ICP lead to vision loss is unclear, although it is hypothesized to involve deformations of the tissues in the optic nerve head (ONH). Recently, understanding the effect of ICP alterations on ocular tissues has become a major concern for NASA, where 42 of astronauts that partake in long duration space missions suffer from VIIP syndrome. Astronauts with VIIP syndrome suffer from visual impairment and changes in ocular anatomy that persist after returning to earth (1). It is hypothesized that the cephalad fluid shift that occurs upon entering microgravity increases ICP, which leads to an altered biomechanical environment in the posterior globe and optic nerve sheath, and subsequently VIIP syndrome. Our goal was to develop a finite element (FE) model to simulate the acute effects of elevated ICP on the posterior eye. Here, we simulated how inter-individual differences affect the deformation of ONH tissues. Further, we examined how several different geometries influenced deformations when exposed to elevated ICP.

  8. Optical coherence tomography in retinitis pigmentosa: reproducibility and capacity to detect macular and retinal nerve fiber layer thickness alterations.

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    Garcia-Martin, Elena; Pinilla, Isabel; Sancho, Eva; Almarcegui, Carmen; Dolz, Isabel; Rodriguez-Mena, Diego; Fuertes, Isabel; Cuenca, Nicolas

    2012-09-01

    To evaluate the ability of time-domain and Fourier-domain optical coherence tomographies (OCTs) to detect macular and retinal nerve fiber layer atrophies in retinitis pigmentosa (RP). To test the intrasession reproducibility using three OCT instruments (Stratus, Cirrus, and Spectralis). Eighty eyes of 80 subjects (40 RP patients and 40 healthy subjects) underwent a visual field examination, together with 3 macular scans and 3 optic disk evaluations by the same experienced examiner using 3 OCT instruments. Differences between healthy and RP eyes were compared. The relationship between measurements with each OCT instrument was evaluated. Repeatability was studied by intraclass correlation coefficients and coefficients of variation. Macular and retinal nerve fiber layer atrophies were detected in RP patients for all OCT parameters. Macular and retinal nerve fiber layer thicknesses, as determined by the different OCTs, were correlated but significantly different (P < 0.05). Reproducibility was moderately high using Stratus, good using Cirrus and Spectralis, and excellent using the Tru-track technology of Spectralis. In RP eyes, measurements showed higher variability compared with healthy eyes. Differences in thickness measurements existed between OCT instruments, despite there being a high degree of correlation. Fourier-domain OCT can be considered a valid and repeatability technique to detect retinal nerve fiber layer atrophy in RP patients.

  9. Retinal ganglion cell survival and axon regeneration after optic nerve injury in naked mole-rats.

    Science.gov (United States)

    Park, Kevin K; Luo, Xueting; Mooney, Skyler J; Yungher, Benjamin J; Belin, Stephane; Wang, Chen; Holmes, Melissa M; He, Zhigang

    2017-02-01

    In the adult mammalian central nervous system (CNS), axonal damage often triggers neuronal cell death and glial activation, with very limited spontaneous axon regeneration. In this study, we performed optic nerve injury in adult naked mole-rats, the longest living rodent, with a maximum life span exceeding 30 years, and found that injury responses in this species are quite distinct from those in other mammalian species. In contrast to what is seen in other mammals, the majority of injured retinal ganglion cells (RGCs) survive with relatively high spontaneous axon regeneration. Furthermore, injured RGCs display activated signal transducer and activator of transcription-3 (STAT3), whereas astrocytes in the optic nerve robustly occupy and fill the lesion area days after injury. These neuron-intrinsic and -extrinsic injury responses are reminiscent of those in "cold-blooded" animals, such as fish and amphibians, suggesting that the naked mole-rat is a powerful model for exploring the mechanisms of neuronal injury responses and axon regeneration in mammals. J. Comp. Neurol. 525:380-388, 2017. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  10. Ganglioglioma of the trigeminal nerve: MRI

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    Athale, S.; Jinkins, J.R. [Neuroradiology Section, The University of Texas Health Science Center at San Antonio, 7703 F. Curl Drive, San Antonio, TX 78284-7800 (United States); Hallet, K.K. [Neuropathology Department, The University of Texas Health Science Center at San Antonio, Texas (United States)

    1999-08-01

    Ganglioglioma of the cranial nerves is extremely rare; only a few cases involving the optic nerves have been reported. We present a case of ganglioglioma of the trigeminal nerve, which was isointense with the brain stem on all MRI sequences and showed no contrast enhancement. (orig.) With 2 figs., 6 refs.

  11. Evaluation of retinal nerve fiber layer thickness measurements using optical coherence tomography in patients with tobacco-alcohol-induced toxic optic neuropathy

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    Moura Frederico

    2010-01-01

    Full Text Available Three patients with progressive visual loss, chronic alcoholism and tabagism were submitted to a complete neuro-ophthalmic examination and to retinal nerve fiber layer (RNFL measurements using optical coherence tomography (OCT scanning. Two patients showed marked RNFL loss in the temporal sector of the optic disc. However, a third patient presented RNFL measurements within or above normal limits, based on the Stratus-OCT normative database. Such findings may be due to possible RNFL edema similar to the one that may occur in the acute phase of toxic optic neuropathies. Stratus-OCT was able to detect RNFL loss in the papillomacular bundle of patients with tobacco-alcohol-induced toxic optic neuropathy. However, interpretation must be careful when OCT does not show abnormality in order to prevent diagnostic confusion, since overestimation of RNFL thickness measurements is possible in such cases.

  12. Gene therapy for inherited retinal and optic nerve degenerations.

    Science.gov (United States)

    Moore, Nicholas A; Morral, Nuria; Ciulla, Thomas A; Bracha, Peter

    2018-01-01

    The eye is a target for investigational gene therapy due to the monogenic nature of many inherited retinal and optic nerve degenerations (IRD), its accessibility, tight blood-ocular barrier, the ability to non-invasively monitor for functional and anatomic outcomes, as well as its relative immune privileged state.Vectors currently used in IRD clinical trials include adeno-associated virus (AAV), small single-stranded DNA viruses, and lentivirus, RNA viruses of the retrovirus family. Both can transduce non-dividing cells, but AAV are non-integrating, while lentivirus integrate into the host cell genome, and have a larger transgene capacity. Areas covered: This review covers Leber's congenital amaurosis, choroideremia, retinitis pigmentosa, Usher syndrome, Stargardt disease, Leber's hereditary optic neuropathy, Achromatopsia, and X-linked retinoschisis. Expert opinion: Despite great potential, gene therapy for IRD raises many questions, including the potential for less invasive intravitreal versus subretinal delivery, efficacy, safety, and longevity of response, as well as acceptance of novel study endpoints by regulatory bodies, patients, clinicians, and payers. Also, ultimate adoption of gene therapy for IRD will require widespread genetic screening to identify and diagnose patients based on genotype instead of phenotype.

  13. Bone Marrow-Derived Cells as a Therapeutic Approach to Optic Nerve Diseases

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    Louise A. Mesentier-Louro

    2016-01-01

    Full Text Available Following optic nerve injury associated with acute or progressive diseases, retinal ganglion cells (RGCs of adult mammals degenerate and undergo apoptosis. These diseases have limited therapeutic options, due to the low inherent capacity of RGCs to regenerate and due to the inhibitory milieu of the central nervous system. Among the numerous treatment approaches investigated to stimulate neuronal survival and axonal extension, cell transplantation emerges as a promising option. This review focuses on cell therapies with bone marrow mononuclear cells and bone marrow-derived mesenchymal stem cells, which have shown positive therapeutic effects in animal models of optic neuropathies. Different aspects of available preclinical studies are analyzed, including cell distribution, potential doses, routes of administration, and mechanisms of action. Finally, published and ongoing clinical trials are summarized.

  14. Spectral domain optical coherence tomography cross-sectional image of optic nerve head during intraocular pressure elevation

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    Ji Young Lee

    2014-12-01

    Full Text Available AIM: To analyze changes of the optic nerve head (ONH and peripapillary region during intraocular pressure (IOP elevation in patients using spectral domain optical coherence tomography (SD-OCT.METHODS: Both an optic disc 200×200 cube scan and a high-definition 5-line raster scan were obtained from open angle glaucoma patients presented with monocular elevation of IOP (≥30 mm Hg using SD-OCT. Additional baseline characteristics included age, gender, diagnosis, best-corrected visual acuity, refractive error, findings of slit lamp biomicroscopy, findings of dilated stereoscopic examination of the ONH and fundus, IOP, pachymetry findings, and the results of visual field.RESULTS: The 24 patients were selected and divided into two groups:group 1 patients had no history of IOP elevation or glaucoma (n=14, and group 2 patients did have history of IOP elevation or glaucoma (n=10. In each patient, the study eye with elevated IOP was classified into group H (high, and the fellow eye was classified into group L (low. The mean deviation (MD differed significantly between groups H and L when all eyes were considered (P=0.047 and in group 2 (P=0.042, not in group 1 (P=0.893. Retinal nerve fiber layer (RNFL average thickness (P=0.050, rim area (P=0.015, vertical cup/disc ratio (P=0.011, cup volume (P=0.028, inferior quadrant RNFL thickness (P=0.017, and clock-hour (1, 5, and 6 RNFL thicknesses (P=0.050, 0.012, and 0.018, respectively, cup depth (P=0.008, central prelaminar layer thickness (P=0.023, mid-inferior prelaminar layer thickness (P=0.023, and nasal retinal slope (P=0.034 were significantly different between the eyes with groups H and L.CONCLUSION:RNFL average thickness, rim area, vertical cup/disc ratio, cup volume, inferior quadrant RNFL thickness, and clock-hour (1, 5, and 6 RNFL thicknesses significantly changed during acute IOP elevation.

  15. Does Laser Surgery Interfere with Optical Nerve Identification in Maxillofacial Hard and Soft Tissue?—An Experimental Ex Vivo Study

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    Bastian Bergauer

    2015-10-01

    Full Text Available The protection of sensitive structures (e.g., nerves from iatrogenic damage is of major importance when performing laser surgical procedures. Especially in the head and neck area both function and esthetics can be affected to a great extent. Despite its many benefits, the surgical utilization of a laser is therefore still limited to superficial tissue ablation. A remote feedback system which guides the laser in a tissue-specific way would provide a remedy. In this context, it has been shown that nerval structures can be specifically recognized by their optical diffuse reflectance spectra both before and after laser ablation. However, for a translation of these findings to the actual laser ablation process, a nerve protection within the laser pulse is of utmost significance. Thus, it was the aim of the study to evaluate, if the process of Er:YAG laser surgery—which comes with spray water cooling, angulation of the probe (60° and optical process emissions—interferes with optical tissue differentiation. For the first time, no stable conditions but the ongoing process of laser tissue ablation was examined. Therefore, six different tissue types (nerve, skin, muscle, fat, cortical and cancellous bone were acquired from 15 pig heads. Measurements were performed during Er:YAG laser ablation. Diffuse reflectance spectra (4500, wavelength range: 350–650 nm where acquired. Principal component analysis (PCA and quadratic discriminant analysis (QDA were calculated for classification purposes. The clinical highly relevant differentiation between nerve and bone was performed correctly with an AUC of 95.3% (cortial bone respectively 92.4% (cancellous bone. The identification of nerve tissue against the biological very similar fat tissue yielded good results with an AUC value of 83.4% (sensitivity: 72.3%, specificity: of 82.3%. This clearly demonstrates that nerve identification by diffuse reflectance spectroscopy works reliably in the ongoing process of

  16. High-resolution imaging of the retinal nerve fiber layer in normal eyes using adaptive optics scanning laser ophthalmoscopy.

    Science.gov (United States)

    Takayama, Kohei; Ooto, Sotaro; Hangai, Masanori; Arakawa, Naoko; Oshima, Susumu; Shibata, Naohisa; Hanebuchi, Masaaki; Inoue, Takashi; Yoshimura, Nagahisa

    2012-01-01

    To conduct high-resolution imaging of the retinal nerve fiber layer (RNFL) in normal eyes using adaptive optics scanning laser ophthalmoscopy (AO-SLO). AO-SLO images were obtained in 20 normal eyes at multiple locations in the posterior polar area and a circular path with a 3-4-mm diameter around the optic disc. For each eye, images focused on the RNFL were recorded and a montage of AO-SLO images was created. AO-SLO images for all eyes showed many hyperreflective bundles in the RNFL. Hyperreflective bundles above or below the fovea were seen in an arch from the temporal periphery on either side of a horizontal dividing line to the optic disc. The dark lines among the hyperreflective bundles were narrower around the optic disc compared with those in the temporal raphe. The hyperreflective bundles corresponded with the direction of the striations on SLO red-free images. The resolution and contrast of the bundles were much higher in AO-SLO images than in red-free fundus photography or SLO red-free images. The mean hyperreflective bundle width around the optic disc had a double-humped shape; the bundles at the temporal and nasal sides of the optic disc were narrower than those above and below the optic disc (Poptical coherence tomography correlated with the hyperreflective bundle widths on AO-SLO (Pfiber bundles and Müller cell septa. The widths of the nerve fiber bundles appear to be proportional to the RNFL thickness at equivalent distances from the optic disc.

  17. Nervus terminalis, olfactory nerve, and optic nerve representation of luteinizing hormone-releasing hormone in primates.

    Science.gov (United States)

    Witkin, J W

    1987-01-01

    The luteinizing hormone-releasing hormone (LHRH) system was examined immunocytochemically in olfactory bulbs of adult monkeys, including two New World species (squirrel monkey, Saimiri sciureus and owl monkey, Aotus trivirgatus) and one Old World species (cynomolgus macaque, Macaca fasciculata), and in the brain and nasal region of a fetal rhesus macaque Macaca mulatta. LHRH neurons and fibers were found sparsely distributed in the olfactory bulbs in all adult monkeys. There was more LHRH in the accessory olfactory bulb (which is absent in Old World monkeys). In the fetal macaque there was a rich distribution of LHRH neurons and fibers along the pathway of the nervus terminalis, anterior and ventral to the olfactory bulb, and in the nasal septum, with fibers branching into the olfactory epithelium. In addition, there were LHRH neurons and fibers in the optic nerve.

  18. Analysis of the enamel hypoplasia using micro-CT scanner versus classical method.

    Science.gov (United States)

    Marchewka, Justyna; Skrzat, Janusz; Wróbel, Andrzej

    2014-01-01

    This article demonstrates the use of micro-CT scanning of the teeth surface for recognizing and evaluating severity of the enamel hypoplasia. To test capabilities of the microtomography versus classical method of evaluation hypoplastic defects of the enamel we selected two human teeth (C, M(2)) showing different types of enamel hypoplasia: linear, pits, and groove. Examined samples derive from archeological material dated on XVII-XVIII AD and excavated in Poland. In the current study we proved that micro-CT scanning is a powerful technique not only for imaging all kinds of the enamel hypoplasia but also allows to perform accurate measurements of the enamel defects. We figure out that contrary to the classical method of scoring enamel defects, the micro-computed tomography yields adequate data which serve for estimating the length of stress episode and length of interval between them.

  19. Short term reproducibility of a high contrast 3-D isotropic optic nerve imaging sequence in healthy controls

    Science.gov (United States)

    Harrigan, Robert L.; Smith, Alex K.; Mawn, Louise A.; Smith, Seth A.; Landman, Bennett A.

    2016-03-01

    The optic nerve (ON) plays a crucial role in human vision transporting all visual information from the retina to the brain for higher order processing. There are many diseases that affect the ON structure such as optic neuritis, anterior ischemic optic neuropathy and multiple sclerosis. Because the ON is the sole pathway for visual information from the retina to areas of higher level processing, measures of ON damage have been shown to correlate well with visual deficits. Increased intracranial pressure has been shown to correlate with the size of the cerebrospinal fluid (CSF) surrounding the ON. These measures are generally taken at an arbitrary point along the nerve and do not account for changes along the length of the ON. We propose a high contrast and high-resolution 3-D acquired isotropic imaging sequence optimized for ON imaging. We have acquired scan-rescan data using the optimized sequence and a current standard of care protocol for 10 subjects. We show that this sequence has superior contrast-to-noise ratio to the current standard of care while achieving a factor of 11 higher resolution. We apply a previously published automatic pipeline to segment the ON and CSF sheath and measure the size of each individually. We show that these measures of ON size have lower short- term reproducibility than the population variance and the variability along the length of the nerve. We find that the proposed imaging protocol is (1) useful in detecting population differences and local changes and (2) a promising tool for investigating biomarkers related to structural changes of the ON.

  20. Bilateral optic nerve head drusen with chorioretinal coloboma in the right eye

    Directory of Open Access Journals (Sweden)

    Ali Reza Dehghani

    2016-01-01

    Full Text Available Chorioretinal coloboma is a congenital defect of the eye caused by improper closure of the embryonic fissure. Optic nerve head drusen (ONHD are white calcareous deposits that are generally asymptomatic. We report a very rare association of both in a healthy patient with no any systemic syndrome. A 16-year-old man was referred to our clinic from suffering blurred vision. Best corrected visual acuity of the right eye was 6/10 and 10/10 in the left one. External ocular and slit lamp examination were normal. Dilated ophthalmoscopy showed marked swelling in both optic nerves and chorioretinal coloboma in the right eye inferiorly. Ultrasonography showed an echodense structure with acoustic shadowing in both eyes consistent with buried ONHD. Visual field testing showed normal field in the left eye and moderate superior field depression in the right eye corresponding to inferior coloboma in funduscopy. Results of general medical and neurologic, cardiologic, and other examinations were normal. To the best our knowledge combination of bilateral ONHD and unilateral chorioretinal coloboma in a healthy patient with no any systemic syndrome has not been published in the literature. We reported this very rare association and recommended examine eyes and other body organs. In such cases that coloboma is associated with ONHD, we should keep in mind Noonan syndrome. The diagnosis of Noonan syndrome is clinical and confirm by the consultant pediatricians and clinical geneticists.

  1. Rhabdomyolysis in pontocerebellar hypoplasia type 2 (PCH-2)

    NARCIS (Netherlands)

    Barth, Peter G.; Ryan, Monique M.; Webster, Richard I.; Aronica, Eleonora; Kan, Alex; Ramkema, Marja; Jardine, Philip; Poll-The, Bwee Tien

    2008-01-01

    Pontocerebellar hypoplasia type 2, an autosomal recessive neurodegeneration with prenatal onset, is characterised by progressive microcephaly and chorea/dystonia and has not previously been associated with muscular involvement. The gene associated with PCH-2 is unknown. An episode of rhabdomyolysis

  2. Variations in retinal nerve fiber layer measurements on optical coherence tomography after implantation of trifocal intraocular lens.

    Science.gov (United States)

    García-Bella, Javier; Martínez de la Casa, José M; Talavero González, Paula; Fernández-Vigo, José I; Valcarce Rial, Laura; García-Feijóo, Julián

    2018-01-01

    To establish the changes produced after implantation of a trifocal intraocular lens (IOL) on retinal nerve fiber layer measurements performed with Fourier-domain optical coherence tomography (OCT). This prospective study included 100 eyes of 50 patients with bilateral cataract in surgical range, no other associated ocular involvement, refractive errors between +5 and -5 spherical diopters, and less than 1.5 D of corneal astigmatism. The eyes were operated by phacoemulsification with implantation of 2 different trifocal IOLs (FineVision and AT LISA tri 839MP) in randomized equal groups. Cirrus OCT and Spectralis OCT were performed before surgery and 3 months later. Both analyzed the thickness of the nerve fiber layer and thickness divided by quadrants (6 in case of Spectralis and 4 in case of Cirrus HD). The mean age of patients was 67.5 ± 5.8 years. The global nerve fiber layer thickness measured with Spectralis OCT was 96.77 μm before surgery and 99.55 μm after. With Cirrus OCT, the global thickness was 85.29 μm before surgery and 89.77 μm after. Statistically significant differences in global thickness measurements between preimplantation and postimplantation of the IOL were found with both OCT in the 2 groups. Statistically significant differences were also found in temporal and superior quadrants. The implantation of a diffractive trifocal IOL alters the results of the optic nerve fiber layer on Fourier-domain OCT in these patients, which should be taken into account in the posterior study of these patients.

  3. Ametropia, retinal anatomy, and OCT abnormality patterns in glaucoma. 2. Impacts of optic nerve head parameters

    Science.gov (United States)

    Baniasadi, Neda; Wang, Mengyu; Wang, Hui; Jin, Qingying; Elze, Tobias

    2017-12-01

    Clinicians use retinal nerve fiber layer thickness (RNFLT) measured by optical coherence tomography (OCT) as an adjunct to glaucoma diagnosis. Ametropia is accompanied by changes to the optic nerve head (ONH), which may affect how OCT machines mark RNFLT measurements as abnormal. These changes in abnormality patterns may bias glaucoma diagnosis. Here, we investigate the relationship between OCT abnormality patterns and the following ONH-related and ametropia-associated parameters on 421 eyes of glaucoma patients: optic disc tilt and torsion, central retinal vessel trunk location (CRVTL), and nasal and temporal retinal curvature adjacent to ONH, quantified as nasal/temporal slopes of the inner limiting membrane. We applied multivariate logistic regression with abnormality marks as regressands to 40,401 locations of the peripapillary region and generated spatial maps of locations of false positive/negative abnormality marks independent of glaucoma severity. Effects of torsion and temporal slope were negligible. The effect of tilt could be explained by covariation with ametropia. For CRVTL/nasal slope, abnormality pattern shifts at 7.2%/23.5% of the peripapillary region were detected, respectively, independent of glaucoma severity and ametropia. Therefore, CRVTL and nasal curvature should be included in OCT RNFLT norms. Our spatial location maps may aid clinicians to improve diagnostic accuracy.

  4. Neuromuscular ultrasound of cranial nerves.

    Science.gov (United States)

    Tawfik, Eman A; Walker, Francis O; Cartwright, Michael S

    2015-04-01

    Ultrasound of cranial nerves is a novel subdomain of neuromuscular ultrasound (NMUS) which may provide additional value in the assessment of cranial nerves in different neuromuscular disorders. Whilst NMUS of peripheral nerves has been studied, NMUS of cranial nerves is considered in its initial stage of research, thus, there is a need to summarize the research results achieved to date. Detailed scanning protocols, which assist in mastery of the techniques, are briefly mentioned in the few reference textbooks available in the field. This review article focuses on ultrasound scanning techniques of the 4 accessible cranial nerves: optic, facial, vagus and spinal accessory nerves. The relevant literatures and potential future applications are discussed.

  5. Intertooth patterns of hypoplasia expression: implications for childhood health in the classic Maya collapse.

    Science.gov (United States)

    Wright, L E

    1997-02-01

    Enamel hypoplasias, which record interacting stresses of nutrition and illness during the period of tooth formation, are a key tool in the study of childhood health in prehistory. But interpretation of the age of peak morbidity is complicated by differences in susceptibility to stress both between tooth positions and within a single tooth. Here, hypoplasias are used to evaluate the prevailing ecological model for the collapse of Classic Period Lowland Maya civilization, circa AD 900. Hypoplasias were recorded in the full dentition of 160 adult skeletons from six archaeological sites in the Pasion River region of Guatemala. Instead of constructing a composite scale of stress experience, teeth are considered separately by position in the analysis. No statistical differences are found in the proportion of teeth affected by hypoplasia between "Early," Late Classic, and Terminal Classic Periods for anterior teeth considered to be most susceptible to stress, indicating stability in the overall stress loads affecting children of the three chronological periods. However, hypoplasia trends in posterior teeth may imply a change in the ontogenetic-timing of more severe stress episodes during the final occupation and perhaps herald a shift in child-care practices. These results provide little support for the ecological model of collapse but do call to attention the potential of posterior teeth to reveal subtle changes in childhood morbidity when consideredindividually.

  6. Optic nerve tolerance to single and fractionated radiation simulating radiosurgery: a rabbit model using visual evoked potentials, fundoscopy and histology

    International Nuclear Information System (INIS)

    Bastin, Kenneth; Mehta, Minesh

    1997-01-01

    Purpose/Objective: To develop a rabbit model enabling single or fractionated optic nerve irradiation, measure post-irradiation visual evoked potentials (VEP), fundoscopic and histopathologic changes, and relate different radiation dosing fractions to these measurable physiologic changes. Materials and Methods: Forty male New Zealand white rabbits underwent surgical right orbital prolapse with template-guided optic nerve irradiation using an iridium-192 high dose rate afterloader. Rabbits were randomized into single fraction groups (0 (control), 10, 12.5, 15, 20, and 30 Gy (3 per group); or two fraction groups of 0 (control) 5, 7.5, 10, 15, and 20 Gy (3 per group); or three fractions groups of 10 and 15 Gy (2 per group). Bilateral fundoscopy and pattern-reversal VEPs (0.5 and 1 c/deg, 1 hertz) were performed at 6 and 12 months (mos) following scheduled irradiation. VEP peaks (P1) were measured. Sacrifice and necropsy followed 12 month evaluation, allowing for histological changes. Results: Excluding deaths from anesthesia (2), CNS mite infection (2), sepsis, pyothorax, 'undetermined' and technically non-analyzable VEP recordings, 24 complete rabbit data sets were evaluated. Fundoscopy demonstrated no gross changes at any dose. Histopathology demonstrated generalized optic nerve atrophy without radiation dose correlation. Among single fraction groups, VEP showed a 6 mos post-irradiation P1 prolongation only in the 20 and 30 Gy groups (maximum 67%). At 12 mos lower dose single fraction groups had a prolonged P1 peak. All fractionated groups above 5 Gy x 2 had P1 prolongation times at 6 mos (maximum 46% in the 15 Gy x 3 data set) but by 12 mos these groups had non-measurable, deteriorated VEPs. Correlating VEP P1 latency with the calculated linear quadratic formula (LQM) biologically equivalent dose (BED,α/β=3) for each group demonstrated a general correlation (t-Test P<.001) as shown: Conclusion: Using a rabbit model for selective optic nerve irradiation we conclude

  7. Edaravone Prevents Retinal Degeneration in Adult Mice Following Optic Nerve Injury.

    Science.gov (United States)

    Akiyama, Goichi; Azuchi, Yuriko; Guo, Xiaoli; Noro, Takahiko; Kimura, Atsuko; Harada, Chikako; Namekata, Kazuhiko; Harada, Takayuki

    2017-09-01

    To assess the therapeutic potential of edaravone, a free radical scavenger that is used for the treatment of acute brain infarction and amyotrophic lateral sclerosis, in a mouse model of optic nerve injury (ONI). Two microliters of edaravone (7.2 mM) or vehicle were injected intraocularly 3 minutes after ONI. Optical coherence tomography, retrograde labeling of retinal ganglion cells (RGCs), histopathology, and immunohistochemical analyses of phosphorylated apoptosis signal-regulating kinase-1 (ASK1) and p38 mitogen-activated protein kinase (MAPK) in the retina were performed after ONI. Reactive oxygen species (ROS) levels were assessed with a CellROX Green Reagent. Edaravone ameliorated ONI-induced ROS production, RGC death, and inner retinal degeneration. Also, activation of the ASK1-p38 MAPK pathway that induces RGC death following ONI was suppressed with edaravone treatment. The results of this study suggest that intraocular administration of edaravone may be a useful treatment for posttraumatic complications.

  8. Anatomic relationship of the optic nerve channel with sphenoid al sinus: a computed tomography study; Relacao anatomica do nervo optico com o seio esfenoidal: estudo por tomografia computadorizada

    Energy Technology Data Exchange (ETDEWEB)

    Dias, Paulo Cesar J. [Universidade Federal de Sao Paulo (UNIFESP/EPM), SP (Brazil); Albernaz, Pedro Luiz M. [Universidade Federal de Sao Paulo (UNIFESP/EPM), SP (Brazil). Dept. de Otorrinolaringologia e Disturbios da Comunicacao Humana; Yamashida, Helio K. [Universidade Federal de Sao Paulo (UNIFESP/EPM), SP (Brazil). Dept. de Diagnostico por Imagem

    2004-09-15

    The sphenoid sinus, out of the facial sinuses, is certainly the most neglected as far as diagnosis is concerned. The surgical approach requires a detailed anatomical knowledge, considering the serious complications resulting from injuries of vital structures adjacent to this region. Aim: the objective of our research is to evaluate the anatomic relationship of the optic nerve with the sphenoid sinus making use of the computed tomography. Study design: series report. Material and method: the authors present a retrospective analysis of 203 computed tomographies of facial sinus belonging to individuals of both sexes aged 14 and over. The examinations were evaluated observing the course of the optic nerve, obtained through the degree of its projection on the wall of the sphenoid sinus. The method used was the modified classification of Delano, evaluating the absence of bone reduction (dehiscence) of the optic nerve in the sphenoid sinus. We analysed the degree of pneumatization of the sphenoid sinus, using Hammer's classification adapted by Guerrero, apart from the pneumatization of the anterior clinoidal process and pterigoid and the presence of the Onodi cell. Results: most of the patients (78.96%) presented their optic nerve with a type 1 course. Type 2 was observed in 16.83% of the patients, type 3 in 3.47% and type 4 in 0.74%. The presence of dehiscence of the optic nerve in the wall of the sphenoid sinus was evidenced in 21.29% of the cases. Related to pneumatization, we noticed that the pre-selar type was observed in 6.44%, the post-selar type appeared in 39.11%, the selar type appeared in 54.45%, and the apneumatized type was not observed in any of the cases. The pneumatization of the anterior clinoidal process was verified in 10.64% of the cases, while the pterigoid process was apparent in 21.29% of the cases. The Onodi cell (sphenoetmoidal) was found in 7.92% of the cases. Conclusion: the presence of dehiscence of the optic nerve is related with the

  9. Radiation therapy in and about the retina, optic nerve, and anterior visual pathway: psychophysical assessment

    International Nuclear Information System (INIS)

    Fitzgerald, C.R.; Enoch, J.M.; Temme, L.A.

    1981-01-01

    Visual changes may develop in patients receiving radiation therapy for malignant neoplasms in and about the optic nerve and anterior visual pathway. Cases have been studied using a series of psychophysical tests, including kinetic perimetry, increment threshold determinations, Flashing Repeat Static Test, and sustained- and transient-like functions. A characteristic time-dependent reduction in sensitivity has been identified in these patients. This finding, in addition to the presence of nerve fiber bundle defects, appears to place the pathologic changes in the axon of the ganglion cell posterior to the lamina cribrosa. Any change in the sustained- and transient-like functions, the organization of which appears to be in the neural retina, was seen only if a concomitant radiation retinopathy was identified

  10. Radiation therapy in and about the retina, optic nerve, and anterior visual pathway. Psychophysical assessment

    International Nuclear Information System (INIS)

    Fitzgerald, C.R.; Enoch, J.M.; Temme, L.A.

    1981-01-01

    Visual changes may develop in patients receiving radiation therapy for malignant neoplasms in and about the optic nerve and anterior visual pathway. Cases have been studied using a series of psychophysical tests, including kinetic perimetry, increment threshold determinations, Flashing Repeat Static Test, and sustained- and transient-like functions. A characteristic time-dependent reduction in sensitivity has been identified in these patients. This finding, in addition to the presence of nerve fiber bundle defects, appears to place the pathologic changes in the axon of the ganglion cell posterior to the lamina cribrosa. Any change in the sustained- and transient-like functions, the organization of which appears to be in the neural retina, was seen only if a concomitant radiation retinopathy was identified

  11. Causes of congenital unilateral pulmonary hypoplasia

    International Nuclear Information System (INIS)

    Currarino, G.; Williams, B.; Children's Medical Center, Dallas, TX

    1985-01-01

    A review of the roentgenograms and clinical records of 33 children with primary congenital underdevelopment of one lung showed that 9 patients had simple pulmonary hypoplasia, 8 had anomalous venous return to the right atrium or the inferior vena cava (scimitar syndrome), 7 had an absence of ipsilateral pulmonary artery, 7 had an accessory diaphragm, and 2 had a pulmonary sequestration adjacent to a small diaphragmatic hernia. (orig.)

  12. Use of repeat anterior maxillary distraction to correct residual midface hypoplasia in cleft patients.

    Science.gov (United States)

    Richardson, Sunil; Krishna, Shreya; Bansal, Avi

    2017-12-01

    The study was designed to evaluate the efficacy of performing a second, repeat anterior maxillary distraction (AMD) to treat residual cleft maxillary hypoplasia. Five patients between the ages of 12 to 15 years with a history of AMD and with residual cleft maxillary hypoplasia were included in the study. Inclusion was irrespective of gender, type of cleft lip and palate, and the amount of advancement needed. Repeat AMD was executed in these patients 4 to 5 years after the primary AMD procedure to correct the cleft maxillary hypoplasia that had developed since the initial procedure. Orthopantomogram (OPG) and lateral cephalograms were taken for evaluation preoperatively, immediately after distraction, after consolidation, and one year postoperatively. The data obtained was tabulated and a Mann Whitney U-test was used for statistical comparisons. At the time of presentation, a residual maxillary hypoplasia was observed with a well maintained distraction gap on the OPG which ruled out the occurrence of a relapse. Favorable movement of the segments without any resistance was seen in all patients. Mean maxillary advancement of 10.56 mm was achieved at repeat AMD. Statistically significant increases in midfacial length, SNA angle, and nasion perpendicular to point A distance was achieved ( P =0.012, P =0.011, and P =0.012, respectively). Good profile was achieved for all patients. Minimal transient complications, for example anterior open bite and bleeding episodes, were managed. Addressing the problem of cleft maxillary hypoplasia at an early age (12-15 years) is beneficial for the child. Residual hypoplasia may develop in some patients, which may require additional corrective procedures. The results of our study show that AMD can be repeated when residual deformity develops with the previous procedure having no negative impact on the results of the repeat procedure.

  13. Enamel hypoplasia and its role in identification of individuals: A review of literature

    Science.gov (United States)

    Kanchan, Tanuj; Machado, Meghna; Rao, Ashwin; Krishan, Kewal; Garg, Arun K.

    2015-01-01

    Identification of individuals is the mainstay of any forensic investigation especially in cases of mass disasters when mutilated remains are brought for examination. Dental examination helps in establishing the identity of an individual and thus, has played a vital role in forensic investigation process since long. In this regard, description on the role of enamel hypoplasia is limited in the literature. The present article reviews the literature on the enamel hypoplasia and discusses its utility in forensic identification. Enamel hypoplasia is a surface defect of the tooth crown caused by disturbance of enamel matrix secretion. Enamel defects can be congenital or acquired. In cases of mass disasters, or when the body is completely charred, putrefied and mutilated beyond recognition, the unique dental features can help in identification of the victims. PMID:26097340

  14. Diffuse reflectance spectroscopy for optical nerve identification. Preliminary ex vivo results for feedback controlled oral and maxillofacial laser surgery

    Science.gov (United States)

    Stelzle, Florian; Zam, Azhar; Adler, Werner; Douplik, Alexandre; Tangermann-Gerk, Katja; Nkenke, Emeka; Neukam, Friedrich Wilhelm; Schmidt, Michael

    Objective: Laser surgery has many advantages. However, due to a lack of haptic feedback it is accompanied by the risk of iatrogenic nerve damage. The aim of this study was to evaluate the possibilities of optical nerve identification by diffuse reflectance spectroscopy to set the base for a feedback control system to enhance nerve preservation in oral and maxillofacial laser surgery. Materials and Methods: Diffuse reflectance spectra of nerve tissue, skin, mucosa, fat tissue, muscle, cartilage and bone (15120 spectra) of ex vivo pig heads were acquired in the wavelength range of 350-650 nm. Tissue differentiation was performed by principal components analysis (PCA) followed by linear discriminant analysis (LDA). Specificity and sensitivity were calculated by receiver operating characteristic (ROC) analysis and the area under curve (AUC). Results: Nerve tissue could correctly be identified and differed from skin, mucosa, fat tissue, muscle, cartilage and bone in more than 90% of the cases (AUC results) with a specificity of over 78% and a sensitivity of more than 86%. Conclusion: Nerve tissue can be identified by diffuse reflectance spectroscopy with high precision and reliability. The results may set the base for a feedback system to prevent iatrogenic nerve damage performing oral and maxillofacial laser surgery.

  15. Ultrastructure of the extracellular matrix of bovine dura mater, optic nerve sheath and sclera.

    OpenAIRE

    Raspanti, M; Marchini, M; Della Pasqua, V; Strocchi, R; Ruggeri, A

    1992-01-01

    The sclera, the outermost sheath of the optic nerve and the dura mater have been investigated histologically and ultrastructurally. Although these tissues appear very similar under the light microscope, being dense connective tissues mainly composed of collagen bundles and a limited amount of cells and elastic fibres, they exhibit subtle differences on electron microscopy. In the dura and sclera collagen appears in the form of large, nonuniform fibrils, similar to those commonly found in tend...

  16. Septo-optic dysplasia plus: clinical presentation and magnetic resonance imaging findings

    International Nuclear Information System (INIS)

    Matushita Junior, Joao Paulo K.; Tiel, Chan; Py, Marco; Batista, Raquel Ribeiro; Gasparetto, Emerson L.

    2010-01-01

    Septo-optic dysplasia (SOD) is a heterogeneous developmental malformation characterized by optic nerve hypoplasia associated with dysgenesis of the septum pellucidum and other cerebral malformations. The clinical manifestations include psychomotor retardation, visual impairment, thermoregulatory disturbances, conjugated hyperbilirubinemia and seizures. In 2000, Miller et al. first named the association of SOD and cortical dysplasia as SOD-plus. In this report, all the cases had severe clinical impairment, presenting global developmental delay and spastic motor deficits. Subsequent reports of SOD-plus also stressed the psychomotor development delay, spastic motor deficits and seizures seen in these patients, emphasizing the severity of the brain involvement. Recently, Kwak et al. reported a case of SOD-plus presenting with cortical dysplasia involving the insular cortex bilaterally. However, differently from all the previous reports, the patient had no signs or symptoms of cortical dysfunction, except for one episode of seizure. We report an additional case of SOD-plus, which presented extensive cortical malformation, with no signs or symptoms of cortical dysfunction. We suggest that similar to the classical form of SOD, the clinical presentation in patients with SOD-plus can also range from mild to extremely severe. (author)

  17. Septo-optic dysplasia plus: clinical presentation and magnetic resonance imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Matushita Junior, Joao Paulo K. [Instituto de Pos-Graduacao Medica Carlos Chagas (IPGMCC), Rio de Janeiro, RJ (Brazil); Tiel, Chan; Py, Marco [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Inst. de Neurologia Deolindo Couto; Batista, Raquel Ribeiro [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Hospital Universitario Clementino Fraga Filho; Gasparetto, Emerson L., E-mail: egasparetto@gmail.co [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Dept. de Radiologia

    2010-04-15

    Septo-optic dysplasia (SOD) is a heterogeneous developmental malformation characterized by optic nerve hypoplasia associated with dysgenesis of the septum pellucidum and other cerebral malformations. The clinical manifestations include psychomotor retardation, visual impairment, thermoregulatory disturbances, conjugated hyperbilirubinemia and seizures. In 2000, Miller et al. first named the association of SOD and cortical dysplasia as SOD-plus. In this report, all the cases had severe clinical impairment, presenting global developmental delay and spastic motor deficits. Subsequent reports of SOD-plus also stressed the psychomotor development delay, spastic motor deficits and seizures seen in these patients, emphasizing the severity of the brain involvement. Recently, Kwak et al. reported a case of SOD-plus presenting with cortical dysplasia involving the insular cortex bilaterally. However, differently from all the previous reports, the patient had no signs or symptoms of cortical dysfunction, except for one episode of seizure. We report an additional case of SOD-plus, which presented extensive cortical malformation, with no signs or symptoms of cortical dysfunction. We suggest that similar to the classical form of SOD, the clinical presentation in patients with SOD-plus can also range from mild to extremely severe. (author)

  18. A novel PLP1 mutation associated with optic nerve enlargement in two siblings with Pelizaeus-Merzbacher disease: A new MRI finding.

    Science.gov (United States)

    Pavlidou, Efterpi; Ramachandran, Vijaya; Govender, Veronica; Wilson, Clare; Das, Rini; Vlachou, Victoria; Pavlou, Evangelos; Saggar, Anand; Mankad, Kshitij; Kinali, Maria

    2017-03-01

    Pelizaeus-Merzbacher disease (PMD) is a rare, X-linked disorder characterized by hypomyelination of the Central Nervous System due to mutations in the PLP1 gene. Certain mutations of the PLP1 gene correlate with specific clinical phenotypes and neuroimaging findings. We herein report a novel mutation of the PLP1 gene in two siblings with PMD associated with a rare and protean neuroimaging finding of optic nerve enlargement. To the best of our knowledge this is the first time that this novel mutation H133P of PLP1 gene is identified and clinically associated with optic nerve enlargement in PMD patients. Copyright © 2016 The Japanese Society of Child Neurology. All rights reserved.

  19. Cerebellar Hypoplasia and Dysmorphia in Neurofibromatosis Type 1.

    Science.gov (United States)

    Toelle, Sandra P; Poretti, Andrea; Weber, Peter; Seute, Tatjana; Bromberg, Jacoline E C; Scheer, Ianina; Boltshauser, Eugen

    2015-12-01

    Unidentified bright objects (UBO) and tumors are well-known cerebellar abnormalities in neurofibromatosis type 1 (NF1). Literature reports on malformative cerebellar anomalies in neurofibromatosis type 1 (NF1), however, are scant. We retrospectively studied the clinical and neuroimaging findings of 5 patients with NF1 (4 females, age 6 to 29 years at last follow-up) and cerebellar anomalies. Cerebellar symptoms on neurological examination were mild or even not evident whereas learning disabilities were more or less pronounced in four patients. Two patients had cerebellar hypoplasia (diffusely enlarged cerebellar interfoliar spaces) and three cerebellar dysmorphias involving mainly one cerebellar hemisphere. In NF1, malformative cerebellar anomalies are rare (estimated prevalence of about 1%), but most likely underestimated and easily overlooked, because physicians tend to focus on more prevalent, obvious, and well-known findings such as optic pathway gliomas, other tumors, and UBO. This kind of cerebellar anomaly in NF1 has most likely a malformative origin, but the exact pathogenesis is unknown. The individual clinical significance is difficult to determine. We suggest that cerebellar anomalies should be systematically evaluated in neuroimaging studies of NF1 patients.

  20. The effect of the menstrual cycle on the optic nerve head analysis of migrainous women.

    Science.gov (United States)

    Yucel, Iclal; Akar, Munire; Durukan, A; Akar, Yusuf; Taskin, Omur; Dora, Babur; Yilmaz, Nurgul

    2005-03-01

    To determine the effect of the menstrual cycle on the optic nerve head topographic analysis of normally menstruating migrainous women. Randomly selected one eye of 44 migrainous and 49 healthy control women with regular menstrual cycles were included in the study. All subjects underwent complete ocular examination. Optic nervehead topographic analysis were performed using a confocal scanning laser ophthalmoscope, HRT II (Heidelberg Retinal Tomograph II, software version 1.6;Heidelberg Engineering, Heidelberg, Germany). They were repeated for two times during the menstrual cycle: in follicular phase (7th to 10th day of the cycle) and in the luteal phase (days 3 to 4 before the menstrual bleeding). Serum estradiol, progesterone, and luteinizing hormone measurements were repeated at each menstrual phase. The mean age of migrainous and control subjects were 31.5 + 5.1 years and 33.4 +/- 3.7 years, respectively (P > 0.05). Their mean disc areas were 2.26 +/- 0.46 mm(2) and 1.95 +/- 0.39 mm(2), respectively(P 0.05). The parameter rim volume decreased, while the parameters cup volume and cup shape measure increased significantly in the luteal phase of the migrainous women (all P values cup parameters during the menstrual cycle of the migrainous women. Further clinical trials on ocular blood flow changes during the menstrual cycle of the migrainous women may highlight the role of sex steroids in the optic nerve head of the migrainous women.

  1. Diffusion tensor imaging of occult injury of optic radiation following optic neuritis in multiple sclerosis.

    Science.gov (United States)

    Chen, Jiafeng; Zhu, Lijun; Li, He; Lu, Ziwen; Chen, Xin; Fang, Shaokuan

    2016-10-01

    Multiple sclerosis (MS) is easily detected by routine magnetic resonance imaging (MRI). However, it is not possible to detect early or occult lesions in MS by routine MRI, and this may explain the inconsistency between the severity of the lesions found by MRI and the degree of clinical disability of patients with MS. The present study included 10 patients with relapsing-remitting MS and 10 healthy volunteers. Each patient underwent routine 3.0 T MRI, diffusion tensor imaging (DTI), and diffusion tensor tractography (DTT). Optic nerve and optic radiation were analyzed by DTI and DTT. The fractional anisotropy (FA), mean diffusivity (MD), λ // , and λ ┴ values were measured. In the 10 patients with MS, 7 optic nerves were affected, and 13 optic nerves were not affected. Cranial MRI showed that optic nerve thickening and hyperintensity occurred in 2 patients with MS. In the directionally encoded color maps, a hypointensive green signal in the optic nerve was observed in 3 patients with MS. The FA values were significantly lower and the MD, λ // , and λ ┴ values were significantly higher in the affected and unaffected optic nerves and optic radiations in patients with MS in comparison with controls (P0.05). Diffusion tensor imaging is sensitive in the detection of occult injury of the optic nerve and optic radiation following optic neuritis. Diffusion tensor imaging may be a useful tool for the early diagnosis, treatment and management of MS.

  2. Focal dermal hypoplasia: A rare case report

    Directory of Open Access Journals (Sweden)

    Sahana M Srinivas

    2015-01-01

    Full Text Available Focal dermal hypoplasia (Goltz syndrome is a rare genetic multisystem disorder primarily involving the skin, skeletal system, eyes, and face. We report the case of an eight-month-old female child who presented with multiple hypopigmented atrophic macules along the lines of blaschko, skeletal anomalies, umbilical hernia, developmental delay, hypoplastic nails, syndactyly, and lobster claw deformity characteristic of Goltz syndrome.

  3. Rat optic nerve head anatomy within 3D histomorphometric reconstructions of normal control eyes.

    Science.gov (United States)

    Pazos, Marta; Yang, Hongli; Gardiner, Stuart K; Cepurna, William O; Johnson, Elaine C; Morrison, John C; Burgoyne, Claude F

    2015-10-01

    The purpose of this study is to three-dimensionally (3D) characterize the principal macroscopic and microscopic relationships within the rat optic nerve head (ONH) and quantify them in normal control eyes. Perfusion-fixed, trephinated ONH from 8 normal control eyes of 8 Brown Norway Rats were 3D histomorphometrically reconstructed, visualized, delineated and parameterized. The rat ONH consists of 2 scleral openings, (a superior neurovascular and inferior arterial) separated by a thin connective tissue strip we have termed the "scleral sling". Within the superior opening, the nerve abuts a prominent extension of Bruch's Membrane (BM) superiorly and is surrounded by a vascular plexus, as it passes through the sclera, that is a continuous from the choroid into and through the dural sheath and contains the central retinal vein (CRV), (inferiorly). The inferior scleral opening contains the central retinal artery and three long posterior ciliary arteries which obliquely pass through the sclera to obtain the choroid. Bruch's Membrane Opening (BMO) is irregular and vertically elongated, enclosing the nerve (superiorly) and CRV and CRA (inferiorly). Overall mean BMO Depth, BMO Area, Choroidal Thickness and peripapillary Scleral Thickness were 29 μm, 56.5 × 10(3) μm(2), 57 μm and 104 μm respectively. Mean anterior scleral canal opening (ASCO) and posterior scleral canal opening (PSCO) radii were 201 ± 15 μm and 204 ± 16 μm, respectively. Mean optic nerve area at the ASCO and PSCO were 46.3 × 10(3)±4.4 × 10(3) μm(2) and 44.1 × 10(3)±4.5 × 10(3) μm(2) respectively. In conclusion, the 3D complexity of the rat ONH and the extent to which it differs from the primate have been under-appreciated within previous 2D studies. Properly understood, these anatomic differences may provide new insights into the relative susceptibilities of the rat and primate ONH to elevated intraocular pressure. Copyright © 2015 Elsevier Ltd. All rights reserved.

  4. Prenatal evaluation of the middle ear and diagnosis of middle ear hypoplasia using MRI

    Energy Technology Data Exchange (ETDEWEB)

    Katorza, Eldad; Nahama-Allouche, Catherine; Ducou le Pointe, Hubert; Garel, Catherine [Hopital d' Enfants Armand-Trousseau, Service de Radiologie, Paris (France); Castaigne, Vanina [Hopital Saint-Antoine, Service de Gynecologie-Obstetrique, Paris (France); Gonzales, Marie; Marlin, Sandrine [Hopital d' Enfants Armand-Trousseau, Service de Genetique et Embryologie medicales, Paris (France); Galliani, Eva [Hopital d' Enfants Armand-Trousseau, Service de Chirurgie maxillo-faciale, Paris (France); Jouannic, Jean-Marie; Rosenblatt, Jonathan [Hopital d' Enfants Armand-Trousseau, Service de Gynecologie-Obstetrique, Centre pluridisciplinaire de diagnostic prenatal, Paris (France)

    2011-05-15

    Analysis of the middle ear with fetal MRI has not been previously reported. To show the contribution of fetal MRI to middle ear imaging. The tympanic cavity was evaluated in 108 fetal cerebral MRI examinations (facial and/or cerebral malformation excluded) and in two cases, one of Treacher Collins syndrome (case 1) and the other of oculo-auriculo-vertebral (OUV) spectrum (case 2) with middle ear hypoplasia identified by MRI at 27 and 36 weeks' gestation, respectively. In all 108 fetuses (mean gestational age 32.5 weeks), the tympanic cavity and T2 hypointensity related to the ossicles were well visualised on both sides. Case 1 had micro/retrognathia and bilateral external ear deformity and case 2 had retrognathism with a left low-set and deformed ear. MRI made it possible to recognize the marked hypoplasia of the tympanic cavity, which was bilateral in case 1 and unilateral in case 2. Both syndromes are characterized by craniofacial abnormalities including middle ear hypoplasia, which cannot be diagnosed with US. The middle ear cavity can be visualized with fetal MRI. We emphasize the use of this imaging modality in the diagnosis of middle ear hypoplasia. (orig.)

  5. Malformation of the eighth cranial nerve in children.

    Science.gov (United States)

    de Paula-Vernetta, Carlos; Muñoz-Fernández, Noelia; Mas-Estellés, Fernando; Guzmán-Calvete, Abel; Cavallé-Garrido, Laura; Morera-Pérez, Constantino

    2016-01-01

    Prevalence of congenital sensorineural hearing loss (SNHL) is approximately 1.5-6 in every 1,000 newborns. Dysfunction of the auditory nerve (auditory neuropathy) may be involved in up to 1%-10% of cases; hearing losses because of vestibulocochlear nerve (VCN) aplasia are less frequent. The objectives of this study were to describe clinical manifestations, hearing thresholds and aetiology of children with SNHL and VCN aplasia. We present 34 children (mean age 20 months) with auditory nerve malformation and profound HL taken from a sample of 385 children implanted in a 10-year period. We studied demographic characteristics, hearing, genetics, risk factors and associated malformations (Casselman's and Sennaroglu's classifications). Data were processed using a bivariate descriptive statistical analysis (P<.05). Of all the cases, 58.8% were bilateral (IIa/IIa and I/I were the most common). Of the unilateral cases, IIb was the most frequent. Auditory screening showed a sensitivity of 77.4%. A relationship among bilateral cases and systemic pathology was observed. We found a statistically significant difference when comparing hearing loss impairment and patients with different types of aplasia as defined by Casselman's classification. Computed tomography (CT) scan yielded a sensitivity of 46.3% and a specificity of 85.7%. However, magnetic resonance imaging (MRI) was the most sensitive imaging test. Ten percent of the children in a cochlear implant study had aplasia or hypoplasia of the auditory nerve. The degree of auditory loss was directly related to the different types of aplasia (Casselman's classification) Although CT scan and MRI are complementary, the MRI is the test of choice for detecting auditory nerve malformation. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

  6. Rapid diagnosis of retina and optic nerve abnormalities in canine patients with and without cataracts using chromatic pupil light reflex testing.

    Science.gov (United States)

    Grozdanic, Sinisa D; Kecova, Helga; Lazic, Tatjana

    2013-09-01

    To develop fast and reliable testing routines for diagnosing retina and optic nerve diseases in canine cataract patients based on chromatic properties of the pupillary light reflex response. Seventy-seven canine patients with a history of cataract and decreased vision (43 patients with cataracts and no evidence of retina or optic nerve disease, 21 patients with cataracts and retinal degeneration [RD], 13 patients with cataracts and retinal detachment [RDT]), 11 canine patients with optic neuritis (ON) and 23 healthy dogs were examined using chromatic pupillary light reflex (cPLR) analysis with red and blue light and electroretinography. Electroretinography analysis showed statistically significant deficits in a- and b-wave amplitudes in dogs with cataracts and RD, or cataracts and RDT, when compared to dogs with cataracts without evidence of retinal abnormalities. Evaluation of b-wave amplitudes showed that presence of 78.5-μV (or lower) amplitudes had high sensitivity of 100% (95% CI: 87.2-100%) and high specificity of 96.7% (95% CI: 88.4-100%) in RD and RDT. Evaluation of cPLR responses using red light showed that presence of the pupil end constriction diameter of 5.5 mm (or higher) had moderately high sensitivity of 76.5% (95% CI: 50.1-93.2%) and high specificity of 100% (95% CI: 91.2-100%) in detecting RD and RDT. Optic neuritis patients had absent cPLR responses, regardless of the visual status. Chromatic evaluation of the pupillary light reflex is a rapid and accurate test for diagnosing retina and optic nerve diseases in canine patients. © 2012 American College of Veterinary Ophthalmologists.

  7. Hypoplasia of the thumb. Clinical presentation and reconstruction

    International Nuclear Information System (INIS)

    Vergara A, Enrique M

    2008-01-01

    In the genesis of the partial or total absence of the thumb they are genetic, environmental factors or a combination of both. It is take part of a syndrome or to be isolated and frequently associated with problems of radial longitudinal deficiency of the forearm. Objective. The purpose of this study is shown the experience, the focus of the processing and the results obtained since the point esthetic and functional view. The most it accepted classification is the proposal by Blauth that helps to determine the forecast and the processing. Materials and methods. it is a work type series of cases in 22 children with hypoplasia of the thumb, with a minimum of 12 months, (average 28 months). In 15 cases there were association of radial dysplasia or another anomaly among them 4 patients with VATER, and the 7 remaining they corresponded to hypoplasia of the thumb as only entity. We carried out tendon transfer, with opening of the first comisure in 2 patients with hypoplasia type II. In 3 patients, with hypoplasia type III A, one carries out corner opening, transfer of the superficial flexor of the 4 finger to correct instability of the articulation MF and opposition of the thumb, and transfer for extension of the thumb. In 17 cases one carries out politicization of the index. Results. The outcome was evaluated in: non pinch, lateral pinch and fingertip pinch; the grade of opposition like good, minimal and non opposition, and the aesthetic result according to the satisfaction of the parents in bad, regular and good. The five children reconstructed with transfers of tendons and comisure opening had good result. In 17 children with politicization one patient had a necrosis, of the 16 remaining a good or acceptable result was obtained. Discussion. It is not easy to follow a good system of measure of the functional results. We find that a practical way to evaluate was the clip, opposition and aesthetics. Previous to the surgeries it is required to evaluate alterations

  8. Optic nerve compression as a late complication of a hydrogel explant with silicone encircling band.

    Science.gov (United States)

    Crama, Niels; Kluijtmans, Leo; Klevering, B Jeroen

    2018-06-01

    To present a complication of compressive optic neuropathy caused by a swollen hydrogel explant and posteriorly displaced silicone encircling band. A 72-year-old female patient presented with progressive visual loss and a tilted optic disc. Her medical history included a retinal detachment in 1993 that was treated with a hydrogel explant under a solid silicone encircling band. Visual acuity had decreased from 6/10 to 6/20 and perimetry showed a scotoma in the temporal superior quadrant. On Magnetic Resonance Imaging (MRI), compression of the optic nerve by a displaced silicone encircling band inferior nasally in combination with a swollen episcleral hydrogel explant was observed. Surgical removal of the hydrogel explant and silicone encircling band was uneventful and resulted in improvement of visual acuity and visual field loss. This is the first report on compressive optic neuropathy caused by swelling of a hydrogel explant resulting in a dislocated silicone encircling band. The loss of visual function resolved upon removal of the explant and encircling band.

  9. [Pulmonary hypoplasia: An analysis of cases over a 20-year period].

    Science.gov (United States)

    Delgado-Peña, Yanny Paola; Torrent-Vernetta, Alba; Sacoto, Gabriela; de Mir-Messa, Inés; Rovira-Amigo, Sandra; Gartner, Silvia; Moreno-Galdó, Antonio; Molino-Gahete, José Andrés; Castillo-Salinas, Felíx

    2016-08-01

    Pulmonary hypoplasia is the most frequent congenital anomaly associated with perinatal mortality. A retrospective and descriptive review was conducted on cases of patients diagnosed with pulmonary hypoplasia between 1995 and 2014 in a tertiary university hospital. An analysis was made of the prenatal imaging, clinical manifestations, post-natal diagnostic tests, treatment and management, long-term follow up, and survival data. A total of 60 cases were identified, all of them with prenatal imaging. Sixteen patients required foetal surgery. Congenital diaphragmatic hernia was the most frequent diagnosis. Main clinical presentation was respiratory distress with severe hypoxemia and high requirements of mechanical ventilation. Mortality rate was 47% within first 60 days of life, and 75% for the first day of life. Pneumonia and recurrent bronchitis episodes were observed during follow-up. They had a lung function obstructive pattern, and their quality of life and exercise tolerance was good. High neonatal mortality and significant long-term morbidity associated with pulmonary hypoplasia requires an early diagnosis and a specialised multidisciplinary team management. Copyright © 2015 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  10. Effects of high-intensity interval training on optic nerve head and macular perfusion using optical coherence tomography angiography in healthy adults.

    Science.gov (United States)

    Schmitz, Boris; Nelis, Pieter; Rolfes, Florian; Alnawaiseh, Maged; Klose, Andreas; Krüger, Michael; Eter, Nicole; Brand, Stefan-Martin; Alten, Florian

    2018-07-01

    High-intensity interval training (HIIT) has been identified to be efficient for increasing health-related fitness in general and in lifestyle-induced chronic diseases such as hypertension, obesity and metabolic syndrome. This study aimed to evaluate HIIT effects on optic nerve head (ONH) and macular perfusion in healthy adults using optical coherence tomography angiography (OCTA). Fifty-eight healthy participants (22.0 ± 2.02 years, 40 females (69.0%)) performed a 4-week HIIT with two exercise sessions/week: Group 1, 4 × 30 HIIT, running at maximal speed (all-out) for 4 × 30 s with 30 s active recovery, Group 2, 8 × 15 HIIT, running at maximal speed (all-out) for 8 × 15 s with 15 s active recovery. OCTA of the ONH and the macula was performed at baseline and follow-up to detect changes of the foveal avascular zone (FAZ). Flow density was evaluated in the superficial and deep plexus of the central macula, in the radial peripapillary capillary layer, the nerve head layer of the disc region and of the peripapillary region. The mean deep FAZ area and flow density of the superficial layer decreased by 14.00 ± 13.02% and 1.26 ± 3.20%, respectively, in response to overall HIIT (pre vs. post p HIIT may be performed to induce changes in ophthalmic measures such as FAZ and nerve head perfusion. OCTA imaging of the central retina and the ONH could represent a sensitive tool for the early detection of systemic vascular changes. Copyright © 2018 Elsevier B.V. All rights reserved.

  11. Practical Considerations for Optic Nerve Estimation in Telemedicine

    Energy Technology Data Exchange (ETDEWEB)

    Karnowski, Thomas Paul [ORNL; Aykac, Deniz [ORNL; Chaum, Edward [ORNL; Giancardo, Luca [ORNL; Li, Yaquin [University of Tennessee, Knoxville (UTK); Tobin Jr, Kenneth William [ORNL

    2009-01-01

    The projected increase in diabetes in the United States and worldwide has created a need for broad-based, inexpensive screening for diabetic retinopathy (DR), an eye disease which can lead to vision impairment. A telemedicine network with retina cameras and automated quality control, physiological feature location, and lesion / anomaly detection is a low-cost way of achieving broad-based screening. In this work we report on the effect of quality estimation on an optic nerve (ON) detection method with a confidence metric. We report on an improvement of the fusion technique using a data set from an ophthalmologists practice then show the results of the method as a function of image quality on a set of images from an on-line telemedicine network collected in Spring 2009 and another broad-based screening program. We show that the fusion method, combined with quality estimation processing, can improve detection performance and also provide a method for utilizing a physician-in-the-loop for images that may exceed the capabilities of automated processing.

  12. Perspectives of optic nerve prostheses.

    Science.gov (United States)

    Lane, Frank John; Nitsch, Kristian; Huyck, Margaret; Troyk, Philip; Schug, Ken

    2016-01-01

    A number of projects exist that are investigating the ability to restore visual percepts for individuals who are blind through a visual prosthesis. While many projects have reported the results from a technical basis, very little exists in the professional literature on the human experience of visual implant technology. The current study uses an ethnographic methodological approach to document the experiences of the research participants and study personnel of a optic nerve vision prosthesis project in Brussels, Belgium. The findings have implications for motivation for participating in clinical trials, ethical safeguards of participants and the role of the participant in a research study. Implications for Rehabilitation Rehabilitation practitioners are often solicited by prospective participants to assist in evaluating a clinical trial before making a decision about participation. Rehabilitation professionals should be aware that: The decision to participate in a clinical trial is ultimately up to the individual participant. However, participants should be aware that family members might experience stress from of a lack of knowledge about the research study. The more opportunities a participant has to share thoughts and feelings about the research study with investigators will likely result in a positive overall experience. Ethical safeguards put in place to protect the interests of an individual participant may have the opposite effect and create stress. Rehabilitation professionals can play an important role as participant advocates from recruitment through termination of the research study. Participant hope is an important component of participation in a research study. Information provided to participants by investigators during the consent process should be balanced carefully with potential benefits, so it does not destroy a participant's hope.

  13. Nonthermal ablation with microbubble-enhanced focused ultrasound close to the optic tract without affecting nerve function.

    Science.gov (United States)

    McDannold, Nathan; Zhang, Yong-Zhi; Power, Chanikarn; Jolesz, Ferenc; Vykhodtseva, Natalia

    2013-11-01

    Tumors at the skull base are challenging for both resection and radiosurgery given the presence of critical adjacent structures, such as cranial nerves, blood vessels, and brainstem. Magnetic resonance imaging-guided thermal ablation via laser or other methods has been evaluated as a minimally invasive alternative to these techniques in the brain. Focused ultrasound (FUS) offers a noninvasive method of thermal ablation; however, skull heating limits currently available technology to ablation at regions distant from the skull bone. Here, the authors evaluated a method that circumvents this problem by combining the FUS exposures with injected microbubble-based ultrasound contrast agent. These microbubbles concentrate the ultrasound-induced effects on the vasculature, enabling an ablation method that does not cause significant heating of the brain or skull. In 29 rats, a 525-kHz FUS transducer was used to ablate tissue structures at the skull base that were centered on or adjacent to the optic tract or chiasm. Low-intensity, low-duty-cycle ultrasound exposures (sonications) were applied for 5 minutes after intravenous injection of an ultrasound contrast agent (Definity, Lantheus Medical Imaging Inc.). Using histological analysis and visual evoked potential (VEP) measurements, the authors determined whether structural or functional damage was induced in the optic tract or chiasm. Overall, while the sonications produced a well-defined lesion in the gray matter targets, the adjacent tract and chiasm had comparatively little or no damage. No significant changes (p > 0.05) were found in the magnitude or latency of the VEP recordings, either immediately after sonication or at later times up to 4 weeks after sonication, and no delayed effects were evident in the histological features of the optic nerve and retina. This technique, which selectively targets the intravascular microbubbles, appears to be a promising method of noninvasively producing sharply demarcated lesions in

  14. Sensory stimulation for lowering intraocular pressure, improving blood flow to the optic nerve and neuroprotection in primary open-angle glaucoma.

    Science.gov (United States)

    Rom, Edith

    2013-12-01

    Primary open-angle glaucoma is a group of optic neuropathies that can lead to irreversible blindness. Sensory stimulation in the form of acupuncture or ear acupressure may contribute to protecting patients from blindness when used as a complementary method to orthodox treatment in the form of drops, laser or surgery. The objective of this article is to provide a narrative overview of the available literature up to July 2012. It summarises reported evidence on the potential beneficial effects of sensory stimulation for glaucoma. Sensory stimulation appears to significantly enhance the pressure-lowering effect of orthodox treatments. Studies suggest that it may also improve blood flow to the eye and optic nerve head. Furthermore, it may play a role in neuroprotection through regulating nerve growth factor and brain-derived neurotrophic factor and their receptors, thereby encouraging the survival pathway in contrast to the pathway to apoptosis. Blood flow and neuroprotection are areas that are not directly influenced by orthodox treatment modalities. Numerous different treatment protocols were used to investigate the effect of sensory stimulation on intraocular pressure, blood flow or neuroprotection of the retina and optic nerve in the animal model and human pilot studies. Objective outcomes were reported to have been evaluated with Goldmann tonometry, Doppler ultrasound techniques and electrophysiology (pattern electroretinography, visually evoked potentials), and supported with histological studies in the animal model. Taken together, reported evidence from these studies strongly suggests that sensory stimulation is worthy of further research.

  15. Early Methylprednisolone Treatment Can Stabilize the Blood-Optic Nerve Barrier in a Rat Model of Anterior Ischemic Optic Neuropathy (rAION).

    Science.gov (United States)

    Huang, Tzu-Lun; Wen, Yao-Tseng; Chang, Chung-Hsing; Chang, Shu-Wen; Lin, Kuan-Hung; Tsai, Rong-Kung

    2017-03-01

    We investigated whether methylprednisolone (MP) treatment halting retinal ganglion cell (RGC) death and having anti-inflammatory effect over a narrow therapeutic window affects the integrity of the blood-optic nerve barrier (BOB) in a rat model of ischemic optic neuropathy (rAION). The optic nerve (ON) vascular permeability was determined by Evans blue extravasation. Changes in the levels of TNF-α and IL-1β cytokines were analyzed using quantitative RT-PCR (qRT-PCR) from day 1 to day 5 post-rAION. Rats were treated with MP starting on days 0, 1, 2, and 7 post-rAION. The survival and apoptosis of the RGCs were determined by fluoroGold labeling and TUNEL assay, and the visual function was assessed with flash visual-evoked potentials (FVEPs) 4 weeks postinfarct. Inflammation of the ON was detected by immunohistochemical staining of ED1. Macrophage recruitment in the ON was significantly reduced, which was compatible with the reduction in ON vascular permeability, after MP treatment starting on days 0 and 1 postinsult compared to PBS treatment (both, P < 0.05). There was significant reduction in TNF-α and IL-1β expression in MP-treated rats (all, P < 0.05). The survival number and antiapoptotic effect on RGCs, and the P1-N2 FVEP amplitude significantly improved with MP treatment starting on days 0 and 1 (all, P < 0.05). Early treatment with MP halts RGC death and mitigates macrophage infiltration with decreased expression of proinflammatory cytokines in acute rAION. The very narrow therapeutic window is related to the quick stabilization of the disrupted BOB by early application of MP.

  16. Mechanisms of cadmium-caused eye hypoplasia and hypopigmentation in zebrafish embryos

    International Nuclear Information System (INIS)

    Zhang, Ting; Zhou, Xin-Ying; Ma, Xu-Fa; Liu, Jing-Xia

    2015-01-01

    Highlights: Using high-throughput in situ hybridization screening, we found that genes labeling the neural crest and its derivative pigment cells were sensitive to cadmium toxicity during zebrafish organogenesis, which might contribute to the molecular mechanisms underlying the phenotype defects of head and eye hypoplasia and hypopigmentation in cadmium-exposed embryos. Based on neural crest markers, we identified the doses and times of cadmium exposure that cause damage to the zebrafish organogenesis, and we also found that compounds BIO or RA could neutralize the toxic effects of cadmium. - Abstract: Cadmium-caused head and eye hypoplasia and hypopigmentation has been recognized for a long time, but knowledge of the underlying mechanisms is limited. In this study, we found that high mortality occurred in exposed embryos after 24 hpf, when cadmium (Cd) dosage was above 17.8 μM. Using high-throughput in situ hybridization screening, we found that genes labelling the neural crest and its derivative pigment cells exhibited obviously reduced expression in Cd-exposed embryos from 24 hpf, 2 days earlier than head and eye hypoplasia and hypopigmentation occurred. Moreover, based on expression of crestin, a neural crest marker, we found that embryos before the gastrula stage were more sensitive to cadmium toxicity and that damage caused by Cd on embryogenesis was dosage dependent. In addition, by phenotype observation and detection of neural crest and pigment cell markers, we found that BIO and retinoic acid (RA) could neutralize the toxic effects of Cd on zebrafish embryogenesis. In this study, we first determined that Cd blocked the formation of the neural crest and inhibited specification of pigment cells, which might contribute to the molecular mechanisms underlying the phenotype defects of head and eye hypoplasia and hypopigmentation in Cd-exposed embryos. Moreover, we found that compounds BIO or RA could neutralize the toxic effects of Cd.

  17. Mechanisms of cadmium-caused eye hypoplasia and hypopigmentation in zebrafish embryos

    Energy Technology Data Exchange (ETDEWEB)

    Zhang, Ting, E-mail: zting@webmail.hzau.edu.cn; Zhou, Xin-Ying, E-mail: 290356082@qq.com; Ma, Xu-Fa, E-mail: xufama@mail.hzau.edu.cn; Liu, Jing-Xia, E-mail: ichliu@mail.hzau.edu.cn

    2015-10-15

    Highlights: Using high-throughput in situ hybridization screening, we found that genes labeling the neural crest and its derivative pigment cells were sensitive to cadmium toxicity during zebrafish organogenesis, which might contribute to the molecular mechanisms underlying the phenotype defects of head and eye hypoplasia and hypopigmentation in cadmium-exposed embryos. Based on neural crest markers, we identified the doses and times of cadmium exposure that cause damage to the zebrafish organogenesis, and we also found that compounds BIO or RA could neutralize the toxic effects of cadmium. - Abstract: Cadmium-caused head and eye hypoplasia and hypopigmentation has been recognized for a long time, but knowledge of the underlying mechanisms is limited. In this study, we found that high mortality occurred in exposed embryos after 24 hpf, when cadmium (Cd) dosage was above 17.8 μM. Using high-throughput in situ hybridization screening, we found that genes labelling the neural crest and its derivative pigment cells exhibited obviously reduced expression in Cd-exposed embryos from 24 hpf, 2 days earlier than head and eye hypoplasia and hypopigmentation occurred. Moreover, based on expression of crestin, a neural crest marker, we found that embryos before the gastrula stage were more sensitive to cadmium toxicity and that damage caused by Cd on embryogenesis was dosage dependent. In addition, by phenotype observation and detection of neural crest and pigment cell markers, we found that BIO and retinoic acid (RA) could neutralize the toxic effects of Cd on zebrafish embryogenesis. In this study, we first determined that Cd blocked the formation of the neural crest and inhibited specification of pigment cells, which might contribute to the molecular mechanisms underlying the phenotype defects of head and eye hypoplasia and hypopigmentation in Cd-exposed embryos. Moreover, we found that compounds BIO or RA could neutralize the toxic effects of Cd.

  18. Findings of Optical Coherence Tomography of Retinal Nerve Fiber Layer in Two Common Types of Multiple Sclerosis

    Directory of Open Access Journals (Sweden)

    Gholamali Yousefipour

    2016-06-01

    Full Text Available Multiple sclerosis (MS is the most prevalent disease caused by the inflammatory demyelinating process that causes progressive nervous system degeneration over the time. Optical Coherence Tomography (OCT is a non-invasive optical imaging technology, which can measure the thickness of retinal nerve fiber layer as well as the diameter of the macula. The purpose of the study is evaluation OCT findings in two common types of multiple sclerosis. For doing the cross-sectional study, 63 patients with two prevalent types of multiple sclerosis (35 patients with Relapse Remitting Multiple Sclerosis (RRMS and 28 patients with Secondary Progressive Multiple Sclerosis (SPMS were evaluated for 6 months. Exclusion criteria of the study were a history of optic neuritis, suffering from diabetes mellitus, hypertension, ocular disease, and the presence of other neurologic degenerative diseases. Then, the thickness of retinal nerve fiber layer (RNFL, as well as thickness and volume of the macula, were measured in the patients using OCT technology. The disability rate of patients was evaluated according to Expanded Disability Status Scale (EDSS. Finally, data was analyzed by means of SPSS software. Overall, 35 patients with RRMS (with mean age of 32.37+10.01, average disease period of 3.81+3.42 and mean EDSS of 1.84+0.45 and 28 patients with SPMS (with mean age of 39.21+9.33, average disease period of 11.32+5.87 and mean EDSS of 5.12+1.46 were assessed and compared in terms of retinal nerve fiber layer and size and thickness of macula. In all of these sections, the thicknesses were smaller in SPMS patients than patients with RRMS. But, there was a significant difference in total thickness (81.82µm versus 96.03µm with P=0.04 and thickness of temporal sector (54.5 µm versus 69.34 µm with P=0.04 of retinal nerve fiber layer and macular size at the superior sector of external ring (1.48 mm³ versus 1.58 mm³ with P=0.03, and nasal sector of external ring surrounding

  19. Are All Retinal Nerve Fiber Layer Defects on Optic Coherence Tomography Glaucomatous?

    Science.gov (United States)

    Gür Güngör, Sirel; Ahmet, Akman

    2017-10-01

    In this study, we investigated the patients who were referred to our clinic with a prediagnosis of glaucoma based on retinal nerve fiber layer (RNFL) defects on optic coherence tomography (OCT) but were determined to have nonglaucomatous RNLF defects upon detailed examination. The ophthalmic examination notes, OCT images, Heidelberg retinal tomography (HRT) II and fundus photographs of 357 patients were retrospectively evaluated. Final diagnoses of these patients were investigated. Of the 357 patients, 216 (60.5%) were diagnosed as open angle glaucoma, 33 (9.2%) as low-tension glaucoma, 39 (10.9%) as pre-perimetric glaucoma. The ophthalmic examinations of 14 patients (3.9%) were normal and there were no RNFL defects in OCT examinations after dilatation. In 39 patients (10.9%), the ophthalmic and optic disc examinations were completely normal and no etiologic factor explaining RNFL defects was found. Twenty-two eyes of 16 patients (4.5%) were included in this study (the mean age was 53.8±11.5 years; 9 men and 7 women). After detailed questioning of the medical history and systemic and neurologic examinations, a diagnosis of ischemic optic neuropathy was made in 11 eyes (10 patients) (2.8%), optic neuritis in 3 eyes (2 patients) (0.6%), optic disc drusen in 4 eyes (2 patients) (0.6%), pseudotumor cerebri in 2 eyes (1 patient) (0.3%), and cerebral palsy in 2 eyes (1 patient) (0.3%). Decrease in RNFL thickness on OCT images alone may be misleading in glaucoma examination. In cases where optic disc cupping is not evident, diagnosis should not be based on OCT RNFL examinations alone, and the patient's medical history, detailed ophthalmic examination, OCT optic disc parameters, HRT, and visual field tests should all be carefully evaluated together.

  20. Can sonographic measurement of optic nerve sheath diameter be used to detect raised intracranial pressure in patients with tuberculous meningitis? A prospective observational study

    International Nuclear Information System (INIS)

    Sangani, Shruti V; Parikh, Samira

    2015-01-01

    CNS Tuberculosis can manifest as meningitis, arachnoiditis and a tuberculoma. The rupture of a tubercle into the subarachnoid space leads to Tuberculosis Meningitis (TBME); the resulting hypersensitivity reaction can lead to an elevation of the intracranial pressure and hydrocephalus. While bedside optic nerve sheath diameter (ONSD) ultrasonography (USG) can be a sensitive screening test for elevated intracranial pressure in adult head injury, little is known regarding ONSD measurements in Tuberculosis Meningitis. The aim of this study was to determine whether patients with TBME had dilation of the optic nerve sheath, as detected by ocular USG performed in the emergency department (ED). We conducted a prospective, observational study on adult ED patients with suspected TBME. Patients underwent USG measurements of the optic nerve followed by MRI. The ONSD was measured 3 mm behind the globe in each eye. MRI evidence of basilar meningeal enhancement and any degree of hydrocephalus was suggestive of TBME. Those patients without evidence of hydrocephalus subsequently underwent a lumbar puncture to confirm the diagnosis. Exclusion criteria were age less than 18 and obvious ocular pathology. In total, the optic nerve sheath diameters of 25 adults with confirmed TBME were measured. These measurements were compared with 120 control patients. The upper limit of normal ONSD was 4.37 mm in control group. Those patients with TBME had a mean ONSD of 5.81 mm (SD 0.42). These results confirm that patients with tuberculosis meningitis have an ONSD in excess of the control data (P < 0.001). The evaluation of the ONSD is a simple non-invasive and potentially useful tool in the assessment of adults suspected of having TBME

  1. A Case of Fatal Pulmonary Hypoplasia with Congenital Diaphragmatic Hernia, Thoracic Myelomeningocele, and Thoracic Dysplasia.

    Science.gov (United States)

    Ito, Ai; Fujinaga, Hideshi; Matsui, Sachiko; Tago, Kumiko; Iwasaki, Yuka; Fujino, Shuhei; Nagasawa, Junko; Amari, Shoichiro; Kaneshige, Masao; Wada, Yuka; Takahashi, Shigehiro; Tsukamoto, Keiko; Miyazaki, Osamu; Yoshioka, Takako; Ishiguro, Akira; Ito, Yushi

    2017-10-01

    Background  Congenital diaphragmatic hernia (CDH) is fatal in severe cases of pulmonary hypoplasia. We experienced a fatal case of pulmonary hypoplasia due to CDH, thoracic myelomeningocele (MMC), and thoracic dysplasia. This constellation of anomalies has not been previously reported. Case Report  A male infant with a prenatal diagnosis of thoracic MMC with severe hydrocephalus and scoliosis was born at 36 weeks of gestation. CDH was found after birth and the patient died of respiratory failure due to pulmonary hypoplasia and persistent pulmonary hypertension of the newborn at 30 hours of age despite neonatal intensive care. An autopsy revealed a left CDH without herniation of the liver or stomach into the thoracic cavity, severe hydrocephalus, Chiari malformation type II, MMC with spina bifida from Th4 to Th12, hemivertebrae, fused ribs, deformities of the thoracic cage and legs, short trunk, and agenesis of the left kidney. Conclusion  We speculate that two factors may be associated with the severe pulmonary hypoplasia: decreased thoracic space due to the herniation of visceral organs caused by CDH and thoracic dysplasia due to skeletal deformity and severe scoliosis.

  2. Radioinduced optic nerve neuropathy after treatment for nasopharynx cancer. About two cases and literature review

    International Nuclear Information System (INIS)

    Mnejja, W.; Siala, W.; Daoud, J.; Fki, J.; Ghorbel, M.; Frikha, M.

    2007-01-01

    The radioinduced neuropathy of the optic nerve is a rare and delayed complication. Its incidence is difficult to evaluate in the literature. It depends on the whole irradiation dose, fractionation and irradiated volume. Currently, it does not exist any efficient treatment, only the prevention play an important part on avoiding the high doses, and the broad irradiation volumes. The innovating techniques using conformal radiotherapy with or without modulated intensity could contribute to reduce this toxicity incidence. (N.C.)

  3. Prenatal and neonatal variables associated with enamel hypoplasia in deciduous teeth in low birth weight preterm infants

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    Kátia Maria Dmytraczenko Franco

    2007-12-01

    Full Text Available This study investigated possible prenatal and neonatal variables that may influence the prevalence of tooth enamel hypoplasia in preterm and low birth weight children (LBW and a matched control group of term children with normal birth weight (NBW. The study sample consisted of 61 children born preterm and with LBW examined at 18-34 months of age. The control group was formed by 61 infants born full term and with NBW examined at 31-35 months of age. All children were born at the Center of Integrated Attention of Women's Health (CAISM-UNICAMP. FDI criteria were followed for dental examination. Medical data was collected retrospectively from hospital records. Among preterms, 57.4% had some type of developmental defects of enamel (DDE, 52.5 % had opacities and 21.3 % presented hypoplasia. Among full-term children, 24.6% presented DDE, 24.6% had opacities and 3.3% had hypoplasia. LBW preterm infants presented a higher prevalence of hypoplasia than NBW controls. The deciduous teeth most affected by hypoplasia were maxillary incisors. There was no significant association with prenatal variables; among neonatal variables there was a significant association with respiratory distress syndrome and neurological examination at discharge with an altered result.

  4. Hypoplasia of bone marrow and cytopenia in non-hemic diseases

    International Nuclear Information System (INIS)

    Turbina, N.S.

    1987-01-01

    Common (stereotype) and different links in pathogenesis of hemodepressions pronounced in the form of cytopenia and/or hypoplasia of hemopoiesis are considered. Pathogenesis and signs of the diseases, as well as their diagnosis, are studied in detail

  5. Preliminary MRI study in patients with congenital complex strabismus

    International Nuclear Information System (INIS)

    Man Fengyuan; Wang Zhenchang; Zhao Bo; Zhu Lei; Xian Junfang; Wang Shijun; Jiao Yonghong; Wu Xiao; Zhao Kanxing

    2009-01-01

    Objective: Although the ocular motility examination has been traditionally used in the diagnosis of complex strabismus resulting from cranial nerve (CN) and extraocular muscles (EOM) abnormalities, magnetic resonance imaging (MRI) now permits the direct imaging of lesions in CNs and EOMs. Methods: Twenty-six patients with complex strabismus underwent MRI examination on 1.5 T MR unit (Twinspeed, GE). Nerves to EOMs were imaged with T 1 weighted in orbits in all patients using phase array surface coils. Results: Patients with Duane syndrome (15 cases, 19 eyes) all exhibited absence or hypoplasia of abducens nerve (CN6), always with mild hypoplasia and apparent misdirection of oculomotor nerve (CN3) to the lateral rectus muscle in the orbit, and there were no hypoplasia of EOMs. Patients with congenital fibrosis of EOMs (9 cases, 16 eyes) exhibited severe hypoplasia of CN3 and CN6, and EOMs appeared hypoptasia to a different degree, particularly severe for the superior rectus and levated palpted muscles. Multiple nerves displayed aplasia in patients with Mobius syndrome (1 ease, 2 eyes) and there was abnormal branch from CN3 to lateral rectus. The tendons of bilateral superior oblique muscles were thin in the patients with Brown syndrome (1 case, 2 eyes). Conclusion: MRI can directly demonstrate absence or hypoplasia of CNs and corresponding EOM abnormalities in congenital complex strabismus, which suggests that the mechanism of congenital complex strabismus is perhaps abnormal innervation or displasia of the ocular motor nerves. (authors)

  6. X-linked congenital adrenal hypoplasia associated with hypospadias in an Egyptian baby: a case report

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    Metwalley Kotb

    2012-12-01

    Full Text Available Abstract Introduction X-linked congenital adrenal hypoplasia is a rare developmental disorder of the human adrenal cortex and is caused by deletion or mutation of the dosage-sensitive sex reversal adrenal hypoplasia congenita critical region of the X chromosome, gene 1 (DAX-1 gene. Most affected children present with failure to thrive, salt wasting and hypoglycemic convulsions in the first months of life. Hypospadias affects approximately one in 250 live male births. Mutations in the mastermind-like domain-containing 1 (MAMLD1 gene have been implicated as one of the causes of hypospadias in children. To the best of our knowledge, an association between congenital adrenal hypoplasia due to a DAX-1 mutation and hypospadias due to mutation of the MAMLD1 gene has not previously been reported in the literature. Case presentation A 35-day-old male Egyptian baby was referred to our institution for the evaluation of a two-week history of recurrent vomiting associated with electrolyte imbalance. On examination, our patient was found to have hypotension and dehydration. A genital examination showed distal penile hypospadias with chordee and normal testes. He had hyponatremia, hyperkalemia, hypoglycemia and metabolic acidosis. Endocrinological investigations revealed low levels of cortisol, 17-hydroxyprogesterone and aldosterone, with a high level of adrenocorticotrophic hormone. A provisional diagnosis of congenital adrenal hypoplasia associated with hypospadias was made. A molecular genetics study confirmed the diagnosis of X-linked congenital adrenal hypoplasia due to DAX-1 mutations and hypospadias due to MAMLD1 mutation. He was started on hydrocortisone and fludrocortisone treatment. After three weeks of treatment, his symptoms improved and his blood sugar, sodium, potassium and cortisol levels normalized. Conclusions We report the case of an Egyptian baby with an association of congenital adrenal hypoplasia due to DAX-1 mutation and hypospadias due

  7. Optic nerve compression as a late complication of a hydrogel explant with silicone encircling band

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    Niels Crama

    2018-06-01

    Full Text Available Purpose: To present a complication of compressive optic neuropathy caused by a swollen hydrogel explant and posteriorly displaced silicone encircling band. Observations: A 72-year-old female patient presented with progressive visual loss and a tilted optic disc. Her medical history included a retinal detachment in 1993 that was treated with a hydrogel explant under a solid silicone encircling band. Visual acuity had decreased from 6/10 to 6/20 and perimetry showed a scotoma in the temporal superior quadrant. On Magnetic Resonance Imaging (MRI, compression of the optic nerve by a displaced silicone encircling band inferior nasally in combination with a swollen episcleral hydrogel explant was observed. Surgical removal of the hydrogel explant and silicone encircling band was uneventful and resulted in improvement of visual acuity and visual field loss. Conclusions and importance: This is the first report on compressive optic neuropathy caused by swelling of a hydrogel explant resulting in a dislocated silicone encircling band. The loss of visual function resolved upon removal of the explant and encircling band. Keywords: Retinal detachment, Tilted disc, Optic neuropathy, Miragel, Explant, Encircling band

  8. Evaluation of Macular Ganglion Cell Complex and Peripapillary Retinal Nerve Fiber Layer in Primary Craniopharyngioma by Fourier-Domain Optical Coherence Tomography.

    Science.gov (United States)

    Yang, Liu; Qu, Yuanzhen; Lu, Wen; Liu, Fengjun

    2016-07-03

    BACKGROUND The aim of this study was to compare the differences in macular ganglion cell complex (GCC) and peripapillary retinal nerve fiber layer (pRNFL) in child and adult patients with primary craniopharyngioma by Fourier-domain optical coherence tomography (FD-OCT) and to evaluate their significance in the diagnosis of primary craniopharyngioma. MATERIAL AND METHODS Ninety-six participants were divided into 3 groups: 32 in the child craniopharyngioma group (CCG) and 32 in the adult craniopharyngioma group (ACG) who were treated in Beijing Tiantan Hospital between November 2013 and October 2014, and 32 in the normal group (NG). All subjects were scanned by FD-OCT to map GCC and pRNFL thicknesses. Spearman correlation coefficient was used to assess the correlation between GCC and pRNFL thickness, and pRNFL thickness and optic nerve head (ONH) parameters, including horizontal cup-disc ratio (HCDR), vertical cup-disc ratio (VCDR), optic disc area (ODA), and cup area (CA), respectively. RESULTS The correlation between GCC and pRNFL thickness in the CCG was slightly stronger compared with the ACG. A significant difference in GCC thickness was observed among the CCG, ACG, and NG. Although the pRNFL thickness in both the CCG and ACG was significantly higher than that in NG, no significant difference in pRNFL thickness was detected between the 2 craniopharyngioma groups. The average, superior, and inferior pRNFL thicknesses were negatively correlated with VCDR in the CCG (in double eyes) and ACG (only in left eyes). CONCLUSIONS GCC was more sensitive than pRNFL in detecting optic nerve damage in the eyes of craniopharyngioma patients. A thinner pRNFL was especially correlated with VCDR in child craniopharyngioma patients.

  9. Hypoplasia of the internal carotid artery with intercavernous anastomosis

    International Nuclear Information System (INIS)

    Chen, C.J.; Wang, L.J.; Wong, Y.C.; Chen, S.T.; Hsieh, F.Y.

    1998-01-01

    We report a symptomatic case of unilateral hypoplasia of the internal carotid artery with an intercavernous anastomosis, a very rare developmental anomaly. The symptoms were caused by occlusion of the proximal middle cerebral artery which possibly related to the haemodynamic stress caused by the anomalous intercavernous anastomosis. (orig.)

  10. Treatment of enamel hypoplasia in a patient with Usher syndrome.

    Science.gov (United States)

    de la Peña, Victor Alonso; Valea, Martín Caserío

    2011-08-01

    Usher syndrome (USH) is a group of autosomal recessive diseases characterized by the association of retinitis pigmentosa with sensorineural hearing loss. There are three types of USH. In addition, in people with USH and hypoplasia, the thickness of the enamel is reduced. The authors describe a case of a patient with USH type II associated with severe enamel hypoplasia and multiple unerupted teeth. The authors placed direct composite crowns and extracted severely affected and impacted molars. There is little information available on the oral pathologies of USH. Because the authors did not know how the patient's condition would progress and the patient still was growing, the authors treated the patient conservatively by placing direct composite crowns. The treatment has met both esthetic and functional expectations for 10 years. Copyright © 2011 American Dental Association. All rights reserved.

  11. Spatial organization of lipids in the human retina and optic nerve by MALDI imaging mass spectrometry.

    Science.gov (United States)

    Zemski Berry, Karin A; Gordon, William C; Murphy, Robert C; Bazan, Nicolas G

    2014-03-01

    MALDI imaging mass spectrometry (IMS) was used to characterize lipid species within sections of human eyes. Common phospholipids that are abundant in most tissues were not highly localized and observed throughout the accessory tissue, optic nerve, and retina. Triacylglycerols were highly localized in accessory tissue, whereas sulfatide and plasmalogen glycerophosphoethanolamine (PE) lipids with a monounsaturated fatty acid were found enriched in the optic nerve. Additionally, several lipids were associated solely with the inner retina, photoreceptors, or retinal pigment epithelium (RPE); a plasmalogen PE lipid containing DHA (22:6), PE(P-18:0/22:6), was present exclusively in the inner retina, and DHA-containing glycerophosphatidylcholine (PC) and PE lipids were found solely in photoreceptors. PC lipids containing very long chain (VLC)-PUFAs were detected in photoreceptors despite their low abundance in the retina. Ceramide lipids and the bis-retinoid, N-retinylidene-N-retinylethanolamine, was tentatively identified and found only in the RPE. This MALDI IMS study readily revealed the location of many lipids that have been associated with degenerative retinal diseases. Complex lipid localization within retinal tissue provides a global view of lipid organization and initial evidence for specific functions in localized regions, offering opportunities to assess their significance in retinal diseases, such as macular degeneration, where lipids have been implicated in the disease process.

  12. Controversy in the management of optic nerve glioma

    International Nuclear Information System (INIS)

    Weiss, L.; Sagerman, R.H.; King, G.A.; Chung, C.T.; Dubowy, R.L.

    1987-01-01

    The records of 16 patients with optic nerve glioma treated between 1961 and 1984 were reviewed. All patients except two had extension of tumor beyond the chiasm to the hypothalamus, adjacent brain and/or along the posterior optic tract. Eleven of 16 cases were biopsy-proven, two patients had craniotomy and visual inspection but no biopsy was performed, and in two cases the biopsy was not diagnostic. Fourteen patients received radiation therapy, usually consisting of 50 Gy in 5 weeks (range 40-56 Gy), one patient was treated surgically and one with chemotherapy. With a follow-up of 1 to 20 years, 7 of the 14 patients irradiated are alive, three patients are dead of disease at 3, 6 and 9 years post-treatment, three were lost to follow-up at 1, 8, and 8 years, and one is dead of intercurrent disease at 5.5 years. Overall vision was improved in five patients and stable in seven following treatment. In two patients, vision could not be evaluated because of young age at presentation. Four patients had recurrences. One was retreated with 30 Gy in 3 weeks and shows no evidence of disease at 20 years. The three other patients died of their disease. There is controversy over the best treatment for these patients. Based on these results and a review of the literature, the authors recommendation is to irradiate tumors with extension beyond the chiasm at the time of presentation rather than waiting for increasing symptoms because function that is lost may not always be recovered. Chemotherapy needs to be further investigated but holds promise, especially for the younger children

  13. [Risk factors for teeth aplasia and hypoplasia in cleft lip and palate children].

    Science.gov (United States)

    Korolenkova, M V; Starikova, N V; Ageeva, L V

    2016-01-01

    The aim of the study was to assess the significance of environmental risk factors for teeth aplasia and hypoplasia in cleft lip and palate children. Two hundred and forty-seven cleft lip and palate (CLP) children were enrolled in the study including 105 (42.5%) with bilateral CLP and 57.5% with unilateral CLP. The mean age was 11.2±4.9 years. Teeth condition was assessed clinically and radiologically. The impact of risk factors for teeth anomalies was analyzed by retrospective data obtained from computer database (absence of preoperative orthopedic treatment, palatal defects after primary palatoplasty and type of primary procedures). Surgical trauma by early periosteoplasty (at the age of 3-4 months), excessive scarring and tissue traction due to absence of early orthopedic treatment and palatal defect were associated with significantly higher incidence of incisors hypoplasia (both developmental enamel defects and microdentia) and aplasia of central incisors not seen in the other study subgroups. Incisors aplasia and hypoplasia in CLP patients do not always have disembryogenic origin but may depend on external environmental factors, including surgical trauma.

  14. Improved automatic optic nerve radius estimation from high resolution MRI

    Science.gov (United States)

    Harrigan, Robert L.; Smith, Alex K.; Mawn, Louise A.; Smith, Seth A.; Landman, Bennett A.

    2017-02-01

    The optic nerve (ON) is a vital structure in the human visual system and transports all visual information from the retina to the cortex for higher order processing. Due to the lack of redundancy in the visual pathway, measures of ON damage have been shown to correlate well with visual deficits. These measures are typically taken at an arbitrary anatomically defined point along the nerve and do not characterize changes along the length of the ON. We propose a fully automated, three-dimensionally consistent technique building upon a previous independent slice-wise technique to estimate the radius of the ON and surrounding cerebrospinal fluid (CSF) on high-resolution heavily T2-weighted isotropic MRI. We show that by constraining results to be three-dimensionally consistent this technique produces more anatomically viable results. We compare this technique with the previously published slice-wise technique using a short-term reproducibility data set, 10 subjects, follow-up <1 month, and show that the new method is more reproducible in the center of the ON. The center of the ON contains the most accurate imaging because it lacks confounders such as motion and frontal lobe interference. Long-term reproducibility, 5 subjects, follow-up of approximately 11 months, is also investigated with this new technique and shown to be similar to short-term reproducibility, indicating that the ON does not change substantially within 11 months. The increased accuracy of this new technique provides increased power when searching for anatomical changes in ON size amongst patient populations.

  15. Indirect veneer treatment of anterior maxillary teeth with enamel hypoplasia

    Directory of Open Access Journals (Sweden)

    Devi Eka Juniarti

    2010-09-01

    Full Text Available Background: Nowadays, aesthetic rehabilitation becomes a necessity. It is affected by patient’s background, especially career, social and economic status. The aesthetic abnormality of anterior teeth i.e discoloration, malposition and malformation can affect patient’s appearance, especially during smile. These dental abnormalities, as a result, can decrease patient’s performance. Dental malformation, for instance, can be caused by developmental tooth defect, such as enamel hypoplasia. Enamel hypoplasia is a developmental defect caused by the lack of matrix amount which leads to thin and porous enamel. Enamel hypoplasia can also be caused by matrix calcification disturbance starting from the formation and development of enamel matrix causing defect and permanent changes which can occur on one or more tooth. Purpose: The aim of the study is to improve dental discoloration and tooth surface texture on anterior maxillary teeth with enamel hypoplasia by using indirect veneer with porcelain material. Case: A 20 years-old woman with enamel hypoplasia came to the Dental Hospital, Faculty of Dentistry Airlangga University. The patient wanted to improve her anterior maxillary teeth. It is clinically known that there were some opaque white spots (chalky spotted and porous on anterior teeth’s surface. Case management: Indirect veneer with porcelain material had been chosen as a restoration treatment which has excellent aesthetics and strength, and did not cause gingival irritation. As a result, the treatment could improve the confidence of the patient, and could also make their function normal. Conclusion: Indirect veneer is an effective treatment, which can improve patient’s appearance and self confidence.Latar belakang: Saat ini perbaikan estetik menjadi suatu kebutuhan. Kebutuhan akan estetik dipengaruhi latar belakang penderita, terutama karir, status sosial dan ekonomi. Hal ini disebabkan, kelainan estetik seperti diskolorasi, malposisi

  16. Interocular symmetry of retinal nerve fiber layer and optic nerve head parameters measured by Cirrus high-definition optical coherence tomography in a normal pediatric population.

    Science.gov (United States)

    Pawar, Neelam; Maheshwari, Devendra; Ravindran, Meenakshi; Ramakrishnan, Renagappa

    2017-10-01

    To determine interocular differences in the retinal nerve fiber layer (RNFL) and optic nerve head (ONH) parameters in a pediatric population using Cirrus high-definition optical coherence tomography (HD-OCT). Seventy normal Indian children aged 5-17 years presenting to the Pediatric Clinic were included in this observational cross-sectional study. All subjects underwent a comprehensive ophthalmologic examination and an evaluation of the RNFL and ONH by Cirrus HD-OCT. Differences between the right and left eyes were calculated and values were compared by means of a paired t-test. Subjects were also divided into two groups based on age (under or over 10 years of age). Interocular differences in RNFL and ONH parameters together with sex and age variations for these differences were determined. The mean age of studied pediatric population was 11.83 ± 3.3 years (range 5-17). Average RNFL thickness was 94.46 ± 8.7 μm (± SD) (range 77-111). Differences in the average RNFL between right and left eyes were not statistically significant (P = 0.060). Superior quadrant RNFL was thicker in the left eye and temporal quadrant was thicker in the right eye. Among ONH parameters, there were no statistically significant differences in any parameters, except vertical cup-disc (CD) ratio which was significant (P = 0.007). The 2.5%-97.5% limits of asymmetry were 9 μm for average RNFL, 0.14 for average CD ratio, and 0.22 for vertical CD ratio. Mean interocular RNFL thickness differences in superior, superior nasal, and temporal superior quadrants were 10.61 (P sex, while only significant differences with age were observed in 12 clock hour sector analysis, mainly in nasal inferior and inferior quadrant. We report the degree of interocular symmetry of RNFL and ONH parameters measured by Cirrus HD-OCT in a healthy pediatric population. The normal interocular RNFL asymmetry should not exceed 9 μm and vertical CD ratio beyond 0.22 should be considered for further investigations. The

  17. Mechanisms of cadmium-caused eye hypoplasia and hypopigmentation in zebrafish embryos.

    Science.gov (United States)

    Zhang, Ting; Zhou, Xin-Ying; Ma, Xu-Fa; Liu, Jing-Xia

    2015-10-01

    Cadmium-caused head and eye hypoplasia and hypopigmentation has been recognized for a long time, but knowledge of the underlying mechanisms is limited. In this study, we found that high mortality occurred in exposed embryos after 24 hpf, when cadmium (Cd) dosage was above 17.8 μM. Using high-throughput in situ hybridization screening, we found that genes labelling the neural crest and its derivative pigment cells exhibited obviously reduced expression in Cd-exposed embryos from 24 hpf, 2 days earlier than head and eye hypoplasia and hypopigmentation occurred. Moreover, based on expression of crestin, a neural crest marker, we found that embryos before the gastrula stage were more sensitive to cadmium toxicity and that damage caused by Cd on embryogenesis was dosage dependent. In addition, by phenotype observation and detection of neural crest and pigment cell markers, we found that BIO and retinoic acid (RA) could neutralize the toxic effects of Cd on zebrafish embryogenesis. In this study, we first determined that Cd blocked the formation of the neural crest and inhibited specification of pigment cells, which might contribute to the molecular mechanisms underlying the phenotype defects of head and eye hypoplasia and hypopigmentation in Cd-exposed embryos. Moreover, we found that compounds BIO or RA could neutralize the toxic effects of Cd. Copyright © 2015 Elsevier B.V. All rights reserved.

  18. The helical three-dimensional CT in the diagnosis of torticollis with occipitocondylar hypoplasia

    International Nuclear Information System (INIS)

    Ilkko, E.; Tikkakoski, T.; Pyhtinen, J.

    1998-01-01

    Congenital anomalies of the atlanto-occipital and atlantoaxial joints are rare. Those most commonly reported are atlantoaxial instability, basilar impression, anomalies of the odontoid process, laxity of the transverse atlantal ligament and atlanto-occipital fusion. Occipital condylar hypoplasia is infrequent and difficult to recognise. We recently diagnosed it using helical 3D CT in association with torticollis in two patients. The first patient had a several year history of torticollis. The second patient had acute cervical lymphadenitis associated with post-operative torticollis. 3D CT distinctly revealed atlantoaxial subluxation with hypoplasia of the occipital condyles in both cases

  19. Polycystic Ovary Syndrome: Aggressive or Protective Factor for the Retina? Evaluation of Macular Thickness and Retinal Nerve Fiber Layers Using High-Definition Optical Coherence Tomography

    Directory of Open Access Journals (Sweden)

    José Edvan de Souza-Júnior

    2015-01-01

    Full Text Available Objective. To compare macular thickness (MT and retinal nerve fiber layers (RNFL between women with polycystic ovary syndrome (PCOS and healthy women. Materials and Methods. The study included 45 women with PCOS and 47 ovulatory women undergoing clinical-gynecological and ophthalmic evaluations, including measurement of MT, RNFL, and optic disc parameters using optical coherence tomography. Results. The superior RNFL around the optic nerve was significantly thicker in PCOS than in healthy volunteers (P=0.036. After stratification according to insulin resistance, the temporal inner macula (TIM, the inferior inner macula (IIM, the nasal inner macula (NIM, and the nasal outer macula (NOM were significantly thicker in PCOS group than in control group (P<0.05. Both the presence of obesity associated with insulin resistance (P=0.037 and glucose intolerance (P=0.001 were associated with significant increase in the PC1 mean score, relative to MT. A significant increase in the PC2 mean score occurred when considering the presence of metabolic syndrome (P<0.0001. There was a significant interaction between obesity and inflammation in a decreasing mean PC2 score relative to macular RNFL thickness (P=0.034. Conclusion. Decreased macular RNFL thickness and increased total MT are associated with metabolic abnormalities, while increased RNFL thickness around the optic nerve is associated with hormonal changes inherent in PCOS.

  20. The significance of a hypoplastic bony canal for the cochlear nerve in patients with sensorineural hearing loss: CT and MRI findings

    International Nuclear Information System (INIS)

    Choi, Yoon Jung; Park, Sang Yoo; Kim, Myung Soon; Sung, Ki Jun

    2004-01-01

    The purpose of this study is to evaluate the significance of the hypoplastic canal for the cochlear nerve in patients with sensorineural hearing loss (SNHL) and the relationship between the hypoplastic bony canal and aplasia or hypoplasia of the cochlear nerve. A retrospective review of high resolution temporal CT(HRCT) and MRI findings was conducted. The narrow bony canal of the cochlear nerve and the relative size of the internal auditory canal were correlated with the cochlear nerve deficiency on MRI. The comparative size of the component nerves (facial, cochlear, superior vestibular, inferior vestibular nerve), and the relative size of the internal auditory canal and the bony canal of the cochlear nerve were measured. The clinical history and the results of the clinical examination were reviewed for each patient. High resolution MRI showed aplasia of the common vestibulocochlear nerve in one patient and a deficiency of the cochlear nerve in 9 patients. These abnormalities occurred in association with a prominent narrowing of the canal for the cochlear nerve and a stenosis of the internal auditory canal, which was observed on temporal bone CT in 9 patients with congenital SNHL. Three patients had normal IAC, despite the presence of a hypoplastic cochlear nerve on the side on which they had SNHL. In one patient, the narrowing of the canal for the cochlear nerve and internal auditory canal were not found to be associated with acquired SNHL. The hypoplastic bony canal for the cochlear nerve might be more highly indicative of congenital cochlear nerve deficiency than that of the narrow internal auditory canal, and the position of the crista falciformis should also be carefully

  1. The significance of a hypoplastic bony canal for the cochlear nerve in patients with sensorineural hearing loss: CT and MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Yoon Jung; Park, Sang Yoo; Kim, Myung Soon; Sung, Ki Jun [College of Medicine, Yonsei Univ., Wonju (Korea, Republic of)

    2004-04-01

    The purpose of this study is to evaluate the significance of the hypoplastic canal for the cochlear nerve in patients with sensorineural hearing loss (SNHL) and the relationship between the hypoplastic bony canal and aplasia or hypoplasia of the cochlear nerve. A retrospective review of high resolution temporal CT(HRCT) and MRI findings was conducted. The narrow bony canal of the cochlear nerve and the relative size of the internal auditory canal were correlated with the cochlear nerve deficiency on MRI. The comparative size of the component nerves (facial, cochlear, superior vestibular, inferior vestibular nerve), and the relative size of the internal auditory canal and the bony canal of the cochlear nerve were measured. The clinical history and the results of the clinical examination were reviewed for each patient. High resolution MRI showed aplasia of the common vestibulocochlear nerve in one patient and a deficiency of the cochlear nerve in 9 patients. These abnormalities occurred in association with a prominent narrowing of the canal for the cochlear nerve and a stenosis of the internal auditory canal, which was observed on temporal bone CT in 9 patients with congenital SNHL. Three patients had normal IAC, despite the presence of a hypoplastic cochlear nerve on the side on which they had SNHL. In one patient, the narrowing of the canal for the cochlear nerve and internal auditory canal were not found to be associated with acquired SNHL. The hypoplastic bony canal for the cochlear nerve might be more highly indicative of congenital cochlear nerve deficiency than that of the narrow internal auditory canal, and the position of the crista falciformis should also be carefully.

  2. Pontoneocerebellar hypoplasia: definition of MR features

    International Nuclear Information System (INIS)

    Goasdoue, P.; Lalande, G.; Adamsbaum, C.; Rodriguez, D.; Moutard, M.-L.; Robain, O.

    2001-01-01

    We report five patients with pontoneocerebellar hypoplasia. Because the issue of cerebellar malformations is a difficult subject, we tried to define criteria for diagnosis on MRI: a thin flat pons with disappearance of the anterior curve, a small cerebellum with predominant flattening of the hemispheres and shortened cerebellar fissures, in contrast to atrophy. The posterior fossa is not enlarged. We emphasize the probable late onset of the disease in fetal life because of the demonstration of the abnormalities at US during the last trimester of the pregnancy in one patient. Prenatal diagnosis is important because of possible autosomal recessive transmission. (orig.)

  3. Tolerance of cranial nerves of the cavernous sinus to radiosurgery

    International Nuclear Information System (INIS)

    Tishler, R.B.; Loeffler, J.S.; Alexander, E. III; Kooy, H.M.; Lunsford, L.D.; Duma, C.; Flickinger, J.C.

    1993-01-01

    Stereotactic radiosurgery is becoming a more accepted treatment option for benign, deep seated intracranial lesions. However, little is known about the effects of large single fractions of radiation on cranial nerves. This study was undertaken to assess the effect of radiosurgery on the cranial nerves of the cavernous sinus. The authors examined the tolerance of cranial nerves (II-VI) following radiosurgery for 62 patients (42/62 with meningiomas) treated for lesions within or near the cavernous sinus. Twenty-nine patients were treated with a modified 6 MV linear accelerator (Joint Center for Radiation Therapy) and 33 were treated with the Gamma Knife (University of Pittsburgh). Three-dimensional treatment plans were retrospectively reviewed and maximum doses were calculated for the cavernous sinus and the optic nerve and chiasm. Median follow-up was 19 months (range 3-49). New cranial neuropathies developed in 12 patients from 3-41 months following radiosurgery. Four of these complications involved injury to the optic system and 8 (3/8 transient) were the result of injury to the sensory or motor nerves of the cavernous sinus. There was no clear relationship between the maximum dose to the cavernous sinus and the development of complications for cranial nerves III-VI over the dose range used (1000-4000 cGy). For the optic apparatus, there was a significantly increased incidence of complications with dose. Four of 17 patients (24%) receiving greater than 800 cGy to any part of the optic apparatus developed visual complications compared with 0/35 who received less than 800 cGy (p = 0.009). Radiosurgery using tumor-controlling doses of up to 4000 cGy appears to be a relatively safe technique in treating lesions within or near the sensory and motor nerves (III-VI) of the cavernous sinus. The dose to the optic apparatus should be limited to under 800 cGy. 21 refs., 4 tabs

  4. A case of atypical McCune-Albright syndrome requiring optic nerve decompression.

    Science.gov (United States)

    Yavuzer, R; Khilnani, R; Jackson, I T; Audet, B

    1999-10-01

    McCune-Albright syndrome (MAS) is a disease of noninheritable, genetic origin defined by the triad of café-au-lait pigmentation of the skin, precocious puberty, and polyostotic fibrous dysplasia. This syndrome, which affects young girls primarily, has also been reported with other endocrinopathies, and rarely with acromegaly and hyperprolactinemia. The fibrous dysplasia in MAS is of the polyostotic type and, apart from the characteristic sites such as the proximal aspects of the femur and the pelvis, the craniofacial region is frequently involved. A male patient with MAS presented with juvenile gigantism, precocious puberty, pituitary adenoma-secreting growth hormone and prolactin, hypothalamic pituitary gonadal and thyroid dysfunction, and polyostotic fibrous dysplasia causing optic nerve compression. Visual deterioration and its surgical management are presented.

  5. Ectopic KIT copy number variation underlies impaired migration of primordial germ cells associated with gonadal hypoplasia in cattle (Bos taurus.

    Directory of Open Access Journals (Sweden)

    Heli Venhoranta

    Full Text Available Impaired migration of primordial germ cells during embryonic development causes hereditary gonadal hypoplasia in both sexes of Northern Finncattle and Swedish Mountain cattle. The affected gonads exhibit a lack of or, in rare cases, a reduced number of germ cells. Most affected animals present left-sided gonadal hypoplasia. However, right-sided and bilateral cases are also found. This type of gonadal hypoplasia prevails in animals with white coat colour. Previous studies indicated that gonadal hypoplasia is inherited in an autosomal recessive fashion with incomplete penetrance. In order to identify genetic regions underlying gonadal hypoplasia, a genome-wide association study (GWAS and a copy number variation (CNV analysis were performed with 94 animals, including 21 affected animals, using bovine 777,962 SNP arrays. The GWAS and CNV results revealed two significantly associated regions on bovine chromosomes (BTA 29 and 6, respectively (P=2.19 x 10(-13 and P=5.65 x 10(-6. Subsequent cytogenetic and PCR analyses demonstrated that homozygosity of a ~500 kb chromosomal segment translocated from BTA6 to BTA29 (Cs29 allele is the underlying genetic mechanism responsible for gonadal hypoplasia. The duplicated segment includes the KIT gene that is known to regulate the migration of germ cells and precursors of melanocytes. This duplication is also one of the two translocations associated with colour sidedness in various cattle breeds.

  6. Imaging of macrophage dynamics with optical coherence tomography in anterior ischemic optic neuropathy.

    Science.gov (United States)

    Kokona, Despina; Häner, Nathanael U; Ebneter, Andreas; Zinkernagel, Martin S

    2017-01-01

    Anterior ischemic optic neuropathy (AION) is a relatively common cause of visual loss and results from hypoperfusion of the small arteries of the anterior portion of the optic nerve. AION is the leading cause of sudden optic nerve related vision loss with approximately 10 cases per 100'000 in the population over 50 years. To date there is no established treatment for AION and therefore a better understanding of the events occurring at the level of the optic nerve head (ONH) would be important to design future therapeutic strategies. The optical properties of the eye allow imaging of the optic nerve in vivo, which is a part of the CNS, during ischemia. Experimentally laser induced optic neuropathy (eLiON) displays similar anatomical features as anterior ischemic optic neuropathy in humans. After laser induced optic neuropathy we show that hyperreflective dots in optical coherence tomography correspond to mononuclear cells in histology. Using fluorescence-activated flow cytometry (FACS) we found these cells to peak one week after eLiON. These observations were translated to OCT findings in patients with AION, where similar dynamics of hyperreflective dots at the ONH were identified. Our data suggests that activated macrophages can be identified as hyperreflective dots in OCT. Copyright © 2016 The Authors. Published by Elsevier Ltd.. All rights reserved.

  7. Palatoglossal fusion with cleft palate and hypoplasia of cerebellar vermis

    Directory of Open Access Journals (Sweden)

    Shailesh Solanki

    2016-01-01

    Full Text Available A new-born male presented within 12 h of birth with respiratory distress. On examination and workup, he had palatoglossal fusion, cleft palate and hypoplasia of the cerebellar vermis. A 2.5 Fr endotracheal tube was inserted into the pharynx through nostril as a nasopharyngeal stent, following which his respiratory distress improved. Once child was optimised, then feeding was started by nasogastric tube and feeds were tolerated well. Elective tracheostomy and gastrostomy were done, followed by release of adhesions between the tongue and palate at a later stage. Review of literature suggests that palatoglossal fusion is uncommon and presents as an emergency. Mostly, these oral synechiae are associated with digital and/or cardiac anomaly. Other disorders associated with intra-oral synechiae include congenital alveolar synechiae, van der Woude syndrome, popliteal pterygium syndrome and oromandibular limb hypogenesis syndrome. The authors report a hitherto undescribed association of palatoglossal fusion with cleft palate and hypoplasia of the cerebellar vermis.

  8. The helical three-dimensional CT in the diagnosis of torticollis with occipitocondylar hypoplasia

    International Nuclear Information System (INIS)

    Ilkko, E.; Tikkakoski, T.; Pyhtinen, J.

    1998-01-01

    Congenital anomalies of the atlanto-occipital and atlantoaxial joints are rare. Those most commonly reported are atlantoaxial instability, basilar impression, anomalies of the odontoid process, laxity of the transverse atlantal ligament and atlanto-occipital fusion. Occipital condylar hypoplasia is infrequent and difficult to recognise. We recently diagnosed it using helical 3D CT in association with torticollis in two patients. The first patient had a several year history of torticollis. The second patient had acute cervical lymphadenitis associated with post-operative torticollis. 3D CT distinctly revealed atlantoaxial subluxation with hypoplasia of the occipital condyles in both cases. (Copyright (c) 1998 Elsevier Science B.V., Amsterdam. All rights reserved.)

  9. Severe hypoplasia of the omasal laminae in a Japanese Black steer with chronic bloat--a case report.

    Science.gov (United States)

    Takagi, Mitsuhiro; Mukai, Shuhei; Fushimi, Yasuo; Matsushita, Kouhei; Miyoshi, Nobuaki; Yasuda, Nobuhiro; Kitajima, Hideo; Takamure, Senro; Matsushita, Toshihiko; Kitamura, Nobuo; Deguchi, Eisaburo

    2007-12-01

    An 11-month-old Japanese Black steer with chronic bloat underwent clinical and histological analyses. During the observation period, it showed normal appetite and fecal volume but persistent chronic bloat symptoms. Compared to controls, the steer's feces contained undigested large straws. Necropsy revealed normal rumen, reticulum, and abomasum but a small omasum. The rumen, reticulum, and abomasum mucosa was normal, with well-developed ruminal papillae. However, severe hypoplasia of the omasal laminae was observed along with hypoplasia reticular groove and ruminoreticular fold. The contents of the reticulum, omasum, and abomasums comprised undigested large sized hay particles. The omasum papillae showed no pathological abnormalities. This is a rare case of a steer with chronic bloat probably caused by severe hypoplasia of the omasal laminae.

  10. Retinal Nerve Fiber Layer and Peripapillary Choroidal Thicknesses in Non-Glaucomatous Unilateral Optic Atrophy Compared with Unilateral Advanced Pseudoexfoliative Glaucoma.

    Science.gov (United States)

    Kucukevcilioglu, Murat; Ayyildiz, Onder; Aykas, Seckin; Gokce, Gokcen; Koylu, Mehmet Talay; Ozgonul, Cem; Ozge, Gokhan; Mumcuoglu, Tarkan; Yumusak, Erhan

    2017-02-01

    To investigate retinal nerve fiber layer thickness (RNFL-T) and peripapillary choroidal thickness (PC-T) in non-glaucomatous optic atrophy (OA) patients in comparison with unaffected and control eyes, furthermore, to compare thickness profiles with unilateral pseudoexfoliative advanced glaucoma. Thirty-three eyes with OA (Group A), 33 unaffected fellow eyes (Group B), 25 right eyes of 25 control subjects (Group C), and 15 eyes with advanced glaucoma (Group D) were enrolled. RNFL-T was measured in six regions by spectral-domain optical coherence tomography. Enhanced depth imaging optical coherence tomography was obtained to evaluate PC-T in corresponding regions. RNFL-T was significantly lower in Group A than in Groups B and C globally and at all peripapillary regions (all p temporal > nasal > inferior) profiles were almost identical to that in unaffected fellow eyes and control eyes. However, Group D showed different patterns with less regional differences in RNFL-T, and the greatest value of PC-T in nasal quadrant. Besides retinal nerve fiber layer thinning, non-glaucomatous OA is associated with choroidal thinning. The RNFL-T and PC-T profiles in advanced glaucoma eyes differed from the common patterns seen among OA eyes, unaffected fellow eyes, and control eyes.

  11. Visual loss and optic nerve head swelling in thiamine deficiency without prolonged dietary deficiency

    Directory of Open Access Journals (Sweden)

    Gratton SM

    2014-05-01

    Full Text Available Sean M Gratton, Byron L LamBascom Palmer Eye Institute, University of Miami, Miller School of Medicine, Miami, FL, USAAbstract: Visual loss due to optic neuropathy is a rare manifestation of thiamine deficiency. We report a case of a 39-year-old woman with a body mass index (BMI of 29 kg/m2 who developed visual loss and bilateral optic nerve head swelling after a short, self-limited gastrointestinal illness. She was disoriented and inattentive and had absent ankle jerk reflexes, diminished sensation in both legs below the knees, and marked truncal ataxia. Magnetic resonance imaging (MRI showed increased T2-signal in the medial thalami and mammillary bodies. The serum thiamine level was 8 nmol/L (normal 8–30. The diagnosis of thiamine deficiency was made, and the patient’s vision and neurologic symptoms improved significantly with intramuscular thiamine treatment. Thiamine deficiency can occur in the absence of an obvious predisposing factor such as alcoholism or low body weight. The clinician must be aware of the factors that govern vitamin availability and maintain a high index of suspicion to make the diagnosis in such cases.Keywords: optic neuropathy, nutritional deficiency

  12. Wfs1- deficient rats develop primary symptoms of Wolfram syndrome: insulin-dependent diabetes, optic nerve atrophy and medullary degeneration.

    Science.gov (United States)

    Plaas, Mario; Seppa, Kadri; Reimets, Riin; Jagomäe, Toomas; Toots, Maarja; Koppel, Tuuliki; Vallisoo, Tuuli; Nigul, Mait; Heinla, Indrek; Meier, Riho; Kaasik, Allen; Piirsoo, Andres; Hickey, Miriam A; Terasmaa, Anton; Vasar, Eero

    2017-08-31

    Wolfram syndrome (WS) is a rare autosomal-recessive disorder that is caused by mutations in the WFS1 gene and is characterized by juvenile-onset diabetes, optic atrophy, hearing loss and a number of other complications. Here, we describe the creation and phenotype of Wfs1 mutant rats, in which exon 5 of the Wfs1 gene is deleted, resulting in a loss of 27 amino acids from the WFS1 protein sequence. These Wfs1-ex5-KO232 rats show progressive glucose intolerance, which culminates in the development of diabetes mellitus, glycosuria, hyperglycaemia and severe body weight loss by 12 months of age. Beta cell mass is reduced in older mutant rats, which is accompanied by decreased glucose-stimulated insulin secretion from 3 months of age. Medullary volume is decreased in older Wfs1-ex5-KO232 rats, with the largest decreases at the level of the inferior olive. Finally, older Wfs1-ex5-KO232 rats show retinal gliosis and optic nerve atrophy at 15 months of age. Electron microscopy revealed axonal degeneration and disorganization of the myelin in the optic nerves of older Wfs1-ex5-KO232 rats. The phenotype of Wfs1-ex5-KO232 rats indicates that they have the core symptoms of WS. Therefore, we present a novel rat model of WS.

  13. Computed tomography in optic neuritis

    International Nuclear Information System (INIS)

    Fujitani, Ken; Hakuba, Akira; Tsujimoto, Takeshi; Yasui, Toshihiro; Sakamoto, Hiroaki

    1979-01-01

    In two patients with optic neuritis, CT scans showed common features which were helpful in diagnosis. These consisted of an enlargement of the optic nerve and an increased density of the optic nerve after contrast infusion. A 49-year-old man was admitted with a two-week duration of pain and a progressive visual loss in his right eye. On admission, an enlarged central scotoma was noted, and only a flashlight was perceived at the periphery in the right eye. The right optic disc was swollen with perivascular bleeding. Plain skull radiographs and orbital tomographs were normal. An orbital CT scan, however, revealed an enlargement of the right optic nerve, the density of which was increased after the infusion of a contrast medium. After steroid therapy, the CT scan showed a normal optic nerve following improvement in the vision of the right eye. A 44-year-old man was referred to this hospital because of a visual loss in his right eye which had been noted one month before. The right eye, with a central scotoma, had only visual acuity enough to perceive hand motion. The right pupil responded poorly to light. The right optic disc was hyperemic. Plain skull radiographs were normal. The orbital CT scan, however, showed an enlargement of the right optic nerve and exhibited contrast enhancement. Steroid therapy resulted in an improvement of the visual disturbance, and a follow-up CT scan showed normal. Two months later, though, the visual acuity had decreased and a visual field defect was noted in his left eye. The orbital CT scan revealed an enlargement of the left optic nerve. The administration of steroid again improved the visual field defect in the left eye, and the CT scan again showed normal. (author)

  14. Evaluation of acute radiation optic neuropathy by B-scan ultrasonography

    International Nuclear Information System (INIS)

    Lovato, A.A.; Char, D.H.; Quivey, J.M.; Castro, J.R.

    1990-01-01

    We studied the accuracy of B-scan ultrasonography to diagnose radiation-induced optic neuropathy in 15 patients with uveal melanoma. Optic neuropathy was diagnosed by an observer masked as to clinical and photographic data. We analyzed planimetry area measurements of the retrobulbar nerve before and after irradiation. The retrobulbar area of the optic nerve shadow on B-scan was quantitated with a sonic digitizer. Increased optic nerve shadow area was confirmed in 13 of 15 patients who had radiation optic neuropathy (P less than .004). The correct diagnosis was confirmed when the results of ultrasound were compared to fundus photography and fluorescein angiography. In 13 patients there was acute radiation optic neuropathy. Two patients did not show an enlarged retrobulbar optic nerve, and the clinical appearance suggested early progression to optic atrophy. Ultrasonography documents the enlargement of the optic nerve caused by acute radiation changes

  15. Congenital bicuspid stenosis with left ventricular hypoplasia in a kitten.

    Science.gov (United States)

    van Nie, C J; van Messel, M A; Straatman, T J

    1980-01-15

    Congenital bicuspid stenosis with left ventricular hypoplasia was diagnosed in a kitten. Clinical weakness, dyspnoea and marked cardiomegaly (X rays) were related to postmortem findings. The cardiomegaly had resulted from an enlargement of the left auricular appendage. It is supposed the cardiomegaly developed after the closing of the foramen ovale.

  16. Genetics Home Reference: optic atrophy type 1

    Science.gov (United States)

    ... Nerve Atrophy Encyclopedia: Visual Acuity Test Health Topic: Color Blindness Health Topic: Optic Nerve Disorders Genetic and Rare ... Disease InfoSearch: Optic atrophy 1 Kids Health: What's Color Blindness? MalaCards: autosomal dominant optic atrophy, classic form Merck ...

  17. Prune belly syndrome with urethral hypoplasia and vesico-cutaneous fistula: A case report and review of literature.

    Science.gov (United States)

    Sarhan, Osama M; Al-Ghanbar, Mustafa S; Nakshabandi, Ziad M

    2013-10-01

    Association between Prune belly syndrome (PBS) and urethral hypoplasia is an unusual condition. It is usually fatal unless there is a communication between the fetal bladder and the amniotic sac. We report a case of PBS with urethral hypoplasia and congenital vesico-cutaneous fistula in a male neonate. Patient underwent cutaneous vesicostomy and was discharged for close follow up of his renal function and for future reconstruction.

  18. Short limbed dwarfism, genital hypoplasia, sparse hair, and vertebral anomalies: a variant of Ellis-van Creveld syndrome?

    OpenAIRE

    Fryns, J P; Moerman, P

    1993-01-01

    A male newborn with acromesomelic short limbed dwarfism, genital hypoplasia, and vertebral anomalies is reported. As the child had an important number of clinical and radiological symptoms seen in patients with Ellis-van Creveld syndrome, we raise the question of whether he may represent a variant example of this syndrome despite the absence of cardinal symptoms such as postaxial polydactyly and ectodermal changes (nail hypoplasia).

  19. Optic nerve histopathology in a case of Wolfram Syndrome: a mitochondrial pattern of axonal loss.

    Science.gov (United States)

    Ross-Cisneros, Fred N; Pan, Billy X; Silva, Ruwan A; Miller, Neil R; Albini, Thomas A; Tranebjaerg, Lisbeth; Rendtorff, Nanna D; Lodahl, Marianne; Moraes-Filho, Milton N; Moraes, Milton N; Salomao, Solange R; Berezovsky, Adriana; Belfort, Rubens; Carelli, Valerio; Sadun, Alfredo A

    2013-11-01

    Mitochondrial dysfunction in Wolfram Syndrome (WS) is controversial and optic neuropathy, a cardinal clinical manifestation, is poorly characterized. We here describe the histopathological features in postmortem retinas and optic nerves (ONs) from one patient with WS, testing the hypothesis that mitochondrial dysfunction underlies the pathology. Eyes and retrobulbar ONs were obtained at autopsy from a WS patient, and compared with those of a Leber hereditary optic neuropathy (LHON) patient and one healthy control. Retinas were stained with hematoxylin & eosin for general morphology and ONs were immunostained for myelin basic protein (MBP). Immunostained ONs were examined in four "quadrants": superior, inferior, nasal, and temporal. The WS retinas displayed a severe loss of retinal ganglion cells in the macular region similar to the LHON retina, but not in the control. The WS ONs, immunostained for MBP, revealed a zone of degeneration in the temporal and inferior quadrants. This pattern was similar to that seen in the LHON ONs but not in the control. Thus, the WS patient displayed a distinct pattern of optic atrophy observed bilaterally in the temporal and inferior quadrants of the ONs. This arrangement of axonal degeneration, involving primarily the papillomacular bundle, closely resembled LHON and other mitochondrial optic neuropathies, supporting that mitochondrial dysfunction underlies its pathogenesis. Copyright © 2013 Elsevier B.V. All rights reserved.

  20. Evaluation of Retinal Nerve Fiber Layer Thinning in Myopic Glaucoma: Impact of Optic Disc Morphology.

    Science.gov (United States)

    Na, Kyeong Ik; Lee, Won June; Kim, Young Kook; Park, Ki Ho; Jeoung, Jin Wook

    2017-12-01

    The purpose of this study was to investigate the role of optic disc torsion on the rate of progressive retinal nerve fiber layer (RNFL) thinning in patients with myopic open-angle glaucoma. We included 102 patients with myopic open-angle glaucoma accompanied by glaucomatous damage confined to a single hemiretina who were followed up over a 5-year period. We divided the subjects into three groups according to the presence or absence of optic disc torsion and the correspondence between the direction of optic disc torsion and the location of glaucomatous damage: torsion with reverse correspondence group (eyes showing inferior optic disc torsion with glaucomatous damage in the superior quadrant or eyes showing superior torsion with damage in the inferior quadrant), no torsion group, and torsion with correspondence group (eyes showing inferior optic disc torsion with glaucomatous damage in the inferior quadrant or eyes showing superior torsion with damage in the superior quadrant). Changes in the peripapillary RNFL thickness (pRNFLT), evaluated using linear mixed model analysis, were compared among the three groups to determine the relationship between optic disc torsion and pRNFLT changes. Among the total of 102 subjects, 13 eyes (12.7%) exhibited optic disc torsion with reverse correspondence, 59 (57.8%) did not exhibit optic disc torsion, and 30 (29.4%) exhibited optic disc torsion with correspondence. pRNFL thinning in the quadrant with glaucomatous damage was significantly faster in the torsion with correspondence group (-1.66 μm/y) than those in the no torsion (-1.14 μm/y; P = 0.032) and torsion with reverse correspondence (-0.50 μm/y; P optic disc torsion-glaucomatous damage correspondence is an important prognostic factor for patients with myopic open-angle glaucoma.

  1. Parvovirus associated cerebellar hypoplasia and hydrocephalus in day-old broiler chickens

    Science.gov (United States)

    Cerebellar hypoplasia and hydrocephalus were detected in day-old broiler chickens. Brains of chickens evaluated at necropsy appeared to be abnormal; some were disfigured and cerebellae appeared to be smaller than normal. Histopathologic examination of brains revealed cerebellar folia that were sho...

  2. Ischemic stroke in patient with existing congenital hypoplasia of the middle cerebral artery

    International Nuclear Information System (INIS)

    Manchev, I.; Manolova, T.; Manchev, L.

    2015-01-01

    Presented is a clinical case of a woman 29 years old with ischemic stroke (IS), which has developed abruptly in existing congenital hypoplasia and occlusion of the middle cerebral artery. There are no other well or less well documented risk factors for cerebrovascular disease. In family history noted that the father of the patient died suddenly at the age of 45 years from stroke, also without evidence of vascular disease. On magnetic resonance imaging (MRI) of the brain is found high signal zone in the left nucleus lentiformis. We discussed the possibilities for implementing conventional angiography and eventually surgical procedures unfortunately rejected due to the high risk to the patient. Key words: Ischemic Stroke. Magnetic Resonance Imaging. Hypoplasia

  3. A photon-driven micromotor can direct nerve fibre growth

    Science.gov (United States)

    Wu, Tao; Nieminen, Timo A.; Mohanty, Samarendra; Miotke, Jill; Meyer, Ronald L.; Rubinsztein-Dunlop, Halina; Berns, Michael W.

    2012-01-01

    Axonal path-finding is important in the development of the nervous system, nerve repair and nerve regeneration. The behaviour of the growth cone at the tip of the growing axon determines the direction of axonal growth and migration. We have developed an optical-based system to control the direction of growth of individual axons (nerve fibres) using laser-driven spinning birefringent spheres. One or two optical traps position birefringent beads adjacent to growth cones of cultured goldfish retinal ganglion cell axons. Circularly polarized light with angular momentum causes the trapped bead to spin. This creates a localized microfluidic flow generating an estimated 0.17 pN shear force against the growth cone that turns in response to the shear. The direction of axonal growth can be precisely manipulated by changing the rotation direction and position of this optically driven micromotor. A physical model estimating the shear force density on the axon is described.

  4. Cerebral white matter hypoplasia

    International Nuclear Information System (INIS)

    Dietrich, R.B.; Shields, W.D.; Sankar, R.

    1990-01-01

    This paper demonstrates the MR imaging findings in children with cerebral white matter hypoplasia (CWMH). The MR studies of four children, aged 3-7 y (mean age, 2.3 y) with a diagnosis of CWMH were reviewed. In all cases multiplanar T1-weighted and T2-weighted spin-echo images were obtained. All children had similar histories of severe developmental delay and nonprogressive neurologic deficits despite normal gestational and birth histories. In two cases there was a history of maternal cocaine abuse. Autopsy correlation was available in one child. The MR images of all four children demonstrated diffuse lack of white matter and enlarged ventricles but normal-appearing gray matter. The corpus callosum, although completely formed, was severely thinned. There was no evidence of gliosis or porencephaly, and the distribution of myelin deposition was normal for age in all cases. Autopsy finding in one child correlated exactly with the MR finding

  5. In Vivo Changes in Lamina Cribrosa Microarchitecture and Optic Nerve Head Structure in Early Experimental Glaucoma.

    Directory of Open Access Journals (Sweden)

    Kevin M Ivers

    Full Text Available The lamina cribrosa likely plays an important role in retinal ganglion cell axon injury in glaucoma. We sought to (1 better understand optic nerve head (ONH structure and anterior lamina cribrosa surface (ALCS microarchitecture between fellow eyes of living, normal non-human primates and (2 characterize the time-course of in vivo structural changes in the ONH, ALCS microarchitecture, and retinal nerve fiber layer thickness (RNFLT in non-human primate eyes with early experimental glaucoma (EG. Spectral domain optical coherence tomography (SDOCT images of the ONH were acquired cross-sectionally in six bilaterally normal rhesus monkeys, and before and approximately every two weeks after inducing unilateral EG in seven rhesus monkeys. ONH parameters and RNFLT were quantified from segmented SDOCT images. Mean ALCS pore area, elongation and nearest neighbor distance (NND were quantified globally, in sectors and regionally from adaptive optics scanning laser ophthalmoscope images. In bilaterally normal monkeys, ONH parameters were similar between fellow eyes with few inter-eye differences in ALCS pore parameters. In EG monkeys, an increase in mean ALCS Depth (ALCSD was the first structural change measured in 6 of 7 EG eyes. A decrease in mean minimum rim width (MRW simultaneously accompanied this early change in 4 of 6 EG eyes and was the first structural change in the 7th EG eye. Mean ALCS pore parameters were among the first or second changes measured in 4 EG eyes. Mean ALCS pore area and NND increased in superotemporal and temporal sectors and in central and peripheral regions at the first time-point of change in ALCS pore geometry. RNFLT and/or mean ALCS radius of curvature were typically the last parameters to initially change. Survival analyses found mean ALCSD was the only parameter to significantly show an initial change prior to the first measured loss in RNFLT across EG eyes.

  6. Prune belly syndrome with urethral hypoplasia and vesico-cutaneous fistula: A case report and review of literature

    Directory of Open Access Journals (Sweden)

    Osama M Sarhan

    2013-01-01

    Full Text Available Association between Prune belly syndrome (PBS and urethral hypoplasia is an unusual condition. It is usually fatal unless there is a communication between the fetal bladder and the amniotic sac. We report a case of PBS with urethral hypoplasia and congenital vesico-cutaneous fistula in a male neonate. Patient underwent cutaneous vesicostomy and was discharged for close follow up of his renal function and for future reconstruction.

  7. Short limbed dwarfism, genital hypoplasia, sparse hair, and vertebral anomalies: a variant of Ellis-van Creveld syndrome?

    Science.gov (United States)

    Fryns, J P; Moerman, P

    1993-01-01

    A male newborn with acromesomelic short limbed dwarfism, genital hypoplasia, and vertebral anomalies is reported. As the child had an important number of clinical and radiological symptoms seen in patients with Ellis-van Creveld syndrome, we raise the question of whether he may represent a variant example of this syndrome despite the absence of cardinal symptoms such as postaxial polydactyly and ectodermal changes (nail hypoplasia). Images PMID:8487282

  8. WDR73 missense mutation causes infantile onset intellectual disability and cerebellar hypoplasia in a consanguineous family.

    Science.gov (United States)

    Jiang, Chen; Gai, Nan; Zou, Yongyi; Zheng, Yu; Ma, Ruiyu; Wei, Xianda; Liang, Desheng; Wu, Lingqian

    2017-01-01

    Galloway-Mowat syndrome (GMS) is a very rare autosomal-recessive disorder characterized by nephrotic syndrome associated with microcephaly, and various central nervous system abnormalities, mostly cerebral hypoplasia or cerebellar atrophy, intellectual disability and neural-migration defects. WDR73 is the only gene known to cause GMS, and has never been implicated in other disease. Here we present a Chinese consanguineous family with infantile onset intellectual disability and cerebellar hypoplasia but no microcephaly. Whole exome sequencing identified a WDR73 p.W371G missense mutation. The mutation is confirmed to be segregated in this family by Sanger sequencing according to a recessive inheritance pattern. It is predicted to be deleterious by multiple algorithms and affect highly conserved site. Structural modeling revealed conformational differences between the wild type protein and the p.W371G protein. Real-time PCR and Western blotting revealed altered mRNA and protein levels in mutated samples. Our study indicates the novel WDR73 p.W371G missense mutation causes infantile onset intellectual disability and cerebellar hypoplasia in recessive mode of inheritance. Our findings imply that microcephaly is a variable phenotype in WDR73-related disease, suggest WDR73 to be a candidate gene of severe intellectual disability and cerebellar hypoplasia, and expand the molecular spectrum of WDR73-related disease. Copyright © 2016 Elsevier B.V. All rights reserved.

  9. Diffusion MR Imaging of Postoperative Bilateral Acute Ischemic Optic Neuropathy

    International Nuclear Information System (INIS)

    Park, Ju Young; Lee, In Ho; Song, Chang June; Hwang, Hee Youn

    2012-01-01

    A 57-year-old woman experienced bilateral acute ischemic optic neuropathy after spine surgery. Routine MR imaging sequence, T2-weighted image, showed subtle high signal intensity on bilateral optic nerves. A contrast-enhanced T1 weighted image showed enhancement along the bilateral optic nerve sheath. Moreover, diffusion-weighted image (DWI) and an apparent diffusion coefficient map showed markedly restricted diffusion on bilateral optic nerves. Although MR findings of T2-weighted and contrast enhanced T1-weighted images may be nonspecific, the DWI finding of cytotoxic edema of bilateral optic nerves will be helpful for the diagnosis of acute ischemic optic neuropathy after spine surgery.

  10. Diffusion MR Imaging of Postoperative Bilateral Acute Ischemic Optic Neuropathy

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    Park, Ju Young; Lee, In Ho; Song, Chang June [Chungnam National University Hospital, Daejeon (Korea, Republic of); Hwang, Hee Youn [Eulji University Hospital, Daejeon(Korea, Republic of)

    2012-03-15

    A 57-year-old woman experienced bilateral acute ischemic optic neuropathy after spine surgery. Routine MR imaging sequence, T2-weighted image, showed subtle high signal intensity on bilateral optic nerves. A contrast-enhanced T1 weighted image showed enhancement along the bilateral optic nerve sheath. Moreover, diffusion-weighted image (DWI) and an apparent diffusion coefficient map showed markedly restricted diffusion on bilateral optic nerves. Although MR findings of T2-weighted and contrast enhanced T1-weighted images may be nonspecific, the DWI finding of cytotoxic edema of bilateral optic nerves will be helpful for the diagnosis of acute ischemic optic neuropathy after spine surgery.

  11. PATHOGENESIS OF OPTIC DISC EDEMA IN RAISED INTRACRANIAL PRESSURE

    Science.gov (United States)

    Hayreh, Sohan Singh

    2015-01-01

    Optic disc edema in raised intracranial pressure was first described in 1853. Ever since, there has been a plethora of controversial hypotheses to explain its pathogenesis. I have explored the subject comprehensively by doing basic, experimental and clinical studies. My objective was to investigate the fundamentals of the subject, to test the validity of the previous theories, and finally, based on all these studies, to find a logical explanation for the pathogenesis. My studies included the following issues pertinent to the pathogenesis of optic disc edema in raised intracranial pressure: the anatomy and blood supply of the optic nerve, the roles of the sheath of the optic nerve, of the centripetal flow of fluids along the optic nerve, of compression of the central retinal vein, and of acute intracranial hypertension and its associated effects. I found that, contrary to some previous claims, an acute rise of intracranial pressure was not quickly followed by production of optic disc edema. Then, in rhesus monkeys, I produced experimentally chronic intracranial hypertension by slowly increasing in size space-occupying lesions, in different parts of the brain. Those produced raised cerebrospinal fluid pressure (CSFP) and optic disc edema, identical to those seen in patients with elevated CSFP. Having achieved that, I investigated various aspects of optic disc edema by ophthalmoscopy, stereoscopic color fundus photography and fluorescein fundus angiography, and light microscopic, electron microscopic, horseradish peroxidase and axoplasmic transport studies, and evaluated the effect of opening the sheath of the optic nerve on the optic disc edema. This latter study showed that opening the sheath resulted in resolution of optic disc edema on the side of the sheath fenestration, in spite of high intracranial CSFP, proving that a rise of CSFP in the sheath was the essential pre-requisite for the development of optic disc edema. I also investigated optic disc edema with

  12. Comparison of peripapillary retinal nerve fiber layer loss and visual outcome in fellow eyes following sequential bilateral non-arteritic anterior ischemic optic neuropathy.

    Science.gov (United States)

    Dotan, Gad; Kesler, Anat; Naftaliev, Elvira; Skarf, Barry

    2015-05-01

    To report on the correlation of structural damage to the axons of the optic nerve and visual outcome following bilateral non-arteritic anterior ischemic optic neuropathy. A retrospective review of the medical records of 25 patients with bilateral sequential non-arteritic anterior ischemic optic neuropathy was performed. Outcome measures were peripapillary retinal nerve fiber layer thickness measured with the Stratus optical coherence tomography scanner, visual acuity and visual field loss. Median peripapillary retinal nerve fiber layer (RNFL) thickness, mean deviation (MD) of visual field, and visual acuity of initially involved NAION eyes (54.00 µm, -17.77 decibels (dB), 0.4, respectively) were comparable to the same parameters measured following development of second NAION event in the other eye (53.70 µm, p = 0.740; -16.83 dB, p = 0.692; 0.4, p = 0.942, respectively). In patients with bilateral NAION, there was a significant correlation of peripapillary RNFL thickness (r = 0.583, p = 0.002) and MD of the visual field (r = 0.457, p = 0.042) for the pairs of affected eyes, whereas a poor correlation was found in visual acuity of these eyes (r = 0.279, p = 0.176). Peripapillary RNFL thickness following NAION was positively correlated with MD of visual field (r = 0.312, p = 0.043) and negatively correlated with logMAR visual acuity (r = -0.365, p = 0.009). In patients who experience bilateral NAION, the magnitude of RNFL loss is similar in each eye. There is a greater similarity in visual field loss than in visual acuity between the two affected eyes with NAION of the same individual.

  13. Invasão do nervo óptico por melanoma peripapilar: relato de caso Optic nerve invasion by juxtapapillary melanoma: case report

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    Eduardo Ferrari Marback

    2003-06-01

    Full Text Available Tumores pigmentados localizados sobre o disco óptico são raros e representam desafio diagnóstico. Paciente masculino, 60 anos, apresenta baixa da acuidade visual no olho esquerdo devido à lesão pigmentada que cobre o disco óptico. Foi indicada a enucleação com recusa pelo paciente. O quadro evoluiu com descolamento de retina. Examinado em outro serviço teve indicação de vitrectomia também recusada. Retorna aos nossos cuidados; feita a enucleação o diagnóstico anatomopatológico revelou melanoma maligno da coróide com invasão pós-laminar do nervo óptico. A importância prognóstica da invasão do nervo óptico por melanoma da coróide ainda não está totalmente esclarecida. Embora raro, tumor pigmentado cobrindo o nervo óptico pode representar melanoma maligno. O diagnóstico diferencial destes casos é geralmente difícil, porém seu reconhecimento à ultra-sonografia ocular é patente e descolamento de retina associado é sinal de atividade tumoral. Os riscos de disseminação da doença exigem atenção na suspeita diagnóstica e conduta precisa.Small-pigmented lesions over the optic disc are very rare and may represent a diagnostic challenge. To report a case of a small malignant choroidal melanoma invading the optic nerve. A 60-year-old male presents with low vision in the left eye due to a small, pigmented lesion over the optic disc. At first the patient refused enucleation. One month later, after further drop in visual acuity, the patient was seen at another service, diagnosed as having a retinal detachment, and pars plana vitrectomy was proposed but also refused by the patient. Returning to our service, the eye was enucleated and a final diagnosis of choroidal melanoma with post-laminar optic nerve invasion was made. Although rare, pigmented lesions over the optic disc may represent a malignant melanoma. The prognostic significance of optic nerve invasion by choroidal melanoma is not clear yet. The differential

  14. Narrow, duplicated internal auditory canal

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    Ferreira, T. [Servico de Neurorradiologia, Hospital Garcia de Orta, Avenida Torrado da Silva, 2801-951, Almada (Portugal); Shayestehfar, B. [Department of Radiology, UCLA Oliveview School of Medicine, Los Angeles, California (United States); Lufkin, R. [Department of Radiology, UCLA School of Medicine, Los Angeles, California (United States)

    2003-05-01

    A narrow internal auditory canal (IAC) constitutes a relative contraindication to cochlear implantation because it is associated with aplasia or hypoplasia of the vestibulocochlear nerve or its cochlear branch. We report an unusual case of a narrow, duplicated IAC, divided by a bony septum into a superior relatively large portion and an inferior stenotic portion, in which we could identify only the facial nerve. This case adds support to the association between a narrow IAC and aplasia or hypoplasia of the vestibulocochlear nerve. The normal facial nerve argues against the hypothesis that the narrow IAC is the result of a primary bony defect which inhibits the growth of the vestibulocochlear nerve. (orig.)

  15. Multifocal visual evoked potential in optic neuritis, ischemic optic neuropathy and compressive optic neuropathy

    Science.gov (United States)

    Jayaraman, Manju; Gandhi, Rashmin Anilkumar; Ravi, Priya; Sen, Parveen

    2014-01-01

    Purpose: To investigate the effect of optic neuritis (ON), ischemic optic neuropathy (ION) and compressive optic neuropathy (CON) on multifocal visual evoked potential (mfVEP) amplitudes and latencies, and to compare the parameters among three optic nerve disorders. Materials and Methods: mfVEP was recorded for 71 eyes of controls and 48 eyes of optic nerve disorders with subgroups of optic neuritis (ON, n = 21 eyes), ischemic optic neuropathy (ION, n = 14 eyes), and compressive optic neuropathy (CON, n = 13 eyes). The size of defect in mfVEP amplitude probability plots and relative latency plots were analyzed. The pattern of the defect in amplitude probability plot was classified according to the visual field profile of optic neuritis treatment trail (ONTT). Results: Median of mfVEP amplitude (log SNR) averaged across 60 sectors were reduced in ON (0.17 (0.13-0.33)), ION (0.14 (0.12-0.21)) and CON (0.21 (0.14-0.30)) when compared to controls. The median mfVEP relative latencies compared to controls were significantly prolonged in ON and CON group of 10.53 (2.62-15.50) ms and 5.73 (2.67-14.14) ms respectively compared to ION group (2.06 (-4.09-13.02)). The common mfVEP amplitude defects observed in probability plots were diffuse pattern in ON, inferior altitudinal defect in ION and temporal hemianopia in CON eyes. Conclusions: Optic nerve disorders cause reduction in mfVEP amplitudes. The extent of delayed latency noted in ischemic optic neuropathy was significantly lesser compared to subjects with optic neuritis and compressive optic neuropathy. mfVEP amplitudes can be used to objectively assess the topography of the visual field defect. PMID:24088641

  16. Characterization of Pax2 expression in the goldfish optic nerve head during retina regeneration.

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    Marta Parrilla

    Full Text Available The Pax2 transcription factor plays a crucial role in axon-guidance and astrocyte differentiation in the optic nerve head (ONH during vertebrate visual system development. However, little is known about its function during regeneration. The fish visual system is in continuous growth and can regenerate. Müller cells and astrocytes of the retina and ONH play an important role in these processes. We demonstrate that pax2a in goldfish is highly conserved and at least two pax2a transcripts are expressed in the optic nerve. Moreover, we show two different astrocyte populations in goldfish: Pax2(+ astrocytes located in the ONH and S100(+ astrocytes distributed throughout the retina and the ONH. After peripheral growth zone (PGZ cryolesion, both Pax2(+ and S100(+ astrocytes have different responses. At 7 days after injury the number of Pax2(+ cells is reduced and coincides with the absence of young axons. In contrast, there is an increase of S100(+ astrocytes in the retina surrounding the ONH and S100(+ processes in the ONH. At 15 days post injury, the PGZ starts to regenerate and the number of S100(+ astrocytes increases in this region. Moreover, the regenerating axons reach the ONH and the pax2a gene expression levels and the number of Pax2(+ cells increase. At the same time, S100(+/GFAP(+/GS(+ astrocytes located in the posterior ONH react strongly. In the course of the regeneration, Müller cell vitreal processes surrounding the ONH are primarily disorganized and later increase in number. During the whole regenerative process we detect a source of Pax2(+/PCNA(+ astrocytes surrounding the posterior ONH. We demonstrate that pax2a expression and the Pax2(+ astrocyte population in the ONH are modified during the PGZ regeneration, suggesting that they could play an important role in this process.

  17. Study of the neuroprotective effects and mechanisms of Tianma Gouteng Decoction on retinal ganglion cells in rat optic nerve crush model

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    Fan-Tao Lyu

    2018-01-01

    Full Text Available AIM: To observe the mechanism of Tianma Gouteng Decoction on the protein molecular level in the optic nerve crush model rats. METHODS: Totally 36 participants 36 male Wistar rats were divided randomly into six groups(6 in every group: normal control group, negative control group, Tianma Gouteng Decoction treatment groups(con-centrations were 0.6g/mL, 1.2g/mL, 2.4g/mL respictivelyand ginkgo biloba tablets positive control group(concentrations was 1.2mg/mL. Nothing was done in the normal control group. The optic nerve of right eye in the other groups was done with the optic nerve crush model. Normal control group and negative control group was treated only with water. The average grey scale values of the N-methyl-D-aspartic acid receptor 2B(NMDA2Breceptor protein, beta - amyloid protein(Aβin the average grey scale values were detected. RESULTS: The average grey scale value of Tianma Gouteng Decoction in low, medium and high dose groups about NMDA2B receptor protein was significantly less than that of the negative control group(all PP=0.092, 0.411, 0.676, the difference between normal control group and negative control group was significant(PP=0.030, 0.001. The low dose group than the negative control group was not obviously(P=0.614. The high dose group was not significantly different from the positive control group(P=0.927, the difference between normal control group and negative control group was significant(PCONCLUSION: Tianma Gouteng Decoction can go through the decrease of the NMDA2B receptor protein expression and the control of beta-amyloid deposition to reduce the retinal ganglion cell injury and apoptosis.

  18. Sensitivity and specificity of monochromatic photography of the ocular fundus in differentiating optic nerve head drusen and optic disc oedema: optic disc drusen and oedema.

    Science.gov (United States)

    Gili, Pablo; Flores-Rodríguez, Patricia; Yangüela, Julio; Orduña-Azcona, Javier; Martín-Ríos, María Dolores

    2013-03-01

    Evaluation of the efficacy of monochromatic photography of the ocular fundus in differentiating optic nerve head drusen (ONHD) and optic disc oedema (ODE). Sixty-six patients with ONHD, 31 patients with ODE and 70 healthy subjects were studied. Colour and monochromatic fundus photography with different filters (green, red and autofluorescence) were performed. The results were analysed blindly by two observers. The sensitivity, specificity and interobserver agreement (k) of each test were assessed. Colour photography offers 65.5 % sensitivity and 100 % specificity for the diagnosis of ONHD. Monochromatic photography improves sensitivity and specificity and provides similar results: green filter (71.20 % sensitivity, 96.70 % specificity), red filter (80.30 % sensitivity, 96.80 % specificity), and autofluorescence technique (87.8 % sensitivity, 100 % specificity). The interobserver agreement was good with all techniques used: autofluorescence (k = 0.957), green filter (k = 0.897), red filter (k = 0.818) and colour (k = 0.809). Monochromatic fundus photography permits ONHD and ODE to be differentiated, with good sensitivity and very high specificity. The best results were obtained with autofluorescence and red filter study.

  19. Urgent Optic Nerve Decompression via an Endoscopic Endonasal Transsphenoidal Approach for Craniopharyngioma in a 12-Month-Old Infant: A Case Report.

    Science.gov (United States)

    Shibata, Teishiki; Tanikawa, Motoki; Sakata, Tomohiro; Mase, Mitsuhito

    2018-01-01

    Craniopharyngiomas are benign tumors and account for approximately 5.6-13% of all intracranial tumors in children. Diagnosis of pediatric craniopharyngioma is often delayed until the tumor becomes relatively large and manifests severe visual and/or endocrine disturbance. Endoscopic endonasal approaches have recently been introduced to surgery for craniopharyngioma. These techniques, however, have rarely been utilized in patients affected with craniopharyngioma as young as 1 year old. This report documents a 12-month-old male infant with sellar craniopharyngioma who presented with acute total vision loss. To increase the chances of visual recovery, an endoscopic endonasal optic nerve decompression was performed as an urgent procedure. After decompression, which resulted in improvement of his visual disturbance, gross total resection of the tumor was undertaken through an anterior interhemispheric approach at a later date. Tumor mass reduction through an endoscopic endonasal transsphenoidal approach followed by secondary radical total resection under craniotomy was considered to be useful in cases such as this when urgent optic nerve decompression is required. © 2018 S. Karger AG, Basel.

  20. Posterior communicating artery hypoplasia as a risk factor for acute ischemic stroke in the absence of carotid artery occlusion.

    Science.gov (United States)

    Chuang, Yu-Ming; Liu, Chih-Yang; Pan, Po-Jung; Lin, Ching-Po

    2008-12-01

    Posterior communicating artery (PCoA) hypoplasia is a fetal variant of the Circle of Willis. According to angiograms and autopsy reports, this congenital variation is found in 6-21% of the general population. PCoA hypoplasia only becomes a risk factor for ischemic stroke in the presence of ipsilateral internal carotid artery (ICA) occlusion. The aim of our study was to determine the role of PCoA hypoplasia in acute ischemic stroke in the absence of ICA occlusion. We examined 310 acute ischemic stroke patients (mean age+/-standard deviation; 68.9+/-15.6 years). Cerebral magnetic resonance angiography was performed within 72 hours of ischemic stroke onset. For comparison, a risk factor-matched control group was recruited. Conditional logistic regression was used to calculate odds ratios (OR) and 95% confidence intervals (CI) to estimate the independent effect of potential risk factors. The overall incidence of PCoA hypoplasia in our experimental group was 19.35% (n=60), which was significantly higher than in the control group (8.20%, n=22, p=0.036, OR, 3.21; 95% CI, 1.43-9.62). The most common ischemic event was ipsilateral thalamic lacunar infarctions with or without occipital lobe involvement. Based on our results, PCoA hypoplasia appears to be a contributor to the risk of ischemic stroke, even in the absence of ICA occlusion. This risk is especially pronounced for strokes involving arteries that penetrate the thalamus.

  1. Stem Cell Ophthalmology Treatment Study (SCOTS for retinal and optic nerve diseases: a preliminary report

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    Jeffrey N Weiss

    2015-01-01

    Full Text Available In this report, we present the results of a single patient with optic neuropathy treated within the Stem Cell Ophthalmology Treatment Study (SCOTS. SCOTS is an Institutional Review Board approved clinical trial and is the largest ophthalmology stem cell study registered at the National Institutes of Health to date- www.clinicaltrials.gov Identifier NCT 01920867. SCOTS utilizes autologous bone marrow-derived stem cells in the treatment of optic nerve and retinal diseases. Pre- and post-treatment comprehensive eye exams were independently performed at the Wilmer Eye Institute at the Johns Hopkins Hospital, USA. A 27 year old female patient had lost vision approximately 5 years prior to enrollment in SCOTS. Pre-treatment best-corrected visual acuity at the Wilmer Eye Institute was 20/800 Right Eye (OD and 20/4,000 Left Eye (OS. Four months following treatment in SCOTS, the central visual acuity had improved to 20/100 OD and 20/40 OS.

  2. Correction of axial deformity during lengthening in fibular hypoplasia: Hexapodal versus monorail external fixation.

    Science.gov (United States)

    Chalopin, A; Geffroy, L; Pesenti, S; Hamel, A; Launay, F

    2017-09-01

    Childhood fibular hypoplasia is a rare pathology which may or may not involve limb-length discrepancy and axial deformity in one or more dimensions. The objective of the present study was to compare the quality of the axial correction achieved in lengthening procedures by hexapodal versus monorail external fixators. The hypothesis was that the hexapodal fixator provides more precise correction. A retrospective multicenter study included 52 children with fibular hypoplasia. Seventy-two tibias were analyzed, in 2 groups: 52 using a hexapodal fixator, and 20 using a monorail fixator. Mean age was 10.2 years. Mean lengthening was 5.7cm. Deformities were analyzed and measured in 3 dimensions and classified in 4 preoperative types and 4 post-lengthening types according to residual deformity. Complete correction was achieved in 26 tibias in the hexapodal group (50%) and 2 tibias in the monorail group (10%). Mean post-correction mechanical axis deviation was smaller in the hexapodal group: 12.83mm, versus 14.29mm in the monorail group. Mean post-correction mechanical lateral distal femoral angle was 87.5° in the hexapodal group, versus 84.3° in the monorail group (P=0.002), and mean mechanical medial proximal tibial angle 86.9° versus 89.5°, respectively (P=0.015). No previous studies focused on this congenital pathology in lengthening and axial correction programs for childhood lower-limb deformity. The present study found the hexapodal fixator to be more effective in conserving or restoring mechanical axes during progressive bone lengthening for fibular hypoplasia. The hexapodal fixator met the requirements of limb-length equalization in childhood congenital lower-limb hypoplasia, providing better axial correction than the monorail fixator. IV. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  3. The Quantitative Measurements of Vascular Density and Flow Area of Optic Nerve Head Using Optical Coherence Tomography Angiography.

    Science.gov (United States)

    Bazvand, Fatemeh; Mirshahi, Reza; Fadakar, Kaveh; Faghihi, Houshangh; Sabour, Siamak; Ghassemi, Fariba

    2017-08-01

    The purpose of this study was to evaluate the vascular density (VD) and the flow area on optic nerve head (ONH) and peripapillary area, and the impact of age and sex using optical coherence tomography angiography (OCTA) in healthy human subjects. Both eyes of each volunteer were scanned by an RTVue XR Avanti; Optovue with OCTA using the split-spectrum amplitude-decorrelation angiography algorithm technique. Masked graders evaluated enface angiodisc OCTA data. The flow area of ONH and the VD were automatically calculated. A total of 79 eyes of patients with a mean age of 37.03±11.27 were examined. The total ONH (papillary and peripapillary) area VD was 56.03%±4.55%. The flow area of the ONH was 1.74±0.10 mm/1.34 mm. The temporal and inferotemporal peripapillary VD was different between male and female patients. Increasing age causes some changes in the flow area of the ONH and the papillary VD from the third to the fourth decade (analysis of variance test; P<0.05). A normal quantitative database of the flow area and VD of the papillary and peripapillary area, obtained by RTVue XR with OCT angiography technique, is presented here.

  4. Visual Outcome and Tumor Control After Conformal Radiotherapy for Patients With Optic Nerve Sheath Meningioma

    International Nuclear Information System (INIS)

    Arvold, Nils D.; Lessell, Simmons; Bussiere, Marc; Beaudette, Kevin; Rizzo, Joseph F.; Loeffler, Jay S.; Shih, Helen A.

    2009-01-01

    Purpose: Optic nerve sheath meningioma (ONSM) is a rare tumor that almost uniformly leads to visual dysfunction and even blindness without intervention. Because surgical extirpation carries a high risk of postoperative blindness, vision-sparing treatment strategies are desirable. Methods and Materials: We retrospectively reviewed the outcomes of 25 patients (25 optic nerves) with ONSM, treated at a single institution with conformal fractionated radiotherapy by either stereotactic photon or proton radiation. Primary endpoints were local control and visual acuity. Results: The patients presented with symptoms of visual loss (21) or orbital pain (3) or were incidentally diagnosed by imaging (3). The mean age was 44 years, and 64% were female patients. The indication for treatment was the development or progression of symptoms. Of the patients, 13 were treated with photons, 9 were treated with protons, and 3 received a combination of photons and protons. The median dose delivered was 50.4 gray equivalents (range, 45-59.4 gray equivalents). Median follow-up after radiotherapy was 30 months (range, 3-168 months), with 3 patients lost to follow-up. At most recent follow-up, 21 of 22 patients (95%) had improved (14) or stable (7) visual acuity. One patient had worsened visual acuity after initial postirradiation improvement. Of the 22 patients, 20 (95%) had no radiographic progression. Three patients had evidence of asymptomatic, limited retinopathy on ophthalmologic examination, and one had recurrent ONSM 11 years after treatment. Conclusions: Highly conformal, fractionated radiation therapy for symptomatic primary ONSM provides tumor control and improvement in visual function in most cases, with minimal treatment-induced morbidity. Longer follow-up is needed to assess the durability of tumor control and treatment-related late effects.

  5. Hepatocyte growth factor promotes long-term survival and axonal regeneration of retinal ganglion cells after optic nerve injury: comparison with CNTF and BDNF.

    Science.gov (United States)

    Wong, Wai-Kai; Cheung, Anny Wan-Suen; Yu, Sau-Wai; Sha, Ou; Cho, Eric Yu Pang

    2014-10-01

    Different trophic factors are known to promote retinal ganglion cell survival and regeneration, but each had their own limitations. We report that hepatocyte growth factor (HGF) confers distinct advantages in supporting ganglion cell survival and axonal regeneration, when compared to two well-established trophic factors ciliary neurotrophic factor (CNTF) and brain-derived neurotrophic factor (BDNF). Ganglion cells in adult hamster were injured by cutting the optic nerve. HGF, CNTF, or BDNF was injected at different dosages intravitreally after injury. Ganglion cell survival was quantified at 7, 14, or 28 days postinjury. Peripheral nerve (PN) grafting to the cut optic nerve of the growth factor-injected eye was performed either immediately after injury or delayed until 7 days post-injury. Expression of heat-shock protein 27 and changes in microglia numbers were quantified in different growth factor groups. The cellular distribution of c-Met in the retina was examined by anti-c-Met immunostaining. Hepatocyte Growth Factor (HGF) was equally potent as BDNF in promoting short-term survival (up to 14 days post-injury) and also supported survival at 28 days post-injury when ganglion cells treated by CNTF or BDNF failed to be sustained. When grafting was performed without delay, HGF stimulated twice the number of axons to regenerate compared with control but was less potent than CNTF. However, in PN grafting delayed for 7 days after optic nerve injury, HGF maintained a better propensity of ganglion cells to regenerate than CNTF. Unlike CNTF, HGF application did not increase HSP27 expression in ganglion cells. Microglia proliferation was prolonged in HGF-treated retinas compared with CNTF or BDNF. C-Met was localized to both ganglion cells and Muller cells, suggesting HGF could be neuroprotective via interacting with both neurons and glia. Compared with CNTF or BDNF, HGF is advantageous in sustaining long-term ganglion cell survival and their propensity to respond to

  6. Prospective Study on Retinal Nerve Fibre Layer Thickness Changes in Isolated Unilateral Retrobulbar Optic Neuritis

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    Gordon S. K. Yau

    2013-01-01

    Full Text Available Purpose. To investigate the retinal nerve fibre layer (RNFL thickness after unilateral acute optic neuritis using optical coherence tomography (OCT. Patients and Methods. This prospective cohort study recruited consecutive patients with a first episode of isolated, unilateral acute optic neuritis. RNFL thickness and visual acuity (VA of the attack and normal fellow eye were measured at presentation and 3 months in both the treatment and nontreatment groups. Results. 11 subjects received systemic steroids and 9 were treated conservatively. The baseline RNFL thickness was similar in the attack and fellow eye (P≥0.4. At 3 months, the attack eye had a thinner temporal (P=0.02 and average (P=0.05 RNFL compared to the fellow eye. At 3 months, the attack eye had significant RNFL thinning in the 4 quadrants and average thickness (P≤0.0002 compared to baseline. The RNFL thickness between the treatment and nontreatment groups was similar at baseline and 3 months (P≥0.1. Treatment offered better VA at 3 months (0.1 ± 0.2 versus 0.3 ± 0.2 LogMAR, P=0.04. Conclusion. Generalized RNFL thinning occurred at 3 months after a first episode of acute optic neuritis most significantly in the temporal quadrant and average thickness. Visual improvement with treatment was independent of RNFL thickness.

  7. Treatment of severe maxillary cleft hypoplasia in a case with missing premaxilla with anterior maxillary distraction using tooth-borne hyrax appliance

    Directory of Open Access Journals (Sweden)

    Akshai Shetty

    2015-01-01

    Full Text Available Cleft orthodontics generally poses a challenge and a missing premaxilla adds to the difficulty in managing them. The lack of bone support and anterior teeth in a case with missing premaxilla accounts not only for difficulty in rehabilitation but also in increasing the maxillary hypoplasia. This article presents a case report where planned orthodontic and surgical management using distraction has helped treat a severe maxillary hypoplasia in a patient with missing premaxilla. The treatment plan and method can be used to treat severe maxillary hypoplasia and yield reasonably acceptable results for such patients.

  8. Urethral hydrodistension for management of urethral hypoplasia in prune belly syndrome: long-term results.

    Science.gov (United States)

    Kajbafzadeh, Abdol-Mohammad; Rasouli, Mohammad Reza; Dianat, SeyedSaeid; Nezami, Behtash G; Mahboubi, Amir Hassan; Sina, Alireza

    2010-11-01

    The aim of the study was to evaluate the efficacy and safety of urethral hydrodistension for management of urethral hypoplasia in prune belly syndrome (PBS). During a 10-year period, 7 infants with PBS and urethral hypoplasia presented either with open urachus or surgically created urinary diversion referred to our hospital. Five milliliters of normal saline was pushed via a 22-gauge plastic angiocatheter into the urethra with simultaneous finger pressure on the perineum to occlude the proximal urethra that was repeated with higher volumes of the solution (up to 20 mL). The procedure was continued until a 6F or 8F feeding tube catheter confirmed the urethral patency. Hydrodistension was repeated in 3-month intervals till complete patency was confirmed by imaging. Median age of the infants was 6 (1-8) months. All urethral hydrodistension were successful after 1 to 3 sessions. Follow-up imaging studies showed significant improvement in all patients except one. Natural and surgically created urinary diversions were closed in 6 infants. The hydrodistension create an equal and constant pressure into the urethral wall without any urethral damage. This technique can be considered along with the other available methods for management of urethral hypoplasia in selected cases of PBS. Copyright © 2010 Elsevier Inc. All rights reserved.

  9. Size of the Optic Nerve Head and Its Relationship with the Thickness of the Macular Ganglion Cell Complex and Peripapillary Retinal Nerve Fiber Layer in Patients with Primary Open Angle Glaucoma

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    Nobuko Enomoto

    2015-01-01

    Full Text Available Purpose. To evaluate the relationships among the optic nerve head (ONH area, macular ganglion cell complex (mGCC thickness, circumpapillary retinal nerve fiber layer (cpRNFL thickness, and visual field defects in patients with primary open angle glaucoma (POAG. Methods. This retrospective study included 90 eyes of 90 patients with POAG. The ONH area, rim area, mGCC thickness, and cpRNFL thickness were measured using optical coherence tomography. Mean deviation (MD was measured using standard automated perimetry. The relationships among clinical factors including age, refraction, the ONH area, the rim area, the mGCC thickness, the cpRNFL thickness, and MD were evaluated using correlation coefficients and multiple regression analyses. Results. The significant correlation of the ONH area with refraction (r=0.362, P<0.001, the mGCC thickness (r=0.225, P=0.033, and the cpRNFL thickness (r=0.253, P=0.016 was found. Multiple regression analysis showed that the ONH area, rim area, and MD were selected as significant contributing factors to explain the mGCC thickness and cpRNFL thickness. No factor was selected to explain MD. Conclusions. The ONH area, in other words, the disc size itself may affect the mGCC thickness and cpRNFL thickness in POAG patients.

  10. Visual electrophysiology in children

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    Jelka Brecelj

    2005-10-01

    Full Text Available Background: Electrophysiological assessment of vision in children helps to recognise abnormal development of the visual system when it is still susceptible to medication and eventual correction. Visual electrophysiology provides information about the function of the retina (retinal pigment epithelium, cone and rod receptors, bipolar, amacrine, and ganglion cells, optic nerve, chiasmal and postchiasmal visual pathway, and visual cortex.Methods: Electroretinograms (ERG and visual evoked potentials (VEP are recorded non-invasively; in infants are recorded simultaneously ERG with skin electrodes, while in older children separately ERG with HK loop electrode in accordance with ISCEV (International Society for Clinical Electrophysiology of Vision recommendations.Results: Clinical and electrophysiological changes in children with nystagmus, Leber’s congenital amaurosis, achromatopsia, congenital stationary night blindness, progressive retinal dystrophies, optic nerve hypoplasia, albinism, achiasmia, optic neuritis and visual pathway tumours are presented.Conclusions: Electrophysiological tests can help to indicate the nature and the location of dysfunction in unclear ophthalmological and/or neurological cases.

  11. The use of internal maxillary distraction for maxillary hypoplasia: a preliminary report.

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    Van Sickels, Joseph E; Madsen, Mathew J; Cunningham, Larry L; Bird, Douglas

    2006-12-01

    Distraction osteogenesis is a useful alternative to advance the maxilla in complicated cases of maxillary hypoplasia. The purpose of this article is to review the workup, experience, and preliminary results with the use of internal distraction osteogenesis for maxillary hypoplasia at one teaching institution. Over a 5-year period, more than 300 patients with craniofacial and dentofacial defects have undergone oral and maxillofacial surgery at our center to correct their skeletal discrepancies. Of these, 10 have had maxillary distraction osteogenesis done with internal distractors. Follow-up of 6 months or more was available for 8 patients. Stereolithographic models were used to bend distractors prior to surgery in 6 patients. Latency prior to the start of distraction was 3 to 7 days and varied with the age of the patient. Distraction occurred at approximately 1 mm per day with an average distraction length of 8.5 mm (range, 6-10 mm). Excellent occlusal results were obtained in 5 patients. Major complications including nonunion and failure to achieve acceptable occlusal results were observed in 3 patients. Minor complications including pain and loosening of the distracter devices were observed in 2 patients, but did not appear to affect the esthetic and functional results. Distraction osteogenesis is a useful alternative to traditional orthognathic surgery to treat maxillary hypoplasia. Internal distractions are attractive to patients, but are more difficult to place and can cause discomfort to patients when trying to achieve an ideal primary vector of distraction. Stereolithographic models can help with placement of the device. Changes in design of distractors may help with patient discomfort.

  12. Comprehensive evaluation of peripheral nerve regeneration in the acute healing phase using tissue clearing and optical microscopy in a rodent model.

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    Yookyung Jung

    Full Text Available Peripheral nerve injury (PNI, a common injury in both the civilian and military arenas, is usually associated with high healthcare costs and with patients enduring slow recovery times, diminished quality of life, and potential long-term disability. Patients with PNI typically undergo complex interventions but the factors that govern optimal response are not fully characterized. A fundamental understanding of the cellular and tissue-level events in the immediate postoperative period is essential for improving treatment and optimizing repair. Here, we demonstrate a comprehensive imaging approach to evaluate peripheral nerve axonal regeneration in a rodent PNI model using a tissue clearing method to improve depth penetration while preserving neural architecture. Sciatic nerve transaction and end-to-end repair were performed in both wild type and thy-1 GFP rats. The nerves were harvested at time points after repair before undergoing whole mount immunofluorescence staining and tissue clearing. By increasing the optic depth penetration, tissue clearing allowed the visualization and evaluation of Wallerian degeneration and nerve regrowth throughout entire sciatic nerves with subcellular resolution. The tissue clearing protocol did not affect immunofluorescence labeling and no observable decrease in the fluorescence signal was observed. Large-area, high-resolution tissue volumes could be quantified to provide structural and connectivity information not available from current gold-standard approaches for evaluating axonal regeneration following PNI. The results are suggestive of observed behavioral recovery in vivo after neurorrhaphy, providing a method of evaluating axonal regeneration following repair that can serve as an adjunct to current standard outcomes measurements. This study demonstrates that tissue clearing following whole mount immunofluorescence staining enables the complete visualization and quantitative evaluation of axons throughout

  13. Prevalence of dental anomalies and enamel hypoplasia in primary dentition among preschool children of West Godavari District, Andhra Pradesh -A cross - sectional study

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    Suzan Sahana

    2013-01-01

    Full Text Available Background: It is axiomatic that Pediatric dental anomalies and enamel hypoplasia (E.H are routinely encountered in primary dentition and early detection and prudent management of the condition facilitates normal occlusal development. Objectives: To determine the prevalence of various dental anomalies and enamel hypoplasia in preschool children between two to six years of age. Materials & Method: A total of 1898 children, between two to six years were randomly selected and screened for dental anomalies and enamel hypoplasia The chi square test was used to analyze the data statistically. Results: The overall prevalence rate of dental anomalies and enamel hypoplasia in this study was 0.63% and 8.95% respectively. Double teeth were the most frequently reported dental anomaly while supernumerary teeth were least reported. None of them reported with hypodontia.

  14. Ischio-pubic-patellar hypoplasia: is it a new syndrome?

    International Nuclear Information System (INIS)

    Habboub, H.K.; Thneibat, W.A.

    1997-01-01

    Aplasia/hypoplasia of the patella has been described as an isolated finding or, more commonly, as a part of congenital syndromes. We describe here bilateral absence of the patella in an 11-year-old girl with absence of the ischial and inferior pubic rami bilaterally. Other associated skeletal and soft-tissue deformities are also reported. To our knowledge, the constellation of these findings has not been described previously and represents a unique syndrome. (orig.). With 1 fig

  15. Serial Diffusion Tensor Imaging of the Optic Radiations after Acute Optic Neuritis

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    Scott C. Kolbe

    2016-01-01

    Full Text Available Previous studies have reported diffusion tensor imaging (DTI changes within the optic radiations of patients after optic neuritis (ON. We aimed to study optic radiation DTI changes over 12 months following acute ON and to study correlations between DTI parameters and damage to the optic nerve and primary visual cortex (V1. We measured DTI parameters [fractional anisotropy (FA, axial diffusivity (AD, radial diffusivity (RD, and mean diffusivity (MD] from the optic radiations of 38 acute ON patients at presentation and 6 and 12 months after acute ON. In addition, we measured retinal nerve fibre layer thickness, visual evoked potential amplitude, optic radiation lesion load, and V1 thickness. At baseline, FA was reduced and RD and MD were increased compared to control. Over 12 months, FA reduced in patients at an average rate of −2.6% per annum (control = −0.51%; p=0.006. Change in FA, RD, and MD correlated with V1 thinning over 12 months (FA: R=0.450, p=0.006; RD: R=-0.428, p=0.009; MD: R=-0.365, p=0.029. In patients with no optic radiation lesions, AD significantly correlated with RNFL thinning at 12 months (R=0.489, p=0.039. In conclusion, DTI can detect optic radiation changes over 12 months following acute ON that correlate with optic nerve and V1 damage.

  16. Recurrent Vitreous Hemorrhage from an Optic Nerve Retinal Arterial Macroaneurysm

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    Jessica F. Yang

    2017-10-01

    Full Text Available Objective: To report a case of recurrent vitreous hemorrhage from an optic nerve retinal arterial macroaneurysm (ONRAM successfully treated with intraoperative endolaser. Patient and Methods: A 92-year-old woman on oral aspirin and warfarin anticoagulation for atrial fibrillation developed three episodes of dense vitreous hemorrhage from an ONRAM. Due to failure of the vitreous hemorrhage to clear spontaneously, a total of three pars plana vitrectomy (PPV procedures were performed along with a 1.25-mg intravitreal bevacizumab injection after the third episode of hemorrhage. During the third PPV procedure, a 25-gauge 532-nm green diode laser endoprobe was used to deliver low-power (100 mW and long-duration (500 ms laser spots directly on the ONRAM to induce intraoperative shrinkage of the ONRAM. Results: After the endolaser treatment, the macroaneurysm showed involution due to fibrosis without any adverse effects on retinal circulation or visual field defect. No recurrence of vitreous hemorrhage was noted after 2 years of follow-up. Conclusion: Oral anticoagulant use may have been responsible for the atypical clinical course in our patient. Laser photocoagulation, including intraoperative endolaser photocoagulation, may be considered in selected cases of symptomatic ONRAMs.

  17. Unilateral hypoplasia with contralateral hypertrophy of anterior belly of digastric muscle: a case report.

    Science.gov (United States)

    Ochoa-Escudero, Martin; Juliano, Amy F

    2016-10-01

    Anomalies of the anterior belly of the digastric muscle (DM) are uncommon. We present a case of hypoplasia of the anterior belly of the left DM with hypertrophy of the anterior belly of the contralateral DM. The importance of recognizing this finding is to differentiate hypoplasia of the anterior belly of the DM from denervation atrophy, and not to confuse contralateral hypertrophy with a submental mass or lymphadenopathy. In denervation atrophy of the anterior belly of the DM, associated atrophy of the ipsilateral mylohyoid muscle is present. Hypertrophy of the anterior belly of the contralateral DM can be differentiated from a submental mass or lymphadenopathy by recognizing its isodensity on computed tomography and isointensity on magnetic resonance imaging to other muscles, without abnormal contrast enhancement.

  18. Optic Disc and Retinal Nerve Fiber Layer Thickness Evaluation of the Fellow Eyes in Non-Arteritic Ischemic Optic Neuropathy

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    Medine Yılmaz Dağ

    2015-05-01

    Full Text Available Objectives: To examine the fellow eyes in unilateral non-arteritic ischemic optic neuropathy (NAION and to compare their optic disc parameters and peripapillary retinal nerve fiber layer (RNFL thickness with age-and refraction-matched normal controll subjects, using Heidelberg Retinal Tomograph 2 (HRT II. Materials and Methods: The fellow eyes of 40 patients with typical unilateral NAION (study group and one randomly chosen eye of 42 age-, sex-, and refraction-matched normal control subjects were enrolled in the study. Optic disc morphologic features (average disc area, cup area, rim area, disc volume, rim volume, cup/disc area ratio, cup depth and peripapillary RNFL thickness were evaluated using HRT II, a confoal scanning ophtalmoscopy. Results: In the study group, there were 26 (65% men and 14 (35% women, whereas there were 27 (64% men and 15 (36% women in the control group (Chi square test, p=0.89. Mean age of the patients in the study and control groups was 59.4±10.3 and 57.7±9.1 years, respectively (T test, p=0.72. There was not any statistically significant difference regarding mean spheric equivalent between the two groups (Mann-Whitney U-test, p=0.203. The NAION unaffected fellow eyes had significantly smaller disc areas, cup areas, cup volumes, cup-disc area ratios (vertical and lineer, and cup depths than the control eyes (Mann-Whitney U-test; p<0.05, whereas there was no significant difference in the RNFL thickness between the two. Conclusion: A comparison of the fellow eyes in patients with unilateral NAION and the control eyes showed a significant difference in optic disc parameters and the morphology of RNFL. These differences could be important in the pathogenesis of NAION and needs to have further investigated. (Turk J Ophthalmol 2015; 45: 111-114

  19. Measurement of wavefront aberrations in cortex and peripheral nerve using a two-photon excitation guidestar

    Science.gov (United States)

    Futia, Gregory L.; Fontaine, Arjun; McCullough, Connor; Ozbay, Baris N.; George, Nickolas M.; Caldwell, John; Restrepo, Diego; Weir, Richard; Gibson, Emily A.

    2018-02-01

    Neural-machine interfaces using optogenetics are of interest due to their minimal invasiveness and potential for parallel read in and read out of activity. One possible biological target for such an interface is the peripheral nerve, where axonlevel imaging or stimulation could greatly improve interfacing with artificial limbs or enable neuron/fascicle level neuromodulation in the vagus nerve. Two-photon imaging has been successful in imaging brain activity using genetically encoded calcium or voltage indicators, but in the peripheral nerve, this is severely limited by scattering and aberrations from myelin. We employ a Shack-Hartman wavefront sensor and two-photon excitation guidestar to quantify optical scattering and aberrations in peripheral nerves and cortex. The sciatic and vagus nerves, and cortex from a ChAT-Cre ChR-eYFP transgenic mouse were excised and imaged directly. In peripheral nerves, defocus was the strongest aberration followed by astigmatism and coma. Peripheral nerve had orders of magnitude higher aberration compared with cortex. These results point to the potential of adaptive optics for increasing the depth of two-photon access into peripheral nerves.

  20. Indirect veneer treatment of anterior maxillary teeth with enamel hypoplasia

    OpenAIRE

    Juniarti, Devi Eka

    2010-01-01

    Background: Nowadays, aesthetic rehabilitation becomes a necessity. It is affected by patient’s background, especially career, social and economic status. The aesthetic abnormality of anterior teeth i.e discoloration, malposition and malformation can affect patient’s appearance, especially during smile. These dental abnormalities, as a result, can decrease patient’s performance. Dental malformation, for instance, can be caused by developmental tooth defect, such as enamel hypoplasia. Enamel h...

  1. Optic chiasm in the species of order Clupeiformes, family Clupeidae: optic chiasm of Spratelloides gracilis shows an opposite laterality to that of Etrumeus teres.

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    Mogi, Kazue; Misawa, Kazuya; Utsunomiya, Kentaro; Kawada, Yuta; Yamazaki, Toshihisa; Takeuchi, Shigeo; Toyoizumi, Ryuji

    2009-09-01

    In most teleost fishes, the optic nerves decussate completely as they project to the mesencephalic region. Examination of the decussation pattern of 25 species from 11 different orders in Pisces revealed that each species shows a specific chiasmic type. In 11 species out of the 25, laterality of the chiasmic pattern was not determined; in half of the individuals examined, the left optic nerve ran dorsally to the right optic nerve, while in the other half, the right optic nerve was dorsal. In eight other species the optic nerves from both eyes branched into several bundles at the chiasmic point, and intercalated to form a complicated decussation pattern. In the present study we report our findings that Spratelloides gracilis, of the order Clupeiformes, family Clupeidae, shows a particular laterality of decussation: the left optic nerve ran dorsally to the right (n=200/202). In contrast, Etrumeus teres, of the same order and family, had a strong preference of the opposite (complementary) chiasmic pattern to that of S. gracilis (n=59/59), revealing that these two species display opposite left-right optic chiasm patterning. As far as we investigated, other species of Clupeiformes have not shown left-right preference in the decussation pattern. We conclude that the opposite laterality of the optic chiasms of these two closely related species, S. gracilis and E. teres, enables investigation of species-specific laterality in fishes of symmetric shapes.

  2. Correlation of Optic Nerve Microcirculation with Papillomacular Bundle Structure in Treatment Naive Normal Tension Glaucoma

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    Wataru Kobayashi

    2014-01-01

    Full Text Available Purpose. To assess the association between optic nerve head (ONH microcirculation, central papillomacular bundle (CPB structure, and visual function in eyes with treatment naive normal tension glaucoma (NTG. Methods. This study included 40 eyes of 40 patients with NTG and 20 eyes of 20 normal patients. We used laser speckle flowgraphy (LSFG to measure mean blur rate (MBR in all eyes and calculated the ratio of MBR in the horizontal quadrants of tissue area ONH (temporal/nasal ratio of MBR in the tissue area: T/N MT. Clinical findings also included retinal nerve fiber layer thickness (RNFLT and ganglion cell complex thickness (GCCT in the CPB and macular areas, best-corrected visual acuity (BCVA, mean deviation (MD, and refractive error. Results. T/N MT was correlated with both BCVA and MD. The OCT parameters most highly correlated with T/N MT were macular RNFLT and mid-CPB RNFLT. Furthermore, T/N MT, mid-CPB RNFLT, and macular RNFLT were higher in NTG than in normal eyes. A discrimination analysis revealed that T/N MT and refractive error were independent factors indicating NTG. Conclusions. Our results suggest that T/N MT is a candidate biomarker of NTG. Furthermore, T/N MT reflects visual function, including acuity and sensitivity, and CPB structure.

  3. Automatic computer-aided diagnosis of retinal nerve fiber layer defects using fundus photographs in optic neuropathy.

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    Oh, Ji Eun; Yang, Hee Kyung; Kim, Kwang Gi; Hwang, Jeong-Min

    2015-05-01

    To evaluate the validity of an automatic computer-aided diagnosis (CAD) system for detection of retinal nerve fiber layer (RNFL) defects on fundus photographs of glaucomatous and nonglaucomatous optic neuropathy. We have proposed an automatic detection method for RNFL defects on fundus photographs in various cases of glaucomatous and nonglaucomatous optic neuropathy. In order to detect the vertical dark bands as candidate RNFL defects, the nonuniform illumination of the fundus image was corrected, the blood vessels were removed, and the images were converted to polar coordinates with the center of the optic disc. False positives (FPs) were reduced by using knowledge-based rules. The sensitivity and FP rates for all images were calculated. We tested 98 fundus photographs with 140 RNFL defects and 100 fundus photographs of healthy normal subjects. The proposed method achieved a sensitivity of 90% and a 0.67 FP rate per image and worked well with RNFL defects with variable depths and widths, with uniformly high detection rates regardless of the angular widths of the RNFL defects. The average detection accuracy was approximately 0.94. The overall diagnostic accuracy of the proposed algorithm for detecting RNFL defects among 98 patients and 100 healthy individuals was 86% sensitivity and 75% specificity. The proposed CAD system successfully detected RNFL defects in optic neuropathies. Thus, the proposed algorithm is useful for the detection of RNFL defects.

  4. Diffuse large B-cell lymphoma chemotherapy reveals a combined immunodeficiency syndrome in cartilage hair hypoplasia.

    Science.gov (United States)

    Nguyen, Alexandre; Martin Silva, Nicolas; de Boysson, Hubert; Damaj, Gandhi; Aouba, Achille

    2018-04-24

    Cartilage hair hypoplasia (CHH) is a rare autosomal recessive ribosomopathy characterised by skeletal and integumentary system manifestations. It may also present with varied forms and intensities of haematopoietic and/or immune disorders. We report a 27-year-old female who presented a picture of combined immunodeficiency after receiving an adriamycin-based chemotherapy regimen followed by autologous stem cell transplantation. Her medical history indicated neonatal dwarfism, recurrent ear, nose and throat and respiratory infections, and hypogammaglobulinaemia, which were suggestive of a primary minor B-cell immune deficiency. Taken together, the diagnosis of cartilage hair hypoplasia was suspected and confirmed by means of molecular biological analysis. Here, we discuss the causal relationship and molecular mechanisms existing between both primary immunodeficiency and lymphoma conditions and between chemotherapy cytotoxicity and aggravation of the immune system and associated hematopoietic dysfunction, considering the role of all these components in light of the initially undiagnosed cartilage hair hypoplasia. Finally, this case highlights the importance of screening for primary immunodeficiencies in the setting of a diagnosis of lymphoma and/or dwarfism; moreover, CHH must be distinguished from other causes of small size; its diagnosis and complete check-up must include the molecular characterisation, and its management must be global in collaboration with haematologists, immunologists and internists.

  5. MRI findings and correlative study of MRI and visual evoked potentials in optic neuritis

    International Nuclear Information System (INIS)

    Yan Fei; Li Jing; Wang Zhenchang; Liu Shoubin; Zhang Xiaojun

    2008-01-01

    Objective: To investigate the effective MRI sequences and describe the correlation between MRI and visual evoked potential (VEP) in diagnosing optic neuritis. Methods: One hundred and fifty-four eyes with visual impairment of 98 patients with diagnoses of optic neuritis, papillitis, multiple sclerosis and Devic's disease underwent MRI and VEP examination. The MRI findings were analyzed and correlated with VEP results and clinical presentation by using X 2 test, wilco xon test and Kappa test. Results Out of the 154 sick eyes, 56 eyes presented thickened optic nerves, 76 eyes had normal diameter of the optic nerve, and 22 eyes had thin optic nerves. A total of 132 optic nerves showed abnormally high signal in STIR sequences, including involvement of intraocular segment in 7, intraorbital segment in 1.35, intracanalicular segment in 109, intracranial segment in 97, optic chiasm in 56, and optic tract in 23. A total of 54 patients underwent postcontrast MRI. Seventy-four optic nerves of 87 eyes showed enhancement. Among the 196 eyes of 98 patients, 132 eyes presented visual impairment and simultaneous abnormal MR signal of the optic nerve, and 26 eyes had both normal vision and normal MR signal of optic nerve. The consistency of MRI findings and vision status was 80. 61% (Kappa 0.453,P 1 -weighted MR sequence combined with fat- suppression are helpful in diagnosis of optic neuritis. VEP is helpful in diagnosing optic neuritis and in finding subclinical visual problem. The MRI combined with VEP could improve the diagnostic accuracy of optic neuritis. (authors)

  6. Measurement of retinal nerve fiber layer thickness in eyes with optic disc swelling by using scanning laser polarimetry and optical coherence tomography.

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    Hata, Masayuki; Miyamoto, Kazuaki; Oishi, Akio; Kimura, Yugo; Nakagawa, Satoko; Horii, Takahiro; Yoshimura, Nagahisa

    2014-01-01

    The retinal nerve fiber layer thickness (RNFLT) in patients with optic disc swelling of different etiologies was compared using scanning laser polarimetry (SLP) and spectral-domain optical coherence tomography (OCT). Forty-seven patients with optic disc swelling participated in the cross-sectional study. Both GDx SLP (enhanced corneal compensation) and Spectralis spectral-domain OCT measurements of RNFLT were made in 19 eyes with papilledema (PE), ten eyes with optic neuritis (ON), and 18 eyes with nonarteritic anterior ischemic optic neuropathy (NAION) at the neuro-ophthalmology clinic at Kyoto University Hospital. Differences in SLP (SLP-RNFLT) and OCT (OCT-RNFLT) measurements among different etiologies were investigated. No statistical differences in average OCT-RNFLT among PE, ON, and NAION patients were noted. Average SLP-RNFLT in NAION patients was smaller than in PE (P<0.01) or ON (P=0.02) patients. When RNFLT in each retinal quadrant was compared, no difference among etiologies was noted on OCT, but on SLP, the superior quadrant was thinner in NAION than in PE (P<0.001) or ON (P=0.001) patients. Compared with age-adjusted normative data of SLP-RNFLT, average SLP-RNFLT in PE (P<0.01) and ON (P<0.01) patients was greater. Superior SLP-RNFLT in NAION patients was smaller (P=0.026). The ratio of average SLP-RNFLT to average OCT-RNFLT was smaller in NAION than in PE (P=0.001) patients. In the setting of RNFL thickening, despite increased light retardance in PE and ON eyes, SLP revealed that NAION eyes have less retardance, possibly associated with ischemic axonal loss.

  7. Radiation optic neuropathy after megavoltage external-beam irradiation: Analysis of time-dose factors

    International Nuclear Information System (INIS)

    Parsons, J.T.; Bova, F.J.; Million, R.R.

    1994-01-01

    To investigate the risk of radiation-induced optic neuropathy according to total radiotherapy dose and fraction size, based on both retrospective and prospectively collected data. Between October 1964 and May 1989, 215 optic nerves in 131 patients received fractionated external-beam irradiation during the treatment of primary extracranial head and neck tumors. All patients had a minimum of 3 years of ophthalmologic follow-up (range, 3 to 21 years). The clinical end point was visual acuity of 20/100 or worse as a result of optic nerve injury. Anterior ischemic optic neuropathy developed in five nerves (at mean and median times of 32 and 30 months, respectively, and a range of 2-4 years). Retrobulbar optic neuropathy developed in 12 nerves (at mean and median times of 47 and 28 months, respectively, and a range of 1-14 years). No injuries were observed in 106 optic nerves that received a total dose of <59 Gy. Among nerves that received doses of ≥ 60 Gy, the dose per fraction was more important than the total dose in producing optic neuropathy. The 15-year actuarial risk of optic compared with 47% when given in fraction sizes ≥1.9 Gy. The data also suggest an increased risk of optic nerve injury with increasing age. As there is no effective treatment of radiation-induced optic neuropathy, efforts should be directed at its prevention by minimizing the total dose, paying attention to the dose per fraction to the nerve, and using reduced field techniques where appropriate to limit the volume of tissues that receive high-dose irradiation. 32 refs., 5 figs., 5 tabs

  8. Laser-activated protein solder for peripheral nerve repair

    Science.gov (United States)

    Trickett, Rodney I.; Lauto, Antonio; Dawes, Judith M.; Owen, Earl R.

    1995-05-01

    A 100 micrometers core optical fiber-coupled 75 mW diode laser operating at a wavelength of 800 nm has been used in conjunction with a protein solder to stripe weld severed rat tibial nerves, reducing the long operating time required for microsurgical nerve repair. Welding is produced by selective laser denaturation of the albumin based solder which contains the dye indocyanine green. Operating time for laser soldering was 10 +/- 5 min. (n equals 20) compared to 23 +/- 9 min. (n equals 10) for microsuturing. The laser solder technique resulted in patent welds with a tensile strength of 15 +/- 5 g, while microsutured nerves had a tensile strength of 40 +/- 10 g. Histopathology of the laser soldered nerves, conducted immediately after surgery, displayed solder adhesion to the outer membrane with minimal damage to the inner axons of the nerves. An in vivo study is under way comparing laser solder repaired tibial nerves to conventional microsuture repair. At the time of submission 15 laser soldered nerves and 7 sutured nerves were characterized at 3 months and showed successful regeneration with compound muscle action potentials of 27 +/- 8 mV and 29 +/- 8 mW respectively. A faster, less damaging and long lasting laser based anastomotic technique is presented.

  9. Morphometric Optic Nerve Head Analysis in Glaucoma Patients: A Comparison between the Simultaneous Nonmydriatic Stereoscopic Fundus Camera (Kowa Nonmyd WX3D and the Heidelberg Scanning Laser Ophthalmoscope (HRT III

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    Siegfried Mariacher

    2016-01-01

    Full Text Available Purpose. To investigate the agreement between morphometric optic nerve head parameters assessed with the confocal laser ophthalmoscope HRT III and the stereoscopic fundus camera Kowa nonmyd WX3D retrospectively. Methods. Morphometric optic nerve head parameters of 40 eyes of 40 patients with primary open angle glaucoma were analyzed regarding their vertical cup-to-disc-ratio (CDR. Vertical CDR, disc area, cup volume, rim volume, and maximum cup depth were assessed with both devices by one examiner. Mean bias and limits of agreement (95% CI were obtained using scatter plots and Bland-Altman analysis. Results. Overall vertical CDR comparison between HRT III and Kowa nonmyd WX3D measurements showed a mean difference (limits of agreement of −0.06 (−0.36 to 0.24. For the CDR < 0.5 group (n=24 mean difference in vertical CDR was −0.14 (−0.34 to 0.06 and for the CDR ≥ 0.5 group (n=16 0.06 (−0.21 to 0.34. Conclusion. This study showed a good agreement between Kowa nonmyd WX3D and HRT III with regard to widely used optic nerve head parameters in patients with glaucomatous optic neuropathy. However, data from Kowa nonmyd WX3D exhibited the tendency to measure larger CDR values than HRT III in the group with CDR < 0.5 group and lower CDR values in the group with CDR ≥ 0.5.

  10. Head-down posture in glaucoma suspects induces changes in IOP, systemic pressure and PERG that predict future loss of optic nerve tissue

    Science.gov (United States)

    Porciatti, Vittorio; Feuer, William J.; Monsalve, Pedro; Triolo, Giacinto; Vazquez, Luis; McSoley, John; Ventura, Lori M.

    2017-01-01

    Purpose To obtain pilot data on posture-induced changes of IOP, systemic pressure and pattern electroretinogram (PERG) predictive of future optic nerve tissue loss glaucoma suspects (GS). Methods Mean peripapillary retinal fiber layer thickness (RNFLT) was measured with OCT two times/year in 28 GS aged 58 ± 8.9 years over 5.0 ± 0.73 years. All patients had a baseline PERG, IOP and brachial blood pressure measurements in the seated and – 10 degrees head-down-body-tilt position (HDT). Outcome measures were seated/HDT PERG amplitude and phase, IOP, mean arterial blood pressure (MAP), and estimated ocular perfusion pressure (OPP). An additional group of 11 similarly-aged controls (SAC) aged 56.9 ± 13 years was tested for comparison. Results While all GS had initial RNFLT in the normal range, 9/28 of them developed significant (P blood pressure, together with their changes upon HDT, may have predictive value for future loss of optic nerve tissue in GS. This study supports the rationale for a full-scale clinical trial to identify patients at high-risk of development of glaucoma. PMID:28263259

  11. Radiation optic neuropathy

    International Nuclear Information System (INIS)

    Kline, L.B.; Kim, J.Y.; Ceballos, R.

    1985-01-01

    Following surgery for pituitary adenoma, radiation therapy is an accepted treatment in reducing tumor recurrence. However, a potential therapeutic complication is delayed radionecrosis of perisellar neural structures, including the optic nerves and chiasm. This particular cause of visual loss, radiation optic neuropathy (RON), has not been emphasized in the ophthalmologic literature. Four cases of RON seen in the past five years are reported. Diagnostic criteria include: (1) acute visual loss (monocular or binocular), (2) visual field defects indicating optic nerve or chiasmal dysfunction, (3) absence of optic disc edema, (4) onset usually within three years of therapy (peak: 1-1 1/2 years), and (5) no computed tomographic evidence of visual pathway compression. Pathologic findings, differential diagnosis and therapy will be discussed in outlining the clinical profile of RON

  12. A detailed study of enamel hypoplasia in a post-medieval adolescent of known age and sex.

    Science.gov (United States)

    King, T; Hillson, S; Humphrey, L T

    2002-01-01

    Developmental disturbances that affect the secretion of enamel matrix can cause defective enamel structure. Linear hypoplasia is one type of enamel defect and manifests itself as a furrow that runs around the circumference of the tooth. Such defects range in size from the microscopic to those that are several millimetres wide. Enamel defects have been widely used by anthropologists for the investigation of growth disruptions in past populations, as they provide a permanent record of disturbances during much of a child's developmental period. This is a detailed case study of enamel growth disruptions in a 15-year-old female from the 18th and 19th century crypt of Christ Church, Spitalfields. The method used relates linear enamel hypoplasia to the incremental structures in the enamel surface, the perikymata, in order to investigate the timing of growth disturbances. Linear enamel hypoplasia was defined here as a greater than expected spacing between neighbouring pairs of perikymata. In addition, this study used recently published histological data on the precise timing of tooth development to establish chronologies for growth disruptions. Defects were matched in at least two teeth with overlapping developmental schedules to ensure that systemic disturbances, as opposed to localised traumas, were identified. Thirteen enamel defects were matched between five different teeth from the same individual from Spitalfields. Most linear enamel hypoplasias were evident on the anterior dentition. Using an 8-day average perikymata periodicity, the age at first defect in this individual was calculated as 1.5 years and the last growth disruption occurred when she was 4.6 years of age. The distribution of the defects was examined to identify any seasonal pattern in the occurrence of the growth disturbances.

  13. Osteopathia striata: a characteristic X-ray finding in focal dermal hypoplasia (Goltz-Gorlin syndrome)

    Energy Technology Data Exchange (ETDEWEB)

    Barthels, W.; Boepple, D.; Petzel, H.

    1982-12-01

    Two cases of the very rare Goltz-Gorlin syndrome are presented. The relationship of osteopathia striata and focal dermal hypoplasia is discussed, and it is concluded that the osteopathia striata represents the characteristic picture of this ectopic mesodermal abnormality.

  14. Peripapillary Retinal Nerve Fiber Layer Thickness Corresponds to Drusen Location and Extent of Visual Field Defects in Superficial and Buried Optic Disc Drusen.

    Science.gov (United States)

    Malmqvist, Lasse; Wegener, Marianne; Sander, Birgit A; Hamann, Steffen

    2016-03-01

    Optic disc drusen (ODD) are hyaline deposits located within the optic nerve head. Peripapillary retinal nerve fiber layer (RNFL) thinning is associated with the high prevalence of visual field defects seen in ODD patients. The goal of this study was to investigate the characteristics of patients with ODD and to compare the peripapillary RNFL thickness to the extent of visual field defects and anatomic location (superficial or buried) of ODD. Retrospective, cross sectional study. A total of 149 eyes of 84 ODD patients were evaluated. Sixty-five percent were female and 76% had bilateral ODD. Of 149 eyes, 109 had superficial ODD and 40 had buried ODD. Peripapillary RNFL thinning was seen in 83.6% of eyes, where optical coherence tomography was performed (n = 61). Eyes with superficial ODD had greater mean peripapillary RNFL thinning (P ≤ 0.0001) and visual field defects (P = 0.002) than eyes with buried ODD. There was a correlation between mean peripapillary RNFL thinning and visual field defects as measured by perimetric mean deviation (R-0.66; P = 0.0001). The most frequent visual field abnormalities were arcuate and partial arcuate defects. Peripapillary RNFL thickness correlates with anatomic location (superficial or buried) of ODD. Frequency and extent of visual field defects corresponded with anatomic location of ODD and peripapillary RNFL thickness, suggesting increased axonal damage in patients with superficial ODD.

  15. Non-Invasive Study of Nerve Fibres using Laser Interference Microscopy

    DEFF Research Database (Denmark)

    Brazhe, A. R.; Brazhe, N. A.; Rodionova, N. N.

    2008-01-01

    This paper presents the results of a laser interference microscopy study of the morphology and dynamical properties of myelinated nerve fibres. We describe the principles of operation of the phase-modulated laser interference microscope and show how this novel technique allows us to obtain...... information non-invasively about the internal structure of different regions of a nerve fibre. We also analyse the temporal variations in the internal optical properties in order to detect the rhythmic activity in the nerve fibre at different time scales and to shed light on the underlying biological...

  16. Terminal nerve: cranial nerve zero

    Directory of Open Access Journals (Sweden)

    Jorge Eduardo Duque Parra

    2006-12-01

    Full Text Available It has been stated, in different types of texts, that there are only twelve pairs of cranial nerves. Such texts exclude the existence of another cranial pair, the terminal nerve or even cranial zero. This paper considers the mentioned nerve like a cranial pair, specifying both its connections and its functional role in the migration of liberating neurons of the gonadotropic hormone (Gn RH. In this paper is also stated the hypothesis of the phylogenetic existence of a cerebral sector and a common nerve that integrates the terminal nerve with the olfactory nerves and the vomeronasals nerves which seem to carry out the odors detection function as well as in the food search, pheromone detection and nasal vascular regulation.

  17. Unusual right ventricle aneurysm and dysplastic pulmonary valve with mitral valve hypoplasia

    Directory of Open Access Journals (Sweden)

    Ozge Pamukcu

    2013-01-01

    Full Text Available We report a newborn with an unusual combination of aneurysmally dilated thin-walled right ventricle with hypertrophy of the apical muscles of the right ventricle. There was narrow pulmonary annulus, pulmonary regurgitation, and hypoplasia of the mitral valve and left ventricle. We propose that this heart represents a partial form of Uhl`s anomaly.

  18. POSITIONAL VARIATION OF OPTIC NERVE IN RELATION TO SPHENOID SINUSES AND ITS ASSOCIATION WITH PNEUMATISATION OF ANTERIOR CLINOID PROCESS : A RADIOLOGICAL STUDY

    Directory of Open Access Journals (Sweden)

    Santhana Lakshmi

    2015-08-01

    Full Text Available OBJECTIVE: The posterior most among the paranasal sinuses, the sphenoid sinuses exhibit high variability in their structure, pneumatisation and relation to surrounding neurovascular structures. The protrusion of optic nerve (ON into the superolateral wall of the sinus has been reported in literature wit h varied incidence. The pneumatisation of sphenoid sinus and its extension to anterior clinoid process (ACP is also been mentioned in few studies. The variability in the incidence and the inconsistency in the association between optic nerve protrusion and degree of pneumatisation seen in studies done in different ethnicity and with paucity of Indian studies necessitated this study on positional variation of optic nerve in relation to sphenoid sinuses and its association with pneumatisation of anterior clin oid process in South Indian ethnicity. THE METHODS: CT scan images in coronal section collected from 114 patients with sinusitis with in the age group of 16 - 64 years belonging to both sexes were studied. The CT images were evaluated for the position of ON with sphenoid sinuses, protrusion of it into the sinus walls, bony dehiscence, pneumatisation of ACP. The position of ON was classified into Delano’s four types and their incidence noted. RESULTS: Type 1 position of ON was observed predominantly in 65.8% s ides while Type 2, 3, 4 were seen in 29.8%, 1.8% and 2.6% sides respectively out of 228 sides studied. Associated bony dehiscence was noted in only 5 out of 228sides (2.1% studied. The pneumatisation of ACP was observed in 23.6% of the CT scans studied. T he association between ON protrusion and ACP pneumatisation was found to be statistically significant with P= 0.008 . CONCLUSION: The varying position of ON, its protrusion with or without dehiscence in to the sphenoid sinus wall with statistically signific ant association with ACP pneumatisation in south Indian ethnicity warrants a systematic CT evaluation pre operatively to ensure

  19. Osteopathia striata: A characteristic X-ray finding in focal dermal hypoplasia (Goltz-Gorlin syndrome)

    International Nuclear Information System (INIS)

    Barthels, W.; Boepple, D.; Petzel, H.

    1982-01-01

    Two cases of the very rare Goltz-Gorlin syndrome are presented. The relationship of osteopathia striata and focal dermal hypoplasia is discussed, and it is concluded that the osteopathia striata represents the characteristic picture of this ectopic mesodermal abnormality. (orig.)

  20. Thanatophoric dysplasia type II with encephalocele and aortic hypoplasia diagnosed in an anatomical specimen

    NARCIS (Netherlands)

    Jap-A-Joe, Simone M. E. A. A.; Oostra, Roelof-Jan; Maas, Mario; Stoker, Jaap; van der Horst, Chantal M. A. M.

    2003-01-01

    A hitherto unknown combination of congenital anomalies was found in an anatomical specimen of a female neonate. External examination and additional CT and MRI studies showed thanatophoric dysplasia type II with cloverleaf skull and concomitant parietal meningoencephalocele and hypoplasia of the

  1. Lesion discrimination in optic neuritis using high-resolution fat-suppressed fast spin-echo MRI

    International Nuclear Information System (INIS)

    Gass, A.; Moseley, I.F.; Barker, G.J.; Jones, S.; MacManus, D.; McDonald, W.I.; Miller, D.H.

    1996-01-01

    Fast spin-echo (FSE) is a new sequence with acquisition times currently down to one-sixteenth of those obtained with conventional spin-echo sequences, which allows high-resolution (512 x 512 matrix) images to be acquired in an acceptable time. We compared the higher resolution of FSE with the medium resolution of a short inversion-time inversion-recovery (STIR) sequence in depicting the optic nerves of healthy controls and patients with optic neuritis. Optic nerve MRI examinations were performed in 18 patients with optic neuritis and 10 normal controls. Two sequences were obtained coronally: fat-suppressed FSE (FSE TR 3250 ms/TEef 68 ms, echo-train length 16, 4 excitations, 24 cm rectangular field of view, 3 mm interleaved contiguous slices, in-plane resolution 0.5 x 0.5 mm) and STIR (TR 2000 ms/TE 50 ms/TI 175 ms, in-plane resolution 0.8 x 0.8 mm, slice thickness 5 mm). FSE demonstrated much more anatomical detail than STIR, e. g. distinction of optic nerve and sheath. Lesions were seen in 20 of 21 symptomatic nerves using FSE and in 18 of 21 using STIR. Nerve swelling or partial cross-sectional lesions of the optic nerve were each seen only on FSE in 3 cases. Fat-suppressed FSE imaging of the optic nerve improves anatomical definition and increases lesion detection in optic neuritis. (orig.). With 5 figs

  2. Blaschko Linear Enamel Defects - A Marker for Focal Dermal Hypoplasia: Case Report of Focal Dermal Hypoplasia

    Directory of Open Access Journals (Sweden)

    Stefan Gysin

    2015-05-01

    Full Text Available Focal dermal hypoplasia (FDH is a rare genetic skin disorder. The inheritance of FDH or Goltz-Gorlin syndrome is X-linked dominant and the disease is associated with a PORCN gene mutation. This gene plays a key role in the Wnt pathway, which has an impact on embryonic development. Every tissue derived from meso- and ectoderm can be affected. Patients suffer from cutaneous, ocular, osseous, oral and dental defects. The skin and dental alterations manifest along the Blaschko lines. We present a woman (born in 1962 suffering from FDH with congenital skin changes and Blaschko linear enamel defects. Typical symptoms (e.g. fat herniations, scoliosis, syndactyly, microphthalmia, caries and alopecia plus vertical grooving of all teeth gave a first indication. Molecular genetic testing confirmed the definitive diagnosis of FDH. We hypothesize that, in the context of typical skin changes, visible Blaschko lines on the teeth in the form of vertical grooves are almost pathognomonic for FDH.

  3. Diffusion MR Imaging of Postoperative Bilateral Acute Ischemic Optic Neuropathy

    International Nuclear Information System (INIS)

    Kannan, Anusha; Srinivasan, Sivasubramanian

    2012-01-01

    We read with great interest, the case report on ischemic optic neuropathy (1). We would like to add a few points concerning the blood supply of the optic nerve and the correlation with the development of post-operative ischemic neuropathy. Actually, the perioperative or post-operative vision loss (postoperative ischemic neuropathy) is most likely due to ischemic optic neuropathy. Ischemic optic neuropathy (2) is classified as an anterior ischemic optic neuropathy (AION) and posterior ischemic optic neuropathy (PION). This classification is based on the fact that blood supply (2) to the anterior segment of the optic nerve (part of the optic nerve in the scleral canal and the optic disc) is supplied by short posterior ciliary vessels or anastamotic ring branches around the optic nerve. The posterior part of the optic canal is relatively less perfused, and is supplied by ophthalmic artery and central fibres are perfused by a central retinal artery. So, in the post-operative period, the posterior part of the optic nerve is more vulnerable for ischemia, especially, after major surgeries (3), one of the theories being hypotension or anaemia (2) and resultant decreased perfusion. The onset of PION is slower than the anterior ischemic optic neuropathy. AION on the other hand, is usually spontaneous (idiopathic) or due to arteritis, and is usually sudden in its onset. The reported case is most likely a case of PION. The role of imaging, especially the diffusion weighted magnetic resonance imaging, is very important because the ophthalmoscopic findings in early stages of PION is normal, and it may delay the diagnosis. On the other hand, edema of the disc is usually seen in the early stages of AION.

  4. Diffusion MR Imaging of Postoperative Bilateral Acute Ischemic Optic Neuropathy

    Energy Technology Data Exchange (ETDEWEB)

    Kannan, Anusha; Srinivasan, Sivasubramanian [Khoo Teck Puat Hospital, Singapore (Singapore)

    2012-09-15

    We read with great interest, the case report on ischemic optic neuropathy (1). We would like to add a few points concerning the blood supply of the optic nerve and the correlation with the development of post-operative ischemic neuropathy. Actually, the perioperative or post-operative vision loss (postoperative ischemic neuropathy) is most likely due to ischemic optic neuropathy. Ischemic optic neuropathy (2) is classified as an anterior ischemic optic neuropathy (AION) and posterior ischemic optic neuropathy (PION). This classification is based on the fact that blood supply (2) to the anterior segment of the optic nerve (part of the optic nerve in the scleral canal and the optic disc) is supplied by short posterior ciliary vessels or anastamotic ring branches around the optic nerve. The posterior part of the optic canal is relatively less perfused, and is supplied by ophthalmic artery and central fibres are perfused by a central retinal artery. So, in the post-operative period, the posterior part of the optic nerve is more vulnerable for ischemia, especially, after major surgeries (3), one of the theories being hypotension or anaemia (2) and resultant decreased perfusion. The onset of PION is slower than the anterior ischemic optic neuropathy. AION on the other hand, is usually spontaneous (idiopathic) or due to arteritis, and is usually sudden in its onset. The reported case is most likely a case of PION. The role of imaging, especially the diffusion weighted magnetic resonance imaging, is very important because the ophthalmoscopic findings in early stages of PION is normal, and it may delay the diagnosis. On the other hand, edema of the disc is usually seen in the early stages of AION.

  5. Fibre optic confocal imaging (FOCI) of keratinocytes, blood vessels and nerves in hairless mouse skin in vivo

    Science.gov (United States)

    BUSSAU, L. J.; VO, L. T.; DELANEY, P. M.; PAPWORTH, G. D.; BARKLA, D. H.; KING, R. G.

    1998-01-01

    Fibre optic confocal imaging (FOCI) enabled subsurface fluorescence microscopy of the skin of hairless mice in vivo. Application of acridine orange enabled imaging of the layers of the epidermis. The corneocytes of the stratum corneum, the keratinocytes in the basal layers and redundant hair follicles were visualised at depths greater than 100 μm. Cellular and nuclear membranes of keratinocytes of the skin were visualised by the use of acridine orange and DIOC5(3). Imaging of the skin after injection of FITC-dextran revealed an extensive network of blood vessels with a size range up to 20 μm. Blood cells could be seen moving through dermal vessels and the blood circulation through the dermal vascular bed was video-taped. The fluorescent dye 4-di-2-ASP showed the presence of nerves fibres around the hair follicles and subsurface blood vessels. Comparison was made between images obtained in vivo using FOCI and in vitro scanning electron microscopy and conventional histology. FOCI offers the potential to study dynamic events in vivo, such as blood flow, skin growth, nerve regeneration and many pathological processes, in ways which have not previously been possible. PMID:9643419

  6. Enamel hypoplasia in the deciduous teeth of great apes: variation in prevalence and timing of defects.

    Science.gov (United States)

    Lukacs, J R

    2001-11-01

    The prevalence of enamel hypoplasia in the deciduous teeth of great apes has the potential to reveal episodes of physiological stress in early stages of ontogenetic development. However, little is known about enamel defects of deciduous teeth in great apes. Unresolved questions addressed in this study are: Do hypoplastic enamel defects occur with equal frequency in different groups of great apes? Are enamel hypoplasias more prevalent in the deciduous teeth of male or female apes? During what phase of dental development do enamel defects tend to form? And, what part of the dental crown is most commonly affected? To answer these questions, infant and juvenile skulls of two sympatric genera of great apes (Gorilla and Pan) were examined for dental enamel hypoplasias. Specimens from the Powell-Cotton Museum (Quex Park, UK; n = 107) are reported here, and compared with prior findings based on my examination of juvenile apes at the Cleveland Museum of Natural History (Hamman-Todd Collection; n = 100) and Smithsonian Institution (National Museum of Natural History; n = 36). All deciduous teeth were examined by the author with a x10 hand lens, in oblique incandescent light. Defects were classified using Fédération Dentaire International (FDI)/Defects of Dental Enamel (DDE) standards; defect size and location on the tooth crown were measured and marked on dental outline charts. Enamel defects of ape deciduous teeth are most common on the labial surface of canine teeth. While deciduous incisor and molar teeth consistently exhibit similar defects with prevalences of approximately 10%, canines average between 70-75%. Position of enamel defects on the canine crown was analyzed by dividing it into three zones (apical, middle, and cervical) and calculating defect prevalence by zone. Among gorillas, enamel hypoplasia prevalence increases progressively from the apical zone (low) to the middle zone to the cervical zone (highest), in both maxillary and mandibular canine teeth

  7. Management of Cleft Maxillary Hypoplasia with Anterior Maxillary Distraction: Our Experience

    OpenAIRE

    Chacko, Tojan; Vinod, Sankar; Mani, Varghese; George, Arun; Sivaprasad, K. K.

    2013-01-01

    Maxillary hypoplasia is a common developmental problem in cleft lip and palate deformities. Since 1970s these deformities have traditionally been corrected by means of orthognathic surgery. Management of skeletal deformities in the maxillofacial region has been an important challenge for maxillofacial surgeons and orthodontists. Distraction osteogenesis is a surgical technique that uses body’s own repairing mechanisms for optimal reconstruction of the tissues. We present four cases of anterio...

  8. Growth hormone treatment in cartilage-hair hypoplasia: effects on growth and the immune system.

    NARCIS (Netherlands)

    Bocca, G.; Weemaes, C.M.R.; Burgt, C.J.A.M. van der; Otten, B.J.

    2004-01-01

    Cartilage-hair hypoplasia (CHH) is a rare autosomal recessive disorder characterized by metaphyseal chondrodysplasia with severe growth retardation and impaired immunity. We studied the effects of growth hormone treatment on growth parameters and the immune system in four children with CHH. The

  9. Exposure to As, Cd and Pb-mixture impairs myelin and axon development in rat brain, optic nerve and retina

    Energy Technology Data Exchange (ETDEWEB)

    Rai, Nagendra Kumar; Ashok, Anushruti [Academy of Scientific and Innovative Research (India); Developmental Toxicology, Council of Scientific and Industrial Research-Indian Institute of Toxicology Research (CSIR-IITR) (India); Rai, Asit; Tripathi, Sachin [Developmental Toxicology, Council of Scientific and Industrial Research-Indian Institute of Toxicology Research (CSIR-IITR) (India); Nagar, Geet Kumar [Endocrinology, CSIR-Central Drug Research Institute (CSIR-CDRI) (India); Mitra, Kalyan [Electron Microscopy Unit, CSIR-CDRI, Lucknow 226001 (India); Bandyopadhyay, Sanghamitra, E-mail: sanghmitra@iitr.res.in [Academy of Scientific and Innovative Research (India); Developmental Toxicology, Council of Scientific and Industrial Research-Indian Institute of Toxicology Research (CSIR-IITR) (India)

    2013-12-01

    Arsenic (As), lead (Pb) and cadmium (Cd) are the major metal contaminants of ground water in India. We have reported the toxic effect of their mixture (metal mixture, MM), at human relevant doses, on developing rat astrocytes. Astrocyte damage has been shown to be associated with myelin disintegration in CNS. We, therefore, hypothesized that the MM would perturb myelinating white matter in cerebral cortex, optic nerve (O.N.) and retina. We observed modulation in the levels of myelin and axon proteins, such as myelin basic protein (MBP), proteolipid protein, 2′-, 3′-cyclic-nucleotide-3′-phosphodiesterase, myelin-associated glycoprotein and neurofilament (NF) in the brain of developing rats. Dose and time-dependent synergistic toxic effect was noted. The MBP- and NF-immunolabeling, as well as luxol-fast blue (LFB) staining demonstrated a reduction in the area of intact myelin-fiber, and an increase in vacuolated axons, especially in the corpus-callosum. Transmission electron microscopy (TEM) of O.N. revealed a reduction in myelin thickness and axon-density. The immunolabeling with MBP, NF, and LFB staining in O.N. supported the TEM data. The hematoxylin and eosin staining of retina displayed a decrease in the thickness of nerve-fiber, plexiform-layer, and retinal ganglion cell (RGC) count. Investigating the mechanism revealed a loss in glutamine synthetase activity in the cerebral cortex and O.N., and a fall in the brain derived neurotrophic factor in retina. An enhanced apoptosis in MBP, NF and Brn3b-containing cells justified the diminution in myelinating axons in CNS. Our findings for the first time indicate white matter damage by MM, which may have significance in neurodevelopmental-pediatrics, neurotoxicology and retinal-cell biology. - Highlights: • As, Cd and Pb-mixture, at human relevant dose, demyelinate developing rat CNS. • The attenuation in myelin and axon is synergistic. • The optic nerve and brain demonstrate reduced glutamine synthetase.

  10. Exposure to As, Cd and Pb-mixture impairs myelin and axon development in rat brain, optic nerve and retina

    International Nuclear Information System (INIS)

    Rai, Nagendra Kumar; Ashok, Anushruti; Rai, Asit; Tripathi, Sachin; Nagar, Geet Kumar; Mitra, Kalyan; Bandyopadhyay, Sanghamitra

    2013-01-01

    Arsenic (As), lead (Pb) and cadmium (Cd) are the major metal contaminants of ground water in India. We have reported the toxic effect of their mixture (metal mixture, MM), at human relevant doses, on developing rat astrocytes. Astrocyte damage has been shown to be associated with myelin disintegration in CNS. We, therefore, hypothesized that the MM would perturb myelinating white matter in cerebral cortex, optic nerve (O.N.) and retina. We observed modulation in the levels of myelin and axon proteins, such as myelin basic protein (MBP), proteolipid protein, 2′-, 3′-cyclic-nucleotide-3′-phosphodiesterase, myelin-associated glycoprotein and neurofilament (NF) in the brain of developing rats. Dose and time-dependent synergistic toxic effect was noted. The MBP- and NF-immunolabeling, as well as luxol-fast blue (LFB) staining demonstrated a reduction in the area of intact myelin-fiber, and an increase in vacuolated axons, especially in the corpus-callosum. Transmission electron microscopy (TEM) of O.N. revealed a reduction in myelin thickness and axon-density. The immunolabeling with MBP, NF, and LFB staining in O.N. supported the TEM data. The hematoxylin and eosin staining of retina displayed a decrease in the thickness of nerve-fiber, plexiform-layer, and retinal ganglion cell (RGC) count. Investigating the mechanism revealed a loss in glutamine synthetase activity in the cerebral cortex and O.N., and a fall in the brain derived neurotrophic factor in retina. An enhanced apoptosis in MBP, NF and Brn3b-containing cells justified the diminution in myelinating axons in CNS. Our findings for the first time indicate white matter damage by MM, which may have significance in neurodevelopmental-pediatrics, neurotoxicology and retinal-cell biology. - Highlights: • As, Cd and Pb-mixture, at human relevant dose, demyelinate developing rat CNS. • The attenuation in myelin and axon is synergistic. • The optic nerve and brain demonstrate reduced glutamine synthetase.

  11. Optic Disc and Optic Cup Segmentation Methodologies for Glaucoma Image Detection: A Survey

    Directory of Open Access Journals (Sweden)

    Ahmed Almazroa

    2015-01-01

    Full Text Available Glaucoma is the second leading cause of loss of vision in the world. Examining the head of optic nerve (cup-to-disc ratio is very important for diagnosing glaucoma and for patient monitoring after diagnosis. Images of optic disc and optic cup are acquired by fundus camera as well as Optical Coherence Tomography. The optic disc and optic cup segmentation techniques are used to isolate the relevant parts of the retinal image and to calculate the cup-to-disc ratio. The main objective of this paper is to review segmentation methodologies and techniques for the disc and cup boundaries which are utilized to calculate the disc and cup geometrical parameters automatically and accurately to help the professionals in the glaucoma to have a wide view and more details about the optic nerve head structure using retinal fundus images. We provide a brief description of each technique, highlighting its classification and performance metrics. The current and future research directions are summarized and discussed.

  12. Optic Disc and Optic Cup Segmentation Methodologies for Glaucoma Image Detection: A Survey

    Science.gov (United States)

    Almazroa, Ahmed; Burman, Ritambhar; Raahemifar, Kaamran; Lakshminarayanan, Vasudevan

    2015-01-01

    Glaucoma is the second leading cause of loss of vision in the world. Examining the head of optic nerve (cup-to-disc ratio) is very important for diagnosing glaucoma and for patient monitoring after diagnosis. Images of optic disc and optic cup are acquired by fundus camera as well as Optical Coherence Tomography. The optic disc and optic cup segmentation techniques are used to isolate the relevant parts of the retinal image and to calculate the cup-to-disc ratio. The main objective of this paper is to review segmentation methodologies and techniques for the disc and cup boundaries which are utilized to calculate the disc and cup geometrical parameters automatically and accurately to help the professionals in the glaucoma to have a wide view and more details about the optic nerve head structure using retinal fundus images. We provide a brief description of each technique, highlighting its classification and performance metrics. The current and future research directions are summarized and discussed. PMID:26688751

  13. Variations of retinal nerve fiber layer thickness and ganglion cell-inner plexiform layer thickness according to the torsion direction of optic disc.

    Science.gov (United States)

    Lee, Kang Hoon; Kim, Chan Yun; Kim, Na Rae

    2014-02-20

    To examine the relationship between the optic disc torsion and peripapillary retinal nerve fiber layer (RNFL) thickness through a comparison with the macular ganglion cell inner plexiform layer complex (GCIPL) thickness measured by Cirrus optical coherence tomography (OCT). Ninety-four eyes of 94 subjects with optic disc torsion and 114 eyes of 114 subjects without optic disc torsion were enrolled prospectively. The participants underwent fundus photography and OCT imaging in peripapillary RNFL mode and macular GCIPL mode. The participants were divided into groups according to the presence or absence of optic disc torsion. The eyes with optic disc torsion were further divided into supranasal torsion and inferotemporal torsion groups according to the direction of optic disc torsion. The mean RNFL and GCIPL thicknesses for the quadrants and subsectors were compared. The superior and inferior peak locations of the RNFL were also measured according to the torsion direction. The temporal RNFL thickness was significantly thicker in inferotemporal torsion, whereas the GCIPL thickness at all segments was unaffected. The inferotemporal optic torsion had more temporally positioned superior peak locations of the RNFL than the nontorsion and supranasal-torted optic disc. Thickening of the temporal RNFL with a temporal shift in the superior peak within the eyes with inferotemporal optic disc torsion can lead to interpretation errors. The ganglion cell analysis algorithm can assist in differentiating eyes with optic disc torsion.

  14. Optical coherence tomography angiography in acute arteritic and non-arteritic anterior ischemic optic neuropathy.

    Science.gov (United States)

    Balducci, Nicole; Morara, Mariachiara; Veronese, Chiara; Barboni, Piero; Casadei, Nicoletta Lelli; Savini, Giacomo; Parisi, Vincenzo; Sadun, Alfredo A; Ciardella, Antonio

    2017-11-01

    The purpose of our study was to describe the feature of acute non-arteritic or arteritic anterior ischemic optic neuropathy (NA-AION and A-AION) using optical coherence tomography angiography (OCT-A) and to compare it with fluorescein angiography (FA) and indocyanine green angiography (ICGA). In this retrospective, observational case-control study four NA-AION patients and one A-AION patient were examined by FA, ICGA and OCT-A within 2 weeks from disease presentation. The characteristics of the images were analyzed. Optic nerve head (ONH) and radial peripapillary capillaries (RPC) vessel densities (VDs) were compared between NA-AION and controls. In two of four NA-AION cases and in the A-AION patient, OCT-A clearly identified the boundary of the ischemic area at the level of the optic nerve head, which was comparable to optic disc filling defects detected by FA. In the other two NA-AION cases, a generalized leakage from the disc was visible with FA, yet OCT-A still demonstrated sectorial peripapillary capillary network reduction. Both ONH and RPC VDs were reduced in NA-AION patients, when compared to controls. OCT-A was able to identify microvascular defects and VD reduction in cases of acute optic disc edema due to NA-AION and A-AION. OCT-A provides additional information in ischemic conditions of the optic nerve head.

  15. End-to-side neurorrhaphy repairs peripheral nerve injury: sensory nerve induces motor nerve regeneration.

    Science.gov (United States)

    Yu, Qing; Zhang, She-Hong; Wang, Tao; Peng, Feng; Han, Dong; Gu, Yu-Dong

    2017-10-01

    End-to-side neurorrhaphy is an option in the treatment of the long segment defects of a nerve. It involves suturing the distal stump of the disconnected nerve (recipient nerve) to the side of the intimate adjacent nerve (donor nerve). However, the motor-sensory specificity after end-to-side neurorrhaphy remains unclear. This study sought to evaluate whether cutaneous sensory nerve regeneration induces motor nerves after end-to-side neurorrhaphy. Thirty rats were randomized into three groups: (1) end-to-side neurorrhaphy using the ulnar nerve (mixed sensory and motor) as the donor nerve and the cutaneous antebrachii medialis nerve as the recipient nerve; (2) the sham group: ulnar nerve and cutaneous antebrachii medialis nerve were just exposed; and (3) the transected nerve group: cutaneous antebrachii medialis nerve was transected and the stumps were turned over and tied. At 5 months, acetylcholinesterase staining results showed that 34% ± 16% of the myelinated axons were stained in the end-to-side group, and none of the myelinated axons were stained in either the sham or transected nerve groups. Retrograde fluorescent tracing of spinal motor neurons and dorsal root ganglion showed the proportion of motor neurons from the cutaneous antebrachii medialis nerve of the end-to-side group was 21% ± 5%. In contrast, no motor neurons from the cutaneous antebrachii medialis nerve of the sham group and transected nerve group were found in the spinal cord segment. These results confirmed that motor neuron regeneration occurred after cutaneous nerve end-to-side neurorrhaphy.

  16. Herniated gyrus rectus causing idiopathic compression of the optic chiasm.

    Science.gov (United States)

    Smith, Jacob; Jack, Megan M; Peterson, Jeremy C; Chamoun, Roukoz B

    2017-02-01

    Anomalies in the frontal lobe can interfere with visual function by compression of the optic chiasm and nerve. The gyrus rectus is located at the anterior cranial fossa floor superior to the intracranial optic nerves and chiasm. Compression of these structures by the gyrus rectus is often caused by neoplastic or dysplastic growth in the area. We report a rare case of a herniated gyrus rectus impinged on the optic chiasm and nerve without a clear pathological cause for the herniation. Copyright © 2016 Elsevier B.V. All rights reserved.

  17. Primary extranodal marginal zone lymphoma of the uvea associated with massive diffuse epibulbar extension and focal infiltration of the optic nerve and meninges, clinically presented as uveitis masquerade syndrome: a case report.

    Science.gov (United States)

    Rasić, D M; Stanković, Z; Terzić, T; Kovacević, D; Koturović, Z; Marković, V

    2010-09-01

    To report a clinical, histopathological and immunohistochemical findings in a case of primary extranodal marginal zone lymphoma of the uvea associated with massive diffuse extraocular episcleral extension and focal infiltration of the optic nerve and meninges, clinically presented as longstanding uveitis masquerade syndrome. Interventional case reports with histopathological correlation. We describe a 80-year-old male patient with a 3-year history of chronic recurrent hypertensive (pan) uveitis associated with ocular pain, unresponsive to topical and systemic anti-inflammatory, immunosuppressive, antibiotic/antiviral and antiglaucomatous therapy. Because the eye was not salvageable with conservative treatment, enucleation of blind and painful eye was performed. Findings from histopathological and immunohistochemistry examination of the enucleated eye showed an extranodal marginal zone lymphoma of the uveal tract with massive epibulbar extension and optic nerve and meningeal penetration. During almost 3 years of clinical course and 6 months after the enucleation, there were no systemic manifestations of lymphoma, and patient has not required subsequent treatment. Primary lymphoproliferative lesions of the uvea, comprising the iris, ciliary body and choroid are very rare, associated with epibulbar extension extremely and with optic nerve and menigeal penetration exceptionally. Despite its rarity, primary lymphoma of the uvea should be included in the differential diagnosis particularly in older patients with longstanding recurrent uveitis.

  18. Maxillary hypoplasia in the cleft patient: contribution of orthodontic dental space closure to orthognathic surgery.

    Science.gov (United States)

    Lee, Justine C; Slack, Ginger C; Walker, Ryann; Graves, Lindsay; Yen, Sandra; Woo, Jessica; Ambaram, Rishal; Martz, Martin G; Kawamoto, Henry K; Bradley, James P

    2014-02-01

    Cleft lip and palate surgery in the developing child is known to be associated with maxillary hypoplasia. However, the effects of nonsurgical manipulations on maxillary growth have not been well investigated. The authors present the contribution of orthodontic dental space closure with canine substitution to maxillary hypoplasia and the need for orthognathic surgery. Cleft lip/palate and cleft palate patients older than 15 years of age were reviewed for dental anomalies, orthodontic canine substitution, and Le Fort I advancement. Skeletal relationships of the maxilla to the skull base (SNA), mandible (ANB), and facial height were determined on lateral cephalograms. Logistic regression analyses were performed to estimate odds ratios. Ninety-five patients were reviewed (mean age, 18.1 years). In 65 patients with congenitally missing teeth, 55 percent with patent dental spaces required Le Fort I advancement. In contrast, 89 percent who underwent canine substitution required Le Fort I advancement (p = 0.004). Canine substitution is associated with a statistically significant increase in maxillary retrognathia when compared with dental space preservation on lateral cephalograms (mean SNA, 75.2 and 79.0, respectively; p = 0.006). Adjusting for missing dentition, logistic regression analyses demonstrated that canine substitution is an independent predictor for orthognathic surgery (OR, 6.47) and maxillary retrusion defined by SNA orthodontic cleft closure using canine substitution with maxillary hypoplasia and subsequent Le Fort I advancement, and suggest systematic criteria for management of cleft-related dental agenesis. Therapeutic, III.

  19. Monoamine oxidase enzymes and oxidative stress in the rat optic nerve: age-related changes.

    Science.gov (United States)

    Nebbioso, Marcella; Pascarella, Antonia; Cavallotti, Carlo; Pescosolido, Nicola

    2012-12-01

    In this study, age-related changes in the monoamine oxidases (MAO) were studied in the optic nerve (ON) of both young and aged male rats. The aim of the study was to assess the role of MAO in age-related changes in the rat ON and explain the mechanisms of neuroprotection mediated by MAO-B-specific inhibitors. Fifteen three month old and fifteen 26 month old Sprague-Dawley rats were used. The animals were killed by terminal anaesthesia. Staining of MAO, quantitative analysis of images, biochemical assays and statistical analysis of data were carried out. Samples of the ON were washed in water, fixed in Bowen fluid, dehydrated and embedded in Entellan. Histological sections were stained for MAO-enzymatic activities. The specificity of the reaction was evaluated by incubating control sections in a medium either without substrate or without dye. The quantitative analysis of images was carried out at the same magnification and the same lighting using a Zeiss photomicroscope. The histochemical findings were compared with the biochemical results. After enzymatic staining, MAO could be demonstrated in the ON fibres of both young and aged animals; however, MAO were increased in the nerve fibres of the elderly rats. These morphological findings were confirmed biochemically. The possibility that age-related changes in MAO levels may be attributed to impaired energy production mechanisms and/or represent the consequence of reduced energy needs is discussed. © 2012 The Authors. International Journal of Experimental Pathology © 2012 International Journal of Experimental Pathology.

  20. Unilateral blindness with third cranial nerve palsy and abnormal enhancement of extraocular muscles on magnetic resonance imaging of orbit after the ingestion of methanol.

    Science.gov (United States)

    Chung, Tae Nyoung; Kim, Sun Wook; Park, Yoo Seok; Park, Incheol

    2010-05-01

    Methanol is generally known to cause visual impairment and various systemic manifestations. There are a few reported specific findings for methanol intoxication on magnetic resonance imaging (MRI) of the brain. A case is reported of unilateral blindness with third cranial nerve palsy oculus sinister (OS) after the ingestion of methanol. Unilateral damage of the retina and optic nerve were confirmed by fundoscopy, flourescein angiography, visual evoked potential and electroretinogram. The optic nerve and extraocular muscles (superior rectus, medial rectus, inferior rectus and inferior oblique muscle) were enhanced by gadolinium-DTPA on MRI of the orbit. This is the first case report of permanent monocular blindness with confirmed unilateral damage of the retina and optic nerve, combined with third cranial nerve palsy after methanol ingestion.

  1. Anterior maxillary segmental distraction in the treatment of severe maxillary hypoplasia secondary to cleft lip and palate.

    Science.gov (United States)

    Li, Hongliang; Dai, Jiewen; Si, Jiawen; Zhang, Jianfei; Wang, Minjiao; Shen, Steve Guofang; Yu, Hongbo

    2015-01-01

    Anterior maxillary segmental distraction (AMSD) is an effective surgical procedure in the treatment of maxillary hypoplasia secondary to cleft lip and palate. Its unique advantage of preserving velopharyngeal function makes this procedure widely applied. In this study, the application of AMSD was described and its long-term stability was explored. Eight patients with severe maxillary hypoplasia secondary to CLP were included in this study. They were treated with AMSD using rigid external distraction (RED) device. Cephalometric analysis was performed twice at three time points for evaluation: before surgery (T1), after distraction (T2), and 2 years after treatment (T3). One-way analysis of variance was used to assess the differences statistically. All the distractions completed smoothly, and maxilla was distracted efficiently. The value of SNA, NA-FH, Ptm-A, U1-PP, overjet and PP (ANS-PNS) increased significantly after the AMSD procedure (P 0.05). Changes of palatopharyngeal depth and soft palatal length were insignificant. AMSD with RED device provided an effective way to correct maxillary hypoplasia secondary to CLP, extended the palatal and arch length, avoided damage on velopharyngeal closure function and reduced the relapse rate. It is a promising and valuable technique in this potentially complicated procedure.

  2. Novel occurrence of axenfeld: Rieger syndrome in a patient with blepharophimosis ptosis epicanthus inversus syndrome

    Directory of Open Access Journals (Sweden)

    Bhavin M Shah

    2014-01-01

    Full Text Available Blepharophimosis ptosis epicanthus inversus syndrome (BPES is a complex eyelid malformation characterized by the classical tetrad of blepharophimosis, telecanthus, ptosis, and epicanthus inversus. It has been reported to be associated with other ocular anomalies such as euryblepharon, strabismus, nystagmus, amblyopia, microphthalmos, lacrimal drainage apparatus abnormality, extra ocular muscle abnormalities, microcornea, trabecular dysgenesis, optic nerve hypoplasias, and colobomas of the optic disk. We describe a case of BPES with Axenfeld-Rieger syndrome, a neurocristopathy characterized by maldevelopment of the anterior segment with predisposition to development of glaucoma. Interestingly, both syndromes are caused by mutations in the same class of genes, namely the winged-helix/forked transcription factors (FOX involved in a variety of developmental processes.

  3. Influence of atypical retardation pattern on the peripapillary retinal nerve fibre distribution assessed by scanning laser polarimetry and optical coherence tomography.

    Science.gov (United States)

    Schrems, W A; Laemmer, R; Hoesl, L M; Horn, F K; Mardin, C Y; Kruse, F E; Tornow, R P

    2011-10-01

    To investigate the influence of atypical retardation pattern (ARP) on the distribution of peripapillary retinal nerve fibre layer (RNFL) thickness measured with scanning laser polarimetry in healthy individuals and to compare these results with RNFL thickness from spectral domain optical coherence tomography (OCT) in the same subjects. 120 healthy subjects were investigated in this study. All volunteers received detailed ophthalmological examination, GDx variable corneal compensation (VCC) and Spectralis-OCT. The subjects were divided into four subgroups according to their typical scan score (TSS): very typical with TSS=100, typical with 99 ≥ TSS ≥ 91, less typical with 90 ≥ TSS ≥ 81 and atypical with TSS ≤ 80. Deviations from very typical normal values were calculated for 32 sectors for each group. There was a systematic variation of the RNFL thickness deviation around the optic nerve head in the atypical group for the GDxVCC results. The highest percentage deviation of about 96% appeared temporal with decreasing deviation towards the superior and inferior sectors, and nasal sectors exhibited a deviation of 30%. Percentage deviations from very typical RNFL values decreased with increasing TSS. No systematic variation could be found if the RNFL thickness deviation between different TSS-groups was compared with the OCT results. The ARP has a major impact on the peripapillary RNFL distribution assessed by GDx VCC; thus, the TSS should be included in the standard printout.

  4. Laser-activated protein bands for peripheral nerve repair

    Science.gov (United States)

    Lauto, Antonio; Trickett, Rodney I.; Malik, Richard; Dawes, Judith M.; Owen, Earl R.

    1996-01-01

    A 100 micrometer core optical fiber-coupled 75 mW diode laser operating at a wavelength of 800 nm has been used in conjunction with a protein solder to stripe weld severed rat tibial nerves, reducing the long operating time required for microsurgical nerve repair. Welding is produced by selective laser denaturation of the protein based solder which contains the dye indocyanine green. Operating time for laser soldering was 10 plus or minus 5 min. (n equals 24) compared to 23 plus or minus 9 min (n equals 13) for microsuturing. The laser solder technique resulted in patent welds with a tensile strength of 15 plus or minus 5 g, while microsutured nerves had a tensile strength of 40 plus or minus 10 g. Histopathology of the laser soldered nerves, conducted immediately after surgery, displayed solder adhesion to the outer membrane with minimal damage to the inner axons of the nerves. An in vivo study, with a total of fifty-seven adult male wistar rats, compared laser solder repaired tibial nerves to conventional microsuture repair. Twenty-four laser soldered nerves and thirteen sutured nerves were characterized at three months and showed successful regeneration with average compound muscle action potentials (CMAP) of 2.4 plus or minus 0.7 mV and 2.7 plus or minus 0.8 mV respectively. Histopathology of the in vivo study, confirmed the comparable regeneration of axons in laser and suture operated nerves. A faster, less damaging and long lasting laser based anastomotic technique is presented.

  5. Watery and dark axons in Wallerian degeneration of the opossum's optic nerve: different patterns of cytoskeletal breakdown?

    Directory of Open Access Journals (Sweden)

    MARCELO S. NARCISO

    2001-06-01

    Full Text Available In this paper we report a qualitative morphological analysis of Wallerian degeneration in a marsupial. Right optic nerves of opossums Didelphis marsupialis were crushed with a fine forceps and after 24, 48, 72, 96 and 168 hours the animals were anaesthetized and perfused with fixative. The optic nerves were immersed in fixative and processed for routine transmission electron microscopy. Among the early alterations typical of axonal degeneration, we observed nerve fibers with focal degeneration of the axoplasmic cytoskeleton, watery degeneration and dark degeneration, the latter being prevalent at 168 hours after crush. Our results point to a gradual disintegration of the axoplasmic cytoskeleton, opposed to the previous view of an "all-or-nothing'' process (Griffin et al 1995. We also report that, due to an unknown mechanism, fibers show either a dark or watery pattern of axonal degeneration, as observed in axon profiles. We also observed fibers undergoing early myelin breakdown in the absence of axonal alterations.Neste trabalho, relatamos uma análise morfológica qualitativa da degeneração Walleriana em um marsupial. Os nervos ópticos direito de gambás da espécie Didelphis marsupialis foram esmagados com uma pinça fina. Após 24, 48, 72, 96 e 168 horas, os animais foram anestesiados e perfundidos com fixador. A seguir, os nervos foram imersos em fixador e processados para microscopia eletrônica de rotina. Entre as alterações precoces típicas da degeneração, observamos fibras nervosas com degeneração focal do citoesqueleto axoplasmático, degeneração aquosa e degeneração escura, com o último tipo prevalente às 168 horas após esmagamento. Nossos resultados indicam uma desintegração gradual do citoesqueleto axoplasmático, oposta à prévia visão de um processo "tudo-ou-nada''. Relatamos também que, devido a um mecanismo desconhecido, as fibras mostram ou um padrão aquoso ou um padrão escuro de degeneração axonal

  6. Estimating the accuracy of optic nerve sheath diameter measurement using a pocket-sized, handheld ultrasound on a simulation model.

    Science.gov (United States)

    Johnson, Garrett G R J; Zeiler, Frederick A; Unger, Bertram; Hansen, Gregory; Karakitsos, Dimitrios; Gillman, Lawrence M

    2016-12-01

    Ultrasound measurement of optic nerve sheath diameter (ONSD) appears to be a promising, rapid, non-invasive bedside tool for identification of elevated intra-cranial pressure. With improvements in ultrasound technology, machines are becoming smaller; however, it is unclear if these ultra-portable handheld units have the resolution to make these measurements precisely. In this study, we estimate the accuracy of ONSD measurement in a pocket-sized ultrasound unit. Utilizing a locally developed, previously validated model of the eye, ONSD was measured by two expert observers, three times with two machines and on five models with different optic nerve sheath sizes. A pocket ultrasound (Vscan, GE Healthcare) and a standard portable ultrasound (M-Turbo, SonoSite) were used to measure the models. Data was analyzed by Bland-Altman plot and intra-class correlation coefficient (ICC). The ICC between raters for the SonoSite was 0.878, and for the Vscan was 0.826. The between-machine agreement ICC was 0.752. Bland-Altman agreement analysis between the two ultrasound methods showed an even spread across the range of sheath sizes, and that the Vscan tended to read on average 0.33 mm higher than the SonoSite for each measurement, with a standard deviation of 0.65 mm. Accurate ONSD measurement may be possible utilizing pocket-sized, handheld ultrasound devices despite their small screen size, lower resolution, and lower probe frequencies. Further study in human subjects is warranted for all newer handheld ultrasound models as they become available on the market.

  7. Congenital bilateral perisylvian syndrome with pituitary hypoplasia and ectopic neurohypophysis

    Energy Technology Data Exchange (ETDEWEB)

    Yekeler, Ensar; Genchellac, Hakan; Dursun, Memduh; Acunas, Gulden [Istanbul Faculty of Medicine, Department of Radiology, Istanbul (Turkey); Ozmen, Meral [Istanbul Faculty of Medicine, Department of Paediatric Neurology, Istanbul (Turkey)

    2004-11-01

    Congenital bilateral perisylvian syndrome (CBPS) is a congenital neurological syndrome characterized by pseudobulbar palsy, cognitive deficits and bilateral perisylvian abnormalities observed on imaging. The described abnormality in CBPS is polymicrogyria located in the frontal, parietal, and/or occipital lobes. A few syndromes or abnormalities associated with this syndrome have been documented. Pituitary abnormalities are rare disorders. Association of CBPS with pituitary abnormalities has not been reported previously. In this case, a combination of bilateral perisylvian polymicrogyria with pituitary hypoplasia and ectopic neurohypophysis, caused by a possible single common insult, is presented. (orig.)

  8. Congenital bilateral perisylvian syndrome with pituitary hypoplasia and ectopic neurohypophysis

    International Nuclear Information System (INIS)

    Yekeler, Ensar; Genchellac, Hakan; Dursun, Memduh; Acunas, Gulden; Ozmen, Meral

    2004-01-01

    Congenital bilateral perisylvian syndrome (CBPS) is a congenital neurological syndrome characterized by pseudobulbar palsy, cognitive deficits and bilateral perisylvian abnormalities observed on imaging. The described abnormality in CBPS is polymicrogyria located in the frontal, parietal, and/or occipital lobes. A few syndromes or abnormalities associated with this syndrome have been documented. Pituitary abnormalities are rare disorders. Association of CBPS with pituitary abnormalities has not been reported previously. In this case, a combination of bilateral perisylvian polymicrogyria with pituitary hypoplasia and ectopic neurohypophysis, caused by a possible single common insult, is presented. (orig.)

  9. Distraction osteogenesis of radiation-induced orbitozygomatic hypoplasia.

    Science.gov (United States)

    Grover, Ramon; Murray, Dylan; Fialkov, Jeffrey A

    2008-05-01

    In the last decade, the application of distraction osteogenesis to the craniofacial skeleton has grown to include not only deformities of the mandible, but of the midface, palate, dentoalveolar region, and calvarium. A major advantage of distraction osteogenesis lies in the simultaneous soft tissue histogenesis that accompanies the bony distraction process, allowing for potentially lower relapse rates and improved cosmesis. Although this may seem appropriately suited to irradiation-induced deformities of both hard and soft tissues, there is little in the literature as to the efficacy of this technique in patients who have received radiotherapy. To introduce an effective application of this technology, and highlight some advantages and disadvantages of its application in the irradiated craniofacial skeleton, we present a case of distraction osteogenesis of the orbitozygomatic complex in a patient with radiation induced orbitozygomatic hypoplasia.

  10. Primary Sjoegren's syndrome initially manifested by optic neuritis: MRI findings

    International Nuclear Information System (INIS)

    Kadota, Y.; Tokumaru, A.M.; Kohyama, S.; Okizuka, H.; Kaji, T.; Kusano, S.; Kamakura, K.

    2002-01-01

    We herein describe the MRI findings in a patient clinically diagnosed with primary Sjoegren's syndrome (SjS) initially manifested by retrobulbar optic neuritis. A 63-year-old woman suddenly had left ocular pain and progressive visual disturbance. MR T2-weighted images revealed hyperintensity in the left optic nerve, with swelling. Contrast-enhanced T1-weighted images showed no abnormal enhancement. Follow-up MRI 6 months after admission revealed no significant changes in the affected optic nerve. To our knowledge, optic neuritis as a complication of SjS has been reported in ten patients [1, 2, 3, 4, 5, 6] and MRI findings in only one of them [6]. We thought MR images were useful for visualizing optic nerve involvement in SjS and observing its course. (orig.)

  11. Normal computed tomographic anatomy of the cisterns and cranial nerves

    International Nuclear Information System (INIS)

    Manelfe, C.; Bonafe, A.

    1980-01-01

    This study, based on the normal CT anatomy of the cisterns investigated with Metrizamide, aims at attempting to find out with accuracy which plane of section is the most suitable for the investigation of each group of cisterns (posterior fossa, mesencephalon, suprasellar). Moreover we felt it necessary to include our study the normal appearance of the cranial nerves as their normal CT anatymy - optic nerves expected - is not well known yet. (orig./AJ) [de

  12. Application of mitomycin C after endoscopic lysis of congenital laryngeal web combined with epiglottic hypoplasia in a middle-aged man.

    Science.gov (United States)

    Roh, Jong-Lyel

    2006-04-01

    Laryngeal webs and epiglottic hypoplasias are uncommon congenital anomalies. Anterior glottic web combined with epiglottic hypoplasia was found in a middle-aged man presenting with hoarseness and dyspnea on exertion. This can be considered as a unique isolated defect of the larynx during early fetal development. The laryngeal web can be successfully treated in a single stage with endoscopic lysis and topical application of mitomycin C for prevention of anterior glottic restenosis. This case and prior reports suggest that the novel approach may be effective in the treatment of laryngeal webs.

  13. Unilateral hypoglossal nerve atrophy as a late complication of radiation therapy of head and neck carcinoma: a report of four cases and a review of the literature on peripheral and cranial nerve damages after radiation therapy

    International Nuclear Information System (INIS)

    Cheng, V.S.T.; Schulz, M.D.

    1975-01-01

    The case histories of four patients who developed hemiatrophy of the tongue from 3 to 9 years after a course of curative radiation therapy for carcinomas of the head and neck are presented. These patients were subsequently followed from 1 1 / 2 to 6 years without local recurrence of the tumor, distant metastasis, or involvement of other cranial nerves, indicative of only a unilateral hypoglossal nerve atrophy. A review of the literature showed that peripheral and cranial nerve damages after radiation therapy have been reported for the optic nerve, hypoglossal nerve, oculomotor nerve, abducens nerve, recurrent laryngeal nerve, brachial plexus nerves, and peripheral nerves of the extremities. Review of clinical and experimental data indicated that in most cases, the damages were probably caused by extensive connective tissue fibrosis around and infiltrating the nerve trunks. Three possible types of peripheral and cranial nerve damages after radiation therapy are identified. (U.S.)

  14. Optic neuropathies: the tip of the neurodegeneration iceberg

    Science.gov (United States)

    Carelli, Valerio; La Morgia, Chiara; Ross-Cisneros, Fred N.; Sadun, Alfredo A.

    2017-01-01

    Abstract The optic nerve and the cells that give origin to its 1.2 million axons, the retinal ganglion cells (RGCs), are particularly vulnerable to neurodegeneration related to mitochondrial dysfunction. Optic neuropathies may range from non-syndromic genetic entities, to rare syndromic multisystem diseases with optic atrophy such as mitochondrial encephalomyopathies, to age-related neurodegenerative diseases such as Alzheimer’s and Parkinson’s disease where optic nerve involvement has, until recently, been a relatively overlooked feature. New tools are available to thoroughly investigate optic nerve function, allowing unparalleled access to this part of the central nervous system. Understanding the molecular pathophysiology of RGC neurodegeneration and optic atrophy, is key to broadly understanding the pathogenesis of neurodegenerative disorders, for monitoring their progression in describing the natural history, and ultimately as outcome measures to evaluate therapies. In this review, the different layers, from molecular to anatomical, that may contribute to RGC neurodegeneration and optic atrophy are tackled in an integrated way, considering all relevant players. These include RGC dendrites, cell bodies and axons, the unmyelinated retinal nerve fiber layer and the myelinated post-laminar axons, as well as olygodendrocytes and astrocytes, looked for unconventional functions. Dysfunctional mitochondrial dynamics, transport, homeostatic control of mitobiogenesis and mitophagic removal, as well as specific propensity to apoptosis may target differently cell types and anatomical settings. Ultimately, we can envisage new investigative approaches and therapeutic options that will speed the early diagnosis of neurodegenerative diseases and their cure. PMID:28977448

  15. Development and Functional Organization of the Cranial Nerves in Lampreys.

    Science.gov (United States)

    Pombal, Manuel A; Megías, Manuel

    2018-04-16

    Lampreys, together with hagfishes, are the only extant representatives of the oldest branch of vertebrates, the agnathans, which are the sister group of gnathostomes; therefore, studies on these animals are of great evolutionary significance. Lampreys exhibit a particular life cycle with remarkable changes in their behavior, concomitant, in part, with important modifications in the head and its musculature, which might influence the development of the cranial nerves. In this context, some cranial nerves such as the optic nerve and the ocular motor nerves, which develop slowly during an extremely long larval period lasting more than five years, have been more thoroughly investigated; however, much less experimental information is available about others, such as the facial or the hypoglossal nerves. In addition, the possible existence of a "true" accessory nerve in these animals is still a matter of conjecture. Although growing in last decades, investigations on the physiology of the lamprey cranial nerves is scanty. This review focuses on past and recent findings that have contributed to characterize the anatomical organization of the cranial nerves in lampreys, including their components and nuclei, and their relations in the brain; in addition, comments on their development and functional role are also included. Anat Rec, 2018. © 2018 Wiley Periodicals, Inc. © 2018 Wiley Periodicals, Inc.

  16. Combined use of high-definition and volumetric optical coherence tomography for the segmentation of neural canal opening in cases of optic nerve edema

    Science.gov (United States)

    Wang, Jui-Kai; Kardon, Randy H.; Garvin, Mona K.

    2015-03-01

    In cases of optic-nerve-head edema, the presence of the swelling reduces the visibility of the underlying neural canal opening (NCO) within spectral-domain optical coherence tomography (SD-OCT) volumes. Consequently, traditional SD-OCT-based NCO segmentation methods often overestimate the size of the NCO. The visibility of the NCO can be improved using high-definition 2D raster scans, but such scans do not provide 3D contextual image information. In this work, we present a semi-automated approach for the segmentation of the NCO in cases of optic disc edema by combining image information from volumetric and high-definition raster SD-OCT image sequences. In particular, for each subject, five high-definition OCT B-scans and the OCT volume are first separately segmented, and then the five high-definition B-scans are automatically registered to the OCT volume. Next, six NCO points are placed (manually, in this work) in the central three high-definition OCT B-scans (two points for each central B-scans) and are automatically transferred into the OCT volume. Utilizing a combination of these mapped points and the 3D image information from the volumetric scans, a graph-based approach is used to identify the complete NCO on the OCT en-face image. The segmented NCO points using the new approach were significantly closer to expert-marked points than the segmented NCO points using a traditional approach (root mean square differences in pixels: 5.34 vs. 21.71, p < 0.001).

  17. Three-dimensional-fast imaging employing steady-state acquisition and T2-weighted fast spin-echo magnetic resonance sequences on visualization of cranial nerves Ⅲ-Ⅻ

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    @@ Because of the small diameter and complex anatomic course of the cranial nerves except for the optic nerve,mgeminal nerve,facial nerve,and cochlear and vestibular nerve,other cranial nerves are difficult to be visualized in magnetic resonance imaging (MRI) scanning with conventional thickness (5-10 mm).

  18. Bilateral familial vertical Duane Syndrome with synergistic convergence, aberrant trigeminal innervation, and facial hypoplasia

    Directory of Open Access Journals (Sweden)

    Malvika Gupta

    2014-01-01

    Full Text Available A 5-year-old girl presented with bilateral familial vertical  Duane retraction syndrome with alternating esotropia, elevation deficit, Marcus gunn phenomenon, and facial hypoplasia. Abnormal adducting downshoots on attempting abduction suggestive of a synergistic convergence were noted. Hypothesis suggests aberrant innervations or peripheral anatomic connections between inferior and medial recti.

  19. Liver segment IV hypoplasia as a risk factor for bile duct injury.

    Science.gov (United States)

    Mercado, Miguel Angel; Franssen, Bernardo; Arriola, Juan Carlos; Garcia-Badiola, Artemio; Arámburo, Rigoberto; Elnecavé, Alejandro; Cortés-González, Rubén

    2011-09-01

    Bile duct injury remains constant in the era of laparoscopic cholecystectomy and misidentification of structures remains one of the most common causes of such injuries. Abnormalities in liver segment IV, which is fully visible during laparoscopic cholecystectomy, may contribute to misidentification as proposed herein. We describe the case of a 36-year-old female who had a bile duct injury during a laparoscopic cholecystectomy where the surgeon noticed an unusually small distance between the gallbladder and the round ligament. We define hypoplasia of liver segment IV as well as describe the variation of the biliary anatomy in the case. We also intend to fit it in a broader spectrum of developmental anomalies that have both hyopoplasia of some portion of the liver and variations in gallbladder and bile duct anatomy that may contribute to bile duct injury. To our knowledge, hypoplasia of liver segment IV has not been suggested in the literature as a risk factor for bile duct injury except in the extreme case of a left-sided gallbladder. Surgeons should be vigilant during laparoscopic cholecystectomy when they become aware of an unusually small distance between the gallbladder bed and the round ligament prior to beginning their dissection, variations in the common bile duct and cystic duct should be expected.

  20. Leukemic optic neuropathy.

    Science.gov (United States)

    Brown, G C; Shields, J A; Augsburger, J J; Serota, F T; Koch, P

    1981-03-01

    The clinical course and ophthalmic manifestations of an eight year old child with acute undifferentiated leukemia and unilateral blindness secondary to leukemic optic nerve head infiltration are described. At autopsy the involved nerve head and peripapillary retina demonstrated massive leukemic cell infiltration and hemorrhagic necrosis. This manifestation of leukemia is quite uncommon and prognosis for life in such cases is poor with existing methods of therapy.