Congenital unilateral absence of the pulmonary artery in adults

International Nuclear Information System (INIS)

Gonzalez Garcia, Mauricio; Escalante Mora, Hector A; Lozano Castillo, Alfonso J

2000-01-01

Unilateral absence of a pulmonary artery is a rare anomaly. It occurs with pulmonary ipsilateral hypoplasia and it's frequently associated with other cardiovascular malformations. The majority of the cases are diagnosed in childhood. This is a case report of two adult patients of the Hospital Central de la Policia Nacional in Bogota, Colombia, with unilateral absence of the pulmonary artery one isolated and the other with patent ductus arteriosus. We describe the clinical and roentgenographic findings of this congenital anomaly

Genetics of human hydrocephalus

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Williams, Michael A.; Rigamonti, Daniele

2006-01-01

Human hydrocephalus is a common medical condition that is characterized by abnormalities in the flow or resorption of cerebrospinal fluid (CSF), resulting in ventricular dilatation. Human hydrocephalus can be classified into two clinical forms, congenital and acquired. Hydrocephalus is one of the complex and multifactorial neurological disorders. A growing body of evidence indicates that genetic factors play a major role in the pathogenesis of hydrocephalus. An understanding of the genetic components and mechanism of this complex disorder may offer us significant insights into the molecular etiology of impaired brain development and an accumulation of the cerebrospinal fluid in cerebral compartments during the pathogenesis of hydrocephalus. Genetic studies in animal models have started to open the way for understanding the underlying pathology of hydrocephalus. At least 43 mutants/loci linked to hereditary hydrocephalus have been identified in animal models and humans. Up to date, 9 genes associated with hydrocephalus have been identified in animal models. In contrast, only one such gene has been identified in humans. Most of known hydrocephalus gene products are the important cytokines, growth factors or related molecules in the cellular signal pathways during early brain development. The current molecular genetic evidence from animal models indicate that in the early development stage, impaired and abnormal brain development caused by abnormal cellular signaling and functioning, all these cellular and developmental events would eventually lead to the congenital hydrocephalus. Owing to our very primitive knowledge of the genetics and molecular pathogenesis of human hydrocephalus, it is difficult to evaluate whether data gained from animal models can be extrapolated to humans. Initiation of a large population genetics study in humans will certainly provide invaluable information about the molecular and cellular etiology and the developmental mechanisms of human

A pilot study using residual newborn dried blood spots to assess the potential role of cytomegalovirus and Toxoplasma gondii in the etiology of congenital hydrocephalus.

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Simeone, Regina M; Rasmussen, Sonja A; Mei, Joanne V; Dollard, Sheila C; Frias, Jaime L; Shaw, Gary M; Canfield, Mark A; Meyer, Robert E; Jones, Jeffrey L; Lorey, Fred; Honein, Margaret A

2013-07-01

Congenital hydrocephalus is a condition characterized by accumulation of cerebrospinal fluid in the ventricles of the brain. Prenatal infections are risk factors for some birth defects. This pilot study investigated whether residual dried blood spots (DBS) could be used to assess infections as risk factors for birth defects by examining the associations between prenatal infection with Toxoplasma gondii (T. gondii) or cytomegalovirus (CMV) with congenital hydrocephalus. Case-infants with hydrocephalus (N=410) were identified among live-born infants using birth defects surveillance systems in California, North Carolina, and Texas. Control-infants without birth defects were randomly selected from the same geographic areas and time periods as case-infants (N=448). We tested residual DBS from case- and control-infants for T. gondii immunoglobulin M and CMV DNA. When possible, we calculated crude odds ratios (cORs) and confidence intervals (CIs). Evidence for prenatal T. gondii infection was more common among case-infants (1.2%) than control-infants (0%; p=0.11), and evidence for prenatal CMV infection was higher among case-infants (1.5%) than control-infants (0.7%; cOR: 2.3; 95% CI: 0.48, 13.99). Prenatal infections with T. gondii and CMV occurred more often among infants with congenital hydrocephalus than control-infants, although differences were not statistically significant. This pilot study highlighted some challenges in using DBS to examine associations between certain infections and birth defects, particularly related to reduced sensitivity and specimen storage conditions. Further study with increased numbers of specimens and higher quality specimens should be considered to understand better the contribution of these infections to the occurrence of congenital hydrocephalus. Copyright © 2013 Wiley Periodicals, Inc.

Monocular oral reading after treatment of dense congenital unilateral cataract

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Birch, Eileen E.; Cheng, Christina; Christina, V; Stager, David R.

2010-01-01

Background Good long-term visual acuity outcomes for children with dense congenital unilateral cataracts have been reported following early surgery and good compliance with postoperative amblyopia therapy. However, treated eyes rarely achieve normal visual acuity and there has been no formal evaluation of the utility of the treated eye for reading. Methods Eighteen children previously treated for dense congenital unilateral cataract were tested monocularly with the Gray Oral Reading Test, 4th edition (GORT-4) at 7 to 13 years of age using two passages for each eye, one at grade level and one at +1 above grade level. In addition, right eyes of 55 normal children age 7 to 13 served as a control group. The GORT-4 assesses reading rate, accuracy, fluency, and comprehension. Results Visual acuity of treated eyes ranged from 0.1 to 2.0 logMAR and of fellow eyes from −0.1 to 0.2 logMAR. Treated eyes scored significantly lower than fellow and normal control eyes on all scales at grade level and at +1 above grade level. Monocular reading rate, accuracy, fluency, and comprehension were correlated with visual acuity of treated eyes (rs = −0.575 to −0.875, p < 0.005). Treated eyes with 0.1-0.3 logMAR visual acuity did not differ from fellow or normal control eyes in rate, accuracy, fluency, or comprehension when reading at grade level or at +1 above grade level. Fellow eyes did not differ from normal controls on any reading scale. Conclusions Excellent visual acuity outcomes following treatment of dense congenital unilateral cataracts are associated with normal reading ability of the treated eye in school-age children. PMID:20603057

Congenital Unilateral Hypoplasia of Depressor Anguli Oris

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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.

Medfødt hydrocephalus--forekomst og prognose. Mortalitet og morbiditet i en populationsbaseret opgørelse

DEFF Research Database (Denmark)

Christensen, Jacob Haaber; Hansen, Lars Kjaersgård; Garne, Ester

2003-01-01

The aim was to describe the prevalence and to estimate the prognosis of congenital hydrocephalus (HC) in fetuses and children.......The aim was to describe the prevalence and to estimate the prognosis of congenital hydrocephalus (HC) in fetuses and children....

Changes in auditory perceptions and cortex resulting from hearing recovery after extended congenital unilateral hearing loss

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Jill B Firszt

2013-12-01

Full Text Available Monaural hearing induces auditory system reorganization. Imbalanced input also degrades time-intensity cues for sound localization and signal segregation for listening in noise. While there have been studies of bilateral auditory deprivation and later hearing restoration (e.g. cochlear implants, less is known about unilateral auditory deprivation and subsequent hearing improvement. We investigated effects of long-term congenital unilateral hearing loss on localization, speech understanding, and cortical organization following hearing recovery. Hearing in the congenitally affected ear of a 41 year old female improved significantly after stapedotomy and reconstruction. Pre-operative hearing threshold levels showed unilateral, mixed, moderately-severe to profound hearing loss. The contralateral ear had hearing threshold levels within normal limits. Testing was completed prior to, and three and nine months after surgery. Measurements were of sound localization with intensity-roved stimuli and speech recognition in various noise conditions. We also evoked magnetic resonance signals with monaural stimulation to the unaffected ear. Activation magnitudes were determined in core, belt, and parabelt auditory cortex regions via an interrupted single event design. Hearing improvement following 40 years of congenital unilateral hearing loss resulted in substantially improved sound localization and speech recognition in noise. Auditory cortex also reorganized. Contralateral auditory cortex responses were increased after hearing recovery and the extent of activated cortex was bilateral, including a greater portion of the posterior superior temporal plane. Thus, prolonged predominant monaural stimulation did not prevent auditory system changes consequent to restored binaural hearing. Results support future research of unilateral auditory deprivation effects and plasticity, with consideration for length of deprivation, age at hearing correction, degree and type

Sonographic pattern of hydrocephalus among the under five children in Sokoto North Western Nigeria

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Sule Ahmed Saidu

2015-01-01

Full Text Available Background: Hydrocephalus among children is an important medical problem in view of its neurological sequelae in the growing child. This situation is compounded by the acute shortage of neurosurgeons in third world countries like Nigeria; hence, the need for its early detection and proper management. Objective: Evaluation of the ultrasound (US appearances in children under 5 years of age presenting with clinical signs of raised intracranial pressure suggestive of hydrocephalus. Patients and Methods: A retrospective review of transfontanelle US scans done in 64 children (39 boys and 25 girls attending the Department of Radiology, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria over a period of 2 years was carried out. The patients had a mean age of 5.0 ± 4.6 months (range: 1–60 months at the time of their US examination. Some of the clinical indications for US scan included: Congenital hydrocephalus, encephalocele, meningomyelocele, and meningitis. All scans were performed through the anterior fontanelle using SIUI Apogee 800 PLUS scanner with a curvilinear probe using multi-frequency transducer of 2–5 MHz. Results: Fifty-two patients (81.3% had hydrocephalus of congenital origin. Eleven cases (17.2% had postmeningitic hydrocephalus while only 1 case (1.6% was posthemorrhagic. Twenty-five patients (48.0% of the congenital cases were due to cerebral aqueduct stenosis. Eleven (21.2% of the congenital cases were from obstruction at the exit foramina of Luschka and Magendie resulting in the communicating type of hydrocephalus. Conclusion: Hydrocephalus is a known cause of neurological morbidity among infants in developing countries. Majority of the cases are congenital in origin and most commonly due to cerebral aqueduct stenosis. Transfontanelle US is cheap, affordable, nonhazardous, and more accessible than other imaging modalities. It should serve as the first-line investigation of infants with suspected hydrocephalus for early

Anoftalmia clínica bilateral associada à hidrocefalia congênita em cão Bilateral clinical anophthalmia associated with congenital hydrocephalus in the dog

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Mariana Isa Poci Palumbo

2011-07-01

Full Text Available A ausência completa do bulbo ocular é muito rara em cães e gatos, enquanto a hidrocefalia é comumente observada como distúrbio congênito em cães de raças miniatura ou braquicefálicas, com menos de um ano de idade. O presente trabalho relata a ocorrência de anoftalmia clínica bilateral associada à hidrocefalia congênita em um cão da raça poodle, sendo este o primeiro relato de caso da associação dessas alterações no Brasil.The complete absence of the eyeball is rare in dogs and cats, and hydrocephalus is commonly seen as a congenital disorder in toy or brachycephalic dogs before one year old. This paper describes for the first time in Brazil the occurrence of bilateral clinical anophthalmia associated to congenital hydrocephalus in a dog.

Congenital hydrocephalus and abnormal subcommissural organ development in Sox3 transgenic mice.

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Kristie Lee

Full Text Available Congenital hydrocephalus (CH is a life-threatening medical condition in which excessive accumulation of CSF leads to ventricular expansion and increased intracranial pressure. Stenosis (blockage of the Sylvian aqueduct (Aq; the narrow passageway that connects the third and fourth ventricles is a common form of CH in humans, although the genetic basis of this condition is unknown. Mouse models of CH indicate that Aq stenosis is associated with abnormal development of the subcommmissural organ (SCO a small secretory organ located at the dorsal midline of the caudal diencephalon. Glycoproteins secreted by the SCO generate Reissner's fibre (RF, a thread-like structure that descends into the Aq and is thought to maintain its patency. However, despite the importance of SCO function in CSF homeostasis, the genetic program that controls SCO development is poorly understood. Here, we show that the X-linked transcription factor SOX3 is expressed in the murine SCO throughout its development and in the mature organ. Importantly, overexpression of Sox3 in the dorsal diencephalic midline of transgenic mice induces CH via a dose-dependent mechanism. Histological, gene expression and cellular proliferation studies indicate that Sox3 overexpression disrupts the development of the SCO primordium through inhibition of diencephalic roof plate identity without inducing programmed cell death. This study provides further evidence that SCO function is essential for the prevention of hydrocephalus and indicates that overexpression of Sox3 in the dorsal midline alters progenitor cell differentiation in a dose-dependent manner.

Endoscopic third ventriculostomy for obstructive hydrocephalus

International Nuclear Information System (INIS)

Ali, M.; Usman, M.; Khan, Z.; Khan, K.M.; Hussain, R.; Khanzada, K.

2013-01-01

To determine the success rate of endoscopic third ventriculostomy (ETV) for treating obstructive hydrocephalus. Study Design: Cross-sectional observational study. Place and Duration of Study: The Neurosurgery Department of PGMI, Lady Reading Hospital, Peshawar, from May 2010 to November 2011. Methodology: Patients with obstructive hydrocephalus due to aqueductal stenosis, tectal and non-tectal tumour and already shunted patients for obstructive hydrocephalus presented with blocked shunt were included in the study. Patients with congenital hydrocephalus and secondary to meningitis were excluded. Endoscopic third ventriculostomy was performed. Success, complications and mortality was noted. Data was analyzed by descriptive statistics using SPSS software version 17. Results: There were 155 patients including 72 males and 83 females with ratio of 1: 1.33. Success rate was 71%. Indication of surgery was obstructive hydrocephalus due to aqueductal stenosis, posterior fossa tumour, brain stem and CP angle tumour. Complications were seen in 18 patients including mortality in 3 patients. Conclusion: ETV is effective, safe and successful procedure in patients with obstructive hydrocephalus. It may be used as replacement procedure of ventriculo-peritoneal shunt as initial line of management in selected patients. (author)

Comparison of hydrocephalus appearance at spinaldysraphia.

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Elshani, Besnik; Lenjani, Basri

2013-01-01

Congenital malformation of spinal dysraphism followed by hydrocephalus are phenomenon reveals during intrauterine child growth. Prime objective of this work was to present Comparison of hydrocephalus appearance at spinal dysraphism respectively at its meningocele and myelomeningocele forms in Neurosurgery Clinic in UCC in Prishtina. It is perfected with retrospective and prospective method precisely of its epidemiologic part summarizing notices from patients' histories which in 2000-2006 are hospitalized in Neurosurgery Clinic from (QFLPK)--Pediatric Clinic and Children Box (Department)--Gynecology Clinic and from Sanitary Regional Center throughout Kosova. Our study objects were two groups, as the first group 90 patients with spinal dysraphism where neurosurgery operations were done and classified types of dysraphism. At myelomeningocele hydrocephalus has dominated and in a percent of appearance and as acute of its active form was 97% of hydrocephalus form where subjected to cerebrospinal liquid derivation with ventriculo -peritoneal shunt in comparison with meningocele we do not have involvation of spinal nerve element, hydrocephalus takes active form with intervention indication in 60% of cases. Reflection in shown deficit aspect is totally different at myelomeningoceles where lower paraplegia dominate more than paraparesis. The second patient operative technique developed by hydrocephalus with neurosurgical intervention indication has to do with placing of (VP) ventriculo- peritoneal system (shant) at myelomeningoceles with hydrocephalus 58 cases and 12 cases meningoceles with hydrocephalus. Post operative meningitis (shant meningitis): from 70 operated cases of hydrocephalus with spinal dysraphism shunts complications from all types are just cases. Finally that appearance of hydrocephalus compared at spinal dysraphism dominate at myellomeningoceles as in notice time aspect, it is persisting and further acute, with vital motivation for neurosurgical

Dusp16 Deficiency Causes Congenital Obstructive Hydrocephalus and Brain Overgrowth by Expansion of the Neural Progenitor Pool

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Ksenija Zega

2017-11-01

Full Text Available Hydrocephalus can occur in children alone or in combination with other neurodevelopmental disorders that are often associated with brain overgrowth. Despite the severity of these disorders, the molecular and cellular mechanisms underlying these pathologies and their comorbidity are poorly understood. Here, we studied the consequences of genetically inactivating in mice dual-specificity phosphatase 16 (Dusp16, which is known to negatively regulate mitogen-activated protein kinases (MAPKs and which has never previously been implicated in brain development and disorders. Mouse mutants lacking a functional Dusp16 gene (Dusp16−/− developed fully-penetrant congenital obstructive hydrocephalus together with brain overgrowth. The midbrain aqueduct in Dusp16−/− mutants was obstructed during mid-gestation by an expansion of neural progenitors, and during later gestational stages by neurons resulting in a blockage of cerebrospinal fluid (CSF outflow. In contrast, the roof plate and ependymal cells developed normally. We identified a delayed cell cycle exit of neural progenitors in Dusp16−/− mutants as a cause of progenitor overproliferation during mid-gestation. At later gestational stages, this expanded neural progenitor pool generated an increased number of neurons associated with enlarged brain volume. Taken together, we found that Dusp16 plays a critical role in neurogenesis by balancing neural progenitor cell proliferation and neural differentiation. Moreover our results suggest that a lack of functional Dusp16 could play a central role in the molecular mechanisms linking brain overgrowth and hydrocephalus.

Enlarged thalamostriate vein causing unilateral Monro foramen obstruction. Case report.

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Leonardo, Jody; Grand, Walter

2009-06-01

Causes of unilateral hydrocephalus resulting from an obstruction at the Monro foramen include foraminal atresia, tumors, gliosis, contralateral shunting, and infectious and inflammatory conditions. However, few reports in the literature cite vascular lesions as the cause of the obstruction. To their knowledge, the authors present the first report of unilateral hydrocephalus occurring due to an abnormally enlarged thalamostriate vein independent of an arteriovenous malformation or developmental venous angioma. The condition was treated successfully by endoscopic septum pellucidum fenestration. A 28-year-old man was referred for evaluation due to a 10-year history of chronic headaches that worsened in severity over the past year. A CT scan of the head revealed unilateral right ventricular dilation. Cranial MR imaging with and without contrast administration showed a dilated right thalamostriate-internal vein complex without any evidence of associated arteriovenous malformation or venous angioma. Endoscopic exploration of the right lateral ventricle showed an enlarged subependymal thalamostriate vein obstructing the Monro foramen. An endoscopic fenestration of the septum pellucidum was performed, resulting in alleviation of the patient's symptoms. Abnormally enlarged venous structures may cause obstructive unilateral hydrocephalus and can be a rare cause of chronic, intermittent headaches in adults. Endoscopic fenestration of the septum pellucidum is an effective treatment.

Fluid Distribution Pattern in Adult-Onset Congenital, Idiopathic, and Secondary Normal-Pressure Hydrocephalus: Implications for Clinical Care.

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Yamada, Shigeki; Ishikawa, Masatsune; Yamamoto, Kazuo

2017-01-01

In spite of growing evidence of idiopathic normal-pressure hydrocephalus (NPH), a viewpoint about clinical care for idiopathic NPH is still controversial. A continuous divergence of viewpoints might be due to confusing classifications of idiopathic and adult-onset congenital NPH. To elucidate the classification of NPH, we propose that adult-onset congenital NPH should be explicitly distinguished from idiopathic and secondary NPH. On the basis of conventional CT scan or MRI, idiopathic NPH was defined as narrow sulci at the high convexity in concurrent with enlargement of the ventricles, basal cistern and Sylvian fissure, whereas adult-onset congenital NPH was defined as huge ventricles without high-convexity tightness. We compared clinical characteristics and cerebrospinal fluid distribution among 85 patients diagnosed with idiopathic NPH, 17 patients with secondary NPH, and 7 patients with adult-onset congenital NPH. All patients underwent 3-T MRI examinations and tap-tests. The volumes of ventricles and subarachnoid spaces were measured using a 3D workstation based on T2-weighted 3D sequences. The mean intracranial volume for the patients with adult-onset congenital NPH was almost 100 mL larger than the volumes for patients with idiopathic and secondary NPH. Compared with the patients with idiopathic or secondary NPH, patients with adult-onset congenital NPH exhibited larger ventricles but normal sized subarachnoid spaces. The mean volume ratio of the high-convexity subarachnoid space was significantly less in idiopathic NPH than in adult-onset congenital NPH, whereas the mean volume ratio of the basal cistern and Sylvian fissure in idiopathic NPH was >2 times larger than that in adult-onset congenital NPH. The symptoms of gait disturbance, cognitive impairment, and urinary incontinence in patients with adult-onset congenital NPH tended to progress more slowly compared to their progress in patients with idiopathic NPH. Cerebrospinal fluid distributions and

Congenital constriction of the foramen of Monro

International Nuclear Information System (INIS)

Marions, O.; Boethius, J.

1986-01-01

We report two cases of hydrocephalus in adults. The radiological investigations and direct inspection during surgery in one of the cases indicate that the hydrocephalus is caused in both cases by a benign stricture in the region of the foramen of Monro and that this constriction is congenital. This origin of hydrocephalus has not been reported previously in adults. (orig.)

Papilledema in children with hydrocephalus: incidence and associated factors.

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Lee, Haeng Jin; Phi, Ji Hoon; Kim, Seung-Ki; Wang, Kyu-Chang; Kim, Seong-Joon

2017-06-01

OBJECTIVE The aim of this study was to report the incidence of and the factors associated with papilledema in children with hydrocephalus. METHODS Patients younger than 15 years of age who had been diagnosed with hydrocephalus and treated by extra-ventricular drainage or ventriculoperitoneal shunt surgery between 2005 and 2015 were retrospectively reviewed. Factors including patient age and sex, etiology of hydrocephalus, duration of signs or symptoms, intracranial pressure (ICP), and presence of papilledema were evaluated. RESULTS Forty-six patients, whose mean age was 6.3 ± 4.7 years, were included in the study. The 19 patients without papilledema had a mean age of 2.7 ± 2.7 years, and the 27 patients with papilledema had a mean age of 8.8 ± 4.2 years (p hydrocephalus were tumor (59%), congenital anomaly (19%), hemorrhage (13%), and infection (9%). CONCLUSIONS Papilledema was more common in patients who were older, who had higher ICP, and whose hydrocephalus had been induced by brain tumor. However, since papilledema was absent in 41% of the children with hydrocephalus, papilledema's absence does not ensure the absence of hydrocephalus, especially in younger patients.

Mixed Feelings of Children and Adolescents with Unilateral Congenital Below Elbow Deficiency : An Online Focus Group Study

NARCIS (Netherlands)

de Jong, I.G.M.; Reinders-Messelink, H.A.; Janssen, W.G.M.; Poelma, M.J.; van Wijk, I.; van der Sluis, C.K.

2012-01-01

The existing literature is inconsistent about the psychosocial functioning of children and adolescents with Unilateral Congenital Below Elbow Deficiency (UCBED). The objective of this qualitative study was to explore the psychosocial functioning of children and adolescents with UCBED in terms of

Mixed feelings of children and adolescents with unilateral congenital below elbow deficiency: An online focus group study

NARCIS (Netherlands)

I.G.M. de Jong (Ingrid); H.A. Reinders-Messelink (Heleen); W.G.M. Janssen (Wim); M.J. Poelma (Margriet); I. van Wijk (Iris); C.K. van der Sluis (Corry)

2012-01-01

textabstractThe existing literature is inconsistent about the psychosocial functioning of children and adolescents with Unilateral Congenital Below Elbow Deficiency (UCBED). The objective of this qualitative study was to explore the psychosocial functioning of children and adolescents with UCBED in

DTI study of Children with Congenital Hydrocephalus: 1 Year Post-Surgical Outcomes

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Mangano, Francesco T.; Altaye, Mekibib; McKinstry, Robert C.; Shimony, Joshua S.; Powell, Stephanie K.; Phillips, Jannel M.; Barnard, Holly; Limbrick, David D.; Holland, Scott K.; Jones, Blaise V.; Dodd, Jonathon; Simpson, Sarah; Deanna, Mercer; Rajagopal, Akila; Bidwell, Sarah; Yuan, Weihong

2016-01-01

Object To investigate white matter structural abnormalities using diffusion tensor imaging (DTI) in children with hydrocephalus before CSF diversionary surgery (including ventriculoperitoneal shunting and endoscopic third ventriculoscopy) and the course of recovery post-surgery in association with neuropsychological and behavioral outcome. Methods This was a prospective study that included 54 children with congenital hydrocephalus (21F/33M; age range: 0.03–194.5 months) who underwent surgery and 64 normal controls (30F/34M, age range: 0.30–197.75 months). DTI and neurodevelopmental outcome data were collected once in the control group and at pre-surgery, 3-month, and 12-month post-surgery in the patients. DTI measures, including fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) values were extracted from the genu of corpus callosum (gCC) and the posterior limb of internal capsule (PLIC). Group analysis was performed first cross-sectionally to quantify DTI abnormalities at three time points by comparing the controls and the patients group at the three time points separately. Longitudinal comparisons were conducted pairwise between different time points in patients whose data were acquired at multiple time points. Neurodevelopmental data were collected and analyzed using the Adaptive Behavior Assessment System, Second Edition (ABAS-II) and the Bayley Scales of Infant Development, Third Edition (Bayley-III). Correlation analyses were performed between DTI and behavioral outcomes. Results Significant DTI abnormalities were found in both the gCC (lower FA and higher MD, AD, and RD) and the PLIC (higher FA, lower AD and RD) at pre-surgery. The DTI measures in the gCC remained mostly abnormal at 3-month and 12-month post-surgery. The DTI abnormalities in the PLIC were significant in FA and AD at 3-month post-surgery but did nor persist when tested at 12-month post-surgery. Significant longitudinal DTI changes in the

Clinical analysis of aqueductal stenosis in patients with hydrocephalus in a Kenyan setting.

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Kaur, Loyal Poonamjeet; Munyiri, Nderitu Joseph; Dismus, Wekesa Vincent

2017-01-01

Aqueductal stenosis is the commonest cause of congenital hydrocephalus. The scope of this paper is to highlight the disease burden of hydrocephalus attributed to aqueductal stenosis which still remains unknown in our setting. In a descriptive cross-sectional study, 258 records of patients diagnosed with hydrocephalus were analyzed after ethical approval from Kenyatta National Hospital- University of Nairobi (KNH-UON) ethics and research committee from January 2010 to May 2016. Patients with a diagnosis of hydrocephalus due to aqueductal stenosis were included in this study. Patients age, sex, mode of delivery, associated comorbidities, presenting complaints, neurosurgical intervention performed, Kafarnosky score were recorded. Data were divided into 2 sets based on the patient's age i.e. whether 12 years. Data were recorded on google data collection form and analyzed using Google spreadsheets. Out of 258 cases of hydrocephalus, 52 had aqueductal stenosis. Male to female sex ratio for this condition was 3:2. There were 25 cases 12 years old who were diagnosed with hydrocephalus due to aqueductal stenosis. Associated conditions were bilateral congenital talipes equinovarus, spina bifida, Arnold Chairi malformations, meningitis and HIV. The presenting complaints differed according to the age groups. Neurosurgical interventions included Endoscopic Third Ventriculostomy (ETV) in 21 cases, insertion of Ventriculoperitoneal (VP) shunt and ETV were done in 3 cases while the rest had only insertion of VP shunt. The Kafanosky score improve from hydrocephalus. Clinical presentation differs according to patients age. Accurate diagnosis and treatment remain a cardinal to improving patient outcome.

A rare combination: congenital factor VII deficiency with Chiari malformation.

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Bay, Ali; Aktekin, Elif; Erkutlu, Ibrahim

2015-12-01

Congenital factor (VII) deficiency is a rare bleeding disorder. We present a patient with congenital FVII deficiency and congenital hydrocephalus who underwent a ventriculoperitoneal shunt operation and needed no prophylaxis after the procedure.

High incidence of hydrocephalus following prenatal exposure to X-irradiation at early gestational stage in mice

International Nuclear Information System (INIS)

Aolad, H.; Inouye, Minoru; Hayasaka, Shizu; Darmanto, W.; Murata, Yoshiharu

1998-01-01

Hydrocephalus is one of the severe brain anomalies. Several causes of congenital hydrocephalus have been reported and, it is known that radiation is one of those. The current study was designed to obtain postnatally viable hydrocephalic offspring at a high incidence following X-radiation. This finding will be helpful to elucidate the mechanism of congenital hydrocephalus caused by X-radiation. Twenty pregnant Slc:ICR mice, 5 in each group, were exposed to X-irradiation at a dose of 1.0 Gy on gestational days 7 (G7), G8, G9 or G10. The incidence of hydrocephalus was high in the group exposed on G7. An additional 21 pregnant mice were then exposed to a dose of 1.2 Gy, 1.3 Gy, 1.4 Gy or 1.5 Gy X-radiation on G7. The highest incidence of hydrocephalic offspring was found following exposure to 1.4 Gy X-radiation. (author)

Double elevator weakening for unilateral congenital superior oblique palsy with ipsilateral superior rectus contracture and lax superior oblique tendon.

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Khan, Arif O

2012-06-01

In unilateral congenital superior oblique palsy, a large hypertropia is sometimes associated with ipsilateral contracture of the superior rectus muscle and apparent overaction of the contralateral superior oblique. Ipsilateral double elevator weakening is one surgical approach; however, this procedure could compromise supraduction. We report a series of three consecutive patients who underwent ipsilateral superior rectus and inferior oblique recessions for unilateral superior oblique palsy. Intraoperatively, all three patients were found to have a lax ipsilateral superior oblique tendon. Postoperatively, all three patients had satisfactory correction of the hypertropia and abnormal head position with minimal supraduction defect. This procedure seems to be an acceptable initial surgical option for treating congenital superior oblique muscle palsy with ipsilateral contracture of the superior rectus muscle, even when the ipsilateral superior oblique tendon is lax. Copyright © 2012 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.

[Importance of occlusion therapy for amblyopia in partial unilateral congenital cataracts that are discovered late].

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Denion, E; Dedes, V; Bonne, M; Labalette, P; Berger, C; Guilbert, F; Bouckehove, S; Rouland, J-F

2004-11-01

The aim of this study is to investigate the importance of occlusion therapy for amblyopia in patients with partial unilateral congenital cataracts that were discovered after 24 months of age. A retrospective study was conducted on 11 patients, each of whom underwent a clinical examination including a cycloplegic refraction with atropine. The average age when the cataract was diagnosed was 35 months. The average distance visual acuity was 6/78 and the average near visual acuity was 35/175. Occlusion therapy using adhesive patches was started after refractive error correction. In two cases, observance was mediocre. Ametropia was found in every patient, with anisometropia in nine patients (alpha occlusion therapy for amblyopia, the average visual acuity significantly improved to 6/22 in distance vision (alpha Amblyopia is related to lens opacities as well as frequently associated anisometropia. Functional improvement is greater in near vision than in distance vision. With occlusion therapy for amblyopia, accommodation is preserved. This factor is of utmost importance as near vision is preferential in young children. This study provides an opportunity to recall the importance of refraction and occlusion therapy for amblyopia, which must be systematically attempted in cases of partial unilateral congenital cataracts before considering a surgical procedure.

The role of bone conduction hearing aids in congenital unilateral hearing loss: A systematic review.

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Liu, C Carrie; Livingstone, Devon; Yunker, Warren K

2017-03-01

To systematically review the literature on the audiological and/or quality of life benefits of a bone conduction hearing aid (BCHA) in children with congenital unilateral conductive or sensorineural deafness. A systematic search was performed according to the PRISMA guidelines using the PubMed, Medline, and Embase databases. Data were collected on the following outcomes of interest: speech reception threshold, speech discrimination, sound localization, and quality of life measures. Given the heterogeneity of the data for quantitative analysis, the results are qualitatively summarized. Eight studies were included in the review. Four studies examined the audiological outcomes associated with bone conduction hearing aid implantation. There was a consistent gain in speech reception thresholds and speech discrimination, especially in noisy environments. Results pertaining to sound localization was inconsistent. The studies that examined quality of life measures reported a high usage rate of BCHAs among children. Quality of life improvements are reported with suggested benefit in the subdomain of learning. Given the potential benefits of a BCHA, along with the fact that it can be safely trialed using a headband, it is reasonable to trial a BCHA in children with congenital unilateral deafness. Should the trial offer audiological and/or quality of life benefits for the individual child, then BCHA implantation can be considered. Copyright © 2017 Elsevier B.V. All rights reserved.

Diffuse choroid plexus hyperplasia: an under-diagnosed cause of hydrocephalus in children?

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Aziz, Azian Abd.; Coleman, Lee [Royal Children' s Hospital Melbourne, Department of Medical Imaging, Parkville, Victoria (Australia); Morokoff, Andrew; Maixner, Wirginia [Royal Children' s Hospital Melbourne, Department of Neurosurgery, Parkville (Australia)

2005-08-01

Hydrocephalus is a common neurological disorder in children and the result of a variety of causes. However, with the advancement of imaging modalities, particularly MRI, previously reported rarer causes of hydrocephalus in children are now being more readily appreciated. We report an 11-year-old boy with diffuse villous hyperplasia of the choroid plexus. He had a ventriculo-peritoneal (VP) shunt in-situ and a prior diagnosis from infancy of congenital aqueduct stenosis as the cause of his hydrocephalus. His current presentation was with further shunt dysfunction. CT and MRI demonstrated enlarged choroid plexuses but did not confirm aqueduct stenosis. CSF overproduction was demonstrated from the externalized ventricular drain. The enlarged choroid plexuses were surgically resected and histology confirmed choroid plexus hyperplasia. Identification of choroid plexus hyperplasia is important since the neurosurgical management of hydrocephalus is not VP shunt insertion, but resection of the hyperplastic choroid plexus. (orig.)

Diffuse choroid plexus hyperplasia: an under-diagnosed cause of hydrocephalus in children?

International Nuclear Information System (INIS)

Aziz, Azian Abd.; Coleman, Lee; Morokoff, Andrew; Maixner, Wirginia

2005-01-01

Hydrocephalus is a common neurological disorder in children and the result of a variety of causes. However, with the advancement of imaging modalities, particularly MRI, previously reported rarer causes of hydrocephalus in children are now being more readily appreciated. We report an 11-year-old boy with diffuse villous hyperplasia of the choroid plexus. He had a ventriculo-peritoneal (VP) shunt in-situ and a prior diagnosis from infancy of congenital aqueduct stenosis as the cause of his hydrocephalus. His current presentation was with further shunt dysfunction. CT and MRI demonstrated enlarged choroid plexuses but did not confirm aqueduct stenosis. CSF overproduction was demonstrated from the externalized ventricular drain. The enlarged choroid plexuses were surgically resected and histology confirmed choroid plexus hyperplasia. Identification of choroid plexus hyperplasia is important since the neurosurgical management of hydrocephalus is not VP shunt insertion, but resection of the hyperplastic choroid plexus. (orig.)

Radionuclide cisternography in diagnostics of obstruction hydrocephalus in introduced ventricculoperitoneal shunt

International Nuclear Information System (INIS)

Cernochova, I.; Lipina, R.

2004-01-01

To confirm or exclude a diagnosis of internal obstruction hydrocephalus in an 8-year old girl with an introduced ventriculoperitoneal (V-P) shunt Establishing the V-P shunt is indisputably the route of choice in non-communicating hydrocephalus. The existence of a V-P shunt, however, is connected with numerous risks, especially of injuries, infection as well as increased intraabdominal pressure - e.g. in pregnancy. The development of endoscopy in neurosurgery allows the creation of communication via the bottom of the third ventricle with basal cisterns, and the subsequent cancellation of a V-P shunt. We describe the case of an 8-year old girl with congenital internal hydrocephalus with an assumed obliteration of the Sylvian aqueduct with an established V-P shunt. An MR scan described the membrane in the area of the Sylvian aqueduct, but the disproportion between the dilatation of the lateral ventricles, third ventricle and fourth ventricle led the physician to doubt as to the accuracy of the diagnosis of internal obstruction hydrocephalus. Therefore we performed a radionuclide cisternography (in a modified manner), which proved an existing communication between the third and fourth cerebral ventricle and which contradicted the clinical diagnosis of obstruction hydrocephalus. (author)

Mixed Feelings of Children and Adolescents with Unilateral Congenital Below Elbow Deficiency: An Online Focus Group Study

OpenAIRE

de Jong, Ingrid G.M.; Reinders-Messelink, Heleen A.; Janssen, Wim G.M.; Poelma, Margriet J.; van Wijk, Iris; van der Sluis, Corry K.

2012-01-01

textabstractThe existing literature is inconsistent about the psychosocial functioning of children and adolescents with Unilateral Congenital Below Elbow Deficiency (UCBED). The objective of this qualitative study was to explore the psychosocial functioning of children and adolescents with UCBED in terms of their feelings about the deficiency and what helps them to cope with those feelings. Additionally, the perspectives of prosthesis wearers and non-wearers were compared, as were the perspec...

The Unilateral Below Elbow Test: a function test for children with unilateral congenital below elbow deficiency.

Science.gov (United States)

Bagley, Anita M; Molitor, Fred; Wagner, Lisa V; Tomhave, Wendy; James, Michelle A

2006-07-01

The Unilateral Below Elbow Test (UBET) was developed to evaluate function in bimanual activities for both the prosthesis wearer and non-wearer. Nine tasks were chosen for each of four age-specific categories defined by development stages of hand function (2-4y, 5-7y, 8-10y, and 11-21y). Two scales, Completion of Task and Method of Use, were designed to rate performance. To measure reliability, four occupational therapists scored samples of videotaped UBET performances. For Completion of Task, an interval scale, agreement in scoring was measured with interclass correlation coefficients (ICC; n=9; five females, four males). For Method of Use, a nominal scale, chance-adjusted association was calculated with Cohen's kappa coefficients (interobserver n=198; 111 females, 87 males; intraobserver n=93; 56 females, 37 males). For Completion of Task, the average ICC was 0.87 for the prosthesis-on condition, and 0.85 for the prosthesis-off condition. ICCs exceeded 0.80 for eight out of nine tasks for the two older age groups, but for only five out of nine tasks in the younger age groups. Higher inter- and intraobserver kappa coefficients for Method of Use resulted when scoring children with their prostheses on versus off. The oldest age group had lower kappa values than the other three groups. The UBET is recommended for the functional evaluation of Completion of Task in children with unilateral congenital below elbow deficiency with and without their prostheses. Method of Use scoring can evaluate individuals for directed therapy interventions or prosthetic training.

Cirurgia da catarata infantil unilateral Unilateral pediatric cataract surgery

Directory of Open Access Journals (Sweden)

Adriana Maria Drummond Brandão

2008-04-01

Full Text Available OBJETIVO: Analisar os resultados visuais de uma série de crianças operadas de catarata unilateral. MÉTODOS: Um estudo retrospectivo foi realizado através da análise de 35 prontuários médicos do Serviço de Catarata Congênita da UNIFESP/EPM. RESULTADOS: Quanto à etiologia, a primeira causa de catarata foi idiopática, a segunda causa foi o trauma e a terceira foi a rubéola congênita. Em 51,4% dos olhos tinham acuidade visual pré-operatória de ausência de fixação. E em 42,8% dos casos operados a acuidade visual final foi igual ou melhor que 20/200. DISCUSSÃO: Embora a cirurgia em catarata unilateral seja motivo de controvérsias entre os oftalmologistas, obteve-se melhora de acuidade visual em número significativo de casos.PURPOSE: To analyze the results in a series of children submitted to unilateral cataract surgery. METHODS: A retrospective study was conducted through the analysis of 35 patient files from the Congenital Cataract Service of UNIFESP/EPM. RESULTS: The main cause of unilateral cataract was idiopathic, the second cause was ocular trauma and the third cause was congenital rubella. Initial visual acuity was very poor in 51.4% of the cases (did not fix or follow, and the best corrected final visual acuity was better than 20/200 in 42.8% of the eyes. DISCUSSION: Although controversial, the surgical treatment of unilateral cataract, in this study, showed improvement in many cases.

Unilateral fixation for treatment of occipitocervical instability in children with congenital vertebral anomalies of the craniocervical junction.

Science.gov (United States)

Mazur, Marcus D; Ravindra, Vijay M; Brockmeyer, Douglas L

2015-04-01

OBJECT Patients with occipitocervical (OC) instability from congenital vertebral anomalies (CVAs) of the craniocervical junction (CCJ) often have bony abnormalities that make instrumentation placement difficult. Within this patient population, some bilateral instrumentation constructs either fail or are not feasible, and a unilateral construct must be used. The authors describe the surgical management and outcomes of this disorder in patients in whom unilateral fixation constructs were used to treat OC instability. METHODS From a database of OC fusion procedures, the authors identified patients who underwent unilateral fixation for the management of OC instability. Patient characteristics, surgical details, and radiographic outcomes were reviewed. In each patient, CT scans were performed at least 4 months after surgery to evaluate for fusion. RESULTS Eight patients with CVAs of the CCJ underwent unilateral fixation for the treatment of OC instability. For 4 patients, the procedure occurred after a bilateral OC construct failed or infection forced hardware removal. For the remainder, it was the primary procedure. Two patients required reoperation for hardware revision and 1 developed nonunion requiring revision of the bone graft. Ultimately, 7 patients demonstrated osseous fusion on CT scans and 1 had a stable fibrous union. CONCLUSIONS These findings demonstrate that a unilateral OC fixation is effective for the treatment of OC instability in children with CVAs of the CCJ in whom bilateral screw placement fails or is not feasible.

Normal pressure hydrocephalus

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Hydrocephalus - occult; Hydrocephalus - idiopathic; Hydrocephalus - adult; Hydrocephalus - communicating; Dementia - hydrocephalus; NPH ... Ferri FF. Normal pressure hydrocephalus. In: Ferri FF, ed. ... Elsevier; 2016:chap 648. Rosenberg GA. Brain edema and disorders ...

Unilateral congenital corneal keloid with anterior segment mesenchymal dysgenesis and subluxated lens: case report and review of literature.

Science.gov (United States)

Vanathi, M; Sen, Seema; Panda, Anita; Dada, Tanuj; Behera, Geeta; Khokhar, Sudharshan

2007-01-01

To report the unusual association of unilateral congenital corneal keloid with anterior-segment mesenchymal dysgenesis and bilateral subluxated lens. A 20-year old man presented with a mass lesion involving the left cornea. The corneal lesion had been present since birth. On biomicroscopic examination, a well-defined vascularized, grayish-white mass occupying the whole of the left cornea was seen. The right eye showed multiple peripheral corneal opacities with iridocorneal adhesions, a poorly defined supranasal limbus, and a subluxated lens. Excision biopsy of the mass was done for histopathologic examination. Histopathologic examination of the excised corneal mass showed features consistent with that of a corneal keloid: thickened keratinized epithelium, absent Bowman membrane layer, and fibrovascular hyperplasia composed of hyalinized collagen fibers with irregular orientation of the collagen lamellae. During penetrating keratoplasty of the left eye, an anomalous iris pattern with poorly defined angle and a supranasal subluxated lens was also observed. Extraction of the subluxated lens was also done. The graft failed subsequent to a nonhealing persistent epithelial defect. Our case report highlights the rare association of a unilateral congenital corneal keloid with anterior-segment mesenchymal dysgenesis and bilateral subluxated lens.

Hydrocephalus

International Nuclear Information System (INIS)

Bradley, W.G.

1990-01-01

In benign external hydrocephalus, the increased pressure due to mild communicating hydrocephalus both increases ventricular size and increases the accumulation of fluid over the convexities in the subarachnoid space, shown in a six-month-old child. Since the sutures can separate in this age group, the extra-axial space represents an additional site of CSF accumulation. Atrophy is generally distinguishable from hydrocephalus by either CT or MRI on the basis of associated enlargement of the cortical sulci. When both are enlarged, ventricular enlargement out of proportion to sulcal enlargement is considered indicative of hydrocephalus

Encephalocele development from a congenital meningocele: case report.

Science.gov (United States)

Gandhoke, Gurpreet S; Goldschmidt, Ezequiel; Kellogg, Robert; Greene, Stephanie

2017-11-01

A fetal MRI study obtained at 21 weeks' gestation revealed a suboccipital meningocele without hydrocephalus. One day after term birth, MRI demonstrated an acquired cerebellar encephalocele, and MRI obtained 5 months later showed progressive enlargement of the encephalocele, still without obvious hydrocephalus. The patient underwent an operation in which an external ventricular drain was placed, the grossly normal cerebellum was reduced into the posterior fossa without resection, and the dural defect was closed. The drain was weaned out over 5 days, and no ventriculoperitoneal shunt was placed. Postoperative MR images revealed normal cerebellum and no hydrocephalus. The patient is developmentally normal. Meningocele and encephalocele are embryologically distinct. An acquired encephalocele could develop from hydrocephalus (which was not present in this case), or secondary to the lower resistance to expansion into the dural defect of the meningocele relative to the resistance to expansion of the fetal skull. The cerebellar tissue was normal in this case, and was thus preserved. The developmental prognosis is excellent. To the authors' knowledge, this is the first reported case of this occurrence. It is important to differentiate between congenital and acquired encephalocele etiologies, because resection of the cerebellar tissue in an acquired encephalocele (as is routinely done in cases of congenital encephalocele) would be expected to result in neurological deficits.

Congenital anomalies of the neonatal head

International Nuclear Information System (INIS)

Benson, C.B.; Teele, R.L.; Dobkin, G.R.; Fine, C.; Bundy, A.L.; Doubilet, P.M.

1987-01-01

US is a noninvasive modality that has proved highly valuable in the assessment of congenital intracranial anomalies in the neonate. The patterns of malformation must be familiar to those who obtain and interpret neonatal cranial sonograms. The authors present a variety of cases of congenital anomalies studied with US, including agenesis of the corpus callosum, Dandy-Walker malformation, occipital enecphalocele, congenital hydrocephalus, vein of Galen aneurysm, hydranencephaly, holoprosencephaly, absence of the septum pellucidum, schizencephaly, and hypoplastic cerebellum. Correlation with other radiographic modalities and pathologic follow-up, available in a majority of cases, is included

Sound Localization in Patients With Congenital Unilateral Conductive Hearing Loss With a Transcutaneous Bone Conduction Implant.

Science.gov (United States)

Vyskocil, Erich; Liepins, Rudolfs; Kaider, Alexandra; Blineder, Michaela; Hamzavi, Sasan

2017-03-01

There is no consensus regarding the benefit of implantable hearing aids in congenital unilateral conductive hearing loss (UCHL). This study aimed to measure sound source localization performance in patients with congenital UCHL and contralateral normal hearing who received a new bone conduction implant. Evaluation of within-subject performance differences for sound source localization in a horizontal plane. Tertiary referral center. Five patients with atresia of the external auditory canal and contralateral normal hearing implanted with transcutaneous bone conduction implant at the Medical University of Vienna were tested. Activated/deactivated implant. Sound source localization test; localization performance quantified using the root mean square (RMS) error. Sound source localization ability was highly variable among individual subjects, with RMS errors ranging from 21 to 40 degrees. Horizontal plane localization performance in aided conditions showed statistically significant improvement compared with the unaided conditions, with RMS errors ranging from 17 to 27 degrees. The mean RMS error decreased by a factor of 0.71 (p conduction implant. Some patients with congenital UCHL might be capable of developing improved horizontal plane localization abilities with the binaural cues provided by this device.

Neurolues congênita associada a hidrocefalia Congenital neurosyphilis associated with hydrocephalus

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Julinho Aisen

1970-09-01

Full Text Available É apresentado um caso de neurolues congênita (meningovascular e parenquimatosa associada a hidrocefalia do tipo bloqueado. A natureza luética da afecção foi confirmada por exames paraclínicos e histopatológico. O registro do caso se justifica pelo fato da não existência na literatura compulsada de outro semelhante comprovado histopatològicamente. A hidrocefalia ocorreu em conseqüência do bloqueio do sistema ventricular pelo processo inflamatório de natureza crônica.A case of meningovascular and parenchymatous neurosyphilis associated with a non-communicating hydrocephalus is reported. The syphilitic nature of the disease was confirmed by laboratory and histopathological findings. The authors were unable to find in the medical literature other reports of histologically documented cases of hydrocephalus secondary to a block of the ventricular system determined by neurosyphilis.

Unilateral CHARGE association

NARCIS (Netherlands)

Trip, J; van Stuijvenberg, M; Dikkers, FG; Pijnenburg, MWH

A case with a predominantly unilateral CHARGE association is reported. The CHARGE association refers to a combination of congenital malformations. This boy had left-sided anomalies consisting of choanal atresia. coloboma and peripheral facial palsy. The infant had a frontal encephalocele. an anomaly

Ventriculoperitoneal shunt for treatment of hydrocephalus in a French bulldog puppy

Science.gov (United States)

Giacinti, Jolene A.

2016-01-01

A 6.5-week-old bulldog was presented with lethargy, anorexia, and stunted growth. A domed skull, ventrolateral strabismus, hypermetria, and delayed hopping were observed. Congenital hydrocephalus was diagnosed and a ventriculoperitoneal shunt was placed. After surgery, a shunt obstruction occurred but resolved with treatment. The puppy responded well and neurological deficits continued to improve after surgery. PMID:26933271

Hydrocephalus due to multiple ependymal malformations is caused by mutations in the MPDZ gene.

Science.gov (United States)

Saugier-Veber, Pascale; Marguet, Florent; Lecoquierre, François; Adle-Biassette, Homa; Guimiot, Fabien; Cipriani, Sara; Patrier, Sophie; Brasseur-Daudruy, Marie; Goldenberg, Alice; Layet, Valérie; Capri, Yline; Gérard, Marion; Frébourg, Thierry; Laquerrière, Annie

2017-05-01

Congenital hydrocephalus is considered as either acquired due to haemorrhage, infection or neoplasia or as of developmental nature and is divided into two subgroups, communicating and obstructive. Congenital hydrocephalus is either syndromic or non-syndromic, and in the latter no cause is found in more than half of the patients. In patients with isolated hydrocephalus, L1CAM mutations represent the most common aetiology. More recently, a founder mutation has also been reported in the MPDZ gene in foetuses presenting massive hydrocephalus, but the neuropathology remains unknown. We describe here three novel homozygous null mutations in the MPDZ gene in foetuses whose post-mortem examination has revealed a homogeneous phenotype characterized by multiple ependymal malformations along the aqueduct of Sylvius, the third and fourth ventricles as well as the central canal of the medulla, consisting in multifocal rosettes with immature cell accumulation in the vicinity of ependymal lining early detached from the ventricular zone. MPDZ also named MUPP1 is an essential component of tight junctions which are expressed from early brain development in the choroid plexuses and ependyma. Alterations in the formation of tight junctions within the ependyma very likely account for the lesions observed and highlight for the first time that primary multifocal ependymal malformations of the ventricular system is genetically determined in humans. Therefore, MPDZ sequencing should be performed when neuropathological examination reveals multifocal ependymal rosette formation within the aqueduct of Sylvius, of the third and fourth ventricles and of the central canal of the medulla.

Hydrocephalus and intestinal aganglionosis : Is L1CAM a modifier gene in Hirschsprung disease?

NARCIS (Netherlands)

Parisi, MA; Kapur, RP; Neilson, [No Value; Hofstra, RMW; Holloway, LW; Michaelis, RC; Leppig, KA

2002-01-01

Congenital hydrocephalus associated with aqueductal stenosis and/or agenesis of the corpus callosum has been described in newborn males with mutations in L1CAM, a gene that encodes a neural cell adhesion molecule. These males usually have severe mental retardation and may have spastic paraplegia and

Congenital unilateral absence of the upper extremity may give rise to a specific kind of thoracolumbar curve.

Science.gov (United States)

Olgun, Z Deniz; Demirkiran, Gokhan; Polly, David; Yazici, Muharrem

2018-03-01

There is an increased incidence of scoliosis in patients with congenital malformations of the upper extremity even in the absence of overt vertebral abnormalities. In this case series, we summarize the curve characteristics of four patients presenting to two spine surgery clinics with unilateral amelia or phocomelia and a progressive scoliotic curve with the apex on the side of deficiency. All patients required orthopedic intervention for their curves. Amelia and phocomelia are severe congenital malformations of the upper limb affecting trunk balance and, conceivably, causing scoliosis with the absence of counterbalancing weight on the affected side. The combination of upper limb absence and same-sided scoliosis in these patients may provide a clue of the mechanical factors causing scoliosis in other disorders. In this article, we attempt to define this exceptional deformity, theorize on its etiology, and draw attention to this particular combination of problems. Case series; Level IV.

Imaging of the ventriculoperitoneal shunt-related complications in infants and children with hydrocephalus

International Nuclear Information System (INIS)

Jeon, Se Ok; Kim, Ji Hye; Oh, Eun Young; Hwang, Hee Young; Lee, Seon Kyu; Lee, Eun Joo; Kwak, Min Sook; Kim, Hyung Sik

1999-01-01

To evaluate the frequency and imaging findings of various ventriculo-peritoneal shunt-related complications in pediatric patients with hydrocephalus. We retrospectively reviewed 246 plain radiographs, three shuntograms, 53 ultrasounds, 133 CT scans, and 24 MR images obtained before and after the ventriculo-peritoneal shunt procedure in 33 pediatric patients with hydrocephalus. Using preoperative images, the etiology of the hydrocephalus was assessed. Changes in the size and shape of the ventricles, the location and continuity of shunt apparatus, and the presence of any abnormal enhancement, hemorrhage, edema or tissue loss, or other findings of complications, were analyzed on postoperative images ; the frequency and imaging findings of shunt-related complications such as shunt malfunction, infection, homorrhage or isolated ventricle, and complications caused by overdrainage, were thus evaluated. The frequency of such complications was analyzed according to the etiology of the hydrocephalus, and in addition, medical records were reviewed and correlated with imaging findings. In 18 of the 33 patients(54%), a total of 31 complications was detected. These were present in four of five cases (80%) of hydrocephalus caused by meningitis and ventriculitis, seven of twelve (58%) intraventricular hemorrhage, two of four (50%) unknown cases, three of nine (33%) congenital malformations, one of two (50%) tumors, and one (100%) congenital infection. Shunt malfunction was most common (n=15), and was accompanied by findings of enlarged ventricles, periventricular and peritubal edema, and abnormal location of the shunt tube. Symptoms and signs of increased intracranial pressure were also noted. Subdural hemorrhage and infection were present in four cases each ; findings of infection were enhancement of the ventricular wall, meninges, and parenchyma, as well as sonographically noted intraventricular septation and increased ventricular wall echo. Isolated lateral ventricle (n=4) or 4

Clinical and morphological characteristics of malformations in infants with congenital cytomegalovirus infection and congenital toxoplasmosis

Directory of Open Access Journals (Sweden)

L. Yu. Barycheva

2015-01-01

Full Text Available The results of following up infants with intrauterine infections and malformations were retrospectively analyzed. Infants with malformations were diagnosed as having congenital cytomegalovirus infection and congenital toxoplasmosis in 127 and 69 cases, respectively. The aim of the study was to characterize malformations in infants with congenital cytomegalovirus and congenital Toxoplasma infections. The infants with malformations in congenital cytomegalovirus infection were found to have higher mortality rates (61,4% than those with congenital toxoplasmosis (34,8%. Postmortem analysis indicated that there was a predominance of embryopathies in infants with congenital cytomegalovirus infection and that of fetopathies in those with congenital toxoplasmosis. The dead infants with congenital cytomegalovirus infection had more commonly developed visceral defects, including heart diseases, pneumopathies, gastrointestinal and genitourinary abnormalities; fetopathies of the central nervous system and eye were prevalent in congenital toxoplasmosis. The surviving children with congenital toxoplasmosis were more frequently observed to have disabling CNS and ocular sequels as obstructive hydrocephalus, infantile cerebral palsy, complete or partial blindness, and cerebrasthenic disorders than those with congenital cytomegalovirus infection. 

Hydrocephalus

Science.gov (United States)

... drowsiness, irritability, or other changes in personality or cognition including memory loss. Symptoms of normal pressure hydrocephalus ... example, if the symptoms of hydrocephalus return), medical attention should be sought immediately. top What is the ...

HYDROCEPHALUS IN THREE JUVENILE NORTH AMERICAN BLACK BEARS (URSUS AMERICANUS).

Science.gov (United States)

Ferguson, Sylvia H; Novak, Janelle; Hecht, Silke; Craig, Linden E

2016-06-01

Hydrocephalus has been reported in a variety of species, including the North American black bear ( Ursus americanus ). This report describes three cases of hydrocephalus in this species from wild bears aged 3-4 mo considered retrospectively from necropsy records of one institution. Clinical signs included cortical blindness and ataxia. Primary gross findings were doming of the skull, gyri compression and flattening, and lateral ventricle dilation. Two cases had severe bilateral ventricular dilation with loss of the septum pellucidum; atrophy of the surrounding corpus callosum; and bilateral periventricular tears involving the caudate nuclei, internal capsule, and adjacent cerebrum. Histologically, the cases with periventricular tearing had severe axonal loss and degeneration, malacia, hemorrhage, and variable periventricular astrocytosis. All cases were likely congenital, given the bears' age and lack of an apparent acquired obstruction.

Residual symptoms after surgery for unilateral congenital superior oblique palsy.

Science.gov (United States)

Caca, Ihsan; Sahin, Alparslan; Cingu, Abdullah; Ari, Seyhmus; Akbas, Umut

2012-01-01

To establish the surgical results and residual symptoms in 48 cases with unilateral congenital superior oblique muscle palsy that had surgical intervention to the vertical muscles alone. Myectomy and concomitant disinsertion of the inferior oblique (IO) muscle was performed in 38 cases and myectomy and concomitant IO disinsertion and recession of the superior rectus muscle in the ipsilateral eye was performed in 10 cases. The preoperative and postoperative vertical deviation values and surgical results were compared. Of the patients who had myectomy and concomitant IO disinsertion, 74% achieved an "excellent" result, 21% a "good" result, and 5% a "poor" result postoperatively. The difference in deviation between preoperative and postoperative values was statistically significant (P < .001). Of the patients who had myectomy and concomitant inferior oblique disinsertion and ipsilateral superior rectus recession, 50% achieved an "excellent" result, 20% a "good" result, and 30% a "poor" result postoperatively. The difference in deviation between preoperative and postoperative values was statistically significant (P < .001). Both procedures are effective and successful in patients with superior oblique muscle palsy, but a secondary surgery may be required. Copyright 2012, SLACK Incorporated.

SNX27 Deletion Causes Hydrocephalus by Impairing Ependymal Cell Differentiation and Ciliogenesis.

Science.gov (United States)

Wang, Xin; Zhou, Ying; Wang, Jian; Tseng, I-Chu; Huang, Timothy; Zhao, Yingjun; Zheng, Qiuyang; Gao, Yue; Luo, Hong; Zhang, Xian; Bu, Guojun; Hong, Wanjin; Xu, Huaxi

2016-12-14

therapeutic interest to congenital hydrocephalus. Copyright © 2016 the authors 0270-6474/16/3612586-12$15.00/0.

Complete dorsal pancreatic agenesis and unilateral renal agenesis.

Science.gov (United States)

Moreira, Adriana; Carvalho, André; Portugal, Inês; Jesus, José Miguel

2018-02-01

Dorsal pancreatic agenesis is a very rare congenital anomaly. Unilateral renal agenesis, on the other hand, is a relatively common congenital anomaly, although its etiology is not fully understood. Renal and pancreatic embryologic development appears to be nonrelated. We report a case of a 34-year-old man who was referred to our hospital for evaluation of cholestasis and microalbuminuria. Ultrasound and magnetic resonance imaging examinations showed empty right renal fossa and absence of the pancreatic neck, body, and tail. Our case report is the second case of a dorsal pancreatic agenesis and unilateral renal agenesis in a young male patient.

Congenital zygomatico-mandibular fusion (pseudo-syngnathia?) in conjunction with unilateral anophthalmia: review of terminology and classification.

Science.gov (United States)

Tauro, David P; Kallappanavar, N K; Kiran, H Y; Girhe, Vijaykumar J

2012-09-01

Syngnathia per se is a rare congenital disorder. A literature survey reveals a total of 26 cases of syngnathia in the English literature since 1936, of which only seven cases involved fusion of the ascending ramus of the mandible to the posterior portion of the maxilla and zygomatic complex. The remaining 19 involved fusion of the alveolar ridges of the maxilla and mandible. This is a unique case of fusion of the mandible to the zygomatic complex presenting with a unilateral anophthalmic orbit in an 18-day-old neonate. The use of the term syngnathia has been reviewed and a modification in classification has been suggested.

Normal Pressure Hydrocephalus (NPH)

Science.gov (United States)

... local chapter Join our online community Normal Pressure Hydrocephalus (NPH) Normal pressure hydrocephalus is a brain disorder ... Symptoms Diagnosis Causes & risks Treatments About Normal Pressure Hydrocephalus Normal pressure hydrocephalus occurs when excess cerebrospinal fluid ...

Frontal and occipital horn ratio is associated with multifocal intraparenchymal hemorrhages in neonatal shunted hydrocephalus.

Science.gov (United States)

Oushy, Soliman; Parker, Jonathon J; Campbell, Kristen; Palmer, Claire; Wilkinson, Corbett; Stence, Nicholas V; Handler, Michael H; Mirsky, David M

2017-11-01

OBJECTIVE Placement of a cerebrospinal fluid diversion device (i.e., shunt) is a routine pediatric neurosurgical procedure, often performed in the first weeks of life for treatment of congenital hydrocephalus. In the postoperative period, shunt placement may be complicated by subdural, catheter tract, parenchymal, and intraventricular hemorrhages. The authors observed a subset of infants and neonates who developed multifocal intraparenchymal hemorrhages (MIPH) following shunt placement and sought to determine any predisposing perioperative variables. METHODS A retrospective review of the electronic medical record at a tertiary-care children's hospital was performed for the period 1998-2015. Inclusion criteria consisted of shunt placement, age hydrocephalus. Markers of severity of ventriculomegaly (FOR) and ventricular response to CSF diversion (∆FOR) were significantly associated with occurrence of MIPH. Choice of shunt and etiology of hydrocephalus were also significantly associated with MIPH. After adjusting for corrected age, etiology of hydrocephalus, and shunt setting, the authors found that ∆FOR after shunting was still associated with MIPH. A prospective study of MIPH prevention strategies and assessment of possible implications for patient outcomes is needed.

An unusual cause of hydrocephalus: aqueductal developmental venous anomaly

Energy Technology Data Exchange (ETDEWEB)

Yagmurlu, Banu; Fitoz, Suat; Atasoy, Cetin; Erden, ilhan [Ankara University School of Medicine, Department of Radiology, Ankara (Turkey); Deda, Gulhis; Unal, Ozlem [Ankara University School of Medicine, Division of Pediatric Neurology, Ankara (Turkey)

2005-06-01

Vascular malformations are infrequent causes of aqueductal stenoses, developmental venous anomaly (DVA) being the rarest among them. DVAs, also known as venous angiomas, are congenital in origin and characterized by dilatation of vessels in the superficial and deep venous system. Although they are usually clinically silent, they can be complicated by hemorrhage, seizures and neurologic deficits. Herein, we report MR imaging findings of a 7-year-old girl whose hydrocephalus was due to an abnormal vein coursing through the aqueduct. (orig.)

Cerebrospinal fluid circulation and hydrocephalus.

Science.gov (United States)

Leinonen, Ville; Vanninen, Ritva; Rauramaa, Tuomas

2017-01-01

Hydrocephalus (HC) is classically defined as dynamic imbalance between the production and absorption of cerebrospinal fluid (CSF) leading to enlarged ventricles. Potential causative factors include various brain disorders like tumors causing obstruction of CSF flow within the ventricular system or the subarachnoid space. Classification of HC is based on the site of CSF flow obstruction guiding optimal treatment, with endoscopic third ventriculostomy in intraventricular obstruction and CSF shunt in communicating HC. Another clinically relevant classification is acute and chronic; the most frequent chronic form is idiopathic normal-pressure hydrocephalus (iNPH). The reported incidence of HC varies according to the study population and classification used. The incidence of congenital HC is approximately 0.4-0.6/1,000 newborns and the annual incidence of iNPH varies from 0.5/100,000 to 5.5/100,000. Radiologically, ventricular dilatation may be nonspecific, and differentiation of iNPH from other neurodegenerative diseases may be ambiguous. There are no known specific microscopic findings of HC but a systematic neuropathologic examination is needed to detect comorbid diseases and possible etiologic factors of HC. Depending on the etiology of HC, there are several nonspecific signs potentially to be seen. Copyright © 2017 Elsevier B.V. All rights reserved.

Unilateral Congenital Cataract: Clinical Profile and Presentation.

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Khokhar, Sudarshan; Jose, Cijin P; Sihota, Ramanjit; Midha, Neha

2018-03-01

To study the clinical profile and presentation of children with unilateral cataract. In this hospital-based, observational, cross-sectional study, patients 15 years of age or younger who presented with unilateral cataract were recruited. Cases of cataract secondary to causes such as trauma or uveitis were excluded. Age at detection and presentation, distance from the treatment center, presenting complaints, cataract morphology, and biometry were noted for each case. A total of 76 patients were recruited. Most patients presented with complaints of leukocoria. Persistent fetal vasculature accounted for 27.6% of cases and was the most common identifiable cause of cataract in this study. Subsequently, patients were divided into two groups: no persistent fetal vasculature (control) and persistent fetal vasculature. A male predominance was noted in both groups. The mean age at detection was 27.58 ± 37.02 and 6.17 ± 8.42 months and the mean age at presentation was 55.613 ± 45.21 and 14.83 ± 17.75 months in the control and persistent fetal vasculature groups, respectively. In the persistent fetal vasculature group, a significant difference was noted in the axial length, keratometry, and corneal diameter between the affected and normal eyes (P = .027, .00176, and .0114, respectively). In the control group, this difference was observed only in keratometry readings (P = .0464). The mean distance traveled by patients to reach the treatment center was 211 km. Persistent fetal vasculature is an important and less identified cause of unilateral cataract. A significant delay is noted in the detection and presentation of unilateral cataract. [J Pediatr Ophthalmol Strabismus. 2018;55(2):107-112.]. Copyright 2017, SLACK Incorporated.

Our initial experience with ventriculo-epiplooic shunt in treatment of hydrocephalus in two centers.

Science.gov (United States)

Grigorean, Valentin Titus; Sandu, Aurelia Mihaela; Popescu, Mihai; Florian, Ioan Stefan; Lupascu, Cristian Dumitru; Ursulescu, Corina Lupascu

Hydrocephalus represents impairment in cerebrospinal fluid (CSF) dynamics. If the treatment of hydrocephalus is considered difficult, the repeated revisions of ventriculo-peritoneal (VP) shunts are even more challenging. The aim of this article is to evaluate the efficiency of ventriculo-epiplooic (VEp) shunt as a feasible alternative in hydrocephalic patients. A technical modification regarding the insertion of peritoneal catheter was imagined: midline laparotomy 8-10cm long was performed in order to open the peritoneal cavity; the great omentum was dissected between its two layers; we placed the distal end of the catheter between the two epiplooic layers; a fenestration of 4cm in diameter into the visceral layer was also performed. A retrospective study of medical records of 15 consecutive patients with hydrocephalus treated with VEp shunt is also presented. Between 2008 and 2014 we performed VEp shunt in 15 patients: 5 with congenital hydrocephalus, 8 with secondary hydrocephalus and 2 with normal pressure hydrocephalus. There were 7 men and 8 women. VEp shunt was performed in 13 patients with multiple distal shunt failures and in 2 patients, with history of abdominal surgery, as de novo extracranial drainage procedure. The outcome was favorable in all cases, with no significant postoperative complications. VEp shunt is a new, safe and efficient surgical technique for the treatment of hydrocephalus. VEp shunt is indicated in patients with history of recurrent distal shunt failures, and in patients with history of open abdominal surgery and high risk for developing abdominal complications. Copyright © 2017 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

Twelve-year prevalence of common neonatal congenital malformations in Zhejiang Province, China.

Science.gov (United States)

Sun, Ge; Xu, Zhe-Ming; Liang, Jian-Feng; Li, Lin; Tang, Da-Xing

2011-11-01

One of the challenges that pediatricians face when examining birth defects is to understand the trends in its occurrence and provide clues to etiology. This study was undertaken to retrospectively assess the prevalence of 10 common neonatal congenital malformations by reviewing a database of all deliveries from 28 weeks up until 7 days of birth from January 1998 to December 2009 in Zhejiang Province, China. Ten common neonatal congenital malformations were selected for analysis. The incidence and the Cochran-Armitage Trend were assessed via SAS9.2. A P value ≤0.05 was considered statistically significant. Of 83 888 perinatals, 374 (4.46‰) suffered from congenital heart diseases (CHD), 77 (0.92‰) from congenital hydrocephalus, 32 (0.38‰) from intestinal atresia/stenosis, 36 (0.43‰) from anorectal malformations, 149 (1.78‰) from kidney malformations, 139 (3.31‰) from hypospadias (male), 178 (2.12‰) from orofacial clefts (OFC), 188 (2.24‰) from polydactyly, 62 (0.74‰) from syndactyly, and 269 (3.21‰) from accessory auricle anomaly. Their trend of prevalence varied as follows: CHD, P=0.0026; hydrocephalus, P=0.0042; intestinal atresia/stenosis, P=0.0103; anorectal malformations, P=0.4332; kidney malformations, Pmalformations (Pmalformations (P=0.4332), polydactyly (P=0.0867) and syndactyly (P=0.1941). The incidences of CHD, intestinal atresia/stenosis, kidney malformations, hypospadias, OFC, and accessory auricle anomaly have increased in the last 12 years, but the incidences of anorectal malformations, polydactyly and syndactyly remain stable. The incidence of hydrocephalus shows a downward trend.

Botulinum toxin for treating unilateral apraxia of eyelid opening in a patient with congenital myotonia

Directory of Open Access Journals (Sweden)

Estrella Fernández

Full Text Available ABSTRACT A 37-year-old female presented with severe apraxia of lid opening (ALO affecting the right upper lid associated with Becker congenital myotonia (MC. The patient had a history of right upper lid ptosis for 25 years that was exacerbated over the previous month with severe incapacity to open her right eye. No other associated neurological or ophthalmic symptoms were observed. The patient was treated with botulinum toxin (BoNT-A injection into the pretarsal and lateral canthus region of the orbicularis oculi of the affected eyelid. Treatment with BoNT-A is an effective method of managing ALO in Becker MC. This is the first case of unilateral ALO in the course of Becker MC that was successfully treated with injections of botulinum toxin.

Auditory agnosia due to long-term severe hydrocephalus caused by spina bifida - specific auditory pathway versus nonspecific auditory pathway.

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Zhang, Qing; Kaga, Kimitaka; Hayashi, Akimasa

2011-07-01

A 27-year-old female showed auditory agnosia after long-term severe hydrocephalus due to congenital spina bifida. After years of hydrocephalus, she gradually suffered from hearing loss in her right ear at 19 years of age, followed by her left ear. During the time when she retained some ability to hear, she experienced severe difficulty in distinguishing verbal, environmental, and musical instrumental sounds. However, her auditory brainstem response and distortion product otoacoustic emissions were largely intact in the left ear. Her bilateral auditory cortices were preserved, as shown by neuroimaging, whereas her auditory radiations were severely damaged owing to progressive hydrocephalus. Although she had a complete bilateral hearing loss, she felt great pleasure when exposed to music. After years of self-training to read lips, she regained fluent ability to communicate. Clinical manifestations of this patient indicate that auditory agnosia can occur after long-term hydrocephalus due to spina bifida; the secondary auditory pathway may play a role in both auditory perception and hearing rehabilitation.

Biomarkers in adult posthemorrhagic hydrocephalus.

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Hua, Cong; Zhao, Gang

2017-08-01

Posthemorrhagic hydrocephalus is a severe complication following intracranial hemorrhage. Posthemorrhagic hydrocephalus is often associated with high morbidity and mortality and serves as an important clinical predictor of adverse outcomes after intracranial hemorrhage. Currently, no effective medical intervention exists to improve functional outcomes in posthemorrhagic hydrocephalus patients because little is still known about the mechanisms of posthemorrhagic hydrocephalus pathogenesis. Because a better understanding of the posthemorrhagic hydrocephalus pathogenesis would facilitate development of clinical treatments, this is an active research area. The purpose of this review is to describe recent progress in elucidation of molecular mechanisms that cause posthemorrhagic hydrocephalus. What we are certain of is that the entry of blood into the ventricular system and subarachnoid space results in release of lytic blood products which cause a series of physiological and pathological changes in the brain. Blood components that can be linked to pathology would serve as disease biomarkers. From studies of posthemorrhagic hydrocephalus, such biomarkers are known to mutually synergize to initiate and promote posthemorrhagic hydrocephalus progression. These findings suggest that modulation of biomarker expression or function may benefit posthemorrhagic hydrocephalus patients.

Neonatal hydrocephalus is a result of a block in folate handling and metabolism involving 10-formyltetrahydrofolate dehydrogenase.

Science.gov (United States)

Naz, Naila; Jimenez, Alicia Requena; Sanjuan-Vilaplana, Anna; Gurney, Megan; Miyan, Jaleel

2016-08-01

Folate is vital in a range of biological processes and folate deficiency is associated with neurodevelopmental disorders such as neural tube defects and hydrocephalus (HC). 10-formyl-tetrahydrofolate-dehydrogenase (FDH) is a key regulator for folate availability and metabolic interconversion for the supply of 1-carbon groups. In previous studies, we found a deficiency of FDH in CSF associated with the developmental deficit in congenital and neonatal HC. In this study, we therefore aimed to investigate the role of FDH in folate transport and metabolism during the brain development of the congenital hydrocephalic Texas (H-Tx) rat and normal (Sprague-Dawley) rats. We show that at embryonic (E) stage E18 and E20, FDH-positive cells and/or vesicles derived from the cortex can bind methyl-folate similarly to folate receptor alpha, the main folate transporter. Hydrocephalic rats expressed diminished nuclear FDH in both liver and brain at all postnatal (P) ages tested (P5, P15, and P20) together with a parallel increase in hepatic nuclear methyl-folate at P5 and cerebral methylfolate at P15 and P20. A similar relationship was found between FDH and 5-methyl cytosine, the main marker for DNA methylation. The data indicated that FDH binds and transports methylfolate in the brain and that decreased liver and brain nuclear expression of FDH is linked with decreased DNA methylation which could be a key factor in the developmental deficits associated with congenital and neonatal HC. Folate deficiency is associated with neurodevelopmental disorders such as neural tube defects and hydrocephalus. 10-formyl-tetrahydrofolate-dehydrogenase (FDH) is a key regulator for folate availability and metabolic interconversion. We show that FDH binds and transports methylfolate in the brain. Moreover, we found that a deficiency of FDH in the nucleus of brain and liver is linked with decreased DNA methylation which could be a key factor in the developmental deficits associated with congenital and

Tratamiento simultáneo de anquilosis temporomandibular unilateral congénita y del microlaterognatismo asociado Simultaneously treament of congenital unilateral temporomandibular ankylosis and consecutive microlatherognathism

Directory of Open Access Journals (Sweden)

José Felipe Basulto Varela

2013-03-01

Full Text Available Este artículo tiene como objetivo reportar los resultados obtenidos a corto y largo plazo, del tratamiento con enfoque multidisciplinario de una anquilosis unilateral congénita de la articulación temporomandibular asociada a un síndrome de malformación embrionaria, en un niño de 12 años de edad, en el que se utilizó un distractor externo bidimensional con un doble propósito: como fijador para mantener el espacio logrado con la artroplastia y como distractor para elongar la rama mandibular hipotrófica, activado 5 días después de la osteotomía, con el objetivo de eliminar la anquilosis y el microlaterognatismo mandíbular consecutivo de ella, simultaneamente de manera funcional y dinámica.The paper reports the short- and long-term results obtained from the multidisciplinary treatment of a congenital unilateral ankylosis of the temporomandibular joint associated to an embryonic malformation in a 12-year-old boy, using an external bidimensional distraction device with a two-fold purpose: as fixator to maintain the space achieved by arthroplasty, and as distractor to elongate the hypotrophic mandibular branch, activated 5 days after osteotomy, with the purpose of eliminating ankylosis and consecutive mandibular microlaterognatism, both functionally and dynamically.

Hydrocephalus (For Parents)

Science.gov (United States)

... any of the ventricles, or something disturbs the recycling, CSF backs up and causes hydrocephalus. Hydrocephalus can ... and/or other repetitive eye movements Changes in personality, loss of new developmental abilities (like speaking or ...

Disruption of the mouse Jhy gene causes abnormal ciliary microtubule patterning and juvenile hydrocephalus

Science.gov (United States)

Appelbe, Oliver K.; Bollman, Bryan; Attarwala, Ali; Triebes, Lindy A.; Muniz-Talavera, Hilmarie; Curry, Daniel J.; Schmidt, Jennifer V.

2013-01-01

SUMMARY Congenital hydrocephalus, the accumulation of excess cerebrospinal fluid (CSF) in the ventricles of the brain, affects one of every 1,000 children born today, making it one of the most common human developmental disorders. Genetic causes of hydrocephalus are poorly understood in humans, but animal models suggest a broad genetic program underlying the regulation of CSF balance. In this study, the random integration of a transgene into the mouse genome led to the development of an early onset and rapidly progressive hydrocephalus. Juvenile hydrocephalus transgenic mice (JhylacZ) inherit communicating hydrocephalus in an autosomal recessive fashion with dilation of the lateral ventricles observed as early as postnatal day 1.5. Ventricular dilation increases in severity over time, becoming fatal at 4-8 weeks of age. The ependymal cilia lining the lateral ventricles are morphologically abnormal and reduced in number in JhylacZ/lacZ brains, and ultrastructural analysis revealed disorganization of the expected 9+2 microtubule pattern. Rather, the majority of JhylacZ/lacZ cilia develop axonemes with 9+0 or 8+2 microtubule structures. Disruption of an unstudied gene, 4931429I11Rik (now named Jhy) appears to underlie the hydrocephalus of JhylacZ/lacZ mice, and the Jhy transcript and protein are decreased in JhylacZ/lacZ mice. Partial phenotypic rescue was achieved in JhylacZ/lacZ mice by the introduction of a bacterial artificial chromosome (BAC) carrying 60-70% of the JHY protein coding sequence. Jhy is evolutionarily conserved from humans to basal vertebrates, but the predicted JHY protein lacks identifiable functional domains. Ongoing studies are directed at uncovering the physiological function of JHY and its role in CSF homeostasis. PMID:23906841

National Hydrocephalus Foundation

Science.gov (United States)

... Mission, History & Philosophy of NHF Treatment of Hydrocephalus What is a Shunt? Treatment Third Ventriculostomy Shunt Malfunction Prognosis and Research Medical Dictionary Resources Success Stories Blessing in Disguise Our Shining Star So Grateful Living with Hydrocephalus Fetal MRI Advancements ...

Gevolgen van hydrocephalus

NARCIS (Netherlands)

Tromp, Cornelis Nicolaas

1984-01-01

Hydrocephalus is een aandoening van het centrale zenuwstelsel die de clinicus vrij lang voor grote problemen ten aanzien van de behandeling heeft qesteld. Door de toepassing van de ventriculo-atriale shunttechniek zijn de behandelingsmogelijkheden van hydrocephalus sterk verbeterd. Deze studie

Antemortem diagnosis of hydrocephalus in two Congo African grey parrots (Psittacus erithacus erithacus) by means of computed tomography.

Science.gov (United States)

Thurber, Mary I; Mans, Christoph; Fazio, Constance; Waller, Ken; Rylander, Helena; Pinkerton, Marie E

2015-04-01

A 7-year-old and a 10-year-old Congo African grey parrot (Psittacus erithacus erithacus; parrots 1 and 2, respectively) were evaluated because of neurologic deficits. Parrot 1 had an 8- to 9-month history of lethargy and anorexia, with a recent history of a suspected seizure. Parrot 2 had a 6-month history of decreased activity and vocalizing, with an extended history of excessive water intake; a water deprivation test ruled out diabetes insipidus, and psychogenic polydipsia was suspected. Both birds had ophthalmologic asymmetry, with anisocoria detected in parrot 1 and unilateral blindness in parrot 2. Metal gastrointestinal foreign bodies were observed on whole-body radiographs of both birds, but blood lead concentrations were below the range indicated for lead toxicosis. Findings on CT of the head were consistent with hydrocephalus in both cases. Parrot 1 received supportive care and died 3 months after the diagnosis of hydrocephalus. Parrot 2 was treated with omeprazole and prednisolone for 10 days without any improvement in neurologic deficits; euthanasia was elected, and hydrocephalus was confirmed on necropsy. No underlying or concurrent disease was identified. Hydrocephalus should be considered a differential diagnosis for parrots evaluated because of CNS signs. Computed tomography was an excellent screening tool to diagnose hydrocephalus in these patients. Compared with MRI, CT is more frequently available and offers reduced scanning times, reduced cost, and less concern for interference from metallic foreign bodies.

Health-related quality of life in adults with congenital unilateral upper limb deficiency in Norway. A cross-sectional study.

Science.gov (United States)

Johansen, Heidi; Østlie, Kristin; Andersen, Liv Øinæs; Rand-Hendriksen, Svend

2016-11-01

To examine subjective health-related quality of life (HRQoL) in adults with congenital unilateral upper limb deficiency (UULD) in Norway and to explore the associations between demographic and clinical factors and HRQoL. Cross-sectional study comparing HRQoL, measured by SF-36, among adults with UULD and an age- and gender-matched control group from the Norwegian general population (NGP). Seventy-seven respondents, median age 42 years (range: 20-82); 71% were women. Most had left-sided (61%), below elbow (53%), transverse (73%) deficiency. Compared to the NGP, the UULD group reported reduced HRQoL on all SF-36 subscales except for the role emotional (RE) scale (p=0.321), mental health (MH) (p=0.055) and mental component summary (MCS) (p=0.064). The greatest difference was on the bodily pain (BP) scale (point difference of 20.0). Multiple linear regression models showed significant association between several physical- and mental SF-36 subscales and occupational status, occurrence of comorbidity and chronic pain. Persons with UULD reported reduced HRQoL on most SF-36 subscales, mostly in the physical health domain. Employment status, occurrence of comorbidity and chronic pain seem to have a negative impact on the HRQoL. Measures that can reduce pain and loss of function should be given particular attention in UULD rehabilitation. Implications for Rehabilitation Persons with congenital unilateral upper limb deficiency (UULD) who experience pain and discomfort should seek professional help for evaluating their everyday coping strategies. Professionals who meet persons with UULD should examine anomalies, comorbidity, pain and employment status before choosing advices and actions. Individually adapted grip-improving devices, environments, physical exercise and pain management programs should be implemented early to reduce pain, loss of function and decreased HRQoL. A multidisciplinary approach is often necessary when counseling persons with UULD.

Unilateral pulmonary veins atresia: evaluation by computed tomography; Atresia unilateral das veias pulmonares: avaliacao por tomografia computadorizada

Energy Technology Data Exchange (ETDEWEB)

Eifer, Diego Andre; Arsego, Felipe Veras, E-mail: felipesoarestorres@gmail.com [Hospital de Clinicas de Porto Alegre (HCPA), RS (Brazil). Serv. de Radiologia; Torres, Felipe Soares [Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS (Brazil). Escola de Medicina

2013-11-15

Unilateral pulmonary vein atresia is a rare congenital condition. In addition to cardiac malformations or pulmonary hypertension, patients may present with recurrent pulmonary infections or hemoptysis in childhood or adolescence. The authors report a case where the typical findings of such condition were observed at computed tomography in an adult patient. (author)

Congenital TORCH infections of the brain--CT manifestation (with analysis of 7 cases)

International Nuclear Information System (INIS)

Li Xin; Li Minglin; Yang Zhiyong

1997-01-01

To study the neuropathologic changes and CT manifestations in congenital TORCH infection of the brain. Analysis of 7 cases of congenital TORCH infection of the neonates and infants demonstrated by serum examination was performed. There were congenital toxoplasmosis 3 cases, congenital syncytial virus infection 1 case, congenital rubella virus infection 1 case, congenital cytomegalovirus infection 2 cases, and congenital herpes simplex virus infection 1 case. Cerebral hypoplasia, ventricular dilatation or hydrocephalus, subependymal and parenchymal calcifications, microcephalic focal cortical migration anomalies, schizencephaly polymicrogyria, et al, were demonstrated by CT with congenital TORCH infection. The earlier the infection, the more severe the brain developmental anomalies. The extent and appearance of calcification in brain were related to the degree, extent and course of TORCH infection. Basal ganglia calcification of unknown cause in infant was suggestive of congenital TORCH infection. Typical CT manifestations together with clinical picture may suggest congenital TORCH infection, while serological test can be diagnostic

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.)

Unilateral testicular tumour associated to congenital adrenal hyperplasia: Failure of specific tumoral molecular markers to discriminate between adrenal rest and leydigioma.

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Fenichel, P; Bstandig, B; Roger, C; Chevallier, D; Michels, J-F; Sadoul, J-L; Hieronimus, S; Brucker-Davis, F

2008-11-01

Testicular adrenal rest tumours are frequently associated with congenital adrenal hyperplasia (CAH). These ACTH-dependent tumours cannot be easily distinguished histologically from Leydig-cell tumours. We report the case of a 30-year-old man who was explored for infertility, azoospermia and unilateral testicular tumour. High levels of 17-OH progesterone and ACTH, low cortisol and undetectable gonadotropins levels, associated to bilateral adrenal hyperplasia, led to the diagnosis of CAH by 21-OH deficiency with a composite heterozygoty. The testicular tumour was first considered as adrenal rest. However, histological analysis of this unilateral painful tumour showed a steroid-hormone-secreting cell proliferation with atypical and frequent mitosis. To discriminate between a benign adrenal rest tumour and a possible malignant leydigioma, tumoral expression of specific gene products was analyzed by RT-PCR. No 11-beta-hydroxylase nor ACTH receptor mRNAs could be found in the tumour, which did not behave like usual adrenal rest cells. For this unilateral testicular tumour, the lack of adrenal-specific markers associated with a high rate of mitosis and pleiomorphism supported a leydigian origin with malignant potential. However, lack of tumoral LH-R mRNA expression and a tumour-free 3-year follow-up led us to retain the diagnosis of adrenal rest tumour with loss of adrenal gene expression and progressive autonomous behaviour.

A mutation in Ccdc39 causes neonatal hydrocephalus with abnormal motile cilia development in mice.

Science.gov (United States)

Abdelhamed, Zakia; Vuong, Shawn M; Hill, Lauren; Shula, Crystal; Timms, Andrew; Beier, David; Campbell, Kenneth; Mangano, Francesco T; Stottmann, Rolf W; Goto, June

2018-01-09

Pediatric hydrocephalus is characterized by an abnormal accumulation of cerebrospinal fluid (CSF) and is one of the most common congenital brain abnormalities. However, little is known about the molecular and cellular mechanisms regulating CSF flow in the developing brain. Through whole-genome sequencing analysis, we report that a homozygous splice site mutation in coiled-coil domain containing 39 ( Ccdc39 ) is responsible for early postnatal hydrocephalus in the progressive hydrocephal us ( prh ) mouse mutant. Ccdc39 is selectively expressed in embryonic choroid plexus and ependymal cells on the medial wall of the forebrain ventricle, and the protein is localized to the axoneme of motile cilia. The Ccdc39 prh/prh ependymal cells develop shorter cilia with disorganized microtubules lacking the axonemal inner arm dynein. Using high-speed video microscopy, we show that an orchestrated ependymal ciliary beating pattern controls unidirectional CSF flow on the ventricular surface, which generates bulk CSF flow in the developing brain. Collectively, our data provide the first evidence for involvement of Ccdc39 in hydrocephalus and suggest that the proper development of medial wall ependymal cilia is crucial for normal mouse brain development. © 2018. Published by The Company of Biologists Ltd.

Uptake of [14C]deoxyglucose into brain of young rats with inherited hydrocephalus

International Nuclear Information System (INIS)

Richards, H.K.; Bucknall, R.M.; Jones, H.C.; Pickard, J.D.

1989-01-01

The effect of hydrocephalus on cerebral glucose utilization as reflected by deoxyglucose uptake has been examined in rats with inherited hydrocephalus at 10, 20, and 28 days after birth using a semiquantitative method. Injection of [14C]deoxyglucose intraperitoneally was followed by freezing the brain, sectioning, and quantitative autoradiography of 10 brain regions. Brain [14C] concentration, cortical thickness, and plasma glucose concentrations were measured. Maximal thinning of the cerebral cortex had already occurred by 10 days after birth, although obvious symptoms such as gait disturbance developed after 20 days. In control rats, the cerebral isotope concentration was lower and more homogeneous at 10 days than at 20 or 28 days, which may be a reflection of the use of metabolic substrates other than glucose in younger animals. In order to make comparisons between control and hydrocephalic groups, tissue isotope concentrations were normalized to cerebellar cortex which was not affected by the hydrocephalus at any age. In hydrocephalic rats at 10 and 20 days, the concentration of [14C] was lower in all areas except the inferior colliculi and pons but the reduction was only significant in the sensory-motor cortex at 10 days and in the caudate nuclei at 20 days. By 28 days after birth, all areas except the cerebellum (six cortical regions, inferior colliculi, pons, and caudate) had significantly lower isotope concentrations in the hydrocephalic group. It is concluded that cerebral glucose metabolism is significantly reduced by 28 days after birth in H-Tx rats with congenital hydrocephalus and that less marked reductions occur prior to 28 days

Hydrocephalus in spina bifida

African Journals Online (AJOL)

deep concern. It is therefore .... Natural history of hydrocephalus in children with spinal open neural tube defect. Surg. Neurol Int ... fluid shunt infection: e Hydrocephalus Clinical Research Network Quality Improvement Initiative. J Neurosurg ...

Cognitive disorders in children's hydrocephalus.

Science.gov (United States)

Zielińska, Dorota; Rajtar-Zembaty, Anna; Starowicz-Filip, Anna

Hydrocephalus is defined as an increase of volume of cerebrospinal fluid in the ventricular system of the brain. It develops as a result of cerebrospinal fluid flow disorder due to dysfunctions of absorption or, less frequently, as a result of the increase of its production. Hydrocephalus may lead to various cognitive dysfunctions in children. In order to determine cognitive functioning in children with hydrocephalus, the authors reviewed available literature while investigating this subject. The profile of cognitive disorders in children with hydrocephalus may include a wide spectrum of dysfunctions and the process of neuropsychological assessment may be very demanding. The most frequently described cognitive disorders within children's hydrocephalus include attention, executive, memory, visual, spatial or linguistic dysfunctions, as well as behavioral problems. Copyright © 2017 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

Study on the progression of congenital scoliosis and the experimental production of the congenital scoliosis induced by /sup 60/Co irradiation in rats

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Noda, Y [Tokushima Univ. (Japan). School of Medicine

1977-08-01

In order to clarify the pathological mechanism of the development of congenital spinal deformities, seventy six cases with congenital spinal deformity were analysed clinically, and vertebral anomalies were produced experimentally in rats of the Wistar strain by giving /sup 60/Co irradiation (200 R) to the mother in the ninth, tenth and eleventh days of the pregnancy. The congenital vertebral anomalies were classified embryologically into the following eight groups, (1) Wedge vertebra, (2) Fused wedge vertebra, (3) Hemivertebra, (4) Fused hemivertebra, (5) Unilateral segmental failure, (6) Bilateral segmental failure, (7) Butterfly vertebra and (8) Mixed type. Out of these anomalies, the unilateral segmental failure and the imbalanced multiple hemivertebrae and wedge vertebrae proved to show a marked development of the scoliotic deformities. Vertebral anomalies were confirmed in 26 of the 101 newborn infants whose mothers were treated with /sup 60/Co irradiation in the 10th or 11th day of the pregnancy. The vertebral anomalies were either unilateral segmental failure or butterfly vertebrae located mostly at the lower thoracic, lumbar or sacral spine. A scoliotic deformity of the spine developed during the rapid growth of the body in all rats with unilateral segmental failure. From the histological examinations, it was revealed that the asymmetrical potency of the growth in the malformed vertebra was a factor causing the spinal deformities.

CT findings of central nervous system in congenital syphilis infant

International Nuclear Information System (INIS)

Yang Cheng; Yang Xinghui; Wang Man

2005-01-01

Objective: To investigate the CT features of the central nervous system in congenital syphilis infant. Methods: CT findings of central nervous system in 11 infants with clinically proved congenital syphilis were analyzed retrospectively. Results: CT findings in 10 syphilis neonates were diffuse hypodense lesions in the white matter, with subarachnoid and intra-encephalic hemorrhage in 3 and 1 cases, respectively. One 2-month-old syphilis infant case and 5 cases of follow-up after 45 days to 6 months of treatment demonstrated bilateral widened sulci and cistern with enlarged ventricles in 3 of them. Conclusion: CT findings of the central nervous system in congenital syphilis infant are similar to those of hypoxic-ischemic encephalopathy in neonates, and extra-encephalic hydrocephalus or brain hypogenesis ensues later on. (authors)

Periventricular hyperintensity in children with hydrocephalus

International Nuclear Information System (INIS)

Akbari, S.H.A.; Ragan, Dustin K.; Limbrick, David D.; McKinstry, Robert C.; Shimony, Joshua S.; Altaye, Mekibib; Yuan, Weihong; Holland, Scott K.; Mangano, Francesco T.

2015-01-01

Magnetic resonance images of children with hydrocephalus often include a rim of hyperintensity in the periventricular white matter (halo). The purpose of this study was to decide between the hypothesis that the halo is caused by cerebrospinal fluid (CSF) flow during the cardiac cycle, and the alternate hypothesis that the halo is caused by anatomical changes (stretching and compression of white matter). Participants were selected from a multicenter imaging study of pediatric hydrocephalus. We compared 19 children with hydrocephalus to a group of 52 controls. We quantified ventricle enlargement using the frontal-occipital horn ratio. We conducted qualitative and quantitative analysis of diffusion tensor imaging in the corpus callosum and posterior limb of the internal capsule. Parameters included the fractional anisotropy (FA), mean diffusivity, axial diffusivity and radial diffusivity. The halo was seen in 16 of the 19 children with hydrocephalus but not in the controls. The corpus callosum of the hydrocephalus group demonstrated FA values that were significantly decreased from those in the control group (P = 4 . 10 -6 ), and highly significant increases were seen in the mean diffusivity and radial diffusivity in the hydrocephalus group. In the posterior limb of the internal capsule the FA values of the hydrocephalus group were higher than those for the control group (P = 0.002), and higher values in the hydrocephalus group were also noted in the axial diffusivity. We noted correlations between the diffusion parameters and the frontal-occipital horn ratio. Our results strongly support the hypothesis that the halo finding in hydrocephalus is caused by structural changes rather than pulsatile CSF flow. (orig.)

Features of Somatic and Reproductive Status of Women, who Gave Birth to a Child with Congenital Malformations, and Their Identifiсation Using «Case — Control» Method According to Data of Lviv Regional Clinical Perinatal Center in 2007–2009

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Zborovska N.V. Zborovska N.V.

2012-04-01

While comparing a range of reproductive anamnesis indices there was noticed a higher percentage of dead children, stillbirths, anemia in pregnancy, gestosis in the first half of pregnancy, gestosis in the second half of pregnancy, abnormal labors in mothers who gave birth to a child with congenital malformations than in women of control group. According to ultrasound diagnosis congenital abnormalities in pregnancy were detected in 31.6 % of cases. There were diagnosed 4 cases of Arnold — Chiari syndrome (spina bifida and internal hydrocephalus; and 3 cases of each: hydrocephalus, gastroschisis, small intestinal atresia, and 2 cases of each: congenital malformations, hypoplastic left heart syndrome, Down’s syndrome, congenital diaphragmatic hernia, omphalocele, congenital rectal atresia, and 1 case of each: spina bifida, congenital absence of forearm and hand, left ventricular rabdomioma, encephalocele.

Cerebrospinal fluid hypersecretion in pediatric hydrocephalus.

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Karimy, Jason K; Duran, Daniel; Hu, Jamie K; Gavankar, Charuta; Gaillard, Jonathan R; Bayri, Yasar; Rice, Hunter; DiLuna, Michael L; Gerzanich, Volodymyr; Marc Simard, J; Kahle, Kristopher T

2016-11-01

Hydrocephalus, despite its heterogeneous causes, is ultimately a disease of disordered CSF homeostasis that results in pathological expansion of the cerebral ventricles. Our current understanding of the pathophysiology of hydrocephalus is inadequate but evolving. Over this past century, the majority of hydrocephalus cases has been explained by functional or anatomical obstructions to bulk CSF flow. More recently, hydrodynamic models of hydrocephalus have emphasized the role of abnormal intracranial pulsations in disease pathogenesis. Here, the authors review the molecular mechanisms of CSF secretion by the choroid plexus epithelium, the most efficient and actively secreting epithelium in the human body, and provide experimental and clinical evidence for the role of increased CSF production in hydrocephalus. Although the choroid plexus epithelium might have only an indirect influence on the pathogenesis of many types of pediatric hydrocephalus, the ability to modify CSF secretion with drugs newer than acetazolamide or furosemide would be an invaluable component of future therapies to alleviate permanent shunt dependence. Investigation into the human genetics of developmental hydrocephalus and choroid plexus hyperplasia, and the molecular physiology of the ion channels and transporters responsible for CSF secretion, might yield novel targets that could be exploited for pharmacotherapeutic intervention.

Compensated or progressive hydrocephalus?

NARCIS (Netherlands)

Leliefeld, P.H.

2012-01-01

OBJECTIVE: Raised intracranial pressure (ICP) that is associated with hydrocephalus may lead to alterations in cerebral hemodynamics and ischemic changes in the brain. In infants with hydrocephalus, defining the right moment for surgical intervention based on clinical signs alone, can sometimes be a

Ventricular differences between hydrostatic hydrocephalus and hydrocephalus Ex Vacuo by computed tomography

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LeMay, M; Hochberg, F H [Massachusetts General Hospital, Boston (USA). Radiology Dept.; Massachusetts General Hospital, Boston (USA). Dept. of Neurology; Harvard Medical School, Boston, MA (USA))

1979-01-01

Transaxial CT scans of 100 patients with hydrostatic hydrocephalus and 50 patients with hydrocephalus ex vacuo have been reviewed with respect to measurements of: frontal horn ratio, width of the temporal horns, width of the third ventricle, width of cerebral fissures and sulci. The diagnosis of hydrostatic hydrocephalus is probable when (a) both temporal tips are visualized and measure 3 mm or greater in width and the sylvian and interhemispheric fissures and cerebral sulci are not visible, or (b) there is visualization of temporal horn tips measuring 2 mm or greater in width and the lateral ventricles are symmetrically enlarged with the frontal horn ratio 0.50 or more.

Hydrocephalus secondary to subarachnoid hemorrhage

International Nuclear Information System (INIS)

Koga, Nobunori; Nakamura, Saburo; Kushi, Hidehiko; Yamamoto, Takamitsu; Tsubokawa, Takashi; Moriyasu, Nobuo

1982-01-01

The relationship between the extension and severity of subarachnoid hemorrhage, as demonstrated by computed tomography (CT), and hydrocephalus secondary to subarachnoid hemorrhage was studied. In 94 cases with subarachnoid hemorrhage, as analyzed by CT scan performed within 7 days after onset, high-density areas in the subarachnoid space were recognized in 61 cases (64%) and secondary hydrocephalus occurred in 22 cases (23%). 17 cases died within 2 weeks, before the occurrence of the hydrocephalus. The CT findings of subarachnoid hemorrhage was classified into 5 types, according to its severity and extension; especially the degree of high density in the basal cistern and/or cisterns around the brain stem was remarked. Secondary hydrocephalus after subarachnoid hemorrhage was observed in 90% of the cases; they had a density higher than a CT number of 60 in the basal cistern and/or cisterns around the brain stem (Type V). The mean interval between the onset of subarachnoid hemorrhage and the appearance of hydrocephalus was 20.6 days. We conclude that a significantly high density of extravasated blood in the subarachnoid space, especially in the basal cistern and/or the cisterns around the brain stem, can be predictive of secondary hydrocephalus after subarachnoid hemorrhage. (author)

Periventricular hyperintensity in children with hydrocephalus

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Akbari, S.H.A.; Ragan, Dustin K. [St. Louis Children' s Hospital, Washington University School of Medicine, Department of Neurological Surgery, St. Louis, MO (United States); Limbrick, David D. [St. Louis Children' s Hospital, Washington University School of Medicine, Department of Neurological Surgery, St. Louis, MO (United States); McKinstry, Robert C.; Shimony, Joshua S. [St. Louis Children' s Hospital, Washington University School of Medicine, Mallinckrodt Institute of Radiology, St. Louis, MO (United States); Altaye, Mekibib [Cincinnati Children' s Hospital, Division of Biostatistics and Epidemiology, Cincinnati, OH (United States); Yuan, Weihong; Holland, Scott K. [Cincinnati Children' s Hospital, Department of Pediatric Radiology, Cincinnati, OH (United States); Mangano, Francesco T. [Cincinnati Children' s Hospital, Department of Pediatric Neurological Surgery, Cincinnati, OH (United States)

2015-08-15

Magnetic resonance images of children with hydrocephalus often include a rim of hyperintensity in the periventricular white matter (halo). The purpose of this study was to decide between the hypothesis that the halo is caused by cerebrospinal fluid (CSF) flow during the cardiac cycle, and the alternate hypothesis that the halo is caused by anatomical changes (stretching and compression of white matter). Participants were selected from a multicenter imaging study of pediatric hydrocephalus. We compared 19 children with hydrocephalus to a group of 52 controls. We quantified ventricle enlargement using the frontal-occipital horn ratio. We conducted qualitative and quantitative analysis of diffusion tensor imaging in the corpus callosum and posterior limb of the internal capsule. Parameters included the fractional anisotropy (FA), mean diffusivity, axial diffusivity and radial diffusivity. The halo was seen in 16 of the 19 children with hydrocephalus but not in the controls. The corpus callosum of the hydrocephalus group demonstrated FA values that were significantly decreased from those in the control group (P = 4 . 10{sup -6}), and highly significant increases were seen in the mean diffusivity and radial diffusivity in the hydrocephalus group. In the posterior limb of the internal capsule the FA values of the hydrocephalus group were higher than those for the control group (P = 0.002), and higher values in the hydrocephalus group were also noted in the axial diffusivity. We noted correlations between the diffusion parameters and the frontal-occipital horn ratio. Our results strongly support the hypothesis that the halo finding in hydrocephalus is caused by structural changes rather than pulsatile CSF flow. (orig.)

Neurosonography of hydrocephalus in infants

International Nuclear Information System (INIS)

Shackelford, G.D.

1986-01-01

Transfontanel cranial ultrasonography reliably delineates ventricular size and anatomy in small infants. In these children, it is an excellent primary imaging technique for evaluation of the many clinical problems related to ventricular dilatation. Sonography can be useful for: detecting ventriculomegaly, differentiating nonobstructive ventricular dilatation from obstructive enlargement (hydrocephalus), determining the cause of hydrocephalus; aiding in the temporary management of patients with hydrocephalus; and aiding in the management of patients with permanent ventricular shunts. (orig.)

Trends in hospitalization of preterm infants with intraventricular hemorrhage and hydrocephalus in the United States, 2000-2010.

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Christian, Eisha A; Jin, Diana L; Attenello, Frank; Wen, Timothy; Cen, Steven; Mack, William J; Krieger, Mark D; McComb, J Gordon

2016-03-01

OBJECT Even with improved prenatal and neonatal care, intraventricular hemorrhage (IVH) occurs in approximately 25%-30% of preterm infants, with a subset of these patients developing hydrocephalus. This study was undertaken to describe current trends in hospitalization of preterm infants with posthemorrhagic hydrocephalus (PHH) using the Nationwide Inpatient Sample (NIS) and the Kids' Inpatient Database (KID). METHODS The KID and NIS were combined to generate data for the years 2000-2010. All neonatal discharges with ICD-9-CM codes for preterm birth with IVH alone or with IVH and hydrocephalus were included. RESULTS There were 147,823 preterm neonates with IVH, and 9% of this group developed hydrocephalus during the same admission. Of patients with Grade 3 and 4 IVH, 25% and 28%, respectively, developed hydrocephalus in comparison with 1% and 4% of patients with Grade 1 and 2 IVH, respectively. Thirty-eight percent of patients with PHH had permanent ventricular shunts inserted. Mortality rates were 4%, 10%, 18%, and 40%, respectively, for Grade 1, 2, 3, and 4 IVH during initial hospitalization. Length of stay has been trending upward for both groups of IVH (49 days in 2000, 56 days in 2010) and PHH (59 days in 2000, 70 days in 2010). The average hospital cost per patient (adjusted for inflation) has also increased, from $201,578 to $353,554 (for IVH) and $260,077 to $495,697 (for PHH) over 11 years. CONCLUSIONS The number of neonates admitted with IVH has increased despite a decrease in the number of preterm births. Rates of hydrocephalus and mortality correlated closely with IVH grade. The incidence of hydrocephalus in preterm infants with IVH remained stable between 8% and 10%. Over an 11-year period, there was a progressive increase in hospital cost and length of stay for preterm neonates with IVH and PHH that may be explained by a concurrent increase in the proportion of patients with congenital cardiac anomalies.

MR imaging features of hydrocephalus

International Nuclear Information System (INIS)

Zinn, W.; George, A.E.; Leon, M.J. de; Pinto, R.S.; Litt, A.W.; Kricheff, I.I.

1990-01-01

This paper compares the midsagittal dimensions of the third and lateral ventricles on MR images in cases of known hydrocephalus and atrophy. Cranial MR studies of 55 age-matched patients, 21 with known hydrocephalus and 28 with atrophy were retrospectively reviewed. Measurements of the genu-to-splenium diameter (G-S) and anterior commissure to corpus callosum distance (AC-CC) were obtained. A volumetric index (VI) was calculated as (G-S) x (AC-CC), and a ratio was calculated as (AC-CC)/(G-S). The volumetric index (VI) was 22% larger in the hydrocephalus group (Student two-tail t test, P > .001,t = -4.23). Sixty-two percent of hydrocephalus patients had either ratios greater than 56% or VIs of a least 20 cm 2

Congenital asymmetric crying face: a case report

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Semra Kara

2011-12-01

Full Text Available Congenital asymmetric crying face is an anomalia caused by unilateral absence or weakness of depressor anguli oris muscle The major finding of the disease is the absence or weakness in the outer and lower movement of the commissure during crying. The other expression muscles are normal and the face is symmetric at rest. The asymmetry in congenital asymmetric crying face is most evident during infancy but decreases by age. Congenital asymmetric crying face can be associated with cervicofacial, musclebone, respiratory, genitourinary and central nervous system anomalia. It is diagnosed by physical examination. This paper presents a six days old infant with Congenital asymmetric crying face and discusses the case in terms of diagnosis and disease features.

Hydrocephalus

International Nuclear Information System (INIS)

Gado, M.H.

1987-01-01

Both hydrocephalus and atrophy imply enlargement of cerebrospinal fluid (CSF) spaces; however, the management is totally different, and the two entities must be carefully differentiated from each other. The following topics are examined; (1) CSF dynamics. The main bulk of CSF production takes place in the lateral ventricles by the choroidal plexuses. Unidirectional flow of CSF brings it into basal cisterns and over cerebral hemispheres. The main bulk of CSF absorption occurs in the superior sagittal sinus via the arachnoid villi. (2) Classification by etiology increased production of CSF vs. obstruction to CSF flow. (3) The diagnosis of hydrocephaulus with CT or MR imaging. Signs of hydrocephalus include enlargement of the ventricles, sparing of sulci, ballooning of the temporal horns, periventricular edema, and progresssion with time. (4) The radiologic evaluation of results of shunt surgery

Shortfalls in pediatric hydrocephalus clinical outcome analysis.

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Walid, Mohammad Sami; Robinson, Joe Sam

2012-06-01

In this paper, we used search engine technology to study outcome analysis and cost awareness of child hydrocephalus in the literature. The aggregate hospital charges of hydrocephalus treatment procedures for patients years old was extracted from the Nationwide Inpatient Sample (NIS) data. Hydrocephalus literature was probed through the PubMed biomedical search engine. Aggregate hospital charges associated with ventriculo-peritoneal shunting as the principle procedure for patients years old have increased 1.7-fold over a 13-year period to 235.6 million in 2009. Hospital discharges, however, decreased from 3,390 in 1997 to 2,525 in 2009 (25.5% decrease over 13 years). The number of papers in English language indexed by PubMed in relation to child hydrocephalus in humans increased from 81 papers in 1996 to 133 in 2010 (1.6-fold increase), totaling 1,694 over 15 years. Randomized controlled trials published in relation to child hydrocephalus totaled 16 over the same period (0.94% of child hydrocephalus papers). Papers related to child hydrocephalus with "costs and cost analysis" as medical subject heading totaled 13 papers (0.77%). Over the past 15 years, disappointingly the number of printed child hydrocephalus papers appeared to have only plateaued. Strikingly, only a very small number of these papers were directed toward randomized control studies, the sine qua non of high-grade clinical evidence. Moreover, very few papers make reference to cost analysis or economics in the treatment of hydrocephalus - an issue coming increasingly before the nation at this point.

Quantitative RI cisternography in hydrocephalus

International Nuclear Information System (INIS)

Hara, Masao; Kayamori, Ryo; Sato, Kazuaki; Miyazawa, N.

1975-01-01

Ventriculography and cisterography with 131 I-HSA, and 167 Yb-DTPA were performed in patients with obstructive and normal-pressure hydrocephalus. Radioactivity in the head, blood counts and excretions in the urine were determined. In the patients with obstructive hydrocephalus, the radioactivity reduction in the head was late by the ventricular injection, while it was normal by the lumbar puncture. In the patients with normal-pressure hydrocephalus, the radioactivity reduction curve varied both by the ventricular injection and the lumbar puncture. However, these did not indicate the results of the shunt operation. The external determination of the rate of reduction of radioactivity in the head were parallel with blood radioactivity the urinary excretion rate. In the patients with normal-pressure hydrocephalus, the absorption rate of the spinal fluid decreased independently from the radioactivity curve in the head. (Saito, K.)

Hydrocephalus in cerebral venous thrombosis

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Zuurbier, Susanna M.; van den Berg, René; Troost, Dirk; Majoie, Charles B.; Stam, Jan; Coutinho, Jonathan M.

2015-01-01

Increased intracranial pressure is common in cerebral venous thrombosis (CVT), but hydrocephalus is rarely reported in these patients. We examined the frequency, pathophysiology and associated clinical manifestations of hydrocephalus in patients with CVT admitted to our hospital between 2000 and

Mathematics skills in good readers with hydrocephalus.

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Barnes, Marcia A; Pengelly, Sarah; Dennis, Maureen; Wilkinson, Margaret; Rogers, Tracey; Faulkner, Heather

2002-01-01

Children with hydrocephalus have poor math skills. We investigated the nature of their arithmetic computation errors by comparing written subtraction errors in good readers with hydrocephalus, typically developing good readers of the same age, and younger children matched for math level to the children with hydrocephalus. Children with hydrocephalus made more procedural errors (although not more fact retrieval or visual-spatial errors) than age-matched controls; they made the same number of procedural errors as younger, math-level matched children. We also investigated a broad range of math abilities, and found that children with hydrocephalus performed more poorly than age-matched controls on tests of geometry and applied math skills such as estimation and problem solving. Computation deficits in children with hydrocephalus reflect delayed development of procedural knowledge. Problems in specific math domains such as geometry and applied math, were associated with deficits in constituent cognitive skills such as visual spatial competence, memory, and general knowledge.

Importância da avaliação genético-clínica na hidrocefalia Importance of the clinical genetics evaluation on hydrocephalus

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Marta Wey-Vieira

2004-06-01

Full Text Available Os objetivos deste estudo foram caracterizar a presença de possíveis quadros de etiologia genética entre portadores de hidrocefalia congênita de etiologia não anteriormente esclarecida e confirmar aqueles com etiologia identificada previamente. A casuística compôs-se de 16 pacientes portadores de hidrocefalia congênita. O protocolo de investigação incluiu anamnese, investigação de história familial, exame clínico-dismorfológico, tomografia computadorizada ou ressonância magnética de sistema nervoso central, radiografia vertebral simples, cariótipo e estudo dismorfológico. Para análise dos resultados, a casuística foi dividida em dois grupos. O Grupo I (3M:6F caracterizado por indivíduos com hidrocefalia e sinais clínicos inespecíficos; o Grupo II (7M, em que os indivíduos apresentavam hidrocefalia congênita e sinais sugestivos do espectro da doença L1. Orientação genética específica foi possível em 11 casos. Os resultados demonstram a heterogeneidade etiológica envolvida na hidrocefalia, evidenciando a necessidade de avaliação clínico-dismorfológica como instrumento complementar na investigação dessa condição clínica.The aim of this study was to characterize the possibility of genetic etiology in a group of individuals with congenital hydrocephalus in which the etiology was indeterminate and to confirm that earlier diagnosed. The casuistry was composed by 16 individuals with congenital hydrocephalus. Investigation protocol included anamnesis, familial investigation, physical examination, computerized tomography or magnetic resonance image of head, vertebral column X-ray, karyotype and dysmorphological study. Results were analyzed in two groups. In Group I (3M:9F was composed by hydrocephalus associated with unspecific signs. Group II (7 males had findings of epectrum of L1 disease. Genetic counseling could be offered in 11 cases. These results demonstrate the great etiological heterogeneity of congenital

Phenotypic characteristics of hydrocephalus in stillborn Friesian foals.

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Sipma, K D; Cornillie, P; Saulez, M N; Stout, T A E; Voorhout, G; Back, W

2013-11-01

Hydrocephalus is uncommon in horses. However, in recent years, it has become clear that the prevalence of hydrocephalus is greater in Friesian horses than in other breeds probably due to their limited gene pool. Before identification of candidate genes that predispose to the development of hydrocephalus in Friesian horses can be pursued, an in-depth, phenotypic, pathological description of the condition in Friesians would be of great benefit. Our study aimed to characterize the morphology of hydrocephalus in Friesian horses, to support further investigation of the genetic background of this condition. Four stillborn Friesian foals with hydrocephalus were examined macroscopically and microscopically and compared with 2 normal stillborn Friesian foals without hydrocephalus. In all clinical cases, tetraventricular and venous dilatations were observed, together with malformation of the petrosal bone and, as a result, narrowing of the jugular foramen. These observations suggest a communicative hydrocephalus with a diminished absorption of cerebrospinal fluid into the systemic circulation at the venous sinuses due to a distorted, nonfunctional jugular foramen. This type of hydrocephalus is also recognized in humans and dogs and has been linked genetically to chondrodysplasia; this has already been recognized in dwarfism, which is another monogenetic defect in Friesian horses.

Monaural Congenital Deafness Affects Aural Dominance and Degrades Binaural Processing

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Tillein, Jochen; Hubka, Peter; Kral, Andrej

2016-01-01

Cortical development extensively depends on sensory experience. Effects of congenital monaural and binaural deafness on cortical aural dominance and representation of binaural cues were investigated in the present study. We used an animal model that precisely mimics the clinical scenario of unilateral cochlear implantation in an individual with single-sided congenital deafness. Multiunit responses in cortical field A1 to cochlear implant stimulation were studied in normal-hearing cats, bilaterally congenitally deaf cats (CDCs), and unilaterally deaf cats (uCDCs). Binaural deafness reduced cortical responsiveness and decreased response thresholds and dynamic range. In contrast to CDCs, in uCDCs, cortical responsiveness was not reduced, but hemispheric-specific reorganization of aural dominance and binaural interactions were observed. Deafness led to a substantial drop in binaural facilitation in CDCs and uCDCs, demonstrating the inevitable role of experience for a binaural benefit. Sensitivity to interaural time differences was more reduced in uCDCs than in CDCs, particularly at the hemisphere ipsilateral to the hearing ear. Compared with binaural deafness, unilateral hearing prevented nonspecific reduction in cortical responsiveness, but extensively reorganized aural dominance and binaural responses. The deaf ear remained coupled with the cortex in uCDCs, demonstrating a significant difference to deprivation amblyopia in the visual system. PMID:26803166

Monaural Congenital Deafness Affects Aural Dominance and Degrades Binaural Processing.

Science.gov (United States)

Tillein, Jochen; Hubka, Peter; Kral, Andrej

2016-04-01

Cortical development extensively depends on sensory experience. Effects of congenital monaural and binaural deafness on cortical aural dominance and representation of binaural cues were investigated in the present study. We used an animal model that precisely mimics the clinical scenario of unilateral cochlear implantation in an individual with single-sided congenital deafness. Multiunit responses in cortical field A1 to cochlear implant stimulation were studied in normal-hearing cats, bilaterally congenitally deaf cats (CDCs), and unilaterally deaf cats (uCDCs). Binaural deafness reduced cortical responsiveness and decreased response thresholds and dynamic range. In contrast to CDCs, in uCDCs, cortical responsiveness was not reduced, but hemispheric-specific reorganization of aural dominance and binaural interactions were observed. Deafness led to a substantial drop in binaural facilitation in CDCs and uCDCs, demonstrating the inevitable role of experience for a binaural benefit. Sensitivity to interaural time differences was more reduced in uCDCs than in CDCs, particularly at the hemisphere ipsilateral to the hearing ear. Compared with binaural deafness, unilateral hearing prevented nonspecific reduction in cortical responsiveness, but extensively reorganized aural dominance and binaural responses. The deaf ear remained coupled with the cortex in uCDCs, demonstrating a significant difference to deprivation amblyopia in the visual system. © The Author 2016. Published by Oxford University Press.

Three-year experience with the Sophono in children with congenital conductive unilateral hearing loss: tolerability, audiometry, and sound localization compared to a bone-anchored hearing aid.

Science.gov (United States)

Nelissen, Rik C; Agterberg, Martijn J H; Hol, Myrthe K S; Snik, Ad F M

2016-10-01

Bone conduction devices (BCDs) are advocated as an amplification option for patients with congenital conductive unilateral hearing loss (UHL), while other treatment options could also be considered. The current study compared a transcutaneous BCD (Sophono) with a percutaneous BCD (bone-anchored hearing aid, BAHA) in 12 children with congenital conductive UHL. Tolerability, audiometry, and sound localization abilities with both types of BCD were studied retrospectively. The mean follow-up was 3.6 years for the Sophono users (n = 6) and 4.7 years for the BAHA users (n = 6). In each group, two patients had stopped using their BCD. Tolerability was favorable for the Sophono. Aided thresholds with the Sophono were unsatisfactory, as they did not reach under a mean pure tone average of 30 dB HL. Sound localization generally improved with both the Sophono and the BAHA, although localization abilities did not reach the level of normal hearing children. These findings, together with previously reported outcomes, are important to take into account when counseling patients and their caretakers. The selection of a suitable amplification option should always be made deliberately and on individual basis for each patient in this diverse group of children with congenital conductive UHL.

Agenesis of internal carotid artery associated with congenital anterior hypopituitarism

International Nuclear Information System (INIS)

Moon, W.-J.; Porto, L.; Lanfermann, H.; Zanella, F.E.; Weis, R.

2002-01-01

We report a rare case of unilateral agenesis of the internal carotid artery in association with congenital anterior hypopituitarism. The collateral circulation is supplied by a transsellar intercavernous anastomotic vessel connecting the internal carotid arteries. These abnormalities are well depicted on MRI and MRA. The agenesis of the internal carotid artery may explain the pathogenesis of some of congenital anterior hypopituitarism. (orig.)

Congenital ankyloblepharon in a leopard gecko (Eublepharis macularius).

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Rival, Franck

2015-01-01

A 6-month-old leopard gecko with unilateral partially fused eyelids since birth was presented for examination. A diagnosis of congenital ankyloblepharon was made and surgical correction was performed successfully. © 2014 American College of Veterinary Ophthalmologists.

An update on research priorities in hydrocephalus: overview of the third National Institutes of Health-sponsored symposium "Opportunities for Hydrocephalus Research: Pathways to Better Outcomes".

Science.gov (United States)

McAllister, James P; Williams, Michael A; Walker, Marion L; Kestle, John R W; Relkin, Norman R; Anderson, Amy M; Gross, Paul H; Browd, Samuel R

2015-12-01

Building on previous National Institutes of Health-sponsored symposia on hydrocephalus research, "Opportunities for Hydrocephalus Research: Pathways to Better Outcomes" was held in Seattle, Washington, July 9-11, 2012. Plenary sessions were organized into four major themes, each with two subtopics: Causes of Hydrocephalus (Genetics and Pathophysiological Modifications); Diagnosis of Hydrocephalus (Biomarkers and Neuroimaging); Treatment of Hydrocephalus (Bioengineering Advances and Surgical Treatments); and Outcome in Hydrocephalus (Neuropsychological and Neurological). International experts gave plenary talks, and extensive group discussions were held for each of the major themes. The conference emphasized patient-centered care and translational research, with the main objective to arrive at a consensus on priorities in hydrocephalus that have the potential to impact patient care in the next 5 years. The current state of hydrocephalus research and treatment was presented, and the following priorities for research were recommended for each theme. 1) Causes of Hydrocephalus-CSF absorption, production, and related drug therapies; pathogenesis of human hydrocephalus; improved animal and in vitro models of hydrocephalus; developmental and macromolecular transport mechanisms; biomechanical changes in hydrocephalus; and age-dependent mechanisms in the development of hydrocephalus. 2) Diagnosis of Hydrocephalus-implementation of a standardized set of protocols and a shared repository of technical information; prospective studies of multimodal techniques including MRI and CSF biomarkers to test potential pharmacological treatments; and quantitative and cost-effective CSF assessment techniques. 3) Treatment of Hydrocephalus-improved bioengineering efforts to reduce proximal catheter and overall shunt failure; external or implantable diagnostics and support for the biological infrastructure research that informs these efforts; and evidence-based surgical standardization with

Treatment of Class II subdivision malocclusion with congenitally missing upper lateral incisors: A case report

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Siddharth Mehta

2014-01-01

Full Text Available Orthodontic treatment for patients with unilateral or bilateral congenitally missing lateral incisor poses a challenge mainly with regard to treatment planning. The use of a diagnostic setup is one of the most important aids in the decision-making process. Two alternatives, orthodontic space closure or space opening for prosthetic replacement exist. The present case report shows use of the microimplant for unilateral upper molar distalization and space closure in a Class-II division 1 subdivision malocclusion case with bilateral congenitally missing upper lateral incisors.

Hydrocephalus: a rare initial manifestation of sporadic intramedullary hemangioblastoma : Intramedullary hemangioblastoma presenting as hydrocephalus.

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Morais, Barbara Albuquerque; Cardeal, Daniel Dante; Ribeiro E Ribeiro, Renan; Frassetto, Fernando Pereira; Andrade, Fernanda Goncalves; Matushita, Hamilton; Teixeira, Manoel Jacobsen

2017-08-01

Intramedullary hemangioblastomas are rare benign vascular tumors, infrequent in pediatric patients. Clinical symptoms vary according to the age of presentation, tumor size, location, and concomitant syringomyelia. This is the second reported case of hemangioblastoma presenting with acute hydrocephalus. A 3-month-old infant with acute hydrocephalus was asymptomatic after a ventriculoperitoneal shunt was placed. She returned 3 months later with irritability, acute paraplegia, and respiratory distress. Magnetic resonance imaging (MRI) showed an intramedullary T8-T9 tumor with syringomyelia. She underwent surgical resection with good results during the 6-month follow-up. Intramedullary tumors may present as hydrocephalus and other nonspecific symptoms, with invariably delayed diagnosis in children, but must be considered in suspicious cases.

Infantile hydrocephalus: a review of epidemiology, classification and causes

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Tully, Hannah M; Dobyns, William B

2015-01-01

Hydrocephalus is a common but complex condition caused by physical or functional obstruction of CSF flow that leads to progressive ventricular dilatation. Though hydrocephalus was recently estimated to affect 1.1 in 1,000 infants, there have been few systematic assessments of the causes of hydrocephalus in this age group, which makes it a challenging condition to approach as a scientist or as a clinician. Here, we review contemporary literature on the epidemiology, classification and pathogenesis of infantile hydrocephalus. We describe the major environmental and genetic causes of hydrocephalus, with the goal of providing a framework to assess infants with hydrocephalus and guide future research. PMID:24932902

Classification of hydrocephalus: critical analysis of classification categories and advantages of "Multi-categorical Hydrocephalus Classification" (Mc HC).

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Oi, Shizuo

2011-10-01

Hydrocephalus is a complex pathophysiology with disturbed cerebrospinal fluid (CSF) circulation. There are numerous numbers of classification trials published focusing on various criteria, such as associated anomalies/underlying lesions, CSF circulation/intracranial pressure patterns, clinical features, and other categories. However, no definitive classification exists comprehensively to cover the variety of these aspects. The new classification of hydrocephalus, "Multi-categorical Hydrocephalus Classification" (Mc HC), was invented and developed to cover the entire aspects of hydrocephalus with all considerable classification items and categories. Ten categories include "Mc HC" category I: onset (age, phase), II: cause, III: underlying lesion, IV: symptomatology, V: pathophysiology 1-CSF circulation, VI: pathophysiology 2-ICP dynamics, VII: chronology, VII: post-shunt, VIII: post-endoscopic third ventriculostomy, and X: others. From a 100-year search of publication related to the classification of hydrocephalus, 14 representative publications were reviewed and divided into the 10 categories. The Baumkuchen classification graph made from the round o'clock classification demonstrated the historical tendency of deviation to the categories in pathophysiology, either CSF or ICP dynamics. In the preliminary clinical application, it was concluded that "Mc HC" is extremely effective in expressing the individual state with various categories in the past and present condition or among the compatible cases of hydrocephalus along with the possible chronological change in the future.

Unilateral hearing during development: hemispheric specificity in plastic reorganizations.

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Kral, Andrej; Heid, Silvia; Hubka, Peter; Tillein, Jochen

2013-01-01

The present study investigates the hemispheric contributions of neuronal reorganization following early single-sided hearing (unilateral deafness). The experiments were performed on ten cats from our colony of deaf white cats. Two were identified in early hearing screening as unilaterally congenitally deaf. The remaining eight were bilaterally congenitally deaf, unilaterally implanted at different ages with a cochlear implant. Implanted animals were chronically stimulated using a single-channel portable signal processor for two to five months. Microelectrode recordings were performed at the primary auditory cortex under stimulation at the hearing and deaf ear with bilateral cochlear implants. Local field potentials (LFPs) were compared at the cortex ipsilateral and contralateral to the hearing ear. The focus of the study was on the morphology and the onset latency of the LFPs. With respect to morphology of LFPs, pronounced hemisphere-specific effects were observed. Morphology of amplitude-normalized LFPs for stimulation of the deaf and the hearing ear was similar for responses recorded at the same hemisphere. However, when comparisons were performed between the hemispheres, the morphology was more dissimilar even though the same ear was stimulated. This demonstrates hemispheric specificity of some cortical adaptations irrespective of the ear stimulated. The results suggest a specific adaptation process at the hemisphere ipsilateral to the hearing ear, involving specific (down-regulated inhibitory) mechanisms not found in the contralateral hemisphere. Finally, onset latencies revealed that the sensitive period for the cortex ipsilateral to the hearing ear is shorter than that for the contralateral cortex. Unilateral hearing experience leads to a functionally-asymmetric brain with different neuronal reorganizations and different sensitive periods involved.

A systematic review of the risks factors associated with the onset and natural progression of hydrocephalus.

Science.gov (United States)

Walsh, Stephanie; Donnan, Jennifer; Morrissey, Andrea; Sikora, Lindsey; Bowen, Sonya; Collins, Kayla; MacDonald, Don

2017-07-01

The purpose of this study was to systematically assess and synthesize the world literature on risk factors for the onset and natural progression of hydrocephalus, thereby providing a basis for policy makers to identify appropriate risk management measures to mitigate the burden of disease in Canada. Evidence for risk factors was limited for both onset and progression. Two meta-analyses that examined a risk factor for onset met the inclusion criteria. One found a significant protective effect of prenatal vitamins among case control studies, but not cohort/randomized controlled trials (RCTs). The second found maternal obesity to be a significant risk factor for congenital hydrocephalus. Significant risk factors among 25 observational studies included: biological (multiple births, maternal parity, common cold with fever, maternal thyroid disease, family history, preterm birth, hypertension, ischemic heart disease, ischemic ECG changes, higher cerebrospinal fluid protein concentration following vestibular schwannoma); lifestyle (maternal obesity, high-density lipoprotein (HDL) cholesterol, maternal diabetes, maternal age), healthcare-related (caesarean section, interhospital transfer, drainage duration following subarachnoid hemorrhage, proximity to midline for craniectomy following traumatic brain injury); pharmaceutical (prenatal exposure to: tribenoside, metronidazole, anesthesia, opioids); and environmental (altitude, paternal occupation). Three studies reported on genetic risk factors: no significant associations were found. There are major gaps in the literature with respect to risk factors for the natural progression of hydrocephalus. Only two observational studies were included and three factors reported. Many risk factors for the onset of hydrocephalus have been studied; for most, evidence remains limited or inconclusive. More work is needed to confirm any causal associations and better inform policy. Copyright © 2016. Published by Elsevier B.V.

[Analyses of the clinical characteristics of unilateral conductive hearing loss with intact tympanic membrane].

Science.gov (United States)

Tang, Chaoying; Zhang, Jishuai; Han, Weiju; Shen, Weidong; Liu, Jun; Hou, Zhaohui; Dai, Pu; Yang, Shiming; Han, Dongyi

2016-05-01

To analyze the clinical characteristics of unilateral conductive hearing loss with intact tympanic membrane, and summarize the key diagnostic points, differential diagnosis and observe the effects of surgical treatment. We reviewed data from 82 patients with unilateral conductive hearing loss with intact tympanic membranes who accepted the exploratory tympanotomy from April 2011 to September 2013. There were 41 males and 41 females, aged from 7 to 66( averaged 26.5±13.7)years, with a history of one month to 50 years. The history, clinical symptoms, audiological evaluation, high resolution temporal bone CT, the results of surgical exploration and hearing reconstruction were analyzed. The exploratory tympanotomy revealed 43 cases of congenital middle ear malformations (52.4%), 22 cases of otosclerosis (26.8%), eight cases of congenital cholesteatoma (9.8%), six cases of trauma induced conductive hearing loss (7.3%), three cases of congenital ossicular malformations with congenital cholesteatoma (3.7%). Progressive hearing loss was common in patients with otosclerosis and congenital cholesteatoma, and patients with congenital middle ear malformations described their hearing loss since childhood. High resolution temporal bone CT of congenital middle ear malformation, trauma induced conductive hearing loss, congenital cholesteatoma diagnosis rate was 40.0%, 50.0%, and 83.3% respectively. The preoperative air-conductive threshold of patients with absence of the oval window were increased to (66.9±1.1)dBHL, the preoperative bone-conductive threshold achieved (28.3±10.4)dBHL at 2 000 Hz. While patients with stapes fixation and that with ossicular chain discontinuity were (27.2±9.7)dBHL and (17.8±8.8)dBHL(P=0.000)respectively. Through the tympanic exploration with endaural incision under the microscope, different hearing reconstruction were applied according to different lesions. After the operation, the hearing level of 52 patients with return visit were improved, the

Our experience in the management of infantile hydrocephalus: A study on thirty-five regrouped cases in Yaounde, Cameroon

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F F Mouafo Tambo

2011-01-01

Full Text Available Background: Hydrocephalus is a frequent central nervous system disorder in children, and despite its importance, it has not been sufficiently studied in developing countries. Patients and Methods: A prospective and descriptive study on 35 cases of infantile hydrocephalus was carried out in the neurosurgery unit of the Yaounde Central Hospital, from March 2008 to January 2010. Results: The mean age of the patients was 6.69΁1.58 months, and the majority of them were in the 0-6 months age group (71.43%. The most frequent causes were congenital malformations, with stenosis of the aqueduct of Sylvius being the most represented (31.43%. As radiological workup, a CT scan was done in more than half of the cases (57.15%, and ventriculo-peritoneal shunting was the main surgical method of management used (94.29%. Infectious complications were observed in 22.86% of our cases. Conclusion: Hydrocephalus is a frequent disorder in this Cameroonian setting affecting mostly the 0-6months age group. For early diagnosis to be made, the head circumference of neonates should be routinely measured in the labour room and followed-up in all medical visits. To avert complications following surgery, rigorous surgical procedures with effective asepsis and appropriate methods and materials for shunting should be used.

Subdural fluid collection and hydrocephalus following cervical schwannoma resection: hydrocephalus resolution after spinal pseudomeningocele repair: case report.

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Benedetto, Nicola; Cagnazzo, Federico; Gambacciani, Carlo; Perrini, Paolo

2016-12-01

The authors report the case of a 31-year-old man who developed neck pain and headache 2 months after the uncomplicated resection of a cervical schwannoma. MR imaging revealed infratentorial subdural fluid collections and obstructive hydrocephalus associated with cervical pseudomeningocele. The clinical symptoms, subdural fluid collections, and ventricular dilation resolved after surgical correction of the pseudomeningocele. This report emphasizes that hydrocephalus may be related to disorders of cerebrospinal fluid flow dynamics induced by cervical pseudomeningocele. In these rare cases, both the hydrocephalus and the symptoms are resolved by the simple correction of the pseudomeningocele.

Long-term Compliance and Satisfaction With Percutaneous Bone Conduction Devices in Patients With Congenital Unilateral Conductive Hearing Loss.

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Nelissen, Rik C; Mylanus, Emmanuel A M; Cremers, Cor W R J; Hol, Myrthe K S; Snik, Ad F M

2015-06-01

Patients with congenital unilateral conductive hearing loss (UCHL) can either be watchful monitored or treated surgically through the fitting of a percutaneous bone conduction device (BCD) or, in some cases, atresia repair. The current study evaluated the long-term compliance and satisfaction with a percutaneous BCD in this specific population. Fifty-three consecutive patients with congenital UCHL treated with a percutaneous BCD in our tertiary referral center between 1998 and 2011 were identified. Clinical and audiological data were retrospectively gathered from the patients' files. The patients were interviewed by telephone about their current device usage status and were asked to complete the Speech, Spatial and Qualities of Hearing Scale (SSQ). Compliance with the BCD was 56.6% after a mean follow-up of 7 years. The mean age at implantation of the users (22 years) was significantly higher than that of the nonusers (10 years). The mean time of device usage before the patients stopped using the BCD was 5 years. The primary reasons mentioned for quitting the BCD were experiencing excess background noise and/or subjectively not receiving enough benefit. Objectively measured features of binaural processing affected by the BCD were found to correlate with long-term BCD usage. The SSQ revealed significant improvement in the aided condition compared with the nonaided condition in the users, in contrast to the nonusers. The current disappointing long-term compliance figures indicate the need for an even more careful and individualized approach with life-long follow-up when fitting BCDs in this specific population, especially in children.

Surgical treatment reduces blood pressure in children with unilateral congenital hydronephrosis.

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Al-Mashhadi, Ammar; Nevéus, Tryggve; Stenberg, Arne; Karanikas, Birgitta; Persson, A Erik G; Carlström, Mattias; Wåhlin, Nils

2015-04-01

Renal disorders can cause hypertension, but less is known about the influence of hydronephrosis on blood pressure. Hydronephrosis due to pelvo-ureteric junction obstruction (PUJO) is a fairly common condition (incidence in newborns of 0.5-1%). Although hypertensive effects of hydronephrosis have been suggested, this has not been substantiated by prospective studies in humans [1-3]. Experimental studies with PUJO have shown that animals with induced hydronephrosis develop salt-sensitive hypertension, which strongly correlate to the degree of obstruction [4-7]. Moreover, relief of the obstruction normalized blood pressure [8]. In this first prospective study our aim was to study the blood pressure pattern in pediatric patients with hydronephrosis before and after surgical correction of the ureteral obstruction. Specifically, we investigated if preoperative blood pressure is reduced after surgery and if split renal function and renographic excretion curves provide any prognostic information. Twelve patients with unilateral congenital hydronephrosis were included in this prospective study. Ambulatory blood pressure (24 h) was measured preoperatively and six months after surgery. Preoperative evaluations of bilateral renal function by Tc99m-MAG3 scintigraphy, and renography curves, classified according to O'Reilly, were also performed. As shown in the summary figure, postoperative systolic (103 ± 2 mmHg) and diastolic (62 ± 2 mmHg) blood pressure were significantly lower than those obtained preoperatively (110 ± 4 and 69 ± 2 mmHg, respectively), whereas no changes in circadian variation or pulse pressure were observed. Renal functional share of the hydronephrotic kidney ranged from 11 to 55%. There was no correlation between the degree of renal function impairment and the preoperative excretory pattern, or between the preoperative excretory pattern and the blood pressure reduction postoperatively. However, preoperative MAG3 function of the affected kidney correlated

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.

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

Long term ocular and neurological involvement in severe congenital toxoplasmosis.

Science.gov (United States)

Meenken, C; Assies, J; van Nieuwenhuizen, O; Holwerda-van der Maat, W G; van Schooneveld, M J; Delleman, W J; Kinds, G; Rothova, A

1995-06-01

This study was set up to determine the long term ocular and systemic sequelae in patients with severe congenital toxoplasmosis. Cross sectional and retrospective study of 17 patients with severe congenital toxoplasmosis. In addition to chorioretinitis (100%), the most common abnormal ocular features were optic nerve atrophy (83%), visual acuity of less than 0.1 (85%), strabismus, and microphthalmos. In 50% of cases we observed iridic abnormalities and about 40% developed a cataract. Overt endocrinological disease, diagnosed in five of 15 patients, included panhypopituitarism (n = 2), gonadal failure with dwarfism (n = 1), precocious puberty with dwarfism and thyroid deficiency (n = 1), and diabetes mellitus and thyroid deficiency (n = 1). The observed endocrinological involvement was associated in all cases with obstructive hydrocephalus with a dilated third ventricle and optic nerve atrophy. The recognition of long term ocular, neurological, and endocrinological sequelae of congenital toxoplasmosis is important for medical management of these severely handicapped patients.

Prevalence of hydrocephalus in 157 patients with vestibular schwannoma

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Rogg, Jeffrey M.; Ahn, S.H.; Tung, G.A. [Rhode Island Hospital, Department of Diagnostic Imaging, Providence, Rhode Island (United States); Reinert, S.E. [Rhode Island Hospital, Lifespan Medical Computing, Providence, Rhode Island (United States); Noren, G. [Rhode Island Hospital, Department of Neurosurgery, Providence, Rhode Island (United States)

2005-05-01

The purpose of this study was to determine the prevalence of hydrocephalus in patients with vestibular schwannoma. A second objective was to investigate possible etiologies for hydrocephalus in this population by attempting to correlate the incidence and severity of hydrocephalus with tumor volume and extent of fourth ventricular compression. The MRI examinations of 157 adult patients with vestibular schwannoma were retrospectively reviewed. Tumor size was quantified, and the presence of accompanying hydrocephalus was assessed, categorized as communicating type or non-communicating type and then rated as mild, moderate or severe (grades 1-3). Next, the degree of fourth ventricular distortion caused by tumor mass effect was evaluated and categorized as mild, moderate or severe (grades 1-3). Spearman's rank correlation coefficient was used to test the relationships between tumor volume and (1) the extent of fourth ventricular effacement and (2) severity of hydrocephalus. Hydrocephalus was present in 28/157 (18%) cases and was categorized as mild in 11/28 (39%), moderate in 15/28 (54%) and severe in 2/28 (7%). Communicating-type hydrocephalus was present in 17/28 (61%) and non-communicating type in 11/28 (39%). There was a positive correlation between the grade of non-communicating hydrocephalus and tumor volume (r=0.38; P<0.001) and between the severity of fourth ventricular compression and extent of hydrocephalus in this group(r=0.43; P<0.001). (orig.)

The Risk Factors of Symptomatic Communicating Hydrocephalus After Stereotactic Radiosurgery for Unilateral Vestibular Schwannoma: The Implication of Brain Atrophy

International Nuclear Information System (INIS)

Han, Jung Ho; Kim, Dong Gyu; Chung, Hyun-Tai; Paek, Sun Ha; Park, Chul-Kee; Kim, Chae-Yong; Hwang, Seung-Sik; Park, Jeong-Hoon; Kim, Young-Hoon; Kim, Jin Wook; Kim, Yong Hwy; Song, Sang Woo; Kim, In Kyung; Jung, Hee-Won

2012-01-01

Purpose: To identify the effect of brain atrophy on the development of symptomatic communicating hydrocephalus (SCHCP) after stereotactic radiosurgery (SRS) for sporadic unilateral vestibular schwannomas (VS). Methods and Materials: A total of 444 patients with VS were treated with SRS as a primary treatment. One hundred eighty-one patients (40.8%) were male, and the mean age of the patients was 53 ± 13 years (range, 11–81 years). The mean follow-up duration was 56.8 ± 35.8 months (range, 12–160 months). The mean tumor volume was 2.78 ± 3.33 cm 3 (range, 0.03–23.30 cm 3 ). The cross-sectional area of the lateral ventricles (CALV), defined as the combined area of the lateral ventricles at the level of the mammillary body, was measured on coronal T1-weighted magnetic resonance images as an indicator of brain atrophy. Results: At distant follow-up, a total of 25 (5.6%) patients had SCHCP. The median time to symptom development was 7 months (range, 1–48 months). The mean CALV was 334.0 ± 194.0 mm 2 (range, 44.70–1170 mm 2 ). The intraclass correlation coefficient was 0.988 (95% confidence interval [CI], 0.976–0.994; p < 0.001). In multivariate analysis, the CALV had a significant relationship with the development of SCHCP (p < 0.001; odds ration [OR] = 1.005; 95% CI, 1.002–1.007). Tumor volume and female sex also had a significant association (p < 0.001; OR = 1.246; 95% CI, 1.103–1.409; p < 0.009; OR = 7.256; 95% CI, 1.656–31.797, respectively). However, age failed to show any relationship with the development of SCHCP (p = 0.364). Conclusion: Brain atrophy may be related to de novo SCHCP after SRS, especially in female patients with a large VS. Follow-up surveillance should be individualized, considering the risk factors involved for each patient, for prompt diagnosis of SCHCP.

The Risk Factors of Symptomatic Communicating Hydrocephalus After Stereotactic Radiosurgery for Unilateral Vestibular Schwannoma: The Implication of Brain Atrophy

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Han, Jung Ho [Department of Neurosurgery, Seoul National University Bundang Hospital, Gyeonggi-do (Korea, Republic of); Department of Neurosurgery, Seoul National University College of Medicine, Seoul (Korea, Republic of); Kim, Dong Gyu, E-mail: gknife@plaza.snu.ac.kr [Department of Neurosurgery, Seoul National University Hospital, Seoul (Korea, Republic of); Department of Neurosurgery, Seoul National University College of Medicine, Seoul (Korea, Republic of); Chung, Hyun-Tai; Paek, Sun Ha; Park, Chul-Kee [Department of Neurosurgery, Seoul National University Hospital, Seoul (Korea, Republic of); Department of Neurosurgery, Seoul National University College of Medicine, Seoul (Korea, Republic of); Kim, Chae-Yong [Department of Neurosurgery, Seoul National University Bundang Hospital, Gyeonggi-do (Korea, Republic of); Department of Neurosurgery, Seoul National University College of Medicine, Seoul (Korea, Republic of); Hwang, Seung-Sik [Department of Social and Preventive Medicine, Inha University School of Medicine, Incheon (Korea, Republic of); Park, Jeong-Hoon [Department of Neurosurgery, Seoul National University Bundang Hospital, Gyeonggi-do (Korea, Republic of); Kim, Young-Hoon [Department of Neurosurgery, Seoul National University Bundang Hospital, Gyeonggi-do (Korea, Republic of); Department of Neurosurgery, Seoul National University College of Medicine, Seoul (Korea, Republic of); Kim, Jin Wook; Kim, Yong Hwy; Song, Sang Woo; Kim, In Kyung; Jung, Hee-Won [Department of Neurosurgery, Seoul National University Hospital, Seoul (Korea, Republic of); Department of Neurosurgery, Seoul National University College of Medicine, Seoul (Korea, Republic of)

2012-11-15

Purpose: To identify the effect of brain atrophy on the development of symptomatic communicating hydrocephalus (SCHCP) after stereotactic radiosurgery (SRS) for sporadic unilateral vestibular schwannomas (VS). Methods and Materials: A total of 444 patients with VS were treated with SRS as a primary treatment. One hundred eighty-one patients (40.8%) were male, and the mean age of the patients was 53 {+-} 13 years (range, 11-81 years). The mean follow-up duration was 56.8 {+-} 35.8 months (range, 12-160 months). The mean tumor volume was 2.78 {+-} 3.33 cm{sup 3} (range, 0.03-23.30 cm{sup 3}). The cross-sectional area of the lateral ventricles (CALV), defined as the combined area of the lateral ventricles at the level of the mammillary body, was measured on coronal T1-weighted magnetic resonance images as an indicator of brain atrophy. Results: At distant follow-up, a total of 25 (5.6%) patients had SCHCP. The median time to symptom development was 7 months (range, 1-48 months). The mean CALV was 334.0 {+-} 194.0 mm{sup 2} (range, 44.70-1170 mm{sup 2}). The intraclass correlation coefficient was 0.988 (95% confidence interval [CI], 0.976-0.994; p < 0.001). In multivariate analysis, the CALV had a significant relationship with the development of SCHCP (p < 0.001; odds ration [OR] = 1.005; 95% CI, 1.002-1.007). Tumor volume and female sex also had a significant association (p < 0.001; OR = 1.246; 95% CI, 1.103-1.409; p < 0.009; OR = 7.256; 95% CI, 1.656-31.797, respectively). However, age failed to show any relationship with the development of SCHCP (p = 0.364). Conclusion: Brain atrophy may be related to de novo SCHCP after SRS, especially in female patients with a large VS. Follow-up surveillance should be individualized, considering the risk factors involved for each patient, for prompt diagnosis of SCHCP.

Unilateral hearing during development: hemispheric specificity in plastic reorganizations

Directory of Open Access Journals (Sweden)

Andrej eKral

2013-11-01

Full Text Available The present study investigates the hemispheric contributions of neuronal reorganization following early single-sided hearing (unilateral deafness. The experiments were performed on ten cats from our colony of deaf white cats. Two were identified in early hearing screening as unilaterally congenitally deaf. The remaining eight were bilaterally congenitally deaf, unilaterally implanted at different ages with a cochlear implant. Implanted animals were chronically stimulated using a single-channel portable signal processor for two to five months. Microelectrode recordings were performed at the primary auditory cortex under stimulation at the hearing and deaf ear with bilateral cochlear implants. Local field potentials (LFPs were compared at the cortex ipsilateral and contralateral to the hearing ear. The focus of the study was on the morphology and the onset latency of the LFPs. The data revealed that effects of hearing experience were more pronounced when stimulating the hearing ear. With respect to morphology of LFPs, pronounced hemisphere-specific effects were observed. Morphology of amplitude-normalized LFPs for stimulation of the deaf and the hearing ear was similar for responses recorded at the same hemisphere. However, when comparisons were performed between the hemispheres, the morphology was more dissimilar even though the same ear was stimulated. This demonstrates hemispheric specificity of some cortical adaptations irrespective of the ear stimulated. The results suggest a specific adaptation process at the hemisphere ipsilateral to the hearing ear, involving specific (down-regulated inhibitory mechanisms not found in the contralateral hemisphere. Finally, onset latencies revealed that the sensitive period for the cortex ipsilateral to the hearing ear is shorter than that for the contralateral cortex. Unilateral hearing experience leads to a functionally-asymmetric brain with different neuronal reorganizations and different sensitive

Congenital tumors of the central nervous system

International Nuclear Information System (INIS)

Severino, Mariasavina; Schwartz, Erin S.; Thurnher, Majda M.; Rydland, Jana; Nikas, Ioannis; Rossi, Andrea

2010-01-01

Congenital tumors of the central nervous system (CNS) are often arbitrarily divided into ''definitely congenital'' (present or producing symptoms at birth), ''probably congenital'' (present or producing symptoms within the first week of life), and ''possibly congenital'' (present or producing symptoms within the first 6 months of life). They represent less than 2% of all childhood brain tumors. The clinical features of newborns include an enlarged head circumference, associated hydrocephalus, and asymmetric skull growth. At birth, a large head or a tense fontanel is the presenting sign in up to 85% of patients. Neurological symptoms as initial symptoms are comparatively rare. The prenatal diagnosis of congenital CNS tumors, while based on ultrasonography, has significantly benefited from the introduction of prenatal magnetic resonance imaging studies. Teratomas constitute about one third to one half of these tumors and are the most common neonatal brain tumor. They are often immature because of primitive neural elements and, rarely, a component of mixed malignant germ cell tumors. Other tumors include astrocytomas, choroid plexus papilloma, primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, and medulloblastomas. Less common histologies include craniopharyngiomas and ependymomas. There is a strong predilection for supratentorial locations, different from tumors of infants and children. Differential diagnoses include spontaneous intracranial hemorrhage that can occur in the presence of coagulation factor deficiency or underlying vascular malformations, and congenital brain malformations, especially giant heterotopia. The prognosis for patients with congenital tumors is generally poor, usually because of the massive size of the tumor. However, tumors can be resected successfully if they are small and favorably located. The most favorable outcomes are achieved with choroid plexus tumors, where aggressive surgical treatment leads to disease

Diagnosis and differential diagnosis of hydrocephalus in adults

International Nuclear Information System (INIS)

Langner, Soenke; Mensel, Birger; Kuehn, Jens Peter; Kirsch, Michael; Fleck, Steffen; Baldauf, Joerg

2017-01-01

Hydrocephalus is caused by an imbalance of production and absorption of cerebrospinal fluid (CSF) or obstruction of its pathways, resulting in ventricular dilatation and increased intracranial pressure. Imaging plays a crucial role in the diagnosis, differential diagnosis and planning of treatment. This review article presents the different types of hydrocephalus und their typical imaging appearance, describes imaging techniques, and discusses differential diagnoses of the different forms of hydrocephalus. Imaging plays a central role in the diagnosis of hydrocephalus. While magnetic resonance (MR) imaging is the first-line imaging modality, computed tomography (CT) is often the first-line imaging test in emergency patients.

Congenital Pseudoarthrosis of the clavicle - Case report

International Nuclear Information System (INIS)

Holguin Maldonado, Esteban; Rossellli Cock, Pablo

2006-01-01

Congenital pseudoarthrosis of the clavicle (CPC) is a tare malformation of the scapular waist of unknown etiology. The lesion is usually unilateral and right sided. It is seldom symptomatic and X rays confirm the diagnosis. Treatment is usually non surgical, unless functional or cosmetic compromise of the shoulder is present

Unrecognised ventriculitis/meningitis presenting as hydrocephalus in infancy.

Science.gov (United States)

Udani, Vrajesh; Udani, Soonu; Merani, Rohan; Bavdekar, Manisha

2003-09-01

Infantile hydrocephalus due to unrecognized neonatal-onset meningitis/ventriculitis, was studied retrospectively using 1991-1998 chart review. Seventy two patients with hydrocephalus were reviewed. Thirteen infants had hydrocephalus associated with active meningitis/ventriculitis which had remained unrecognized. Active meningitis/ventriculitis was confirmed by the finding of an abnormal lumbar and ventricular CSF with or without positive culture. All had perinatal risk factors and 10/13 had been given antibiotics in the postnatal period. 6/13 infants appeared to be well. The most common presentation was increasing head size. All lumbar and ventricular CSFs were abnormal and 10/13 had positive cultures as well. Imaging revealed hydrocephalus in all. The infants were treated with antibiotics for a mean of 32.8 days before VP shunting. 7/11 were severely disabled. Unrecognized active meningitis/ventriculitis is an important cause of infantile hydrocephalus.

[Congenital toxoplasmosis: clinical manifestation, treatment and follow-up] [Article in Italian] • Il neonato con toxoplasmosi congenita: clinica, terapia e follow-up

Directory of Open Access Journals (Sweden)

Lina Bollani

2014-01-01

Full Text Available Toxoplamosis is a parasitic zoonosis which occurs worldwide, but is prevalent in Europe, South America and Africa. When infection occurs for the first time during pregnancy, mother to child transmission of the parasite can cause congenital toxoplasmosis. Rate of congenital infection ranges from less than 0.1 to approximately 1 per 1,000 live births. The risk of transmission depends on the gestational age at the time of maternal infection. A diagnosis of congenital toxoplasmosis is usually considered in infants who present: hydrocephalus, chorioretinitis, and intracranial calcifications, but this triade is very rare. Approximately 85% of the infants with congenital toxoplasmosis are clinically normal at birth; however, sequelae of infection may become apparent only months or even years later. Chorioretinitis is the main complication of congenital toxoplasmosis, late onset retinal lesions and relapse can appear many years after birth, but the overall ocular prognosis is satisfactory when infection is identified and treated accordingly. Fortunately, serious neonatal forms and severe neurological impairment have become rare, but prompt treatment of children with convulsions, abnormal muscle tone, hydrocephalus, may improve the prognosis and result in almost normal outcome. For infants who have congenital toxoplasmosis, treatment soon after birth for 1 year with pyrimetamine, sulfadiazine and leukoverin led to remarkable resolution of serious, active disease. A long follow-up is necessary to assess the long-term outcome of children and young adults with congenital toxoplasmosis, that is favourable for the majority of cases. Epidemiological surveillance needs to be improved in order to determine the effectiveness of prevention programs.Articoli Selezionati del “3° Convegno Pediatrico del Medio Campidano” · Guspini · 25 Maggio 2013Guest Editor: Roberto Antonucci

Diagnosis and Differential Diagnosis of Hydrocephalus in Adults.

Science.gov (United States)

Langner, Sönke; Fleck, Steffen; Baldauf, Jörg; Mensel, Birger; Kühn, Jens Peter; Kirsch, Michael

2017-08-01

Purpose  Hydrocephalus is caused by an imbalance of production and absorption of cerebrospinal fluid (CSF) or obstruction of its pathways, resulting in ventricular dilatation and increased intracranial pressure. Imaging plays a crucial role in the diagnosis, differential diagnosis and planning of treatment. Methods  This review article presents the different types of hydrocephalus und their typical imaging appearance, describes imaging techniques, and discusses differential diagnoses of the different forms of hydrocephalus. Results and Conclusion  Imaging plays a central role in the diagnosis of hydrocephalus. While magnetic resonance (MR) imaging is the first-line imaging modality, computed tomography (CT) is often the first-line imaging test in emergency patients. Key points   · Occlusive hydrocephalus is caused by obstruction of CSF pathways.. · Malabsorptive hydrocephalus is caused by impaired CSF absorption.. · The MR imaging protocol should always include sagittal high-resolution T2-weighted images.. · When an inflammatory etiology is suspected, imaging with contrast agent administration is necessary.. Citation Format · Langner S, Fleck S, Baldauf J et al. Diagnosis and Differential Diagnosis of Hydrocephalus in Adults. Fortschr Röntgenstr 2017; 189: 728 - 739. © Georg Thieme Verlag KG Stuttgart · New York.

236 children with developmental hydrocephalus: causes and clinical consequences

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Tully, Hannah M; Ishak, Gisele E; Rue, Tessa C; Dempsey, Jennifer C; Browd, Samuel R; Millen, Kathleen J; Doherty, Dan; Dobyns, William B

2016-01-01

Few systematic assessments of developmental forms of hydrocephalus exist. We reviewed MRIs and clinical records of patients with infancy-onset hydrocephalus. Among 411 infants, 236 had hydrocephalus with no recognizable extrinsic cause. These children were assigned to one of five subtypes and compared on the basis of clinical characteristics, developmental and surgical outcomes. At an average age of 5.3 years, 72% of children were walking independently and 87% could eat by mouth. 18% had epilepsy. Distinct patterns of associated malformations and syndromes were observed within each subtype. On average, children with aqueductal obstruction, cysts and encephaloceles had worse clinical outcomes than those with other forms of developmental hydrocephalus. 53% of surgically-treated patients experienced at least one shunt failure, but hydrocephalus associated with posterior fossa crowding required fewer shunt revisions. We conclude that each subtype of developmental hydrocephalus is associated with distinct clinical characteristics, syndromology, and outcomes, suggesting differences in underlying mechanisms. PMID:26184484

The value of early and comprehensive diagnoses in a human fetus with hydrocephalus and progressive obliteration of the aqueduct of Sylvius: Case Report.

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Ortega, Eduardo; Muñoz, Rosa I; Luza, Nelly; Guerra, Francisco; Guerra, Monserrat; Vio, Karin; Henzi, Roberto; Jaque, Jaime; Rodriguez, Sara; McAllister, James P; Rodriguez, Esteban

2016-04-11

progressive stenosis of the SA initiated during the embryonic period may have resulted from ependymal disruption of the cerebral aqueduct and dysfunction of the subcommissural organ. The analysis of subcommissural organ glycoproteins present in the CSF may be a valuable diagnostic tool for the pathogenesis of congenital hydrocephalus.

Magnetic resonance (MR) imaging in hydrocephalus

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Tamaki, Norihiko; Nagashima, Tatsuya; Shirakuni, Takayuki; Masumura, Michio; Matsumoto, Satoshi

1985-01-01

The correlation between the findings on MR images and CT findings, clinical symptoms, and the results of various specific investigations was determined. MR imaging was more sensitive in demonstrating the pathology of the periventricular tissue in hydrocephalus than was CT scanning, although the overall correlation between MR and CT findings was good. The authors classified the periventricular high-signal intensities (PVHI) of MR images into 4 types according to the extension and grade of the PVHI. Type 0 has no PVHI. Type I has a band of the PVHI around the wall of the lateral ventricles, including the corpus callosum and the septum pellucidum. In Type III, PVHI extended half of the width of the periventricular white matter, while in Type IV PVHI occupied the entire white matter. CT scans showed periventricular lucency (PVL) only in the periventricular white matter, especially surrounding the anterior horn, but the PVHI was visible not only in wider areas of the entire periventricular white matter, but also in the corpus callosum and the septum pellucidum. The PVHI seen in case of hydrocephalus due to posterior fossa tumors was characterized by PVHI of Type I; the PVHI disappeared after CSF shunting in all cases. There was no PVHI in the cases of marked hydrocephalus due to aqueductal stenosis, which appeared to be compensated for a long period. In the cases of normal-pressure hydrocephalus, there were various types of PVHI. There was no correlation between the types of PVHI and the grade of ventriculomegaly. There was, however, a close correlation between the types of PVHI on MR images and the clinical symptoms, and the cerebral blood flow in cases of normal-pressure hydrocephalus. In some patients with normal-pressure hydrocephalus, the PVHI improved and the cerebral blood flow increased after cerebrospinal fluid shunting. (J.P.N.)

Factors Associated with Acute and Chronic Hydrocephalus in Nonaneurysmal Subarachnoid Hemorrhage.

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Kang, Peter; Raya, Amanda; Zipfel, Gregory J; Dhar, Rajat

2016-02-01

Hydrocephalus requiring external ventricular drain (EVD) or shunt placement commonly complicates aneurysmal subarachnoid hemorrhage (SAH), but its frequency is not as well known for nonaneurysmal SAH (NA-SAH). Those with diffuse bleeding may have greater risk of hydrocephalus compared to those with a perimesencephalic pattern. We evaluated the frequency of hydrocephalus in NA-SAH and whether imaging factors could predict the need for EVD and shunting. We collected admission clinical and imaging variables for 105 NA-SAH patients, including bicaudate index (BI), Hijdra sum score (HSS), intraventricular hemorrhage (IVH) score, modified Fisher scale (mFS), and bleeding pattern. Hydrocephalus was categorized as acute (need for EVD) or chronic (shunt). We applied logistic regression to determine whether hydrocephalus risk was independently related to bleeding pattern or mediated through blood volume or ventriculomegaly. Acute hydrocephalus was seen in 26 (25%) patients but was more common with diffuse (15/28, 54%) versus perimesencephalic (10/59, 17%, p hydrocephalus had worse clinical grade and higher BI, HSS, and IVH scores. Adjusting the relationship between hydrocephalus and diffuse bleeding for HSS (but not BI) nullified this association. Nine (35%) patients requiring EVD eventually required shunting for chronic hydrocephalus, which was associated with greater blood burden but not poor clinical grade. Acute hydrocephalus occurs in one-quarter of NA-SAH patients. The greater risk in diffuse bleeding appears to be mediated by greater cisternal blood volume but not by greater ventriculomegaly. Imaging characteristics may aid in anticipatory management of hydrocephalus in NA-SAH.

Communicating hydrocephalus. Cisternographic and neuropathologic studies

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Price, D.L.; James, E.; Sperber, E.; Strecker, E.P.

1976-01-01

Chronic communicating hydrocephalus was produced in adult dogs by injection of silastic into the subarachnoid space. Electron microscopy was used to verify the sequence of pathologic changes in the ventricular wall. The pathologic findings were correlated with cisternographic images and measurements of cerebrospinal fluid (CSF) pressure. Early in hydrocephalus, the CSF pressure was increased and cisternograms showed ventricular entry and clearing; the ependyma was stretched and fluid accumulated in subependymal regions. In animals with chronic hydrocephalus, the CSF pressure was normal and cisternograms disclosed radioactivity persisting in the ventricles. At this time the ependyma was severely damaged, the subependymal white matter showed enlargement of the extracellular space, and degenerative changes were present in axons and myelin sheaths

MR elastography of hydrocephalus

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Pattison, Adam J.; Lollis, S. Scott; Perrinez, Phillip R.; Weaver, John B.; Paulsen, Keith D.

2009-02-01

Hydrocephalus occurs due to a blockage in the transmission of cerebrospinal fluid (CSF) in either the ventricles or subarachnoid space. Characteristics of this condition include increased intracranial pressure, which can result in neurologic deterioration [1]. Magnetic resonance elastography (MRE) is an imaging technique that estimates the mechanical properties of tissue in vivo. While some investigations of brain tissue have been performed using MRE [2,3,4,5], the effects due to changes in interstitial pressure and fluid content on the mechanical properties of the brain remain unknown. The purpose of this work is to assess the potential of MRE to differentiate between the reconstructed properties of normal and hydrocephalic brains. MRE data was acquired in 18 female feline subjects, 12 of which received kaolin injections resulting in an acute form of hydrocephalus. In each animal, four MRE scans were performed during the process including one pre-injection and three post-injection scans. The elastic parameters were obtained using a subzone-based reconstruction algorithm that solves Navier's equations for linearly elastic materials [6]. The remaining cats were used as controls, injected with saline instead of kaolin. To determine the state of hydrocephalus, ventricular volume was estimated from segmenting anatomical images. The mean ventricular volume of hydrocephalic cats significantly increased (P ~ 0.5) for the control cats. Also, there was an observable increase in the recorded elastic shear modulus of brain tissue in the normal and hydrocephalic acquisitions. Results suggest that MRE is able to detect changes in the mechanical properties of brain tissue resulting from kaolin-induced hydrocephalus, indicating the need for further study.

Congenital tumors of the central nervous system

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Severino, Mariasavina [G. Gaslini Children' s Hospital, Department of Neuroradiology, Genoa (Italy); Schwartz, Erin S. [The Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); Thurnher, Majda M. [Medical University of Vienna, Department of Radiology, Vienna (Austria); Rydland, Jana [MR Center, St. Olav' s Hospital HF, Trondheim (Norway); Nikas, Ioannis [Agia Sophia Children' s Hospital, Imaging Department, Athens (Greece); Rossi, Andrea [G. Gaslini Children' s Hospital, Department of Neuroradiology, Genoa (Italy); G. Gaslini Children' s Hospital, Department of Pediatric Neuroradiology, Genoa (Italy)

2010-06-15

Congenital tumors of the central nervous system (CNS) are often arbitrarily divided into ''definitely congenital'' (present or producing symptoms at birth), ''probably congenital'' (present or producing symptoms within the first week of life), and ''possibly congenital'' (present or producing symptoms within the first 6 months of life). They represent less than 2% of all childhood brain tumors. The clinical features of newborns include an enlarged head circumference, associated hydrocephalus, and asymmetric skull growth. At birth, a large head or a tense fontanel is the presenting sign in up to 85% of patients. Neurological symptoms as initial symptoms are comparatively rare. The prenatal diagnosis of congenital CNS tumors, while based on ultrasonography, has significantly benefited from the introduction of prenatal magnetic resonance imaging studies. Teratomas constitute about one third to one half of these tumors and are the most common neonatal brain tumor. They are often immature because of primitive neural elements and, rarely, a component of mixed malignant germ cell tumors. Other tumors include astrocytomas, choroid plexus papilloma, primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, and medulloblastomas. Less common histologies include craniopharyngiomas and ependymomas. There is a strong predilection for supratentorial locations, different from tumors of infants and children. Differential diagnoses include spontaneous intracranial hemorrhage that can occur in the presence of coagulation factor deficiency or underlying vascular malformations, and congenital brain malformations, especially giant heterotopia. The prognosis for patients with congenital tumors is generally poor, usually because of the massive size of the tumor. However, tumors can be resected successfully if they are small and favorably located. The most favorable outcomes are achieved with choroid plexus tumors

Brain scintigraphy in patients with hydrocephalus

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Kadowaki, Hirotaka; Imanaga, Hirohisa; Kitamura, Koichi

1975-01-01

Brain scintigraphy is a simple, innocuous and valuable diagnostic method. Particulary in children, in order to minimize damage by examination, brain scintigraphy should be the first examination for patients, before other diagnostic methods such as cerebral angiography and pneumoencephalography. In the 3 years between Jan. 1970 and Dec. 1972, 235 children under the age of 15 years had brain scintigraphy with sup(99m)Tc pertechnetate at the Tokyo Women's Medical College Hospital. The authors especially attended to the findings of scintigrams in children with hydrocephalus. 29 children with non-neoplastic hydrocephalus were scanned with sup(99m)Tc pertechnetate: noncommunicating hydrocephalus; 9; hydroencephalodysplasia; 4; Chiari Arnold malformation; 1; cranium bifidum; 2; communicating hydrocephalis; 13. In noncommunicating hydrocephalus, the midline structure on the scintigram appeared thicker than the normal, because activity in the superior sagittal sinus was relatively high compared to the reduction of activity in the enlarged ventricles. In cases of very severe hydrocephalus, the basal ganglia was shown as a clear hot spot on the scintigram, because R. I. activity in the basal ganglia was relatively high compared to the reduction of R. I. activity in the enlarged ventricles. In cases of hydroencephalodysplasia, the midline structure on the scintigram shifted to the side opposite the dysplasia, as the result of pressure from a large CSF collection. In cases of cranium bifidum, the scintigram showed an elevation of the transverse sinus and the confluent sinus. (auth.)

Epidemiology of adult-onset hydrocephalus: institutional experience with 2001 patients.

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Bir, Shyamal C; Patra, Devi Prasad; Maiti, Tanmoy K; Sun, Hai; Guthikonda, Bharat; Notarianni, Christina; Nanda, Anil

2016-09-01

OBJECTIVE Adult-onset hydrocephalus is not commonly discussed in the literature, especially regarding its demographic distribution. In contrast to pediatric hydrocephalus, which is related to a primary CSF pathway defect, its development in adults is often secondary to other pathologies. In this study, the authors investigated the epidemiology of adult-onset hydrocephalus as it pertains to different etiologies and in reference to age, sex, and race distributions. METHODS The authors retrospectively reviewed the clinical notes of 2001 patients with adult-onset hydrocephalus who presented to Louisiana State University Health Sciences Center within a 25-year span. Significant differences between the groups were analyzed by a chi-square test; p hydrocephalus in this population was 77 ± 30 per year, with a significant increase in incidence in the past decade (55 ± 3 [1990-2003] vs 102 ± 6 [2004-2015]; p Hydrocephalus in a majority of the patients had a vascular etiology (45.5%) or was a result of a tumor (30.2%). The incidence of hydrocephalus in different age groups varied according to various pathologies. The incidence was significantly higher in males with normal-pressure hydrocephalus (p = 0.03) or head injury (p = 0.01) and higher in females with pseudotumor cerebri (p hydrocephalus was significantly higher in Caucasian patients (p = 0.0002) than in those of any other race. CONCLUSIONS Knowledge of the demographic variations in adult-onset hydrocephalus is helpful in achieving better risk stratification and better managing the disease in patients. For general applicability, these results should be validated in a large-scale meta-analysis based on a national population database.

Diagnosis, treatment and long-term outcome in fetal hydrocephalus

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Yamasaki, Mami; Nonaka, Masahiro; Bamba, Yohei; Teramoto, Chika; Ban, Chiaki; Pooh, Ritsuko

2011-01-01

The objective of this study was to evaluate the method of prenatally estimating an appropriate clinical outcome in fetal hydrocephalus. Retrospective study, single institute (Osaka National Hospital). Hundred and seventeen cases with fetal hydrocephalus treated at Osaka National Hospital from 1992 to 2010 were analysed. Of the 117 cases analysed, 38% are diagnosed as isolated ventriculomegaly (IVM), 51% as other types of malformation (30 cases of myelomeningocele, 4 cases of holoprosencephaly, 4 of Dandy Walker syndrome, 10 of arachnoid cyst and 6 of encephalocele etc.) and 11% as secondary hydrocephalus. They are diagnosed between 17 and 40 weeks of gestation (average 27 weeks), 17% diagnosed between 17 and 21 weeks, 30% between 22 and 27 weeks and 53% after 28 weeks. With the exception of 9 aborted cases and 30 unknown cases too young to be evaluated or lost due to lack of follow-up, final outcome was analyzed in 78 cases. Of these 78 cases, 15% died in utero or after birth, 23% showed severe retardation, 17% moderate retardation, 26% mild retardation, and 19% showed good outcome. Long term consequences were mostly influenced by basic disease and accompanied anomalies. Hydrocephalus associated with arachnoid cyst, atresia of Monro, corpus callosum agenesis and hydrocephalus due to fetal intracranial hemorrhage are categorized in the good outcome group. On the other hand, holoprosencephaly, hydrocephalus associated with encephalocele, syndromic hydrocephalus and hydrocephalus due to fetal virus infection are categorized in the poor outcome group. In order to accurate diagnosis and proper counseling, establishment of diagnosis protocol and treatment policy for fetal hydrocephalus including not only fetal sonography, fetal MRI, toxoplasma, rubella, cytomegalovirus, herpes simplex (TORCH) screening test but also chromosomal and gene testing is required. (author)

New concept of the pathogenesis and therapeutic orientation of acquired communicating hydrocephalus.

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Xu, Hao

2016-09-01

Hydrocephalus is a common medical condition characterized by abnormalities in the secretion, circulation and absorption of cerebrospinal fluid (CSF), resulting in ventricle dilatation. For the communicating hydrocephalus, without etiological treatment, its pathogenesis has been considered as a research emphasis. Many factors can damage the CSF system and trigger communicating hydrocephalus, including tumor surgery and hydrocephalus neurological diseases, such as brain trauma, infection, ICH and SAH. But according to our clinical experience, a big proportion of patients do not develop hydrocephalus. That is because the absorbing ability of CSF can compensate within a certain range. If the damage exceeds that range, hydrocephalus will occur. Once it occurs, it is not likely to be reversed, so a shunt surgery is always needed. Therefore, we believe that our orientation could transform the treatment of patient who has already showed hydrocephalus symptoms to the prevention of the occurrence in the patient with high risk of hydrocephalus. Based on the hypothesis above, we first divide the process of hydrocephalus into three stages and we believe that hydrocephalus are possible be reversed or halted in stage 1 and 2. The new concept of the pathogenesis in hydrocephalus will enrich our understanding and provide new insights to the therapeutic orientation. In conclusion, the future research direction should be the prevention of hydrocephalus, which should take a long period from the immediate occurrence of brain injury to several months or even years after the injury.

Post-hemispherectomy hydrocephalus: results of a comprehensive, multi-institutional review

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Lew, Sean M.; Matthews, Anne E.; Hartman, Adam L.; Haranhalli, Neil

2012-01-01

SUMMARY Purpose Hemispherectomy surgery for medically intractable epilepsy is known to cause hydrocephalus in a subset of patients. Existing data regarding the incidence of, and risk factors for developing, post-hemispherectomy hydrocephalus has been limited by the relatively small number of cases performed by any single center. Our goal was to better understand this phenomenon and to identify risk factors that may predispose patients to developing hydrocephalus after hemispherectomy surgery. Methods Fifteen pediatric epilepsy centers participated in this study. A retrospective chart review was performed on all available patients who had hemispherectomy surgery. Data collected included surgical techniques, etiology of seizures, prior brain surgery, symptoms and signs of hydrocephalus, timing of shunt placement and basic demographics. Key findings Data were collected from 736 patients who underwent hemispherectomy surgery between 1986 and 2011. Forty-six patients had pre-existing shunted hydrocephalus and were excluded from analysis, yielding 690 patients for this study. One hundred sixty-two patients (23%) required hydrocephalus treatment. The timing of hydrocephalus ranged from the immediate post-operative period to 8.5 years after surgery, with 43 patients (27%) receiving shunts more than 90 days after surgery. Multivariate regression analysis revealed anatomic hemispherectomies (OR 4.1, phydrocephalus. There was a trend towards significance for the use of hemostatic agents (O.R. 2.2, p=0.07) and the involvement of basal ganglia or thalamus in the resection (O.R. 2.2, p=0.08) as risk factors. Significance Hydrocephalus is a common sequela of hemispherectomy surgery. Surgical technique and prior brain surgery influence the occurrence of post-hemispherectomy hydrocephalus. A significant portion of patients develop hydrocephalus on a delayed basis, indicating the need for long-term surveillance. PMID:23106378

MRI and hydrocephalus in childhood

International Nuclear Information System (INIS)

Britton, J.; Marsh, H.; Kendall, B.; Kingsley, D.

1988-01-01

In six young patients presenting with raised intracranial pressure during the period of a year, CT revealed the presence of hydrocephalus, but not the cause. Magnetic Resonance Imaging not only showed the site and nature of the obstructing lesion, but also detected additional clinically silent spinal cord tumors in five of the patients. The place of MRI in the diagnosis of diseases involving the region of the cranio-cervical junction and in the elucidation of 'unexplained hydrocephalus' is considered. (orig.)

CT and MR imaging features of hydrocephalus

International Nuclear Information System (INIS)

Shier, C.K.; George, A.E.; de Leon, M.J.; Stylopoulos, L.A.; Pinto, R.S.

1989-01-01

Sylvian fissure and sulcal enlargement is generally perceived as indicative of cortical atrophy and has been used by surgeons in cases of suspected hydrocephalus as a criterion for exclusion from ventricular shunting procedure. The authors have observed sylvian fissure collapse following ventricular shunting in several patients with communicating hydrocephalus (CH). The purpose of this study was to determine the incidence of this finding in patients with CH. The pre- and postshunt CT and MR images of 30 patients with communicating hydrocephalus were reviewed. As anticipated, after shunting a diminution in caliber of the lateral ventricle bodies, temporal horns, and third ventricle occurred in a majority of cases. However, sulcal width paradoxically decreased in 13% of cases after shunt, and sylvian fissure size decreased in seven patients after shunt (23%). In summary, large sylvian fissures and focally dilated sulci do not rule out the presence of hydrocephalus and may in fact act as cerebrospinal fluid reservoirs in cases of obstruction higher along the cerebral convexities

Hydrocephalus-history of surgical treatment over the centuries

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Milojević Aleksandar J.

2012-01-01

Full Text Available To trace the history of the treatment of hydrocephalus is to document the parallel development of medicine as a whole; when one reviews the treatment of hydrocephalus, the integral relationship between basic science and therapy is reaffirmed. The treatment of hydrocephalus, over the centuries, underwent three stages of evolution. Prior to the late 19 th century, treatment for 'water on the brain' involved more observation than intervention. During antiquity, middle ages and renaissance, hydrocephalus was not understood. Medical treatment was useless; surgery was hopeless. The second stage extends from the 19th century to the end of the first half of the 20 th century. Cerebrospinal fluid circulation was now understood; surgery however, remained inefficient, but some patients survived with arrested hydrocephalus. The third stage begins in the nineteen fifties with the development of silicone shunts with a valve. Surgery transforms the prognosis of hydrocephalus, but the number of post-operative complications creates new problems. The different attempts that have been made during these past two decades to solve these problems are reviewed. They have resulted in a reduction of the mechanical and infectious complications. CSF over drainage has been minimized. Percutaneous ventriculo-cisternostomies have in some cases replaced shunts. In the future, to improve outcome in these hydrocephalics, surgery, when indicated, should be performed as early as possible. Knowledge and prevention of the causes of hydrocephalus should be developed. As we progress further in this new millennium, it is appropriate to reflect on the past understanding and treatment of this disorder, review strategies to curb this disease process, and consider therapies and possibly cures that will be available in the future.

Congenital toxoplasmosis presenting as central diabetes insipidus in an infant: a case report.

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Mohamed, Sarar; Osman, Abdaldafae; Al Jurayyan, Nasir A; Al Nemri, Abdulrahman; Salih, Mustafa A M

2014-03-28

Congenital toxoplasmosis has a wide range of presentation at birth varying from severe neurological features such as hydrocephalus and chorioretinitis to a well appearing baby, who may develop complications late in infancy. While neuroendocrine abnormalities associated with congenital toxoplasmosis are uncommon, isolated central diabetes insipidus is extremely rare. Here, we report on a female infant who presented with fever, convulsions, and polyuria. Examination revealed weight and length below the 3rd centile along with signs of severe dehydration. Fundal examination showed bilateral chorioretinitis. This infant developed hypernatremia together with increased serum osmolality and decreased both urine osmolality and specific gravity consistent with central diabetes insipidus. Serology for toxoplasma specific immunoglobulin M was high for both the mother and the baby and polymerase chain reaction for toxoplasma deoxyribonucleic acid was positive in the infant confirming congenital toxoplasmosis. Brain computerized tomography scans demonstrated ventriculomegaly associated with cerebral and cortical calcifications. Fluid and electrolyte abnormalities responded to nasal vasopressin therapy. This report highlights central diabetes inspidus as a rare presentation of congenital toxoplasmosis.

Hydrocephalus after decompressive craniectomy for malignant hemispheric cerebral infarction.

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Wang, Qiang-Ping; Ma, Jun-Peng; Zhou, Zhang-Ming; Yang, Min; You, Chao

2016-08-01

Several studies have investigated the incidence and risk factors of hydrocephalus after decompressive craniectomy (DC) for malignant hemispheric cerebral infarction. However, the results are controversial. Therefore, the following is a retrospective cohort study to determine the incidence and risk factors of hydrocephalus after DC for malignant hemispheric cerebral infarction. From January 2004 to June 2014, patients at two medical centres in south-west China, who underwent DC for malignant hemispheric cerebral infarction, were included. The patients' clinical and radiologic findings were retrospectively reviewed. A chi-square test, Mann-Whitney U-test and logistic regression model were used to identify the risk factors. A total of 128 patients were included in the study. The incidence of ventriculomegaly and shunt-dependent hydrocephalus were 42.2% (54/128) and 14.8% (19/128), respectively. Lower preoperative Glasgow Coma Scale (GCS) score and presence of subarachnoid haemorrhage (SAH) were factors significantly associated with the development of post-operative hydrocephalus after DC. Cerebral infarction patients receiving DC have a moderate tendency to suffer from post-operative hydrocephalus. A poor GCS score and the presence of SAH were significantly associated with the development of hydrocephalus after DC.

Decompressive craniectomy and hydrocephalus: proposal of a therapeutic flow chart.

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Peraio, Simone; Calcagni, Maria Lucia; Mattoli, Maria Vittoria; Marziali, Giammaria; DE Bonis, Pasquale; Pompucci, Angelo; Anile, Carmelo; Mangiola, Annunziato

2017-12-01

Decompressive craniectomy (DC) may be necessary to save the lives of patients suffering from intracranial hypertension. However, this procedure is not complication-free. Its two main complications are hydrocephalus and the sinking skin-flap syndrome (SSFS). The radiological findings and the clinical evaluation may be not enough to decide when and/or how to treat hydrocephalus in a decompressed patient. SSFS and hydrocephalus may be not unrelated. In fact, a patient affected by hydrocephalus, after the ventriculo-peritoneal shunt, can develop SSFS; on the other hand, SSFS per se can cause hydrocephalus. Treating hydrocephalus in decompressed patients can be challenging. Radiological findings and clinical evaluation may not be enough to define the most appropriate therapeutic strategy. Cerebrospinal fluid (CSF) dynamics and metabolic evaluations can represent important diagnostic tools for assessing the need of a CSF shunt in patients with a poor baseline neurologic status. Based on our experience, we propose a flow chart for treating decompressed patients affected by ventriculomegaly.

Ruptured Massa Intermedia Secondary to Hydrocephalus.

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El Damaty, Ahmed; Langner, Soenke; Schroeder, Henry W S

2017-01-01

We report a case of ruptured massa intermedia (MI) as a sequela of hydrocephalus. A single case report is presented describing the sequelae of tumor bed hematoma after a posterior fossa hemangioblastoma resection in which the patient bled 3 days after surgery, resulting in secondary hydrocephalus and subsequently dilatation of the third ventricle, which resulted in rupture of the MI. The patient was managed on emergency basis with an external ventricular drain then endoscopically with a third ventriculostomy and clot extraction. Absent MI is not uncommon in hydrocephalic patients, and it is assumed to be the result of rupture from acute dilatation of the third ventricle. Our case report proves this assumption and documents the presence and absence of the MI before and after developing hydrocephalus. Copyright © 2016 Elsevier Inc. All rights reserved.

Congenital club foot in a teaching hospital in Lagos, Nigeria.

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Adewole, O A; Giwa, S O; Kayode, M O; Shoga, M O; Balogun, R A

2009-06-01

Congenital club foot has been sparsely reported in literature in Nigeria, although it has been reported as the commonest congenital musculoskeletal abnormality. This study enumerates the point prevalence of this disease in a university teaching hospital in Lagos. Better understanding of the epidemiology in our community should improve awareness, and influence management. Between June 2005 and July 2006, 72 consecutive patients with congenital club feet were seen in the orthopaedic clinic of our Hospital. Demographic data, birth weight, family history, birth facility, maternal age and associated congenital anomalies were recorded and analysed using Statistical Programme for Social Sciences (SPSS) version 15. A total of 72 patients were seen, 28 of whom had bilateral club feet resulting in a total of 100 feet. There were 38 males and 34 females. Only 29% presented in the first month of life and 28% in the second month. Maternal ages ranged between 19 and 38 years and no family history of congenital club foot was given,. Babies delivered outside the orthodox medical system (churches, traditional healers, home etc) constituted 28%. The commonest associated congenital anomalies were tibia hemimelia, hydrocephalus, inguinal hernia and umbilical hernia. A default rate of 28% was observed during treatment. Congenital club foot may not be uncommon in Nigeria. Late presentation and high default rate before correction of the deformity were observed. Establishment of special club foot clinics should reduce the default rate. Training of healthcare workers in maternity units as well as Public awareness should encourage early referral to specialists.

Spontaneous subconjunctival abscess in congenital lamellar ichthyosis

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Shivanand C Bubanale

2018-01-01

Full Text Available Congenital lamellar ichthyosis is an autosomal recessive, heterogeneous disorder presenting at birth with generalized skin involvement. The most common ophthalmic manifestation noted is bilateral ectropion of the lower eyelids. A 1-month-old female neonate, the second born of a nonconsanguineous marriage, presented with 4 days' history of redness, discharge, and swelling in the right eye. There was severe right upper eyelid ectropion, conjunctival injection, chemosis, a subconjunctival mass on the temporal bulbar conjunctiva spontaneously draining pus and corneal haze. The anterior chamber, iris, lens and fundus appeared normal. Congenital lamellar ichthyosis was suspected because of scaling and excessive dryness of the entire body. The occurrence of a spontaneous subconjunctival abscess is not known in lamellar ichthyosis. We thus report the management of a rare case of unilateral upper eyelid ectropion, subconjunctival abscess with orbital cellulitis in congenital lamellar ichthyosis.

Normal Pressure Hydrocephalus

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... improves the chance of a good recovery. Without treatment, symptoms may worsen and cause death. What research is being done? The NINDS conducts and supports research on neurological disorders, including normal pressure hydrocephalus. Research on disorders such ...

Two types of congenital hydrocephalus induced in rats by X-irradiation in utero: electron microscopic study on the telencephalic wall

International Nuclear Information System (INIS)

Takeuchi, I.K.; Murakami, U.

1979-01-01

Stage-specific incidences of congenital hydrocephalus induced by X-irradiation of pregnant rats showed a bimodal distribution. At a dose level of 200 R, 100% hydrocephalic offspring were obtained by irradiation at embryonic days 11 and 14. When pregnant rats were subjected to 200 R X-irradiation at embryonic day 11, numerous ventricular cells of the telencephalic wall of the embryo became necrotic during the first 2 hours post-irradiation, but the paraventricular cell-to-cell interconnexions made up of zonulae adhaerentes were less affected. Mitosis took place in the surviving paraventricular surface cells throughout subsequent development. The full-term fetus exhibited little change in the cytoarchitectural arrangement of neural cells and neuropils, although it was only about half the thickness of the untreated control. After 200 R X-irradiation at embryonic day 14, most of the ventricular cells became necrotic within 6 hours. The paraventricular cell-to-cell interconnexions were completely destroyed, and never repaired in subsequent development. Mitosis took place either freely in cell clusters, or in rosettes which formed randomly in the telencephalic wall between 48 and 72 hours post-irradiation. The resulting telencephalic wall of the full-term fetus was also about half the thickness of the control. In the outer part of the tissue, the cortical plate made up of differentiating neuroblasts was hypoplastic, but the inner half was filled with numerous heterotopic masses of pleomorphic cells and bundles of primitive axons. The ependymal layer at the paraventricular surface was never formed. Whether the paraventricular zonulae adhaerentes were destroyed or not by X-irradiation was considered to be an important factor in the determination of the subsequent cytoarchitectural organization of the telencephalic wall. (author)

Controversy about Management of Hydrocephalus - Shunt vs. Endoscopic Third Ventriculostomy.

Science.gov (United States)

Kumar, Vikas; Bodeliwala, Shaam; Singh, Daljit

2017-08-01

The best management of hydrocephalus is still controversial in the twenty-first century. Shunt treatment for hydrocephalus is the most common procedure performed in neurosurgical practice and is associated with the highest complications rate. But during the last 2 decades, the treatment of hydrocephalus has improved with better shunt devices available today, increased facilities for investigations and newer approaches like endoscopic third ventriculostomy. The recent advances in development of better endoscopes have provided the patient and treating doctor with an option for an alternative surgery for treatment of hydrocephalus.

Increased risk of hydrocephalus in long-term dialysis patients.

Science.gov (United States)

Wang, I-Kuan; Lin, Cheng-Li; Cheng, Yu-Kai; Chou, Che-Yi; Liang, Chih-Chia; Yen, Tzung-Hai; Sung, Fung-Chang

2016-05-01

The risk of hydrocephalus in end-stage renal disease (ESRD) patients on dialysis has not been studied in depth. Using Taiwan National Health Insurance claims data, we identified 29 684 incident ESRD patients from 2000 to 2010, including 10 030 peritoneal dialysis (PD) patients and 19 654 hemodialysis (HD) patients. The control cohort consisted of 118 736 people randomly selected from those without kidney disease, frequency matched with ESRD patients by age, sex and index year. We also established propensity score-matched cohorts with 10 014 PD and 10 014 HD patients. The incidence rates and hazard ratios (HRs) of hydrocephalus were calculated until the end of 2011. Incidence rates of hydrocephalus were greater in HD and PD patients than in controls (8.44 and 11.0 versus 4.11 per 10 000 person-years, respectively), with an adjusted HR of 1.86 [95% confidence interval (CI) 1.43-2.41] for all ESRD patients compared with controls. A higher proportion of hydrocephalus patients underwent surgical bypass to relieve hydrocephalus in ESRD patients than controls, 40.7% (46/113) versus 24.5% (67/273), with an adjusted odds ratio of 2.11 (95% CI 1.33-3.36). Compared with controls, the adjusted HRs of communicating hydrocephalus for HD and PD patients were 1.77 (95% CI 1.22-2.55) and 2.51 (95% CI 1.61-3.89), respectively. The propensity score-matched analysis showed an HR of 0.72 (95% CI 0.42-1.23) for hydrocephalus in HD patients compared with PD patients. Patients with ESRD are at an increased risk of hydrocephalus. The risk difference between HD and PD patients is not significant. © The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

Congenital hearing loss. Is CT enough? | Agha | Alexandria Journal ...

African Journals Online (AJOL)

Patients and methods: This is a prospective study including 60 patients, 24 males and 36 females aged from 1 to 7 years, who were presented by unilateral or bilateral congenital conductive (CHL) or/and sensorineural hearing loss (SNHL). All patients were evaluated by HRCT scan with post-processing multiplanar ...

Vertebrobasilar dolichoectasia induced hydrocephalus: the water-hammer effect

Directory of Open Access Journals (Sweden)

Vaso Zisimopoulou

2015-05-01

Full Text Available Vertebrobasilar dolichoectasia is a clinical entity associated rarely with obstructive hydrocephalus. We present a 48-year old male with a profound dilatation of the ventricular system due to a dolichoectatic basilar artery, as appeared in imaging studies. The patient suffered from longstanding hydrocephalus and presenile dementia. The underlying mechanism for obstructive hydrocephalus due to vertebrobasilar dolichoectasia is considered to be both a water-hammer effect and a direct compression of adjacent structures. We suggest prompt surgical intervention upon diagnosis as a first choice treatment in order to avoid further complications.

Mechanisms of hydrocephalus in cerebral cysticercosis: implications for therapy

International Nuclear Information System (INIS)

Estanol, B.; Kleriga, E.; Loyo, M.; Mateos, H.; Lombardo, L.; Gordon, F.; Saguchi, A.F.

1983-01-01

Patients with hydrocephalus secondary to cerebral cysticercosis are a highly heterogeneous group. The mechanisms of hydrocephalus in these patients are multiple. Intraventricular cysts may be found in the 3rd and 4th ventricles, the sylvian aqueduct, and the foramen of Monro. Intraventricular cysts can be suspected when the 3rd and 4th ventricles or aqueduct remain enlarged despite shunting. Intraventricular contrast medium demonstrates the presence of the parasites. The intraventricular cysts should be removed surgically. Hydrocephalus due to cisternal cysticercosis can be diagnosed by isotope cisternography. These patients should receive shunts, but the long term prognosis is probably poor. Guidelines for the management of hydrocephalus due to cysticercosis are suggested

Mechanisms of hydrocephalus in cerebral cysticercosis: implications for therapy

Energy Technology Data Exchange (ETDEWEB)

Estanol, B.; Kleriga, E.; Loyo, M.; Mateos, H.; Lombardo, L.; Gordon, F.; Saguchi, A.F.

1983-08-01

Patients with hydrocephalus secondary to cerebral cysticercosis are a highly heterogeneous group. The mechanisms of hydrocephalus in these patients are multiple. Intraventricular cysts may be found in the 3rd and 4th ventricles, the sylvian aqueduct, and the foramen of Monro. Intraventricular cysts can be suspected when the 3rd and 4th ventricles or aqueduct remain enlarged despite shunting. Intraventricular contrast medium demonstrates the presence of the parasites. The intraventricular cysts should be removed surgically. Hydrocephalus due to cisternal cysticercosis can be diagnosed by isotope cisternography. These patients should receive shunts, but the long term prognosis is probably poor. Guidelines for the management of hydrocephalus due to cysticercosis are suggested.

Impact of operation details on hydrocephalus after decompressive craniectomy

Science.gov (United States)

Wang, Qiang-Ping; Ma, Jun-Peng; Zhou, Zhang-Ming; You, Chao

2016-01-01

Objective: To evaluate the correlation between the distance of craniectomy from the midline and hydrocephalus after DC. Methods: The following electronic databases were searched from their inception to June 2015: Cochrane Library, MEDLINE, Science Direct, EMBASE, Scopus, Google Scholar, the Chinese Biomedical Database (CBM), and the Chinese National Knowledge Infrastructure (CNKI). All randomized clinical trials, prospective cohort, retrospective observational cohort, and case-control studies investigating the relationship between distance of craniectomy from the midline and hydrocephalus after DC were enrolled. The Cochrane Collaboration’s software RevMan 5.3 was used for meta-analysis. Results: Six retrospective cohort studies involving 462 participants were included. Pooled analysis of 4 studies suggested that craniectomy close to the midline (hydrocephalus (odds ratio [OR] = 3.61, 95% confidence interval [CI]: 1.3 - 9.97, p=0.01). However, meta-analysis of 4 studies did not find statistical differences when comparing the distance of craniectomy from the midline in the hydrocephalus group and that in the non-hydrocephalus group (OR = −0.14, 95% CI: −0.44 - 0.15, p=0.34). Conclusions: Available evidence was insufficient to support the theory that craniectomy close to the midline increases the risk of developing hydrocephalus after DC. Well-conducted randomized clinical trials are required to verify this issue. PMID:26818161

Pediatric hydrocephalus outcomes: a review

Directory of Open Access Journals (Sweden)

Vinchon Matthieu

2012-08-01

Full Text Available Abstract The outcome of pediatric hydrocephalus, including surgical complications, neurological sequelae and academic achievement, has been the matter of many studies. However, much uncertainty remains, regarding the very long-term and social outcome, and the determinants of complications and clinical outcome. In this paper, we review the different facets of outcome, including surgical outcome (shunt failure, infection and independence, and complications of endoscopy, clinical outcome (neurological, sensory, cognitive sequels, epilepsy, schooling and social integration. We then provide a brief review of the English-language literature and highlighting selected studies that provide information on the outcome and sequelae of pediatric hydrocephalus, and the impact of predictive variables on outcome. Mortality caused by hydrocephalus and its treatments is between 0 and 3%, depending on the duration of follow-up. Shunt event-free survival (EFS is about 70% at one year and 40% at ten years. The EFS after endoscopic third ventriculostomy (ETV appears better but likely benefits from selection bias and long-term figures are not available. Shunt infection affects between 5 and 8% of surgeries, and 15 to 30% of patients according to the duration of follow-up. Shunt independence can be achieved in 3 to 9% of patients, but the definition of this varies. Broad variations in the prevalence of cognitive sequelae, affecting 12 to 50% of children, and difficulties at school, affecting between 20 and 60%, attest of disparities among studies in their clinical evaluation. Epilepsy, affecting 6 to 30% of patients, has a serious impact on outcome. In adulthood, social integration is poor in a substantial number of patients but data are sparse. Few controlled prospective studies exist regarding hydrocephalus outcomes; in their absence, largely retrospective studies must be used to evaluate the long-term consequences of hydrocephalus and its treatments. This review

Angle closure glaucoma in congenital ectropion uvea

Directory of Open Access Journals (Sweden)

Grace M. Wang

2018-06-01

Full Text Available Purpose: Congenital ectropion uvea is a rare anomaly, which is associated with open, but dysplastic iridocorneal angles that cause childhood glaucoma. Herein, we present 3 cases of angle-closure glaucoma in children with congenital ectropion uvea. Observations: Three children were initially diagnosed with unilateral glaucoma secondary to congenital ectropion uvea at 7, 8 and 13 years of age. The three cases showed 360° of ectropion uvea and iris stromal atrophy in the affected eye. In one case, we have photographic documentation of progression to complete angle closure, which necessitated placement of a glaucoma drainage device 3 years after combined trabeculotomy and trabeculectomy. The 2 other cases, which presented as complete angle closure, also underwent glaucoma drainage device implantation. All three cases had early glaucoma drainage device encapsulation (within 4 months and required additional surgery (cycloablation or trabeculectomy. Conclusions and importance: Congenital ectropion uvea can be associated with angle-closure glaucoma, and placement of glaucoma drainage devices in all 3 of our cases showed early failure due to plate encapsulation. Glaucoma in congenital ectropion uvea requires attention to angle configuration and often requires multiple surgeries to obtain intraocular pressure control. Keywords: Congenital ectropion uvea, Juvenile glaucoma, Angle-closure glaucoma, Glaucoma drainage device

Hydrocephalus in adults with community-acquired bacterial meningitis

NARCIS (Netherlands)

Soemirien Kasanmoentalib, E.; Brouwer, Matthijs C.; van der Ende, Arie; van de Beek, Diederik

2010-01-01

Objective: To evaluate the occurrence, treatment, and outcome of hydrocephalus complicating community-acquired bacterial meningitis in adults. Methods: Case series from a prospective nationwide cohort study from Dutch hospitals from 2006 to 2009. Results: Hydrocephalus was diagnosed in 26 of 577

Comparison of CSF Distribution between Idiopathic Normal Pressure Hydrocephalus and Alzheimer Disease.

Science.gov (United States)

Yamada, S; Ishikawa, M; Yamamoto, K

2016-07-01

CSF volumes in the basal cistern and Sylvian fissure are increased in both idiopathic normal pressure hydrocephalus and Alzheimer disease, though the differences in these volumes in idiopathic normal pressure hydrocephalus and Alzheimer disease have not been well-described. Using CSF segmentation and volume quantification, we compared the distribution of CSF in idiopathic normal pressure hydrocephalus and Alzheimer disease. CSF volumes were extracted from T2-weighted 3D spin-echo sequences on 3T MR imaging and quantified semi-automatically. We compared the volumes and ratios of the ventricles and subarachnoid spaces after classification in 30 patients diagnosed with idiopathic normal pressure hydrocephalus, 10 with concurrent idiopathic normal pressure hydrocephalus and Alzheimer disease, 18 with Alzheimer disease, and 26 control subjects 60 years of age or older. Brain to ventricle ratios at the anterior and posterior commissure levels and 3D volumetric convexity cistern to ventricle ratios were useful indices for the differential diagnosis of idiopathic normal pressure hydrocephalus or idiopathic normal pressure hydrocephalus with Alzheimer disease from Alzheimer disease, similar to the z-Evans index and callosal angle. The most distinctive characteristics of the CSF distribution in idiopathic normal pressure hydrocephalus were small convexity subarachnoid spaces and the large volume of the basal cistern and Sylvian fissure. The distribution of the subarachnoid spaces in the idiopathic normal pressure hydrocephalus with Alzheimer disease group was the most deformed among these 3 groups, though the mean ventricular volume of the idiopathic normal pressure hydrocephalus with Alzheimer disease group was intermediate between that of the idiopathic normal pressure hydrocephalus and Alzheimer disease groups. The z-axial expansion of the lateral ventricle and compression of the brain just above the ventricle were the common findings in the parameters for differentiating

Angle closure glaucoma in congenital ectropion uvea.

Science.gov (United States)

Wang, Grace M; Thuente, Daniel; Bohnsack, Brenda L

2018-06-01

Congenital ectropion uvea is a rare anomaly, which is associated with open, but dysplastic iridocorneal angles that cause childhood glaucoma. Herein, we present 3 cases of angle-closure glaucoma in children with congenital ectropion uvea. Three children were initially diagnosed with unilateral glaucoma secondary to congenital ectropion uvea at 7, 8 and 13 years of age. The three cases showed 360° of ectropion uvea and iris stromal atrophy in the affected eye. In one case, we have photographic documentation of progression to complete angle closure, which necessitated placement of a glaucoma drainage device 3 years after combined trabeculotomy and trabeculectomy. The 2 other cases, which presented as complete angle closure, also underwent glaucoma drainage device implantation. All three cases had early glaucoma drainage device encapsulation (within 4 months) and required additional surgery (cycloablation or trabeculectomy). Congenital ectropion uvea can be associated with angle-closure glaucoma, and placement of glaucoma drainage devices in all 3 of our cases showed early failure due to plate encapsulation. Glaucoma in congenital ectropion uvea requires attention to angle configuration and often requires multiple surgeries to obtain intraocular pressure control.

Experimental hydrocephalus following mechanical increment of intraventricular pulse pressure.

Science.gov (United States)

Di Rocco, C; Pettorossi, V E; Caldarelli, M; Mancinelli, R; Velardi, F

1977-11-15

Experimental hydrocephalus has been induced in lambs by artificial increase of the amplitude of intraventricular cerebrospinal fluid (CSF) oscillations related to arterial pulsations, without concomitant changes of the mean CSF-pressure. The characteristics of this hydrocephalus demonstrate that the intraventricular CSF-pulsations can play a role in the genesis of ventricular dilation. Such a method may be used to produce an original model of hydrocephalus independent of changes of CSF-circulation or absorption.

Mechanisms of Hydrocephalus after Neonatal and Adult Intraventricular Hemorrhage

OpenAIRE

Strahle, Jennifer; Garton, Hugh J.L.; Maher, Cormac O.; Muraszko, Karin M.; Keep, Richard F.; Xi, Guohua

2012-01-01

Intraventricular hemorrhage (IVH) is a cause of significant morbidity and mortality and is an independent predictor of a worse outcome in intracerebral hemorrhage (ICH) and germinal matrix hemorrhage (GMH). IVH may result in both injuries to the brain as well as hydrocephalus. This paper reviews evidence on the mechanisms and potential treatments for IVH-induced hydrocephalus. One frequently cited theory to explain hydrocephalus after IVH involves obliteration of the arachnoid villi by microt...

Helical CT of congenital ossicular anomalies

International Nuclear Information System (INIS)

Osada, Hisato; Machida, Kikuo; Honda, Norinari

2001-01-01

Since January 1996 to December 2000, 26 cases of congenital ossicular anomaly could be diagnosed with helical CT. All cases were unilateral. In 8 patients with malformation of the external ear, CT showed malleoincudal fixation (n=5), malleoincudal fixation and deformed incuts long process (n=1), deformed incus long process (n=1), and partial fusion of malleus neck and incus long process (n=1). In 18 patients with normal external ear, CT showed defect of the incus long process (n=5), defect of both the incus long process and stapes superstructure (n=8, 2 patients with congenital cholesteatoma, 1 with hypoplastic oval window), defect of the stapes superstructure (n=2, 1 patient with oval window absence), defect of the malleus manubrium (n=1), ossification of the stampede's tendon (n=1), and monopod stapes (n=1). Helical CT can evaluate the auditory ossicular chain in detail and is useful for diagnosing congenital ossicular anomaly. (author)

Communicating hydrocephalus due to cerebral venous sinus thrombosis treated with ventriculoperitoneal shunt

Directory of Open Access Journals (Sweden)

Rahul T Chakor

2012-01-01

Full Text Available Cerebral venous sinus thrombosis (CVT is a rare cerebrovascular disease with variable presentation. CVT rarely causes hydrocephalus. Communicating hydrocephalus due to CVT is extremely rare. We describe a patient of CVT presenting with chronic headache and communicating hydrocephalus. The patient was successfully treated with ventriculoperitoneal (VP shunt. A 40 year old man presented with moderate to severe headache since six months and progressive visual loss since two months. Head Computed tomogram showed mild hydrocephalus without obstruction. Lumbar puncture (LP demonstrated elevated pressure but was otherwise normal. Magnetic resonance venogram showed extensive CVT. Repeated CSF drainage and thecoperitoneal shunt did not relieve the severe headache hence a VP shunt was placed. Post shunt headache subsided with resolution of hydrocephalus. CVT can present as communicating hydrocephalus. Gradual reduction of intra-ventricular pressure by repeated LPs followed by VP shunt can safely treat hydrocephalus due to CVT.

Low-Field Magnetic Resonance Imaging of Canine Hydrocephalus

Directory of Open Access Journals (Sweden)

Z. Adamiak* and M. Jaskólska and A. Pomianowski1

2012-01-01

Full Text Available The aim of presented study was to evaluate selected surface spine coil, and low-field magnetic resonance (MR selected sequences in diagnosing hydrocephalus in dogs. This paper discusses 19 dogs (14 canine patients with hydrocephalus and 5 healthy dogs, of five breeds, subjected to low-field magnetic resonance imaging (MRI of hydrocephalus. Area of the lateral ventricles and brain were examined in dogs with hydrocephalus using low-field MRI (at 0.25 Tesla. The MRI of FSE REL, SE, FLAIR, STIR, 3D HYCE, T3DT1, GE STIR 3D and 3D SHARC sequences with an indication of the most effective sequences are described. Additionally, coils for MR were compared, and models for infusion anesthesia were described. As a result of performed study all estimated sequences were diagnostically useful. However, spinal coil No. 2 (ESAOTE was the most optimal for examining and positioning the cranium.

Childhood hydrocephalus – is radiological morphology associated with etiology

DEFF Research Database (Denmark)

Foss-Skiftesvik, Jon; Andresen, Morten; Juhler, Marianne

2013-01-01

Clinicians use a non-standardized, intuitive approach when correlating radiological morphology and etiology of hydrocephalus.......Clinicians use a non-standardized, intuitive approach when correlating radiological morphology and etiology of hydrocephalus....

Cerebral hemodynamics and functional prognosis in hydrocephalus

International Nuclear Information System (INIS)

Hirai, Osamu; Nishikawa, Michio; Watanabe, Shu; Yamakawa, Hiroyasu; Kinoshita, Yoshimasa; Uno, Akira; Handa, Hajime

1989-01-01

The functional outcome of cerebral hemodynamics in the chronic stage of juvenile hydrocephalus was determined using single photon emission computed tomography (SPECT). Five patients including three with aqueductal stenosis, one with post-meningitic hydrocephalus, and one case with hydrocephalus having developed after repair of a huge occipital encephalocele. Early images of cerebral blood flow (CBF) were obtained 25 minutes after intravenous injection of 123-I-iodoamphetamine (IMP), and late images were scanned 3 hours later. Cerebral blood volume (CBV) was also measured using 99m Tc in three patients. Twenty cases with adult communicating hydrocephalus were also investigated from the point of view of shunt effectiveness. Although there was no remarkable change in the cerebrovascular bed in the juvenile cases, CBF of the remnant brain parenchyma was good irrespective of the degree of ventricular dilatation. There was a periventricular-related IMP uptake in each case; however, it somehow matched the ventricular span. Functional outcome one to 23 years after the initial shunt operation was good in every case, despite multiple shunt revisions. Redistribution on late images had no bearing on clinical states. In adult cases, 8 patients with effective shunting demonstrated a relatively localized periventricular low perfusion, with preoperative increased cerebrospinal fluid (CSF) pressure. On the contrary, 12 patients with no improvement with or without ventricular-reduced IMP uptake, despite low CSF pressure. The present study indicates that periventricular hemodynamics may play an important role in cerebral function compromised by hydrocephalus. (J.P.N.)

Cerebral hemodynamics and functional prognosis in hydrocephalus

Energy Technology Data Exchange (ETDEWEB)

Hirai, Osamu; Nishikawa, Michio; Watanabe, Shu; Yamakawa, Hiroyasu; Kinoshita, Yoshimasa; Uno, Akira; Handa, Hajime (Hamamatsu Rosai Hospital, Shizuoka (Japan))

1989-11-01

The functional outcome of cerebral hemodynamics in the chronic stage of juvenile hydrocephalus was determined using single photon emission computed tomography (SPECT). Five patients including three with aqueductal stenosis, one with post-meningitic hydrocephalus, and one case with hydrocephalus having developed after repair of a huge occipital encephalocele. Early images of cerebral blood flow (CBF) were obtained 25 minutes after intravenous injection of 123-I-iodoamphetamine (IMP), and late images were scanned 3 hours later. Cerebral blood volume (CBV) was also measured using {sup 99m}Tc in three patients. Twenty cases with adult communicating hydrocephalus were also investigated from the point of view of shunt effectiveness. Although there was no remarkable change in the cerebrovascular bed in the juvenile cases, CBF of the remnant brain parenchyma was good irrespective of the degree of ventricular dilatation. There was a periventricular-related IMP uptake in each case; however, it somehow matched the ventricular span. Functional outcome one to 23 years after the initial shunt operation was good in every case, despite multiple shunt revisions. Redistribution on late images had no bearing on clinical states. In adult cases, 8 patients with effective shunting demonstrated a relatively localized periventricular low perfusion, with preoperative increased cerebrospinal fluid (CSF) pressure. On the contrary, 12 patients with no improvement with or without ventricular-reduced IMP uptake, despite low CSF pressure. The present study indicates that periventricular hemodynamics may play an important role in cerebral function compromised by hydrocephalus. (J.P.N.).

Congenital anomalies of the pulmonary arteries: spectrum of findings on computed tomography.

Science.gov (United States)

Bueno, J; Flors, L; Mejía, M

Congenital anomalies of the pulmonary arteries are uncommon. They can occur in isolation or in association with congenital heart defects. Isolated congenital anomalies remain undiscovered until they are reported as incidental findings on imaging tests, usually not until adolescence. We review the embryological development and normal anatomy of the pulmonary arteries as well as the spectrum of computed tomography findings for various congenital anomalies: unilateral interruption of the pulmonary artery, anomalous origin of the left pulmonary artery (pulmonary artery sling), idiopathic aneurysm of the pulmonary artery, and other anomalies associated with congenital heart defects. Congenital anomalies of the pulmonary arteries represent a diagnostic challenge for clinicians and radiologists. Computed tomography is useful for their diagnosis, and general radiologists need to be familiar with their imaging appearance because they are often discovered incidentally. Copyright © 2016 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

Meaning Construction and Integration in Children with Hydrocephalus

Science.gov (United States)

Barnes, Marcia A.; Faulkner, Heather; Wilkinson, Margaret; Dennis, Maureen

2004-01-01

Text comprehension processes were investigated in children with hydrocephalus, a neurodevelopmental disorder associated with good word decoding, but deficient reading comprehension. In Experiment 1, hydrocephalus and control groups were similar in processes related to activating word meanings and using context to enhance meaning. The hydrocephalus…

Unilateral hyperplasia of the left posterior arch and associated vertebral schisis at C6 level

Energy Technology Data Exchange (ETDEWEB)

Esposito, Giuseppe; Bonis, Pasquale de; Tamburrini, Gianpiero; Massimi, Luca; Rocco, Concezio di [Catholic University, School of Medicine, Department of Pediatric Neurosurgery, Rome (Italy); Byvaltsev, Vadim [Irkutsk Railway Clinical Hospital, Department of Neurosurgery, Irkutsk (Russian Federation); Leone, Antonio [Catholic University, School of Medicine, Department of Bioimaging and Radiological Sciences, Rome (Italy)

2009-12-15

We report on a 5-year-old girl with unilateral hyperplasia of the left posterior arch of C6 associated with spina bifida occulta at the same level. Anteroposterior and lateral radiographs of the cervical spine showed hypertrophy of the left lamina as well as overgrowth and elongation of the left spinous process of the sixth cervical vertebra. Computed tomography (CT) examination better depicted this congenital variant and clearly showed the associated schisis of the posterior arch at the same level. Magnetic resonance (MR) imaging examination ruled out other spinal anomalies. The neck pain, the young age of the patient and the local aesthetic abnormality contributed to the surgical indication. To the best of our knowledge, this is the first case in the English literature of unilateral hyperplasia of a posterior cervical arch. Only one previous study has reported a similar congenital anomaly, but it was limited to the left side of the spinous process. (orig.)

Unilateral hyperplasia of the left posterior arch and associated vertebral schisis at C6 level

International Nuclear Information System (INIS)

Esposito, Giuseppe; Bonis, Pasquale de; Tamburrini, Gianpiero; Massimi, Luca; Rocco, Concezio di; Byvaltsev, Vadim; Leone, Antonio

2009-01-01

We report on a 5-year-old girl with unilateral hyperplasia of the left posterior arch of C6 associated with spina bifida occulta at the same level. Anteroposterior and lateral radiographs of the cervical spine showed hypertrophy of the left lamina as well as overgrowth and elongation of the left spinous process of the sixth cervical vertebra. Computed tomography (CT) examination better depicted this congenital variant and clearly showed the associated schisis of the posterior arch at the same level. Magnetic resonance (MR) imaging examination ruled out other spinal anomalies. The neck pain, the young age of the patient and the local aesthetic abnormality contributed to the surgical indication. To the best of our knowledge, this is the first case in the English literature of unilateral hyperplasia of a posterior cervical arch. Only one previous study has reported a similar congenital anomaly, but it was limited to the left side of the spinous process. (orig.)

Disturbance of cerebral neuronal migration following congenital parvovirus B19 infection

NARCIS (Netherlands)

Pistorius, Lourens Rasmus; Smal, Jaime; de Haan, Timo Robert; Page-Christiaens, Godelieve C. M. L.; Verboon-Maciolek, Malgorzata; Oepkes, Dick; de Vries, Linda S.

2008-01-01

We describe the clinical course of an infant who presented with severe fetal anemia and fetal hydrops following congenital parvovirus B19 infection before 16 gestational weeks. The fetus was treated by cordocentesis and intrauterine transfusion at 18 weeks. The infant demonstrated mild unilateral

Congenital Becker's nevus with a familial association.

Science.gov (United States)

Book, S E; Glass, A T; Laude, T A

1997-01-01

Becker's nevus is a unilateral, hyperpigmented cutaneous hamartoma usually with hypertrichosis. It occurs predominantly in boys, becoming apparent during adolescence, although several cases of congenital Becker's nevus have been reported. Rarely it may be familial and as such is transmitted in an autosomal dominant pattern. We report a 16-month-old black boy with a hyperpigmented patch on his right shoulder and upper pectoral area that extended down his arm. The patient's father has a similar lesion with hair on his left shoulder which has been present since childhood. Histology of the child's lesion was consistent with Becker's nevus. We believe this to be the first reported case of a congenital Becker's nevus with a familial association.

Huge hydrocephalus: definition, management, and complications.

Science.gov (United States)

Faghih Jouibari, Morteza; Baradaran, Nazanin; Shams Amiri, Rouzbeh; Nejat, Farideh; El Khashab, Mostafa

2011-01-01

Lack of comprehensive knowledge and numerous socioeconomic problems may make the parents leave hydrocephalic children untreated, leading to progressive hydrocephalus and eventual unordinary big head. Management of huge hydrocephalus (HH) differs from common hydrocephalus. We present our experience in the management of these children. HH is defined as head circumference larger than the height of the infant. Nine infants with HH have been shunted in Children's Hospital Medical Center and followed up for 0.5 to 7 years. The most common cause of hydrocephalus was aqueductal stenosis. The mean age of patients during shunting was 3 months. The head circumference ranged from 56 to 94 cm with the average of 67 cm. Cognitive statuses were appropriate based on their age in five patients. Motor development was normal only in one patient. Complications were found in most cases which included subdural effusion (six patients), shunt infection (four patients), skin injury (three patients), proximal catheter coming out of ventricle to the subdural space (two patients), and shunt exposure (one patient). Three patients died due to shunt infection and sepsis. Numerous complications may occur in patients with HH after shunt operation such as subdural effusion, ventricular collapse, electrolyte disturbance, skull deformity, scalp injury, and shunt infection. Mental and motor disabilities are very common in patients with HH. Many of these complications can be related to overdrainage; therefore, drainage control using programmable shunts is advisable.

CT manifestation of congenital toxoplasmosis infection of the brain (report of 42 cases)

International Nuclear Information System (INIS)

Wang Zhenyu; Li Shuxin; Feng Kun

1997-01-01

To improve the recognition and diagnosis of congenital toxoplasmosis infection of the brain, forty-two cases of congenital toxoplasmosis infection of the brain verified by serological tests and initially investigated by CT were retrospectively studied. The main diagnostic feature of the entity included: (1) Widely scattered small nodular or curvilinear calcifications involving the basal ganglia, subependymal region and the frontal or parietal lobes; (2) Small patches of low density foci located at the paraventricular and gray-white matter junction area with some enhancement surrounding the foci after contrast media administration; (3) Evidence of obstructive hydrocephalus and (4) Complications of CNS malformation or developmental problems. Conclusion: CT was one of the best methods for the diagnosis of this entity, however, it should be closely correlated with the results from serological tests

Management and functional outcome of childhood hydrocephalus at ...

African Journals Online (AJOL)

Background: Hydrocephalus is a pathological condition caused by abnormalities of production or absorption of CSF in the brain. The goal of this study was to evaluate the current management and outcome of children (aged 13 years and below) who were treated for hydrocephalus in a single institution in Kenya. Methods: A ...

Comparison between the incidence of right and left sided congenital torticollis

International Nuclear Information System (INIS)

Aslam, S.; Bashir, M.S.; Hussain, S.I.

2013-01-01

Congenital torticollis is an intriguing condition of unknown origin, characterized by unilateral shortening and tightness of the sternocleidomastoid muscle. Patients usually present with head tilt, facial asymmetry and plagiocephaly. A sternomastoid mass or tumor may or may not be clinically apparent. Untreated, cervical function and facial cosmesis may be severely compromised. Objective: My study is aimed at establishing a comparison between the incidence of right versus left sided congenital torticollis. Method: This observational study included 30 patients of congenital torticollis that completed the questionnaire. The data was collected from patients coming to the Physiotherapy and Orthopedic departments of Children Hospital, Lahore. Results: Results showed that right side was involved in 19 (63.3%) patients and left side was involved in 11 (36.7%) patients. Out of 30 patients, 14 (46.7%) were male, of which 8 had right sided congenital torticollis and 6 had left sided congenital torticollis, and 16 (53.3%) were female, of which right sided congenital torticollis and 5 had left sided congenital torticollis. Conclusion: Hence it is concluded that incidence of right sided congenital torticollis is more common than left sided congenital torticollis. The incidence of con-genital torticollis is higher in females than in males. (author)

Long-term hydrocephalus alters the cytoarchitecture of the adult subventricular zone

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Campos-Ordoñez, Tania; Herranz-Pérez, Vicente; Chaichana, Kaisorn L.; Rincon-Torroella, Jordina; Rigamonti, Daniele; García-Verdugo, Jose M.; Quiñones-Hinojosa, Alfredo; Gonzalez-Perez, Oscar

2014-01-01

Hydrocephalus can develop secondarily to a disturbance in production, flow and/or absorption of cerebrospinal fluid. Experimental models of hydrocephalus, especially subacute and chronic hydrocephalus, are few and limited, and the effects of hydrocephalus on the subventricular zone are unclear. The aim of this study was to analyze the effects of long-term obstructive hydrocephalus on the subventricular zone, which is the neurogenic niche lining the lateral ventricles. We developed a new method to induce hydrocephalus by obstructing the aqueduct of Sylvius in the mouse brain, thus simulating aqueductal stenosis in humans. In 120-day-old rodents (n = 18 per group), the degree of ventricular dilatation and cellular composition of the subventricular zone were studied by immunofluorescence and transmission electron microscopy. In adult patients (age > 18 years), the sizes of the subventricular zone, corpus callosum, and internal capsule were analyzed by magnetic resonance images obtained from patients with and without aqueductal stenosis (n=25 per group). Mice with 60-day hydrocephalus had a reduced number of Ki67+ and doublecortin+ cells on immunofluorescence, as well as decreased number of neural progenitors and neuroblasts in the subventricular zone on electron microscopy analysis as compared to non-hydrocephalic mice. Remarkably, a number of extracellular matrix structures (fractones) contacting the ventricular lumen and blood vessels were also observed around the subventricular zone in mice with hydrocephalus. In humans, the widths of the subventricular zone, corpus callosum, and internal capsule in patients with aqueductal stenosis were significantly smaller than age and gender-matched patients without aqueductal stenosis. In summary, supratentorial hydrocephalus reduces the proliferation rate of neural progenitors and modifies the cytoarchitecture and extracellular matrix compounds of the subventricular zone. In humans, this similar process reduces the

Predictors and outcomes of shunt-dependent hydrocephalus in patients with aneurysmal sub-arachnoid hemorrhage

Science.gov (United States)

2012-01-01

Background Hydrocephalus following spontaneous aneurysmal sub-arachnoid hemorrhage (SAH) is often associated with unfavorable outcome. This study aimed to determine the potential risk factors and outcomes of shunt-dependent hydrocephalus in aneurysmal SAH patients but without hydrocephalus upon arrival at the hospital. Methods One hundred and sixty-eight aneurysmal SAH patients were evaluated. Using functional scores, those without hydrocephalus upon arrival at the hospital were compared to those already with hydrocephalus on admission, those who developed it during hospitalization, and those who did not develop it throughout their hospital stay. The Glasgow Coma Score, modified Fisher SAH grade, and World Federation of Neurosurgical Societies grade were determined at the emergency room. Therapeutic outcomes immediately after discharge and 18 months after were assessed using the Glasgow Outcome Score. Results Hydrocephalus accounted for 61.9% (104/168) of all episodes, including 82 with initial hydrocephalus on admission and 22 with subsequent hydrocephalus. Both the presence of intra-ventricular hemorrhage on admission and post-operative intra-cerebral hemorrhage were independently associated with shunt-dependent hydrocephalus in patients without hydrocephalus on admission. After a minimum 1.5 years of follow-up, the mean Glasgow outcome score was 3.33 ± 1.40 for patients with shunt-dependent hydrocephalus and 4.21 ± 1.19 for those without. Conclusions The presence of intra-ventricular hemorrhage, lower mean Glasgow Coma Scale score, and higher mean scores of the modified Fisher SAH and World Federation of Neurosurgical grading on admission imply risk of shunt-dependent hydrocephalus in patients without initial hydrocephalus. These patients have worse short- and long-term outcomes and longer hospitalization. PMID:22765765

Risk factors for hydrocephalus and neurological deficit in children born with an encephalocele.

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Da Silva, Stephanie L; Jeelani, Yasser; Dang, Ha; Krieger, Mark D; McComb, J Gordon

2015-04-01

There is a known association of hydrocephalus with encephaloceles. Risk factors for hydrocephalus and neurological deficit were ascertained in a series of patients born with an encephalocele. A retrospective analysis was undertaken of patients treated for encephaloceles at Children's Hospital Los Angeles between 1994 and 2012. The following factors were evaluated for their prognostic value: age at presentation, sex, location of encephalocele, size, contents, microcephaly, presence of hydrocephalus, CSF leak, associated cranial anomalies, and neurological outcome. Seventy children were identified, including 38 girls and 32 boys. The median age at presentation was 2 months. The mean follow-up duration was 3.7 years. Encephalocele location was classified as anterior (n = 14) or posterior (n = 56) to the coronal suture. The average maximum encephalocele diameter was 4 cm (range 0.5-23 cm). Forty-seven encephaloceles contained neural tissue. Eight infants presented at birth with CSF leaking from the encephalocele, with 1 being infected. Six patients presented with hydrocephalus, while 11 developed progressive hydrocephalus postoperatively. On univariate analysis, the presence of neural tissue, cranial anomalies, encephalocele size of at least 2 cm, seizure disorder, and microcephaly were each positively associated with hydrocephalus. On multivariate logistic regression modeling, the single prognostic factor for hydrocephalus of borderline statistical significance was the presence of neural tissue (odds ratio [OR] = 5.8, 95% confidence interval [CI] = 0.8-74.0). Fourteen patients had severe developmental delay, 28 had mild/moderate delay, and 28 were neurologically normal. On univariate analysis, the presence of cranial anomalies, larger size of encephalocele, hydrocephalus, and microcephaly were positively associated with neurological deficit. In the multivariable model, the only statistically significant prognostic factor for neurological deficit was presence of

Frequency of Congenital Anomalies in Newborns and Its Relation to Maternal Health in a Tertiary Care Hospital in Peshawar, Pakistan

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Adnan Khan

2015-03-01

Full Text Available Background: Congenital anomalies are a major cause of perinatal and neonatal deaths, both in low- and high-income countries. They are relatively common worldwide, affecting 3% to 5% of live births. Methods: A cross-sectional study was conducted from January 2014 to June 2014 at the Khyber teaching hospital in Peshawar. Specific patient information was obtained from patient records at the beginning of the study. Those individuals found to have at least one birth defect were approached and their attendants (mothers were interviewed. Information regarding various risk factors was collected. Descriptive analyses were carried out. Results: Out of 1062 deliveries, 2.9% (31 of newborns had various congenital anomalies. Hydrocephalus (22.6%, anencephaly (12.9%, and spina bifida (9.7% were major anomalies. The maternal age ranged from 18 years to 46 years (mean: 30 ± 8. Most of the anomalies (35.5% were present in the 26-30 years age group. Out of 31 babies, 6.4% had multiple anomalies. The preponderance of various congenital anomalies was seen in parity 1 (35.4%; parities 2 to 4 had lower incidences (35.4%. The consanguinity rate was 67.7%; only 32.3% of patients were using folic acid. History of passive smoking was positive in 16.1% of cases. Conclusion: Anencephaly and hydrocephalus were the most prominent anomaly detected; early prenatal diagnosis may be helpful in decreasing mortality by offering early termination. Low intake of folic acid and a high consanguinity rate were the most common associated risk factors for congenital anomalies. These risk factors may be reduced by creating awareness regarding the avoidance of consanguineous marriage and promoting the use of folic acid during pregnancy.

Treatment of Class II subdivision malocclusion with congenitally missing upper lateral incisors: A case report

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Siddharth Mehta; Ashima Valiathan; Arun Urala

2014-01-01

Orthodontic treatment for patients with unilateral or bilateral congenitally missing lateral incisor poses a challenge mainly with regard to treatment planning. The use of a diagnostic setup is one of the most important aids in the decision-making process. Two alternatives, orthodontic space closure or space opening for prosthetic replacement exist. The present case report shows use of the microimplant for unilateral upper molar distalization and space closure in a Class-II division 1 subdivi...

Treatment of posthemorrhagic hydrocephalus in premature infants with subcutaneous reservoir drainage.

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Yu, Bo; Li, Shasha; Lin, Zhenlang; Zhang, Nu

2009-01-01

To investigate the effectiveness of subcutaneous reservoir drainage as a treatment for the different types of posthemorrhagic hydrocephalus in premature infants. 11 premature infants with posthemorrhagic hydrocephalus underwent subcutaneous reservoir embedment surgery, and cerebrospinal fluid (CSF) was drained via the reservoir intermittently for 8 weeks. During the period of drainage, ultrasound and computerized tomography were used to measure ventricle size. CSF examinations were performed routinely to assess the presence of intraventricular hemorrhage (IVH) and/or infection. (1) Five infants were diagnosed as having obstructive hydrocephalus; 2 had nearly normal ventricle sizes with treatment and drainage was stopped after 8 weeks, 2 had nearly normal ventricle sizes after 4 more weeks of drainage, and 1 underwent ventriculoperitoneal shunt (V-P shunt) surgery due to failure of ventricle size reduction. (2) Six infants were diagnosed as having communicating hydrocephalus; 4 had further enlarged ventricle size after 8 weeks and underwent V-P shunt surgery, 1 had treatment aborted at week 8 of drainage, and only 1 had moderate reduction of ventricle size after 8 weeks. (3) None of the 11 infants had progressive IVH and/or intracranial infection during drainage. Subcutaneous reservoir drainage is a suitable and safe treatment for posthemorrhagic hydrocephalus in premature infants. It is more effective for obstructive hydrocephalus than for communicating hydrocephalus. Copyright (c) 2009 S. Karger AG, Basel.

Communicating Hydrocephalus Associated with Intracranial Schwannoma Treated by Gamma Knife Radiosurgery.

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Park, Chang Kyu; Lee, Sung Ho; Choi, Man Kyu; Choi, Seok Keun; Park, Bong Jin; Lim, Young Jin

2016-05-01

Gamma knife radiosurgery (GKRS) has been established as an effective and safe treatment for intracranial schwannoma. However, serious complications can occur after GKRS, including hydrocephalus. The pathophysiology and risk factors of this disorder are not yet fully understood. The objective of the study was to assess potential risk factors for hydrocephalus after GKRS. We retrospectively reviewed the medical radiosurgical records of 244 patients who underwent GKRS to treat intracranial schwannoma. The following parameters were analyzed as potential risk factors for hydrocephalus after GKRS: age, sex, target volume, irradiation dose, prior tumor resection, treatment technique, and tumor enhancement pattern. The tumor enhancement pattern was divided into 2 groups: group A (homogeneous enhancement) and group B (heterogeneous or rim enhancement). Of the 244 patients, 14 of them (5.7%) developed communicating hydrocephalus. Communicating hydrocephalus occurred within 2 years after GKRS in most patients (92.8%). No significant association was observed between any of the parameters investigated and the development of hydrocephalus, with the exception of tumor enhancement pattern. Group B exhibited a statistically significant difference by univariate analysis (P = 0.002); this difference was also significant by multivariate analysis (P = 0.006). Because hydrocephalus is curable, patients should be closely monitored for the development of this disorder after GKRS. In particular, patients with intracranial schwannomas with irregular enhancement patterns or cysts should be meticulously observed. Copyright © 2015 Elsevier Inc. All rights reserved.

Unilateral Congenital Knee and Hip Dislocation with Bilateral Clubfoot – A rare Packaging disorder

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Mukesh Tiwari

2013-04-01

Full Text Available ntroduction: Reduced intrauterine space gives rise to ‘packaging disorder’ which may involve joint dislocations or contractures. We present an unique case where mutiple joints were dislocated involving left congenital knee dislocation (CDK, bilateral congenital hip dislocation (CDH and congenital talipes equino varus (CTEVdeformities. Case Report: A preterm baby boy born to mother with diagnosed oligohydramios presented with left CDK bilateral DDH and CTEV. The knee dislocation was treated first with gradual streaching and weekly above knee cast. At 7th week good flexion was achieved at both knees and abduction splint for DDH (using double diaper with ponseti cast for CTEV was done. At one year follow up all joints were reduced and maintained well with baby able to stand with support. Conclusion: Packaging disorders may present with multiple dislocations and deformities. Early intervention with serial casting and manipulation minimises disability and prevents ambulatory problems. In our case there was a good response to manipulation and serial casting. This differs from cases with inherent pathology like arthrogryposis where response to treatment is not so good. Keywords: Congenital genu recurvatum, Develpmental dysplasia hip, CTEV, Clubfoot, serial manipulation, packaging disorders

Simulation of hydrocephalus condition in infant head

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Wijayanti, Erna; Arif, Idam

2014-03-01

Hydrocephalus is a condition of an excessive of cerebrospinal fluid in brain. In this paper, we try to simulate the behavior of hydrocephalus conditions in infant head by using a hydro-elastic model which is combined with orthotropic elastic skull and with the addition of suture that divide the skull into two lobes. The model then gives predictions for the case of stenosis aqueduct by varying the cerebral aqueduct diameter, time constant and brain elastic modulus. The hydrocephalus condition which is shown by the significant value of ventricle displacement, as the result shows, is occurred when the aqueduct is as resistant as brain parenchyma for the flow of cerebrospinal fluid. The decrement of brain elastic modulus causes brain parenchyma displacement value approach ventricle displacement value. The smaller of time constant value causes the smaller value of ventricle displacement.

Two Hundred Thirty-Six Children With Developmental Hydrocephalus: Causes and Clinical Consequences.

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Tully, Hannah M; Ishak, Gisele E; Rue, Tessa C; Dempsey, Jennifer C; Browd, Samuel R; Millen, Kathleen J; Doherty, Dan; Dobyns, William B

2016-03-01

Few systematic assessments of developmental forms of hydrocephalus exist. We reviewed magnetic resonance images (MRIs) and clinical records of patients with infancy-onset hydrocephalus. Among 411 infants, 236 had hydrocephalus with no recognizable extrinsic cause. These children were assigned to 1 of 5 subtypes and compared on the basis of clinical characteristics and developmental and surgical outcomes. At an average age of 5.3 years, 72% of children were walking independently and 87% could eat by mouth; in addition, 18% had epilepsy. Distinct patterns of associated malformations and syndromes were observed within each subtype. On average, children with aqueductal obstruction, cysts, and encephaloceles had worse clinical outcomes than those with other forms of developmental hydrocephalus. Overall, 53% of surgically treated patients experienced at least 1 shunt failure, but hydrocephalus associated with posterior fossa crowding required fewer shunt revisions. We conclude that each subtype of developmental hydrocephalus is associated with distinct clinical characteristics, syndromology, and outcomes, suggesting differences in underlying mechanisms. © The Author(s) 2015.

Preventricular lucency in computerized tomography of various forms of hydrocephalus

International Nuclear Information System (INIS)

Sprung, C.

1981-01-01

602 patients with 8 different types of hydrocephalus have been examined for PVL on the basis of 18,413 CT pictures. The localisation of PVL, in the periventricular white matter of the auterior horns, and its configuration are independent of the type of hydrocephalus and of the intraventricular pressure. In contrast, the intensity and incidence of PVL are a function of the type of hydrocephalus. Factors influencing these two parameters are in order of importance: The intraventricular pressure, the acuteness of the process leading to stenosis (i.e. the rate of development of hydrocephalus), the extent of ventricular enlargement, the compliance of ventricular wall and osseous part of the skull, the volume of the ventricles, and the localisation of the obstructing and displacing process. Independent of the type of hydrocephalus, disappearance of PVL is always well correlated with improved clinical symptoms and, in most cases, also with a reduction of the ventricular volume. As far as the pathomechanism is concerned, the studies suggest passive inflow of liquor through the damaged ependyma rather than an active absorption process. (orig./MG) [de

Idiopathic Normal Pressure Hydrocephalus

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Basant R. Nassar BS

2016-04-01

Full Text Available Idiopathic normal pressure hydrocephalus (iNPH is a potentially reversible neurodegenerative disease commonly characterized by a triad of dementia, gait, and urinary disturbance. Advancements in diagnosis and treatment have aided in properly identifying and improving symptoms in patients. However, a large proportion of iNPH patients remain either undiagnosed or misdiagnosed. Using PubMed search engine of keywords “normal pressure hydrocephalus,” “diagnosis,” “shunt treatment,” “biomarkers,” “gait disturbances,” “cognitive function,” “neuropsychology,” “imaging,” and “pathogenesis,” articles were obtained for this review. The majority of the articles were retrieved from the past 10 years. The purpose of this review article is to aid general practitioners in further understanding current findings on the pathogenesis, diagnosis, and treatment of iNPH.

Communicating hydrocephalus subsequent to purulent meningitis

International Nuclear Information System (INIS)

Suzuki, Hiroshi; Ogawa, Kimio; Hibio, Shuichi; Goto, Kazuhiko; Shiihara, Hiroaki

1984-01-01

Based on CT findings one year after shunting, ventricular dialtion was classified into five degrees for examining prognosis of communicating hydrocephalus subsequent to purulent meningitis. Factors causing and aggravating hydrocephalus were also examined. Patients with hydrocephalus tended to have spasms frequently as the first symptom within one month after birth when there were few characteristic findings. Spasm and disturbance of consciousness occurred frequently during the first week of the occurrence of disease. Large numbers of cells in the spinal fluid and high volume of spinal cord protein were persistent in patients aged one month or less. Chloride transport decreased in patients aged two months or more. The occurrence of syndrome of the pyramidal tract, eye symptoms, movement of head to the left and right, and involuntary movement suggested serious conditions of the disease. Disturbance of movement could be relieved by giving adequate antibiotics as soon as meningitis was discovered within one month after birth and by giving chloramphenicol when symptoms suggesting the development of serious conditions occurred. However, mental retardation and epilepsy could not be prevented. (Namekawa, K.)

Clinical and Surgical Factors Associated With Increased Epilepsy Risk in Children With Hydrocephalus.

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Tully, Hannah M; Kukull, Walter A; Mueller, Beth A

2016-06-01

Children with hydrocephalus are at risk for epilepsy both due to their underlying condition and as a consequence of surgical treatment; however, the relative contributions of these factors remain unknown. The authors sought to characterize epilepsy among children with infancy-onset hydrocephalus and to examine the risks of epilepsy associated with hydrocephalus subtype and with factors related to surgical treatment. We conducted a longitudinal cohort study of all children with infancy-onset hydrocephalus treated at a major regional children's hospital during 2002 to 2012, with follow-up to ascertain risk factors and epilepsy outcome through April 2015. Poisson regression was used to calculate adjusted risk ratios and 95% confidence intervals for associations. Among 379 children with hydrocephalus, 86 (23%) developed epilepsy (mean onset age = 2.7 years), almost one fifth of whom had a history of infantile spasms. Relative to spina bifida-associated hydrocephalus, children with other major hydrocephalus subtypes had fourfold higher risks of developing epilepsy. Among children who underwent surgery, surgical infection doubled the risk of epilepsy (risk ratio = 2.0, 95% confidence interval = 1.4 to 3.0). Epilepsy was associated with surgical failure for intracranial reasons but not extracranial reasons (risk ratio = 1.7, 95% confidence interval = 1.1 to 2.7; risk ratio = 1.1, 95% confidence interval = 0.7 to 1.9, respectively). Epilepsy is common among children with hydrocephalus. Compared with children with spina bifida-associated hydrocephalus, children with other major hydrocephalus subtypes have a markedly increased risk of epilepsy. Surgical infection doubles the risk of epilepsy. Copyright © 2016 Elsevier Inc. All rights reserved.

Unilateral fusion of the odontoid process with the atlas in Klippel-Feil syndrome: a case report

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Park, So Young; Ryu, Kyung Nam; Park, Ji Seon; Suk, Kyung Soo; Han, Mi Young [Kyunghee Medical Center, Seoul (Korea, Republic of)

2006-07-15

Klippel-Feil syndrome (KFS) displays congenital fusion of the cervical vertebrae; it is a relatively common condition and has many associated malformations such as Sprengel's deformity, scoliosis, rib anomalies, congenital defects of the brain or spinal cord, renal anomalies, congenital heart disease, deafness, cleft palate, cranial and facial asymmetry, and enteric cysts. There are various types of cervical fusion observed in KFS. However, fusion of the odontoid process with the atlas is a very rare finding. We report here on a 4-year-old boy with unilateral fusion of a separated odontoid process with the lateral mass of the atlas, and this was associated with a spontaneously closed ventricular septal defect, a small patent ductus arteriosus and a horseshoe kidney.

Technology preferences among caregivers of children with hydrocephalus.

Science.gov (United States)

Naftel, Robert P; Safiano, Nicole A; Falola, Michael I; Shannon, Chevis N; Wellons, John C; Johnston, James M

2013-01-01

The Internet and social media are powerful disseminators of medical information, providing new portals for patient care. The authors of this study evaluated current technology hardware, Internet, and social media use and their socioeconomic relationships among caregivers of children with hydrocephalus. A written survey was completed in the neurosurgical clinics at the University of Alabama at Birmingham by 300 parents of children with shunted hydrocephalus between October 26, 2010, and July 26, 2011. Computer use (94.6%), Internet use (91.7%), smartphone use (56.9%), and Internet research on hydrocephalus (81.9%) were prevalent. However, for each of these four utilizations there was significantly lower access by caregivers of minority races (p = 0.04, 0.03, 0.002, and children with similar conditions. Technology hardware, the Internet, and social media are widely used with some skepticism by parents of children with shunted hydrocephalus. Caregivers are interested in physician-recommended Internet resources. Socioeconomic factors including race, income, and level of education reveal a disparity in access to some of these resources, although all groups have relatively high use. Unlike typical technology use, social media use is breaking down the digital divide among ethnic and socioeconomic groups.

Incidence of environmental and genetic factors causing congenital cataract in Children of Lahore.

Science.gov (United States)

Naz, Shagufta; Sharif, Saima; Badar, Hafsa; Rashid, Farzana; Kaleem, Afshan; Iqtedar, Mehwish

2016-07-01

To check the incidence of environmental and genetic factors causing congenital cataract in infants. The descriptive study was conducted at Layton Rahmatullah Benevolent Trust, Lahore, Pakistan, from October 2013 to April 2014, and comprised children under 15 years of age who had rubella syndrome, herpes simplex, birth trauma, trisomy 21, Nance-Horan syndrome or Lowe's syndrome. Of the 38,000 cases examined, 120(0.3%) patients were diagnosed with congenital cataract. Of them, 52(43.33%)were aged between 2 and 5 years,22(18.33%) <11 years and 10(8.33%) ?15 years. Bilateral congenital cataract was observed in 91(75.83%) patients and unilateral congenital cataract in 29(24.17%). Environmental factors caused 72(62.07%) cases and genetic factors caused 44(37.93%).. Congenital cataract predominated in boys compared to girls. Early diagnosis and adequate therapy requires specific technology, as well as long-term and permanent care..

The influence of decompressive craniectomy on the development of hydrocephalus: a review

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Jun Ding

2014-09-01

Full Text Available Decompressive craniectomy (DC is widely used to treat intracranial hypertension following traumatic brain injury (TBI or cerebral vascular disease. Many studies have discussed complications of this procedure, and hydrocephalus is a common complication of DC. To further evaluate the relationship between DC and hydrocephalus, a review of the literature was performed. Numerous complications may arise after DC, including contusion or hematoma expansion, epilepsy, herniation of the cortex through a bone defect, CSF leakage through the scalp incision, infection, subdural effusion, hydrocephalus and “syndrome of the trephined”. Several hydrocephalus predictors were identified; these included DC, distance from the midline, hygroma, age, injury severity, subarachnoid or intraventricular hemorrhage, delayed time to craniotomy, repeated operation, and duraplasity. However, results differed among studies. The impact of DC on hydrocephalus remains controversial.

A case of congenital corneal keloid.

Science.gov (United States)

Song, Jong-Suk; Kwon, Sangwon; Shyn, Kyung-Hwan

2005-06-01

To describe a case of unilateral comeal keloid and present the clinical and histopathological findings and the management. A 23-year-old Asian male patient was examined for a white spot on the left cornea that had been present since birth. On biomicroscopic examination, a well-demarcated vascularized comeal mass was found located nasal to the center. The pupil was displaced superiorly, and gonioscopic examination showed peripheral iridocomeal adhesion at 12 o'clock. The patient underwent penetrating keratoplasty. Histopathologic study showed a variously thickened epithelial layer, an absence of Bowman's layer, subepithelial fibrovascular hyperplasia, and an absence of dermal elements. These histopathologic findings suggested a congenital comeal keloid. The central graft comea remained clear at 18 months after surgery and the patient was satisfied with the result. Penetrating keratoplasty may be an effective surgical option for congenital keloids in young adult patients.

Tetraventricular hydrocephalus due to ruptured intracranial dermoid cyst

International Nuclear Information System (INIS)

Karabulut, N.; Oguzkurt, L.

2000-01-01

We present the MR imaging findings in a patient who developed tetraventricular hydrocephalus after massive rupture of dermoid cysts with generalized subarachnoid and ventricular spread of cyst contents. The development of tetraventricular hydrocephalus due to ruptured dermoid is a very unusual complication, and was attributed to cyst contents interfering with passage of cerebrospinal fluid through the arachnoid granulations and fourth ventricular outlet foramina. (orig.)

Neuroradiological findings in multiloculated hydrocephalus

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Albanese, V.; Tomasello, F.; Sampaolo, S.; Picozzi, P. (Naples Univ. (Italy). Inst. of Neurosurgery)

1982-01-01

The characteristic neuroradiological features of multiloculated hydrocephalus following neonatal meningitis and ventriculitis in five infants are presented. The role of ventriculography and computerized tomography in detecting multiloculated and enlarged ventricles and paraventricular cavities, as well as complete and incomplete septa, is emphasized for early diagnosis and surgical management. CT scan also provides clues indicating the presence of thin but not visualized septa. It is suggested that post-meningitic hydrocephalus should be repeatedly followed-up for detection of multiloculated transformation. Probably this entity occurs less uncommonly than was thought in the past. The CT scan appears the most helpful and safe procedure for the serial evaluation, but ventriculography may be usefully combined with it in order to plan the most suitable operative approach for fenestration of membranes.

Neuroradiological findings in multiloculated hydrocephalus

International Nuclear Information System (INIS)

Albanese, V.; Tomasello, F.; Sampaolo, S.; Picozzi, P.

1982-01-01

The characteristic neuroradiological features of multiloculated hydrocephalus following neonatal meningitis and ventriculitis in five infants are presented. The role of ventriculography and computerized tomography in detecting multiloculated and enlarged ventricles and paraventricular cavities, as well as complete and incomplete septa, is emphasized for early diagnosis and surgical management. CT scan also provides clues indicating the presence of thin but not visualized septa. It is suggested that post-meningitic hydrocephalus should be repeatedly followed-up for detection of multiloculated transformation. Probably this entity occurs less uncommonly than was thought in the past. The CT scan appears the most helpful and safe procedure for the serial evaluation, but ventriculography may be usefully combined with it in order to plan the most suitable operative approach for fenestration of membranes. (Author)

Allelic mutations of KITLG, encoding KIT ligand, cause asymmetric and unilateral hearing loss and Waardenburg syndrome type 2

NARCIS (Netherlands)

Zazo Seco, C. (Celia); Serrão De Castro, L. (Luciana); J.W.I. van Nierop; Morín, M. (Matías); S.N. Jhangiani (Shalini N.); E.J.J. Verver (Eva J. J.); M. Schraders (Margit); Maiwald, N. (Nadine); Wesdorp, M. (Mieke); H. Venselaar (Hanka); L. Spruijt (Liesbeth); Oostrik, J. (Jaap); J. Schoots (Jeroen); J. van Reeuwijk (Jeroen); Lelieveld, S.H. (Stefan H.); P.L.M. Huygen (Patrick); Insenser, M. (María); R.J. Admiraal (Ronald); R.J.E. Pennings (Ronald J.E.); E.H. Hoefsloot (Lies); A. Arias-Vásquez (Alejandro); J. de Ligt (Joep); H.G. Yntema; Jansen, J.H. (Joop H.); D. Muzny (Donna); G. Huls (Gerwin); M.M. van Rossum (Michelle); J.R. Lupski (James R.); Moreno-Pelayo, M.A. (Miguel Angel); H.P.M. Kunst (Henricus P.M.); H. Kremer (Hannie)

2015-01-01

textabstractLinkage analysis combined with whole-exome sequencing in a large family with congenital and stable non-syndromic unilateral and asymmetric hearing loss (NS-UHL/AHL) revealed a heterozygous truncating mutation, c.286-303delinsT (p.Ser96Ter), in KITLG. This mutation co-segregated with

Isolated Unilateral Absent Branch Pulmonary Artery with Peripheral Pulmonary Stenosis and Coronary Artery Disease

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Sunil Abhishek B

2017-09-01

Full Text Available Isolated Unilateral Absent Pulmonary Artery (UAPA is a rare congenital anomaly. It is usually associated with congenital heart defects. A 45 year old male patient presented with complaints of fever with cough and expectoration for 15 days and retrosternal chest discomfort for the previous 2 days. ECG showed diffuse ST segment depression with T wave inversion in the inferior and lateral leads. Coronary Angiogram done through the right femoral approach revealed diffusely diseased Left Anterior Descending (LAD artery that was totally cut off at the mid segment. The Left Circumflex (LCx artery was providing blood supply to the right middle and lower lung areas. There was another collateral arising from the Left Subclavian Artery supplying the right middle and lower lung areas. The left pulmonary artery was normal, but branches supplying the middle and lower lobes of the right lung were absent and the upper lobe branch had pulmonary stenosis. UAPA is a rare clinical entity; collaterals from coronaries are extremely rare in this condition and till now there has not been any case report of unilateral absent branch pulmonary artery with peripheral stenosis of other branches, on the affected side and associated coronary artery disease.

Multiloculated hydrocephalus: a review of current problems in classification and treatment

DEFF Research Database (Denmark)

Andresen, Morten; Juhler, Marianne

2012-01-01

PURPOSE: Loculated hydrocephalus is a condition in which discrete fluid-filled compartments form in or in relation to the ventricular system of the brain. Both uni- and multiloculated variants exist, with marked differences in outcome. However, several competing and seemingly interchangeable...... of Systematic Reviews, and the U.S. NIH ClinicalTrials.gov database was carried out with the search terms: "multicystic," "multiloculated," "multicompartment," "uniloculated," and "loculated." All were used in conjunction with the search term "hydrocephalus." RESULTS: A single study with a control group......, evidence is in favor of the neuroendoscopic approach. CONCLUSIONS: In order to ensure a consistent nomenclature as well as to guide future research, we propose a new system of classification for loculated hydrocephalus. It acknowledges the differences between uniloculated and multiloculated hydrocephalus...

The value of radionuclide cerebrospinal fluid scintigraphy in the diagnosis and treatment of patients with hydrocephalus

International Nuclear Information System (INIS)

Feng Fang; Wang Hui; Fu Hongliang; Li Jianing; Zou Renjian; Gu Zhenhui; Wu Jingchuan

2009-01-01

Objective: Radionuclide cerebrospinal fluid scintigraphy (RCFS) has been applied to make diagnosis of hydrocephalus clinically for a long time. This study aimed to evaluate clinical value of RCFS in guiding the management of patients with hydrocephalus. Methods: All 13 patients with hydrocephalus (8 adults, 5 children) underwent RCFS and CT/MRI examination before treatment and then follow-up imaging after treatment.Results: Four of 7 patients (6 adults, 1 child) diagnosed as obstructive hydrocephalus by RCFS underwent endoscopic third ventriculostomy (ETV), and 5 of 6 patients (2 adults, 4 children) diagnosed as obstructive hydrocephalus underwent ventriculo peritoneal shunt (VPS). All patients had good recovery after operation and favorable prognosis in follow-up for at least one year. Conclusion: RCFS could differentiate obstructive hydrocephalus from communicating hydrocephalus and had important guiding significance in the treatment of hydrocephalus. (authors)

Investigation of hydrocephalus with three-dimensional constructive interference in steady state MRI

International Nuclear Information System (INIS)

Kurihara, N.; National Sendai Hospital; Takahashi, S.; Higano, S.; Furuta, S.; Umetsu, A.; Tamura, H.; Research Inst. of Brain and Blood Vessels, Akita; Jokura, H.

2000-01-01

We report four patients with various types of hydrocephalus in whom constructive interference in steady state (CISS) MRI disclosed the cause of the hydrocephalus. The imaging clearly delineated an abnormal contour of the ventricular system and intraventricular septa, essential information for surgical planning, including endoscopic surgery. Postoperative CISS images were useful for showing not only regression of hydrocephalus but also the patency of small fenestrations. (orig.)

Hydrocephalus treatment in children: long-term outcome in 975 consecutive patients.

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Beuriat, Pierre-Aurelien; Puget, Stephanie; Cinalli, Giuseppe; Blauwblomme, Thomas; Beccaria, Kevin; Zerah, Michel; Sainte-Rose, Christian

2017-07-01

OBJECTIVE Hydrocephalus remains one of the more common pathologies managed in pediatric neurosurgical units. Endoscopic third ventriculostomy (ETV) has an advantage over ventriculoperitoneal shunting as it enables patients to remain device free. Multiple shunt devices with various valve designs exist, with no one valve proven to be superior to another. The aim of this study was to describe the management of hydrocephalus and its long-term outcome. METHODS The authors retrospectively reviewed the medical records of all patients who had been treated for hydrocephalus at the Hôpital Necker-Enfants Malades in the period from 1985 to 1995. RESULTS Nine hundred seventy-five children had been treated for hydrocephalus. The mean follow-up was 11 ± 7.4 years (mean ± standard deviation). The most common cause of hydrocephalus was tumor related (32.3%), followed by malformative (24.5%) and inflammatory (20.9%) causes. Two hundred eighty patients underwent ETV as the first-line treatment. The procedure was effective in controlling hydrocephalus due to posterior fossa tumors and aqueductal stenosis. Six hundred ninety-five children had initial shunt insertion, with the majority receiving an Orbis-Sigma valve (OSV). The overall OSV shunt survival was 70% at 1 year, 58% at 10 years, and 49% at 20 years. The most common cause for mechanical shunt failure was obstruction (50.7%). Overall shunt survival was statistically different between the OSV and the differential-pressure valve (p = 0.009). CONCLUSIONS Endoscopic third ventriculostomy is effective in the management of childhood hydrocephalus. Its success is directly related to the underlying pathology. In the long term, the OSV has significantly higher event-free shunt survival than the classic differential-pressure valve systems.

Classification of cerebrospinal fluid dynamics in hydrocephalus by computed tomographic cisternography

International Nuclear Information System (INIS)

Tamaki, Norihiko; Kanazawa, Yasuhisa; Asada, Masahiro; Nogaki, Hidekazu; Kusunoki, Tadaki

1980-01-01

Nine patients with normal CSF circulation and 49 patients with hydrocephalus of various etiologies were used to analyse the CSF dynamics by metrizamide CT cisternography. This investigation resulted in a new classification of CSF dynamics in both normal and hydrocephalic patients. We classified the patterns of CSF dynamics into seven types. Types I and II showed normal CSF kinetics, the former having no ventricular reflux, but the latter showing a transient ventricular reflux by 6 hours. The CSF dynamics in cases with communicating hydrocephalus was divided into types III, IV and V, according to the extent of ventricular filling and the convexity flow of the metrizamide. These types seen in communicating hydrocephalus were characterized by the presence of ventricular dilatation and persistent ventricular filling on 24 hours after the intrathecal injection of metrizamide. Type III showed more prominent flow into the convexity subarachnoid space than into the ventricular system. In Type IV the ventricular filling was almost the same extent as the convexity flow. Type V demonstrated a more prominent ventricular filling than convexity flow. Type VI had no intracranial entry of the metrizamide. Type VII, which represents the CSF kinetics in obstructive hydrocephalus, showed either a normal or delayed convexity flow. However no ventricular filling was present in spite of the marked ventricular enlargement. There was a close correlation between this new system of classification and the pathogenesis of hydrocephalus, as well as the various factors associated with the pathology of hydrocephalus. Our CT cisternographic classification of CSF dynamics seems to be of use in evaluating the grade of CSF circulation disturbance and in deciding the method of treatment of hydrocephalus. (author)

Large vestibular schwannomas and hydrocephalus: Lessons learnt from a single centre experience

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Prakash Nair

2014-01-01

Full Text Available Aim: The aim of the following study is to analyze the outcome following surgery in 169 patients with large vestibular schwannoma (VS and to evaluate hydrocephalus as a prognostic factor in patients of the VSs. Subjects and Methods: Retrospective analysis of all cases of VSs admitted to our tertiary neurosurgical center from January 2005 to December 2010 was performed. Comparison of patients who underwent pre-operative cerebrospinal fluid (CSF diversion and those who underwent primary surgery was carried out for post-operative complications and delayed hydrocephalus. Results: A total of 169 patients of VS were seen. The mean age at presentation was 39.03 years (12-72 years. The most common symptom was hearing loss seen in 161 (95.2% cases. Giant VS was seen in 130 (75.5% and hydrocephalus was present in 110 (63.9%. Pre-operative CSF diversion was done in 23 (13.1% patients; 8 (4.6% patients developed gradually symptomatic hydrocephalus following surgery and underwent ventriculoperitoneal shunt. Total surgical excision was done in 92.9% patients and subtotal excision was done in 7% patients. Conclusions: Hydrocephalus occurs in longstanding untreated cases of VS. Hydrocephalus causes no statistically significant increase in post-operative complications like CSF leak and post-operative hematoma. Patients with hydrocephalus presenting with acute symptoms of raised intracranial pressure benefit from CSF diversion. In most patients, tumor resection will restore patency of the CSF pathway and CSF diversion can be avoided.

Radionuclide cisternography in the diagnosis of hydrocephalus type in tuberculous meningitis in children

International Nuclear Information System (INIS)

Zafra B, R.M.

1993-01-01

The radionuclide cisternography permits an accurate diagnosis in hydrocephalus, this is an easy procedure, not traumatic nor expensive. Hydrocephalus is one of the most serious complications of tuberculous meningitis, that, without an opportune treatment, leads to severe sequel and death of the patient. The medical records of thirty patients with diagnosis of tuberculosis meningitis were reviewed, to whom the Neuro-Pediatric Service of the National Institute of Children Health solicited radionuclide cisternography to evaluate hydrocephalus and to determinate type, between january 1990 and october 1992. The hydrocephalus was demonstrated by radionuclide cisternography in 26/30 patients (86,7%) The most frequent age was in children under five years old 21/26 (80,7%) and pre-students in 65,38%. The most common finding was communicating hydrocephalus, pattern type IV in 20/30 patients (66,7%). It is concluded that the radionuclide cisternography is very useful for the diagnosis of hydrocephalus and its type patients with tuberculous meningitis. This exam is recommended for all patients with diagnosis of tuberculous meningitis, with no satisfactory evolution, in order to obtain an early diagnosis of hydrocephalus and its type, and get an opportune medical or surgical treatment. (author). 35 refs., 3 tab., 8 ills

Excess HB-EGF, which promotes VEGF signaling, leads to hydrocephalus

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Shim, Joon W.; Sandlund, Johanna; Hameed, Mustafa Q.; Blazer-Yost, Bonnie; Zhou, Feng C.; Klagsbrun, Michael; Madsen, Joseph R.

2016-01-01

Heparin binding epidermal growth factor-like growth factor (HB-EGF) is an angiogenic factor mediating radial migration of the developing forebrain, while vascular endothelial growth factor (VEGF) is known to influence rostral migratory stream in rodents. Cell migratory defects have been identified in animal models of hydrocephalus; however, the relationship between HB-EGF and hydrocephalus is unclear. We show that mice overexpressing human HB-EGF with β-galactosidase reporter exhibit an elevated VEGF, localization of β-galactosidase outside the subventricular zone (SVZ), subarachnoid hemorrhage, and ventriculomegaly. In Wistar polycystic kidney rats with hydrocephalus, alteration of migratory trajectory is detected. Furthermore, VEGF infusions into the rats result in ventriculomegaly with an increase of SVZ neuroblast in rostral migratory stream, whereas VEGF ligand inhibition prevents it. Our results support the idea that excess HB-EGF leads to a significant elevation of VEGF and ventricular dilatation. These data suggest a potential pathophysiological mechanism that elevated HB-EGF can elicit VEGF induction and hydrocephalus. PMID:27243144

Allelic Mutations of KITLG, Encoding KIT Ligand, Cause Asymmetric and Unilateral Hearing Loss and Waardenburg Syndrome Type 2

NARCIS (Netherlands)

Zazo Seco, C.; Castro, L.S. de; Nierop, J.W. van; Morin, M.; Jhangiani, S.; Verver, E.J.; Schraders, M.; Maiwald, N.; Wesdorp, F.M.; Venselaar, H.; Spruijt, L.; Oostrik, J.; Schoots, J.; Reeuwijk, J. van; Lelieveld, S.H.; Huygen, P.L.M.; Insenser, M.; Admiraal, R.J.C.; Pennings, R.J.E.; Hoefsloot, L.H.; Arias Vasquez, A.; Ligt, J. de; Yntema, H.G.; Jansen, J.H.; Muzny, D.M.; Huls, G.A.; Rossum, M.M. van; Lupski, J.R.; Moreno-Pelayo, M.A.; Kunst, H.P.M.; Kremer, H.

2015-01-01

Linkage analysis combined with whole-exome sequencing in a large family with congenital and stable non-syndromic unilateral and asymmetric hearing loss (NS-UHL/AHL) revealed a heterozygous truncating mutation, c.286_303delinsT (p.Ser96Ter), in KITLG. This mutation co-segregated with NS-UHL/AHL as a

Secondary pediatric encephalocele after ventriculosubgaleal shunting for posthemorrhagic hydrocephalus.

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Seeburg, Daniel; Ahn, Edward; Huisman, Thierry

2014-08-01

Intraventricular hemorrhage and posthemorrhagic hydrocephalus continue to be common complications in very low-birth-weight premature infants, often requiring ventricular shunting for cerebrospinal fluid diversion. We report on two infants with posthemorrhagic hydrocephalus that developed a secondary encephalocele after ventriculosubgaleal shunting. Encephaloceles can act as a source of seizure activity and can result in various additional complications including meningitis, abscess formation, and infarction of herniated brain parenchyma. With continued improvements in neonatal intensive care, the survival of infants with significant medical comorbidities-including those that develop posthemorrhagic hydrocephalus requiring ventricular shunting-continues to increase. It is thus important for the radiologist and treating physician to be aware of this rare, potential complication. Georg Thieme Verlag KG Stuttgart · New York.

Unilateral Autosomal Recessive Anophthalmia in a Patient with Cystic Craniopharyngioma

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Kumar, Amandeep; Bansal, Ankit; Garg, Ajay; Sharma, Bhawani S.

2014-01-01

Abstract Anophthalmia is a rare ocular malformation. It is a genetically determined disorder and is typically associated with syndromes. However, sporadic nonsyndromic familial as well as non-familial cases of anophthalmia have also been reported. Non-syndromic familial cases are usually bilateral and have been attributed to autosomal recessive, autosomal dominant, and X-linked inheritance patterns. The authors hereby report a rare case of autosomal recessive unilateral anophthalmia in a patient with no other associated congenital anomaly. Patient was operated for craniopharyngioma. The clinical, radiological and intraoperative findings are discussed. PMID:27928292

Increased self-diffusion of brain water in hydrocephalus measured by MR imaging

DEFF Research Database (Denmark)

Gideon, P; Thomsen, C; Gjerris, F

1994-01-01

We used MR imaging to measure the apparent brain water self-diffusion in 5 patients with normal pressure hydrocephalus (NPH), in 2 patients with high pressure hydrocephalus (HPH), and in 8 age-matched controls. In all patients with NPH significant elevations of the apparent diffusion coefficients...... white matter, and in one patient reexamined one year after surgery, ADCs were unchanged in nearly all brain regions. The increased ADC values in hydrocephalus patients may be caused by factors such as changes in myelin-associated bound water, increased Virchow-Robin spaces, and increased extracellular...... brain water fraction. For further studies of brain water diffusion in hydrocephalus patients, echo-planar imaging techniques with imaging times of a few seconds may be valuable....

[Hydrocephalus Associated with Small Clinoidal Meningioma that Resolved after Tumor Removal:A Case Report].

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Fujiwara, Hidemoto; Aiba, Toyotaka; Watanabe, Toru; Hiraishi, Tetsuya; Fujii, Yukihiko

2016-12-01

Small meningiomas causing hydrocephalus without obstruction of the ventricular system are rare. Herein, we report a case of small clinoidal meningioma with communicating hydrocephalus, which resolved after tumor removal. A 70-year-old woman presented with a 1-month history of memory disturbance followed by gait disturbance. MR images revealed a right clinoidal meningioma, 2 cm in diameter, and dilatation of the ventricles suggesting communicating hydrocephalus. The cerebrospinal fluid(CSF)pressure was 130 mmH2O, as determined via a lumbar puncture. High concentrations of protein(65mg/dL)were detected in the lumbar CSF. The tumor was completely removed via a frontotemporal craniotomy. Higher protein concentrations(94mg/dL)were detected in the CSF obtained intraoperatively from the sylvian cistern. The histopathological diagnosis was meningothelial meningioma. The patient's symptoms improved markedly after surgery. Postoperative MR images revealed resolution of the hydrocephalus. The lumbar CSF protein concentration returned to normal(43mg/dL). Neither tumor recurrence nor progression of hydrocephalus has been observed for 4 years. Communicating hydrocephalus, associated with a small meningioma at the supratentorial region, has not been described. Previous studies have shown that patients with meningioma may develop communicating hydrocephalus after tumor removal or stereotactic radiosurgery. Thus, it is interesting that the small supratentorial meningioma in our case developed communicating hydrocephalus without any therapeutic intervention. Considering the CSF protein concentration, we speculate that the hydrocephalus was the result of CSF malabsorption associated with high CSF protein concentration and CSF pathway obstruction at the suprasellar cistern caused by the tumor.

Concept and treatment of hydrocephalus in the Greco-Roman and early Arabic medicine.

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Grunert, P; Charalampaki, P; Ayyad, A

2007-10-01

In the ancient medical literature hydrocephalus was not often described although its existence and symptomatology were well known. Most detailed descriptions of hydrocephalus including the surgical treatment are extant in the encyclopaedic works on medicine of the physicians Oreibasios and Aetios from Amida from the 4th and 6th centuries AD, respectively. Because of their broad scientific interests, this type of physicians, typical for the late Roman empire, were known as philosophy-physicians (iota alpha tau rho o sigma o phi iota sigma tau alpha iota). They defined hydrocephalus in contrast to our present understanding as a fluid collection excluding abscesses visible as a bulging tumour localised either outside or inside the skull of an infant. They classified the hydrocephalus similar as stated first by Galen in the 2nd century AD in four types corresponding to the assumed anatomic localisation of the fluid collection: 1st Type between the skin and the pericranium corresponding to the subgaleal haematoma or caput succedaneum of the newborn in our terminology, 2nd Type between the pericranium and the skull corresponding to the cephal haematoma after delivery, 3rd Type between skull and the meninges with increased head circumference, bone sutures being increasingly driven apart corresponding most likely to the hydrocephalus in our understanding, and 4th Type between the menings and the brain characterised by severe neurological deficit with lethal prognosis corresponding probably to all pathologies which were accompanied by an excessive increase of the intracranial pressure with a bulging fontanel. Due to the lack of autopsies in ancient times, the hydrocephalus was never linked to the pathology of the ventricles. All forms of hydrocephalus were believed to be caused by improper handling of the head by the midwife during delivery. Only the extracranial fluid collections, but not hydrocephalus in our sense, were considered to be suitable for surgical treatment

Surgery for congenital choanal atresia.

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Cedin, Antonio C; Atallah, Alvaro N; Andriolo, Régis B; Cruz, Oswaldo L; Pignatari, Shirley N

2012-02-15

Congenital choanal atresia is a rare abnormality characterized by unilateral or bilateral lack of patency of the posterior end of the nasal cavity. With an incidence of 1:5000 to 1:8000 births, it is twice as prevalent in females as it is in males. Surgical procedures aim to provide adequate functional choanal patency and a low rate of restenosis, avoid harm to any structure in development, enable shorter surgery and hospitalization times, and minimize morbidity and mortality. To evaluate the effectiveness and safety of the available surgical techniques for the treatment of congenital choanal atresia in patients with unilateral and bilateral atresia. We searched the Cochrane Ear, Nose and Throat Disorders Group Trials Register; the Cochrane Central Register of Controlled Trials (CENTRAL); PubMed; EMBASE; CINAHL; Web of Science; BIOSIS Previews; Cambridge Scientific Abstracts; ISRCTN and additional sources for published and unpublished trials. The date of the search was 31 January 2011. We planned to include parallel randomized or quasi-randomized controlled trials testing surgical approaches for the treatment of congenital atresia (irrespective of gender and age) that evaluated normal/adequate respiratory function (self reported or preserved nasal airway) and restenosis as the main primary outcomes. We did not consider reoperation and non-congenital atresia (e.g. traumatic, iatrogenic atresias) for inclusion. Three review authors independently assessed the titles and abstracts of the identified articles to determine potential relevance. For dichotomous and continuous variables, we planned to calculate risk ratios (relative risks; RR) and mean differences (MD) with 95% confidence intervals (CI), respectively. We planned to use the random-effects model since we were expecting substantial clinical and methodological heterogeneity. No randomized controlled trials were identified. From the 120 reports retrieved using our search strategy, 46 primary studies had the

Evolution of surgical interventions for hydrocephalus: patient preferences and the need for proper information

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Dr. P. O. Eghwrudjakpor MBBS, DMS, FICS

2010-07-01

Full Text Available The treatment of hydrocephalus has undergone remarkable transformation since it was first documented over two thousand years ago. Currently, the focus of hydrocephalus research is on minimally invasive techniques of treatment. This article reviews the evolution of hydrocephalus therapy, and examines current attitudes towards modern methods. We relied on journal publications, as well as literature on hydrocephalus obtained from the Internet (Google, Yahoo and PUBMED search making use of the following search terms: “hydrocephalus: history; treatment; complications”, “cerebrospinal fluid shunt”, “endoscopic third ventriculostomy: indications of; complications of; advantages; disadvantages; successes; failure”. Numerous medical and surgical approaches have been adopted in the treatment of hydrocephalus in the past. However, the breakthrough that ushered in the modern era of hydrocephalus treatment was the introduction of valve-regulated systems in the middle of the last century. Endoscopic third ventriculostomy has evolved to become an alternative to traditional shunts. Cerebrospinal fluid shunt procedures are very effective in the treatment of hydrocephalus and have radically transformed the outcome of the disorder. However, they have a number of limitations. The alternative to shunts, endoscopic third ventriculostomy, is relatively safe, effective and durable. It does not, however, succeed in every patient; and also has some potentially devastating complications. Preoperative counseling is imperative to ensure that patients are properly guided.

Epidemiology of Benign External Hydrocephalus in Norway-A Population-Based Study.

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Wiig, Ulrikke S; Zahl, Sverre M; Egge, Arild; Helseth, Eirik; Wester, Knut

2017-08-01

Benign external hydrocephalus is defined as a rapidly increasing head circumference (occipitofrontal circumference) with characteristic radiological findings of increased subarachnoid cerebrospinal fluid spaces on neuroimaging. The incidence of benign external hydrocephalus has not been previously reported, and there is no available information on the ratio of benign external hydrocephalus in the population of hydrocephalic children. This study is retrospective and population-based study, geographically covering two health regions in the southern half of Norway with a total mean population of 3.34 million in the ten-year study period, constituting approximately 75% of the Norwegian population. Children with a head circumference crossing two percentiles, or greater than the 97.5th percentile, and with typical imaging findings of enlarged frontal subarachnoid spaces with or without enlarged ventricles were included. Children were excluded if they had a history of head trauma, intracranial hemorrhage, central nervous system infection, other known causes of hydrocephalus, or were born preterm defined as birth before 37 weeks of gestation. A total of 176 children fitting the criteria were identified, giving an incidence of 0.4 per 1000 live births. One hundred fifty-two (86.4%) of the patients were male, and mean age at referral was 7.3 months. Increasing head circumference was the main reason for referral in 158 (89.8%) patients and the only finding in 60 (34.1%) patients. Thirty-seven (21%) children had normal ventricles on imaging; the remainder had increased ventricular size. The incidence of pediatric hydrocephalus in Norway is reported to be 0.75 per 1000 live births, thus benign external hydrocephalus accounts for approximately 50% of hydrocephalic conditions in this population. The incidence of benign external hydrocephalus was found to be 0.4 per 1000 live births in this population. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights

Incidence of Intraventricular Hemorrhage and Post Hemorrhagic Hydrocephalus in Preterm Infants

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Negar Sajjadian

2010-07-01

Full Text Available "nGerminal matrix-intraventricular hemorrhage (IVH is the most common variety of neonatal intracranial hemorrhage and is characteristics of the premature infant. The importance of the lesion relates not only to its high incidence but to their attendant complications (IC: hydrocephalus. Brain sonography is the procedure of choice in diagnosis of germinal matrix- intraventricular hemorrhage and hydrocephalus. In this study we have used brain sonography for detection of intraventricular hemorrhage and post hemorrhagic hydrocephalus and their incidences. The studied population was consisted of premature neonate (birth weight equal or less than 1500g and gestational age equal or less than 37 weeks who admitted in Mofid Hospital NICU (Tehran, Iran during a one year period. For all neonate (including criteria brain sonography in first week of life was done and in presence of IVH, serial Brain sonography was done weekly for detection of hydrocephalus. A total of 57 neonate entered the study. Intraventicular-germinal matrix hemorrhage was seen in 64.4% (35 patients. Forty percent of patients with intraventricular-germinal matrix hemorrhage had grade I, 11% grade II, 25.7% grade III, 2.8% grade VI. Hydrocephalus was detected in 20 percent of patients who had intraventricular-germinal matrix hemorrhage. That incidence of IVH in our study in comparison with other area and situation is higher. Hydrocephaly had direct relation with severity of IVH. This shows that with control of risk factor of IVH, we can control Post hemorrhagic hydrocephalus.

Spontaneous third ventriculostomy 8 years after diagnosis of obstructive hydrocephalus.

Science.gov (United States)

Öğrenci, Ahmet; Ekşi, Murat Şakir; Koban, Orkun

2016-09-01

Spontaneous ventriculostomy is spontaneous rupture of membranes separating the ventricular system from the subarachnoid space in patients with chronic obstructive hydrocephalus that ends with resolution of symptoms. We present a case of spontaneous third ventriculostomy occurred in a 19-year-old girl 8 years after the initial diagnosis of hydrocephalus. An 11-year-old girl applied to the clinic with intermittent headaches. She was neurologically stable with no visual problems. On her brain MRI, obstructive hydrocephalus was observed. Cerebrospinal fluid diversion procedures were recommended, yet the family denied any interventional procedure. She had routine follow-ups with occasional clinical admissions because of ongoing intermittent headaches. On her last clinical visit, 8 years after the first one, she was in well condition with improvement in her headache in the last 4 months. Her new brain MRI showed an active CSF flow between the basal cistern and the third ventricle. In patients with aqueductal stenosis and without any other mass lesion, wait and see protocol might be conveyed in case of mild symptoms of hydrocephalus. However, there is need for large-scaled studies to make a more comprehensive statement for benign obstructive hydrocephalus cases.

Prolonged hydrocephalus induced by intraventricular hemorrhage in rats is reduced by curcumin therapy.

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Qi, Zhihua; Zhang, Huiqin; Fu, Chuhua; Liu, Xiao; Chen, Bo; Dang, Yanwei; Chen, Huayun; Liu, Lijun

2017-01-10

Prolonged hydrocephalus is a major cause of severe disability and death of intraventricular hemorrhage (IVH) patients. However, the therapeutic options to minimize the detrimental effects of post-hemorrhagic hydrocephalus are limited. Curcumin has been reported to confer neuroprotective effects in numerous neurological diseases and injuries, but its role in IVH-induced hydrocephalus has not been determined. The aim of present study was to determine whether curcumin treatment ameliorates blood brain barrier (BBB) damage and reduces the incidence of post-hemorrhagic hydrocephalus in IVH rat model. Autologous blood intraventricular injection was used to establish the IVH model. Our results revealed that repeated intraperitoneal injection of curcumin ameliorated IVH-induced learning and memory deficits as determined by Morris water maze and reduced the incidence of post-hemorrhagic hydrocephalus in a dose-dependent manner at 28 d post-IVH induction. Further, the increased BBB permeability and brain edema induced by IVH were significantly reduced by curcumin administration. In summary, these findings highlighted the important role of curcumin in improving neurological function deficits and protecting against BBB disruption via promoting the neurovascular unit restoration, and thus it reduced the severity of post-hemorrhagic hydrocephalus in the long term. It is believed that curcumin might prove to be an effective therapeutic component in prevent the post-IVH hydrocephalus in the near future. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Hydrocephalus in mice following X-irradiation at early gestational stage. Possibly due to persistent deceleration of cell proliferation

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Aolad, H.M.; Inouye, Minoru; Darmanto, W.; Hayasaka, Shizu; Murata, Yoshiharu [Nagoya Univ. (Japan). Research Inst. of Environmental Medicine

2000-09-01

The pathogenesis of X-ray-induced congenital hydrocephalus was studied. Pregnant mice were irradiated at 1.4 Gy on gestational day 7 (G7). Four hours after irradiation, extensive cell death was evident in the neuroepithelium and underlying mesoderm of the head region, and proliferating cell nuclear antigen (PCNA)-immunoreactive cells almost disappeared. Embryos with thinner lamina terminalis of the telecephalon, when compared with that of the control, were found in the irradiated group on G9. As early as G11 in some irradiated embryos the telencephalic wall was thinner and lateral ventricles were larger than those of the control. The choroid invagination from the lamina terminalis began on G11 in the control brain, but not in the affected brain. During the following development, fetuses with readily apparent hydrocephalus were consistently found among irradiated fetuses. In these brains the brain mantle was thinner, the corpus striatum and thalamic regions were smaller, and lateral ventricles were larger than those of the control. Even on G11 and G13 the frequencies of PCNA-positive cells in the brain mantle and other brain regions were lower in the hydrocephalic brain than those of the control, suggesting a decelerated proliferation of successive cell generations following exposure to X-rays. The cerebral aqueduct was open in the hydrocephalic brain during the fetal period when the lateral ventricles were dilated. The head was vaulted after birth but the cerebral aqueduct was not completely occluded even in these animals. These findings suggested that cell death in the neuroepithelium followed by a persistent deceleration of neural cell proliferation, resulting in the hypoplasia of brain parenchyma with compensatory ventricular dilatation, is important for the establishment of hydrocephalus. (author)

Hydrocephalus in mice following X-irradiation at early gestational stage. Possibly due to persistent deceleration of cell proliferation

International Nuclear Information System (INIS)

Aolad, H.M.; Inouye, Minoru; Darmanto, W.; Hayasaka, Shizu; Murata, Yoshiharu

2000-01-01

The pathogenesis of X-ray-induced congenital hydrocephalus was studied. Pregnant mice were irradiated at 1.4 Gy on gestational day 7 (G7). Four hours after irradiation, extensive cell death was evident in the neuroepithelium and underlying mesoderm of the head region, and proliferating cell nuclear antigen (PCNA)-immunoreactive cells almost disappeared. Embryos with thinner lamina terminalis of the telecephalon, when compared with that of the control, were found in the irradiated group on G9. As early as G11 in some irradiated embryos the telencephalic wall was thinner and lateral ventricles were larger than those of the control. The choroid invagination from the lamina terminalis began on G11 in the control brain, but not in the affected brain. During the following development, fetuses with readily apparent hydrocephalus were consistently found among irradiated fetuses. In these brains the brain mantle was thinner, the corpus striatum and thalamic regions were smaller, and lateral ventricles were larger than those of the control. Even on G11 and G13 the frequencies of PCNA-positive cells in the brain mantle and other brain regions were lower in the hydrocephalic brain than those of the control, suggesting a decelerated proliferation of successive cell generations following exposure to X-rays. The cerebral aqueduct was open in the hydrocephalic brain during the fetal period when the lateral ventricles were dilated. The head was vaulted after birth but the cerebral aqueduct was not completely occluded even in these animals. These findings suggested that cell death in the neuroepithelium followed by a persistent deceleration of neural cell proliferation, resulting in the hypoplasia of brain parenchyma with compensatory ventricular dilatation, is important for the establishment of hydrocephalus. (author)

Mixed feelings of children and adolescents with unilateral congenital below elbow deficiency: an online focus group study.

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Ingrid G M de Jong

Full Text Available The existing literature is inconsistent about the psychosocial functioning of children and adolescents with Unilateral Congenital Below Elbow Deficiency (UCBED. The objective of this qualitative study was to explore the psychosocial functioning of children and adolescents with UCBED in terms of their feelings about the deficiency and what helps them to cope with those feelings. Additionally, the perspectives of prosthesis wearers and non-wearers were compared, as were the perspectives of children, adolescents, parents and health professionals. Online focus group interviews were carried out with 42 children and adolescents (aged 8-12, 13-16 and 17-20, 16 parents and 19 health professionals. Questions were asked about psychosocial functioning, activities, participation, prosthetic use or non-use, and rehabilitation care. This study concerned remarks about psychosocial functioning. Children and adolescents with UCBED had mixed feelings about their deficiency. Both negative and positive feelings were often felt simultaneously and mainly depended on the way people in the children's environment reacted to the deficiency. People staring affected the children negatively, while support from others helped them to cope with the deficiency. Wearing a prosthesis and peer-to-peer contact were also helpful. Non-wearers tended to be more resilient than prosthesis wearers. Wearers wore their prosthesis for cosmetic reasons and to prevent them from negative reactions from the environment. We recommend that rehabilitation teams make parents aware of their great influence on the psychosocial functioning of their child with UCBED, to adjust or extend the currently available psychosocial help, and to encourage peer-to-peer contact.

Mixed feelings of children and adolescents with unilateral congenital below elbow deficiency: an online focus group study.

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de Jong, Ingrid G M; Reinders-Messelink, Heleen A; Janssen, Wim G M; Poelma, Margriet J; van Wijk, Iris; van der Sluis, Corry K

2012-01-01

The existing literature is inconsistent about the psychosocial functioning of children and adolescents with Unilateral Congenital Below Elbow Deficiency (UCBED). The objective of this qualitative study was to explore the psychosocial functioning of children and adolescents with UCBED in terms of their feelings about the deficiency and what helps them to cope with those feelings. Additionally, the perspectives of prosthesis wearers and non-wearers were compared, as were the perspectives of children, adolescents, parents and health professionals. Online focus group interviews were carried out with 42 children and adolescents (aged 8-12, 13-16 and 17-20), 16 parents and 19 health professionals. Questions were asked about psychosocial functioning, activities, participation, prosthetic use or non-use, and rehabilitation care. This study concerned remarks about psychosocial functioning. Children and adolescents with UCBED had mixed feelings about their deficiency. Both negative and positive feelings were often felt simultaneously and mainly depended on the way people in the children's environment reacted to the deficiency. People staring affected the children negatively, while support from others helped them to cope with the deficiency. Wearing a prosthesis and peer-to-peer contact were also helpful. Non-wearers tended to be more resilient than prosthesis wearers. Wearers wore their prosthesis for cosmetic reasons and to prevent them from negative reactions from the environment. We recommend that rehabilitation teams make parents aware of their great influence on the psychosocial functioning of their child with UCBED, to adjust or extend the currently available psychosocial help, and to encourage peer-to-peer contact.

Ehlers-Danlos syndrome type IV : unusual congenital anomalies in a mother and son with a COL3A1 mutation and a normal collagen III protein profile

NARCIS (Netherlands)

Kroes, HY; Pals, G; van Essen, AJ

A mother and son with Ehlers-Danlos syndrome (EDS) type IV and unusual congenital anomalies are described. The congenital anomalies include, in the mother, amniotic band-like constrictions on one hand, a unilateral clubfoot, and macrocephaly owing to normal-pressure hydrocephaly and, in the son, an

Glymphatic MRI in idiopathic normal pressure hydrocephalus.

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Ringstad, Geir; Vatnehol, Svein Are Sirirud; Eide, Per Kristian

2017-10-01

The glymphatic system has in previous studies been shown as fundamental to clearance of waste metabolites from the brain interstitial space, and is proposed to be instrumental in normal ageing and brain pathology such as Alzheimer's disease and brain trauma. Assessment of glymphatic function using magnetic resonance imaging with intrathecal contrast agent as a cerebrospinal fluid tracer has so far been limited to rodents. We aimed to image cerebrospinal fluid flow characteristics and glymphatic function in humans, and applied the methodology in a prospective study of 15 idiopathic normal pressure hydrocephalus patients (mean age 71.3 ± 8.1 years, three female and 12 male) and eight reference subjects (mean age 41.1 + 13.0 years, six female and two male) with suspected cerebrospinal fluid leakage (seven) and intracranial cyst (one). The imaging protocol included T1-weighted magnetic resonance imaging with equal sequence parameters before and at multiple time points through 24 h after intrathecal injection of the contrast agent gadobutrol at the lumbar level. All study subjects were kept in the supine position between examinations during the first day. Gadobutrol enhancement was measured at all imaging time points from regions of interest placed at predefined locations in brain parenchyma, the subarachnoid and intraventricular space, and inside the sagittal sinus. Parameters demonstrating gadobutrol enhancement and clearance in different locations were compared between idiopathic normal pressure hydrocephalus and reference subjects. A characteristic flow pattern in idiopathic normal hydrocephalus was ventricular reflux of gadobutrol from the subarachnoid space followed by transependymal gadobutrol migration. At the brain surfaces, gadobutrol propagated antegradely along large leptomeningeal arteries in all study subjects, and preceded glymphatic enhancement in adjacent brain tissue, indicating a pivotal role of intracranial pulsations for glymphatic function. In

Congenital or torsion-induced absence of Fallopian tubes. Two case reports.

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Paternoster, D M; Costantini, W; Uglietti, A; Vasile, C; Bocconi, L

1998-05-01

Unilateral absence of a uterine tube is an extremely rare finding, for which there are two possible etiopathogenic causes: in some cases it is due to haemorrhage filling of the cavity and its reabsorption as a result of asymptomatic torsion of the uterine tube during adult life, in pediatric age or even during intrauterine life; alternatively, the absence may be congenital, associated with developmental alterations of the mesonephric and paramesonephric ducts. The article presents two cases of fallopian tube absence: a congenital monolateral absence and a tubal torsion during pregnancy. The symptomatology of the torsion of the fallopian tube in pregnancy can be milder than in the classic description with peritoneal reaction and severe clinical alteration. The main risk factors for tubal torsion are: adhesions and inflammatory processes, ovarian cysts, usually of dermoid type, menstrual period, pregnancy, abnormal long mesosalpinx and/or mesovarium, pelvic congestion induced by constipation and disturbed venous blood flow from the adnexa. A congenital defect of the mesonephric duct is followed by a homolateral defect of the paramesonephric duct. The resulting anomaly is characterized by the absence of the uterine tube, uterus-tube angle, kidney and ureter. Partial or total unilateral defects of a paramesonephric duct are more common than aplasia of both ducts. Some authors have suggested that an inadequate blood supply during the descent into the pelvis of the caudal part of the paramesonephric duct might feasibly lead to incomplete tube development.

In need of a patch UP: Recurrent congenital diaphragmatic hernia presenting with a large pleural effusion

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Farhana Shariff

2014-10-01

Full Text Available We report a case of recurrent congenital diaphragmatic hernia (CDH presenting with a large unilateral pleural effusion. A 12-year old boy who had a left sided CDH repaired in the neonatal period, presented with fever, lethargy, and non-productive cough. Chest radiograph demonstrated a loculated pleural effusion. Computed tomography scan revealed recurrent herniation of abdominal contents. To our knowledge, this is the first reported case of a recurrent congenital diaphragmatic hernia presenting with large pleural effusion.

Dyke-Davidoff-Masson syndrome: case report of fetal unilateral ventriculomegaly and hypoplastic left middle cerebral artery

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Piro, Ettore; Piccione, Maria; Marrone, Gianluca; Giuffr?, Mario; Corsello, Giovanni

2013-01-01

Prenatal ultrasonographic detection of unilateral cerebral ventriculomegaly arises suspicion of pathological condition related to cerebrospinal fluid flow obstruction or cerebral parenchimal pathology. Dyke-Davidoff-Masson syndrome is a rare condition characterized by cerebral hemiatrophy, calvarial thickening, skull and facial asymmetry, contralateral hemiparesis, cognitive impairment and seizures. Congenital and acquired types are recognized and have been described, mainly in late childhood...

One-stage release of congenital constriction band in lower limb from new born to 3 years

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Das Sakti

2010-01-01

Full Text Available Background: Congenital constriction band is the most common cause of terminal congenital malformation of a limb and lymphoedema. Superficial bands do not need any treatment, but deeper bands are managed with excision and Z-plasty. The circumferential bands are released in two to three stages to prevent vascular compromise. The purpose of this study was to present the outcome of one-stage release. Materials and Methods: Nineteen children, 12 boys and 7 girls, with 24 congenital constriction bands constituted the clinical material. The mean age at presentation was 57 days (range 12 hours to 3 years Band was unilateral in 14 and bilateral in five limbs. In unilateral cases, right side was involved in nine cases and left side in five. The constriction band is seen at the junction of middle and distal third. The patients having constriction bands in lower limbs and age less than 3 years were included in the study. One stage circumferential release of congenital constriction band was performed. Our youngest patient was operated at the age of six months. Club feet, (n=8 and lymphedema (n=7 were associated anomalies. Club feet and band were released in one stage in three limbs. The results were evaluated by criteria described by Joseph Upton and Cissy Tan. Results: There were 18 excellent, six satisfactory results. No wound problem occurred. No vascular compromise was noted during or after the procedure. On follow-up, distal swelling reduced. Conclusion: One-stage circumferential release of congenital constriction band in lower limbs with or without lymphodema is a safe and easy procedure.

Hydrocephalus and mucopolysaccharidoses: what do we know and what do we not know?

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Dalla Corte, Amauri; de Souza, Carolina F M; Anés, Maurício; Giugliani, Roberto

2017-07-01

The precise incidence of hydrocephalus in patients with mucopolysaccharidoses (MPS) is hard to determine, because the condition lacks a formal, consensus-based definition. The diagnosis of hydrocephalus depends on symptom profile, presence of neuroimaging features, and the outcome of diagnostic tests. Although numerous techniques are used to identify MPS patients who are most likely to have hydrocephalus and respond to treatment, no definitive method exists to prove diagnosis. The authors propose an algorithm to aid in the diagnosis and management of hydrocephalus in MPS patients. The theory of venous hypertension associated with the morphological changes in the skull base and craniocervical junction indicate the need for future neuroimaging studies including cerebrospinal fluid (CSF) and venous flow measurements to monitor hydrocephalus progression and select therapeutic interventions in MPS patients. Preoperative planning should also be based on the increased risk of intraoperative and postoperative hemorrhagic complications.

Congenital agenesis of unilateral parotid gland with ipsilateral type I first branchial cleft anomaly: A rare presentation

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Tripti Maithani; Apoorva Pandey; Seema Acharya

2014-01-01

Aim: To report a rare case of unilateral parotid agenesis with ipsilateral type I first branchial cleft anomaly. Material and methods: A case study with special emphasis on the embryology, outlining the complex developmental process of parotid and branchial arches and highlighting the probable reason for development of such anomalies. Results: The literature states that unilateral parotid agenesis is a rare entity with few reported cases occurring solely or in conjunction with other hea...

Diffusion-weighted imaging in the diagnostic evaluation of the hydrocephalus in patients with acute or chronic increase in cerebral pressure; Diffusionsgewichtete Bildgebung in der Diagnostik des Hydrocephalus - Untersuchungen an Patienten mit akuter und ohne akute Hirndrucksymptomatik

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Dorenbeck, U. [Abt. fuer Neuroradiologie, Universitaetskliniken des Saarlandes, Homburg/Saar (Germany); Inst. fuer Roentgendiagnostik, Universitaetsklinik Regensburg (Germany); Schlaier, J. [Klinik und Poliklinik fuer Neurochirurgie, Universitaetsklinik Regensburg (Germany); Feuerbach, S.; Seitz, J. [Inst. fuer Roentgendiagnostik, Universitaetsklinik Regensburg (Germany)

2005-01-01

Purpose: to investigate whether diffusion-weighted imaging (DWI) in magnetic resonance imaging (MRI) provides additional information about the periventricular white matter for the assessment of hydrocephalus. Materials and methods: sixteen MRI examinations (11 with acutely increased cerebral pressure, 5 without symptoms) on 15 patients with hydrocephalus (4 patients with communicating hydrocephalus and 11 patients with obstructive hydrocephalus) were analyzed. One symptomatic patient subsequently became asymptomatic. We investigated the ''apparent diffusion coefficient'' (ADC) in the subcortical and periventricular white matter. The ADCs of the study patients were compared with those of a healthy control group. Results: symptomatic patients with hydrocephalus, 6/11 showed periventricular edema and a significantly higher ADC values in the periventricular region than in the subcortical white matter. 5/11 symptomatic patients showed significantly higher ADC values even in the absence of periventricular interstitial edema (both groups contained patients with communicating and obstructive hydrocephalus). All 5 asymptomatic patients with hydrocephalus did not have a significantly higher ADC values in the periventricular region. Conclusion: in patients with hydrocephalus and acutely increased cerebral pressure, DWI showed a significantly higher ADC values in the periventricular region even without visible interstitial edema on conventional MRI sequences. (orig.)

First reported case of unilateral Graves' disease in the left lobe of a bilobar thyroid gland.

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Chen, Louis C; Green, Jennifer B

2011-06-01

Unilateral Graves' disease is a rare disease variant that can occur in a bilobar thyroid gland. We report the first documented case of unilateral Graves' disease in the left lobe of a bilobar thyroid gland and review the pertinent literature. A 48-year-old man presented in June 2010 with thyrotoxicosis. I-131 radioisotope uptake was elevated at 33.4%, and scintigraphy revealed that uptake of the radioisotope was uniformly increased in the left lobe of the thyroid gland. Ultrasonography of the thyroid gland revealed a non-nodular, enlarged, and heterogeneous left lobe; Doppler investigation of the lobe showed hypervascularity classically seen in Graves' disease. The right lobe of the thyroid, on the other hand, appeared homogeneous and hypovascular on ultrasonography. Thyroid-stimulating immunoglobulin was significantly elevated at 191% (reference range disease was the most likely diagnosis. As has occasionally been described in the literature, unilateral involvement of the thyroid gland is a rare presentation of Graves' disease. Pre-existing functional or structural differences (either congenital or acquired) between the two lobes may contribute to this rare presentation. To our knowledge, this is the first reported case of unilateral Graves' disease presenting in the left lobe of a bilobar thyroid gland. Although the pathophysiology of unilateral Graves's disease has not been clearly elucidated, clinicians should be aware that Graves' disease can present unilaterally in either lobe of the thyroid gland.

Valved or valveless ventriculoperitoneal shunting in the treatment of post-haemorrhagic hydrocephalus

DEFF Research Database (Denmark)

Andreasen, Trine Hjorslev; Holst, Anders Vedel; Lilja, Alexander

2016-01-01

BACKGROUND: Implant infection and obstruction are major complications for ventriculoperitoneal shunts in patients with post-haemorrhagic hydrocephalus. In an effort to (1) reduce the incidence of these complications, (2) reduce the rate of shunt failure and (3) shorten the duration of neurosurgical...... hospitalisation, we have implemented valveless ventriculoperitoneal shunts at our department for adult patients with post-haemorrhagic hydrocephalus and haemorrhagic cerebrospinal fluid at the time of shunt insertion. METHODS: All adult patients (>18 years old) treated for post-haemorrhagic hydrocephalus.......3 %, p = 0.02), but a higher rate of overdrainage (10.3 % vs 2.6 %, p = 0.04). CONCLUSION: The use of a valveless shunting for patients with post-haemorrhagic hydrocephalus results in shorter duration of neurosurgical hospitalisation and lower rate of shunt infection, although these advantages should...

A Modified Shunt System for the Management of Pyogenic Hydrocephalus

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Abdul Naveed Khan

2017-01-01

Full Text Available BACKGROUND: To describe the structure and efficacy of a modified cerebrospinal fluid (CSF shunt system with Bhatti CSF access chamber and ventriculoperitoneal shunt, for the management of hydrocephalus associated with pyogenic ventriculitis. METHODS: This was an observational study performed at the National Medical Center and Ziauddin Medical University, Karachi, Pakistan. Chemically bonded Bhatti CSF access chamber and ventriculoperitoneal shunt was inserted into 443 patients with hydrocephalus and pyogenic ventriculitis to allow injection of antibiotics into the lateral ventricles. RESULTS: In all patients, symptoms and signs of hydrocephalus were relieved immediately after shunt insertion and infection brought under control within two weeks of intraventricular injection of antibiotics (vancomycin or linezolid, via the CSF access chamber. In two patients peritoneal catheter was blocked by a precipitate of vancomycin. Reduction in the dose of intraventricular vancomycin from 50mg to 20mg and increased dilution (additional 2ml of 0.9% N/S solution prevented recurrence of this complication. All patients recovered from ventriculitis within two weeks of initiating this treatment. Follow-up was from 3 months to 2 years to monitor continued relief of hydrocephalus and absence of recurrence of ventriculitis. CONCLUSION: Bhatti CSF access chamber and the ventriculoperitoneal shunt is the only system available at present that fulfills the management of hydrocephalus associated with pyogenic ventriculitis. It provides continuous internal drainage of CSF combined with access to the ventricles for injection of antibiotics.

Hydrocephalus in a yellow-headed Amazon parrot (Amazona ochrocephala oratrix).

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Keller, Krista A; Guzman, David Sanchez-Migallon; Muthuswamy, Anantharaman; Forrest, Lisa J; Steinberg, Howard; Sladky, Kurt; Petersen, Sophie

2011-09-01

A 37-year-old female yellow-headed Amazon parrot (Amazona ochrocephala oratrix) was presented after a 4-month-period behavior change and intermittent episodes of obtunded mentation. Clinical findings on physical examination included ataxia, a weak grasp, and reluctance to move. Results of magnetic resonance imaging were consistent with severe hydrocephalus without evidence of cerebrospinal fluid obstruction. The bird was treated with tapering dosages of prednisolone over a 4-month period, during which time the episodes did not occur. Discontinuation of treatment was attempted several times but resulted in relapse. After 3.5 years of maintenance treatment with prednisolone, the bird was presented subsequent to a 5-hour episode of obtunded mentation and worsening neurologic signs. Despite increasing the dose of prednisolone and providing additional supportive care, the bird's condition worsened, and euthanasia was elected. Necropsy findings included severe hydrocephalus with significant loss of right cerebral parenchyma and no evidence of cerebrospinal fluid obstruction. Histologic examination of the remaining cerebral parenchyma revealed a moderate, multifocal, cellular infiltrate; encephalomalacia; fibrosis; and hemosiderosis in tissue adjacent to the distended ventricles. Other findings included hepatic vacuolar degeneration. Diagnostic imaging and postmortem findings were consistent with a diagnosis of hydrocephalus ex vacuo. To our knowledge, this is the first report of hydrocephalus in an Amazon parrot as well as the first report of hydrocephalus in any avian species associated with long-term follow-up and prolonged corticosteroid treatment.

Stereotactic CO2 laser therapy for hydrocephalus

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Kozodoy-Pins, Rebecca L.; Harrington, James A.; Zazanis, George A.; Nosko, Michael G.; Lehman, Richard M.

1994-05-01

A new fiber-optic delivery system for CO2 radiation has been used to successfully treat non-communicating hydrocephalus. This system consists of a hollow sapphire waveguide employed in the lumen of a stereotactically-guided neuroendoscope. CO2 gas flows through the bore of the hollow waveguide, creating a path for the laser beam through the cerebrospinal fluid (CSF). This delivery system has the advantages of both visualization and guided CO2 laser radiation without the same 4.3 mm diameter scope. Several patients with hydrocephalus were treated with this new system. The laser was used to create a passage in the floor of the ventricle to allow the flow of CSF from the ventricles to the sub-arachnoid space. Initial postoperative results demonstrated a relief of the clinical symptoms. Long-term results will indicate if this type of therapy will be superior to the use of implanted silicone shunts. Since CO2 laser radiation at 10.6 micrometers is strongly absorbed by the water in tissue and CSF, damage to tissue surrounding the lesion with each laser pulse is limited. The accuracy and safety of this technique may prove it to be an advantageous therapy for obstructive hydrocephalus.

Pediatric Hydrocephalus in Ethiopia: Treatment Failures and Infections: A Hospital-Based, Retrospective Study.

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Laeke, Tsegazeab; Tirsit, Abenezer; Biluts, Hagos; Murali, Deeptha; Wester, Knut

2017-04-01

Treatment of pediatric hydrocephalus in sub-Saharan countries is associated with significant complication rates. The objective of this study is to analyze the management of hydrocephalus and complication rates of surgical intervention in the Ethiopian setting to improve future quality. A retrospective cohort study was conducted in a neurosurgical teaching hospital. Two cohorts separated by 2.5 years were analyzed. A total of 128 (58.6% male and 41.4% female) children with isolated hydrocephalus, meningomyelocele (MMC)-related hydrocephalus, or MMC without hydrocephalus were included. Their age ranged from 1 day to 5 years, for a mean age of 7.2 months (median age 2 months). One hundred thirteen patients had hydrocephalus, of whom 57 (44.5.3%) had isolated hydrocephalus and 56 (43.8%) had hydrocephalus associated with MMC. Seventy-seven (74.7%) patients underwent ventriculoperitoneal (VP) shunting, whereas 24 (23.3%) underwent endoscopic third ventriculostomy (ETV). The incidence of shunt infection was 23.4%. Reoperation was needed in 54 (52.4%) patients, with the most common indication being shunt failure. ETV failed in 14 (58.3%) of the 24 patients undergoing ETV. VP shunt insertions had unacceptably high infection rate despite the presence of a protocol for the procedure. Intraoperative guidelines should be developed further and followed strictly to reduce infections. Such measures should include restricting the number of surgeons performing the procedure. In our opinion, one should avoid insertion of a VP shunt as the primary treatment. ETV has proved to be a good alternative in other studies and the decreasing pattern of ETV failure in our study also suggest ETV as a better alternative to VP shunt. Copyright © 2016 The Author(s). Published by Elsevier Inc. All rights reserved.

Biodriven microsystem for treatment of hydrocephalus

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Joswig, Jurgen; Oswald, Jens; Seifert, Steffen

1995-09-01

A microvalve system made of silicon for use in hydrocephalus therapy is presented, which will provide an excellent intracranial pressure stabilization. Design and processing are described. Testing results (in vitro) are presented.

Morphological and Behavioral Changes in the Pathogenesis of a Novel Mouse Model of Communicating Hydrocephalus

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McMullen, Allison B.; Baidwan, Gurlal S.; McCarthy, Ken D.

2012-01-01

The Ro1 model of hydrocephalus represents an excellent model for studying the pathogenesis of hydrocephalus due to its complete penetrance and inducibility, enabling the investigation of the earliest cellular and histological changes in hydrocephalus prior to overt pathology. Hematoxylin and eosin staining, immunofluorescence and electron microscopy were used to characterize the histopathological events of hydrocephalus in this model. Additionally, a broad battery of behavioral tests was used to investigate behavioral changes in the Ro1 model of hydrocephalus. The earliest histological changes observed in this model were ventriculomegaly and disorganization of the ependymal lining of the aqueduct of Sylvius, which occurred concomitantly. Ventriculomegaly led to thinning of the ependyma, which was associated with periventricular edema and areas of the ventricular wall void of cilia and microvilli. Ependymal denudation was subsequent to severe ventriculomegaly, suggesting that it is an effect, rather than a cause, of hydrocephalus in the Ro1 model. Additionally, there was no closure of the aqueduct of Sylvius or any blockages within the ventricular system, even with severe ventriculomegaly, suggesting that the Ro1 model represents a model of communicating hydrocephalus. Interestingly, even with severe ventriculomegaly, there were no behavioral changes, suggesting that the brain is able to compensate for the structural changes that occur in the pathogenesis of hydrocephalus if the disorder progresses at a sufficiently slow rate. PMID:22291910

Alexander Monro Tertius and his works on hydrocephalus.

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Tubbs, R Shane; Tubbs, Isaiah; Loukas, Marios; Oakes, W Jerry

2015-03-01

The Monros of the University of Edinburgh reigned over anatomy instruction for over a century. The last of these men, Monro Tertius, was the weaker teacher of the family but still contributed to the anatomical and surgical literature. Herein, we describe the life of Alexander Monro Tertius and his writings, particularly on childhood hydrocephalus. Monro Tertius will not be remembered as a great anatomist or teacher. However, he collected and published important books on his observations and those of others on hydrocephalus. These texts contained the knowledge of his era on this topic.

Unilateral Pulmonary Artery Agenesis with Ipsilateral Pulmonary Hypoplasia as Incidental Finding in an Asthmatic Patient

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Catalina Contreras-Arias

2014-01-01

Full Text Available Unilateral absence of a pulmonary artery is an uncommon congenital heart disease. It can be related to respiratory symptoms such as asthma, an unsual nding in some of these patients. This paper reports the case of a 4-year-old male with recurrent respiratory infections and asthma symptoms, in who further studies found agenesia of right pulmonary artery with pulmonary hypoplasia of the same side.

Ventriculosubgaleal shunts for posthemorrhagic hydrocephalus in premature infants.

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Willis, Brian K; Kumar, Cherukuri Ravi; Wylen, Esther L; Nanda, Anil

2005-01-01

The early management of posthemorrhagic hydrocephalus in premature infants is challenging and controversial. These infants need a temporary cerebrospinal fluid (CSF) diversion procedure until they gain adequate weight, and the blood and protein levels in CSF are reasonably low before permanent shunt can be placed. Various options are available with their associated advantages and disadvantages. Ventriculosubgaleal shunts have been recommended as a more physiologic and less invasive means of achieving this goal. We have performed this procedure in 6 premature infants to evaluate their effectiveness and complications. Six consecutive premature infants with posthemorrhagic hydrocephalus underwent placement of ventriculosubgaleal shunts over a 1-year period of time. We reviewed their clinical and imaging progress to assess the ability of the shunt to control hydrocephalus and the complication rates. In all 6 patients, the ventriculosubgaleal shunt controlled the progression of hydrocephalus as assessed by clinical and imaging parameters. A permanent shunt was avoided in 1 patient (16.6%). However, 4 patients developed shunt infections, 1 involving the ventriculosubgaleal shunt itself, and 3 immediately after conversion to ventriculoperitoneal shunt. The total infection rate of the series was 66.6%. All infections were caused by staphylococcus species. There was only a 1% shunt infection rate in our institution for all nonventriculosubgaleal shunts during the same period of time. Placement of ventriculosubgaleal shunts for interim CSF diversion in neonates with posthemorrhagic hydrocephalus is effective as a temporary method of CSF diversion. However, our experience has shown that it is associated with a unacceptably high CSF infection rate. A potential cause for infection is CSF stasis just beneath the extremely thin skin of the premature infants, promoting colonization by skin flora. CSF sampling before conversion to a permanent shunt and replacement of the proximal

Social media networking in pediatric hydrocephalus: a point-prevalence analysis of utilization.

Science.gov (United States)

Elkarim, Ghassan Awad; Alotaibi, Naif M; Samuel, Nardin; Wang, Shelly; Ibrahim, George M; Fallah, Aria; Weil, Alexander G; Kulkarni, Abhaya V

2017-08-01

OBJECTIVE A recent survey has shown that caregivers of children with shunt-treated hydrocephalus frequently use social media networks for support and information gathering. The objective of this study is to describe and assess social media utilization among users interested in hydrocephalus. METHODS Publicly accessible accounts and videos dedicated to the topic of hydrocephalus were comprehensively searched across 3 social media platforms (Facebook, Twitter, and YouTube) throughout March 2016. Summary statistics were calculated on standard metrics of social media popularity. A categorization framework to describe the purpose of pages, groups, accounts, channels, and videos was developed following the screening of 100 titles. Categorized data were analyzed using nonparametric tests for statistical significance. RESULTS The authors' search identified 30 Facebook pages, 213 Facebook groups, 17 Twitter accounts, and 253 YouTube videos. These platforms were run by patients, caregivers, nonprofit foundations, and patient support groups. Most accounts were from the United States (n = 196), followed by the United Kingdom (n = 31), Canada (n = 17), India (n = 15), and Germany (n = 12). The earliest accounts were created in 2007, and a peak of 65 new accounts were created in 2011. The total number of users in Facebook pages exceeded those in Facebook groups (p social media use in the topic of hydrocephalus. Users interested in hydrocephalus seek privacy for support communications and are attracted to treatment procedure and surgical products videos. These findings provide insight into potential avenues of hydrocephalus outreach, support, or advocacy in social media.

Hydrocephalus research funding from the National Institutes of Health: a 10-year perspective.

Science.gov (United States)

Gross, Paul; Reed, Gavin T; Engelmann, Rachel; Kestle, John R W

2014-02-01

Funding of hydrocephalus research is important to the advancement of the field. The goal of this paper is to describe the funding of hydrocephalus research from the National Institutes of Health (NIH) over a recent 10-year period. The NIH online database RePORT (Research Portfolio Online Reporting Tools) was searched using the key word "hydrocephalus." Studies were sorted by relevance to hydrocephalus. The authors analyzed funding by institute, grant type, and scientific approach over time. Over $54 million was awarded to 59 grantees for 66 unique hydrocephalus proposals from 48 institutions from 2002 to 2011. The largest sources of funding were the National Institute of Neurological Disease and Stroke and the National Institute of Child Health and Human Development. Of the total, $22 million went to clinical trials, $15 million to basic science, and $10 million to joint ventures with small business (Small Business Innovation Research or Small Business Technology Transfer). Annual funding varied from $2.3 to $8.1 million and steadily increased in the second half of the observation period. The number of new grants also went from 15 in the first 5 years to 27 in the second 5 years. A large portion of the funding has been for clinical trials. Funding for shunt-device development grew substantially. Support for training of hydrocephalus investigators has been low. Hydrocephalus research funding is low compared with that for other conditions of similar health care burden. In addition to NIH applications, researchers should pursue other funding sources. Small business collaborations appear to present an opportunity for appropriate projects.

Benign communicating hydrocephalus in children

International Nuclear Information System (INIS)

Kendall, B.; Holland, I.

1981-01-01

A review was performed of the computed tomograms (CTs) of 500 children which had been reported as showing widening of the supratentorial subarachnoid spaces with normal cerebral substance. On the basis of this a radiological diagnosis of cerebral atrophy had been made in all but five, who were said to have mengalencephaly. From these, the children with large or abnormally enlarging heads, but normal or only slightly enlarged ventricles, were selected; there were 40 such cases (8%). The clinical condition either improved or remained stable over a period of 2 years; in the majority the scan abnormality regressed (22.5%) or remained static (67.5%). In three cases there was slight progression of the CT changes before stabilisation, but only one case developed classical communicating hydrocephalus necessitating a shunt procedure. This condition is a generally benign and mild form of communicating hydrocephalus, for which an aetiological factor was apparent in about two-thirds of the cases studied. (orig.)

Congenital CMV infection: prevalence in newborns and the impact on hearing deficit.

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Engman, Mona-Lisa; Malm, Gunilla; Engstrom, Lotta; Petersson, Karin; Karltorp, Eva; Tear Fahnehjelm, Kristina; Uhlen, Inger; Guthenberg, Claes; Lewensohn-Fuchs, Ilona

2008-01-01

Congenital cytomegalovirus (CMV) infection is asymptomatic in 90% of infected newborns but approximately 10-20% of these infants are at risk of developing sequelae later, mostly hearing deficit. The aims of the study were to investigate the prevalence of congenital CMV infection in a Swedish population of newborns and investigate the relative risk of hearing deficit in newborns with congenital CMV infection. The dried blood spot (DBS) samples of 6060 newborns in southern Stockholm during 12 months (October 2003-June 2004; August 2004-October 2004) were analysed for CMV DNA by TaqMan based real-time PCR. Hearing deficit was assessed by otoacoustic emission (OAE) within a newborn screening programme. 12 infants out of 6060 or 0.2% (95% CI 0.1-0.3%) had congenital CMV infection. One boy among the 12 infected infants had unilateral hearing loss, indicating that the risk of hearing loss is greatly increased (about 20 times) in CMV infected infants. No child developed ocular complications such as chorioretinopathy during 3 y of follow-up. Congenital CMV has an impact on child health but can easily be overlooked due to lack of signs in the neonatal period. Surveillance for congenital CMV is important in addition to programmes for prevention and treatment.

A proposed role for efflux transporters in the pathogenesis of hydrocephalus

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Krishnamurthy, Satish; Tichenor, Michael D.; Satish, Akhila G.; Lehmann, David B.

2014-01-01

Hydrocephalus is a common brain disorder that is treated only with surgery. The basis for surgical treatment rests on the circulation theory. However, clinical and experimental data to substantiate circulation theory have remained inconclusive. In brain tissue and in the ventricles, we see that osmotic gradients drive water diffusion in water-permeable tissue. As the osmolarity of ventricular CSF increases within the cerebral ventricles, water movement into the ventricles increases and causes hydrocephalus. Macromolecular clearance from the ventricles is a mechanism to establish the normal CSF osmolarity, and therefore ventricular volume. Efflux transporters, (p-glycoprotein), are located along the blood brain barrier and play an important role in the clearance of macromolecules (endobiotics and xenobiotics) from the brain to the blood. There is clinical and experimental data to show that macromolecules are cleared out of the brain in normal and hydrocephalic brains. This article summarizes the existing evidence to support the role of efflux transporters in the pathogenesis of hydrocephalus. The location of p-gp along the pathways of macromolecular clearance and the broad substrate specificity of this abundant transporter to a variety of different macromolecules are reviewed. Involvement of p-gp in the transport of amyloid beta in Alzheimer disease and its relation to normal pressure hydrocephalus is reviewed. Finally, individual variability of p-gp expression might explain the variability in the development of hydrocephalus following intraventricular hemorrhage. PMID:25165050

Pathophysiological study of experimental hydrocephalus with computed tomography (CT) scan

International Nuclear Information System (INIS)

Murata, Takaho

1980-01-01

In order to investigate the pathophysiological changes during a development of hydrocephalus, the observations employing computed tomography (CT) scans and monitorings of intracranial epidural pressure (EDP) were performed in a series of kaolin-induced canine hydrocephalus. According to ''volume index'' of ventricles which was calculated from printed-out CT numbers, great individual variations were recognized in the degree of a ventricular enlargement as well as the rate of EDP. They are thought to be due to the difference in types of hydrocephalus, which have been induced by a discrepancy in the site and degree of an obstruction caused by kaolin. Periventricular lucency (PVL) of various degrees were also detected on CT scans of experimental hydrocephalus. It was always marked in the superolateral angle of frontal horn of the lateral ventricles, and differed in degree from severe to mild. PVLs were distinct in the acute stage with high EDP, and gradually became indistinct and had a tendency to disappear thereafter along with decreased EDP. They immediately disappeared after shunting operation. The pathogenesis of PVL was investigated with histological examinations, as well as by using contrast enhancement, Metrizamide ventriculography, the analysis of linear density profiles, and the measurement of regional cerebral blood flow (rCBF). Consequently, PVLs in hydrocephalus are considered to represent an acute edema or a chronic CSF retention in the periventricular white matter caused by increase of water content. In other words, they are regarded as a sign of present or preceding intraventricular hypertension on CT scan, and may become a clinical indication for shunting operation. (author)

Analysis of risk factors for hydrocephalus development in newborn infants with germinal matrix hemorrhage.

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Romero, L; Ros, B; Arráez, M A; Ríus, F; González, L; Martín, A; Carrasco, A; Segura, M

2015-10-01

The aim of this study was to analyze risk factors for the development of hydrocephalus in newborn infants with germinal matrix (GM) hemorrhage. The study comprised 271 patients admitted to Carlos Haya University Hospital in Malaga with GM hemorrhage. The following data were recorded: gestational age, gender, twin birth, head circumference at birth, weight at birth, and Papile grade. Severe obstetrical (abruption, chorioamnionitis, pregnancy-induced hypertension, tocolytic treatment) and neonatal disorders (respiratory distress syndrome, neonatal infection, coagulation disorder, patent ductus arteriosus, necrotizing enterocolitis) were also recorded. Symptomatic hydrocephalus was diagnosed in the event of a progressive increase in head circumference and ventricular indices requiring shunting. Of the 271 patients, 139 (51%) developed posthemorrhagic ventriculomegaly; 47 patients (17%) developed symptomatic hydrocephalus and needed shunt implantation. We found a significant relationship between the development of symptomatic hydrocephalus and Papile grade, lower gestational age, lower birth weight, twin birth, and neonatal infection. Awareness of risk factors for the development of hydrocephalus in newborn infants with GM hemorrhage should be emphasized in order to enable an early diagnosis of ventriculomegaly and symptomatic hydrocephalus and thus make a correct therapeutic decision.

Comparison of micromorphometric testis tissue structures in antenatal fetuses and patients with congenital cryptorchidism

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K. V. Bunkov

2015-01-01

Full Text Available Investigation of paired organs, testicles in particular, in the antenatal period as compared to congenital cryptorchidism in the context of interrelationship can objectively approach the consideration of the whole set of physiological and pathological processes in them, by taking into account histophysiological identity. The paper gives the results of micromorphometric examinations of the units of a communication system (cellular populations and the areas of parenchymatous and stromal structures (intertubular connective tissue, convoluted seminiferous tubules, and spermatogenic epithelium in two groups: 1 34 antenatal (ANT fetuses at 20–41 weeks' gestation and 2 46 children with congenital unilateral cryptorchidism (CR, in whom testis tissue biopsy specimens were examined at the age of 1 to 14 years. Determination of the similarity of micromorphometric structures (the number of cellular population in the intertubular connective tissue, convoluted seminiferous tubules, and the areas of intertubular connective tissue, spermatogenic epithelium, and capillary lumens was analyzed to discover an identity in the testes tissues of the ANT fetuses versus the children with congenital unilateral CR, by considering the position and site of testicles. The findings indicate that there are synchronous changes of individual similar structures between the right and left testicle during its natural descent into the scrotum in the ANT fetuses and in the patients with congenital CR, which is suggestive of the symmetry and relationship between the contralateral sex glands in different ontogenetic groups. This may testify that there are similar processes occurring between the testis tissues in the children with congenital CR and in the ANT fetuses. The findings may become a ground for further consideration of congenital CR in the context of not only the micromorphometry, but also functional activity of tests tissue structures when comparatively analyzing these

CSF Flow in the Brain in the Context of Normal Pressure Hydrocephalus.

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Bradley, W G

2015-05-01

CSF normally flows back and forth through the aqueduct during the cardiac cycle. During systole, the brain and intracranial vasculature expand and compress the lateral and third ventricles, forcing CSF craniocaudad. During diastole, they contract and flow through the aqueduct reverses. Hyperdynamic CSF flow through the aqueduct is seen when there is ventricular enlargement without cerebral atrophy. Therefore, patients presenting with clinical normal pressure hydrocephalus who have hyperdynamic CSF flow have been found to respond better to ventriculoperitoneal shunting than those with normal or decreased CSF flow. Patients with normal pressure hydrocephalus have also been found to have larger intracranial volumes than sex-matched controls, suggesting that they may have had benign external hydrocephalus as infants. While their arachnoidal granulations clearly have decreased CSF resorptive capacity, it now appears that this is fixed and that the arachnoidal granulations are not merely immature. Such patients appear to develop a parallel pathway for CSF to exit the ventricles through the extracellular space of the brain and the venous side of the glymphatic system. This pathway remains functional until late adulthood when the patient develops deep white matter ischemia, which is characterized histologically by myelin pallor (ie, loss of lipid). The attraction between the bare myelin protein and the CSF increases resistance to the extracellular outflow of CSF, causing it to back up, resulting in hydrocephalus. Thus idiopathic normal pressure hydrocephalus appears to be a "2 hit" disease: benign external hydrocephalus in infancy followed by deep white matter ischemia in late adulthood. © 2015 by American Journal of Neuroradiology.

Normal pressure hydrocephalus. Influences on cerebral hemodynamic and cerebrospinal fluid pressure--chemical autoregulation

International Nuclear Information System (INIS)

Meyer, J.S.; Tachibana, H.; Hardenberg, J.P.; Dowell, R.E. Jr.; Kitagawa, Y.; Mortel, K.F.

1984-01-01

Blood flow in the cerebral gray matter was measured in normal pressure hydrocephalus and Alzheimer disease by 133Xe inhalation. Flow values in the frontal and temporal gray matter increased after lowering cerebrospinal fluid (CSF) pressure by lumbar puncture in normal pressure hydrocephalus (p less than 0.05) and also after shunting. One case with cerebral complications did not improve clinically. In Alzheimer disease the reverse (decreases in flow in the gray matter) occurred after removal of CSF. Normal pressure hydrocephalus was associated with impaired cerebral vasomotor responsiveness during 100% oxygen and 5% carbon dioxide inhalation. This complication was restored toward normal after CSF removal and/or shunting. Cerebral blood flow measurements appear to be useful for confirming the diagnosis of normal pressure hydrocephalus and predicting the clinical benefit from shunting

Impact of timing of cranioplasty on hydrocephalus after decompressive hemicraniectomy in malignant middle cerebral artery infarction.

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Finger, Tobias; Prinz, Vincent; Schreck, Evelyn; Pinczolits, Alexandra; Bayerl, Simon; Liman, Thomas; Woitzik, Johannes; Vajkoczy, Peter

2017-02-01

Patients with malignant middle cerebral artery infarction frequently develop hydrocephalus after decompressive hemicraniectomy. Hydrocephalus itself and known shunt related complications after ventriculo-peritoneal shunt implantation may negatively impact patientś outcome. Here, we aimed to identify factors associated with the development of hydrocephalus after decompressive hemicraniectomy in malignant middle cerebral artery infarction. A total of 99 consecutive patients with the diagnosis of large hemispheric infarctions and the indication for decompressive hemicraniectomy were included. We retrospectively evaluated patient characteristics (gender, age and selected preoperative risk factors), stroke characteristics (side, stroke volume and existing mass effect) and surgical characteristics (size of the bone flap, initial complication rate, time to cranioplasty, complication rate following cranioplasty, type of implant, number of revision surgeries and mortality). Frequency of hydrocephalus development was 10% in our cohort. Patients who developed a hydrocephalus had an earlier time point of bone flap reimplantation compared to the control group (no hydrocephalus=164±104days, hydrocephalus=108±52days, phydrocephalus with a trend towards significance (p=0.08). Communicating hydrocephalus is frequent in patients with malignant middle cerebral artery infarction after decompressive hemicraniectomy. A later time point of cranioplasty might lead to a lower incidence of required shunting procedures in general as we could show in our patient cohort. Copyright © 2016 Elsevier B.V. All rights reserved.

Congenital spinal tumor in a patient with encephalocele and hydrocephalus: a case report

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Mahjoub Fatemeh

2011-01-01

Full Text Available Abstract Introduction Encephalocele is a rare congenital abnormality of the central nervous system, where brain tissue protrudes from a defect in the skull. Some anomalies are associated with encephalocele. However, the association of spinal teratoma and encephalocele has not been reported in the English literature. Case presentation We report the case of an Iranian girl with a history of encephalocele surgery, who, at the age of four years, developed an intramedullary spinal teratoma, and discuss the pathogenesis of this association. Conclusion To the best of our knowledge, this is the first report of an association between encephalocele and spinal teratoma.

Severe aseptic meningitis with hydrocephalus following iotrolan myelography: A case report

Energy Technology Data Exchange (ETDEWEB)

Kim, Jae Hyoung; Ha, Choong Kun; Ahn, In Oak [Gyeongsang National University College of Medicine, Jinju (Korea, Republic of)

1993-05-15

A case of severe aseptic meningitis with communicating hydrocephalus following iotrolan myelography is presented. The patient's condition improved very quickly after corticosteroid therapy. Rapid improvement and absence of pathogenic organisms in the CSF culture strongly favor an aseptic meningitis. This is the first reported case of aseptic meningitis with the secondary development of hydrocephalus caused by iotrolan myelography.

Severe aseptic meningitis with hydrocephalus following iotrolan myelography: A case report

International Nuclear Information System (INIS)

Kim, Jae Hyoung; Ha, Choong Kun; Ahn, In Oak

1993-01-01

A case of severe aseptic meningitis with communicating hydrocephalus following iotrolan myelography is presented. The patient's condition improved very quickly after corticosteroid therapy. Rapid improvement and absence of pathogenic organisms in the CSF culture strongly favor an aseptic meningitis. This is the first reported case of aseptic meningitis with the secondary development of hydrocephalus caused by iotrolan myelography

Diagnosis and differential diagnosis of hydrocephalus in adults; Diagnostik und Differenzialdiagnostik des Hydrozephalus beim Erwachsenen

Energy Technology Data Exchange (ETDEWEB)

Langner, Soenke; Mensel, Birger; Kuehn, Jens Peter; Kirsch, Michael [Univ. Medicine Greifswald (Germany). Inst. for Diagnostic Radiology and Neuroradiology; Fleck, Steffen; Baldauf, Joerg [Univ. Medicine Greifswald (Germany). Dept. of Neurosurgery

2017-08-15

Hydrocephalus is caused by an imbalance of production and absorption of cerebrospinal fluid (CSF) or obstruction of its pathways, resulting in ventricular dilatation and increased intracranial pressure. Imaging plays a crucial role in the diagnosis, differential diagnosis and planning of treatment. This review article presents the different types of hydrocephalus und their typical imaging appearance, describes imaging techniques, and discusses differential diagnoses of the different forms of hydrocephalus. Imaging plays a central role in the diagnosis of hydrocephalus. While magnetic resonance (MR) imaging is the first-line imaging modality, computed tomography (CT) is often the first-line imaging test in emergency patients.

Congenital terminal transverse deformity of upper limb: clinical and radiological findings in a sporadic care.

Science.gov (United States)

Malik, Sajid; Afzal, Muhammad

2013-03-01

Congenital transverse limb anomalies are rare, which affect upper and/or lower limbs and may accompany several syndromic malformations. We present a sporadic male subject with congenital, unilateral transverse arrest of the left hand. The affected arm was observed to be short with reduced zeugopod and truncated palm. Fingers were represented by five bead-like nubbins. Roentgenographic examination revealed short radius and ulna with hypoplastic distal heads, absent carpals/metacarpals, and a hypoplastic bony island in each nubbin. Consanguinity was denied, and the subject had no symptoms in the orofacial, neurological and skeletal systems. Detailed clinical data with literature survey is presented.

Hydrocephalus: an underrated long-term complication of microvascular decompression for trigeminal neuralgia. A single institute experience.

Science.gov (United States)

Muratorio, Francesco; Tringali, G; Levi, V; Ligarotti, G K I; Nazzi, V; Franzini, A A

2016-11-01

Hydrocephalus is a common complication of posterior fossa surgery, but its real incidence after microvascular decompression (MVD) for idiopathic trigeminal neuralgia (TN) still remains unclear. The aim of this study was to focus on the potential association between MVD and hydrocephalus as a surgery-related complication. All patients who underwent MVD procedure for idiopathic TN at our institute between 2009 and 2014 were reviewed to search for early or late postoperative hydrocephalus. There were 259 consecutive patients affected by idiopathic TN who underwent MVD procedure at our institution between 2009 and 2014 (113 men, 146 women; mean age 59 years, range 30-87 years; mean follow-up 40.92 months, range 8-48 months). Nine patients (3.47 %) developed communicating hydrocephalus after hospital discharge and underwent standard ventriculo-peritoneal shunt. No cases of acute hydrocephalus were noticed. Our study suggests that late communicating hydrocephalus may be an underrated potential long-term complication of MVD surgery.

Increased self-diffusion of brain water in hydrocephalus measured by MR imaging

International Nuclear Information System (INIS)

Gideon, P.; Thomsen, C.; Gjerris, F.; Soerensen, P.S.; Henriksen, O.

1994-01-01

We used MR imaging to measure the apparent brain water self-diffusion in 5 patients with normal pressure hydrocephalus (NPH), in 2 patients with high pressure hydrocephalus (HPH), and in 8 agematched controls. In all patients with NPH significant elevations of the apparent diffusion coefficients (ADC) of brain water were found within periventricular white matter, in the corpus callosum, in the internal capsule, within cortical gray matter, and in cerebrospinal fluid, whereas normal ADCs were found within the basal ganglia. In 2 patients with HPH elevated ADCs were found most prominently within white matter and in one patient reexamined one year after surgery. ADCs were unchanged in nearly all brain regions. The increased ADC values in hydrocephalus patients may be caused by factors such as changes in myelin-associated bound water, increased Virchow-Robin spaces, and increased extracellular brain water fraction. For further studies of brain water diffusion in hydrocephalus patients, echo-planar imaging techniques with imaging times of a few seconds may be valuable. (orig.)

Acute infarction of corpus callosum due to transient obstructive hydrocephalus.

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Kaymakamzade, Bahar; Eker, Amber

2016-01-01

Acute ischemia of the corpus callosum (CC) is not a well-known feature in patients with acute hydrocephalus. Herein, we describe a case with acute CC infarction due to another rare entity; transient obstructive hydrocephalus. A 66-year-old male was admitted with sudden onset right-sided hemiparesia. CT demonstrated a hematoma on the left basal ganglia with extension to all ventricles. The following day, the patient's neurological status progressed to coma and developed bilateral pyramidal signs. MRI demonstrated obstructive hydrocephalus and acute diffuse infarction accompanied by elevation of the CC. On the same day there was improvement in his neurological status with significant decrease in ventricular size and complete resolution of the clot in the third ventricle. The mechanism of signal abnormalities is probably related with the neural compression of the CC against the falx. Presumably, the clot causing obstruction in the third ventricle dissolved or decayed by the help of fibrinolytic activity of CSF, which was raised after IVH and caused spontaneous improvement of hydrocephalus. Bilateral neurological symptoms suggest diffuse axonal damage and normalization of the intracranial pressure should be performed on the early onset of clinical detorioration in order to prevent axonal injury. Copyright © 2016 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

Evaluation of lung function changes before and after surfactant application during artificial ventilation in newborn rats with congenital diaphragmatic hernia

NARCIS (Netherlands)

E.C. Scheffers; H. IJsselstijn (Hanneke); R. Tenbrinck (Robert); B.F. Lachmann (Burkhard); J.C. de Jongste (Johan); J.C. Molenaar; D. Tibboel (Dick)

1994-01-01

textabstractPatients with congenital diaphragmatic hernia (CDH) have unilateral or bilateral hypoplasia of the lungs including delayed maturation of the terminal air sacs. Because these lungs are highly susceptible to barotrauma and oxygen toxicity, even in full-term newborns, continued research

Communicating Hydrocephalus Associated with Small- to Medium-Sized Vestibular Schwannomas: Clinical Significance of the Tumor Apparent Diffusion Coefficient Map.

Science.gov (United States)

Taniguchi, Masaaki; Nakai, Tomoaki; Kohta, Masaaki; Kimura, Hidehito; Kohmura, Eiji

2016-10-01

The etiology of hydrocephalus associated with the small- to medium-sized vestibular schwannomas is still controversial. We investigated tumor-specific factors related to the association of hydrocephalus with small- to medium-sized vestibular schwannomas. Among the 77 patients with vestibular schwannoma smaller than 30 mm, 9 patients demonstrated associated communicating hydrocephalus. Patient medical records, radiologic data, and histopathologic specimens were reviewed retrospectively. The age of the patients, and size, mean apparent diffusion coefficient (ADC) value, and histologic features of the tumors were compared with those of patients without hydrocephalus. The symptoms related to hydrocephalus improved in all patients after tumor removal. Both the mean size and ADC values exhibited a statistically significant difference between the tumors with and without hydrocephalus (P hydrocephalus. The increased tumor ADC value was considered to be the result of degenerative change and suggested the involvement of protein sloughing in the etiology of the associated hydrocephalus. Copyright © 2016 Elsevier Inc. All rights reserved.

The clinical application and nursing experience of adjustable shunt valve in treatment for patients with normal pressure hydrocephalus

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YANG Li-rong

2012-02-01

Full Text Available Objective To introduce the application of adjustable shunt valve in treatment for patients with normal pressure hydrocephalus. Methods Twenty-four patients with normal pressure hydrocephalus implanted adjustable shunt valve underwent ventriculo-peritoneal shunt surgery and nursing care. Results After operation, cerebrospinal pressure was regulated for 0-6 (1.88 ± 1.52 times. Clinical symptoms were improved, especially in gait disturbance. Conclusion Treatment of normal pressure hydrocephalus with adjustable shunt valve can alleviate symptoms of hydrocephalus. It is especially suitable for patients with short course and secondary normal hydrocephalus patients.

Radioisotope cisternographic evaluation of hydrocephalus: Comparison with CT, MRI, and clinical findings

International Nuclear Information System (INIS)

Kwon, Soon Tae; Park, Cheong Hee; Kim, Hyeong Yeol; Kim, Dae Hong; Shin, Kyung Suk; Cho, June Sik; Lee, Kang Wook; Kim, Jae Moon

1993-01-01

To evaluate the clinical usefulness of radionuclide(RI) cisternography in patients with hydrocephalus, we retrospectively analyzed RI cisternographic findings of 47 patients by using our classification which was modified from Baum's and correlated them with CT(n=37) or MRI(n=10) findings and clinical outcome in selected patients with hydrocephalus(n=37). Modified RI cisternography patterns of 37 patients were type I in three case(8%), type II in seven(18%), type III-A in six(16%), no case of type III-B, type IV-A in 12(32%), and type IV-B in nine(24%). Ri cisternography enabled to differentiate communicating hydrocephalus(27 cases, 73%) from noncommunicating hydrocephalus(10 case, 27%). There was marked clinical improvement in 17 patients(46%), slight improvement in 11 patients(30%), and no improvement in nine patients(24%). The clinical outcome of patients with RI cisternographic type IV-B was worse than that of other types. CT and MRI could neither predict the clinical outcome nor differentiate type IV-B from type IV-A(P>0.05). Ventricular size index(VSI)was significantly higher in patients with type IV than that with other type(p<0.001). RI cisternographic patterns of communicating hydrocephalous were relatively correlated with clinical outcome(r=-0.53, p=.0010 . VIS(r=0.59, p=.001), and dilation of fourth ventricle(r=0.41, p<0.05). We suggest that our modified classification of RI cisternographic patterns can provide more strict physiological assessment of the CFS dynamics and RI cisternography may be still useful to differentiate communicating hydrocephalus from noncommunicating hydrocephalus and to predict the clinical outcome in conjunction with CT/ MR findings and clinical presentation

Hydrocephalus in neuromyelitis optica

OpenAIRE

Clardy, Stacey L.; Lucchinetti, Claudia F.; Krecke, Karl N.; Lennon, Vanda A.; O'Toole, Orna; Weinshenker, Brian G.; Boyd, Clara D.; Krieger, Stephen; McGraw, Corey; Guo, Yong; Pittock, Sean J.

2014-01-01

A majority of patients with neuromyelitis optica (NMO) spectrum disorders (NMOSD) have MRI brain abnormalities, some of which are “NMO-typical” with localization in aquaporin 4 (AQP4)–rich circumventricular and periaqueductal regions.1 Although uncommon in adult patients, symptomatic brain involvement occurs in approximately 50% of NMO–immunoglobulin G (IgG) seropositive children. Here we report the clinical characteristics, type, and frequency of hydrocephalus in NMOSD.

Hydrocephalus: the role of cerebral aquaporin-4 channels and computational modeling considerations of cerebrospinal fluid.

Science.gov (United States)

Desai, Bhargav; Hsu, Ying; Schneller, Benjamin; Hobbs, Jonathan G; Mehta, Ankit I; Linninger, Andreas

2016-09-01

Aquaporin-4 (AQP4) channels play an important role in brain water homeostasis. Water transport across plasma membranes has a critical role in brain water exchange of the normal and the diseased brain. AQP4 channels are implicated in the pathophysiology of hydrocephalus, a disease of water imbalance that leads to CSF accumulation in the ventricular system. Many molecular aspects of fluid exchange during hydrocephalus have yet to be firmly elucidated, but review of the literature suggests that modulation of AQP4 channel activity is a potentially attractive future pharmaceutical therapy. Drug therapy targeting AQP channels may enable control over water exchange to remove excess CSF through a molecular intervention instead of by mechanical shunting. This article is a review of a vast body of literature on the current understanding of AQP4 channels in relation to hydrocephalus, details regarding molecular aspects of AQP4 channels, possible drug development strategies, and limitations. Advances in medical imaging and computational modeling of CSF dynamics in the setting of hydrocephalus are summarized. Algorithmic developments in computational modeling continue to deepen the understanding of the hydrocephalus disease process and display promising potential benefit as a tool for physicians to evaluate patients with hydrocephalus.

Congenital uronephropathy pattern in children

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Husein Alatas

2001-10-01

Full Text Available To obtain the basic data of congenital uronephropathy pattern and the affecting factors in children, we conducted a cross-sectional study at the Department of Child Health Cipto Mangunkusumo (CM Hospital Jakarta from 1995 to 1999 and 9 teaching hospitals throughout Indonesia. During the study period 134 patients were obtained, 116 patients from the CM Hospital and 18 patients from other teaching hospitals. Most patients (48.8% were below 1 year of age; male were affected more than female (2.4:1. The disorder was classified into two groups, i.e., congenital nephropathy and uropathy. There were 10 children with nephropathy, i.e., 4 with unilateral renal hypoplasia, 3 with polycystic kidney, and 3 with renal agenesis. In the uropathy group, 43 were with hypospadia, 22 with primary reflux vesicoureter, 18 with neurogenic bladder, and 17 with ureteropelvic junction obstruction. The complications found were urinary tract infection (71.2%, chronic renal failure (15.7%, hypertension (3.7%, and acute renal failure (1.5%. Consanguinity, familial disorders, maternal diseases, x-ray exposure and abortion efforts were found in a small proportion of patients. History of drug or herbs use in the first trimester of pregnancy was found in a large proportion of patients, mostly took analgesics (especially acetaminophen. In conclusion, uropathy disorders were much more common than congenital nephropathy. The most common complication was urinary tract infection, followed by chronic renal failure, hypertension, and acute renal failure.

Intelligence Quotient (IQ) in Congenital Strabismus.

Science.gov (United States)

Bagheri, Abbas; Fallahi, Mohammad Reza; Tamannaifard, Shima; Vajebmonfared, Sara; Zonozian, Saideh

2013-04-01

To evaluate intelligence quotient (IQ) in patients with congenital strabismus. All patients with congenital strabismus scheduled for surgery were enrolled consecutively over a one year period in a cross-sectional study and were evaluated for verbal, performance and total IQ scores, and compared to the mean normal IQ of 100±15. During the study period, 109 patients with mean age of 18.4±10.5 (range, 4-63) years were included. Educational status in most patients (80%) was less than high-school. Most patients (80%) lived in urban areas and 46 patients (42.2%) had some degrees of unilateral or bilateral amblyopia. Mean verbal IQ was 87.2±19.6 (range, 45-127), performance IQ was 81±15.5 (range, 44-111) and total IQ was 83.5±18.3 (range, 40-120). Total IQ was significantly lower in comparison to the normal population (PIQ levels. Verbal IQ was insignificantly higher in myopes than emmetropes and hyperopes. IQ was better with vertical deviations and was higher in esotropes than exotropes; however, these differences were not statistically significant (P>0.05 for all comparisons). Patients with congenital strabismus in this study had lower mean IQ scores than the normal population which may be due to genetic background or acquired causes secondary to strabismus.

A characteristic ventricular shape in myelomeningocele-associated hydrocephalus? A CT stereology study

International Nuclear Information System (INIS)

Roost, D. van; Solymosi, L.; Funke, K.

1995-01-01

We measured the volume of the supratentorial ventricles in 39 consecutive children with myelomeningocele (MMC) and associated hydrocephalus, using a stereological method based on the Cavalieri theorem of systematic sampling. We distinguished the following groups: newborns before and after cerebrospinal fluid shunting (14), a somewhat larger group of newborns with an untreated MMC-associated hydrocephalus (25) and a group of shunted children at a mean age of 1.5 years (28). We paid special attention to the shape of the lateral ventricles, looking separately at the anterior and posterior halves. The measurements were compared with a healthy control group (10) and with children with hydrocephalus unrelated to MMC (15). The average volume ratio of the posterior to the anterior half of the lateral ventricles was 1.05 ± 0.39 in non-hydrocephalic children, 1.11 ± 0.55 in untreated hydrocephalic children without MMC, and 2.15 ± 0.65 in MMC-associated hydrocephalus prior to shunting. These ratios did not change significantly after shunting. This confirms our impression that MMC-associated hydrocephalus shows a characteristic shape, with a disproportionate enlargement of the posterior part of the lateral ventricles, in clear contrast to the normal-width frontal horns. This shape is reminiscent of the fetal ventricular shape. It reveals disturbance of brain development in children with MMC, which goes beyond the classic description of the Chiari malformation. (orig.)

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.

Hypoxic ischemia encephalopathy leading to external hydrocephalus and the cerebral atrophy: mechanism and differential diagnosis

International Nuclear Information System (INIS)

Huang Zhenglin; Mo Xiaorong

2002-01-01

Objective: It is a study of the mechanism and differential diagnosis of the infant external hydrocephalus and cerebral atrophy. Methods: In total 84 cases of neonatal hypoxic ischemia encephalopathy followed by infant external hydrocephalus were investigated, among which 26 patients gradually were found having developed cerebral atrophy in follow up. Results: Characteristic dilation of the frontal-parietal subarachnoid space and the adjacent cistern was noted on the CT images of the external hydrocephalus. CT revealed the enlarged ventricle besides the dilated subarachnoid space in the cases of cerebral atrophy, while these two entities were indistinguishable on CT in the early stage. Conclusion: Clinical manifestations make a major differential diagnosis of the external hydrocephalus and cerebral atrophy: tic and mild delayed development of locomotion over major presentation of external hydrocephalus, while cerebral atrophy is featured by remarkable dysnoesia and severe delayed development of locomotion. In addition, hemiplegia and increased muscular tension are presented in a few cases of cerebral atrophy

Application of MR virtual endoscopy in children with hydrocephalus.

Science.gov (United States)

Zhao, Cailei; Yang, Jian; Gan, Yungen; Liu, Jiangang; Tan, Zhen; Liang, Guohua; Meng, Xianlei; Sun, Longwei; Cao, Weiguo

2015-12-01

To evaluate the performance of MR virtual endoscopy (MRVE) in children with hydrocephalus. Clinical and imaging data were collected from 15 pediatric patients with hydrocephalus and 15 normal control children. All hydrocephalus patients were confirmed by ventriculoscopy or CT imaging. The cranial 3D-T1 weighted imaging data from fast spoiled gradient echo scan (FSPGR) were transported to working station. VE images of cerebral ventricular cavity were constructed with Navigator software. Cerebral ventricular MRVE can achieve similar results as ventriculoscopy in demonstrating the morphology of ventricular wall or intracavity lesion. In addition, MRVE can observe the lesion from distal end of obstruction, as well as other areas that are inaccessible to ventriculoscopy. MRVE can also reveal the pathological change of ventricular inner wall surface, and help determine patency of the cerebral aqueduct and fourth ventricle outlet. MR virtual endoscopy provides a non-invasive diagnostic modality that can be used as a supplemental approach to ventriculoscopy. However, its sensitivity and specificity need to be determined in the large study. Copyright © 2015 Elsevier Inc. All rights reserved.

A case of congenital dermal sinus of the occipital region detected by CT at the onset of meningitis

International Nuclear Information System (INIS)

Fujiwara, Katsuhiko; Esumi, Noriko; Nishimura, Osamu

1985-01-01

A 4-year-old girl who was found to have congenital dermal sinus of the occipital region by CT at the second onset of meningitis is reported. CT at the first onset of meningitis (at the age of 3 years and 9 months) revealed only small, oval low density areas in the posterior fossa of the occipital region. CT at the second onset showed an abscess in the posterior fossa and dissociation in the posterior part of the cranium. Skin examination in the occipital region disclosed induration of the median area. Thus, congenital dermal sinus was suspected. Further CT disclosed that the patient subsequently developed hydrocephalus despite the intravenous and intrameningeal administration of antibiotics. Therefore, she underwent excision of a cyst and was pathologically diagnosed as having dermoid associated with abscess in the tip of the dermal sinus, which was followed by induration of the occipital skin. (Namekawa, K.)

Case report: Isolated unilateral pulmonary vein atresia diagnosed on 128-slice multidetector CT

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Rashmi Dixit

2011-01-01

Full Text Available Unilateral pulmonary venous atresia is an uncommon entity that is generally believed to be congenital. Most patients present in infancy or childhood with recurrent chest infections or hemoptysis. Pulmonary angiography is usually used for definitive diagnosis. However, the current multislice CT scanners may obviate the need for pulmonary angiography. We report two cases diagnosed using 128-slice CT angiography. On the CT angiography images both these cases demonstrated absent pulmonary veins on the affected side, with a small pulmonary artery and prominent bronchial or other systemic arterial supply.

Everolimus Alleviates Obstructive Hydrocephalus due to Subependymal Giant Cell Astrocytomas.

Science.gov (United States)

Moavero, Romina; Carai, Andrea; Mastronuzzi, Angela; Marciano, Sara; Graziola, Federica; Vigevano, Federico; Curatolo, Paolo

2017-03-01

Subependymal giant cell astrocytomas (SEGAs) are low-grade tumors affecting up to 20% of patients with tuberous sclerosis complex (TSC). Early neurosurgical resection has been the only standard treatment until few years ago when a better understanding of the molecular pathogenesis of TSC led to the use of mammalian target of rapamycin (mTOR) inhibitors. Surgical resection of SEGAs is still considered as the first line treatment in individuals with symptomatic hydrocephalus and intratumoral hemorrhage. We describe four patients with symptomatic or asymptomatic hydrocephalus who were successfully treated with the mTOR inhibitor everolimus. We collected the clinical data of four consecutive patients presenting with symptomatic or asymptomatic hydrocephalus due to a growth of subependymal giant cell atrocytomas and who could not undergo surgery for different reasons. All patients experienced a clinically significant response to everolimus and an early shrinkage of the SEGA with improvement in ventricular dilatation. Everolimus was well tolerated by all individuals. Our clinical series demonstrate a possible expanding indication for mTOR inhibition in TSC, which can be considered in patients with asymptomatic hydrocephalus or even when the symptoms already appeared. It offers a significant therapeutic alternative to individuals that once would have undergone immediate surgery. Everolimus might also allow postponement of a neurosurgical resection, making it elective with an overall lower risk. Copyright © 2016 Elsevier Inc. All rights reserved.

Edaravone reduces astrogliosis and apoptosis in young rats with kaolin-induced hydrocephalus.

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Garcia, Camila Araújo Bernardino; Catalão, Carlos Henrique Rocha; Machado, Hélio Rubens; Júnior, Ivair Matias; Romeiro, Thais Helena; Peixoto-Santos, José Eduardo; Santos, Marcelo Volpon; da Silva Lopes, Luiza

2017-03-01

We investigated the possible neuroprotective effects of the free radical scavenger edaravone in experimental hydrocephalus. Seven-day-old Wistar rats were divided into three groups: control group (C), untreated hydrocephalic (H), and hydrocephalic treated with edaravone (EH). The H and EH groups were subjected to hydrocephalus induction by 20% kaolin intracisternal injection. The edaravone (20 mg/kg) was administered daily for 14 days from the induction of hydrocephalus. All animals were daily weighed and submitted to behavioral test and assessment by magnetic resonance imaging. After 14 days, the animals were sacrificed and the brain was removed for histological, immunohistochemical, and biochemical studies. The gain weight was similar between groups from the ninth post-induction day. The open field test performance of EH group was better (p  0.01), germinal matrix (p > 0.05), and cerebral cortex (p > 0.05), as compared to H group. We have demonstrated that administration of edaravone for 14 consecutive days after induction of hydrocephalus reduced astrocyte activity and that it has some beneficial effects over apoptotic cell death.

Hydrocephalus is a rare outcome in community-acquired bacterial meningitis in adults

DEFF Research Database (Denmark)

Bodilsen, Jacob; Schønheyder, Henrik Carl; Nielsen, Henrik I

2013-01-01

BACKGROUND: Community-acquired bacterial meningitis (CABM) continues to have a high mortality rate and often results in severe sequelae among survivors. Lately, an increased effort has been focused on describing the neurological complications of meningitis including hydrocephalus. To aid in this ......BACKGROUND: Community-acquired bacterial meningitis (CABM) continues to have a high mortality rate and often results in severe sequelae among survivors. Lately, an increased effort has been focused on describing the neurological complications of meningitis including hydrocephalus. To aid...... in this field of research we set out to ascertain the risk and outcome of hydrocephalus in patients with community-acquired bacterial meningitis (CABM) in North Denmark Region. METHODS: We conducted a retrospective population-based cohort study of CABM cases above 14 years of age. Cases diagnosed during a 13......-year period, 1998 through 2010, were identified in a laboratory register and data were acquired through patient records. Cases not confirmed by culture met other strict inclusion criteria. The diagnosis of hydrocephalus relied upon the radiologists' reports on cranial imaging. Outcome was graded...

Evaluation of mandibular condyles in children with unilateral posterior crossbite

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Edson ILLIPRONTI-FILHO

2015-01-01

Full Text Available The relationship of mandibular condyle dimensions and its association with unilateral posterior crossbite (UPXB has been suggested in the literature. The purpose of this prospective study was to evaluate mandibular condyles on the left and right sides and between crossed and non-crossed sides in the sagittal and coronal planes, using cone-beam computed tomography (CBCT. Twenty CBCT images of 40 temporo mandibular joints (TMJs in individuals in mixed dentition phase, which included 9 males (mean 7.9 years and 11 females (mean 8.2 years, with unilateral posterior crossbite without premature contacts and functional mandibular shifts and with transverse maxillary deficiency. The criteria for sample exclusion included the presence of painful symptoms, facial trauma history, systemic diseases such as juvenile rheumatoid arthritis, mouth opening limitation (< 40 mm, congenital or genetic anomalies, and skeletal asymmetries that may result in TMJ disorders. Dimensional measurements of the condyles between the right and left sides and crossed and non-crossed sides in sagittal and coronal view were made. There was no significant difference between the measurements of the crossed and non-crossed sides in both sagittal and coronal view. These findings suggest that the presence of unilateral posterior crossbite in children with UPXB did not result in changes between the mandibular condyles in the right and left sides or between the crossed and non-crossed sides in the coronal or sagittal plane.

High signal intensity of the septum pellucidum at MR imaging; Significance in hydrocephalus

International Nuclear Information System (INIS)

Yoon, Jeong Hee; Kim, Eun Ha; Chung, Chun Phil; Kim, Chang Soo

1994-01-01

To evaluate the significance of high signal intensity of the septum pellucidum in hydrocephalus on proton density-weighted brain MR images. Authors reviewed the MR images of 418 cases of patients with normal (175 case), hydrocephalic(35 cases), atrophic(58 cases), and other groups(150 cases) retrospectively. We analyzed the signal intensity of the septum pellucidum in the normal group and the incidences of high signal intensities of periventricular area of frontal horn of lateral ventricle(area 1), periventricular area except area 1 (area 2), callososeptal area(area 3), and septum pellucidum(area 4) in the normal and abnormal groups. In the normal group, the septum pellucidum was isointense to the head of caudate nucleus on proton density-weighted image. High signal intensity of the septum pellucidum was seen in 31 cases (22 cases of hydrocephalus, 5 cases of brain atrophy, and 4 cases of others), and showed high specificity(91.4%) for hydrocephalus in spite of low sensitivity(62.9%), as compared with periventricular hyperintensities of other areas. High signal intensity of the septum pellucidum on proton density- weighted image may be caused by transependymal CSF migration in the patients with hydrocephalus, and considered as an additional finding of hydrocephalus in the cases of ventriculomegaly

Obstructive hydrocephalus secondary to odontoid pannus: case report and review of literature.

Science.gov (United States)

Soon, Wai Cheong; Thanabalasundaram, Gopiga; Thant, Kyaw Zayar; Ogbonnaya, Ebere Sunny; Harrisson, Stuart Edward

2018-04-01

Odontoid pannus or periodontoid pseudotumour is associated with a variety of rheumatological conditions. We report a case of an 80-year old man who presented with acute hydrocephalus following an emergency operation to amputate his infected left big toe. Imaging revealed a large tissue mass causing severe compression at the cervico-medullary junction and obstruction of CSF flow. The acute hydrocephalus was presumed to be related to neck manoeuvring during general anaesthesia. Following an initial emergency CSF diversion through external ventricular drainage catheter insertion, the patient subsequently underwent ventriculo-peritoneal insertion and posterior spinal decompression and fixation. To our knowledge, there have been no reported cases of tophaceous gout of the odontoid pannus causing acute hydrocephalus.

Visual outcome after endoscopic third ventriculostomy for hydrocephalus.

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Jung, Ji-Ho; Chai, Yong-Hyun; Jung, Shin; Kim, In-Young; Jang, Woo-Youl; Moon, Kyung-Sub; Kim, Seul-Kee; Chong, Sangjoon; Kim, Seung-Ki; Jung, Tae-Young

2018-02-01

Hydrocephalus-related symptoms are mostly improved after successful endoscopic third ventriculostomy (ETV). However, visual symptoms can be different. This study was focused on visual symptoms. We analyzed the magnetic resonance images (MRI) of the orbit and visual outcomes. From August 2006 to November 2016, 50 patients with hydrocephalus underwent ETV. The male-to-female ratio was 33:17, and the median age was 61 years (range, 5-74 years). There were 18 pediatric and 32 adult patients. Abnormal orbital MRI findings included prominent subarachnoid space around the optic nerves and vertical tortuosity of the optic nerves. We retrospectively analyzed clinical symptoms, causes of hydrocephalus, ETV success score (ETVSS), ETV success rate, ETV complications, orbital MRI findings, and visual impairment score (VIS). The median duration of follow-up was 59 months (range, 3-113 months). The most common symptoms were headache, vomiting, and gait disturbance. Visual symptoms were found in 6 patients (12%). The most common causes of hydrocephalus were posterior fossa tumor in 13 patients, pineal tumor in 12, aqueductal stenosis in 8, thalamic malignant glioma in 7, and tectal glioma in 4. ETVSS was 70 in 3 patients, 80 in 34 patients, and 90 in 13 patients. ETV success rate was 80%. ETVSS 70 showed the trend in short-term survival compared to ETVSS 90 and 80. ETV complications included epidural hematoma requiring operation in one patient, transient hemiparesis in two patients, and infection in two patients. Preoperative abnormal orbital MRI findings were found in 18 patients and postoperative findings in 7 patients. Four of six patients with visual symptoms had abnormal MR findings. Three patients did not show VIS improvement, including two with severe visual symptoms. Patients with severe visual impairment were found to have bad outcomes. The visual symptoms related with increased intracranial pressure should be carefully monitored and controlled to improve outcomes.

Magnetic resonance velocity mapping of 3D cerebrospinal fluid flow dynamics in hydrocephalus: preliminary results

International Nuclear Information System (INIS)

Stadlbauer, Andreas; Salomonowitz, Erich; Brenneis, Christian; Ungersboeck, Karl; Riet, Wilma van der; Buchfelder, Michael; Ganslandt, Oliver

2012-01-01

To investigate the detectability of CSF flow alterations in the ventricular system of patients with hydrocephalus using time-resolved 3D MR velocity mapping. MR velocity mapping was performed in 21 consecutive hydrocephalus patients and 21 age-matched volunteers using a 3D phase-contrast (PC) sequence. Velocity vectors and particle path lines were calculated for visualisation of flow dynamics. CSF flow was classified as ''hypomotile flow'' if it showed attenuated dynamics and as ''hypermotile flow'' if it showed increased dynamics compared with volunteers. Diagnostic efficacy was compared with routine 2D cine PC-MRI. Seven patients showed hypomotile CSF flow: six had non-communicating hydrocephalus due to aqueductal stenosis. One showed oscillating flow between the lateral ventricles after craniotomy for intracranial haemorrhage. Seven patients showed normal flow: six had hydrocephalus ex vacuo due to brain atrophy. One patient who underwent ventriculostomy 10 years ago showed a flow path through the opening. Seven patients showed hypermotile flow: three had normal pressure hydrocephalus, three had dementia, and in one the diagnosis remained unclear. The diagnostic efficacy of velocity mapping was significantly higher except for that of aqueductal stenosis. Our approach may be useful for diagnosis, therapy planning, and follow-up of different kinds of hydrocephalus. (orig.)

Magnetic resonance velocity mapping of 3D cerebrospinal fluid flow dynamics in hydrocephalus: preliminary results

Energy Technology Data Exchange (ETDEWEB)

Stadlbauer, Andreas [Landesklinikum St. Poelten, MR Physics Group, Department of Radiology, St. Poelten (Austria); University of Erlangen-Nuremberg, Department of Neurosurgery, Erlangen (Germany); Salomonowitz, Erich [Landesklinikum St. Poelten, MR Physics Group, Department of Radiology, St. Poelten (Austria); Brenneis, Christian [Landesklinikum St. Poelten, Department of Neurology, St. Poelten (Austria); Ungersboeck, Karl [Landesklinikum St. Poelten, Department of Neurosurgery, St. Poelten (Austria); Riet, Wilma van der [European MRI Consultancy (EMRIC), Strasbourg (France); Buchfelder, Michael; Ganslandt, Oliver [University of Erlangen-Nuremberg, Department of Neurosurgery, Erlangen (Germany)

2012-01-15

To investigate the detectability of CSF flow alterations in the ventricular system of patients with hydrocephalus using time-resolved 3D MR velocity mapping. MR velocity mapping was performed in 21 consecutive hydrocephalus patients and 21 age-matched volunteers using a 3D phase-contrast (PC) sequence. Velocity vectors and particle path lines were calculated for visualisation of flow dynamics. CSF flow was classified as ''hypomotile flow'' if it showed attenuated dynamics and as ''hypermotile flow'' if it showed increased dynamics compared with volunteers. Diagnostic efficacy was compared with routine 2D cine PC-MRI. Seven patients showed hypomotile CSF flow: six had non-communicating hydrocephalus due to aqueductal stenosis. One showed oscillating flow between the lateral ventricles after craniotomy for intracranial haemorrhage. Seven patients showed normal flow: six had hydrocephalus ex vacuo due to brain atrophy. One patient who underwent ventriculostomy 10 years ago showed a flow path through the opening. Seven patients showed hypermotile flow: three had normal pressure hydrocephalus, three had dementia, and in one the diagnosis remained unclear. The diagnostic efficacy of velocity mapping was significantly higher except for that of aqueductal stenosis. Our approach may be useful for diagnosis, therapy planning, and follow-up of different kinds of hydrocephalus. (orig.)

Surgical management of arrested hydrocephalus: Case report, literature review, and 18-month follow-up.

Science.gov (United States)

Hong, Jennifer; Barrena, Benjamin G; Lollis, S Scott; Bauer, David F

2016-12-01

Arrested hydrocephalus is stable ventriculomegaly without evidence of neurologic deterioration or symptoms. Management of arrested hydrocephalus in asymptomatic adults is controversial, with little clinical data. This case highlights the potential for decompensation in adults with arrested hydrocephalus and reviews the literature regarding pathophysiology and management of this clinical entity. A 39 year-old gentleman with arrested hydrocephalus incidentally found during work-up for new-onset seizure and managed conservatively for ten years presented with increasing headache, memory loss, gait instability and urinary and fecal incontinence. Stable massive triventriculomegaly was documented on serial brain imaging, and ophthalmologic exam revealed no papilledema. The patient underwent endoscopic third ventriculostomy with immediate post-operative improvement of headache, resolution of incontinence, and cessation of seizures. At 15 months after surgery, neuropsychiatric testing demonstrated improvement in visuomotor skills, problem solving, verbal fluency and cognitive flexibility compared to his pre-operative baseline. At 18 months after surgery he remained seizure free with full continence and significant improvement in headaches. Early recognition of arrested hydrocephalus and its potential for decompensation may prompt surgical treatment and prevent neurologic deterioration. Copyright © 2016 Elsevier B.V. All rights reserved.

Unilateral renal agenesia in the angiographic material and renovascular hypertension

Energy Technology Data Exchange (ETDEWEB)

Stojanov, D.; Lovasic, I.; Dujmovic, M.; Bobinac, D.

1987-06-01

Analysis of 1216 abdominal aortographies and selective renovasographies undertaken at the Institute of Radiology, Clinical Hospital Centre Rijeka during the period 1979-1985, was performed. 39 (3,2%) unilateral renal agenesias were established, a percentage that is significantly higher than reported by other authors. Analysis of all angiographic parameters of a single kidney was also worked out. A significanlty high percentage (66,7%) of hypertension was diagnosed in this group. Congenital renal failures make their appearance in the early embryological development and are discovered, if compatible with life, most frequently at an advanced age. Statistical data on the frequency during a lifetime are less reliable because a great number of anomalies are never discovered during a person's life. The etiology of variations and congenital failure of renal arteries is unknown in 90% of the cases, and most of the malformations are without characteristic symptomatology. Since the anatomic relations in anomalous kidney are disturbed, the sign of the disease can develop atypical forms causing frequent errors in diagnosis. The kidney is supplied by numerous lateral branches of the medial sacral artery, i.e. the aorta, during embryological development. Later, some of them degenerate or mutually connect themselves, and the definitive kidney has been usually penetrated by only one artery and one vein on the same side (1). If this is not so, i.e. when the obliterations of caudal metanephogenic arteries are only partially present, then the renal arteries emerge from the iliac or lumbar arteries (2,3). Bilateral renal agenesia is extreme and incompatible with life, and hence of small clinical importance. Unilateral agenesia makes its appearance according to various statistics and authors from the obductional materials in ratio 1:1000 (4,5), clinical ones 1:5000 (6, 7, 8) and the angiographically 1:76(9), more exactly 1:35 cases (10).

Unilateral renal agenesia in the angiographic material and renovascular hypertension

International Nuclear Information System (INIS)

Stojanov, D.; Lovasic, I.; Dujmovic, M.; Bobinac, D.

1987-01-01

Analysis of 1216 abdominal aortographies and selective renovasographies undertaken at the Institute of Radiology, Clinical Hospital Centre Rijeka during the period 1979-1985, was performed. 39 (3,2%) unilateral renal agenesias were established, a percentage that is significantly higher than reported by other authors. Analysis of all angiographic parameters of a single kidney was also worked out. A significanlty high percentage (66,7%) of hypertension was diagnosed in this group. Congenital renal failures make their appearance in the early embryological development and are discovered, if compatible with life, most frequently at an advanced age. Statistical data on the frequency during a lifetime are less reliable because a great number of anomalies are never discovered during a person's life. The etiology of variations and congenital failure of renal arteries is unknown in 90% of the cases, and most of the malformations are without characteristic symptomatology. Since the anatomic relations in anomalous kidney are disturbed, the sign of the disease can develop atypical forms causing frequent errors in diagnosis. The kidney is supplied by numerous lateral branches of the medial sacral artery, i.e. the aorta, during embryological development. Later, some of them degenerate or mutually connect themselves, and the definitive kidney has been usually penetrated by only one artery and one vein on the same side (1). If this is not so, i.e. when the obliterations of caudal metanephogenic arteries are only partially present, then the renal arteries emerge from the iliac or lumbar arteries (2,3). Bilateral renal agenesia is extreme and incompatible with life, and hence of small clinical importance. Unilateral agenesia makes its appearance according to various statistics and authors from the obductional materials in ratio 1:1000 (4,5), clinical ones 1:5000 (6, 7, 8) and the angiographically 1:76(9), more exactly 1:35 cases (10). (orig.)

X-linked hydrocephalus : A novel missense mutation in the L1CAM gene

NARCIS (Netherlands)

Sztriha, L; Vos, YJ; Verlind, E; Johansen, J; Berg, B

2002-01-01

X-linked hydrocephalus is associated with mutations in the L1 neuronal cell adhesion molecule gene. L1 protein plays a key role in neurite outgrowth, axonal guidance, and pathfinding during the development of the nervous system. A male is described with X-linked hydrocephalus who had multiple small

Global surgery for pediatric hydrocephalus in the developing world: a review of the history, challenges, and future directions.

Science.gov (United States)

Muir, Ryan T; Wang, Shelly; Warf, Benjamin C

2016-11-01

OBJECTIVE Pediatric hydrocephalus is one of the most common neurosurgical conditions and is a major contributor to the global burden of surgically treatable diseases. Significant health disparities exist for the treatment of hydrocephalus in developing nations due to a combination of medical, environmental, and socioeconomic factors. This review aims to provide the international neurosurgery community with an overview of the current challenges and future directions of neurosurgical care for children with hydrocephalus in low-income countries. METHODS The authors conducted a literature review around the topic of pediatric hydrocephalus in the context of global surgery, the unique challenges to creating access to care in low-income countries, and current international efforts to address the problem. RESULTS Developing countries face the greatest burden of pediatric hydrocephalus due to high birth rates and greater risk of neonatal infections. This burden is related to more general global health challenges, including malnutrition, infectious diseases, maternal and perinatal risk factors, and education gaps. Unique challenges pertaining to the treatment of hydrocephalus in the developing world include a preponderance of postinfectious hydrocephalus, limited resources, and restricted access to neurosurgical care. In the 21st century, several organizations have established programs that provide hydrocephalus treatment and neurosurgical training in Africa, Central and South America, Haiti, and Southeast Asia. These international efforts have employed various models to achieve the goals of providing safe, sustainable, and cost-effective treatment. CONCLUSIONS Broader commitment from the pediatric neurosurgery community, increased funding, public education, surgeon training, and ongoing surgical innovation will be needed to meaningfully address the global burden of untreated hydrocephalus.

Acute hydrocephalus in nonaneurysmal perimesencephalic hemorrhage: evidence of CSF block at the tentorial hiatus

NARCIS (Netherlands)

Rinkel, G. J.; Wijdicks, E. F.; Vermeulen, M.; Tans, J. T.; Hasan, D.; van Gijn, J.

1992-01-01

The acute hydrocephalus in patients with nonaneurysmal perimesencephalic hemorrhage suggests an extraventricular obstruction of CSF flow. We studied the occurrence of acute hydrocephalus and the site of cisternal blood in 40 consecutive patients with perimesencephalic hemorrhage. In all 11 patients

Hydrocephalus associated with subarachnoid hemorrhage

International Nuclear Information System (INIS)

Nosaka, Yoshiki

1981-01-01

Thirteen patients exhibited a communicating hydrocephalus following subarachnoid hemorrhage secondary to ruptured intracranial aneurysms and were treated with shunt procedures. The interval between subarachnoid hemorrhage and surgery averaged 9 weeks. Seven of the patients showed improvement. The prognostic value for surgical management was evaluated on the basis of three different diagnostic examinations (computed tomography (CT), cisternography and constant infusion test). A correct diagnosis was obtained in 78 per cent in cisternography, and 63 per cent in infusion test and CT. All patients responding to surgery showed a typical pattern in cisternography, consisting of ventricular retention of radiopharmaceutical tracer for 48 h or longer in association with no radioactivity over the cerebral hemispheres. The constant infusion test correlated well with typical cisternographic patterns. CT is useful in demonstrating pathophysiological changes in hydrocephalus. Periventricular hypodensity was visible in patients with normal or slightly elevated intracranial pressure, accompanied by fairly rapid deterioration. All of them responded well to shunting. In most cases which benefited from the shunt, the postoperative CT showed not only normal-sized ventricles but also marked regression of the hypodensity over a short period. (author)

Control volume based hydrocephalus research

Science.gov (United States)

Cohen, Benjamin; Voorhees, Abram; Wei, Timothy

2008-11-01

Hydrocephalus is a disease involving excess amounts of cerebral spinal fluid (CSF) in the brain. Recent research has shown correlations to pulsatility of blood flow through the brain. However, the problem to date has presented as too complex for much more than statistical analysis and understanding. This talk will highlight progress on developing a fundamental control volume approach to studying hydrocephalus. The specific goals are to select physiologically control volume(s), develop conservation equations along with the experimental capabilities to accurately quantify terms in those equations. To this end, an in vitro phantom is used as a simplified model of the human brain. The phantom's design consists of a rigid container filled with a compressible gel. The gel has a hollow spherical cavity representing a ventricle and a cylindrical passage representing the aquaducts. A computer controlled piston pump supplies pulsatile volume fluctuations into and out of the flow phantom. MRI is used to measure fluid velocity, and volume change as functions of time. Independent pressure measurements and flow rate measurements are used to calibrate the MRI data. These data are used as a framework for future work with live patients.

Changes in Rat Brain Tissue Microstructure and Stiffness during the Development of Experimental Obstructive Hydrocephalus

Science.gov (United States)

Jugé, Lauriane; Pong, Alice C.; Bongers, Andre; Sinkus, Ralph; Bilston, Lynne E.; Cheng, Shaokoon

2016-01-01

Understanding neural injury in hydrocephalus and how the brain changes during the course of the disease in-vivo remain unclear. This study describes brain deformation, microstructural and mechanical properties changes during obstructive hydrocephalus development in a rat model using multimodal magnetic resonance (MR) imaging. Hydrocephalus was induced in eight Sprague-Dawley rats (4 weeks old) by injecting a kaolin suspension into the cisterna magna. Six sham-injected rats were used as controls. MR imaging (9.4T, Bruker) was performed 1 day before, and at 3, 7 and 16 days post injection. T2-weighted MR images were collected to quantify brain deformation. MR elastography was used to measure brain stiffness, and diffusion tensor imaging (DTI) was conducted to observe brain tissue microstructure. Results showed that the enlargement of the ventricular system was associated with a decrease in the cortical gray matter thickness and caudate-putamen cross-sectional area (P hydrocephalus development, increased space between the white matter tracts was observed in the CC+PVWM (P hydrocephalus development. PMID:26848844

Neuropathology and Structural Changes in Hydrocephalus

Science.gov (United States)

Del Bigio, Marc R.

2010-01-01

In the context of spina bifida, hydrocephalus is usually caused by crowding of the posterior fossa with obstruction to cerebrospinal fluid flow from the forth ventricle, and less often by malformation of the cerebral aqueduct. Enlargement of the cerebral ventricles causes gradual destruction of periventricular white matter axons. Motor, sensory,…

Congenital hydrocephalus following X-irradiation of pregnant rats on an early gestational day

International Nuclear Information System (INIS)

Takeuchi, I.K.; Takeuchi, Y.K.

1986-01-01

When pregnant rats were X-irradiated at a dose of 100 R on gestational day 9.5, a considerable number of postnatally-viable hydrocephalic offspring resulted, all of which were accompanied with bilateral micro- or anophthalmia. Histological studies revealed that the cerebral aqueduct of the congenital hydrocephalic brain was severely stenosed, and the subcommissural organ was reduced in size and displaced at some distance from the anterior end of the cerebral aqueduct. From embryological studies, it was considered that the maldevelopment of the subcommissural organ in the X-irradiated fetus might cause a reduction in the amount of its secretions which function as a cushion preventing complete closure of the cerebral aqueduct during fetal life, resulting in stenosis of the cerebral aqueduct

Acquired Encephalocele With Hydrocephalus and Pineal Region Epidermoid Cyst.

Science.gov (United States)

Toktaş, Zafer Orkun; Yilmaz, Baran; Ekşi, Murat Şakir; Bayoumi, Ahmed B; Akakin, Akin; Yener, Yasin; Demir, Mustafa Kemal; Kiliç, Türker

2016-07-01

A combination of trauma and a missed inflammatory response (nasal operation) concomitant with hydrocephalus and tumor in secondary encephalocele has not been described in the English literature yet. A 38-year-old man was admitted to the clinic with rhinorrhea that started 3 months ago. In his medical history, nothing abnormal was present except a nasal operation performed 1 year ago. Brain magnetic resonance imaging depicted left frontal encephalocele concomitant with obstructive hydrocephalus caused by an epidermoid cyst originated from the pineal region. A 2-staged surgery was planned. In the first stage, a ventriculoperitoneal shunt insertion was conveyed successfully. In the second-stage surgery, the herniated brain tissue was excised, and the frontal sinus was cleansed with serum saline combined with antibiotic. The bony defect and the dura defect were repaired. The patient's presenting complaint recovered fully, and he was discharged to home in a well condition. Acquired encephalocele is a rare entity. In case of rhinorrhea and encephalocele, even in the presence of prior history of nasal surgery, intracranial evaluation should be conveyed to exclude the presence of hydrocephalus and/or tumor. The cranial defect should be repaired to prevent future infections and brain tissue damage.

Surgical management of hydrocephalus secondary to intraventricular hemorrhage in the preterm infant.

Science.gov (United States)

Christian, Eisha A; Melamed, Edward F; Peck, Edwin; Krieger, Mark D; McComb, J Gordon

2016-03-01

OBJECT Posthemorrhagic hydrocephalus (PHH) in the preterm infant remains a major neurological complication of prematurity. The authors first described insertion of a specially designed low-profile subcutaneous ventricular catheter reservoir for temporary management of hydrocephalus in 1983. This report presents the follow-up experience with the surgical management of PHH in this population and describes outcomes both in infants who were stable for permanent shunt insertion and those initially temporized with a ventricular reservoir (VR) prior to permanent ventriculoperitoneal (VP)/ventriculoatrial (VA) shunt placement. METHODS A retrospective review was undertaken of the medical records of all premature infants surgically treated for posthemorrhagic hydrocephalus (PHH) between 1997 and 2012 at Children's Hospital Los Angeles. RESULTS Over 14 years, 91 preterm infants with PHH were identified. Fifty neonates received temporizing measures via a VR that was serially tapped for varying time periods. For the remaining 41 premature infants, VP/VA shunt placement was the first procedure. Patients with a temporizing measure as their initial procedure had undergone CSF diversion significantly earlier in life than those who had permanent shunting as the initial procedure (29 vs 56 days after birth, p hydrocephalus and shunt infection did not statistically differ between the 2 groups. CONCLUSIONS Patients with initial VR insertion as a temporizing measure received a CSF diversion procedure significantly earlier than those who received a permanent shunt as their initial procedure. Otherwise, the outcomes with regard to shunt revisions, loculated hydrocephalus, and shunt infection were not different for the 2 groups.

Radionuclide blood levels during cisternography of patients with normal-pressure hydrocephalus or Alzheimer's disease

International Nuclear Information System (INIS)

Mahaley, M.S. Jr.; Wilkinson, R.H. Jr.; Sivalingham, S.; Friedman, H.; Tyson, W.; Goodrich, J.K.

1974-01-01

Various diagnostic procedures were compared during investigations of 37 dementia patients undergoing differential study for normal-pressure hydrocephalus or Alzheimer's disease. A diminished radionuclide level in the blood, with abnormal cisternography and pneumoencephalography, provided the most valuable diagnostic evidence of normal-pressure hydrocephalus. (U.S.)

Hydrocephalus induces dynamic spatiotemporal regulation of aquaporin-4 expression in the rat brain

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Praetorius Jeppe

2010-11-01

Full Text Available Abstract Background The water channel protein aquaporin-4 (AQP4 is reported to be of possible major importance for accessory cerebrospinal fluid (CSF circulation pathways. We hypothesized that changes in AQP4 expression in specific brain regions correspond to the severity and duration of hydrocephalus. Methods Hydrocephalus was induced in adult rats (~8 weeks by intracisternal kaolin injection and evaluated after two days, one week and two weeks. Using magnetic resonance imaging (MRI we quantified lateral ventricular volume, water diffusion and blood-brain barrier properties in hydrocephalic and control animals. The brains were analysed for AQP4 density by western blotting and localisation by immunohistochemistry. Double fluorescence labelling was used to study cell specific origin of AQP4. Results Lateral ventricular volume was significantly increased over control at all time points after induction and the periventricular apparent diffusion coefficient (ADC value significantly increased after one and two weeks of hydrocephalus. Relative AQP4 density was significantly decreased in both cortex and periventricular region after two days and normalized after one week. After two weeks, periventricular AQP4 expression was significantly increased. Relative periventricular AQP4 density was significantly correlated to lateral ventricular volume. AQP4 immunohistochemical analysis demonstrated the morphological expression pattern of AQP4 in hydrocephalus in astrocytes and ventricular ependyma. AQP4 co-localized with astrocytic glial fibrillary acidic protein (GFAP in glia limitans. In vascular structures, AQP4 co-localized to astroglia but not to microglia or endothelial cells. Conclusions AQP4 levels are significantly altered in a time and region dependent manner in kaolin-induced hydrocephalus. The presented data suggest that AQP4 could play an important neurodefensive role, and may be a promising future pharmaceutical target in hydrocephalus and CSF

Obstructive Hydrocephalus Secondary to Enlarged Virchow-Robin Spaces: A Rare Cause of Pulsatile Tinnitus.

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Donaldson, Christopher; Chatha, Gurkirat; Chandra, Ronil V; Goldschlager, Tony

2017-05-01

Obstructive hydrocephalus secondary to enlarged Virchow-Robin Spaces (VRS) is a rare entity, with only a few cases reported in the literature. Presenting symptoms vary widely from headaches to dizziness. We report a case of a 31-year-old man who presented with pulsatile tinnitus and magnetic resonance imaging showing obstructive hydrocephalus secondary to tumefactive VRS. After a cerebrospinal fluid diversion procedure in the form of an endoscopic third ventriculostomy, he had almost complete resolution of his symptoms. This is the first case of obstructive hydrocephalus secondary to enlarged VRS, presenting with pulsatile tinnitus. Copyright © 2017 Elsevier Inc. All rights reserved.

Cine MR CSF flow study in hydrocephalus: What are the valuable parmeters?

International Nuclear Information System (INIS)

Choi, Hye Young; Kim, Myung Hyun

1997-01-01

To evaluate the changes in diagnosis of intracranial cerebrospinal fluid (CSF) dynamics in the hydrocephalus, we studied the various parameters of cine phase contrast (PC) magnetic resonance (MR) CSF flow images in cases of acutely progressive hydrocephalus, comparing them with those in normal CSF circulation. The MR images were obtained with a 1.5T (GE Signa, GE Medical Systems, Milwaukee, USA) unit using the 2 dimensional cine PC sequence with peripheral gating and gradient recalled echo imaging in ten cases of non-obstructive hydrocephalus(NOH), three of obstructive hydrocephalus(OH), and ten controls. The time-echo time-flip angle employed were 50 to 80 msec-11 to 15 msec-12 to 15 degrees. Temporal velocity information relating to cervical pericord CSF spaces, third and fourth ventricles, and the aqueduct were plotted as wave forms which were then analyzed for configuration, amplitude parameters(Vmax, Vmin, Vdif), and temporal parameters(R-S, R-SMV, R-D, R-DMV). The statistical significance of each parameter was examined using the paired t-test. All patients with OH underwent endoscopic third ventriculostomy, whereas all with NOH underwent shunting procedures. In five ROIs, distinct reproducible configuration features were obtained at aqueduct and cervical pericord spaces, but not at ventricular levels. We determined the statistically significant differences between controls and hydrocephalus patients using temporal, rather than amplitude parameters. In NOH, the graph showed R-DMV shortening (p<0.01) at the anterior cervical pericord space. In OH, there were R-DMV shortening (p<0.05) was seen at the anterior cervical pericord space, and R-DMV shortening (p<0.02) at the posterior cervical pericord space. In one case of OH, a typical change of configuration, mirror image, was obtained at aqueduct level, and in all OH cases, the level of obstruction could be determined. The results of cine PC MR CSF flow study may be valuable for points for determining the level

Repeated peritoneal catheter blockage caused by neurocysticercosis following ventriculoperitoneal shunt placement for hydrocephalus

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Zhi Hua Li

2018-01-01

Full Text Available Cerebral cysticercosis is common, but the possibility for repeated occurrence of peritoneal catheter blockage caused by neurocysticercosis (NCC after two revisions following ventriculoperitoneal shunt placement for hydrocephalus is unusual. Herein, we describe one rare case in which peritoneal catheter revision was performed two times unsuccessfully. Endoscopic cysternostomy rather than peritoneal catheter adjustment was performed successfully, and histopathological examination of excised cystic samples confirmed NCC in our hospital. The present case highlights the need for awareness of NCC as a possible etiology of hydrocephalus, especially in developing countries. Uncommon findings in both lateral ventricles following low-field magnetic resonance imaging scans as well as the rarity of this infection involved in unusual location play important roles in misdiagnosis and incorrect treatment for hydrocephalus; thus, endoscopic cysternostomy, rather than multiple shunt adjustment of the peritoneal end, is recommended in the selected patient. To the best of our knowledge, this is the first report describing the misdiagnosis and inappropriate treatment of hydrocephalus caused by cerebral cysticercosis in China.

Prevalence of dental anomalies in children with cleft lip and unilateral and bilateral cleft lip and palate.

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Rullo, R; Festa, V M; Rullo, R; Addabbo, F; Chiodini, P; Vitale, M; Perillo, L

2015-09-01

To examine the prevalence of different types of dental anomalies in children with nonsyndromic cleft lip, unilateral cleft lip-palate, and bilateral cleft lip-palate. A sample of 90 patients (aged 4-20 years) affected by isolated cleft lip, unilateral and bilateral cleft lip and palate was examined. Cleft patients were classified into one of three groups according to cleft type: (1) Unilateral Cleft Lip-Palate, (2) Bilateral Cleft Lip-Palate, and (3) Cleft Lip. Intraoral exams, panoramic radiographs and dental casts, were used to analyse the prevalence of the various dental anomalies included in this study. There were no statistically significant differences between patients with cleft lip, unilateral cleft lip and palate and bilateral cleft lip and palate. The congenital absence of the cleft-side lateral incisor was observed in 40% of the sample, and a total of 30% patients showed supernumerary teeth at the incisors region. Second premolar agenesis was found in 4.4% of patients, whereas in 18.9% of the sample there was an ectopic dental eruption. Lateral or central incisors rotation was noted in 31.1% of the sample, while shape anomaly, lateral incisor microdontia, and enamel hypoplasia were detected respectively in 25.6%, 5.6% and 18.9% of cleft patients. High prevalence of different dental anomalies in children with cleft lip and unilateral and bilateral cleft lip and palate has been confirmed. This study, in particular, shows the presence of ectopic and rotated teeth in the cleft area.

Diffusion-weighted imaging in the diagnostic evaluation of the hydrocephalus in patients with acute or chronic increase in cerebral pressure

International Nuclear Information System (INIS)

Dorenbeck, U.; Schlaier, J.; Feuerbach, S.; Seitz, J.

2005-01-01

Purpose: to investigate whether diffusion-weighted imaging (DWI) in magnetic resonance imaging (MRI) provides additional information about the periventricular white matter for the assessment of hydrocephalus. Materials and methods: sixteen MRI examinations (11 with acutely increased cerebral pressure, 5 without symptoms) on 15 patients with hydrocephalus (4 patients with communicating hydrocephalus and 11 patients with obstructive hydrocephalus) were analyzed. One symptomatic patient subsequently became asymptomatic. We investigated the ''apparent diffusion coefficient'' (ADC) in the subcortical and periventricular white matter. The ADCs of the study patients were compared with those of a healthy control group. Results: symptomatic patients with hydrocephalus, 6/11 showed periventricular edema and a significantly higher ADC values in the periventricular region than in the subcortical white matter. 5/11 symptomatic patients showed significantly higher ADC values even in the absence of periventricular interstitial edema (both groups contained patients with communicating and obstructive hydrocephalus). All 5 asymptomatic patients with hydrocephalus did not have a significantly higher ADC values in the periventricular region. Conclusion: in patients with hydrocephalus and acutely increased cerebral pressure, DWI showed a significantly higher ADC values in the periventricular region even without visible interstitial edema on conventional MRI sequences. (orig.)

Hydrocephalus secondary to obstruction of the lateral apertures in two dogs.

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Kent, M; Glass, E N; Haley, A C; Shaikh, L S; Sequel, M; Blas-Machado, U; Bishop, T M; Holmes, S P; Platt, S R

2016-11-01

Traditionally, hydrocephalus is divided into communicating or non-communicating (obstructive) based on the identification of a blockage of cerebrospinal fluid (CSF) flow through the ventricular system. Hydrocephalus ex vacuo refers to ventricular enlargement as a consequence of neuroparenchymal loss. Hydrocephalus related to obstruction of the lateral apertures of the fourth ventricles has rarely been described. The clinicopathologic findings in two dogs with hydrocephalus secondary to obstruction of the lateral apertures of the fourth ventricle are reported. Signs were associated with a caudal cervical spinal cord lesion in one dog and a caudal brain stem lesion in the other dog. Magnetic resonance imaging (MRI) disclosed dilation of the ventricular system, including the lateral recesses of the fourth ventricle. In one dog, postmortem ventriculography confirmed obstruction of the lateral apertures. Microscopic changes were identified in the choroid plexus in both dogs, yet a definitive cause of the obstructions was not identified. The MRI findings in both dogs are similar to membranous occlusion of the lateral and median apertures in human patients. MRI detection of dilation of the entire ventricular system in the absence of an identifiable cause should prompt consideration of an obstruction of the lateral apertures. In future cases, therapeutic interventions aimed at re-establishing CSF flow or ventriculoperitoneal catheterisation should be considered. © 2016 Australian Veterinary Association.

Left hemisphere structural connectivity abnormality in pediatric hydrocephalus patients following surgery.

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Yuan, Weihong; Meller, Artur; Shimony, Joshua S; Nash, Tiffany; Jones, Blaise V; Holland, Scott K; Altaye, Mekibib; Barnard, Holly; Phillips, Jannel; Powell, Stephanie; McKinstry, Robert C; Limbrick, David D; Rajagopal, Akila; Mangano, Francesco T

2016-01-01

Neuroimaging research in surgically treated pediatric hydrocephalus patients remains challenging due to the artifact caused by programmable shunt. Our previous study has demonstrated significant alterations in the whole brain white matter structural connectivity based on diffusion tensor imaging (DTI) and graph theoretical analysis in children with hydrocephalus prior to surgery or in surgically treated children without programmable shunts. This study seeks to investigate the impact of brain injury on the topological features in the left hemisphere, contratelateral to the shunt placement, which will avoid the influence of shunt artifacts and makes further group comparisons feasible for children with programmable shunt valves. Three groups of children (34 in the control group, 12 in the 3-month post-surgery group, and 24 in the 12-month post-surgery group, age between 1 and 18 years) were included in the study. The structural connectivity data processing and analysis were performed based on DTI and graph theoretical analysis. Specific procedures were revised to include only left brain imaging data in normalization, parcellation, and fiber counting from DTI tractography. Our results showed that, when compared to controls, children with hydrocephalus in both the 3-month and 12-month post-surgery groups had significantly lower normalized clustering coefficient, lower small-worldness, and higher global efficiency (all p  hydrocephalus surgically treated with programmable shunts.

Fractionated Stereotactic Radiotherapy in the Treatment of Vestibular Schwannoma (Acoustic Neuroma): Predicting the Risk of Hydrocephalus

International Nuclear Information System (INIS)

Powell, Ceri; Micallef, Caroline; Gonsalves, Adam; Wharram, Bev; Ashley, Sue; Brada, Michael

2011-01-01

Purpose: To determine the incidence and predictive factors for the development of hydrocephalus in patients with acoustic neuromas (AN) treated with fractionated stereotactic radiotherapy. Patients and Methods: Seventy-two patients with AN were treated with fractionated stereotactic radiotherapy between 1998 and 2007 (45-50 Gy in 25-30 fractions over 5 to 6 weeks). The pretreatment MRI scan was assessed for tumor characteristics and anatomic distortion independently of subsequent outcome and correlated with the risk of hydrocephalus. Results: At a median follow-up of 49 months (range, 1-120 months), 5-year event-free survival was 95%. Eight patients (11%) developed hydrocephalus within 19 months of radiotherapy, which was successfully treated. On univariate analysis, pretreatment factors predictive of hydrocephalus were maximum diameter (p = 0.005), proximity to midline (p = 0.009), displacement of the fourth ventricle (p = 0.02), partial effacement of the fourth ventricle (p < 0.001), contact with the medulla (p = 0.005), and more brainstem structures (p = 0.004). On multivariate analysis, after adjusting for fourth ventricular effacement, no other variables remained independently associated with hydrocephalus formation. Conclusions: Fractionated stereotactic radiotherapy results in excellent tumor control of AN, albeit with a risk of developing hydrocephalus. Patients at high risk, identified as those with larger tumors with partial effacement of the fourth ventricle before treatment, should be monitored more closely during follow-up. It would also be preferable to offer treatment to patients with progressive AN while the risk of hydrocephalus is low, before the development of marked distortion of fourth ventricle before tumor diameter significantly exceeds 2 cm.

Periventricular hypodensity in communicating hydrocephalus studied by metrizamide CT cisternography

International Nuclear Information System (INIS)

Hiratsuka, Hideo; Okada, Kodai; Takasato, Yoshio; Inaba, Yutaka; Tsuyumu, Matsutaira.

1979-01-01

Modification of periventricular hypodensity in suspected communicating hydrocephalus was investigated by metrizamide CT cisternography. Six to eight ml of metrizamide isotonic solution with a concentration of 170 mgI/ml was given through lumbar puncture and scanning was conducted 1, 3, 6, 24 and 48 hrs after injection. In addition to visual analysis of images, mean value and standard deviation were calculated for Hounsfield units of the regions of interest at the anterolateral angle of frontal horn and the time courses of the attenuation values were statistically compared to each other at each scanning time. The results were classified into four groups: 1) periventricular low density zone with gradual penetration of metrizamide therein; 2) periventricular low density zone without penetration of metrizamide therein; 3) minimal periventricular low density zone with minimal metrizamide penetration; and 4) no periventricular low density zone and no penetration of metrizamide. The presence of a periventricular low density zone with increase of the density after ventricular reflux of metrizamide would be an important criterion for CSF shunting operation in normal pressure hydrocephalus or communicating hydrocephalus. (author)

Lumbar cisternography in evaluation of hydrocephalus in the preterm infant

International Nuclear Information System (INIS)

Donn, S.M.; Roloff, D.W.; Keyes, J.W. Jr.

1983-01-01

Radionuclide lumbar cisternography using indium 111-diethylenetriamine pentaacetic acid (111In-DTPA) and a mobile gamma-camera with a converging collimator was utilized as a bedside procedure to evaluate CSF dynamics and the patency of the cerebral ventricular system in 30 preterm infants with hydrocephalus. Serial images of the brain were obtained at 0, 1, 2, 6, 24, and 48 hours after instillation of the isotope in the lumbar subarachnoid space. Three distinct patterns were seen. Infants with posthemorrhagic hydrocephalus displayed prompt ventricular filling but markedly delayed emptying with minimal flow over the cerebral convexities. Infants with ventriculomegaly secondary to suspected brain atrophy or periventricular leukomalacia demonstrated a pattern of prompt ventricular filling, delayed emptying, but with flow present over the convexities. An infant with noncommunicating hydrocephalus secondary to an Arnold-Chiari malformation showed a pattern of complete obstruction with no ventricular filling. Radionuclide lumbar cisternography appears to be a safe, well-tolerated procedure which produces images of sufficient resolution to provide valuable information about CSF dynamics, delineating basal cisternae, ventricles, and subarachnoid flow paths

Dyke-Davidoff-Masson syndrome: case report of fetal unilateral ventriculomegaly and hypoplastic left middle cerebral artery.

Science.gov (United States)

Piro, Ettore; Piccione, Maria; Marrone, Gianluca; Giuffrè, Mario; Corsello, Giovanni

2013-05-14

Prenatal ultrasonographic detection of unilateral cerebral ventriculomegaly arises suspicion of pathological condition related to cerebrospinal fluid flow obstruction or cerebral parenchimal pathology. Dyke-Davidoff-Masson syndrome is a rare condition characterized by cerebral hemiatrophy, calvarial thickening, skull and facial asymmetry, contralateral hemiparesis, cognitive impairment and seizures. Congenital and acquired types are recognized and have been described, mainly in late childhood, adolescence and adult ages. We describe a female infant with prenatal diagnosis of unilateral left ventriculomegaly in which early brain MRI and contrast enhanced-MRI angiography, showed cerebral left hemiatrophy associated with reduced caliber of the left middle cerebral artery revealing the characteristic findings of the Dyke-Davidoff-Masson syndrome. Prenatal imaging, cerebral vascular anomaly responsible for the cerebral hemiatrophy and the early clinical evolution have never been described before in such a young child and complete the acquired clinical descriptions in older children. Differential diagnosis, genetic investigations, neurophysiologic assessments, short term clinical and developmental follow up are described. Dyke-Davidoff-Masson syndrome must be ruled out in differential diagnosis of fetal unilateral ventriculomegaly. Early clinical assessment, differential diagnosis and cerebral imaging including cerebral MRI angiography allow the clinicians to diagnose also in early infancy this rare condition.

Flow-regulated versus differential pressure-regulated shunt valves for adult patients with normal pressure hydrocephalus

DEFF Research Database (Denmark)

Ziebell, Morten; Wetterslev, Jørn; Tisell, Magnus

2013-01-01

Since 1965 many ventriculo-peritoneal shunt systems have been inserted worldwide to treat hydrocephalus. The most frequent indication in adults is normal pressure hydrocephalus (NPH), a condition that can be difficult to diagnose precisely. Surgical intervention with flow-regulated and differential...

Neurodevelopmental long-term outcome in children with hydrocephalus requiring neonatal surgical treatment.

Science.gov (United States)

Melot, A; Labarre, A; Vanhulle, C; Rondeau, S; Brasseur, M; Gilard, V; Castel, H; Marret, S; Proust, F

2016-04-01

To assess long-term neurodevelopmental outcome in children with hydrocephalus requiring neurosurgical treatment during the neonatal period. This prospective longitudinal population-based study included 43 children with neonatal shunted hydrocephalus. The 43 children were prospectively reviewed in the presence of their parents at the outpatient clinic. Cognitive and motor outcomes were assessed respectively using different Wechsler scales according to age and Gross Motor Function Classification System (GMFCS). Postoperative MRI was routinely performed. The mean gestational age at birth of the 43 consecutive children with neonatal hydrocephalus (sex ratio M/F: 1.39) was 34.5±5.4 weeks of gestation. At mean follow-up of 10.4±4 years, mean total IQ was 73±27.7, with equivalent results in mean verbal and mean performance IQ. Of the 33 children with IQ evaluation, 18 presented an IQ≥85 (41.9%). Efficiency in walking without a mobility device (GMFCS≤2) was obtained in 37 children (86%). Only severity of postoperative ventricular dilation was significantly associated with unfavorable outcome (Evans index>0.37; odds ratio: 0.16, P=0.03). This information could be provided to those families concerned who often experience anxiety when multi-disciplinary management of neonatal hydrocephalus is required. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Effects of Melatonin on the Cerebellum of Infant Rat Following Kaolin-Induced Hydrocephalus: a Histochemical and Immunohistochemical Study.

Science.gov (United States)

Uyanıkgil, Yiğit; Turgut, Mehmet; Baka, Meral

2017-02-01

Hydrocephalus is a developmental disorder causing abnormally collected cerebrospinal fluid within the cerebral ventricles. It leads to bigger skulls and many dysfunctions related to the nervous system. Here, we addressed whether exogenous melatonin administration could reverse the clinical features of kaolin-induced hydrocephalus in infantile rats. A controlled double-blinded study was conducted in 2-week-old 45 Wistar albino rats, which were divided into three groups: Group A, the control group, received intracisternal sham injection with solely the needle insertion; group B, the hydrocephalus group, was treated with isotonic NaCl after kaolin injection; and group C, the hydrocephalus + melatonin group, was given i.p. exogenous melatonin at a dose of 0.5 mg/100 g body weight after kaolin injection. Histological and immunohistochemical analyses were performed after the induction of hydrocephalus and melatonin administration. Glial fibrillary acidic protein was stained by immunohistochemical method. TUNEL method was used to define and quantitate apoptosis in the cerebellar tissues. Statistical analysis was performed by nonparametric Kruskal-Wallis H test, and once significance was determined among means, post hoc pairwise comparisons were carried out using Mann-Whitney U test. We found that melatonin administration significantly ameliorated ratio of substantia grisea area/substantia alba area in the cerebellum of infantile rats. Histologically, there was a significant reduction in the number of cerebellar apoptotic cells after the hydrocephalus induced by kaolin (P cerebellum were reversed by systemic melatonin administration in infantile rats with kaolin-induced hydrocephalus. Nevertheless, further studies are needed to suggest melatonin as a candidate protective drug in children with hydrocephalus.

Contemporary management and surveillance strategy after shunt or endoscopic third ventriculostomy procedures for hydrocephalus.

Science.gov (United States)

Janjua, M Burhan; Hoffman, Caitlin E; Souweidane, Mark M

2017-11-01

The management of hydrocephalus can be challenging even in expert hands. Due to acute presentation, recurrence, accompanying complications, the need for urgent diagnosis; a robust management plan is an absolute necessity. We devised a novel time efficient surveillance strategy during emergency, and clinic follow up settings which has never been described in the literature. We searched all articles embracing management/surveillance protocol on pediatric hydrocephalus utilizing the terms "hydrocephalus follow up" or "surveillance protocol after hydrocephalus treatment". The authors present their own strategy based on vast experience in the hydrocephalus management at a single institution. The need for the diagnostic laboratory testing, age and presentation based radiological imaging, significance of neuro-opthalmological exam, and when to consider the emergent exploration have been discussed in detail. Moreover, a definitive triaging strategy has been described with the help of flow chart diagrams for clinicians, and the neurosurgeons in practice. The triage starts from detail history, physical exam, necessary labs, radiological imaging depending on the presentation, and the age of the child. A quick head CT scan helps after shunt surgery while, a FAST sequence MRI scan (fsMRI) is important in post ETV patients. The need for neuro-opthalmological exam, and the shunt series stays vital in asymptomatic patients during regular follow up. Copyright © 2017 Elsevier Ltd. All rights reserved.

Amblyopia after unilateral infantile cataract extraction after six weeks of age Ambliopia no pós-operatório de catarata congênita unilateral operada após seis semanas de vida

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Fábio Ejzenbaum

2009-10-01

Full Text Available PURPOSE: To determine interocular grating acuity difference in children treated for unilateral infantile cataract. METHODS: A group of 27 children previously treated for unilateral infantile cataract, had their monocular visual acuity measured by sweep visual evoked potentials. Interocular grating acuity difference was calculated as the absolute subtraction of monocular acuity scores. Lens status, opacity severity and eye alignment were considered for analysis. RESULTS: Mean interocular grating acuity difference obtained from unilateral cataract patients was 0.58 ± 0.20 logMAR. This result was significantly larger than 0.10 logMAR used as normative data. Children with severe opacities had a more pronounced amblyopia than the moderate ones. No significant correlation between amblyopia and strabismus or aphakia was found. CONCLUSIONS: Interocular acuity difference in this group of unilateral congenital cataract was more pronounced than previous reports, mainly because of delay in diagnosis, surgery and optical correction.OBJETIVOS: Determinar a diferença interocular da acuidade visual de resolução de grades em crianças operadas de catarata congênita unilateral. MÉTODOS: Um grupo de 27 pacientes operados de catarata congênita unilateral tiveram mensurada sua acuidade visual monocular pelo potencial visual evocado de varredura. A diferença interocular foi calculada pela subtração absoluta das acuidades monoculares. A intensidade da opacificação, implante ou não de lente intraocular e presença de estrabismo foram consideradas para análise. RESULTADOS: A média da diferença interocular foi de 0,58 ± 0,20 logMAR. Esse resultado foi significantemente maior que 0,10 logMAR, valor considerado como média normal nos estudos normativos. Crianças com opacidades intensas tiveram ambliopia mais pronunciada que os casos moderados. Não houve correlação significante entre a intensidade da ambliopia com estrabismo e afacia. CONCLUSÕES: A

Hydrocephalus following severe traumatic brain injury in adults. Incidence, timing, and clinical predictors during rehabilitation

DEFF Research Database (Denmark)

Kammersgaard, Lars Peter; Linnemann, Mia; Tibæk, Maiken

2013-01-01

To investigate timing and clinical predictors that might predict hydrocephalus emerging during rehabilitation until 1 year following severe traumatic brain injury (TBI).......To investigate timing and clinical predictors that might predict hydrocephalus emerging during rehabilitation until 1 year following severe traumatic brain injury (TBI)....

[Constitute, imaging and auditory characteristics of pediatric patients with congenital malformations of inner ear in sensorineural hearing loss].

Science.gov (United States)

Li, Youjin; Yang, Jun; Li, Yun

2011-01-01

To analyze the constitute, imaging and auditory characteristics of pediatric patients with congenital malformations of inner ear in sensorineural hearing loss(SNHL). Imaging and auditory data of 125 cases (225 ears) in 860 pediatric patients with congenital SNHL who referred to ENT department of Shanghai Children Medical Center from February 2005 to January 2010 were retrospectively studied. Congenital malformations of inner ear accounted for 14.5% in 860 pediatric patients with congenital SNHL. Bilateral ear was involved in 98 cases (78.4%), unilateral in 27 cases (21.6%). One hundred and sixty-seven ears (74.2%) were identified as profound deafness, 36 ears (16%) severe deafness and 22 ears (9.8%) moderate deafness in 225 ears, respectively. In present group, large vestibular aqueduct (75.6%) was the most common and next was vestibular deformity (32%), then cochleovestibular deformity (23.1%). Mondini deformity (55.8%) was the most common and common cavity deformity (28.9%) next in cochleovestibular deformity. Profound deafness in cochlea involved congenital malformations of inner ear was significantly more than those in no cochlea involved malformations. The results are of importance for understanding the constitute of congenital malformations of inner ear in pediatric patients with congenital SNHL in China, for etiological diagnosis of congenital SNHL, for intervention including hearing aids or cochlear implant and prognosis.

Craniectomy-associated Progressive Extra-Axial Collections with Treated Hydrocephalus (CAPECTH): redefining a common complication of decompressive craniectomy.

Science.gov (United States)

Nalbach, Stephen V; Ropper, Alexander E; Dunn, Ian F; Gormley, William B

2012-09-01

Extra-axial fluid collections following decompressive craniectomy have been observed in a variety of patient populations. These collections have traditionally been thought to represent extra-axial signs of hydrocephalus, but they often occur even in settings where hydrocephalus has been optimally treated. This study aims to elucidate the phenomenon of extra-axial fluid collections after decompressive craniectomy in patients with treated hydrocephalus, in order to improve identification, classification, prevention and treatment. We retrospectively reviewed all patients at a single institution undergoing decompressive craniectomy for refractory intracranial pressure elevations from June 2007 through December 2009. We identified 39 patients by reviewing clinical reports and imaging. Any patient who died on or prior to the third post-operative day (POD) was excluded. The analysis focused on patients with extra-axial collections and treated hydrocephalus. Twenty-one of 34 (62%) patients developed extra-axial collections and 18 of these developed collections despite ventricular drainage. Subgroup analysis revealed that seven of seven patients (100%) with subarachnoid hemorrhage, and 11 of 14 (79%) with traumatic brain injury developed collections. Extra-axial collections may develop after decompressive craniectomy despite aggressive treatment of communicating hydrocephalus. In these patients, the term "external hydrocephalus" does not appropriately capture the relevant pathophysiology. Instead, we define a new phenomenon, "Craniectomy-associated Progressive Extra-Axial Collections with Treated Hydrocephalus" (CAPECTH), as progressive collections despite aggressive cerebral spinal fluid (CSF) drainage. Our data indicate that early cranioplasty can help prevent the formation and worsening of this condition, presumably by returning normal CSF dynamics. Copyright © 2012 Elsevier Ltd. All rights reserved.

Mental Condition and Ventricular Size in Arrested Hydrocephalus: an Analysis of 29 Shunt‐independent Children

NARCIS (Netherlands)

HOLTZER, G.J.; de LANGE, S.A.; ORBAAN, I.J.C.; GELSEMA, R.

1971-01-01

textabstractMeasurement of the diameter of the ventricular system, in a series of 29 patients with arrested hydrocephalus who had become shunt‐independent, showed that enlargement of the ventricles does not necessarily play a part in the arrest of hydrocephalus, for in many of these cases the

Acute hydrocephalus in a child with a third ventricle arachnoid cyst and coincidental enteroviral meningitis

NARCIS (Netherlands)

Jeltema, Hanne-Rinck; Kuijlen, Jos M. A.; Hoving, Eelco W.

We present a 2.5-year-old child suffering from acute hydrocephalus. First, the child was diagnosed with aseptic viral meningitis. The PCR of the cerebrospinal fluid (CSF) was positive for enterovirus. Subsequently, MRI revealed that the hydrocephalus was caused by a cyst in the third ventricle.

Congenital simple hamartoma of the retinal pigment epithelium: a case report

Directory of Open Access Journals (Sweden)

Mariana Rossi Thorell

2014-04-01

Full Text Available We report the case of a 56-year-old woman who presented for a routine ophthalmological examination without visual symptoms and had a unilateral black retinal lesion that was detected by clinical examination. Fluorescein angiography and optical coherence tomography findings were compatible with a congenital simple hamartoma of the retinal pigment epithelium. It is very important to detect this tumor and differentiate it from other pigmented fundus lesions that can compromise visual function or result in systemic conditions such as those caused by malignant tumors.

Effects of Aerobic Capacity on Thrombin-Induced Hydrocephalus and White Matter Injury.

Science.gov (United States)

Ni, Wei; Gao, Feng; Zheng, Mingzhe; Koch, Lauren G; Britton, Steven L; Keep, Richard F; Xi, Guohua; Hua, Ya

2016-01-01

We have previously shown that intracerebral hemorrhage-induced brain injury is less in rats bred for high aerobic capacity (high capacity runners; HCR) compared with those bred for low aerobic capacity (low capacity runners; LCRs). Thrombin, an essential component in the coagulation cascade, is produced after cerebral hemorrhage. Intraventricular injection of thrombin causes significant hydrocephalus and white matter damage. In the present study, we examined the effect of exercise capacity on thrombin-induced hydrocephalus and white matter damage. Mid-aged (13-month-old) female LCRs (n = 13) and HCRs (n = 12) rats were used in this study. Rats received an intraventricular injection of thrombin (3 U, 50 μl). All rats underwent magnetic resonance imaging (MRI) at 24 h and were then euthanized for brain histology and Western blot. The mortalities were 20 % in LCRs and 33 % in HCRs after thrombin injection (p > 0.05). No rats died after saline injection. Intraventricular thrombin injection resulted in hydrocephalus and periventricular white matter damage as determined on MRI. In LCR rats, thrombin induced significant ventricle enlargement (23.0 ± 2.3 vs12.8 ± 1.9 mm(3) in LCR saline group; p hydrocephalus in rats with low aerobic capacity. A differential effect of thrombin may contribute to differences in the effects of cerebral hemorrhage with aerobic capacity.

An MRI volumetric study for leg muscles in congenital clubfoot.

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Ippolito, Ernesto; Dragoni, Massimiliano; Antonicoli, Marco; Farsetti, Pasquale; Simonetti, Giovanni; Masala, Salvatore

2012-10-01

To investigate both volume and length of the three muscle compartments of the normal and the affected leg in unilateral congenital clubfoot. Volumetric magnetic resonance imaging (VMRI) of the anterior, lateral and postero-medial muscular compartments of both the normal and the clubfoot leg was obtained in three groups of seven patients each, whose mean age was, respectively, 4.8 months, 11.1 months and 4.7 years. At diagnosis, all the unilateral congenital clubfeet had a Pirani score ranging from 4.5 to 5.5 points, and all of them had been treated according to a strict Ponseti protocol. All the feet had percutaneous lengthening of the Achilles tendon. A mean difference in both volume and length was found between the three muscular compartments of the leg, with the muscles of the clubfoot side being thinner and shorter than those of the normal side. The distal tendon of the tibialis anterior, peroneus longus and triceps surae (Achilles tendon) were longer than normal on the clubfoot side. Our study shows that the three muscle compartments of the clubfoot leg are thinner and shorter than normal in the patients of the three groups. The difference in the musculature volume of the postero-medial compartment between the normal and the affected side increased nine-fold from age group 2 to 3, while the difference in length increased by 20 %, thus, showing that the muscles of the postero-medial compartment tend to grow in both thickness and length much less than the muscles of the other leg compartments.

Community Knowledge, Beliefs, Attitudes, and Practices towards Children with Spina Bifida and Hydrocephalus in Uganda

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Bannink, Femke; Stroeken, Koenraad; Idro, Richard; van Hove, Geert

2015-01-01

This article describes the findings of a qualitative study on knowledge, beliefs, attitudes, and practices towards children with spina bifida and hydrocephalus in four regions of Uganda. Focus group discussions and semi-structured interviews were held with parents of children with spina bifida and hydrocephalus, policy-makers, and service…

[Hydrocephalus as initial presentation of a spinal cord tumour in a child

DEFF Research Database (Denmark)

Jorgensen, L.M.; Nysom, K.; Lavard, L.D.

2008-01-01

We report a previously healthy two-year-old girl who initially presented with signs of increased intracranial pressure of vomiting, lethargy and unstable gait. She had communicating hydrocephalus and a ventriculoperitoneal shunt was placed. Two years later the girl developed signs of myelopathy...... and was diagnosed with a spinal cord tumour between Th3 and Th9. We suggest that spinal cord tumour should be considered in patients with increased intracranial pressure or hydrocephalus of unknown origin Udgivelsesdato: 2008/9/15...

BIRTH INJURY RELATED UNILATERAL ANTERIOR NARES PARTIAL FIBROUS ATRESIA: A RARE CASE REPORT

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Jayagar

2016-02-01

Full Text Available Congenital atresia of anterior nares has been rarely reported and it may co-exist along with posterior choanal atresia. (1,2 In our case, birth injury caused by forceps delivery has caused unilateral anterior nares partial atresia. Twenty eight years old male patient came with the complaints of left nostril blocked sensation since birth, aggravated with attacks of upper respiratory tract infections. On examination he had left anterior nares partial atresia caused by fibrous bands as a result of birth injury due to instrumental delivery. Rhinoplasty performed to open-up left nostril and patient relieved of his symptoms and also on cosmetic appearance

Somatic memory and gain increase as preconditions for tinnitus: Insights from congenital deafness.

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Eggermont, Jos J; Kral, Andrej

2016-03-01

Tinnitus is the conscious perception of sound heard in the absence of physical sound sources internal or external to the body. The characterization of tinnitus by its spectrum reflects the missing frequencies originally represented in the hearing loss, i.e., partially or completely deafferented, region. The tinnitus percept, despite a total hearing loss, may thus be dependent on the persisting existence of a somatic memory for the "lost" frequencies. Somatic memory in this context is the reference for phantom sensations attributed to missing sensory surfaces or parts thereof. This raises the question whether tinnitus can exist in congenital deafness, were somatic representations have not been formed. We review the development of tonotopic maps in altricial and precocial animals evidence for a lack of tinnitus in congenital deafness and the effects of cochlear implants on the formation of tonotopic maps in the congenitally deaf. The latter relates to the emergence of tinnitus in these subjects. The reviewed material is consistent with the hypothesis that tinnitus requires an established and actively used somatotopic map that leads to a corresponding somatic memory. The absence of such experience explains the absence of tinnitus in congenital bilateral and unilateral deafness. Copyright © 2015 The Authors. Published by Elsevier B.V. All rights reserved.

Chromosome evaluation of Angus calves with unilateral congenital cleft lip and jaw (cheilognathoschisis).

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Swartz, H A; Vogt, D W; Kintner, L D

1982-04-01

Two purebred Angus calves with unilateral cleft lip and jaw were karyotyped and anatomically described. Both calves, 1 male and 1 female, were from the same farm and were born in March and April 1980. The defect was most pronounced in the female calf. Both calves exhibited an elevated midline of the hard palate. The female calf also manifested characteristics indicative of proportionate dwarfism. Karyotypes were constructed after a 72-hour culture of peripheral blood. The 2N number was 60 with no apparent chromosome abnormality. The coefficient of relationship between the 2 affected calves was calculated to be 0.78%. The cause of the defect could not be ascertained from the limited data available.

NMR images of non-communicating hydrocephalus associated with Dandy-Walker variant and achondroplasia

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Sato, Masaharu; Kuroda, Ryotaro; Watanabe, Masaru; Nakatani, Jiro; Ioku, Masahiko; Irisawa, Minoru; Hamada, Tatsumi; Ishida, Osamu

1988-01-01

Two cases of non-communicating hydrocephalus caused by a relatively rare etiology were reported. They were both diagnosed by means of nuclear magnetic resonance (NMR). The first patient, a 4-month-old boy, had Dandy-Walker variant, showing hypoplasia of the corpus callosum and the inferior vermis, and a large cyst behind the vermis, communicating with the subarachnoid space, as well as hydrocephalus; the fourth ventricle was partially reserved. The second patient, also a 4-month-old boy, had achondroplasia, resulting in a narrow foramen magnum; the disturbance of the outflow of the cerebro-spinal fluid around the cisterna magna was thought to be the cause of hydrocephalus in his case. The validity of NMR was demonstrated in the diagnoses of these conditions, for a high resolution was needed in examining the complicated structure of the posterior fossa. (author)

NMR images of non-communicating hydrocephalus associated with Dandy-Walker variant and achondroplasia

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Sato, Masaharu; Kuroda, Ryotaro; Watanabe, Masaru; Nakatani, Jiro; Ioku, Masahiko; Irisawa, Minoru; Hamada, Tatsumi; Ishida, Osamu

1988-06-01

Two cases of non-communicating hydrocephalus caused by a relatively rare etiology were reported. They were both diagnosed by means of nuclear magnetic resonance (NMR). The first patient, a 4-month-old boy, had Dandy-Walker variant, showing hypoplasia of the corpus callosum and the inferior vermis, and a large cyst behind the vermis, communicating with the subarachnoid space, as well as hydrocephalus; the fourth ventricle was partially reserved. The second patient, also a 4-month-old boy, had achondroplasia, resulting in a narrow foramen magnum; the disturbance of the outflow of the cerebro-spinal fluid around the cisterna magna was thought to be the cause of hydrocephalus in his case. The validity of NMR was demonstrated in the diagnoses of these conditions, for a high resolution was needed in examining the complicated structure of the posterior fossa.

The significance of periventricular lucency on computed tomography: experimental study with canine hydrocephalus

International Nuclear Information System (INIS)

Murata, T.; Handa, H.; Mori, K.; Nakano, Y.

1981-01-01

In order to investigate the pathogenesis of periventricular lucency (PVL) in hydrocephalus, CT scans were performed with monitoring of the epidural pressure in a series of dogs with hydrocephalus induced with kaolin. PVL of various degrees was detected in the experimental animals, which disappeared immediately after a shunting operation. Correlations have been attempted between PVL on CT scans and histological examinations, contrast enhancement studies, metrizamide ventriculography, and measurement of regional cerebral blood flow in the periventricular white matter. PVL in hydrocephalus is considered to represent acute edema or chronic CSF retention in the periventricular white matter caused by an increase of water content. In other words, it is regarded as a sign of existing or preceding intraventricular hypertension on CT scan, and seems to be a reversible phenomenon to some extent. PVL may therefore become an indication for a shunt. (orig.)

Endoscopic Removal of a Bullet That Migrated to the Third Ventricle Causing Hydrocephalus.

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Aydoseli, Aydın; Unal, Tugrul Cem; Aras, Yavuz; Sabanci, Pulat Akın; Altunrende, Emre; Izgi, Nail

2017-09-01

Hydrocephalus caused by an intraventricular bullet is a rare event. We report a case of endoscopic removal of an intraventricular bullet. A 66-year-old man was admitted with a gunshot wound to the head after a suicide attempt. The bullet migrated from the frontal parenchyma to the third ventricle day 4 of admission. On day 21 of admission, the patient developed hydrocephalus with obstruction of the cerebral aqueduct. The bullet was accessed through an endoscopic third ventriculostomy and removed using an endoscope. Hydrocephalus may develop in patients with intraventricular foreign objects. When such objects must be removed, the endoscopic approach is a safe, efficient, and minimally invasive procedure. To our knowledge, this is the first case in the literature of foreign object removal from the ventricle via a transcortical endoscopic approach. Copyright © 2017 Elsevier Inc. All rights reserved.

Demonstration of uneven distribution of intracranial pulsatility in hydrocephalus patients.

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Eide, Per K

2008-11-01

Data from intracranial pressure (ICP) recordings in patients with hydrocephalus were reviewed to determine whether intracranial pulsatility within the cerebrospinal fluid (CSF) of cerebral ventricles (ICP(LV)) may differ from that within the brain parenchyma (ICP(PAR)), and whether pulsatility may differ between noncommunicating ventricles. The authors retrieved data from recordings previously obtained in 7 patients with hydrocephalus (noncommunicating in 4 and communicating in 3) and shunt failure who received both an external ventricular drainage (EVD) and an ICP sensor as part of surveillance during intensive care. Simultaneous ICP(LV) and ICP(PAR) signals were available in 6 cases, and simultaneous signals from the lateral and fourth ventricles (ICP(LV) and ICP4V, respectively) were recorded in 1 case. The recordings with both signals were parsed into 6-second time windows. Pulsatility was characterized by the wave amplitude and rise time coefficient, and differences in pulsatility between the ICP(LV) and ICP(PAR) signals (6 cases) or ICP(LV) and ICP4V signals (1 case) were determined. There was uneven distribution of intracranial pulsatility in all 7 patients, shown as significantly elevated pulsatility (that is, higher wave amplitudes and rise time coefficients) within the ventricles (ICP(LV)) than within brain parenchyma (ICP(PAR)) in 6 patients, and significantly higher pulsatility in the fourth (ICP4V) than in the lateral (ICP(LV)) ventricles in 1 patient. Differences > or = 1 mm Hg in ICP wave amplitude were found in 0.5-100% (median 9.4%) of observations in the 7 patients (total number of 6-second time windows, 68,242). The present observations demonstrate uneven distribution of intracranial pulsatility in patients with hydrocephalus, higher pulse pressure amplitudes within the ventricular CSF (ICP(LV)) than within the brain parenchyma (ICP(PAR)). This may be one mechanism behind ventricular enlargement in hydrocephalus.

Neonate with VACTERL Association and a Branchial Arch Anomaly without Hydrocephalus.

Science.gov (United States)

Velazquez, Danitza; Pereira, Elaine; Havranek, Thomas

2016-03-01

VACTERL (vertebral anomalies, anal atresia, cardiac defect, tracheoesophageal fistula, renal anomaly, limb anomalies) is an association of anomalies with a wide spectrum of phenotypic expression. While the majority of cases are sporadic, there is evidence of an inherited component in a small number of patients as well as the potential influence of nongenetic risk factors (maternal diabetes mellitus). Presence of hydrocephalus has been reported in VACTERL patients (VACTERL-H) in the past, with some displaying branchial arch anomalies. We report the unique case of an infant of diabetic mother with VACTERL association and a branchial arch anomaly-in the absence of hydrocephalus.

Normal-pressure hydrocephalus and the saga of the treatable dementias

International Nuclear Information System (INIS)

Friedland, R.P.

1989-01-01

A case study of a 74-year-old woman is presented which illustrates the difficulty of understanding dementing illnesses. A diagnosis of normal-pressure hydrocephalus (NPH) was made because of the development of abnormal gait, with urinary incontinence and severe, diffuse, white matter lesions on the MRI scan. Computed tomographic, MRI scans and positron emission tomographic images of glucose use are presented. The treatable dementias are a large, multifaceted group of illnesses, of which NPH is one. The author proposes a new term for this disorder commonly known as NPH because the problem with the term normal-pressure hydrocephalus is that the cerebrospinal fluid pressure is not always normal in the disease

Congenital and inherited neurologic diseases in dogs and cats: Legislation and its effect on purchase in Italy

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Annamaria Passantino

2016-05-01

Full Text Available Many of the congenital neurologic diseases can result in incapacity or death of the animal. Some of them, such as idiopathic epilepsy and hydrocephalus, exhibit breed or familial predisposition and a genetic basis was proved or suggested. Some diseases can be presumptively diagnosed after a detailed signalment (breed predisposition, history (e.g. family history because many of these defects have familial tendencies, and through physical exam; other diagnostic methods (radiography, computed tomography, magnetic resonance, electrophysiologic tests, etc. can provide supportive evidence for the congenital defect and help to confirm the diagnosis. Some cases can lead to civil law-suits when the lesions are congenital, but not easily recognizable, or when the lesions are hereditary but tend to became manifest only after some time (more than 12 months after the date of purchase, e.g., after the vice-free guarantee period has expired. Moreover, quite frequently an early diagnosis is not made because there are delays in consulting the veterinarian or the general practitioner veterinarian does not perceive subtle signs. This study was designed to focus on the medico-legal aspects concerning the buying and selling in Italy of dogs and cats affected by congenital and hereditary neurologic diseases that could constitute vice in these animals. While adequate provisions to regulate in detail the various aspects of pet sale have still to be drawn up by legislators, it may be helpful to involve breeders, by obliging them by contract to extend guarantees in the case of hereditary lesions, including neurologic diseases.

Chronic hydrocephalus-induced changes in cerebral blood flow: mediation through cardiac effects.

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Dombrowski, Stephen M; Schenk, Soren; Leichliter, Anna; Leibson, Zack; Fukamachi, Kiyotaka; Luciano, Mark G

2006-10-01

Decreased cerebral blood flow (CBF) in hydrocephalus is believed to be related to increased intracranial pressure (ICP), vascular compression as the result of enlarged ventricles, or impaired metabolic activity. Little attention has been given to the relationship between cardiac function and systemic blood flow in chronic hydrocephalus (CH). Using an experimental model of chronic obstructive hydrocephalus developed in our laboratory, we investigated the relationship between the duration and severity of hydrocephalus and cardiac output (CO), CBF, myocardial tissue perfusion (MTP), and peripheral blood flow (PBF). Blood flow measures were obtained using the microsphere injection method under controlled hemodynamic conditions in experimental CH (n=23) and surgical control (n=8) canines at baseline and at 2, 4, 8, 12, and 16 weeks. Cardiac output measures were made using the Swan-Ganz thermodilution method. Intracranial compliance (ICC) via cerebrospinal fluid (CSF) bolus removal and infusion, and oxygen delivery in CSF and prefrontal cortex (PFC) were also investigated. We observed an initial surgical effect relating to 30% CO reduction and approximately 50% decrease in CBF, MTP, and PBF in both groups 2 weeks postoperatively, which recovered in control animals but continued to decline further in CH animals at 16 weeks. Cerebral blood flow, which was positively correlated with CO (P=0.028), showed no significant relationship with either CSF volume or pressure. Decreased CBF correlated with oxygen deprivation in PFC (P=0.006). Cardiac output was inversely related with ventriculomegaly (P=0.019), but did not correlate with ICP. Decreased CO corresponded to increased ICC, as measured by CSF infusion (P=0.04). Our results suggest that CH may have more of an influence on CO and CBF in the chronic stage than in the early condition, which was dominated by surgical effect. The cause of this late deterioration of cardiac function in hydrocephalus is uncertain, but may reflect

Review: Hydrocephalus and Autism and Occupational Therapy Results, A case Report

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Fatemeh Vojdani

2010-04-01

Full Text Available This is a case report. In this essay, a 4years old boy with "Hydrocephalus and Autism" is presented, who referred to an occupational therapy clinic by a psychiatrist and received occupational therapy interventions. Hydrocephalus together with Autism had caused to severe cognitive, perceptual, motor, communicative and self care problems. Occupational therapy goals were facilitation of development in different areas and making adaptive behavior.finally, his difficulties and dysfunctions decreased noticeably : his eye contact was improved seriously , he could keep almost good communication with his family , hyperactivity and aggressive behaviors are decreased, he could draw simple paintings, could run and ride tricycle. By continued rehabilitation interventions, ultimately he could begin studying in one of Tehran normal state schools. The last following up showed that he could passed fifth level of elementary successfully ( with average, 17 , and now is ready to go to the guidance school. Results of this study show the importance of careful and all-around evaluation of hydrocephalus children, careful combination of therapeutic interventions, cooperation and coordination of all rehabilitation team members, continued following up clients even after discharge, and attracting active cooperation of child parents in occupational therapy successful process.

Congenital Zika Virus Infection: Beyond Neonatal Microcephaly.

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Melo, Adriana Suely de Oliveira; Aguiar, Renato Santana; Amorim, Melania Maria Ramos; Arruda, Monica B; Melo, Fabiana de Oliveira; Ribeiro, Suelem Taís Clementino; Batista, Alba Gean Medeiros; Ferreira, Thales; Dos Santos, Mayra Pereira; Sampaio, Virgínia Vilar; Moura, Sarah Rogéria Martins; Rabello, Luciana Portela; Gonzaga, Clarissa Emanuelle; Malinger, Gustavo; Ximenes, Renato; de Oliveira-Szejnfeld, Patricia Soares; Tovar-Moll, Fernanda; Chimelli, Leila; Silveira, Paola Paz; Delvechio, Rodrigo; Higa, Luiza; Campanati, Loraine; Nogueira, Rita M R; Filippis, Ana Maria Bispo; Szejnfeld, Jacob; Voloch, Carolina Moreira; Ferreira, Orlando C; Brindeiro, Rodrigo M; Tanuri, Amilcar

2016-12-01

Recent studies have reported an increase in the number of fetuses and neonates with microcephaly whose mothers were infected with the Zika virus (ZIKV) during pregnancy. To our knowledge, most reports to date have focused on select aspects of the maternal or fetal infection and fetal effects. To describe the prenatal evolution and perinatal outcomes of 11 neonates who had developmental abnormalities and neurological damage associated with ZIKV infection in Brazil. We observed 11 infants with congenital ZIKV infection from gestation to 6 months in the state of Paraíba, Brazil. Ten of 11 women included in this study presented with symptoms of ZIKV infection during the first half of pregnancy, and all 11 had laboratory evidence of the infection in several tissues by serology or polymerase chain reaction. Brain damage was confirmed through intrauterine ultrasonography and was complemented by magnetic resonance imaging. Histopathological analysis was performed on the placenta and brain tissue from infants who died. The ZIKV genome was investigated in several tissues and sequenced for further phylogenetic analysis. Description of the major lesions caused by ZIKV congenital infection. Of the 11 infants, 7 (63.6%) were female, and the median (SD) maternal age at delivery was 25 (6) years. Three of 11 neonates died, giving a perinatal mortality rate of 27.3%. The median (SD) cephalic perimeter at birth was 31 (3) cm, a value lower than the limit to consider a microcephaly case. In all patients, neurological impairments were identified, including microcephaly, a reduction in cerebral volume, ventriculomegaly, cerebellar hypoplasia, lissencephaly with hydrocephalus, and fetal akinesia deformation sequence (ie, arthrogryposis). Results of limited testing for other causes of microcephaly, such as genetic disorders and viral and bacterial infections, were negative, and the ZIKV genome was found in both maternal and neonatal tissues (eg, amniotic fluid, cord blood, placenta, and

Tuberculous meningitis in an immunocompetent male complicated by hydrocephalus.

Science.gov (United States)

Dunphy, Louise; Shetty, Prashanth; Randhawa, Rabinder; Rani, Kharil Amir; Duodu, Yaw

2016-10-07

A 39-year-old man, born in India but resident in the UK for 10 years, was travelling in America when he became feverish with an altered mentation. He reported a 10-day history of fever, photophobia, headache and fatigue. His medical history included hypothyroidism and migraine. He was a non-smoker, did not consume alcohol and denied a history of drug use. He was transferred to the emergency department. Laboratory investigations confirmed hyponatraemia (sodium 128 mmol/L). A chest radiograph confirmed no focal consolidation. Further investigation with a CT brain was unremarkable. A lumbar puncture was suggestive of viral meningitis, with a raised white cell count, lymphocytosis, high protein and low glucose. His PCR was negative for enterovirus and herpes simplex virus. Further investigation with a CT thorax, abdomen and pelvis demonstrated bilateral upper-lobe infiltrations. A bronchoalveolar lavage was negative for acid alcohol fast bacilli (AAFB). A diagnosis of tuberculous meningitis was rendered following a repeat lumbar puncture. Gram stain revealed AAFB and PCR was also positive. He started antitubercular treatment and corticosteroids. A repeat CT brain demonstrated ventriculomegaly, suggestive of hydrocephalus and an MRI head revealed likely communicating hydrocephalus with basilar enhancement. He was repatriated to the UK. Eleven days post transfer, he became acutely confused and required external ventricular drain insertion. After surgical management of his hydrocephalus, there was no further neurological deterioration. He remains committed to his neurorehabilitation. 2016 BMJ Publishing Group Ltd.

Diffusion tensor imaging with direct cytopathological validation: characterisation of decorin treatment in experimental juvenile communicating hydrocephalus.

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Aojula, Anuriti; Botfield, Hannah; McAllister, James Patterson; Gonzalez, Ana Maria; Abdullah, Osama; Logan, Ann; Sinclair, Alexandra

2016-05-31

In an effort to develop novel treatments for communicating hydrocephalus, we have shown previously that the transforming growth factor-β antagonist, decorin, inhibits subarachnoid fibrosis mediated ventriculomegaly; however decorin's ability to prevent cerebral cytopathology in communicating hydrocephalus has not been fully examined. Furthermore, the capacity for diffusion tensor imaging to act as a proxy measure of cerebral pathology in multiple sclerosis and spinal cord injury has recently been demonstrated. However, the use of diffusion tensor imaging to investigate cytopathological changes in communicating hydrocephalus is yet to occur. Hence, this study aimed to determine whether decorin treatment influences alterations in diffusion tensor imaging parameters and cytopathology in experimental communicating hydrocephalus. Moreover, the study also explored whether diffusion tensor imaging parameters correlate with cellular pathology in communicating hydrocephalus. Accordingly, communicating hydrocephalus was induced by injecting kaolin into the basal cisterns in 3-week old rats followed immediately by 14 days of continuous intraventricular delivery of either human recombinant decorin (n = 5) or vehicle (n = 6). Four rats remained as intact controls and a further four rats served as kaolin only controls. At 14-days post-kaolin, just prior to sacrifice, routine magnetic resonance imaging and magnetic resonance diffusion tensor imaging was conducted and the mean diffusivity, fractional anisotropy, radial and axial diffusivity of seven cerebral regions were assessed by voxel-based analysis in the corpus callosum, periventricular white matter, caudal internal capsule, CA1 hippocampus, and outer and inner parietal cortex. Myelin integrity, gliosis and aquaporin-4 levels were evaluated by post-mortem immunohistochemistry in the CA3 hippocampus and in the caudal brain of the same cerebral structures analysed by diffusion tensor imaging. Decorin significantly

Assessment of hydrocephalus in children based on digital image processing and analysis

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Fabijańska Anna

2014-06-01

Full Text Available Hydrocephalus is a pathological condition of the central nervous system which often affects neonates and young children. It manifests itself as an abnormal accumulation of cerebrospinal fluid within the ventricular system of the brain with its subsequent progression. One of the most important diagnostic methods of identifying hydrocephalus is Computer Tomography (CT. The enlarged ventricular system is clearly visible on CT scans. However, the assessment of the disease progress usually relies on the radiologist’s judgment and manual measurements, which are subjective, cumbersome and have limited accuracy. Therefore, this paper regards the problem of semi-automatic assessment of hydrocephalus using image processing and analysis algorithms. In particular, automated determination of popular indices of the disease progress is considered. Algorithms for the detection, semi-automatic segmentation and numerical description of the lesion are proposed. Specifically, the disease progress is determined using shape analysis algorithms. Numerical results provided by the introduced methods are presented and compared with those calculated manually by a radiologist and a trained operator. The comparison proves the correctness of the introduced approach.

Endoscopic Third Ventriculostomy for the Treatment of Hydrocephalus in a Pediatric Population with Myelomeningocele.

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Rei, Joana; Pereira, Josué; Reis, Carina; Salvador, Sérgio; Vaz, Rui

2017-09-01

Hydrocephalus develops in up to 90% of patients born with myelomeningocele. Although endoscopic third ventriculostomy (ETV) is currently considered the preferred treatment for obstructive hydrocephalus, its results have been inconsistent in patients with myelomeningocele. This study focuses on clinical and radiologic outcomes of ETV in children with hydrocephalus related to myelomeningocele. Medical records of 18 pediatric patients with myelomeningocele treated with ETV from 1998 to 2015 at the Centro Hospitalar São João (Porto, Portugal) were reviewed retrospectively. Patients' caregivers were contacted to evaluate their clinical manifestations before and after surgery regarding signs and symptoms of hydrocephalus and Chiari malformation. Control neuroradiologic imaging of 9 patients was obtained and analyzed. Success of ETV was defined by clinical resolution and radiologic confirmation. ETV was successful in 8 of 18 cases (44.4%). Groups of patients were compared according to age at the time of surgery, with a 40% (2/5) success rate in newborns and a 50% success rate (3/6) in children older than 1 year. Eight patients underwent ETV as a first option, with a 37.5% success rate. Ten patients underwent the procedure after previous ventriculoperitoneal shunt (VPS), 5 for malfunction and 5 for VPS infection with 60% and 40% success rates, respectively. Early postoperative complications occurred in 2 patients. ETV can be performed in patients with myelomeningocele and hydrocephalus with success rates of almost 50%. Prior VPS or VPS malfunction or infection do not contraindicate ETV. If possible, the procedure should be delayed until the patient is at least 1 month old. Copyright © 2017 Elsevier Inc. All rights reserved.

Subdural haematoma complicating shunting for normal pressure hydrocephalus in the setting of concomitant antiplatelet medication

DEFF Research Database (Denmark)

Birkeland, Peter; Lauritsen, Jens; Poulsen, Frantz Rom

2016-01-01

OBJECTIVE: To report on the occurrence and management of subdural haematoma after shunt implantation for normal pressure hydrocephalus and to determine the risk of recurrence in the setting of antiplatelet medication. METHODS: From a consecutive series of 80 patients implanted with a cerebrospinal...... fluid shunt for normal pressure hydrocephalus, records from 11 patients taking antiplatelet drugs, who subsequently had surgery for subdural haematoma were extracted and retrospectively reviewed. RESULTS: Patients were followed up for a mean of 1819 days after shunt implantation. Subdural haematomas...... reoperations done before the subdural collection disappeared. Only one patient had a late recurrence almost 11 years after shunt implantation. CONCLUSIONS: Subdural haematoma in the setting of a ventriculoperitoneal implantation for normal pressure hydrocephalus and concomitant antiplatelet medication can...

Type III occipital condylar fracture presenting with hydrocephalus, vertebral artery injury and vasospasm: case report

International Nuclear Information System (INIS)

Menendez, J.A.; Baskaya, M.K.; Day, M.A.; Nanda, A.

2001-01-01

Occipital condylar fractures (OCF) are rare and have a high mortality rate. We report a patient with OCF who presented with acute hydrocephalus and died from diffuse vasospasm secondary to vertebral artery injury. A 45-year-old man fell 20 feet from a deer stand and landed on his head. CT showed a type III OCF continuing to the anterior rim of the foramen magnum on the left, with a bone fragment pushing into the medulla, causing hydrocephalus. The patient was stabilized, and a four-vessel arteriogram showed diffuse vasospasm with complete occlusion of the left vertebral artery at the level of the OCF. To our knowledge, this is the first documented case of the conjunction of OCF, hydrocephalus, and vasospasm. (orig.)

Association between unilateral or bilateral mastectomy and breast cancer death in patients with unilateral ductal carcinoma.

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Agarwal, Shailesh; Pappas, Lisa; Agarwal, Jayant

2017-01-01

Utilization of bilateral mastectomy for unilateral breast cancer is increasing despite cost and surgical risks with conflicting reports of survival benefit. Current studies evaluating death after bilateral mastectomy have included patients treated both with breast conservation therapy and unilateral mastectomy. In this study, we directly compared breast cancer-specific death of patients who underwent bilateral or unilateral mastectomy for unilateral breast cancer using a matched cohort analysis. This was an observational study of women diagnosed with unilateral breast cancer from 1998 through 2002, using the Surveillance, Epidemiology, and End Results (SEER) database. A 4-to-1 matched cohort of patients was selected including 14,075 patients. Mortality of the groups was compared using Cox proportional hazards models for cause-specific death. A total of 41,510 patients diagnosed with unilateral breast cancer were included. Unilateral mastectomy was performed in 93% of patients, while bilateral mastectomy was performed in the remaining 7% of patients. When 4-to-1 matching was performed, 11,260 unilateral mastectomy and 2,815 bilateral mastectomy patients were included. Patients with bilateral mastectomy did not have a significantly lower hazard of breast cancer-specific death when compared with patients with unilateral mastectomy (hazard ratio: 0.92 vs 1.00, p =0.11). Bilateral mastectomy did not provide a clinically or statistically significant breast cancer-specific mortality benefit over unilateral mastectomy based on a matched cohort analysis of a nationwide population database. These findings should be interpreted in the context of patient preference and alternative benefits of bilateral mastectomy.

Inner ear anomalies causing congenital sensorineural hearing loss: CT and MR imaging findings

International Nuclear Information System (INIS)

Hong, Hyun Sook; Paik, Sang Hyun; Cha, Jang Gyu; Park, Seong Jin; Joh, Joon Hee; Park, Jai Soung; Kim, Dae Ho; Lee, Hae Kyung; Kim, Shi Chan

2005-01-01

Many congenital dysplasias of the osseous labyrinth have been identified, and the differential diagnosis of these dysplasias is essential for delivering proper patient management. We retrospectively reviewed the computed tomography (CT) and magnetic resonance (MR) imaging findings of 20 children who had congenital sensorineural hearing loss. The children included cases of enlarged vestibular aqueduct and endolymphatic sac (n=8), aplasia of the semicircular canal (n=4), lateral semicircular canal-vestibule dysplasia (n=3), common cavity malformations with a large vestibule (n=1), cochlear hypoplasia (n=1), Mondini's dysplasia with large vestibular aqueduct (n=1), Mondini's dysplasia with a large vestibule (n=1), and small internal auditory canal (n=1). Six cases were unilateral. Nine cases had combined deformities, and nine cased had cochlear implants. CT was performed with a 1.0-mm thickness in the direct coronal and axial sections with using bone algorithms. MR was performed with a temporal 3D T2 FSE 10-mm scan and with routine brain images. We describe here the imaging features for the anomalies of the inner ear in patients suffering from congenital sensorineural hearing loss

Genetic and Epigenetic Factors at COL2A1 and ABCA4 Influence Clinical Outcome in Congenital Toxoplasmosis

Science.gov (United States)

Jamieson, Sarra E.; de Roubaix, Lee-Anne; Cortina-Borja, Mario; Tan, Hooi Kuan; Mui, Ernest J.; Cordell, Heather J.; Kirisits, Michael J.; Miller, E. Nancy; Peacock, Christopher S.; Hargrave, Aubrey C.; Coyne, Jessica J.; Boyer, Kenneth; Bessieres, Marie-Hélène; Buffolano, Wilma; Ferret, Nicole; Franck, Jacqueline; Kieffer, François; Meier, Paul; Nowakowska, Dorota E.; Paul, Malgorzata; Peyron, François; Stray-Pedersen, Babill; Prusa, Andrea-Romana; Thulliez, Philippe; Wallon, Martine; Petersen, Eskild; McLeod, Rima; Gilbert, Ruth E.; Blackwell, Jenefer M.

2008-01-01

Background Primary Toxoplasma gondii infection during pregnancy can be transmitted to the fetus. At birth, infected infants may have intracranial calcification, hydrocephalus, and retinochoroiditis, and new ocular lesions can occur at any age after birth. Not all children who acquire infection in utero develop these clinical signs of disease. Whilst severity of disease is influenced by trimester in which infection is acquired by the mother, other factors including genetic predisposition may contribute. Methods and Findings In 457 mother-child pairs from Europe, and 149 child/parent trios from North America, we show that ocular and brain disease in congenital toxoplasmosis associate with polymorphisms in ABCA4 encoding ATP-binding cassette transporter, subfamily A, member 4. Polymorphisms at COL2A1 encoding type II collagen associate only with ocular disease. Both loci showed unusual inheritance patterns for the disease allele when comparing outcomes in heterozygous affected children with outcomes in affected children of heterozygous mothers. Modeling suggested either an effect of mother's genotype, or parent-of-origin effects. Experimental studies showed that both ABCA4 and COL2A1 show isoform-specific epigenetic modifications consistent with imprinting. Conclusions These associations between clinical outcomes of congenital toxoplasmosis and polymorphisms at ABCA4 and COL2A1 provide novel insight into the molecular pathways that can be affected by congenital infection with this parasite. PMID:18523590

Genetic and epigenetic factors at COL2A1 and ABCA4 influence clinical outcome in congenital toxoplasmosis.

Directory of Open Access Journals (Sweden)

Sarra E Jamieson

2008-06-01

Full Text Available Primary Toxoplasma gondii infection during pregnancy can be transmitted to the fetus. At birth, infected infants may have intracranial calcification, hydrocephalus, and retinochoroiditis, and new ocular lesions can occur at any age after birth. Not all children who acquire infection in utero develop these clinical signs of disease. Whilst severity of disease is influenced by trimester in which infection is acquired by the mother, other factors including genetic predisposition may contribute.In 457 mother-child pairs from Europe, and 149 child/parent trios from North America, we show that ocular and brain disease in congenital toxoplasmosis associate with polymorphisms in ABCA4 encoding ATP-binding cassette transporter, subfamily A, member 4. Polymorphisms at COL2A1 encoding type II collagen associate only with ocular disease. Both loci showed unusual inheritance patterns for the disease allele when comparing outcomes in heterozygous affected children with outcomes in affected children of heterozygous mothers. Modeling suggested either an effect of mother's genotype, or parent-of-origin effects. Experimental studies showed that both ABCA4 and COL2A1 show isoform-specific epigenetic modifications consistent with imprinting.These associations between clinical outcomes of congenital toxoplasmosis and polymorphisms at ABCA4 and COL2A1 provide novel insight into the molecular pathways that can be affected by congenital infection with this parasite.

Numerical simulation of cerebrospinal fluid hydrodynamics in the healing process of hydrocephalus patients

Science.gov (United States)

Gholampour, S.; Fatouraee, N.; Seddighi, A. S.; Seddighi, A.

2017-05-01

Three-dimensional computational models of the cerebrospinal fluid (CSF) flow and brain tissue are presented for evaluation of their hydrodynamic conditions before and after shunting for seven patients with non-communicating hydrocephalus. One healthy subject is also modeled to compare deviated patients data to normal conditions. The fluid-solid interaction simulation shows the CSF mean pressure and pressure amplitude (the superior index for evaluation of non-communicating hydrocephalus) in patients at a greater point than those in the healthy subject by 5.3 and 2 times, respectively.

Unilateral initiatives

International Nuclear Information System (INIS)

Anon.

1988-01-01

This paper reports on arms control which is generally thought of in terms of formal negotiations with an opponent, with the resulting agreements embodied in a treaty. This is not surprising, since arms control discussions between opponents are both important and politically visible. There are, however, strong reasons for countries to consider and frequently take unilateral initiatives. To do so is entirely consistent with the established major precepts of arms control which state that arms control is designed to reduce the risk of war, the costs of preparing for war, and the death and destruction if war should come. Unilateral initiatives on what weapons are purchased, which ones are eliminated and how forces are deployed can all relate to these objectives. There are two main categories of motives for unilateral initiatives in arms control. In one category, internal national objectives are the dominant, often sole, driving force; the initiative is undertaken for our own good

[New developments in spastic unilateral cerebral palsy].

Science.gov (United States)

Chabrier, S; Roubertie, A; Allard, D; Bonhomme, C; Gautheron, V

2010-01-01

Hemiplegic (or spastic unilateral) cerebral palsy accounts for about 30% of all cases of cerebral palsy. With a population prevalence of 0.6 per 1000 live births, it is the most common type of cerebral palsy among term-born children and the second most common type after diplegia among preterm infants. Many types of prenatal and perinatal brain injury can lead to congenital hemiplegia and brain MRI is the most useful tool to classify them with accuracy and to provide early prognostic information. Perinatal arterial ischemic stroke thus appears as the leading cause in term infants, whereas encephalopathy of prematurity is the most common cause in premature babies. Other causes include brain malformations, neonatal sinovenous thrombosis, parenchymal hemorrhage (for example due to coagulopathy or alloimmune thrombocytopenia) and the more recently described familial forms of porencephaly associated with mutations in the COL4A1 gene. In adjunction with pharmacologic treatment (botulinium neurotoxin injection), new evidence-based rehabilitational interventions, such as constraint-induced movement therapy and mirror therapy, are increasingly being used.

Complication during Postacute Rehabilitation: Patients with Posttraumatic Hydrocephalus

Science.gov (United States)

Denes, Zoltan; Barsi, Peter; Szel, Istvan; Boros, Erzsebet; Fazekas, Gabor

2011-01-01

The objective of this study was to determine the incidence of posttraumatic hydrocephalus (PTH) among patients in our rehabilitation unit for traumatic brain injury. Furthermore, we aimed to assess the effect of shunt implantation and to identify the postoperative complication rate. This is a retrospective cohort study, in which between 2000 and…

Association of congenital megacalycosis and ipsilateral segmental megaureter

International Nuclear Information System (INIS)

Mandell, G.A.; Keller, M.; Kaplan, J.M.

1987-01-01

Four cases of congenital megacalycosis associated with ipsilateral segmental megaureter in children are presented. This association has not been emphasized, although review of the English and French literature revealed eight such cases. In al cases, the concurrent entities are unilateral, with a left-sided predominance and a male prevalence. The diagnosis of megacalycosis is resumed in four of the cases by normal function and prompt emptying of the calyceal systems on diuretic renography and/or urography. The presence of normal caliber renal pelvis interposed between the dilated collecting system and the distal dilated ureter without evidence of vesicoureteral reflux implies the coexistence of ipsilateral idiopathic megaureter. The patients usually present because of urinary tract infection and/or calculus formation, but respond well to conservative therapy. (orig.)

[Economic hardship and fallout on households of the management of hydrocephalus in Benin].

Science.gov (United States)

Gandaho, Hugues Jean-Thierry; Hounton, Sennen Houesse; Kelani, Amina; Darga, Christian; Hoinsou-Hans, Isaac; Agbani, Florence; Lalya, Francis; Koumakpayi, Sikiratou; Ayivi, Blaise

2017-04-27

Objectives: The socioeconomic profile of households and families of children attending hospital for hydrocephalus were documented and analysed. Main costs related to diagnosis and care were reviewed. The emotional fallout and social well-being of families were also analysed. Methods: This retrospective cross-sectional study (January 2006 to January 2015) was based on costs borne by households and families for neurosurgical care of children with hydrocephalus. Results: Sixty children (1 day to 12 years old) had been hospitalized for hydrocephalus in Cotonou-Benin. In 19 cases, the families were single-parent families. In 44 cases, the parents were self-employed workers or private company employees. Public servants, eligible for national health system assistance, accounted for a mere 16 cases. Twenty six children did not receive any financial support, whereas the total average care-related out-of-pocket expenditure for families during the hospital stay was approximately €1,777 (1,117,500 FCFA), i.e. almost 14 times the average monthly income reported by the parents (82,600 FCFA – approximately €120). After hospitalization, 31 mothers had lost their jobs and 21 couples experienced marital issues and their plans to have children. Twelve recent separations were recorded, as well as one indirect maternal death related to depression. Conclusion: In Benin Republic, surgical care for paediatric hydrocephalus represents catastrophic out-of-pocket expenditures for households and families and other living expenses. Families experience significant emotional fallout with effects on couple relationships and survival.

Megadolichobasilarartery as a rare cause of a hydrocephalus internus -synopsis of modern imaging techniques

International Nuclear Information System (INIS)

Lemke, A.J.; Sander, B.; Benndorf, G.; Balzer, J.; Boerschel, M.F.; Hosten, N.; Sprung, C.; Ricke, J.; Felix, R.; Lanksch, W.R.

1995-01-01

Megadolichobasilarartery (MDB), i.e. the widened, elongated and tortuous course of the basilar artery, has been the topic of numerous publications; about 350 cases have been reported world-wide. It can cause many symptoms; isolated or combined cranial nerve lesions and ischemic or hemorrhagic changes are the most frequent. A hydrocephalus internus is a rare occurrence and many patients do not exhibit any symptoms. To date, angiography, computed tomography, and to an increasing extent magnetic resonance imaging (MRI) are the principal methods for diagnosis of MDB. Angiographic-like representations with CT and MRI are further developments which represent an alternative to angiography. With the help of special MRI sequences, furthermore, non-invasive CSF flow measurements for the etiologic evaluation of a hydrocephalus can be performed. For the example of a patient with MDB and hydrocephalus internus, the possibilities of modern imaging techniques are presented and discussed. (orig.) [de

A 2-D simulation of hydrocephalus in the Foramens of Monro of the human ventricular system

Energy Technology Data Exchange (ETDEWEB)

Ammourah, S.; Aroussi, A. [Univ. of Nottingham, School of Mechanical, Materials, Manufacturing and Management, University Park, Nottingham (United Kingdom)]. E-mail: eaxsaka@nottingham.ac.uk; Vloeberghs, M. [Queen' s Medical Centre, Dept. of Child Health, Nottingham (United Kingdom)

2004-07-01

This study investigates the Cerebrospinal fluid (CSF) flow behavior in a two-dimensional plane (2-D) of the human ventricular system when the hydrocephalus in the Foramens of Monro (F.O.M) occurs. In order to understand the CSF behaviour when the hydrocephalus occurs, it is essential to comprehend its normal flow dynamics i.e. the healthy case with no hydrocephalus. This had been done numerically by reconstructing the ventricular system geometry from the MRI scans and then made a 15{sup o} degree cut in the surgical line to obtain the outlines of the 2-D plane. The healthy cases were solved for the CSF actual flow rate, which is between 100-500 ml/day in 100 incremental steps. The unhealthy cases were studied for the average flow rate, which is 300 ml/day but for blockage ratios of the Foramen diameter of 20%, 50% and 75%. These obstructions are designed to simulate the hydrocephalus. The results show that as the flow rate increases the pressure and the velocity values increases, but no changes in the flow pattern occurs. The maximum pressure on the normal cases occurred in the lateral ventricles and the maximum velocity occurred in the aqueduct neck. The results of the hydrocephalus cases show that as the obstruction degree increases the pressure in the lateral ventricle increases accompanied with a velocity increase in the obstructed Foramen, which may cause unwanted stress on the neighboring tissues. (author)

Account of Haly Abbas regarding the management of hydrocephalus in children: a text from medieval times.

Science.gov (United States)

Aciduman, Ahmet; Arda, Berna; Aşkit, Cağatay; Belen, Deniz; Tuzcu, Kemal

2014-12-01

To present the text on hydrocephalus from Haly Abbas's book Kitāb al-Malikī / Liber Regius (The Royal Book), which was accepted as a classical textbook in the Eastern and Western worlds for a long time. The Arabic (Süleymaniye Manuscript Library, Murad Molla Collection, Nr: 1482 and Būlāḳ, 1294 /1877) and the Latin (Venice, 1492) versions of the related chapter was translated and compared to create an English text. Additionally, relevant literature was reviewed in detail. The text on hydrocephalus in Haly Abbas's The Royal Book virtually resembles Paul of Aegina's work. For hydrocephalic cases where the fluid collects between skin and pericranium, and pericranium and bone, Haly Abbas had made little change in surgical intervention; for the third type, skin incision, he preferred a T-type incision instead of an H-type. Like Paul of Aegina, Haly Abbas also did not advise any surgical intervention for the cases of hydrocephalus, where fluid accumulation is between bone and the dura mater. Haly Abbas's approach to hydrocephalus was as brave as that of his predecessors Antyllus, Oribasius, and Paulus, although the cases they dealt with were almost all cephalic hematomas. Although his chapter on the treatment of water accumulation in the head contains surgical interventions in extracranial hydrocephalic conditions, his account on hydrocephalus is extremely precise and gives adequate detail as in other chapters in his book. Copyright © 2014 Elsevier Inc. All rights reserved.

A 2-D simulation of hydrocephalus in the Foramens of Monro of the human ventricular system

International Nuclear Information System (INIS)

Ammourah, S.; Aroussi, A.; Vloeberghs, M.

2004-01-01

This study investigates the Cerebrospinal fluid (CSF) flow behavior in a two-dimensional plane (2-D) of the human ventricular system when the hydrocephalus in the Foramens of Monro (F.O.M) occurs. In order to understand the CSF behaviour when the hydrocephalus occurs, it is essential to comprehend its normal flow dynamics i.e. the healthy case with no hydrocephalus. This had been done numerically by reconstructing the ventricular system geometry from the MRI scans and then made a 15 o degree cut in the surgical line to obtain the outlines of the 2-D plane. The healthy cases were solved for the CSF actual flow rate, which is between 100-500 ml/day in 100 incremental steps. The unhealthy cases were studied for the average flow rate, which is 300 ml/day but for blockage ratios of the Foramen diameter of 20%, 50% and 75%. These obstructions are designed to simulate the hydrocephalus. The results show that as the flow rate increases the pressure and the velocity values increases, but no changes in the flow pattern occurs. The maximum pressure on the normal cases occurred in the lateral ventricles and the maximum velocity occurred in the aqueduct neck. The results of the hydrocephalus cases show that as the obstruction degree increases the pressure in the lateral ventricle increases accompanied with a velocity increase in the obstructed Foramen, which may cause unwanted stress on the neighboring tissues. (author)

Evaluating the effect of hydrocephalus cause on the manner of changes in the effective parameters and clinical symptoms of the disease.

Science.gov (United States)

Gholampour, Seifollah; Fatouraee, Nasser; Seddighi, Amir Saeed; Seddighi, Afsoun

2017-01-01

In the present study, the heads of 11 normal subjects and 21 patients affected by hydrocephalus due to three different causes were simulated using fluid-structure interaction (FSI). To validate the results, the calculated diagram of CSF velocity in aqueduct of Sylvius (AS) was compared with the similar velocity diagram measured using Cine PC-MRI for the same subject. After ensuring the agreement of results, other outputs such as CSF pressure were calculated non-invasively using FSI. The intracranial pressure and CSF pressure in AS and behind the optic nerve sheath were in patients 5-5.3 times the value in normal subjects and the ventricular system volume in patients was 10.2-11.1 times the value in normal subjects. However, the difference between the coefficient of variation and the maximum value of pressure and volume in different types of hydrocephalus was small. Furthermore, the difference between CSF stroke volumes in various types of hydrocephalus patients was less than 4.4%. Results showed that the intensity of clinical symptoms was similar in patients with similar CSF pressure and the cause of the hydrocephalus disease didn't have any significant effect on the intensity of patients' clinical symptoms and the manner of changes in effective parameters on disease. It was also found that the relation of CSF pressure and volume was 16.7% greater in patients with non-communicating hydrocephalus than in patients with communicating hydrocephalus. These results enhance the insight into hydrocephalus bio-mechanism and can help to choose the proper treatment method for hydrocephalus patients. Copyright © 2016 Elsevier Ltd. All rights reserved.

Noninvasive biomarkers in normal pressure hydrocephalus: evidence for the role of neuroimaging.

Science.gov (United States)

Tarnaris, Andrew; Kitchen, Neil D; Watkins, Laurence D

2009-05-01

Normal pressure hydrocephalus (NPH) represents a treatable form of dementia. Recent estimates of the incidence of this condition are in the region of 5% of patients with dementia. The symptoms of NPH can vary among individuals and may be confused with those of patients with multi-infarct dementia, dementia of the Alzheimer type, or even Parkinson disease. Traditionally the diagnosis of NPH could only be confirmed postoperatively by a favorable outcome to surgical diversion of CSF. The object of this literature review was to examine the role of structural and functional imaging in providing biomarkers of favorable surgical outcome. A Medline search was undertaken for the years 1980-2006, using the following terms: normal pressure hydrocephalus, adult hydrocephalus, chronic hydrocephalus, imaging, neuroimaging, imaging studies, outcomes, surgical outcomes, prognosis, prognostic value, sensitivity, specificity, positive predictive value, negative predictive value, and accuracy. The query revealed 16 studies that correlated imaging with surgical outcomes offering accuracy results. Three studies fulfilled the statistical criteria of a biomarker. A dementia Alzheimer-type pattern on SPECT in patients with idiopathic NPH, the presence of CSF flow void on MR imaging, and the N-acetylaspartate/choline ratio in patients with the secondary form are able to predict surgical outcomes with high accuracy. There is at present Level A evidence for using MR spectroscopy in patients with secondary NPH, and Level B evidence for using SPECT and phase-contrast MR imaging to select patients with idiopathic NPH for shunt placement. The studies, however, need to be repeated by other groups. The current work should act as a platform to design further studies with larger sample sizes.

Agenesia pulmonar unilateral Unilateral pulmonary agenesis

OpenAIRE

Maura Cavada Malcon; Claudio Mattar Malcon; Marina Neves Cavada; Paulo Eduardo Macedo Caruso; Lara Flório Real

2012-01-01

A agenesia pulmonar é uma anomalia congênita rara. Relatamos um caso de um menino de 8 anos de idade com agenesia pulmonar à esquerda sem associação com outras malformações. O diagnóstico foi realizado por achados de imagem quando o paciente apresentou sintomas como tosse, sibilância e dispneia sem melhora do quadro clínico após evolução de 30 dias.Pulmonary agenesis is a rare congenital anomaly. We report the case of an 8-year-old boy with left lung agenesis, without any other congenital mal...

Congenital anophthalmia and microphthalmia: epidemiology and orbitofacial rehabilitation

Directory of Open Access Journals (Sweden)

Llorente-González S

2011-12-01

Full Text Available Sara Llorente-González1, J Peralta-Calvo2, JM Abelairas-Gómez21Ophthalmology Service of Hospital de Torrejón, Madrid, Spain; 2Ophthalmology Service of Hospital Universitario La Paz, Madrid, SpainObjective: To describe the prevalence of congenital anophthalmia and microphthalmia in Hospital Universitario La Paz, and to identify associated risk factors and evaluate cosmetic results in treated and nontreated patients.Methods: A retrospective, descriptive, cross-sectional study of patients treated with orbital expanding techniques (cases and nontreated patients (controls was carried out as a comparative case series study. A total of 36 patients with unilateral or bilateral anophthalmia or microphthalmia as main diagnosis were included; 52 epidemiological and management variables for each patient were analyzed. The study evaluated orbital growth and facial symmetry.Results: The overall cosmetic result in the study’s group of patients was satisfactory: 66.7% showed good or very good orbital growth, and 75% showed good or very good facial symmetry. Controls had better cosmetic outcome but showed more cataracts (P = 0.05, inferior colobomas (P = 0.026, and family history (P = 0.056 than the cases. Controls also showed significantly better orbital growth (P = 0.042 and facial symmetry (P = 0.014 than the cases.Conclusion: This study suggests that the mere presence of a globe (controls still provides better orbitofacial development than the artificial stimulation (cases currently available for patients with congenital anophthalmia and microphthalmia, who receive internal and external orbital rehabilitation.Keywords: cosmetic outcome, orbital rehabilitation, ocular malformation, congenital cataract, persistent fetal vasculature

Hydrocephalus during rehabilitation following severe TBI. Relation to recovery, outcome, and length of stay

DEFF Research Database (Denmark)

Linnemann, Mia; Tibæk, Maiken; Kammersgaard, Lars Peter

2014-01-01

BACKGROUND: Post traumatic hydrocephalus (PTH) is a frequent complication during rehabilitation following severe TBI. However, the diagnosis of PTH is not straightforward and despite shunting recovery may be delayed. OBJECTIVE: To study the influence of PTH on recovery and outcome during rehabili......BACKGROUND: Post traumatic hydrocephalus (PTH) is a frequent complication during rehabilitation following severe TBI. However, the diagnosis of PTH is not straightforward and despite shunting recovery may be delayed. OBJECTIVE: To study the influence of PTH on recovery and outcome during...

["Water Hammer effect": a rare mechanism of hydrocephalus].

Science.gov (United States)

Hage, P; El Helou, A

2012-10-01

We are reporting a case of functional hydrocephalus in a 66-year-old male patient presenting for gait disturbance. The etiology of the disease is a cerebrospinal fluid flow disturbance due to an ectatic basilar artery at the level of Monro foramen. Different pathophysiological mechanisms are discussed below. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

Role of 123I-IMP SPET in the early diagnosis of borderline chronic hydrocephalus after aneurysmal subarachnoid haemorrhage

International Nuclear Information System (INIS)

Ohkuma, Hiroki; Tanaka, Masahiko; Suzuki, Shigeharu; Kondoh, Izumi

2000-01-01

Chronic hydrocephalus after aneurysmal subarachnoid haemorrhage (SAH) is easily diagnosed in most cases. However, the diagnosis is sometimes difficult in borderline cases, in which (a) pathognomonic clinical deterioration due to hydrocephalus is masked by the neurological deficits caused in the acute stage of SAH and (b) ventricular enlargement is not so marked on CT scan. The purpose of this study was to investigate whether or not iodine-123 labelled N-isopropyl-p-iodoamphetamine ( 123 I-IMP) single-photon emission tomography (SPET) is of value for the early diagnosis of borderline chronic hydrocephalus after SAH. Fifteen patients who met the criteria of borderline chronic hydrocephalus were selected for the study, and underwent a shunt operation. The patients were divided into a shunt-effective group and a shunt-ineffective group according to neurological improvement after the shunt operation. 123 I-IMP SPET was performed in the acute stage of SAH, within 1 week before the shunt operation, and 2 weeks after the shunt operation. Regional cerebral blood flow was estimated by the 123 I-IMP autoradiographic method. Pre-shunting periventricular low-perfusion areas showed statistically significant differences between the two groups (P 123 I-IMP SPET can be used for both the early diagnosis of borderline chronic hydrocephalus after SAH and the prediction of shunt effectiveness. (orig.)

Pathophysiological study of chronic subdural hematoma and communicating hydrocephalus with delayed MRI using Gd-DTPA (Magnevist)

Energy Technology Data Exchange (ETDEWEB)

Shinoura, Nobusada; Kondo, Tatsuya; Yamakawa, Kenta; Makiuchi, Tsuneo; Fujii, Kyoichi; Yoshioka, Masumi (National Medical Center of Hospital, Tokyo (Japan))

1991-06-01

Concerning the pathophysiology of chronic subdural hematoma and communicating hydrocephalus, recent studies have been made, but no definitive conclusion has yet been attained. To study their complicated mechanisms, we examined a delayed MRI which was performed 4 hours after the intravenous injection of Gd-diethylenetriaminepentaacetic acid (Gd-DTPA) on 5 cases of subdural hygroma, 3 cases of chronic subdural hematoma after irrigation, one case of hydrocephalus with glioblastoma, and one case of Parkinson syndrome. In every case of subdural hygroma, it was certified that Gd-DTPA was leaked into the cavity of the subdural space. This is perhaps because the outer and inner membranes of the subdural hygroma consist of fibroblasts and of capillary vessels with fenestration; the leakage of blood composition through this fenestration may promote the growth of the membrane and the cavity. The leakage of Gd-DTPA decreased after irrigation, and it did not recur. In the case of hydrocephalus with gioblastoma, there was leakage of Gd-DTPA into the ventricles surrounding the tumor. This may be because of the destruction of the blood-cerebrospinal fluid barrier; perhaps this is associated with the cause of the communicating hydrocephalus. (author).

Pathophysiological study of chronic subdural hematoma and communicating hydrocephalus with delayed MRI using Gd-DTPA (Magnevist)

International Nuclear Information System (INIS)

Shinoura, Nobusada; Kondo, Tatsuya; Yamakawa, Kenta; Makiuchi, Tsuneo; Fujii, Kyoichi; Yoshioka, Masumi

1991-01-01

Concerning the pathophysiology of chronic subdural hematoma and communicating hydrocephalus, recent studies have been made, but no definitive conclusion has yet been attained. To study their complicated mechanisms, we examined a delayed MRI which was performed 4 hours after the intravenous injection of Gd-diethylenetriaminepentaacetic acid (Gd-DTPA) on 5 cases of subdural hygroma, 3 cases of chronic subdural hematoma after irrigation, one case of hydrocephalus with glioblastoma, and one case of Parkinson syndrome. In every case of subdural hygroma, it was certified that Gd-DTPA was leaked into the cavity of the subdural space. This is perhaps because the outer and inner membranes of the subdural hygroma consist of fibroblasts and of capillary vessels with fenestration; the leakage of blood composition through this fenestration may promote the growth of the membrane and the cavity. The leakage of Gd-DTPA decreased after irrigation, and it did not recur. In the case of hydrocephalus with gioblastoma, there was leakage of Gd-DTPA into the ventricles surrounding the tumor. This may be because of the destruction of the blood-cerebrospinal fluid barrier; perhaps this is associated with the cause of the communicating hydrocephalus. (author)

Suprasellar choristoma associated with congenital hydrocephalus, anophthalmia, cleft lip and palate, and clinodactly: a proposed variant of a unique new syndrome

Directory of Open Access Journals (Sweden)

Alysse J. Sever, MD

2015-12-01

Full Text Available A male infant was born with a bilateral cleft lip and/or palate, absent nasal structures, left anophthalmos, right coloboma, and bilateral fifth digit clinodactly. Brain magnetic resonance imaging revealed severe asymmetric hydrocephalus, absent corpus callosum, a suprasellar mass with a high riding third ventricle, and no pituitary gland. He had a normal male karyotype and normal prenatal laboratory testing. He had no significant family history and no renal, vertebral, gastrointestinal, or cardiac malformations. This combination of central nervous system findings, ocular and craniofacial abnormalities, a normal karyotype, and limited skeletal abnormalities to our knowledge has only been previously described once in the literature in association with a disruption in Pax and Sonic Hedgehog protein pathways, and we conclude this patient represents a variant of this described syndrome.

Craniocervical Junction Meningiomas without Hydrocephalus Presenting Solely with Syncope: Report of 2 Cases.

Science.gov (United States)

Champagne, Pierre-Olivier; Bojanowski, Michel W

2018-06-01

To our knowledge, there have not been any reported cases of a meningioma of the craniocervical region presenting solely with syncope as its initial symptom. Only 1 case of meningioma presenting with syncope has been published, but it was associated with hydrocephalus. We report 2 cases of syncope caused by a craniocervical junction meningioma, with syncope being the sole presenting symptom and without hydrocephalus. We discuss the possible pathophysiology, as well as the clinical relevance of this type of presentation. We reviewed the charts, operative details, and imagery of 2 cases of meningioma in the region of the craniocervical junction, with syncope as their sole presenting feature. We also reviewed the literature. In 1 case the syncope occurred spontaneously. In the other, it occurred during a Valsalva maneuver. Both meningiomas were surgically removed via a retromastoid approach. There was no recurrence of syncope following surgery. Following a literature review, we found 1 case of posterior fossa meningioma presenting with syncope, but hydrocephalus was also present. Syncope can be the sole manifestation of a meningioma of the craniocervical junction. Such syncopes are a consequence of transient dysfunction of the autonomous pathways in the medulla and/or of the medulla's output. In the absence of other causes of syncope, a meningioma in this region, even in the absence of hydrocephalus, should not be considered as fortuitous, but rather as the actual cause of syncope. Recognizing this possibility offers the potential for proper diagnosis and appropriate treatment of the syncope. Copyright © 2018 Elsevier Inc. All rights reserved.

Unilateralism in International Law

DEFF Research Database (Denmark)

Hartmann, Jacques

2015-01-01

or foreign territory. Many States, however, viewed the EU’s initiative as a unilateral act in violation of their sovereignty. The EU has since suspended the application of the ETS to foreign aircraft. Even so, this incident raises important questions concerning the legality of unilateral acts under...... Union (EU) decided to act unilaterally to reduce emission from aviation, by including aviation within its Emission Trading System (ETS). Initially, the EU set out to include in the ETS emissions from all major aircraft flying to or from European airports, even when these fly over the high seas...

Latex allergy in an infant with acquired hydrocephalus | Ehiozw ...

African Journals Online (AJOL)

We report the case of a 3 month old male infant with acquired hydrocephalus undergoing ventriculo-peritoneal shunt insertion who developed wheals and suffered a respiratory arrest following contact with latex gloves. The need for anaesthetists to effectively diagnose and properly manage this rare clinical entity is ...

Unilateral retinitis pigmentosa sine pigmento.

Science.gov (United States)

Pearlman, J T; Saxton, J; Hoffman, G

1976-05-01

A patient presented with unilateral findings of night blindness shown by impaired rod function and dark adaptation, constricted visual fields with good central acuity, a barely recordable electro-retinographic b-wave, and a unilaterally impaired electro-oculogram. There were none of the pigmentary changes usually associated with retinitis pigmentosa. The unaffected right eye was normal in all respects. Therefore the case is most probably one of unilateral retinitis pigmentosa sine pigmento.

Temporal bone CT analysis of congenital ear anomalies

International Nuclear Information System (INIS)

Hwang, Jung Won; Moon, Min Joo; Sung, Kyu Bo

1988-01-01

Authors analysed the CT findings of the congenital ear anomalies of twenty-nine patients for 2 years and 3 months. The results were as follows: 1. Most of the patients were under the age of 20 (82.7%) and prevalent in male (72.4%). 2. Clinically, congenital ear anomalies were detected in 20 patients (68.9%), conductive hearing loss in 4, sensorineural hearing loss in 1, and the remained 4 patients were detected incidentally without clinical symptom. 3. In the cases of unilateral involvement of 20 patients, right ear was more common (12/20). Eight of 9 bilateral involvement showed similar degree. 4. The middle ear malformations were found in 22 patients (75.9%) and bilateral in 4 patients. 26 cases of middle ear malformations had been classified by Frey into 4 groups; Group I in 5, Group II in 9, Graoup III in 9 and Group IV in 3. 5. Incidentally found ear anomaly was lateral semicircular canal formed a single cavity with the vestibule in all patients (5 pts.). 6. Inner ear malformations accompanying sensorineural hearing loss were found in 3 patients with bilateral involvement and middle ear malformations were accompanied in 2 patients. The degree of involvement of labyrinth was variable.

Development of acute hydrocephalus does not change brain tissue mechanical properties in adult rats, but in juvenile rats.

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Pong, Alice C; Jugé, Lauriane; Bilston, Lynne E; Cheng, Shaokoon

2017-01-01

Regional changes in brain stiffness were previously demonstrated in an experimental obstructive hydrocephalus juvenile rat model. The open cranial sutures in the juvenile rats have influenced brain compression and mechanical properties during hydrocephalus development and the extent by which closed cranial sutures in adult hydrocephalic rat models affect brain stiffness in-vivo remains unclear. The aims of this study were to determine changes in brain tissue mechanical properties and brain structure size during hydrocephalus development in adult rat with fixed cranial volume and how these changes were related to brain tissue deformation. Hydrocephalus was induced in 9 female ten weeks old Sprague-Dawley rats by injecting 60 μL of a kaolin suspension (25%) into the cisterna magna under anaesthesia. 6 sham-injected age-matched female SD rats were used as controls. MR imaging (9.4T, Bruker) was performed 1 day before and then at 3 days post injection. T2-weighted anatomical MR images were collected to quantify ventricle and brain tissue cross-sectional areas. MR elastography (800 Hz) was used to measure the brain stiffness (G*, shear modulus). Brain tissue in the adult hydrocephalic rats was more compressed than the juvenile hydrocephalic rats because the skulls of the adult hydrocephalic rats were unable to expand like the juvenile rats. In the adult hydrocephalic rats, the cortical gray matter thickness and the caudate-putamen cross-sectional area decreased (Spearman, P hydrocephalus is complex and is not solely dependent on brain tissue deformation. Further studies on the interactions between brain tissue stiffness, deformation, tissue oedema and neural damage are necessary before MRE can be used as a tool to track changes in brain biomechanics in hydrocephalus.

Hydrocephalus, skeletal anomalies, and mental disturbances in a mother and three daughters: A new syndrome

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Ferlini, A.; Zanetti, A.; Milan, M.; Calzolari, E. [Universita di Ferrara, London (United Kingdom)] [and others

1995-12-04

We report on a family in which a mother and her 3 daughters have delayed psychomotor development and/or psychosis, hydrocephalus with white matter alterations, arachnoid cysts, skeletal anomalies consisting of brachydactyly, and Sprengel anomaly. Biochemical and cytogenetic analyses were normal on all 4 patients. The pattern of inheritance, clinical manifestations, and variability of expression suggest that this is a new hydrocephalus syndrome possibly transmitted as an X-linked dominant trait. 24 refs., 6 figs., 1 tab.

Factors associated with strabismus after cataract extraction and primary intraocular lens implantation in congenital cataracts

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Soo Jung Lee

2014-06-01

Full Text Available AIM: To evaluate factors associated with the development of strabismus after cataract extraction and primary intraocular lens implantation.METHODS: The medical records of 122 patients, aged 1.5mo to 9y, who had undergone cataract extraction with primary intraocular lens implantation between January 1993 and August 2011 were reviewed. Fourteen patients (17 eyes with strabismus before cataract surgery were excluded. Patients were divided into those with congenital bilateral cataracts (64 patients, 128 eyes and those with unilateral cataracts (44 patients, 44 eyes. The associations between the development of strabismus and age at cataract surgery, pre- and post-cataract extraction corrected distance visual acuity (CDVA, interocular CDVA difference, nystagmus, surgical method, and secondary cataract were evaluated.RESULTS: Factors significantly associated with the development of strabismus included age at cataract surgery (≤1y, preoperative mean CDVA ≤20/100, presence of nystagmus in the bilateral cataract group and postoperative interocular CDVA difference ＞20/70 in the unilateral group. Postoperative CDVA ≤20/100 and preservation of posterior capsule, and presence of secondary cataract were significant factors in both groups.CONCLUSION: Children with congenital cataracts should be monitored carefully after cataract surgery for the development of strabismus, especially when they underwent surgery at age ≤1y, and they have nystagmus, large postoperative interocular CDVA difference, poor preoperative and postoperative CDVA, preservation of the posterior capsule, or secondary cataract.

Subdural Effusions with Hydrocephalus after Severe Head Injury: Successful Treatment with Ventriculoperitoneal Shunt Placement: Report of 3 Adult Cases

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N. Tzerakis

2010-01-01

Full Text Available Background. Subdural collections of cerebrospinal fluid (CSF with associated hydrocephalus have been described by several different and sometimes inaccurate terms. It has been proposed that a subdural effusion with hydrocephalus (SDEH can be treated effectively with a ventriculoperitoneal shunt (V-P shunt. In this study, we present our experience treating patients with SDEH without directly treating the subdural collection. Methods. We treated three patients with subdural effusions and hydrocephalus as a result of a head injury. All the patients were treated with a V-P shunt despite the fact that there was an extra-axial CSF collection with midline shift. Results. In all of the patients, the subdural effusions subsided and the ventricular dilatation improved in the postoperative period. The final clinical outcome remains difficult to predict and depends not only on the successful CSF diversion but also on the primary and secondary brain insult. Conclusion. Subdural effusions with hydrocephalus can be safely and effectively treated with V-P shunting, without directly treating the subdural effusion which subsides along with the treatment of hydrocephalus. However, it is extremely important to make an accurate diagnosis of an SDEH and differentiate this condition from other subdural collections which require different management.

Behavioural problems and autism in children with hydrocephalus : a population-based study.

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Lindquist, Barbro; Carlsson, Göran; Persson, Eva-Karin; Uvebrant, Paul

2006-06-01

To investigate the prevalence of behavioural problems and autism in a population-based group of children with hydrocephalus and to see whether learning disabilities, cerebral palsy (CP), epilepsy, myelomeningocele (MMC) or preterm birth increase the risk of these problems. In the 107 children with hydrocephalus born in western Sweden in 1989-1993, behaviour was assessed using the Conners' parent rating scales in 66 and the teacher's rating scales in 57. Autism was investigated using the Childhood Autism Rating Scale. Parents rated 67% of the children and teachers 39% of the children as having behavioural problems (>1.5 SD, or T score >65). Learning disabilities increased the risk significantly and almost all the children with CP and/or epilepsy had behavioural problems. Autism was present in nine children (13%), in 20% of those without MMC and in one of 26 with MMC. Autism was significantly more frequent in children with learning disabilities (27% vs. 7%) and in children with CP and/or epilepsy (33% vs. 6%). The majority of children with hydrocephalus have behavioural problems and many have autism. It is therefore important to assess and understand all the aspects of cognition and behaviour in these children in order to minimise disability and enhance participation for the child.

Nonverbal Learning Disability Explained: The Link to Shunted Hydrocephalus

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Rissman, Barbara

2011-01-01

A nonverbal learning disability is believed to be caused by damage, disorder or destruction of neuronal white matter in the brain's right hemisphere and may be seen in persons experiencing a wide range of neurological diseases such as hydrocephalus and other types of brain injury (Harnadek & Rourke 1994). This article probes the relationship…

Prevalence of neural tube defect and hydrocephalus in Northern ...

African Journals Online (AJOL)

All the cases reported in this study were open neural tube defect (NTD). The most common defect was hydrocephalus occurring in 33 patients representing 57.9%, with spinal bifida occurring in 21 patients representing 38.6%. Encephalocele or cranium bifida occurred in only 5.3% (3 patients). Among the spinal bifida cases ...

Disturbances of the liquor circulation: differential diagnosis of the hydrocephalus by radionuclide scintigraphy, pneumoencephalography and determination of the liquor pressure

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Fuhrmeister, U; Boerner, W [Wuerzburg Univ. (F.R. Germany). Neurologische Klinik; Wuerzburg Univ. (F.R. Germany). Nuklearmedizinische Abt.)

1976-06-01

Normal-pressure hydrocephalus (NPH) is a special case of communicating hydrocephalus with disturbed resorption of cerebrospinal fluid. Because of the therapeutic consequences, NPH must be clearly differentiated from cerebral atrophy with compensatory increase of the liquor and from hydrocephalus with well-balanced cerebrospinal fluid. NPH is indicated by: excessive pressure rise after artificial volume load on the liquor space in spinal infusion tests; in the x-ray picture, an internal hydrocephalus; an acute-angled corpus callosum, and a progressive ventricular enlargement following in the pneumoencephalography; ventricular reflux with stasis in radionuclide cisternography. In some limiting cases, the diagnosis can be facilitated by a quantitative functional study of the dynamics of the cerebrospinal fluid with the aid of radionuclides. The biological half-life of /sup 131/I albumin is doubled when the resorption of the cerebrospinal fluid is disturbed.

Disturbances of the liquor circulation: differential diagnosis of the hydrocephalus by radionuclide scintigraphy, pneumoencephalography and determination of the liquor pressure

International Nuclear Information System (INIS)

Fuhrmeister, U.; Boerner, W.; Wuerzburg Univ.

1976-01-01

Normal-pressure hydrocephalus (NPH) is a special case of communicating hydrocephalus with disturbed resorption of cerebrospinal fluid. Because of the therapeutic consequences, NPH must be clearly differentiated from cerebral atrophy with compensatory increase of the liquor and from hydrocephalus with well-balanced cerebrospinal fluid. NPH is indicated by: excessive pressure rise after artificial volume load on the liquor space in spinal infusion tests; in the X-ray picture, an internal hydrocephalus; an acute-angled corpus callosum, and a progressive ventricular enlargement following in the pneumoencephalography; ventricular reflux with stasis in radionuclide cisternography. In some limiting cases, the diagnosis can be facilitated by a quantitative functional study of the dynamics of the cerebrospinal fluid with the aid of radionuclides. The biological half-life of 131 I albumin is doubled when the resorption of the cerebrospinal fluid is disturbed. (orig.) [de

Genetic loci for ventricular dilatation in the LEW/Jms rat with fetal-onset hydrocephalus are influenced by gender and genetic background

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Mayorga David A

2005-06-01

Full Text Available Abstract Background The LEW/Jms rat strain has inherited hydrocephalus, with more males affected than females and an overall expression rate of 28%. This study aimed to determine chromosomal positions for genetic loci causing the hydrocephalus. Methods An F1 backcross was made to the parental LEW/Jms strain from a cross with non-hydrocephalic Fischer 344 rats. BC1 rats were generated for two specific crosses: the first with a male LEW/Jms rat as parent and grandparent, [(F × L × L], designated B group, and the second with a female LEW/Jms rat as the parent and grandparent [L × (L × F], designated C group. All hydrocephalic and a similar number of non-hydrocephalic rats from these two groups were genotyped with microsatellite markers and the data was analyzed separately for each sex by MAPMAKER. Results The frequency of hydrocephalus was not significantly different between the two groups (18.2 and 19.9 %, but there was a significant excess of males in the B group. The mean severity of hydrocephalus, measured as the ventricle-to-brain width ratio, was ranked as B group Conclusion Phenotypic expression of hydrocephalus in Lew/Jms, although not X-linked, has a strong male bias. One, and possibly two chromosomal regions are associated with the hydrocephalus.

Congenital unilateral hydrocele

African Journals Online (AJOL)

ABEOLUGBENGAS

Abstract. Foetal hydrocoele is a condition diagnosed in utero in male foetus. It is characterised by a half-moon fluid image around the testis. We describe a case of ultrasound diagnosis of hydrocele in a woman at 35 weeks of gestation. Diagnosis was confirmed at birth, distinguishing this from the transient cases usually.

Congenital unilateral hydrocele

African Journals Online (AJOL)

ABEOLUGBENGAS

We describe a case of ultrasound diagnosis of hydrocele in a woman at 35 weeks of gestation. Diagnosis was confirmed at birth, distinguishing this from the transient cases usually encountered. This finding during a routine obstetrics scan further highlights the relevance of ultrasound to obstetricians and neonatologists.

Clinical presentation and management of congenital ptosis

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Marenco M

2017-02-01

Full Text Available Marco Marenco,1,* Ilaria Macchi,2,* Iacopo Macchi,3 Emilio Galassi,4 Mina Massaro-Giordano,5 Alessandro Lambiase1 1Department of Sense Organs, University of Rome “Sapienza”, 2Department of Ophthalmology, Campus Bio-Medico University of Rome, Rome, 3Department of Ophthalmology, University of Catania, Catania, 4Ophthalmic Clinic, Department of Ophthalmology, University of L’Aquila, L’Aquila, Italy; 5Department of Ophthalmology, Scheie Eye Institute, University of Pennsylvania, Philadelphia, PA, USA *These authors contributed equally to this work Abstract: Congenital ptosis is a rare condition characterized by lower positioning of the upper eyelid that is present at birth and is a clinical condition that is persistent if not treated. It may be unilateral or bilateral and may be associated with other ocular disorders or systemic conditions, including Marcus Gunn, Horner, and Duane syndromes. It is a benign condition but causes functional, cosmetic, and psychological problems in children. However, not all patients need to undergo surgery, and usually only patients at risk of amblyopia need a prompt surgical correction, while in other cases, surgery can be postponed. The grade of ptosis, the eyelid function, and the amblyopic risk are the parameters that affect the ophthalmologist’s decision on timing of surgery and the surgical technique to be used. In fact, there are several types of surgical techniques to correct a congenital ptosis, although very often more than one is needed to obtain an acceptable result. This paper reviews the causes of congenital ptosis and associated diseases. Particular emphasis is given to surgical management and different procedures available to correct the upper eyelid anomaly and avoid permanent damage to visual function. Keywords: ptosis, extraocular muscle development, neurologic dysfunction, surgical approach

Predictors of shunt-dependent hydrocephalus following aneurysmal subarachnoid hemorrhage: a pilot study in a single Egyptian institute.

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Aboul-Ela, Hashem M; Salah El-Din, Ahmed M; Zaater, Ahmed A; Shehab, Mohamed; El Shahawy, Ossama A

2018-01-01

Acute hydrocephalus can cause neurological deterioration after aneurysmal subarachnoid hemorrhage (aSAH). Predicting which patient would require shunting is challenging. This prospective study was conducted upon twenty patients who suffered acute hydrocephalus due to subarachnoid hemorrhage of ruptured aneurysms. Surgical or non-surgical management of hydrocephalus was conducted. Glasgow Coma scale (GCS) was assessed, and hydrocephalus was graded by bicaudate index. Fisher grade was determined from CT scan. Aneurysm site was determined by conventional or CT angiography. Either surgical clipping or endovascular coiling of aneurysms was performed. Initially, 3 (15%) patients had emergency CSF diversion on admission due to poor GCS on arrival. Initially, the remaining 17 patients were managed conservatively. Five patients did not require any intervention. Twelve patients had external ventricular drainage placement, 4 were weaned, and 8 failed weaning. High bicaudate index (> 0.2) correlated with shunting. Aneurysm site correlated well with shunting (ACoA or PCoA). Patients with fair GCS can be managed conservatively. Any deterioration warrants shifting to CSF diversion. Higher bicaudate index will usually need CSF diversion. The value of Fisher carries no significant value. Aneurysm location (ACoA or PCoA) correlates with an increased incidence of ventriculoperitoneal shunt placement.

Infantile holocord cellular ependymoma with communicating hydrocephalus: unusual presentation of a rare case.

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Aryan, Saritha; Ghosal, Nandita; Aziz, Zarina A; Hegde, A S; Dadlani, Ravi

2014-01-01

We present a case of infantile holocord ependymoma in a 4-month-old boy who presented with infection of ventriculoperitoneal shunt done elsewhere for a communicating hydrocephalus. On magnetic resonance imaging, a diffuse holocord T2-hyperintense, T1-hypointense intramedullary bulky lesion with syringomyelia in the cervical level was seen. To the best of our knowledge, this is the first case of infantile holocord ependymoma. As the extent of morbidity associated with a spinal cord tumor is high, an increased level of suspicion and the need for a complete spinal cord screening in a case of infantile hydrocephalus without obvious clinical and radiological evidence of intracranial abnormality is emphasized.

Intracerebral hemorrhage with intraventricular extension and no hydrocephalus may not increase mortality or severe disability.

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Mahta, Ali; Katz, Paul M; Kamel, Hooman; Azizi, S Ausim

2016-08-01

This paper aimed to test the hypothesis that intraventricular extension of spontaneous intracerebral hemorrhage (ICH) in the absence of hydrocephalus is not associated with increased mortality or severe disability. We performed a retrospective consecutive cohort study of patients with primary spontaneous ICH who were admitted to a single institution. Multivariate logistic regression analysis was used to assess the association of each variable with functional outcome as measured by the modified Rankin Scale (mRS). A total of 164 patients met our inclusion criteria and were included in the study. Only hydrocephalus (p=0.002) and hematoma volume (p=0.006) were significantly associated with mortality or poor functional outcome (mRS of 3 to 6). In contrast, the presence of intraventricular hematoma was not independently associated with poor functional outcome. The presence of intraventricular extension of ICH in the absence of hydrocephalus may not increase mortality or disability. Copyright © 2016 Elsevier Ltd. All rights reserved.

Hydrocephalus caused by conditional ablation of the Pten or beta-catenin gene

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Ohtoshi Akihira

2008-10-01

Full Text Available Abstract To investigate the roles of Pten and β-Catenin in the midbrain, either the Pten gene or the β-catenin gene was conditionally ablated, using Dmbx1 (diencephalon/mesencephalon-expressed brain homeobox gene 1-Cre mice. Homozygous disruption of the Pten or β-catenin gene in Dmbx1-expressing cells caused severe hydrocephalus and mortality during the postnatal period. Conditional deletion of Pten resulted in enlargement of midbrain structures. β-catenin conditional mutant mice showed malformation of the superior and inferior colliculi and stenosis of the midbrain aqueduct. These results demonstrate that both Pten and β-Catenin are essential for proper midbrain development, and provide the direct evidence that mutations of both Pten and β-catenin lead to hydrocephalus.

CT in normal pressure hydrocephalus

International Nuclear Information System (INIS)

Fujita, Katsuzo; Nogaki, Hidekazu; Noda, Masaya; Kusunoki, Tadaki; Tamaki, Norihiko

1981-01-01

CT scans were obtained on 33 patients (age 73y. to 31y.) with the diagnosis of normal pressure hydrocephalus. In each case, the diagnosis was made on the basis of the symptoms, CT and cisternographic findings. Underlying diseases of normal pressure hydrocephalus are ruptured aneurysms (21 cases), arteriovenous malformations (2 cases), head trauma (1 case), cerebrovascular accidents (1 case) and idiopathie (8 cases). Sixteen of 33 patients showed marked improvement, five, moderate or minimal improvement, and twelve, no change. The results were compared with CT findings and clinical response to shunting. CT findings were classified into five types, bases on the degree of periventricular hypodensity (P.V.H.), the extent of brain damage by underlying diseases, and the degree of cortical atrophy. In 17 cases of type (I), CT shows the presence of P.V.H. with or without minimal frontal lobe damage and no cortical atrophy. The good surgical improvements were achieved in all cases of type (I) by shunting. In 4 cases of type (II), CT shows the presence of P.V.H. and severe brain damage without cortical atrophy. The fair clinical improvements were achieved in 2 cases (50%) by shunting. In one case of type (III), CT shows the absence of P.V.H. without brain damage nor cortical atrophy. No clinical improvement was obtained by shunting in this type. In 9 cases of type (IV) with mild cortical atrophy, the fair clinical improvement was achieved in two cases (22%) and no improvement in 7 cases. In 2 cases of type (V) with moderate or marked cortical atrophy, no clinical improvement was obtained by shunting. In conclusion, it appeared from the present study that there was a good correlation between the result of shunting and the type of CT, and clinical response to shunting operation might be predicted by classification of CT findings. (author)

Lung perfusion scintigraphy in congenital heart disease

International Nuclear Information System (INIS)

Sugimura, Hiroshi; Nagamachi, Shigeki; Hoshi, Hiroaki; Jinnouchi, Seishi; Oonishi, Takashi; Futami, Shigemi; Watanabe, Katsushi

1990-01-01

Lung perfusion scintigrams were reviewed retrospectively in 28 patients with congenital heart disease, whose ages ranged from the first year to 16 years with an average age of 5 years and 6 months. Seventy four MBq (2 mCi), 111 MBq (2 mCi), and 185 MBq (5 mCi) of Tc-99m macroaggregated albumin were iv injected in the age groups of 0-3, 4-11, and more than 11 years, respectively. Five minutes later, images were obtained in six projections. Abnormal findings on lung perfusion scintigrams were observed in 13 patients (46%). Of these patients, 8 (29%) had a partially decreased blood flow and 5 (17%) had a decreased blood flow in the unilateral lung. No significant difference in the occurrence of abnormal findings was observed among the age groups, although they tended to occur in younger patients. Sex, underlying conditions, and hemodynamics were also independent of scintigraphically abnormal findings. Even when classifying the patients as having either cyanotic or non-cyanotic heart disease, no significant difference in hemodynamics was observed between the group of abnormal findings and the group of normal findings. Pulmonary arteriography available in all patients failed to reveal abnormal findings, with the exception of pulmonary artery stenosis in 2 patients that corresponded to a decreased blood flow in the unilateral lung. Pulmonary artery stenosis seemed to be responsible for abnormal pulmonary blood flow, although other causes remained uncertain. (N.K.)

Hydrocephalus compacted cortex and hippocampus and altered their output neurons in association with spatial learning and memory deficits in rats.

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Chen, Li-Jin; Wang, Yueh-Jan; Chen, Jeng-Rung; Tseng, Guo-Fang

2017-07-01

Hydrocephalus is a common neurological disorder in children characterized by abnormal dilation of cerebral ventricles as a result of the impairment of cerebrospinal fluid flow or absorption. Clinical presentation of hydrocephalus varies with chronicity and often shows cognitive dysfunction. Here we used a kaolin-induction method in rats and studied the effects of hydrocephalus on cerebral cortex and hippocampus, the two regions highly related to cognition. Hydrocephalus impaired rats' performance in Morris water maze task. Serial three-dimensional reconstruction from sections of the whole brain freshly froze in situ with skull shows that the volumes of both structures were reduced. Morphologically, pyramidal neurons of the somatosensory cortex and hippocampus appear to be distorted. Intracellular dye injection and subsequent three-dimensional reconstruction and analyses revealed that the dendritic arbors of layer III and V cortical pyramid neurons were reduced. The total dendritic length of CA1, but not CA3, pyramidal neurons was also reduced. Dendritic spine densities on both cortical and hippocampal pyramidal neurons were decreased, consistent with our concomitant findings that the expressions of both synaptophysin and postsynaptic density protein 95 were reduced. These cortical and hippocampal changes suggest reductions of excitatory connectivity, which could underlie the learning and memory deficits in hydrocephalus. © 2016 International Society of Neuropathology.

Isolated unilateral absence of the right pulmonary artery in two cats visualized by computed tomography angiography

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Tyler JM Jordan

2016-10-01

Full Text Available Case series summary Two cats were evaluated for progressive exercise intolerance, dyspnea and unilateral infiltrate of the left lung. Computed tomography angiography (CTA revealed absence of the right pulmonary artery in both cats with systemic arterial collateral vessels perfusing the right segmental pulmonary arteries. In one case, the collateral vessels arose from the esophageal artery, while in the other case they derived off the right costocervical trunk. One cat was diagnosed with pulmonary hypertension and was euthanized owing to progressive respiratory distress despite medical management with sildenafil, pimobendan, clopidogrel and furosemide. The other cat, without echocardiographic evidence of pulmonary hypertension, was successfully managed with furosemide and enalapril for more than 4 years. Relevance and novel information CTA allowed visualization of a rare congenital heart malformation, unilateral absence of the right pulmonary artery, in two cats and accurately characterized the source of collateral blood supply to the affected lung. Severe pulmonary hypertension may be a negative prognostic factor in cats with this condition as medical therapy in the cat without evidence of pulmonary hypertension resolved clinical signs, while the cat with severe pulmonary hypertension died from the disease.

Protein and synthetic polymer injection for induction of obstructive hydrocephalus in rats

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Del Bigio Marc R

2007-09-01

Full Text Available Abstract Background The objective of this study was to develop a simple and inexpensive animal model of induced obstructive hydrocephalus with minimal tissue inflammation, as an alternative to kaolin injection. Materials Two-hundred and two male Sprague-Dawley rats aged 3 weeks received intracisternal injections of kaolin (25% suspension, Matrigel, type 1 collagen from rat tail, fibrin glue (Tisseel, n-butyl-cyanoacrylate (NBCA, or ethylene vinyl alcohol copolymer (Onyx-18 and Onyx-34. Magnetic resonance imaging was used to assess ventricle size. Animals were euthanized at 2, 5, 10 and 14 days post-injection for histological analysis. Results Kaolin was associated with 10% mortality and successful induction of hydrocephalus in 97% of survivors (ventricle area proportion 0.168 ± 0.018. Rapidly hardening agents (fibrin glue, NBCA, vinyl polymer had high mortality rates and low success rates in survivors. Only Matrigel had relatively low mortality (17% and moderate success rate (20%. An inflammatory response with macrophages and some lymphocytes was associated with kaolin. There was negligible inflammation associated with Matrigel. A severe inflammatory response with giant cell formation was associated with ethylene vinyl alcohol copolymer. Conclusion Kaolin predictably produces moderate to severe hydrocephalus with a mild chronic inflammatory reaction and fibrosis of the leptomeninges. Other synthetic polymers and biopolymers tested are unreliable and cause different types of inflammation.

Measurement of normal corpus callosum with MRI in Korean adults and morphological change of corpus callosum by grade of hydrocephalus

International Nuclear Information System (INIS)

Song, Dong Hoon; Chang, Seung Kuk; Kim, Jong Deok; Eun, Tchoong Kie; Park, Dong Woo

1995-01-01

To measure the size of normal corpus callosum in each portion using objective and reproducible method with MRI and evaluation of morphological change of corpus callosum by grade of hydrocephalus. Midsagittal T1-weighted MR imaging of the corpus callosum was investigated in 41 volunteers of normal Korean adults and 19 patients with hydrocephalus. Corpus callosum was measured for the anteroposterior length(A), height(B), and the thickness of genu(C), body(D), splenium(E), and the narrowest portion of body(F). And the analysis of morphology and signal intensity of the corpus callosum were also evaluated. Hydrocephalus was graded as mild, moderate, and severe, and comparison of thickness with normal corpus callosum in each portion was done. The mean length and height were 72.3 mm, 28.6 mm in male, and 70.7 mm, 28.9 mm in female. And the mean dimension for C, D, E and F were 13.1 mm, 8 mm, 13.2 mm, 5.2 mm in male, and 12.8 mm, 7.5 mm, 12.3 mm, 5 mm in female. The morphology of normal corpus callosum was 'hook' shaped on midline sagittal T1-weighted image. Narrowing at posterior third portion of body were present on 30 cases(73.2%) and even in thickness of the body in 11 cases(26.8%). The signal intensity of the corpus callosum on midsagittal T1-weighted spin echo image of normal cases was homogeneous hyperintense as compared with cerebral gray matter. In hydrocephalus, A and B were increased and other portions were decreased in thickness. Genu and the narrowest portion of body showed significant difference of thickness according to the grade of hydrocephalus. The mean dimension of all portion of corpus callosum were larger in male than female except for callosal height but not significant statistically with the exception of splenium. Hydrocephalus lead to morphological change of the corpus callosum. Among the portion of corpus callosum, genu and the narrowest portion of the body were thought to be the most sensitive indicators of degree in hydrocephalus

Agenesia pulmonar unilateral Unilateral pulmonary agenesis

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Maura Cavada Malcon

2012-08-01

Full Text Available A agenesia pulmonar é uma anomalia congênita rara. Relatamos um caso de um menino de 8 anos de idade com agenesia pulmonar à esquerda sem associação com outras malformações. O diagnóstico foi realizado por achados de imagem quando o paciente apresentou sintomas como tosse, sibilância e dispneia sem melhora do quadro clínico após evolução de 30 dias.Pulmonary agenesis is a rare congenital anomaly. We report the case of an 8-year-old boy with left lung agenesis, without any other congenital malformations. When the patient presented symptoms, including cough, wheezing, and dyspnea, with no clinical improvement after a period of 30 days, imaging studies were conducted and the diagnosis was made.

Cerebral Blood Flow Changes after Shunt in Hydrocephalus after Aneurysmal Subarachnoid Hemorrhage: Analysis by statistical Parametric Mapping

International Nuclear Information System (INIS)

Hyun, I. Y.; Choi, W. S.; Pak, H. S.

2003-01-01

The purpose of this study was to evaluate the changes of regional cerebral blood flow (rCBF) after shunt operation in patients with hydrocephalus after aneurysmal subarachnoid hemorrhage ba statistical parametric mapping (SPM). Seven patients (4 male, mean age 54 years) with hydrocephalus after aneurysmal subarachnoid hemorrhage underwent a shunt operation. Tc-99m HMPAO SPECT was performed within I week before, and 2 weeks after the shunt operation. All of the SPECT images were spatially transformed to standard space, smoothed, and globally normalized. After spatial and count normalization, rCBF of pre- and post- shunting Tc- 99m HMPAO SPECT was estimated at every voxel using t statistics. The voxels with a P value of less than 0.001 were considered to be significantly different. The shunt operation was effective in all patients. Pre-shunting Tc-99m HMPAO SPECT showed hypoperfusion, predominantly in the periventricular area. After shunt operation, periventricular low perfusion was disappeared. The results of this study show that periventricular CBF is impaired in hydrocephalus after aneurysmal subarachnoid hemorrhage. Significant increase of periventricular CBF after shunt operation suggests the evaluation of periventricular CBF by SPM might be of value for the prediction of shunt effectiveness in hydrocephalus

Cerebral Blood Flow Changes after Shunt in Hydrocephalus after Aneurysmal Subarachnoid Hemorrhage: Analysis by statistical Parametric Mapping

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Hyun, I. Y.; Choi, W. S.; Pak, H. S. [College of Medicine, Univ. of Inhwa, Incheon (Korea, Republic of)

2003-07-01

The purpose of this study was to evaluate the changes of regional cerebral blood flow (rCBF) after shunt operation in patients with hydrocephalus after aneurysmal subarachnoid hemorrhage ba statistical parametric mapping (SPM). Seven patients (4 male, mean age 54 years) with hydrocephalus after aneurysmal subarachnoid hemorrhage underwent a shunt operation. Tc-99m HMPAO SPECT was performed within I week before, and 2 weeks after the shunt operation. All of the SPECT images were spatially transformed to standard space, smoothed, and globally normalized. After spatial and count normalization, rCBF of pre- and post- shunting Tc- 99m HMPAO SPECT was estimated at every voxel using t statistics. The voxels with a P value of less than 0.001 were considered to be significantly different. The shunt operation was effective in all patients. Pre-shunting Tc-99m HMPAO SPECT showed hypoperfusion, predominantly in the periventricular area. After shunt operation, periventricular low perfusion was disappeared. The results of this study show that periventricular CBF is impaired in hydrocephalus after aneurysmal subarachnoid hemorrhage. Significant increase of periventricular CBF after shunt operation suggests the evaluation of periventricular CBF by SPM might be of value for the prediction of shunt effectiveness in hydrocephalus.

Contemporary occurrence of hydrocephalus and Chiari I malformation in sagittal craniosynostosis. Case report and review of the literature.

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Sgulò, Francesco Giovanni; Spennato, Pietro; Aliberti, Ferdinando; Di Martino, Giuliana; Cascone, Daniele; Cinalli, Giuseppe

2017-01-01

Chiari malformation type I (CM-I) and hydrocephalus are often associated with complex craniosynostosis. On the contrary, their simultaneous occurrence in monosutural synostosis is extremely rare. The pathophysiological hypothesis is that they may alter posterior fossa growth and lead to cerebellar tonsil herniation also without skull base primary involvement. Hydrocephalus is multifactorial and may be secondary to fourth ventricle outlet obstruction. The management of these cases is quite complex and not well defined. Cranial vault remodeling should be the only treatment when CM-I is asymptomatic and not related to syringomyelia. Suboccipital decompression should be reserved only in complicated CM-I, usually as a second surgical step following the correction of the supratentorial deformity. In our opinion, the associated hydrocephalus should be treated first in order to normalize intracranial hypertension before opening the cranial sutures. We report the case of a 26-month-old child that presented with sagittal craniosynostosis, hydrocephalus, and CM-I. He was managed by performing endoscopic third ventriculostomy (ETV) first and cranial vault remodeling thereafter. Clinico-radiological outcome was very satisfying. Concerning literature is reviewed; physiopathology and surgical management are discussed.

Control volume based hydrocephalus research; a phantom study

Science.gov (United States)

Cohen, Benjamin; Voorhees, Abram; Madsen, Joseph; Wei, Timothy

2009-11-01

Hydrocephalus is a complex spectrum of neurophysiological disorders involving perturbation of the intracranial contents; primarily increased intraventricular cerebrospinal fluid (CSF) volume and intracranial pressure are observed. CSF dynamics are highly coupled to the cerebral blood flows and pressures as well as the mechanical properties of the brain. Hydrocephalus, as such, is a very complex biological problem. We propose integral control volume analysis as a method of tracking these important interactions using mass and momentum conservation principles. As a first step in applying this methodology in humans, an in vitro phantom is used as a simplified model of the intracranial space. The phantom's design consists of a rigid container filled with a compressible gel. Within the gel a hollow spherical cavity represents the ventricular system and a cylindrical passage represents the spinal canal. A computer controlled piston pump supplies sinusoidal volume fluctuations into and out of the flow phantom. MRI is used to measure fluid velocity and volume change as functions of time. Independent pressure measurements and momentum flow rate measurements are used to calibrate the MRI data. These data are used as a framework for future work with live patients and normal individuals. Flow and pressure measurements on the flow phantom will be presented through the control volume framework.

Radiation doses to children with shunt-treated hydrocephalus

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Holmedal, Lise J. [Helse Fonna, Department of Radiology, Stord Hospital, Stord (Norway); Friberg, Eva G.; Boerretzen, Ingelin; Olerud, Hilde [The Norwegian Radiation Protection Authority, Oesteraas (Norway); Laegreid, Liv [Haukeland University Hospital, Department of Paediatrics, Bergen (Norway); Rosendahl, Karen [University of Bergen, Department of Surgical Sciences, Radiology Section, Bergen (Norway); Great Ormond Street Hospital for Children, Department of Diagnostic Radiology, London (United Kingdom)

2007-12-15

Children with shunt-treated hydrocephalus are still followed routinely with frequent head CT scans. To estimate the effective dose, brain and lens doses from these examinations during childhood, and to assess dose variation per examination. All children born between 1983 and 1995 and treated for hydrocephalus between 1983 and 2002 were included. We retrospectively registered the number of examinations and the applied scan parameters. The effective dose was calculated using mean conversion factors from the CT dose index measured free in air, while doses to the lens and brain were estimated using tabulated CT dose index values measured in a head phantom. A total of 687 CT examinations were performed in 67 children. The mean effective dose, lens dose and brain dose to children over 6 months of age were 1.2 mSv, 52 mGy and 33 mGy, respectively, and the corresponding doses to younger children were 3.2 mSv, 60 mGy and 48 mGy. The effective dose per CT examination varied by a factor of 64. None of the children was exposed to doses known to cause deterministic effects. However, since the threshold for radiation-induced damage is not known with certainty, alternative modalities such as US and MRI should be used whenever possible. (orig.)

Congenital sensorineural deafness in Australian stumpy-tail cattle dogs is an autosomal recessive trait that maps to CFA10.

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Susan Sommerlad

2010-10-01

Full Text Available Congenital sensorineural deafness is an inherited condition found in many dog breeds, including Australian Stumpy-tail Cattle Dogs (ASCD. This deafness is evident in young pups and may affect one ear (unilateral or both ears (bilateral. The genetic locus/loci involved is unknown for all dog breeds. The aims of this study were to determine incidence, inheritance mechanism, and possible association of congenital sensorineural deafness with coat colour in ASCD and to identify the genetic locus underpinning this disease.A total of 315 ASCD were tested for sensorineural deafness using the brain stem auditory evoked response (BAER test. Disease penetrance was estimated directly, using the ratio of unilaterally to bilaterally deaf dogs, and segregation analysis was performed using Mendel. A complete genome screen was undertaken using 325 microsatellites spread throughout the genome, on a pedigree of 50 BAER tested ASCD in which deafness was segregating. Fifty-six dogs (17.8% were deaf, with 17 bilaterally and 39 unilaterally deaf. Unilaterally deaf dogs showed no significant left/right bias (p = 0.19 and no significant difference was observed in frequencies between the sexes (p = 0.18. Penetrance of deafness was estimated as 0.72. Testing the association of red/blue coat colour and deafness without accounting for pedigree structure showed that red dogs were 1.8 times more likely to be deaf (p = 0.045. The within family association between red/blue coat colour and deafness was strongly significant (p = 0.00036, with red coat colour segregating more frequently with deafness (COR = 0.48. The relationship between deafness and coat speckling approached significance (p = 0.07, with the lack of statistical significance possibly due to only four families co-segregating for both deafness and speckling. The deafness phenotype was mapped to CFA10 (maximum linkage peak on CFA10 -log10 p-value = 3.64, as was both coat colour and

Comorbid Normal Pressure Hydrocephalus with Parkinsonism: A Clinical Challenge and Call for Awareness.

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Cucca, A; Biagioni, M C; Sharma, K; Golomb, J; Gilbert, R M; Di Rocco, A; Fleisher, J E

2018-01-01

Idiopathic normal pressure hydrocephalus (iNPH) is the most common cause of hydrocephalus in adults. The diagnosis may be challenging, requiring collaborative efforts between different specialists. According to the International Society for Hydrocephalus and Cerebrospinal Fluid Disorders, iNPH should be considered in the differential of any unexplained gait failure with insidious onset. Recognizing iNPH can be even more difficult in the presence of comorbid neurologic disorders. Among these, idiopathic Parkinson's disease (PD) is one of the major neurologic causes of gait dysfunction in the elderly. Both conditions have their peak prevalence between the 6th and the 7th decade. Importantly, postural instability and gait dysfunction are core clinical features in both iNPH and PD. Therefore, diagnosing iNPH where diagnostic criteria of PD have been met represents an additional clinical challenge. Here, we report a patient with parkinsonism initially consistent with PD who subsequently displayed rapidly progressive postural instability and gait dysfunction leading to the diagnosis of concomitant iNPH. In the following sections, we will review the clinical features of iNPH, as well as the overlapping and discriminating features when degenerative parkinsonism is in the differential diagnosis. Understanding and recognizing the potential for concomitant disease are critical when treating both conditions.

Comorbid Normal Pressure Hydrocephalus with Parkinsonism: A Clinical Challenge and Call for Awareness

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A. Cucca

2018-01-01

Full Text Available Idiopathic normal pressure hydrocephalus (iNPH is the most common cause of hydrocephalus in adults. The diagnosis may be challenging, requiring collaborative efforts between different specialists. According to the International Society for Hydrocephalus and Cerebrospinal Fluid Disorders, iNPH should be considered in the differential of any unexplained gait failure with insidious onset. Recognizing iNPH can be even more difficult in the presence of comorbid neurologic disorders. Among these, idiopathic Parkinson’s disease (PD is one of the major neurologic causes of gait dysfunction in the elderly. Both conditions have their peak prevalence between the 6th and the 7th decade. Importantly, postural instability and gait dysfunction are core clinical features in both iNPH and PD. Therefore, diagnosing iNPH where diagnostic criteria of PD have been met represents an additional clinical challenge. Here, we report a patient with parkinsonism initially consistent with PD who subsequently displayed rapidly progressive postural instability and gait dysfunction leading to the diagnosis of concomitant iNPH. In the following sections, we will review the clinical features of iNPH, as well as the overlapping and discriminating features when degenerative parkinsonism is in the differential diagnosis. Understanding and recognizing the potential for concomitant disease are critical when treating both conditions.

Risk factors for congenital hydrocephalus

DEFF Research Database (Denmark)

Munch, Tina Noergaard; Rasmussen, Marie-Louise Hee; Wohlfahrt, Jan

2014-01-01

. Furthermore, to identify the risk factors unique for isolated CHC as compared to syndromic CHC. METHODS: We established a cohort of all children born in Denmark between 1978 and 2008. Information on CHC and maternal medical diseases were obtained from the National Patient Discharge Register, maternal intake...... increased risk of isolated CHC compared to unexposed children (RR 2.52, 95% CI 1.47 to 4.29) (1.5/1000 born children). Risk factors also found for syndromic CHC were: Male gender, multiples and maternal diabetes. CONCLUSIONS: The higher risk for isolated CHC in first-born children as well as behavioural......-born children, we observed 1193 cases of isolated CHC (0.062/1000) born children. First-borns had an increased risk of isolated CHC compared to later-borns (1.32 95% CI 1.17 to 1.49) (0.72/1000 born children). First trimester exposure to maternal use of antidepressants was associated with a significantly...

Parvovirus associated cerebellar hypoplasia and hydrocephalus in day-old broiler chickens

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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...

Accessory mammary tissue associated with congenital and hereditary nephrourinary malformations.

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Urbani, C E; Betti, R

1996-05-01

The association between polythelia (supernumerary nipple) and kidney and urinary tract malformations (KUTM) is controversial. Some authors reported this association in newborns and infants. Case-control studies dealing with adult subjects are not found in the literature. The purpose of this study is to determine the frequency of the association between accessory mammary tissue (AMT) and congenital and hereditary nephrourinary defects in an adult population compared to a control group. The study was performed in 146 white patients (123 men, 23 women) with AMT out of 2645 subjects consecutively referred to us for physical examination. The following investigations were undertaken: ultrasonographic examination of the abdomen and the kidneys, ECG, echocardiogram, roentgenogram of the vertebral column, urinalysis, and other laboratory tests. A sex- and age-matched control group without any evidence of AMT or lateral displacement of the nipples underwent the same examinations. Kidney and urinary tract malformations were detected in 11 patients with AMT (nine men, two women) and in one control. These data indicate a significantly higher frequency of KUTM in the AMT-affected patients compared to controls (7.53% vs. 0.68%, P < 0.001). A broad spectrum of KUTM was discovered in association with AMT: adult dominant polycystic kidney disease, unilateral renal agenesis, cystic renal dysplasia, familial renal cysts, and congenital stenosis of the pyeloureteral joint. Accessory mammary tissue offers an important clue for congenital and hereditary anomalies of the kidneys and urinary collecting systems. Patients with AMT should, therefore, be extensively examined for the presence of occult nephrouropathies.

Loss of Dishevelleds disrupts planar polarity in ependymal motile cilia and results in hydrocephalus

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Ohata, Shinya; Nakatani, Jin; Herranz-Pérez, Vicente; Cheng, JrGang; Belinson, Haim; Inubushi, Toshiro; Snider, William D.; García-Verdugo, Jose Manuel; Wynshaw-Boris, Anthony; Álvarez-Buylla, Arturo

2014-01-01

SUMMARY Defects in ependymal (E) cells, which line the ventricle and generate cerebrospinal fluid flow through ciliary beating, can cause hydrocephalus. Dishevelled genes (Dvls) are essential for Wnt signaling and Dvl2 has been shown to localize to the rootlet of motile cilia. Using the hGFAP-Cre;Dvl1−/−;2flox/flox;3+/− mouse, we show that compound genetic ablation of Dvls causes hydrocephalus. In hGFAP-Cre;Dvl1−/−;2flox/flox;3+/− mutants, E cells differentiated normally, but the intracellular and intercellular rotational alignments of ependymal motile cilia were disrupted. As a consequence, the fluid flow generated by the hGFAP-Cre;Dvl1−/−;2flox/flox;3+/− E cells was significantly slower than that observed in control mice. Dvls were also required for the proper positioning of motile cilia on the apical surface. Tamoxifen-induced conditional removal of Dvls in adult mice also resulted in defects in intracellular rotational alignment and positioning of ependymal motile cilia. These results suggest that Dvls are continuously required for E cell planar polarity and may prevent hydrocephalus. PMID:25043421

Comparative study of unilateral versus bilateral inferior oblique recession/anteriorization in unilateral inferior oblique overaction.

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Mostafa, Attiat M; Kassem, Rehab R

2018-05-01

To compare the effect of, and the rate of subsequent development of iatrogenic antielevation syndrome after, unilateral versus bilateral inferior oblique graded recession-anteriorization to treat unilateral inferior oblique overaction. Thirty-four patients with unilateral inferior oblique overaction were included in a randomized prospective study. Patients were equally divided into 2 groups. Group UNI underwent unilateral, group BI bilateral, inferior oblique graded recession-anteriorization. A successful outcome was defined as orthotropia, or within 2 ∆ of a residual hypertropia, in the absence of signs of antielevation syndrome, residual inferior oblique overaction, V-pattern, dissociated vertical deviation, or ocular torticollis. A successful outcome was achieved in 11 (64.7%) and 13 (76.5%) patients in groups UNI and BI, respectively (p = 0.452). Antielevation syndrome was diagnosed as the cause of surgical failure in 6 (35.3%) and 2 (11.8%) patients, in groups UNI and BI, respectively (p = 0.106). The cause of surgical failure in the other 2 patients in group BI was due to persistence of ocular torticollis and hypertropia in a patient with superior oblique palsy and a residual V-pattern and hypertropia in the other patient. The differences between unilateral and bilateral inferior oblique graded recession-anteriorization are insignificant. Unilateral surgery has a higher tendency for the subsequent development of antielevation syndrome. Bilateral surgery may still become complicated by antielevation syndrome, although at a lower rate. In addition, bilateral surgery had a higher rate of undercorrection. Further studies on a larger sample are encouraged.

A case of unilateral dysmenorrhea

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Tulon Borah

2010-01-01

Full Text Available Unilateral dysmenorrhea in an adolescent may be associated with uterine malformation. Relevant investigations in suspected cases and timely intervention can prevent future complications in such cases. Here, we present a case of unicornuate uterus with rudimentary horn in an adolescent complaining of unilateral dysmenorrhea.

Infant hydrocephalus in sub-Saharan Africa: the reality on the Tanzanian side of the lake.

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Santos, Maria M; Rubagumya, Derick K; Dominic, Imani; Brighton, Amos; Colombe, Soledad; O'Donnell, Philip; Zubkov, Micaella R; Härtl, Roger

2017-11-01

OBJECTIVE Infant hydrocephalus is estimated to affect more than 100,000 new infants each year in sub-Saharan Africa (SSA). Bugando Medical Centre (BMC), a government-funded and patient cost-shared referral center, serves over 13 million people in the Lake and Western regions of Tanzania. The goals of this study were to characterize the infant population affected by hydrocephalus who presented to BMC and were treated with a ventriculoperitoneal shunt (VPS) to determine the rate of early complications associated with this surgical procedure and to assess its potential risk factors. METHODS Data were prospectively collected from all patients less than 1 year of age who, over a period of 7 months, were diagnosed with hydrocephalus and admitted to BMC for insertion of a primary VPS. Demographic data, maternal history, preoperative studies, surgical procedure, and surgical complications developing by the time of the first follow-up visit were analyzed. Risk factors associated with the surgical complications were determined. RESULTS During the 7-month study period, 125 infants eligible for the study were included in the analysis. Overall, 75% were younger than 6 months of age, and 56% were males. Only 7% of mothers had a gestational ultrasound, 98% did not receive preconception folic acid, and 25% delivered their child at home. In most patients with hydrocephalus the etiology was uncertain (56%), and other patients had postinfectious (22.4%) or myelomeningocele-associated (16%) hydrocephalus. Patients' mean head circumference on admission was 51.4 ± 6.3 cm. Their median age at shunt surgery was 137 days, and 22.4% of the patients were operated on without having undergone radiological assessment. The majority of shunts were placed in a right parietooccipital location. Thirteen patients had undergone a previous intraventricular endoscopic procedure. Overall, at least one surgical complication was found in 33.6% of patients up to the first follow-up assessment (median

Junctional Adhesion Molecule (JAM)-C Deficient C57BL/6 Mice Develop a Severe Hydrocephalus

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Liebner, Stefan; Mittelbronn, Michel; Deutsch, Urban; Enzmann, Gaby; Adams, Ralf H.; Aurrand-Lions, Michel; Plate, Karl H.; Imhof, Beat A.; Engelhardt, Britta

2012-01-01

The junctional adhesion molecule (JAM)-C is a widely expressed adhesion molecule regulating cell adhesion, cell polarity and inflammation. JAM-C expression and function in the central nervous system (CNS) has been poorly characterized to date. Here we show that JAM-C−/− mice backcrossed onto the C57BL/6 genetic background developed a severe hydrocephalus. An in depth immunohistochemical study revealed specific immunostaining for JAM-C in vascular endothelial cells in the CNS parenchyma, the meninges and in the choroid plexus of healthy C57BL/6 mice. Additional JAM-C immunostaining was detected on ependymal cells lining the ventricles and on choroid plexus epithelial cells. Despite the presence of hemorrhages in the brains of JAM-C−/− mice, our study demonstrates that development of the hydrocephalus was not due to a vascular function of JAM-C as endothelial re-expression of JAM-C failed to rescue the hydrocephalus phenotype of JAM-C−/− C57BL/6 mice. Evaluation of cerebrospinal fluid (CSF) circulation within the ventricular system of JAM-C−/− mice excluded occlusion of the cerebral aqueduct as the cause of hydrocephalus development but showed the acquisition of a block or reduction of CSF drainage from the lateral to the 3rd ventricle in JAM-C−/− C57BL/6 mice. Taken together, our study suggests that JAM-C−/− C57BL/6 mice model the important role for JAM-C in brain development and CSF homeostasis as recently observed in humans with a loss-of-function mutation in JAM-C. PMID:23029139

A novel microneedle array for the treatment of hydrocephalus.

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Oh, Jonghyun; Liu, Kewei; Medina, Tim; Kralick, Francis; Noh, Hongseok Moses

2014-06-01

We present a microfabricated 10 by 10 array of microneedles for the treatment of a neurological disease called communicating hydrocephalus. Together with the previously reported microvalve array, the current implantable microneedle array completes the microfabricated arachnoid granulations (MAGs) that mimic the function of normal arachnoid granulations (AGs). The microneedle array was designed to enable the fixation of the MAGs through dura mater membrane in the brain and thus provide a conduit for the flow of cerebrospinal fluid (CSF). Cone-shaped microneedles with hollow channels were fabricated using a series of microfabrication techniques: SU-8 photolithography for tapered geometry, reactive ion etching for sharpening the microneedles, 248 nm deep UV excimer laser machining for creating through-hole inside the microneedles, and metal sputtering for improved rigidity. Puncture tests were conducted using porcine dura mater and the results showed that the fabricated microneedle array is strong enough to pierce the dura mater. The in-vitro biocompatibility test result showed that none of the 100 outlets of the microneedles exposed to the bloodstream were clogged significantly by blood cells. We believe that these test results demonstrate the potential use of the microneedle array as a new treatment of hydrocephalus.

The association of congenital neuroblastoma and congenital heart disease

International Nuclear Information System (INIS)

Bellah, R.; D'Andrea, A.; Children's Hospital, Boston, MA; Darillis, E.; Fellows, K.E.

1989-01-01

Several authors have reported an association between neuroblastoma and congenital heart disease; others contend that, unlike specific wellknown associations between malignancy and congenital defects (Wilm's tumor and aniridia, leukemia and Down's syndrome), no real relationship exists. We present three cases of cyanotic congenital heart disease in which subclinical neuroblastoma was found. We speculate that abnormal neural crest cell migration and development may be a common link between cardiac malformations and congenital neuroblastoma. (orig.)

A hydroelastic model of hydrocephalus

Science.gov (United States)

Smillie, Alan; Sobey, Ian; Molnar, Zoltan

2005-09-01

We combine elements of poroelasticity and of fluid mechanics to construct a mathematical model of the human brain and ventricular system. The model is used to study hydrocephalus, a pathological condition in which the normal flow of the cerebrospinal fluid is disturbed, causing the brain to become deformed. Our model extends recent work in this area by including flow through the aqueduct, by incorporating boundary conditions that we believe accurately represent the anatomy of the brain and by including time dependence. This enables us to construct a quantitative model of the onset, development and treatment of this condition. We formulate and solve the governing equations and boundary conditions for this model and give results that are relevant to clinical observations.

Unilateral hallux valgus: is it true unilaterality, or does it progress to bilateral deformity?

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Young, Ki Won; Park, Young Uk; Kim, Jin Su; Jegal, Hyuk; Lee, Kyung Tai

2013-04-01

This study was undertaken to determine whether unilateral hallux valgus progresses unilaterally and to evaluate the demographics, etiologies, and radiographic findings associated with symptomatic unilateral hallux valgus deformities. Patients treated for hallux valgus between January 2004 and December 2008 were identified, and of these, 33 patients with unilateral deformities were enrolled. Progression of deformities in normal feet were evaluated at last follow-up visit, and the clinical information and radiographic measurements of those with a deformed normal foot or an unchanged normal foot were compared. Thirty-three patients (3.4%) had a unilateral hallux valgus deformity on preoperative radiographs. The mean length of follow up was 4.7 years (range, 2.4-11). Twenty-four cases had no deformity of the normal foot at last follow-up (the unchanged group), but 15 cases had developed hallux valgus deformity (the deformed group). No significant intergroup differences were found in terms of metatarsus adductus angle (P = .412), Meary angle (P = .771), talocalcaneal angle (P = 1.000), or calcaneal pitch angle (P = .267). However, members of the deformed group were significantly younger at disease onset (P = .045), exhibited a curved first metatarsal head (P = .046), and had a larger initial hallux valgus angle (P hallux valgus was found to be over 97.3%, and significant differences were found between the deformed and unchanged groups in terms of age of onset, metatarsal head shape, and hallux valgus angle.

Decorin alleviated chronic hydrocephalus via inhibiting TGF-β1/Smad/CTGF pathway after subarachnoid hemorrhage in rats.

Science.gov (United States)

Yan, Hui; Chen, Yujie; Li, Lingyong; Jiang, Jiaode; Wu, Guangyong; Zuo, Yuchun; Zhang, John H; Feng, Hua; Yan, Xiaoxin; Liu, Fei

2016-01-01

Chronic hydrocephalus is one of the severe complications after subarachnoid hemorrhage (SAH). However, there is no efficient treatment for the prevention of chronic hydrocephalus, partially due to poor understanding of underlying pathogenesis, subarachnoid fibrosis. Transforming growth factor-β1(TGF-β1) is a potent fibrogenic factor implicated in wide range of fibrotic diseases. To investigate whether decorin, a natural antagonist for TGF-β1, protects against subarachnoid fibrosis and chronic hydrocephalus after SAH, two-hemorrhage-injection SAH model was conducted in 6-week-old rats. Recombinant human decorin(rhDecorin) (30ug/2ul) was administered before blood injection and on the 10th day after SAH. TGF-β1, p-Smad2/3, connective tissue growth factor (CTGF), collagen I and pro-collagen I c-terminal propeptide were assessed via western blotting, enzyme-linked immunosorbent assay, radioimmunoassay and immunofluorescence. And neurobehavioral tests and Morris water maze were employed to evaluate long-term neurological functions after SAH. We found that SAH induced heightened activation of TGF-β1/Smad/CTGF axis, presenting as a two peak response of TGF-β1 in cerebrospinal fluid, elevation of TGF-β1, p-Smad2/3, CTGF, collagen I in brain parenchyma and pro-collagen I c-terminal propeptide in cerebrospinal fluid, and increased lateral ventricle index. rhDecorin treatment effectively inhibited up-regulation of TGF-β1, p-Smad2/3, CTGF, collagen I and pro-collagen I c-terminal propeptide after SAH. Moreover, rhDecorin treatment significantly reduced lateral ventricular index and incidence of chronic hydrocephalus after SAH. Importantly, rhDecorin improved neurocognitive deficits after SAH. In conclusion, rhDecorin suppresses extracellular matrix accumulation and following subarachnoid fibrosis via inhibiting TGF-β1/Smad/CTGF pathway, preventing development of hydrocephalus and attenuating long-term neurocognitive defects after SAH. Copyright © 2015 Elsevier B

Prevalence of congenital hereditary sensorineural deafness in Australian Cattle Dogs and associations with coat characteristics and sex

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Sommerlad Susan F

2012-10-01

Full Text Available Abstract Background Congenital hereditary sensorineural deafness (CHSD occurs in many dog breeds, including Australian Cattle Dogs. In some breeds, CHSD is associated with a lack of cochlear melanocytes in the stria vascularis, certain coat characteristics, and potentially, abnormalities in neuroepithelial pigment production. This study investigates phenotypic markers for CHSD in 899 Australian Cattle Dogs. Results Auditory function was tested in 899 Australian Cattle Dogs in family groups using brainstem auditory evoked response testing. Coat colour and patterns, facial and body markings, gender and parental hearing status were recorded. Deafness prevalence among all 899 dogs was 10.8% with 7.5% unilaterally deaf, and 3.3% bilaterally deaf, and amongst pups from completely tested litters (n = 696 was 11.1%, with 7.5% unilaterally deaf, and 3.6% bilaterally deaf. Univariable and multivariable analyses revealed a negative association between deafness and bilateral facial masks (odds ratio 0.2; P ≤ 0.001. Using multivariable logistic animal modelling, the risk of deafness was lower in dogs with pigmented body spots (odds ratio 0.4; P = 0.050. No significant associations were found between deafness and coat colour. Within unilaterally deaf dogs with unilateral facial masks, no association was observed between the side of deafness and side of mask. The side of unilateral deafness was not significantly clustered amongst unilaterally deaf dogs from the same litter. Females were at increased risk of deafness (odds ratio from a logistic animal model 1.9; P = 0.034 after adjusting for any confounding by mask type and pigmented body spots. Conclusions Australian Cattle Dogs suffer from CHSD, and this disease is more common in dogs with mask-free faces, and in those without pigmented body patches. In unilaterally deaf dogs with unilateral masks, the lack of observed association between side of deafness and side of mask suggests that if CHSD is due to

Ongoing search for diagnostic biomarkers in idiopathic normal pressure hydrocephalus.

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Tarnaris, Andrew; Toma, Ahmed K; Kitchen, Neil D; Watkins, Laurence D

2009-12-01

Idiopathic normal pressure hydrocephalus is a syndrome, which typically has a clinical presentation of gait/balance disturbance, often accompanied by cognitive decline and/or urinary incontinence. Its diagnosis is based on relevant history and clinical examination, appropriate imaging findings and physiological testing. The clinical picture of idiopathic normal pressure hydrocephalus may occasionally be difficult to distinguish from that of Alzheimer's dementia, subcortical ischemic vascular dementia and Parkinson's disease. The aim of this article is to systematically review the literature from the last 29 years in order to identify cerebrospinal fluid (CSF) or imaging biomarkers that may aid in the diagnosis of the syndrome. The authors concluded that no CSF or imaging biomarker is currently fulfilling the criteria required to aid in the diagnosis of the condition. However, a few studies have revealed promising CSF and imaging markers that need to be verified by independent groups. The reasons that the progress in this field has been slow so far is also commented on, as well as steps required to apply the current evidence in the design of future studies within the field.

Dandy Walker Syndrome with Unusual Associated Findings in a Fetal Autopsy Study

OpenAIRE

Surekha U Arakeri; Himanshu Mulay

2015-01-01

Dandy Walker Syndrome (DWS) is a congenital brain malformation characterized by hypoplasia or absence of cerebellar vermis, cystic dilatation of fourth ventricle and hydrocephalus. It is frequently associated with other congenital anomalies. Associated central nervous system anomalies such as agenesis of corpus callosum and vermis are associated with poor prognosis. Association of DWS with congenital absence of spleen is life threatening condition and has been reported...

The Management and Education of Children with Spina Bifida and Hydrocephalus.

Science.gov (United States)

Andrews, Robert J.; Elkins, John

The report describes the population of children in Australia with spina bifida and/or hydrocephalus, notes their needs and characteristics, reviews their school placement and social circumstances, and considers future educational services for them. Initial chapters review the literature on medical, psychoeducational, and social-family aspects of…

Posthemorrhagic Hydrocephalus in Newborns: Clinical Characteristics and Role of Ventriculoperitoneal Shunts

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Inn-Chi Lee

2009-02-01

Conclusion: Patients who were shunt-dependent had worse neurodevelopmental outcomes and greater mortality than those without shunts. The results appeared to depend on how far the hydrocephalus had progressed and on the degree of IVH, but the necessity of VP shunts requires reevaluation before they are implanted.

Cervical spine disease may result in a negative lumbar spinal drainage trial in normal pressure hydrocephalus: case report.

Science.gov (United States)

Komotar, Ricardo J; Zacharia, Brad E; Mocco, J; Kaiser, Michael G; Frucht, Stephen J; McKhann, Guy M

2008-10-01

In this case report, we present a patient with normal pressure hydrocephalus in whom a lumbar drainage trial yielded a false-negative result secondary to cervical spondylosis. An 80-year-old woman presented with classic symptoms of normal pressure hydrocephalus as well as evidence of cervical myelopathy. Magnetic resonance imaging of the brain and spine showed enlarged ventricles and single-level cervical canal narrowing. An initial lumbar drainage trial was performed, which revealed negative results. The patient then underwent cervical decompression and fusion. Despite this procedure, the patient's symptoms continued to worsen. A repeat lumbar drainage trial was performed with positive results. Subsequently, a ventriculoperitoneal shunt was placed, resulting in significant improvement of her symptoms. This case report illustrates how altered cerebrospinal fluid flow dynamics may impact the accuracy of the lumbar spinal drainage trial in patients with normal pressure hydrocephalus.

Non-invasive estimation of intracranial pressure. MR-based evaluation in children with hydrocephalus

International Nuclear Information System (INIS)

Muehlmann, M.; Steffinger, D.; Ertl-Wagner, B.; Koerte, I.K.; Peraud, A.; Lehner, M.; Heinen, F.; Alperin, N.

2012-01-01

The intracranial pressure (ICP) is a crucially important parameter for diagnostic and therapeutic decision-making in patients with hydrocephalus. So far there is no standard method to non-invasively assess the ICP. Various approaches to obtain the ICP semi-invasively or non-invasively are discussed and the clinical application of a magnetic resonance imaging (MRI)-based method to estimate ICP (MR-ICP) is demonstrated in a group of pediatric patients with hydrocephalus. Arterial inflow, venous drainage and craniospinal cerebrospinal fluid (CSF) flow were quantified using phase-contrast imaging to derive the MR-ICP. A total of 15 patients with hydrocephalus (n=9 treated with shunt placement or ventriculostomy) underwent MRI on a 3 T scanner applying retrospectively-gated cine phase contrast sequences. Of the patients six had clinical symptoms indicating increased ICP (age 2.5-14.61 years, mean 7.4 years) and nine patients had no clinical signs of elevated ICP (age 2.1-15.9 years; mean 9.8 years; all treated with shunt or ventriculostomy). Median MR-ICP in symptomatic patients was 24.5 mmHg (25th percentile 20.4 mmHg; 75th percentile 44.6 mmHg). Median MR-ICP in patients without acute signs of increased ICP was 9.8 mmHg (25th percentile 8.6 mmHg; 75th percentile 11.4 mmHg). Group differences were significant (p [de

[Idiopathic normal pressure hydrocephalus: High incidence in people over 80 years of age].

Science.gov (United States)

Aragonès, Josep Maria; Altimiras, Jacint; Alonso, Francisco; Roura, Pere; Alfonso, Sebastián; Bajo, Lorena

Idiopathic normal pressure hydrocephalus is usually observed in adults over 60 years of age. The highest incidence of cases is between 70 and 80 years-old, and it could be under-diagnosed in over 80 year-olds. A description is presented on the overall incidence and age group incidence, the delay in the diagnosis, and main outcomes. A descriptive study was performed on patients with idiopathic normal pressure hydrocephalus, in the population of Osona County during the years 2010-2015. The annual incidence rate was 4.43 per 100,000 inhabitants. The incidence increased with age; from 8.09 per 100,000 in the 60 to 69 years age group, to 23.61 per 100,000 in the 70-79 years age group of, and to 37.02 per 100,000 in the 80-89 years age. The delay in the diagnosis was 15.01 ± 10.35 months. All the patients improved after surgery, but only 73.3% of the patients maintained the improvement after one year. Idiopathic normal pressure hydrocephalus is an age related disease and probably underdiagnosed in the elderly. An early diagnosis and a clinical suspicion are essential in patients over 80 years old. Copyright © 2017 SEGG. Publicado por Elsevier España, S.L.U. All rights reserved.

An infant case with hydrocephalus as the initial manifestation of Mycoplasma hominis-associated meningitis.

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Taku, Keisuke; Hoshina, Takayuki; Haro, Kaoru; Ichikawa, Shun; Kinjo, Tadamune; Takahashi, Mayu; Akiba, Daisuke; Fukuda, Kazumasa; Kusuhara, Koichi

2017-10-01

We report an infant with hydrocephalus as the initial manifestation of Mycoplasma hominis-associated meningitis, who recovered without appropriate antimicrobial treatment. The analysis of the 16S rRNA gene by polymerase chain reaction amplification using universal primers and pathogen-specific primers was useful for the diagnosis and the investigation of serial detection status of the pathogen. This method may be helpful for the assessment of the frequency and the prediction of severity in M. hominis-associated central nervous system infection in infants, and investigating the association between M. hominis and the development of hydrocephalus. Copyright © 2017 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

Clinical and surgical management of unilateral prepubertal gynecomastia

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Giuseppe Andrea Ferraro

2014-01-01

CONCLUSION: The exact mechanism of unilateral gynecomastia formation in our case is unclear. The evaluation of unilateral gynecomastia can therefore be complex. In conclusion, the surgical treatment of unilateral gynecomastia requires an individual approach, based on an appropriate diagnostic algorithm.

Rectification of pulsatile stress on soft tissues: a mechanism for normal-pressure hydrocephalus

Science.gov (United States)

Jalikop, Shreyas; Hilgenfeldt, Sascha

2011-11-01

Hydrocephalus is a pathological condition of the brain that occurs when cerebrospinal fluid (CSF) accumulates excessively in the brain cavities, resulting in compression of the brain parenchyma. Counter-intuitively, normal-pressure hydrocephalus (NPH) does not show elevated pressure differences across the compressed parenchyma. We investigate the effects of nonlinear tissue mechanics and periodic driving in this system. The latter is due to the cardiac cycle, which provides significant intracranial pressure and volume flow rate fluctuations. Nonlinear rectification of the periodic driving within a model of fluid flow in poroelastic material can lead to compression or expansion of the parenchyma, and this effect does not rely on changes in the mean intracranial pressure. The rectification effects can occur gradually over several days, in agreement with clinical studies of NPH.

Novel Application of Time-Spatial Labeling Inversion Pulse Magnetic Resonance Imaging for Diagnosis of External Hydrocephalus.

Science.gov (United States)

Nakae, Shunsuke; Murayama, Kazuhiro; Adachi, Kazuhide; Kumai, Tadashi; Abe, Masato; Hirose, Yuichi

2018-01-01

Although a subdural fluid collection frequently is observed, diagnostic methods that differentiate between the subdural collection caused by external hydrocephalus and that caused by subdural hygroma have not been established. Here, we report a case of external hydrocephalus caused by Gliadel-induced eosinophilic meningitis that has been previously reported in only 1 case and can be diagnosed by time-spatial labeling inversion pulse magnetic resonance imaging (time-SLIP MRI). A tumor located in the left temporal was detected incidentally in an 81-year-old man by examination of a head injury. The tumor was surgically resected and diagnosed as a high-grade glioma during the surgery; Gliadel wafers subsequently were implanted. Three weeks after the resection, the patient showed disturbed consciousness, and computed tomography revealed a subdural fluid collection. The out-flow of cerebrospinal through the resection cavity was detected by time-SLIP MRI. Cerebrospinal tests indicated high white blood cell counts and high protein levels, with more than 90% of the white blood cell count comprising eosinophils. Therefore, we suspected that the subdural fluid collection was caused by external hydrocephalus because of Gliadel-induced eosinophilic meningitis. We surgically removed the Gliadel wafers and subsequently performed a surgery to insert a ventriculoperitoneal shunt. Histologic examination indicated eosinophilic accumulation around the Gliadel wafers. The patient's symptoms improved after the insertion of a ventriculoperitoneal shunt. In the present case, time-SLIP MRI was a useful and noninvasive method for diagnosing external hydrocephalus which was caused by eosinophilic meningitis because of Gliadel-induced eosinophilic meningitis. Copyright © 2017 Elsevier Inc. All rights reserved.

Posttraumatic hydrocephalus: a clinical, neuroradiologic, and neuropsychologic assessment of long-term outcome.

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Mazzini, Letizia; Campini, Riccardo; Angelino, Elisabetta; Rognone, Felice; Pastore, Ilaria; Oliveri, Giuseppe

2003-11-01

To detect the clinical and radiologic characteristics of posttraumatic hydrocephalus (PTH), to define its prognostic value, and to assess the effects of shunt surgery. Correlational study on a prospective cohort. Brain injury rehabilitation center. One hundred forty patients with severe traumatic brain injury (TBI) referred to an inpatient intensive rehabilitation unit of primary care in a university-based system. Not applicable. The Glasgow Outcome Scale (GOS), Disability Rating Scale (DRS), FIM instrument, and Neurobehavioural Rating Scale (NRS), as well as single-photon emission computed tomography (SPECT) and magnetic resonance imaging. PTH was found in 45% of patients. Risk factors for PTH were as follows: age (PTBI. It influences functional and behavioral outcome and the appearance of posttraumatic epilepsy. The selection of patients for surgery can be defined principally on a clinical basis. SPECT may be helpful for differentiating ventricular enlargement due to cortical atrophy and hydrocephalus.

Fontanelles - excessively large

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... Hydrocephalus Intrauterine growth retardation (IUGR) Premature birth Rarer causes: Achondroplasia Apert syndrome Cleidocranial dysostosis Congenital rubella Neonatal hypothyroidism Osteogenesis imperfecta Rickets When to Contact a Medical ...

Raquianestesia unilateral com bupivacaína hipobárica Raquianestesia unilateral con bupivacaína hipobárica Unilateral spinal anesthesia with hypobaric bupivacaine

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Luiz Eduardo Imbelloni

2002-09-01

Full Text Available JUSTIFICATIVA E OBJETIVOS: Um bloqueio simpático restrito durante raquianestesia pode minimizar as alterações hemodinâmicas. Teoricamente, o uso de soluções não isobáricas de anestésicos locais pode produzir anestesia unilateral e restringir a desnervação simpática a apenas um lado do corpo. A dose do anestésico local e o tempo que o paciente permanece em decúbito lateral para a realização da raquianestesia unilateral são desconhecidos. O presente estudo prospectivo investiga a incidência de raquianestesia unilateral utilizando bupivacaína a 0,15% preparada a partir de 1,5 ml de solução isobárica de bupivacaína adicionada de 25 µg fentanil, injetada através de agulha 27G tipo Quincke no paciente em decúbito lateral, com membro a ser operado voltado para cima. MÉTODO: Raquianestesia com 0,15% de bupivacaína mais fentanil foi realizada através da agulha 27G Quincke em 22 pacientes estado físico ASA I e II submetidos à cirurgias ortopédicas. A punção subaracnóidea foi realizada com o paciente previamente colocado com o lado a ser operado voltado para cima e foram retirados de 3 a 5 ml de LCR e injetados 5 ml da solução hipobárica na velocidade de 1 ml.15s-1. Bloqueios sensitivo e motor (picada de agulha e escala de 0 a 3 foram comparados entre os lados a ser operado e o contralateral. RESULTADOS: Os bloqueios motor e sensitivo entre o lado operado e o contralateral foram significativamente diferentes em todos os tempos em ambos os grupos. Raquianestesia unilateral foi obtida em 71% dos pacientes. Estabilidade hemodinâmica foi observada em todos os pacientes. Nenhum paciente desenvolveu cefaléia pós-raquianestesia. CONCLUSÕES: A bupivacaína hipobárica a 0,15% (7,5 mg associada ao fentanil proporciona um predominante bloqueio unilateral. Vinte minutos são suficientes para a instalação do bloqueio. As principais vantagens da raquianestesia unilateral são a estabilidade hemodinâmica, a satisfação do

Respiratory Polysomnographic Findings in Patients Treated Primarily for Unilateral Cleft Lip and Palate.

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Sobral, Davi Sandes; Faller, Gustavo Juliane; Collares, Marcus Vinícius Martins

2018-02-01

Cleft lip and palate (CLP) is the most common congenital craniofacial abnormality. Obstructive sleep apnea syndrome (OSAS) is a highly prevalent but underdiagnosed disease and is frequently associated with craniofacial anomalies. There are few studies describing the sleep breathing pattern of children with CLP. This study sought to characterize the respiratory profile of 23 children with unilateral cleft lip and palate, aged 7-12 years, who had undergone cleft lip and nasal repair at age 3-4 months and palatoplasty at 12-15 months, with a particular focus on evaluating the presence of OSAS in children with CLP. Polysomnography was performed and findings were analyzed descriptively. We found a mean and median for apnea/hypopnea index (AHI) of 1.11/h (SD = 0.78) and 0.9/h, respectively. The mean obstructive apnea index (OAI) was 0.27/h (SD = 0.38) and the median, 0.1/h. Nearly 30% of patients had an AHI above 1.4 events/h. There was no significant oxyhemoglobin desaturation in the study group. In this group, the prevalence of OSAS was higher than in noncleft populations when compared to the normality values adopted. This sample of patients with unilateral cleft lip and palate exhibited an increased prevalence of OSAS during the mixed dentition stage. Although the results showed that OSAS was mild, we advise closer observation of these patients. Polysomnography is recommended for the assessment of children with airway abnormalities, to individualize the extent of treatment.

Surgical outcomes for unilateral superior oblique palsy in Chinese population:a retrospective study

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Gordon Shing kin Yau

2015-02-01

Full Text Available AIM: To evaluate the outcome after surgery for unilateral superior oblique (SO palsy in Chinese. METHODS: The medical records of 39 patients that underwent surgery for unilateral SO palsy between January 2003 and December 2012 at Caritas Medical Centre, Hong Kong, were retrospectively reviewed. All surgeries were performed by a single surgeon. Pre-operative assessments for vertical deviation, cyclo-deviation, and Knapp’s classification were obtained to determine the nature and degree of surgical correction. Vertical deviation was measured at 1wk; 1, 6mo and on last follow-up day post-operatively. Cyclo-deviation was measured on last follow-up day post-operatively. RESULTS: During the 10y period, 39 subjects were recruited. The most common etiology was congenital (94.9%. Knapp’s Type III (66.7% and Type I (12.8% classifications were the most common subtypes. To treat SO palsy, the most common surgical procedures were: isolated inferior oblique (IO anteriorization (41.0%, isolated IO myectomy (10.3%, and isolated IO recession (10.3%. At 3.5±2.1y post-operatively, the vertical deviation was significantly reduced (15.1±6.2 PD versus 0.5±1.4 PD, PCONCLUSION: The majority of subjects achieved corrected vertical deviation after a single surgery although there was no improvement in cyclo-deviation. Those with over-correction of primary position deviation had greater preoperative vertical deviation and it may be related to simultaneous multiple muscle surgery.

3D mapping of cerebrospinal fluid local volume changes in patients with hydrocephalus treated by surgery: preliminary study

International Nuclear Information System (INIS)

Hodel, Jerome; Besson, Pierre; Pruvo, Jean-Pierre; Leclerc, Xavier; Rahmouni, Alain; Grandjacques, Benedicte; Luciani, Alain; Petit, Eric; Lebret, Alain; Outteryck, Olivier; Benadjaoud, Mohamed Amine; Maraval, Anne; Decq, Philippe

2014-01-01

To develop automated deformation modelling for the assessment of cerebrospinal fluid (CSF) local volume changes in patients with hydrocephalus treated by surgery. Ventricular and subarachnoid CSF volume changes were mapped by calculating the Jacobian determinant of the deformation fields obtained after non-linear registration of pre- and postoperative images. A total of 31 consecutive patients, 15 with communicating hydrocephalus (CH) and 16 with non-communicating hydrocephalus (NCH), were investigated before and after surgery using a 3D SPACE (sampling perfection with application optimised contrast using different flip-angle evolution) sequence. Two readers assessed CSF volume changes using 3D colour-encoded maps. The Evans index and postoperative volume changes of the lateral ventricles and sylvian fissures were quantified and statistically compared. Before surgery, sylvian fissure and brain ventricle volume differed significantly between CH and NCH (P = 0.001 and P = 0.025, respectively). After surgery, 3D colour-encoded maps allowed for the visual recognition of the CSF volume changes in all patients. The amounts of ventricle volume loss of CH and NCH patients were not significantly different (P = 0.30), whereas readjustment of the sylvian fissure volume was conflicting in CH and NCH patients (P < 0.001). The Evans index correlated with ventricle volume in NCH patients. 3D mapping of CSF volume changes is feasible providing a quantitative follow-up of patients with hydrocephalus. (orig.)

3D mapping of cerebrospinal fluid local volume changes in patients with hydrocephalus treated by surgery: preliminary study

Energy Technology Data Exchange (ETDEWEB)

Hodel, Jerome [Hopital Roger Salengro, Department of Neuroradiology, Lille (France); Hopital Roger Salengro, Service de Neuroradiologie, Lille (France); Besson, Pierre; Pruvo, Jean-Pierre; Leclerc, Xavier [Hopital Roger Salengro, Department of Neuroradiology, Lille (France); Rahmouni, Alain; Grandjacques, Benedicte; Luciani, Alain [Hopital Henri Mondor, Department of Radiology, Creteil (France); Petit, Eric; Lebret, Alain [Signals Images and Intelligent Systems Laboratory, Creteil (France); Outteryck, Olivier [Hopital Roger Salengro, Department of Neurology, Lille (France); Benadjaoud, Mohamed Amine [Radiation Epidemiology Team, CESP, Centre for Research in Epidemiology and Population Health U1018, Villejuif (France); Maraval, Anne [Hopital Henri Mondor, Department of Neuroradiology, Creteil (France); Decq, Philippe [Hopital Henri Mondor, Department of Neurosurgery, Creteil (France)

2014-01-15

To develop automated deformation modelling for the assessment of cerebrospinal fluid (CSF) local volume changes in patients with hydrocephalus treated by surgery. Ventricular and subarachnoid CSF volume changes were mapped by calculating the Jacobian determinant of the deformation fields obtained after non-linear registration of pre- and postoperative images. A total of 31 consecutive patients, 15 with communicating hydrocephalus (CH) and 16 with non-communicating hydrocephalus (NCH), were investigated before and after surgery using a 3D SPACE (sampling perfection with application optimised contrast using different flip-angle evolution) sequence. Two readers assessed CSF volume changes using 3D colour-encoded maps. The Evans index and postoperative volume changes of the lateral ventricles and sylvian fissures were quantified and statistically compared. Before surgery, sylvian fissure and brain ventricle volume differed significantly between CH and NCH (P = 0.001 and P = 0.025, respectively). After surgery, 3D colour-encoded maps allowed for the visual recognition of the CSF volume changes in all patients. The amounts of ventricle volume loss of CH and NCH patients were not significantly different (P = 0.30), whereas readjustment of the sylvian fissure volume was conflicting in CH and NCH patients (P < 0.001). The Evans index correlated with ventricle volume in NCH patients. 3D mapping of CSF volume changes is feasible providing a quantitative follow-up of patients with hydrocephalus. (orig.)

Adjustable valves in normal-pressure hydrocephalus: a retrospective study of 218 patients

DEFF Research Database (Denmark)

Zemack, G.; Rommer, Bertil Roland

2008-01-01

OBJECTIVE: We sought to assess the value of adjusting shunt valve opening pressure, complications, and outcomes with the use of an adjustable shunt valve in the treatment of patients with normal-pressure hydrocephalus (NPH). METHODS: In a single-center retrospective study, 231 adjustable valves...

Congenital malformations of the external and middle ear: high-resolution CT findings of surgical import

International Nuclear Information System (INIS)

Swartz, J.D.; Faerber, E.N.

1985-01-01

The external auditory canal, middle ear, and bulk of the ossicular chain develop from the first branchial groove, first and second branchial arches, and first pharyngeal pouch. Embryologic development of these structures is complex and only rarely are two anomalies identical. This study includes 11 cases of unilateral external auditory canal atresia and two cases of bilateral atresia. Eight cases (four bilateral) of isolated congenital ossicular anomalies are also included. Emphasis is placed on findings of surgical import. All patients were studied with computed tomography only, because it was believed that the bony and soft-tissue detail achieved is superior to that with conventional multidirectional tomography

Surgical Treatment Of Congenital Radioulnar Synostosis In Children: A Case Report

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Achraf El Bakkaly

2018-01-01

Full Text Available Congenital radioulnar synostosis is an uncommon condition, only 400 cases have been reported in the world literature. Often bilateral, it can be recognized from birth by a clinical examination if it is attentive (examination of the prono-supination of the two elbows. It is in fact often discovered later, especially in unilateral forms, in children of school age. The proximal congenital radioulnar synostosis often results in functional, cosmetic limitations of the upper limb especially in the bilateral forms. Rotational osteotomy through the synostosis site is the usual procedure. The techniques and sites envisaged for this surgery are numerous, with multiple difficulties, risks and possible complications. We propose the observation of an eight-year-old boy with a symptomatic form of proximal radioulnar synostosis. The surgical treatment consisted of a resection of the synostosis with rotational transversalosteotomy of the two bones of the forearm completed by a plaster in neutral position. Clinical results, evaluated at an average of twelve months postoperatively, were found to be satisfactorily on aesthetic and functional levels. The aim of our work is to highlight our simple and reliable surgical procedure of the osteotomy of the synostosis maintained by spindles, allowing the functional improvement of the movements of the forearm of our child and thus a normal life.

Psychiatric symptoms are present in most of the patients with idiopathic normal pressure hydrocephalus

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Matheus F. Oliveira

2014-06-01

Full Text Available Normal pressure hydrocephalus (NPH is characterized by gait disturbance, dementia and/or urinary incontinence associated with dilation of ventricular system with normal opening cerebrospinal fluid pressure. Wide scientifical evidence confirms association between NPH and psychiatric symptoms. We selected 35 patients with idiopathic normal pressure hydrocephalus from January 2010 to January 2012 in a Brazilian tertiary hospital and performed a formal psychiatric evaluation to identify psychiatric disorders. Psychiatric disorders were present in 71% of these patients, especially anxiety, depression and psychotic syndromes. NPH patients may develop symptoms with frontal dominance, such as personality changes, anxiety, depression, psychotic syndromes, obsessive compulsive disorder, Othello syndrome; shoplifting and mania. Unusual appearances of NPH symptoms may hinder early diagnosis and consequently proper treatment.

Kniest disease with Pierre Robin syndrome and hydrocephalus

International Nuclear Information System (INIS)

Silengo, M.C.; Davi, G.F.; Bianco, R.; DeMarco, A.; Franceschini, P.

1983-01-01

A 3-month-old male infant with clinical and radiological features of Kniest disease is reported. Additional findings were severe Pierre Robin syndrome and external hydrocephalus. The patient was retarded in mental and motor development. He died at 4 months of age from the complications of tracheostomy. The parents were both normal clinically and radiologically, thus the disease in the child was presumably due to a new mutation. The reported familial cases of Kniest disease suggest autosomal dominant inheritance. The differential diagnosis is discussed in detail. (orig.)

Prevalence, heritability and genetic correlations of congenital sensorineural deafness and pigmentation phenotypes in the Border Collie.

Science.gov (United States)

De Risio, Luisa; Lewis, Tom; Freeman, Julia; de Stefani, Alberta; Matiasek, Lara; Blott, Sarah

2011-06-01

The objectives of this study were to estimate prevalence, heritability and genetic correlations of congenital sensorineural deafness (CSD) and pigmentation phenotypes in the Border Collie. Entire litters of Border Collies that presented to the Animal Health Trust (1994-2008) for assessment of hearing status by brain stem auditory evoked response (BAER) at 4-10 weeks of age were included. Heritability and genetic correlations were estimated using residual maximum likelihood (REML). Of 4143 puppies that met the inclusion criteria, 97.6% had normal hearing status, 2.0% were unilaterally deaf and 0.4% were bilaterally deaf. Heritability of deafness as a trichotomous trait (normal/unilaterally deaf/bilaterally deaf) was estimated at 0.42 using multivariate analysis. Genetic correlations of deafness with iris colour and merle coat colour were 0.58 and 0.26, respectively. These results indicate that there is a significant genetic effect on CSD in Border Collies and that some of the genes determining deafness also influence pigmentation phenotypes. Copyright © 2010 Elsevier Ltd. All rights reserved.

Microstructure of transcallosal motor fibers reflects type of cortical (re-)organization in congenital hemiparesis.

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Juenger, Hendrik; Koerte, Inga K; Muehlmann, Marc; Mayinger, Michael; Mall, Volker; Krägeloh-Mann, Ingeborg; Shenton, Martha E; Berweck, Steffen; Staudt, Martin; Heinen, Florian

2014-11-01

Early unilateral brain lesions can lead to different types of corticospinal (re-)organization of motor networks. In one group of patients, the contralesional hemisphere exerts motor control not only over the contralateral non-paretic hand but also over the (ipsilateral) paretic hand, as the primary motor cortex is (re-)organized in the contralesional hemisphere. Another group of patients with early unilateral lesions shows "normal" contralateral motor projections starting in the lesioned hemisphere. We investigated how these different patterns of cortical (re-)organization affect interhemispheric transcallosal connectivity in patients with congenital hemiparesis. Eight patients with ipsilateral motor projections (group IPSI) versus 7 patients with contralateral motor projections (group CONTRA) underwent magnetic resonance diffusion tensor imaging (DTI). The corpus callosum (CC) was subdivided in 5 areas (I-V) in the mid-sagittal slice and volumetric information. The following diffusion parameters were calculated: fractional anisotropy (FA), trace, radial diffusivity (RD), and axial diffusivity (AD). DTI revealed significantly lower FA, increased trace and RD for group IPSI compared to group CONTRA in area III of the corpus callosum, where transcallosal motor fibers cross the CC. In the directly neighboring area IV, where transcallosal somatosensory fibers cross the CC, no differences were found for these DTI parameters between IPSI and CONTRA. Volume of callosal subsections showed significant differences for area II (connecting premotor cortices) and III, where group IPSI had lower volume. The results of this study demonstrate that the callosal microstructure in patients with congenital hemiparesis reflects the type of cortical (re-)organization. Early lesions disrupting corticospinal motor projections to the paretic hand consecutively affect the development or maintenance of transcallosal motor fibers. Copyright © 2014 European Paediatric Neurology Society

Medication overuse as a cause of chronic headache in shunted hydrocephalus patients

DEFF Research Database (Denmark)

Willer, Lasse; Jensen, R H; Juhler, M

2010-01-01

To highlight the group of hydrocephalus patients known to have a long history of shunt revisions and refractory chronic headache. When a shunt in perfect working order has no effect on headache, other causes of headache should be investigated. In this paper, patients with medication overuse...

The relationship between intellectual skills and the computerised axial tomograms of children with spina bifida and hydrocephalus

International Nuclear Information System (INIS)

Lonton, A.P.

1979-01-01

Cranial CT-scans of 467 patients were graded in degrees of severity of hydrocephalus. The patients were aged from 2-22 years and had spina bifida and/or hydrocephalus or related disorders. Even large increases in the degree of hydrocephalus were found to have an insignificant effect upon verbal IQ, but there was a small but statistically significant effect upon performance scale IQ on the WISC. The abilities most affected were those involving the use of motor and perceptuo-motor skills. Assymmetrical or other abnormal scans were found to be associated with lower IQ. Children with valves were only found to be substantially inferior in skills to those without valves when their ventricles were either very large, or abnormally small. The highest proportion of children with valves was found in the group with the smallest ventricles, followed by the group with the largest ventricles. Very small ventricles are known to be associated with an increased rate of valve complications, and this paper shows that there are psychological disadvantages also. (orig.) [de

The Results of Neuroendoscopic Surgery in Patients with Posttraumatic and Posthemorrhagic Hydrocephalus.

Science.gov (United States)

Chrastina, Jan; Novák, Zdeněk; Zeman, Tomáš; Feitová, Věra; Hrabovský, Dušan; Říha, Ivo

2018-05-01

Posttraumatic hydrocephalus (PTH) and posthemorrhagic hydrocephalus (PHH) were previously considered not suitable for neuroendoscopic treatment. New hydrocephalus theories support possible successful neuroendoscopy in such patients. This study presents the results of neuroendoscopy in PTH and PHH with a background analysis. From 130 hydrocephalic patients after neuroendoscopic surgeries, 35 cases with PTH (n = 11) or PHH (n = 24; acute: n = 9, subacute: n = 10, chronic: n = 5) were found. The success rate (Glasgow Outcome Scale [GOS] score 4 or 5 without shunt) and clinical outcome (GOS score) of endoscopic third ventriculostomy (ETV) were analyzed. During the study period, 34 patients had ventriculoperitoneal shunts implanted, including 2 PTH and 5 PHH patients (all chronic). The success rate of ETV in PTH was 54.5%. In acute PHH, the success rate was 33.3%, 42.8% after excluding devastating hematomas. A post-ETV shunt was implanted in 1 patient (massive subarachnoid hemorrhage [SAH]) with final GOS score of 5. In subacute cases, the ETV success rate was 40% (no post-ETV shunts). In chronic PHH, only 1 patient with a GOS score of 5 was shunt-free (20%). The cause of ETV failure was massive SAH. Low final GOS score was caused by the extent of intracerebral bleeding or extracranial problems. The main indications for primary shunt implantation in PTH and PHH were infectious complications. The rate of good outcomes was 0% in PTH and 40% in PHH. The best results of neuroendoscopy were achieved in PTH and acute PHH. ETV failures were associated with massive SAH; arachnoid cistern blockage and scarring precludes ETV success. Copyright © 2018 Elsevier Inc. All rights reserved.

Characteristics of children with unilateral hearing loss.

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Fitzpatrick, Elizabeth M; Al-Essa, Rakan S; Whittingham, JoAnne; Fitzpatrick, Jessica

2017-11-01

The purpose of this study was to describe the clinical characteristics of children with unilateral hearing loss (UHL), examine deterioration in hearing, and explore amplification decisions. Population-based data were collected prospectively from time of diagnosis. Serial audiograms and amplification details were retrospectively extracted from clinical charts to document the trajectory and management of hearing loss. The study included all children identified with UHL in one region of Canada over a 13-year period (2003-2015) after implementation of universal newborn hearing screening. Of 537 children with permanent hearing loss, 20.1% (108) presented with UHL at diagnosis. They were identified at a median age of 13.9 months (IQR: 2.8, 49.0). Children with congenital loss were identified at 2.8 months (IQR: 2.0, 3.6) and made up 47.2% (n = 51), reflecting that a substantial portion had late-onset, acquired or late-identified loss. A total of 42.4% (n = 39) showed deterioration in hearing, including 16 (17.4%) who developed bilateral loss. By study end, 73.1% (79/108) of children had received amplification recommendations. Up to 20% of children with permanent HL are first diagnosed with UHL. About 40% are at risk for deterioration in hearing either in the impaired ear and/or in the normal hearing ear.

Unilateral Cleft Hand with Cleft Foot

Science.gov (United States)

Baba, Asif Nazir; Bhat, Yasmeen J.; Ahmed, Sheikh Mushtaq; Nazir, Abid

2009-01-01

Congenital anomalies of the hand form an important class of congenital malformations. They have a huge functional importance because of the part played by the hand in the daily activities of a person. The deformities also have significant cosmetic significance and may also be associated with other anomalies. Amongst the congenital anomalies, central deficiency or cleft hand is relatively rare. The association of cleft foot with cleft hand is an even more rare occurance. We present a case report of a 6 year old child, born of a non-consanginous marriage, having congenital central deficiency of ipsilateral hand and foot. PMID:21475543

Left hemisphere structural connectivity abnormality in pediatric hydrocephalus patients following surgery

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Weihong Yuan

2016-01-01

Full Text Available Neuroimaging research in surgically treated pediatric hydrocephalus patients remains challenging due to the artifact caused by programmable shunt. Our previous study has demonstrated significant alterations in the whole brain white matter structural connectivity based on diffusion tensor imaging (DTI and graph theoretical analysis in children with hydrocephalus prior to surgery or in surgically treated children without programmable shunts. This study seeks to investigate the impact of brain injury on the topological features in the left hemisphere, contratelateral to the shunt placement, which will avoid the influence of shunt artifacts and makes further group comparisons feasible for children with programmable shunt valves. Three groups of children (34 in the control group, 12 in the 3-month post-surgery group, and 24 in the 12-month post-surgery group, age between 1 and 18 years were included in the study. The structural connectivity data processing and analysis were performed based on DTI and graph theoretical analysis. Specific procedures were revised to include only left brain imaging data in normalization, parcellation, and fiber counting from DTI tractography. Our results showed that, when compared to controls, children with hydrocephalus in both the 3-month and 12-month post-surgery groups had significantly lower normalized clustering coefficient, lower small-worldness, and higher global efficiency (all p < 0.05, corrected. At a regional level, both patient groups showed significant alteration in one or more regional connectivity measures in a series of brain regions in the left hemisphere (8 and 10 regions in the 3-month post-surgery and the 12-month post-surgery group, respectively, all p < 0.05, corrected. No significant correlation was found between any of the global or regional measures and the contemporaneous neuropsychological outcomes [the General Adaptive Composite (GAC from the Adaptive Behavior Assessment System, Second

A unifying hypothesis for hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida

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Williams Helen

2008-04-01

Full Text Available Abstract This work is a modified version of the Casey Holter Memorial prize essay presented to the Society for Research into Hydrocephalus and Spina Bifida, June 29th 2007, Heidelberg, Germany. It describes the origin and consequences of the Chiari malformation, and proposes that hydrocephalus is caused by inadequate central nervous system (CNS venous drainage. A new hypothesis regarding the pathogenesis, anencephaly and spina bifida is described. Any volume increase in the central nervous system can increase venous pressure. This occurs because veins are compressible and a CNS volume increase may result in reduced venous blood flow. This has the potential to cause progressive increase in cerebrospinal fluid (CSF volume. Venous insufficiency may be caused by any disease that reduces space for venous volume. The flow of CSF has a beneficial effect on venous drainage. In health it moderates central nervous system pressure by moving between the head and spine. Conversely, obstruction to CSF flow causes localised pressure increases, which have an adverse effect on venous drainage. The Chiari malformation is associated with hindbrain herniation, which may be caused by low spinal pressure relative to cranial pressure. In these instances, there are hindbrain-related symptoms caused by cerebellar and brainstem compression. When spinal injury occurs as a result of a Chiari malformation, the primary pathology is posterior fossa hypoplasia, resulting in raised spinal pressure. The small posterior fossa prevents the flow of CSF from the spine to the head as blood enters the central nervous system during movement. Consequently, intermittent increases in spinal pressure caused by movement, result in injury to the spinal cord. It is proposed that posterior fossa hypoplasia, which has origins in fetal life, causes syringomyelia after birth and leads to damage to the spinal cord in spina bifida. It is proposed that hydrocephalus may occur as a result of