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Sample records for meningomyelocele

  1. Intracranial structures of meningomyelocels studied by computed tomography

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    Asada, M.; Tamaki, N.; Matsumoto, S. (Kobe Univ. (Japan). School of Medicine)

    1980-10-01

    We have had experience with forty-two patients with meningomyeloceles; their intracranial structures have been studied and analysed by CT. Six patients (16%) revealed no abnormalities, while thirty-six (82%) had hydrocephalus. All the cases have undergone repair of the meningomyelocele, and thirty-five parties, a shunt operation for hydrocephalus. The CT analysis of the intracranial structures of the meningomyelocele revealed that scaphocephalus was present in 40%, posteriorly dominant ventricular dilatation in the lateral ventricle in 73%, and enlarged massa intermedia in 54%. The following postoperative changes were found: sharp edges of the anterior and posterior horns were found in 81%, prominent dilatation of quadrigeminal and retrothalamic cisterns in 76%, and the interdigitation of the medial cerebral cortex in 69%. In the posterior fossa, a hypoplastic cerebellar tentorium was found in 70% of the cases and a pear-shaped deformity of the upper cerebellum in 62%. The brain stem was enclosed laterally by the anteriorly situated ventral portion of the cerebellum in 74%, and the fourth ventricle was collapsed or narrowed in 76%. An absence of the septum pellucidum was associated in six cases; a quadrigeminal cyst, in two, and a cavum septi pellucidum, in one. CT is a useful and safe apparatus for evaluating the intracranial structures of Arnold-Chiari malformations. Trivial morphological changes, such as the ectopic gray matter, beaking tectum, enlarged accessory commissure, and aqueduct stenosis observed in autopsy cases, are still impossible to demonstrate on CT. It is, however, hoped that with the improvement of the CT image, the sagittal image will become more precise for evaluating the downward displacement of the brain stem and the fourth ventricle.

  2. Intracranial structures of meningomyelocels studied by computed tomography

    International Nuclear Information System (INIS)

    Asada, Masahiro; Tamaki, Norihiko; Matsumoto, Satoshi

    1980-01-01

    We have had experience with forty-two patients with meningomyeloceles; their intracranial structures have been studied and analysed by CT. Six patients (16%) revealed no abnormalities, while thirty-six (82%) had hydrocephalus. All the cases have undergone repair of the meningomyelocele, and thirty-five parties, a shunt operation for hydrocephalus. The CT analysis of the intracranial structures of the meningomyelocele revealed that scaphocephalus was present in 40%, posteriorly dominant ventricular dilatation in the lateral ventricle in 73%, and enlarged massa intermedia in 54%. The following postoperative changes were found: sharp edges of the anterior and posterior horns were found in 81%, prominent dilatation of quadrigeminal and retrothalamic cisterns in 76%, and the interdigitation of the medial cerebral cortex in 69%. In the posterior fossa, a hypoplastic cerebellar tentorium was found in 70% of the cases and a pear-shaped deformity of the upper cerebellum in 62%. The brain stem was enclosed laterally by the anteriorly situated ventral portion of the cerebellum in 74%, and the fourth ventricle was collapsed or narrowed in 76%. An absence of the septum pellucidum was associated in six cases; a quadrigeminal cyst, in two, and a cavum septi pellucidum, in one. CT is a useful and safe apparatus for evaluating the intracranial structures of Arnold-Chiari malformations. Trivial morphological changes, such as the ectopic gray matter, beaking tectum, enlarged accessory commissure, and aqueduct stenosis observed in autopsy cases, are still impossible to demonstrate on CT. It is, however, hoped that with the improvement of the CT image, the sagittal image will become more precise for evaluating the downward displacement of the brain stem and the fourth ventricle. (author)

  3. Pathogenesis of cerebral malformations in human fetuses with meningomyelocele

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    Brouwer Oebele F

    2008-03-01

    Full Text Available Abstract Background Fetal spina bifida aperta (SBA is characterized by a spinal meningomyelocele (MMC and associated with cerebral pathology, such as hydrocephalus and Chiari II malformation. In various animal models, it has been suggested that a loss of ventricular lining (neuroepithelial/ependymal denudation may trigger cerebral pathology. In fetuses with MMC, little is known about neuroepithelial/ependymal denudation and the initiating pathological events. The objective of this study was to investigate whether neuroepithelial/ependymal denudation occurs in human fetuses and neonates with MMC, and if so, whether it is associated with the onset of hydrocephalus. Methods Seven fetuses and 1 neonate (16–40 week gestational age, GA with MMC and 6 fetuses with normal cerebral development (22–41 week GA were included in the study. Identification of fetal MMC and clinical surveillance of fetal head circumference and ventricular width was performed by ultrasound (US. After birth, MMC was confirmed by histology. We characterized hydrocephalus by increased head circumference in association with ventriculomegaly. The median time interval between fetal cerebral ultrasound and fixing tissue for histology was four days. Results At 16 weeks GA, we observed neuroepithelial/ependymal denudation in the aqueduct and telencephalon together with sub-cortical heterotopias in absence of hydrocephalus and/or Chiari II malformation. At 21–34 weeks GA, we observed concurrence of aqueductal neuroepithelial/ependymal denudation and progenitor cell loss with the Chiari II malformation, whereas hydrocephalus was absent. At 37–40 weeks GA, neuroepithelial/ependymal denudation coincided with Chiari II malformation and hydrocephalus. Sub-arachnoidal fibrosis at the convexity was absent in all fetuses but present in the neonate. Conclusion In fetal SBA, neuroepithelial/ependymal denudation in the telencephalon and the aqueduct can occur before Chiari II malformation

  4. Meningomyelocele Repair in a Premature Newborn with Hydrocephalus: Anaesthetic Confronts and Management

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    Sandhya Ghodke

    2017-07-01

    Full Text Available Deficit of neural tube closure in the initial phases of intrauterine development leads to a gamut of abnormalities ranging from spina-bifida occulta, a relatively benign condition, to encephalocele and meningomyelocele, an anomaly in vertebral bodies, spinal cord and sometimes involving brainstem (in cervical meningomyelocele. Meningomyelocele is the most common nonlethal malformation in the spectrum of neural tube deficits. The intrinsic challenges associated with the latter disorder warrants tailor-made approaches for providing anaesthesia to the requisite therapeutic surgical interventions. Pediatric patients pose a set of natural barriers because of their ever budding and maturing neurophysiological status, apart from the central neural disease process. Hence, in order to provide optimal neuro-anaesthetic care, the anaesthesiologist must have the knowledge of the outcomes of various pharmacologic interventions on cerebral aerodynamics apart from his professional experience in pediatric neuroanaesthesia. The current case report accounts for a challenging anaesthetic management in a premature newborn having hydrocephalus and lumbosacral meningomyelocele, presented for surgical repair within four hours of delivery.

  5. Syringohydromyelia following meningomyelocele surgery - role of metrizamide myelography and computed tomography

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    Stanley, P.; Senac, M.O. Jr.; Segall, H.D.; Park, T.S.

    1984-01-01

    Five children developed symptoms attributable to syringohydromyelia several years following neonatal surgery for lumbosacral meningomyelocele. Metrizamide injection followed by computed tomography (CT) was performed in all five patients. In two patients, there was direct opacification of both the subarachnoid space and the syringohydromyelia. In a further two patients, delayed CT demonstrated late opacification of the central cavity from contrast introduced into the subarachnoid space. In the fifth patient via a suboccipital puncture, there was fortuitous filling of a caudally displaced fourth ventricle communicating with a central cavity within the cord, but there was no visualization of the subarachnoid space. Surgery with decompression and plugging of the obex halted the progression of the disease in all the patients. (orig.)

  6. Ultrasonography to visualize the upper urinary tract in children with meningomyelocele.

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    Abrahamsson, Kate; Jodal, Ulf; Stokland, Eira; Nordvall, Anders; Sillén, Ulla

    2006-10-01

    To evaluate the frequency of difficulties when using ultrasonography (US, commonly used to visualize the upper urinary tract) to evaluate renal length and dilatation in children and adolescents with meningomyelocele (MMC), who have excessive obesity or a distorted spine. The records of all children with MMC investigated during 1996-2002 were assessed retrospectively and the last investigation used for analysis. In addition to the US results, the body mass index (BMI) and angulation of the spine were recorded. Of the 160 children investigated, US was not evaluable in 46 (29%), i.e. in 35 (22%) for renal length, in one (1%) for dilatation and in 10 (6%) for both. In 99 patients with a straight spine and mild to moderate angulation, renal length was not measurable in 14 (14%), while dilatation was not evaluable in two (2%). In 61 patients with a severely angled spine, the corresponding values were 31 (51%) and nine (15%), respectively. In eight patients with a BMI of >or= 27 kg/m2 and a straight spine, four of the investigations were not completely evaluable, while six were not in the eight patients with both a BMI of >or= 27 kg/m2 and severe angulation. Both severe spinal angulation and a BMI of >or= 27 kg/m2 significantly reduced the possibility of evaluating the urinary tract by US. However, in an unselected group of children with MMC, dilatation could be evaluated in >90% and renal length in approximately 70%.

  7. Velo-Cardio-Facial syndrome and DiGeorge sequence with meningomyelocele and deletions of the 22q11 region

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    Nickel, R.E.; Pillers, D.M.; Merkens, M.; Magenis, R.E.; Zonana, J. [Oregon Health Sciences Univ., Portland, OR (United States); Driscoll, D.A.; Emanuel, B.S. [Univ. of Pennsylvania Medical Center, Philadelphia, PA (United States)

    1994-10-01

    Approximately 5% of children with neural tube defects (NTDs) have a congenital heart defect and/or cleft lip and palate. The cause of isolated meningomyelocele, congenital heart defects, or cleft lip and palate has been largely thought to be multifactorial. However, chromosomal, teratogenic, and single gene causes of combinations of NTDs with congenital heart defects and/or cleft lip and palate have been reported. We report on 3 patients with meningomyelocele, congenital heart defects, and 22q11 deletions. Two of the children had the clinical diagnosis of velo-cardio-facial syndrome (VCFS); both have bifid uvula. The third child had DiGeorge sequence (DGS). The association of NTDs with 22q11 deletion has not been reported previously. An accurate diagnosis of the 22q11 deletion is critical as this micro-deletion and its associated clinical problems is transmitted as an autosomal dominant trait due to the inheritance of the deletion-bearing chromosome. We recommend that all children with NTDs and congenital heart defects, with or without cleft palate, have cytogenetic and molecular studies performed to detect 22q11 deletions. 31 refs., 3 figs.

  8. Achados oculares em pacientes com mielomeningocele: 72 casos Ocular findings in 72 patients with meningomyelocele

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    Mônica Fialho Cronemberger

    2000-10-01

    Full Text Available Objetivo: Estudar as alterações oculares em pacientes com mielomeningocele. Material e Método: Realizado estudo retrospectivo em 72 pacientes com mielomeningocele, descrevendo as alterações oculares presentes nessa população, além de correlacionar com a presença ou não de hidrocefalia. Resultados: Dos 72 pacientes com mielomeningocele, com idade variando de 5 meses a 18 anos, 64 (88,9% pacientes tinham hidrocefalia. Destes 64 pacientes, 36 (56,3% tinham estrabismo, sendo que 21 (58,3% eram endotrópicos, 13 (36,1% exotrópicos, 1 (2,8% exofórico e 1 (2,8% com estrabismo discinético. Do total dos 72 pacientes estudados 38 (52,8% eram estrábicos. A anisotropia foi encontrada em 16 (22,2% pacientes, sendo que em 14 (87,5% em A e em 2 (12,5% em V. Todos os 16 pacientes com anisotropia apresentavam hidrocefalia. O erro refrativo mais freqüente foi a hipermetropia encontrada em 64 (44,4% olhos. Atrofia óptica foi encontrada em 9 (12,5% pacientes. Conclusão: A mielomeningocele, associada a hidrocefalia, apresenta uma porcentagem de estrabismo maior do que o encontrado na população normal.Purpose: To study ocular findings in patients with meningomyelocele. Methods: A retrospective study of 72 patients with myelomeningocele was performed to evaluate ocular motility disorders, refractive errors and indirect ophthalmoscopy findings. Results: In a group of 72 patients with myelomeningocele, aged 5 month -- 18 years, 64 (88.9% had hydrocephalus. Of this group of 64 patients, 36 (56.3% had strabismus, 21 (58.3% of whom were esotropic, and 13 (36.1% exotropic, 1 (2.8% exophoric and 1 (2.8% presented sometimes esotropia and sometimes exotropia. Of the total of 72 studied patients, 38 (52.8% had strabismus. Anisotropia was found in 16 (22.2% patients, 14 (87.5% with an A pattern and 2 (12.5% with a V pattern. The most frequent refractive error was hyperopia, found in 64 (44.4% eyes. All the 16 patients with anisotropia had hydrocephalus

  9. Spinal cord anomalies in patients with anorectal malformations without severe sacral abnormalities or meningomyelocele: outcomes after expectant, conservative management.

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    Kyrklund, Kristiina; Pakarinen, Mikko P; Taskinen, Seppo; Kivisaari, Reetta; Rintala, Risto J

    2016-12-01

    OBJECTIVE The goal of this study was to determine the significance of spinal cord anomalies (SCAs) in patients with anorectal malformations (ARMs) by comparing the outcomes for bowel function, lower urinary tract symptoms (LUTS), and lower-limb neurological abnormalities to these outcomes in patients with similar ARMs and a normal spinal cord. METHODS The spinal cord MRI records of female patients treated for vestibular and perineal fistula (VF/PF) and male patients with rectourethral fistula (RUF) at a single center between 1983 and 2006 were reviewed. Bowel function and LUTS were assessed by questionnaire. Patients with extensive sacral anomalies or meningomyelocele were excluded. RESULTS Of 89 patients (median age 15 years, range 5-29 years), MRI was available in 90% (n = 80; 40 male patients with RUF), and 80% of patients returned the questionnaire (n = 64; 31 male patients with RUF). Spinal cord anomalies were found in 34%, comprising a filum terminale lipoma in 30%, low conus medullaris in 10%, and thoracolumbar syrinx in 6%. Bowel functional outcomes between patients with SCAs (n = 23) and those with a normal spinal cord (n = 41) were not significantly different for soiling (70% vs 63%), fecal accidents (43% vs 34%), and constipation (57% vs 39%; p = not significant for all). The LUTS, including urge (65% vs 54%), urge incontinence (39% vs 24%), stress incontinence (17% vs 22%), and straining (32% vs 29%) were also comparable between groups (p = not significant for all). No patients developed lower-limb neurological abnormalities. CONCLUSIONS The results suggest that the long-term functional outcomes for patients with SCAs who had VF/PF and RUF may not differ significantly from patients with the same type of ARMs and a normal spinal cord. The results favor a conservative approach to their management in the absence of abnormal neurological findings in the lower limbs.

  10. Effects of reading goals on reading comprehension, reading rate, and allocation of working memory in children and adolescents with spina bifida meningomyelocele

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    ENGLISH, LIANNE; BARNES, MARCIA A.; FLETCHER, JACK M.; DENNIS, MAUREEN; RAGHUBAR, KIMBERLY P.

    2011-01-01

    Spina bifida meningomyelocele (SBM) is a neurodevelopmental disorder associated with intact word decoding and deficient text and discourse comprehension. This study investigated the ability to adjust reading in accordance with specified reading goals in 79 children and adolescents with SBM (9–19 years of age) and 39 controls (8–17 years of age). Both groups demonstrated slower reading times and enhanced comprehension when reading to study or to come up with a title than when reading for specific information or for entertainment. For both groups, verbal working memory contributed to comprehension performance in those reading conditions hypothesized to require more cognitive effort. Despite their sensitivity to the goals of reading, the group with SBM answered fewer comprehension questions correctly across all reading goal conditions. The results are discussed in relation to the hypothesized cognitive underpinnings of comprehension deficits in SBM and to current models of text comprehension. PMID:20338082

  11. Effects of reading goals on reading comprehension, reading rate, and allocation of working memory in children and adolescents with spina bifida meningomyelocele.

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    English, Lianne; Barnes, Marcia A; Fletcher, Jack M; Dennis, Maureen; Raghubar, Kimberly P

    2010-05-01

    Spina bifida meningomyelocele (SBM) is a neurodevelopmental disorder associated with intact word decoding and deficient text and discourse comprehension. This study investigated the ability to adjust reading in accordance with specified reading goals in 79 children and adolescents with SBM (9-19 years of age) and 39 controls (8-17 years of age). Both groups demonstrated slower reading times and enhanced comprehension when reading to study or to come up with a title than when reading for specific information or for entertainment. For both groups, verbal working memory contributed to comprehension performance in those reading conditions hypothesized to require more cognitive effort. Despite their sensitivity to the goals of reading, the group with SBM answered fewer comprehension questions correctly across all reading goal conditions. The results are discussed in relation to the hypothesized cognitive underpinnings of comprehension deficits in SBM and to current models of text comprehension.

  12. Biosynthetic cellulose induces the formation of a neoduramater following pre-natal correction of meningomyelocele in fetal sheep A celulose biossintética induz a formação de uma neoduramáter na correção antenatal da meningomielocele em fetos de ovelhas

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    Rita de Cássia Sanchez e Oliveira

    2007-06-01

    Full Text Available PURPOSE: The aim of this study was to compare the effectiveness of two dura-mater substitutes, namely human acellular dermal matrix (HADM and biosynthetic cellulose (BC, in repairing, in utero, surgically-induced meningomyelocele (MMC in fetal sheep. METHODS: A neural tube defect was created at 74-77 days gestation in 36 fetal sheep. They were divided into 3 groups, the control group that did not receive pre-natal corrective surgery, and the other two groups that received corrective surgery using HADM (Group A or BC (Group B. Both materials were used as a dura-mater substitutes between the neural tissue and the sutured skin. Correction was performed at gestation day 100 and the fetuses were maintained in utero until term. Sheep were sacrificed on gestation day 140. The fetal spine was submitted to macro and microscopic analysis. At microscopy, adherence of the material to the skin and neural tissue was analyzed. RESULTS: In the initial phase (pilot, experimentally-induced MMC was performed on 11 fetuses and 4 survived (37%. In the second phase (study, 25 fetuses received surgery and 17 survived (68%. In the study group, 6 fetuses did not undergo repair (control group, 11 cases were submitted to corrective surgery (experimental group and one fetal loss occurred. Of the surviving cases in the experimental group, 4 constituted Group A and 6 in Group B. Macroscopically, skin and underlying tissues where easily displaced from the BC in all cases it was used; in contrast, HADM adhered to these tissues. To compare the adherence, 4 cases from Group A and 4 in Group B were studied. We observed adherence, host cell migration and vessel proliferation into the HADM all sections from Group A and this aspect was not present in any cases in Group B (p OBJETIVO: Estudar os efeitos do emprego de dois materiais consideravelmente diferentes quanto à origem e custo na correção intra-uterina da meningomielocele criada experimentalmente em feto de ovino. M

  13. Uso da prótese vertical expansível de titânio para costela no tratamento da cifose congênita em portadores de mielomeningocele torácica Uso de la prótesis vertical expansible de titanio para costilla en el tratamiento de la cifosis congénita en portadores de mielomeningocele torácico Use of vertical expandable prosthetic of titanium for the rib for treating congenital kyphosis in thoracic meningomyelocele patients

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    Guilherme Rebechi Zuiani

    2009-09-01

    meningomyelocele patients using vertical expandable prosthetic of titanium for the rib (VEPTR. METHODS: a retrospective study of 19 thoracic meningomyelocele and congenital kyphosis patients that were subjected to the VEPTR treatment between October 2005 and October 2008, with radiographic evaluation and immediate post and pre-operative clinical practice. Also, the duration of surgical procedure, the need for blood transfusion and postoperative complications were assessed. RESULTS: the patients' average age was 70 months (from 32 to 130 months. The average follow-up from patients was 13.5 months (from 2 to 26 months. The average duration of the procedure was 117 minutes (variation between 70 and 195 minutes. All children reached trunk balance, 13 of whom had not showed it in the postoperative period. The average of pre-operation kyphosis was 115° (from 80° to 150° and 77° (from 50° to 104° for postoperative, with an average correction percentage of 31.2% (from 1.1 to 61.5%. The previous pre-operative imbalance of trunk was an average 7.9 cm (from 1.0 to 15.5 cm and 3.4 cm (from 0 to 8 cm for post-operative. The average correction of this imbalance was of 50.4% (from 0 to 100%. Regarding weight, in pre-operative the average was 15.4 kg (from 8 to 30 kg and 20.6 kg (from 8.5 to 35 kg for postoperative. The average gain of weight was of 36.6% (from 9.8 to 100%. Five of the 19 patients (26.3% presented postoperative complications. No patient needed blood transfusion. CONCLUSION: the use of VEPTR in thoracic meningomyelocele and congenital kyphosis patients has proven to be an effective and promising alternative for the control of physical deformity in patients with a potential for growth.

  14. Analysis of the distribution pattern of spinal meningoceles and ...

    African Journals Online (AJOL)

    Background: Spinal meningoceles and meningomyeloceles are part of a group of developmental abnormalities. They are usually midline and could be found throughout the length of the spinal column. Their preferential occurrence along the spine is not clear and their aetiological factors could be numerous. Aim: To ...

  15. Comparative Studies of Spinal Celes in Switzerland, Jamaica, and ...

    African Journals Online (AJOL)

    Clinical Methods and Materials: From 1975 to 2008,17 spinal celes (including 2 meningoceles) were routinely repaired in Imo and Ebonyi States of Nigeria, and 5 in Jamaica,the West Indies; none in Basel, Switzerland. All 20 meningomyeloceles were incontinent of urine and faeces, had severe paraparesis to paraplegia, ...

  16. Neonatal loss of motor function in human spina bifida aperta

    NARCIS (Netherlands)

    Sival, Deborah A; van Weerden, Tiemen W; Vles, Johan S H; Timmer, Albertus; den Dunnen, Wilfred F A; Staal-Schreinemachers, A L; Hoving, Eelco W.; Sollie, Krystyne M; Kranen-Mastenbroek, Vivianne J M; Sauer, Pieter J J; Brouwer, Oebele F

    OBJECTIVE: In neonates with spina bifida aperta (SBA), leg movements innervated by spinal segments located caudal to the meningomyelocele are transiently present. This study in neonates with SBA aimed to determine whether the presence of leg movements indicates functional integrity of neuronal

  17. Spinal hemorrhages are associated with early neonatal motor function loss in human spina bifida aperta

    NARCIS (Netherlands)

    Sival, Deborah; Verbeek, R. J.; Brouwer, O. F.; Sollie, K. M.; Bos, A. F.; den Dunnen, W. F. A.

    Background: In spina bifida aperta (SBA), leg movements caudal to the meningomyelocele are present in utero, but they disappear shortly after birth. It is unclear whether leg movements disappear by impact of the neuro-developmental malformation or by superimposed traumatic damage. If superimposed

  18. Cranial CT signs of the Chiari II malformation

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    Naidich, T.P.; Pudlowski, R.M.

    1980-01-01

    Serial CT scans of 32 patients with proved Chiari II malformations and 19 patients with hydrocephalus and meningomyelocele (presumed to have Chiari II malformation) were reviewed and compared with CT scans from 30 patients with non-chiari aqueductal stenosis to develop criteria for identifying the Chiari II malformation and for differentiating it from other forms of hydrocephalus. Correlation with post-mortem specimens of Chiari II brains provided a pathologic basis for the CT signs observed. (orig.) [de

  19. Imaging of the lumbar spine after diskectomy; Imagerie du rachis lombaire apres discectomie

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    Laredo, J.D.; Wybier, M. [Hopital Lariboisiere, 75 - Paris (France)

    1995-12-31

    The radiological investigation of persistent or recurrent sciatica after lumbar diskectomy essentially consists of demonstrating recurrent disk herniation. Comparison between plain and contrast enhanced CT or MR examinations at the level of the diskectomy is the main step of the radiological survey. The meanings of the various radiological findings are discussed. Other lesions that may induce persistent sciatica after lumbar diskectomy include degenerative narrowing of the lateral recess, spinal instability, stress fracture of the remaining neural arch, pseudo-meningomyelocele after laminectomy. (authors). 34 refs., 5 tabs.

  20. Imaging of the lumbar spine after diskectomy

    International Nuclear Information System (INIS)

    Laredo, J.D.; Wybier, M.

    1995-01-01

    The radiological investigation of persistent or recurrent sciatica after lumbar diskectomy essentially consists of demonstrating recurrent disk herniation. Comparison between plain and contrast enhanced CT or MR examinations at the level of the diskectomy is the main step of the radiological survey. The meanings of the various radiological findings are discussed. Other lesions that may induce persistent sciatica after lumbar diskectomy include degenerative narrowing of the lateral recess, spinal instability, stress fracture of the remaining neural arch, pseudo-meningomyelocele after laminectomy. (authors). 34 refs., 5 tabs

  1. Transverse sinus thrombosis associated with otitis media and mastoiditis

    International Nuclear Information System (INIS)

    Santos, V.M.; Figueiredo, N.C.; Santos, F.H.B.

    2012-01-01

    A case of transverse sinus thrombosis is described in an adolescent male with antecedent meningomyelocele corrected by surgery, and ventriclo-peritoneal derivation. Four months before the occurrence of thrombosis, he presented with bilateral otitis media and mastoiditis, and was treated with antibiotic. Magnetic resonance images were not obtained before referral to our hospital. The patient received full anticoagulation and his clinical course was uneventful. He remains asymptomatic under outpatient surveillance till this report. The aim of reporting the case is to emphasize the role of otologic infections in the origin of intracranial thrombotic phenomena, and highlights the findings of magnetic resonance venography for characterization of intracranial sinus thrombosis. (author)

  2. Congenital spinal malformations

    International Nuclear Information System (INIS)

    Ertl-Wagner, B.B.; Reiser, M.F.

    2001-01-01

    Congenital spinal malformations form a complex and heterogeneous group of disorders whose pathogenesis is best explained embryologically. Radiologically, it is important to formulate a diagnosis when the disorder first becomes symptomatic. However, it is also crucial to detect complications of the disorder or of the respective therapeutic interventions in the further course of the disease such as hydromyelia or re-tethering after repair of a meningomyelocele. Moreover, once a congenital spinal malformation is diagnosed, associated malformations should be sought after. A possible syndromal classification such as in OEIS- or VACTERL-syndromes should also be considered. (orig.) [de

  3. Retrospective analysis of the use of amniotic membranes and xenografts in spinal surgery and anterior cranial fossa operations

    International Nuclear Information System (INIS)

    Jafri Malim Abdullah

    1999-01-01

    To determine the suitability of amniotic membrane an bovine bone xenografts for the use in spinal surgery and anterior cranial for a generations. Fifteen patients with anterior cranial fossa defects and spinal bone fractures received bovine bone xenografts and 10 patients with meningomyeloceles received amniotic membranes (produced by the Malaysian National Tissue Bank) were analysed retrospectively. Clinical criterias like fever, signs of inflammation, breakdown of graft implant, non specific reaction to the nervous tissue were analysed haematological and radiologically. All patients who received the bovine grafts and amniotic membranes did not show any evidence of inflammation or fever. There were no graft implant breakdowns. There was no radiological or clinical evidence of specific or non specific reaction to the nervous tissue after 12-36 months followup Amniotic membranes and bovine xenografts may be used in the healing and reconstruction of spinal and cranial defects. Despite no evidence of rejection and infection after 36 months, a long term followup is still needed

  4. Sirenomelia with associated systemic anomalies: an autopsy pathologic illustration of a series of four cases.

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    Chikkannaiah, Panduranga; Mahadevan, Anita; Gosavi, Manasi; Kangle, Ranjit; Anuradha; Shankar, S K

    2014-07-01

    Sirenomelia, a developmental defect involving the caudal region of the body, is associated with several internal visceral anomalies. We report a detailed spectrum of anomalies in an autopsy study of four fetuses with sirenomelia (gestational ages - 20, 21, 22.4, and 22.5 weeks). Three of the fetuses had single umbilical artery, with genitourinary and gastrointestinal anomalies. Central nervous system anomalies were evident in two of the fetuses, with alobar holoprosencephaly in one and lumbar meningomyelocele in another. The most common gastrointestinal anomaly was blind ended gut (imperforate anus), while esophageal atresia and omphalocele were noted in one case each. Renal hypoplasia was seen in two fetuses, renal agenesis in one and cystic renal dysplasia was noted in one case. Literature regarding pathogenesis of this condition is briefly discussed. Copyright © 2014 Elsevier GmbH. All rights reserved.

  5. Neurogenic Bladder

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    Peter T. Dorsher

    2012-01-01

    Full Text Available Congenital anomalies such as meningomyelocele and diseases/damage of the central, peripheral, or autonomic nervous systems may produce neurogenic bladder dysfunction, which untreated can result in progressive renal damage, adverse physical effects including decubiti and urinary tract infections, and psychological and social sequelae related to urinary incontinence. A comprehensive bladder-retraining program that incorporates appropriate education, training, medication, and surgical interventions can mitigate the adverse consequences of neurogenic bladder dysfunction and improve both quantity and quality of life. The goals of bladder retraining for neurogenic bladder dysfunction are prevention of urinary incontinence, urinary tract infections, detrusor overdistension, and progressive upper urinary tract damage due to chronic, excessive detrusor pressures. Understanding the physiology and pathophysiology of micturition is essential to select appropriate pharmacologic and surgical interventions to achieve these goals. Future perspectives on potential pharmacological, surgical, and regenerative medicine options for treating neurogenic bladder dysfunction are also presented.

  6. Neural Tube Defects and Pregnancy

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    Emine Çoşar

    2009-09-01

    Full Text Available OBJECTIVE: Neural tube defects are congenital malformations those mostly causing life-long morbidities. They are prevented by the periconseptional folic acid usage and prenatal diagnostic methods. MATERIALS-METHODS: Pregnants from Afyonkarahisar and neighbourhood cities applied to our hospital and determined NTD, were investigated. RESULTS: In our obstetrics clinic 1403 delivery were made and 43 of them had fetus with NTD. Among these fetuses 41.3% had meningomyelocel, 17.4% had meningocel, 21.7% had encephalocel, 8.7% had unencephali and 4.3% had iniencephali. CONCLUSION: Incidence of NTD is high in our region and geographic region, nutrition and other socioeconomic factors may be related to the high incidence. Education of the mother and periconceptional folic acid usage may reduce teh incidence of NTD.

  7. Vesicoureteric reflux in children

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    Jameela A Kari

    2013-01-01

    Full Text Available Aim: This study aimed to identify the differences between primary and secondary vesicoureteric reflux (VUR and the effect of associated bladder abnormalities on kidney function. Patients and Methods: We retrospectively reviewed the medical records of children with VUR who were followed up at King Abdulaziz University Hospital from January 2005 to December 2010. The review included results of radiological investigations and kidney function tests. We used Chi-square test for statistical analysis and paired t-test to compare group means for initial and last creatinine levels. Results: Ninety-nine children were included in this study. Twenty (20.2% had primary VUR, 11 had high-grade VUR, while 9 had low-grade reflux. All children with low-grade VUR had normal dimercaptosuccinic acid (DMSA. Renal scars were present in 72% of the children with high-grade VUR. The mean creatinine levels (initial and last for both groups were normal. Seventy-nine (79.8% children had secondary VUR, which was due to posterior urethral valves (PUV (46.8%, neurogenic bladder caused by meningomyelocele (25.3%, non-neurogenic neurogenic bladder (NNB (21.5%, or neurogenic bladder associated with prune belly syndrome (6.3%. Children with NNB, meningomyelocele and PUV had high creatinine at presentation with no considerable worsening of their kidney functions during the last visit. Renal scars were present in 49.4% of the children with secondary VUR. Conclusion: Children with primary VUR and normal bladder had good-functioning kidneys, while those with secondary VUR associated with abnormal bladder caused by NNB, spina bifida or PUV had abnormal kidney functions. DMSA scans were useful in predicting higher grades of VUR in children with primary reflux.

  8. Desempenho funcional de crianças com mielomeningocele Functional performance of children with myelomeningocele

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    Luanda André Collange

    2008-01-01

    Full Text Available Este trabalho visou analisar o impacto dos níveis de lesão, deambulação e alterações neurológicas associadas ao quadro clínico da mielomeningocele sobre o desempenho funcional de seus portadores. Neste estudo transversal prospectivo foram examinadas 40 crianças com mielomeningocele, avaliando-se nível de lesão, deambulação, malformação de Arnold-Chiari, hidrocefalia, hidromielia, medula ancorada e incontinência esfincteriana. O desempenho funcional foi avaliado pelo Pediatric Evaluation of Disability Inventory (PEDI, para um registro quantitativo da capacidade funcional e autonomia nas atividades cotidianas, nas áreas de autocuidado, mobilidade e função social. Os dados coletados foram analisados estatisticamente, estabelecendo-se o nível de significância em pThis study aimed at analysing the impact of level of lesion, locomotion, and neurological changes associated to meningomyelocele clinical framework concerning functional performance. For this prospective cross-sectional study 40 children with meningomyelocele were assessed as to level of lesion, ambulatory ability, Arnold-Chiari malformation, hydrocephalus, hydromyelia, tethered cord, and sphincter incontinence. Functional performance was evaluated by the Pediatric Evaluation of Disability Inventory (PEDI which quantitatively assesses functional performance and independence in daily activities in the areas of self-care, mobility, and social functioning. Collected data were statistically analysed and the significance level set at p<0.05. Lesion levels and ambulatory ability had a significant (p<0.01 impact on scores of all three PEDI areas. Limitation degree was found to be directly proportional to level of lesion. Functional performance was more severely affected in self-care and mobility activities, and the best functional scores being obtained in social functioning. In patients with thoracic lesion level, symptomatic hydromyelia proved to be a significant factor for

  9. Asymmetry and discordance for congenital anomalies in conjoined twins: a report of six cases.

    Science.gov (United States)

    Ornoy, A; Navot, D; Menashi, M; Laufer, N; Chemke, J

    1980-10-01

    Six pairs of conjoined twins have been studied. The first case was a pair of 13-week-old omphalopagus fetuses. One was a holoacradius amorphus and the other had rachischisis and anencephaly. The second case was a pair of omphalopagus twins. One of the twins was macerated and corresponded to a developmental age of 13-14 weeks, while the other was developed to 28-30 weeks of gestation and exhibited urogenital and gastrointestinal defects not found in the smaller twin. In the third case, that of a thoracoomphalopagus, one had cleft lip and palate, pulmonic stenosis, and atresia of the ileocecal valve, while the other did not show these anomalies. In the fourth cae, also omphalopagus twins, one had a lumbosacral meningomyelocele and severe gastrointestinal and urogenital anomalies not found in the second twin. The fifth case was a pair of thoracoomphalopagus twins, sharing a common heart with asymmetrical anomalies. The sixth case was a diprosopus anencephalic conjoined twin. The first pairs of conjoined twins were discordant for several abnormalities in nonshared organs, in addition to having abnormalities of the conjoined organs. It seems that discordance in conjoined twins is not a rare finding. The factors that play a role in discordance of anomalies in conjoined twins are probably similar to the factors in monozygotic twins--i.e., environmental, genetic, and abnormal placental and/or fetal circulation.

  10. The school inclusion and use of assistive technology with myelomeningocele sequelae students: a view of the teachers

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    Caroline Penteado Assis

    2011-06-01

    Full Text Available Myelomeningocele is a congenital malformation that affects the fetus during pregnancy. It can causes numerous consequences for the development of the child and consequently in their schooling process. So, there are several challenges that can be experienced by these children in their inclusion at school. The use of assistive technology in the school context has been implanted with goal to high from the functional abilities of these students. In Brazil are conducted a lot of studies to know the reality of children with myelomeningocele. Therefore, the goal of this study was to investigate, from the teacher ́s perspective of regular and multifunctional class, as was the process of schooling for students with myelomeningocele sequelae and resource utilization of assistive technology in the school context. The participants of this research were seven teachers and four students with myelomeningocele sequels enclosed in regular scholl.The researcher confectioned the instruments used in interviews. Data were analyzed using content of interview analysis. The results reveal that there are present challenges for inclusion of students with myelomeningocele sequelae and that teachers did not use assistive technology resources at school. It is considered that for the real practice of educational inclusion of these students the knowledge about meningomyelocele and various technologies that can be used in the school context must be published.

  11. Validation of the Physical Activity Scale for individuals with physical disabilities.

    Science.gov (United States)

    van den Berg-Emons, Rita J; L'Ortye, Annemiek A; Buffart, Laurien M; Nieuwenhuijsen, Channah; Nooijen, Carla F; Bergen, Michael P; Stam, Henk J; Bussmann, Johannes B

    2011-06-01

    To determine the criterion validity of the Physical Activity Scale for Individuals With Physical Disabilities (PASIPD) by means of daily physical activity levels measured by using a validated accelerometry-based activity monitor in a large group of persons with a physical disability. Cross-sectional. Participants' home environment. Ambulatory and nonambulatory persons with cerebral palsy, meningomyelocele, or spinal cord injury (N=124). Not applicable. Self-reported physical activity level measured by using the PASIPD, a 2-day recall questionnaire, was correlated to objectively measured physical activity level measured by using a validated accelerometry-based activity monitor. Significant Spearman correlation coefficients between the PASIPD and activity monitor outcome measures ranged from .22 to .37. The PASIPD overestimated the duration of physical activity measured by using the activity monitor (mean ± SD, 3.9±2.9 vs 1.5±0.9h/d; PPASIPD correlated poorly with objective measurements using an accelerometry-based activity monitor in people with a physical disability. However, similar low correlations between objective and subjective activity measurements have been found in the general population. Users of the PASIPD should be cautious about overestimating physical activity levels. Copyright © 2011 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

  12. Gross congenital malformation at birth in a government hospital.

    Science.gov (United States)

    Sachdeva, Sandeep; Nanda, Smiti; Bhalla, Kapil; Sachdeva, Ruchi

    2014-01-01

    A hospital-based cross-sectional study was undertaken to determine proportion of gross congenital malformation (GCMF) occurring at intramural births. Rate of GCMF was found to be 16.4/1000 consecutive singleton births (>28 weeks) with three leading malformation as anencephaly (44.68%), talipes equinovarus (17.02%) and meningomyelocele (10.63%). Higher risk of malformed births were noticed amongst un-booked (2.07%) in-comparison to booked (1.01%) mothers; women with low level of education (up to 8 years [2.14%] vs. at least 9 years of schooling [0.82%]); gravida status of at least 3 (2.69%) followed by 1 (1.43%) and 2 (1.0%) respectively; pre-term (5.13%) vs. term (0.66%); cesarean section (4.36%) versus vaginal delivery (0.62%). Mortality was significantly higher among congenitally malformed (17.35%) than normal (0.34%) newborns. With-in study limitation, emergence of neural tube defect as the single largest category of congenital malformation indicates maternal malnutrition (especially folic acid) that needs appropriate attention and management.

  13. Congenital spinal malformations; Kongenitale spinale Malformationen

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    Ertl-Wagner, B.B.; Reiser, M.F. [Klinikum Grosshadern, Ludwig-Maximilians-Univ. Muenchen (Germany). Inst. fuer Klinische Radiologie

    2001-12-01

    Congenital spinal malformations form a complex and heterogeneous group of disorders whose pathogenesis is best explained embryologically. Radiologically, it is important to formulate a diagnosis when the disorder first becomes symptomatic. However, it is also crucial to detect complications of the disorder or of the respective therapeutic interventions in the further course of the disease such as hydromyelia or re-tethering after repair of a meningomyelocele. Moreover, once a congenital spinal malformation is diagnosed, associated malformations should be sought after. A possible syndromal classification such as in OEIS- or VACTERL-syndromes should also be considered. (orig.) [German] Kongenitale spinale Malformationen stellen eine komplexe Gruppe an Stoerungen dar, deren Genese sich am einfachsten aus der Embryologie heraus erklaeren laesst. Bei der klinisch-radiologischen Begutachtung ist zunaechst ihre korrekte Klassifikation im Rahmen der Erstdiagnose wichtig. Im weiteren Verlauf ist es jedoch zudem entscheidend, moegliche Komplikationen wie beispielsweise eine Hydromyelie oder ein Wiederanheften des Myelons nach Operation einer Spina bifida aperta zu erkennen. Zudem sollte bei der Diagnosestellung einer kongenitalen spinalen Malformation immer auch auf assoziierte Fehlbildungen, wie z.B. die Diastematomyelie oder das intraspinale Lipom bei der Spina bifida aperta, sowie auf eine moegliche syndromale Einordnung wie beispielsweise beim OEIS-oder VACTERL-Syndrom geachtet werden. (orig.)

  14. The importance of patient-reported outcome measures in reconstructive urology.

    Science.gov (United States)

    Jackson, Matthew J; N'Dow, James; Pickard, Rob

    2010-11-01

    Patient-reported outcome measures (PROMs) are now recognised as the most appropriate instruments to assess the effectiveness of healthcare interventions from the patient's perspective. The purpose of this review was to identify recent publications describing the use of PROMs following reconstructive urological surgery. A wide systematic search identified only three original articles published in the last 2 years that prospectively assessed effectiveness using a patient-completed condition-specific or generic health-related quality of life (HRQoL) instrument. These publications illustrate the need to administer PROMs at a postoperative interval relevant to the anticipated recovery phase of individual procedures. They also highlight the difference in responsiveness of generic HRQoL instruments to symptomatic improvement between straightforward conditions such as pelviureteric junction obstruction and complex multidimensional conditions such as meningomyelocele. PROMs uptake and awareness is increasing in reconstructive urology but more work is required to demonstrate the effectiveness of surgical procedures for patients and healthcare funders alike. Healthcare policy-makers now rely on these measures to determine whether specific treatments are worth financing and to compare outcomes between institutions.

  15. Use of the Palmaz stent in the treatment of severe tracheomalacia.

    Science.gov (United States)

    Geller, Kenneth A; Wells, Winfield J; Koempel, Jeffrey A; St John, Maie A R

    2004-08-01

    To present our experience with the use of the Palmaz stent in treating cases of severe, life-threatening tracheomalacia, and to report our experience with the use of tracheal stents in patients who have concomitant tracheotomies, we performed a retrospective study in a tertiary-care children's hospital. Nine patients with multiple congenital anomalies including severe tracheomalacia required placement of a Palmaz stent to prolong life. The congenital anomalies included congenital heart disease, congenital lung disease, meningomyelocele, laryngotracheoesophageal cleft, and tracheoesophageal fistula. Three of the patients had concomitant tracheotomies. Each patient had placement of one or more Palmaz stents in the trachea and/or bronchus. Four patients died, and 5 patients are still alive. Three of the 4 patients who died had concomitant tracheotomies and died of complications associated with significant tracheal hemorrhage. The fourth died of pulmonary complications following repeated episodes of pneumonia. None of the 5 patients who are still alive had a concomitant tracheotomy. The Palmaz stent is a useful tool for treating life-threatening tracheomalacia as a final resort in this difficult patient population; however, the use of these stents may lead to subsequent hemorrhage and death, especially in patients with tracheotomies, so their use must be carefully considered.

  16. A case of prenatal diagnosis of fetal hydantoin syndrome by ultrasound

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    Thomaz Rafael Gollop

    1999-06-01

    Full Text Available Fetal hydantoin syndrome (FHS is a set of disruptions occasionally present in fetuses exposed in utero to phenytoin or other anticonvulsants. Administration of phenytoin in early pregnancy may impair proper psychomotor performance expected for children's development. Several combined phenotypic markers delineate the syndrome, but the presence of single clinical signs is more common. There is controversy about the etiology of FHS. Associated disruptions may be related to a deficiency in a detoxifying enzyme (epoxide hydrolase, vascular problems, and/or factors not yet known. Genetic causes are believed to influence susceptibility to the drug. This text reports an unusual pattern of malformations detected in an ultrasound scan (gastroschisis, sacral meningomyelocele, and absence of the right lower limb and in the anatomopathological study (left-side gastroschisis, sacral meningomyelocele, scoliosis, left clubfoot, absence of the right lower limb, and pectus carinatum of a fetus whose mother took phenytoin. These defects may have been provoked by exposure to the drug during embryogenesis. In view of similar malformations observed in cases of prenatal exposure to cocaine, a recognized vasoconstrictor, it is suggested that vascular disruptions of hemodynamic origin constituted the event leading to some of the anomalies caused in the developing embryo. A complication of the chorionic villus sampling procedure, used for cytogenetic analysis, is another possibility.A síndrome da hidantoína fetal consiste em um conjunto de disrupturas por vezes observadas em fetos expostos à fenitoína ou outros anticonvulsivos no período pré-natal. A administração de fenitoína em fase precoce da gravidez pode prejudicar o desempenho psicomotor esperado no desenvolvimento infantil. Diversos indicadores fenotípicos, em conjunto, caracterizam a síndrome, mas a presença de sinais clínicos isolados é mais comum. Há controvérsia quanto à sua etiologia. As

  17. Disostose espôndilo-costal associada a defeitos de fechamento do tubo neural Spondylocostal dysostosis associated with neural tube defects

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    Rafael Fabiano M. Rosa

    2009-09-01

    Full Text Available OBJETIVO: Salientar a relação dos defeitos de fechamento do tubo neural com a disostose espôndilo-costal (DEC por meio da descrição de três pacientes. DESCRIÇÃO DOS CASOS: Paciente 1: menina branca, 22 meses, nascida com mielomeningocele lombar. Na avaliação, apresentava hipotonia, baixa estatura, dolicocefalia, fendas palpebrais oblíquas para cima, pregas epicânticas e tronco curto com tórax assimétrico. A avaliação radiográfica revelou hemivértebras múltiplas, vértebras em borboleta e fusão e ausência de algumas costelas. Paciente 2: menina branca, 22 meses, com moderado atraso do desenvolvimento neuropsicomotor, baixa estatura, olhos profundos, pregas epicânticas, pescoço e tronco curtos com assimetria do tórax, abdome protruso, hemangioma plano na altura da transição lombossacra e fosseta sacral profunda no dorso. A avaliação radiográfica identificou hemivértebras, fusão incompleta de vértebras e vértebras em borboleta, malformações de costelas e espinha bífida oculta em L5/S1. Paciente 3: menina branca, 9 dias de vida, com fendas palpebrais oblíquas para cima, ponte nasal alargada, orelhas baixo implantadas e rotadas posteriormente, tronco curto, tórax assimétrico e meningocele tóraco-lombar. A avaliação radiográfica evidenciou hemivértebras, malformação e ausência de algumas costelas e agenesia diafragmática à esquerda. A tomografia computadorizada de encéfalo mostrou estenose de aqueduto. COMENTÁRIOS: Vários defeitos de fechamento do tubo neural, de espinha bífida oculta a grandes mielomeningoceles, são observados em pacientes com DEC, indicando que tais pacientes devem ser cuidadosamente avaliados quanto à possível presença desses defeitos.OBJECTIVE: To highlight the relationship between neural tube defects and spondylocostal dysostosis (SCD through the description of three patients. CASES DESCRIPTION: Patient 1: white girl, 22 months old, born with a lumbar meningomyelocele. At

  18. The value of fast MR imaging as an adjunct to ultrasound in prenatal diagnosis

    International Nuclear Information System (INIS)

    Breysem, L.; Bosmans, H.; Dymarkowski, S.; Demaerel, P.; Vanbeckevoort, D.; Smet, M.; Schoubroeck, D.Van; Witters, I.; Deprest, J.; Vanhole, C.; Casaer, P.

    2003-01-01

    The aim of this study was to evaluate the role of MR imaging of the fetus to improve sonographic prenatal diagnosis of congenital anomalies. In 40 fetuses (not consecutive cases) with an abnormality diagnosed with ultrasound, additional MR imaging was performed. The basic sequence was a T2-weighted single-shot half Fourier (HASTE) technique. Head, neck, spinal, thoracic, urogenital, and abdominal fetal pathologies were found. This retrospective, observational study compared MR imaging findings with ultrasonographic findings regarding detection, topography, and etiology of the pathology. The MR findings were evaluated as superior, equal to, or inferior compared with US, in consent with the referring gynecologists. The role of these findings in relation to pregnancy management was studied and compared with postnatal follow-up in 30 of 40 babies. Fetal MRI technique was successful in 36 of 39 examinations and provided additional information in 21 of 40 fetuses (one twin pregnancy with two members to evaluate). More precise anatomy and location of fetal pathology (20 of 40 cases) and additional etiologic information (8 of 40 cases) were substantial advantages in cerebrospinal abnormalities [ventriculomegaly, encephalocele, vein of Galen malformation, callosal malformations, meningo(myelo)cele], in retroperitoneal abnormalities (lymphangioma, renal agenesis, multicystic renal dysplasia), and in neck/thoracic pathology [cervical cystic teratoma, congenital hernia diaphragmatica, congenital cystic adenomatoid lung malformation (CCAM)]. This improved parental counseling and pregnancy management in 15 pregnancies. In 3 cases, prenatal MRI findings did not correlate with prenatal ultrasonographic findings or neonatal diagnosis. The MRI provided a more detailed description and insight into fetal anatomy, pathology, and etiology in the vast majority of these selected cases. This improved prenatal parental counseling and postnatal therapeutic planning. (orig.)

  19. The value of fast MR imaging as an adjunct to ultrasound in prenatal diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Breysem, L.; Bosmans, H.; Dymarkowski, S.; Demaerel, P.; Vanbeckevoort, D.; Smet, M. [Department of Radiology, University Hospitals, Herestraat 49, 3000, Leuven (Belgium); Schoubroeck, D.Van; Witters, I.; Deprest, J. [Department of Obstetrics and Gynecology, University Hospitals, Herestraat 49, 3000, Leuven (Belgium); Vanhole, C.; Casaer, P. [Department of Pediatrics, University Hospitals, Herestraat 49, 3000, Leuven (Belgium)

    2003-07-01

    The aim of this study was to evaluate the role of MR imaging of the fetus to improve sonographic prenatal diagnosis of congenital anomalies. In 40 fetuses (not consecutive cases) with an abnormality diagnosed with ultrasound, additional MR imaging was performed. The basic sequence was a T2-weighted single-shot half Fourier (HASTE) technique. Head, neck, spinal, thoracic, urogenital, and abdominal fetal pathologies were found. This retrospective, observational study compared MR imaging findings with ultrasonographic findings regarding detection, topography, and etiology of the pathology. The MR findings were evaluated as superior, equal to, or inferior compared with US, in consent with the referring gynecologists. The role of these findings in relation to pregnancy management was studied and compared with postnatal follow-up in 30 of 40 babies. Fetal MRI technique was successful in 36 of 39 examinations and provided additional information in 21 of 40 fetuses (one twin pregnancy with two members to evaluate). More precise anatomy and location of fetal pathology (20 of 40 cases) and additional etiologic information (8 of 40 cases) were substantial advantages in cerebrospinal abnormalities [ventriculomegaly, encephalocele, vein of Galen malformation, callosal malformations, meningo(myelo)cele], in retroperitoneal abnormalities (lymphangioma, renal agenesis, multicystic renal dysplasia), and in neck/thoracic pathology [cervical cystic teratoma, congenital hernia diaphragmatica, congenital cystic adenomatoid lung malformation (CCAM)]. This improved parental counseling and pregnancy management in 15 pregnancies. In 3 cases, prenatal MRI findings did not correlate with prenatal ultrasonographic findings or neonatal diagnosis. The MRI provided a more detailed description and insight into fetal anatomy, pathology, and etiology in the vast majority of these selected cases. This improved prenatal parental counseling and postnatal therapeutic planning. (orig.)

  20. 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

  1. Neural Tube Defect in Alive Neonates: Incidence Rate and Predisposing Factors

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    F Haghollahi

    2008-06-01

    Full Text Available Background: Neural Tube Defect (NTD characterized by failure of neural tube to close properly be the second most common born defect after congenital heart disease. The most prevalent forms of NTD are Anencephaly and Spinal-bifida. Many factors are involved in this anomaly. New researches suggest environmental factors like radiation, hyperthermia, Vitamin A and acid folic deficiency, anti epileptic drug like Carbamazepine, Phenobarbital, phenytoin, Folic acid antagonist like Sulfasalazine, Triametherine and systemic disease like diabet mellitus, obesity, genetic factors, the most schance 40 to 70 percentages.Methods: In this survey cross sectional study was conducted in five hospitals depend to Tehran university during three years. Study subject identified through review of admission and discharge at major hospital through regular contact with newborn nurseries and birth hospital.Results: In 38473 reported cases, 143 cases have neural tube defect. Among NTD cases, 11.9% of mothers had medical diseases in their previous history such as diabetes mellitus, epilepsy-psychiatric, and disorder-heart diseases. In this study group, 5.6% have preclampsia during pregnancy period. The most common NTD anomaly in this study was anencephaly and meningomyelocele that was different from studies in literature.Conclusion: NTD result from failure of neural tube close threats fetus health up to 28 days after conception. When is often prior to the recognition of pregnancy since many pregnancy are unplanned NTD prevention is best achieve by adequate daily folic acid intake thought of reproductive ages .educational effort to promote daily intake of folic acid supplemental by women of reproductive age and NTD risk factor should be done. Early diagnostic procedure for high risk pregnancy advised.

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

    Science.gov (United States)

    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.

  3. Malformation/dysplasia syndrome (neural tube defect, hypospadias neuroblastoma) associated with an extra dicentric marker chromosome 15 ({open_quotes}inversion duplication 15{close_quotes})

    Energy Technology Data Exchange (ETDEWEB)

    Reitnauer, P.J.; Rao, K.W.; Tepperberg, J.H. [Univ. of North Carolina, Chapel Hill, NC (United States)

    1994-09-01

    Extra dicentric 15 marker chromosomes are associated with variable degrees of mental retardation but not major structural birth defects. We have studied a unique patient, a male infant who was prenatally diagnosed with lumbar meningomyelocele and an extra pseudodicentric marker chromosome: 47,XY,+psu dic(15)t(15;15)(?q12,?q12)mat. Hairy ears and a coronal hypospadias were noted at birth. At three months of age, a stage I thoracic neuroblastoma was primarily resected. Tumor cells, skin fibroblasts and peripheral blood lymphocytes contained the dicentric 15. The mother is mosaic for the marker chromosome. Fluorescence in situ hybridization (FISH) studies using a classic 15 satellite probe (D15Z1 [Oncor]) confirmed the presence of 2 number 15 centromeres in the marker. The marker is felt to be the result of a translocation rather than an inverted duplication because the G-band morphology of the short arm/satellite complexes differ from one another, implying that the arms originate from 2 different number 15s. FISH analysis using cosmid probes for the Prader-Willi/Angelman critical region (D15S11 and GABRB3 [Oncor]) revealed 2 copies of this region, indicating that these loci are duplicated in the marker. Although some features of the patient`s phenotype such as developmental delay and hypotonia have been associated with dicentric chromosome 15 markers, this is the first malformation/dysplasia syndrome or neuroblastoma reported to our knowledge. The association of neuroblastoma with chromosome 15 aberrations in this case provides speculation as to the role of chromosome 15 loci in cell division control.

  4. Desempenho psicolinguístico e escolar de irmãos com mielomeningocele Psycholinguistic and school performance of brothers with myelomeningocele

    Directory of Open Access Journals (Sweden)

    Dionísia Aparecida Cusin Lamônica

    2012-08-01

    Full Text Available TEMA: mielomeningocele e consequências para o desenvolvimento de habilidades psicolinguísticas e escolares. PROCEDIMENTOS: o objetivo deste estudo foi descrever e refletir sobre o desempenho psicolinguístico e escolar de irmãos com mielomeningocele lombar baixa. Participaram do estudo um menino de 14 anos e 5 meses (P1 e sua irmã de 6 anos e 6 meses (P2. Os procedimentos constaram de entrevista com familiares, Observação do Comportamento Comunicativo (OCC, Teste de Vocabulário por Imagens Peabody - TVIP, Teste Illinóis de Habilidades Psicolinguísticas (ITPA, Perfil de Habilidades Fonológicas (PHF, Teste de Desempenho Escolar (TDE, e Reconhecimento de letras e números (RLN. A análise dos resultados foi descritiva respeitando os manuais dos instrumentos. RESULTADOS: a linguagem oral encontra-se sem alteração evidente. No ITPA e PHF, verificou-se que P1 e P2 apresentaram dificuldade nas habilidades de memória, closura auditiva, expressão verbal, combinação de sons e consciência fonológica. P1 ainda apresentou dificuldade na habilidade de recepção visual e P2 nas habilidades de associação auditiva e closura gramatical. Ambas as crianças apresentaram alteração nas atividades de aprendizagem. CONCLUSÃO: os participantes do estudo apresentaram comportamento comunicativo, habilidades de linguagem oral e vocabulário receptivo sem alterações evidentes. P1 e P2 apresentaram alterações nas habilidades psicolinguísticas bem como no desempenho de atividades envolvendo leitura, escrita e aritmética. As dificuldades apresentadas causam impacto importante nas atividades acadêmicas, demonstrando vulnerabilidade cerebral no sistema de suporte para atividade de aprendizagem.BACKGROUND: meningomyelocele and consequences on the development of psycholinguistic and school abilities. PROCEDURES: the purpose of this study was to describe and reflect on the psycholinguistic and school performance of siblings with low lumbar

  5. Abordagem assistencial ao neonato portador de mielomeningocele segundo o modelo de adaptação de Roy Abordaje asistencial al neonato portador de mielomeningocele según el modelo de adaptación de Roy Care delivery to newborns with myelomeningocele according to Roy's adaptation model

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    Eloah de Paula Pessoa Gurgel

    2010-09-01

    través de estímulos, y que las metas e intervenciones implementadas fueron importantes en la sustitución de respuestas ineficaces por adaptativas.This case study aimed to identify the Nursing diagnoses, addressing physiological aspects according to Roy's Adaptation Theory, of a baby with meningomyelocele hospitalized at a Neonatal Intensive Care Unit (NICU, and to elaborate pertinent nursing actions for these aspects. The study was developed at the NICU of the Maternity Teaching Hospital Assis Chateaubriand, located in Fortaleza-Ceará, Brazil, between December 2007 and March 2008, when a script was used to survey data. During the analysis, the following diagnoses were recognized: ineffective breathing pattern; imbalanced nutrition: less than body requirements; impaired skin integrity; risk for infection and disturbed sensory perception. The use of this theory permitted recognizing that the newborn can produce positive or negative responses to stimuli and that the implemented targets and interventions were important to replace ineffective by adaptive responses.

  6. Impacto das malformações congênitas na mortalidade perinatal e neonatal em uma maternidade-escola do Recife Impact of congenital malformations on perinatal and neonatal mortality in an university maternity hospital in Recife

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    Melania Maria Ramos de Amorim

    2006-05-01

    , perinatal mortality, early and late neonatal mortality coefficients were calculated. RESULTS: malformation incidence was of 2.8% (in 4.043 births. Malformation percentages among live births was of 2.7% and among stillbirths of 6.7%. The most frequent malformations involved the central nervous system (principally hydrocephaly and meningomyelocele, the skeletal and muscular system and cardiopathies. There was no association between malformation and gender, but prematurity and low birthweight were more frequent among the malformation cases. It was determined that among malformed infants early neonatal mortality was of 32.7% and late neonatal mortality was of 10.6%. Malformation cases were 6.7% of stillborn babies, 24.2% of early neonatal deaths and 25.8% the total of neonatal deaths. CONCLUSIONS: malformation incidence corresponded to 2.8% of the births. Malformation was the second more frequent neonatal death cause following prematurity.