1/28. meningomyelocele and progressive hydromyelia. Progressive paresis in myelodysplasia.Five cases of myelodysplasia with progressive paraparesis are presented. Three of the five patients developed spasticity, but dissociated sensory loss and loss of sphincter control was not a prominent feature. All were found to have compensated hydrocephalus and extensive communicating hydromyelia. The use of myelography and ventriculography in the diagnosis of hydromyelia is discussed. Ventricular drainage led to clinical improvement in two cases and radiological improvement in one. The relationship of compensated hydrocephalus, meningomyelocele, and progressive hydromyelia postnatally may support the hydrodynamic hypothesis of myelodysplasia.- - - - - - - - - - ranking = 1keywords = meningomyelocele (Clic here for more details about this article) |
2/28. Diastematomyelia associated with ectopic dysplastic renal tissue--report of a rare case.BACKGROUND: Presence of heterotopic dysplastic renal tissue in the lumbosacral region is an extremely uncommon condition. CASE REPORT: We report the first case of diastematomyelia associated with ectopic renal tissue. A 10-month-old male child presented with lipomeningomyelocele associated with spina bifida in the lumbosacral region, and the lipoma was excised. Imaging of the spine at 5 years of age showed spina bifida, bony diastematomyelia, lipomeningomyelocele and a small intraspinal cystic lesion. The boy was then operated upon at the age of 5 years, and histopathological examination of the cystic lesion revealed ectopic dysplastic renal tissue.- - - - - - - - - - ranking = 2keywords = meningomyelocele (Clic here for more details about this article) |
3/28. Multiple coexistent dysraphic pathologies.INTRODUCTION: Four distinct dysraphic anomalies were observed in a single child. While combinations of such anomalies are well recognised, quadruple dysraphic pathology, nevertheless, is extremely uncommon. To our knowledge, no previous cases have been reported in the literature. CASE REPORT: We present the management of a child with a concurrent segmental meningocele, a type-1 split cord malformation (SCM) associated with hemivertebrae, lipomyelomeningoceles in each hemicord of the SCM and a terminal myelocystocele, and we review the literature on potential mechanisms of dysmorphogenesis. DISCUSSION: Existing embryologic hypotheses for the dysraphic spectrum lack experimental evidence and studies in animal models. This case challenges the existing hypotheses and illustrates our incomplete understanding of human terminal spinal cord embryogenesis. Further studies on the morphogenetic basis for these anomalies are required.- - - - - - - - - - ranking = 1.1582751459176keywords = myelomeningocele (Clic here for more details about this article) |
4/28. Chiari II malformation and occult spinal dysraphism. case reports and a review of the literature.We report two cases of children with occult spinal dysraphism who were also found to have many associated brain anomalies seen in the Chiari II malformation. No previous report has commented on the possible association between the Chiari II malformation and the 'closed' form of neural tube defect. One child had symptoms referable to pathology at the craniocervical junction. Neither child had cutaneous stigmata associated with occult spinal dysraphism over the caudal midline spine. These cases, although seemingly rare, lend support to the theories that the association between the Chiari II malformation and patients with myelomeningoceles is due to dysgenesis of the rostral and caudal neural tube. These cases are also important as refutation of earlier theories that link these two entities by proposing that the Chiari II malformation is due to overdrainage of cerebrospinal fluid at the site of myelomeningocele.- - - - - - - - - - ranking = 2.3165502918353keywords = myelomeningocele (Clic here for more details about this article) |
5/28. magnetic resonance imaging of progressive hydrosyringomyelia in two patients with meningomyelocele.Two patients who postoperatively developed extensive multiseptated hydrosyringomyelia following surgical repair of a lumbal meningomyelocele are reported. Since MRI has been available, an increasing number of reports showed that MRI is useful in the diagnosis of hydrosyringomyelia. Hydrosyringomyelia can be considered as a dysraphic lesion. Etiology and pathogenesis of hydrosyringomyelia are still not fully understood. Probably arachnoidal adhesions and cord tethering in both patients may be potential factors in producing cystic degeneration of the underlying structure secondary to ischemia.- - - - - - - - - - ranking = 5keywords = meningomyelocele (Clic here for more details about this article) |
6/28. Second-trimester diagnosis of complete trisomy 9 associated with abnormal maternal serum screen results, open sacral spina bifida and congenital diaphragmatic hernia, and review of the literature.OBJECTIVES: To present the prenatal diagnosis of complete trisomy 9 and to review the literature CASE: A 25-year-old primigravida woman was referred for amniocentesis at 19 weeks' gestation because of abnormal maternal screen results showing an elevated maternal serum alpha-fetoprotein (MSAFP) level and a low maternal serum free beta-human chorionic gonadotrophin (MSfreebeta-hCG) level. RESULTS: Genetic amniocentesis revealed a karyotype of 47,XX, 9 in the amniocytes and an elevated amniotic fluid AFP level. ultrasonography demonstrated intrauterine growth restriction, left congenital diaphragmatic hernia, fetal ascites, a sacral spina bifida, a horseshoe kidney, and absence of amniotic fluid. Ultrafast magnetic resonance imaging scans further depicted detailed anatomical configurations of the major congenital malformations. The pregnancy was terminated subsequently. The proband postnatally manifested characteristic facial dysmorphism, limb deformities, and an open sacral spina bifida with myelomeningocele. cytogenetic analysis of the skin fibroblasts revealed a karyotype of 47,XX, 9. Molecular studies of various uncultured fetal tissues using microsatellite markers confirmed a diagnosis of complete trisomy 9 resulting from a meiotic I nondisjunction error of maternal origin. CONCLUSION: Complete trisomy 9 can be identified prenatally with advanced maternal age, sonographically detected fetal structural abnormalities, and abnormal maternal serum screen results. Fetuses with complete trisomy 9 may be associated with congenital diaphragmatic hernia, an open sacral spina bifida, elevated MSAFP, and low MSfreebeta-hCG. We suggest detailed prenatal imaging investigations and genetic analyses of multiple fetal tissues when a prenatal diagnosis of trisomy 9 is made.- - - - - - - - - - ranking = 1.1582751459176keywords = myelomeningocele (Clic here for more details about this article) |
7/28. Treatment of refractory intracranial hypertension in a spina bifida patient by a concurrent ventricular and cisterna magna-to-peritoneal shunt.CASE REPORT: A 20-year-old female born with a thoracic level myelomeningocele, Chiari II malformation, and hydrocephalus treated at birth developed clinical features of increased intracranial pressure (ICP) due to shunt malfunction. The patient became comatose. Her ICP remained high despite a functioning shunt and even after the ventricular catheter was exteriorized. diagnostic imaging consistently demonstrated slit-like ventricles, a Chiari II malformation, and a tethered spinal cord. We attributed her neurological condition either to brainstem compression or increased ICP related to venous outlet obstruction at the foramen magnum. OUTCOME: The patient improved rapidly after undergoing a Chiari II decompression and placement of a shunt from the cisterna magna and upper cervical subarachnoid space to the peritoneum connected by a "Y" connector to the ventricular catheter. CONCLUSION: The complex hydrocephalus was effectively treated by this concurrent ventricular and cisterna magna-to-peritoneum shunt.- - - - - - - - - - ranking = 1.1582751459176keywords = myelomeningocele (Clic here for more details about this article) |
8/28. Occult spinal dysraphism and Pacinian hamartomas.CASES: The authors report two patients with occult spinal dysraphism who, following histological analysis, were found to harbor associated Pacinian hamartomas. One patient's hamartoma was found in conjunction with their lipomyelomeningocele and the other was combined with a presacral myxopapillary ependymoma associated with a fatty filum terminale. DISCUSSION AND CONCLUSION: We review the literature and believe this to be only the third report of Pacinian hamartomas in association with occult spinal dysraphism. Although Pacinian hamartomas are seemingly rare in conjunction with occult spinal dysraphism, mesodermal elements are often found in this disease entity.- - - - - - - - - - ranking = 1.1582751459176keywords = myelomeningocele (Clic here for more details about this article) |
9/28. Split notochord syndrome variant: prenatal findings and neonatal management.Spilt notochord syndrome is an extremely rare form of spinal dysraphism characterized by a complete cleft of the spine and a persistent communication between endoderm and ectoderm. A variant of split notochord syndrome was diagnosed in a 25-week-old fetus showing a prolapsed congenital colostomy and a spinal cystic lesion. The final diagnosis included protruding colon segment, imperforate anus with a rectourethral fistula and lipomyelomeningocele. The ultrasound features of the condition and the post-natal management are discussed. The neonate was successfully treated with a posterior sagittal anorectoplasty, while the lipomyelomeningocele was resected at a later stage.- - - - - - - - - - ranking = 2.3165502918353keywords = myelomeningocele (Clic here for more details about this article) |
10/28. blindness following ventriculoperitoneal shunt block in a child with spinal dysraphism: case report.A six-year-old boy presented with a week history of increasing headache, visual loss and convulsions. He had repair of myelomeningocele early in life and later had insertion of a ventriculoperitoneal shunt for hydrocephalus after the repair. He was treated for meningitis at the referring hospital without improvement. Computed tomography scan of the brain showed ventricular enlargement. The shunt was found to be broken and blocked and was revised. This was followed by rapid improvement but vision was never regained. Shunt malfunction in patients with spinal dysraphism can lead to visual loss but the features may mimic those of meningitis, and delayed referral and treatment. Early shunt revision should prevent this complication.- - - - - - - - - - ranking = 1.1582751459176keywords = myelomeningocele (Clic here for more details about this article) |
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