1/34. Cutaneomeningospinal angiomatosis (Cobb syndrome) with tethered cord.A newborn presented with a skin-covered lumbar mass with a subcutaneous hemangioma and on a magnetic resonance image (MRI) revealed a tethered spinal cord with a local mass. The mass had signal characteristics compatible with a lipoma. An initial diagnosis of a lipomeningocele with tethered cord was made, and the patient underwent surgical exploration and subtotal resection of the mass. A follow-up MRI revealed that the cord was still tethered, but an additional mass was present. The initial mass with signal characteristics of lipomatous tissue was accompanied by a low-signal mass in the lumbosacral canal, ventral to the cord, and bilateral enlargement of the foramina at the lumbosacral level. Because of a concern for an intraspinal tumor, a second operative intervention was performed. Multiple biopsies of the mass inside the spinal cord, the nerve roots and at the level of the foramina revealed angiomas that had similar pathology in all the specimens. A partial resection of the masses and a release of the tethered cord was performed by sectioning the thickened filum terminale. The diagnosis of Cobb's syndrome was made. The unique association of a tethered cord and the Cobb syndrome is reported here.- - - - - - - - - - ranking = 1keywords = meningocele (Clic here for more details about this article) |
2/34. Segmental spinal cord hypoplasia and meningocele with preservation of medullary function: case report.BACKGROUND: In very rare dysraphic cases, it is not clear whether the primary abnormalities are in the neural elements, or in the bony elements. CASE DESCRIPTION: We describe a case of segmental hypoplasia of the spinal cord, with absent nerve roots in the afflicted segments, and associated meningocele and vertebral abnormalities. We illustrate the arguments for and against the classification of this lesion either as an atypical case of myelomeningocele (MMC) or as a mild case of segmental spinal dysgenesis (SSD). CONCLUSIONS: Possibly, in this exceptional case, the primary defect is in the neural tissue like in more usual cases of MMC and not in the spine, like in segmental spinal dysgenesis.- - - - - - - - - - ranking = 6keywords = meningocele (Clic here for more details about this article) |
3/34. prenatal diagnosis of diastematomyelia.INTRODUCTION: Diastematomyelia, also termed split cord malformation, is a form of occult spinal dysraphism characterized by a cleft in the spinal cord. prenatal diagnosis of this anomaly is possible by ultrasonography (US), and fetal MRI can be used to diagnose the type of diastematomyelia precisely. Diastematomyelia can be isolated or associated with other dysraphisms, segmental anomalies of the vertebral bodies, or visceral malformations (horseshoe or ectopic kidney, utero-ovarian malformation, and anorectal malformation). We present three cases of fetal diastematomyelia investigated using a multimodal prenatal work-up (US, MRI, 3D-CT). CASES: The first case, detected at 20 weeks' gestation, had a lumbar meningocele. At 30 weeks' gestation, direct US visualization revealed the division of the spinal cord into two hemicords. This patient illustrates an isolated type II diastematomyelia with a favorable prognosis. The second case, detected at 22 weeks' gestation, presented with disorganization of bony process of the vertebral column with a midline echogenic bony spur, asymmetrical hemicords, and a foot malposition. Fetal MRI at 26 weeks' gestation and CT/3D reconstructed at 32 weeks' gestation confirmed a type I diastematomyelia with orthopedic malposition. The third case, detected at 22 weeks' gestation, presented with widening of the lumbar canal and scoliosis. Prenatal work-up (US, MRI) disclosed other visceral malformations (pelvic kidney), which led to the assumption of a complex polymalformative syndrome. The pregnancy was terminated. Fetopathologic examination disclosed even more visceral malformations (anal atresia and unicorn uterus).- - - - - - - - - - ranking = 1keywords = meningocele (Clic here for more details about this article) |
4/34. Spinal congenital dermal sinus associated with upper thoracic meningocele. Case Report.The congenital dermal sinus is an abnormal epithelium-lined sinus tract between the skin surface and deeper tissues. It occurs during neurulation when the neural groove closes to form the neural tube on Day 26 of gestation and results from a failure of neuroectoderm to separate from the cutaneous ectoderm. The most frequent location is the lumbosacral area; an upper thoracic location is quite rare. This 37-year-old man presented with headache and numbness in both arms. No specific neurological findings were observed. physical examination revealed a dimple at T-2. radiography and magnetic resonance imaging of the thoracic spine revealed spina bifida at T1-3, a meningocele, and a dermal sinus tract complex. The treatment approach and outcome in this unusual case are presented.- - - - - - - - - - ranking = 5keywords = meningocele (Clic here for more details about this article) |
5/34. Triple neural tube defect--cranium bifidum with rostral and caudal spina bifida--live evidence of multi-site closure of the neural tube in humans.OBJECTIVE: The coexistence of three neural tube defects (NTDs) in a single child is an exceptional event. A review of the literature revealed nine published "double" NTD cases, but no cases of "triple" NTDs have been reported to date. CASE REPORT: The rare case of a two-year-old boy with three distinct NTDs is presented. The boy had a 17x15x15-cm(3) parieto-occipital encephalocele, a small cervical myelomeningocele, and a 11x11x8-cm(3) thoracolumbar myelomeningocele. hydrocephalus and Chiari II malformation accompanied the NTDs. All three lesions were surgically treated with good cosmetic results and satisfactory neurologic outcome. CONCLUSIONS: Current neural tube closure theories and models are reviewed in an attempt to better understand this extremely unusual coexistence. The multi-site closure model is clearly more useful in our understanding of NTDs.- - - - - - - - - - ranking = 2keywords = meningocele (Clic here for more details about this article) |
6/34. Familial lipomyelomeningocele: a further report.Lipomyelomeningocele is a form of spina bifida occulta with a distinct pathogenesis that differentiates it from open neural tube defects. Familial forms are rare and the condition may be polygenic. We report on 2 affected siblings with similar lesions and raise the possibility of an autosomal recessive pattern of inheritance with implications for genetic counselling.- - - - - - - - - - ranking = 5keywords = meningocele (Clic here for more details about this article) |
7/34. 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 = 1keywords = meningocele (Clic here for more details about this article) |
8/34. Serial neuropsychological assessment and evidence of shunt malfunction in spina bifida: a longitudinal case study.Myelomeningocele is often accompanied by hydrocephalus (MMH), making it a potentially unstable neurological condition requiring shunt placement and possible revisions. Serial neuropsychological assessment is an important tool in monitoring children with MMH, as cognitive changes can indicate shunt malfunction and hydrocephalus. We present the case of a girl with MMH who had five neuropsychological assessments (ages 5, 7, 11, 12, and 14). Despite a lack of overt neurological symptoms or report of behavioral decline, testing at age 11 revealed decline in multiple neurobehavioral domains, and imaging at that time showed increased hydrocephalus, requiring shunt revision. Subsequent neuropsychological assessment conducted after a 2-year period of medical stability showed improvement and/or a return to baseline levels in some skill areas (i.e., working memory, verbal memory, visuomotor integration, and sustained attention), yet more lasting impairments in others (i.e., Verbal IQ, processing speed, organization, and response inhibition). These lasting cognitive deficits potentially impact independent completion of complex medical self-care tasks. This pattern of recovery highlights vulnerability of brain systems supporting executive functions in children with hydrocephalus and shunt failure.- - - - - - - - - - ranking = 1keywords = meningocele (Clic here for more details about this article) |
9/34. Retrograde intraventricular hemorrhage caused by a traumatic sacral pseudomeningocele in the presence of spina bifida occulta. Case report.The authors present the case of a large, posttraumatic sacral pseudomeningocele in the presence of spina bifida occulta. A pseudomeningocele in the sacral region is associated with trauma and with marfan syndrome, but only one occurrence has been reported in association with spinal dysraphism. Trauma resulted in bleeding into the pseudomeningocele and retrograde passage of blood and fat into the ventricles. An oculomotor nerve palsy subsequently developed in the patient. The authors suspected a subarachnoid hemorrhage caused by a posterior communicating artery aneurysm, although this hypothesis was refuted on further investigation. The pseudomeningocele was drained by direct exposure of the neck and opening of the sac. Postoperatively, communicating hydrocephalus developed and the patient underwent ventriculoperitoneal shunt placement, resulting in resolution of the cranial nerve palsies. This first report of intradural bleeding from direct trauma to a pseudomeningocele illustrates the rare phenomenon of retrograde passage of blood from the sacral region to the brain. It also illustrates a possible but unlikely differential diagnosis of intraventricular blood and fat.- - - - - - - - - - ranking = 9keywords = meningocele (Clic here for more details about this article) |
10/34. A lateral cervical lipomyelomeningocele associated with diplomyelia.We report a case of a neonate with complex spinal dysraphism and associated anomalies of the axial skeleton. Based on the clinical presentation and radiographic findings, and recent advances in the understanding of human embryogenesis, we formulate a hypothesis that such a presentation is the result of failure of normal gastrulation. A 1-day-old male neonate presented with multiple right-sided anomalies, including hypoplastic right face and decreased movement of the right upper extremity. Radiographic studies demonstrated absent right cervical hemivertebrae, right cervical lipomyelomeningocele, and cervical diplomyelia with right hemicord terminating in a blind pouch. Anterior and posterior cervical and thoracic fusion with instrumentation was performed at the age of 3 years, and on follow-up the patient had improvement in right upper extremity strength. Complex spinal dysraphism is a pathological process that occurs during different stages in human development. We describe a case involving a rare lateral lipomyelomeningocele in the cervical-thoracic area.- - - - - - - - - - ranking = 6keywords = meningocele (Clic here for more details about this article) |
| | Next -> |