1/114. Extrarenal nephroblastic proliferation in spinal dysraphism. A report of 4 cases.Four cases of extrarenal nephrogenic proliferation in the sacrococcygeal region with spinal dysraphism are presented. In two of the cases, features of Wilm's or incipient Wilm's tumor were present. The previous literature on sacrococcygeal nephrogenic tissue is reviewed, and the impact of these findings on the histogenesis of extrarenal sacrococcygeal Wilm's tumor is discussed.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/114. Myelocystocele-cloacal exstrophy in a pedigree with a mitochondrial 12S rRNA mutation, aminoglycoside-induced deafness, pigmentary disturbances, and spinal anomalies.A large Filipino-American family with progressive matrilineal hearing loss, premature graying, depigmented patches, and digital anomalies was ascertained through a survey of a spina bifida clinic for neural crest disorders. deafness followed a matrilineal pattern of inheritance and was associated with the A1555G mutation in the 12S rRNA gene (MTRNR1) in affected individuals as well as unaffected maternal relatives. Several other malformations were found in carriers of the mutation. The proband had a myelocystocele, Arnold-Chiari type I malformation, cloacal exstrophy, and severe early-onset hearing loss. Several family members had premature graying, white forelock, congenital leukoderma with or without telecanthus, somewhat suggestive of a waardenburg syndrome variant. In addition to the patient with myelocystocele, two individuals had scoliosis and one had segmentation defects of spinal vertebrae. The syndromic characteristics reported here are novel for the mitochondrial A1555G substitution, and may result from dysfunction of mitochondrial genes during early development. However, the mitochondrial A1555G mutation is only rarely associated with neural tube defects as it was not found in a screen of 218 additional individuals with spina bifida, four of whom had congenital hearing loss.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
3/114. Bladder cancer arising in a spina bifida patient.We report the case of a 52-year-old patient with spina bifida, neurologic bladder, and a history of recurrent urinary tract infections (UTIs) in whom a bladder cancer was incidentally discovered. Cytology, cystoscopy, and cystography showed nonspecific, extensive inflammatory lesions. Cystography demonstrated a complex of diverticulae and cellules. Pathologic examination of a diverticulectomy specimen revealed a grade III pT3b transitional and squamous cell carcinoma. Because of the similar disease causation (recurrent UTIs, stones, and indwelling catheterization), we suggest extension of the guidelines proposed for patients with spinal cord injuries (ie, annual serial bladder biopsies) to patients with nontraumatic neurogenic bladder.- - - - - - - - - - ranking = 0.2keywords = spinal (Clic here for more details about this article) |
4/114. Intradural spinal lipoma of the conus medullaris without spinal dysraphism.A 42-year-old man suffering from progressive left radicular sensory motor loss (L4 level) underwent neurosurgical repair. neuroimaging (RMI) had led to the diagnosis of schwannoma of the filum terminale with lipomatous component. Histological examination visualized a true mature lipoma associated with numerous bundles of more or less dystrophic nerve fibers. This histological benign tumor raised the problem of the genesis of intradural lipomas of spinal cord.- - - - - - - - - - ranking = 1.8keywords = spinal (Clic here for more details about this article) |
5/114. Terminal Myelocystocele:an unusual presentation.Terminal myelocystocele is an unusual form of occult spinal dysraphism. It consists of a cystic dilatation of a low-lying terminal cord herniated posteriorly through a skin-covered lumbosacral spina bifida. An arachnoid-lined meningocele, continuous with the spinal subarachnoid space, is traversed by the hydromyelic cord. Clinically, this presents with a skin-covered lumbosacral mass, but often no neurological deficit is present. We present a case of terminal myelocystocele in a child born without deficit and without an obvious back mass. diagnosis was delayed until sphincter disturbance and lower limb inequalities developed. We discuss the presentation, imaging and operative findings in this case.- - - - - - - - - - ranking = 0.4keywords = spinal (Clic here for more details about this article) |
6/114. Primary lumbosacral Wilms tumour associated with occult spinal dysraphism.A 4-year-old child presenting with sudden-onset paraplegia and a sacral tumour in association with spina bifida occulta is reported. There were no stigmata of spinal dysraphism at birth. Imaging studies confirmed a sacral tumour with extradural extension up to T10 and spinal dysraphism. The histological features of the extradural and sacral components of the tumour were consistent with a Wilms tumour. The differential diagnosis included a primary sacral teratoma containing Wilms tumour elements or a primary extrarenal Wilms tumour arising in association with a spinal dysraphism. There was no clinical response to chemotherapy or radiotherapy.- - - - - - - - - - ranking = 1.4keywords = spinal (Clic here for more details about this article) |
7/114. Split cord malformation with diastematomyelia presenting as neurogenic claudication in an adult: a case report.STUDY DESIGN: This is a report of a rare presentation of a split cord malformation with diastometamyelia. OBJECTIVES: This report draws attention to the fact that the only manifestation of diastmetamyelia in the adult patient may be neurogenic claudication. SUMMARY OF BACKGROUND DATA: patients with split cord malformations and diastometamyelia rarely have symptomatic onset in adulthood. When present, a traumatic event leading to an acute neurologic change is the usual presentation. methods: An adult patient presented with symptoms of neurogenic claudication in the left leg. magnetic resonance imaging examination showed a split cord malformation and diastometamylia at L3-L4 with spinal stenosis of the left hemicord. Decompressive laminectomy and subtotal resection of the bony spur were performed. RESULTS: Two years after decompression, the patient has complete resolution of his leg symptoms and is back to work. CONCLUSIONS: Neurogenic claudication without any objective neurologic deficit or neurocutaneous stigmas of an underlying spinal cord abnormality may be the only presentation in the adult with diastometamyelia. decompression to relieve both clinical and radiologic evidence of spinal stenosis obtained excellent outcome.- - - - - - - - - - ranking = 0.6keywords = spinal (Clic here for more details about this article) |
8/114. Infantile arachnoid cyst compressing the sacral nerve root associated with spina bifida and lipoma--case report.A 2-year-old boy presented with a rare sacral arachnoid cyst manifesting as gait disturbance. neuroimaging revealed an intradural cyst in the sacral nerve root sheath associated with spina bifida occulta and a lipoma at the same level. At surgery, the conus medullaris was situated at the L-1 level and not tethered. The highly pressurized arachnoid cyst had exposed the dural sheath of the left S-2 nerve root and compressed the adjacent nerves. An S-2 nerve root pierced through the cyst. There was no communication between the cyst and spinal arachnoid space. We thought the one-way valve mechanism had contributed to the cyst enlargement and the nerve compression. Radical resection of the cyst was not attempted. A cyst-subarachnoid shunt was placed to release the intracystic pressure. Postoperatively, his gait disturbance improved and no deterioration occurred during the 4-year follow up. Both tethered cord syndrome and sacral arachnoid cyst in the nerve root sheath should be considered in pediatric progressive gait disturbance. Cyst-subarachnoid shunt is an alternative method to cyst resection or fenestration to achieve neurological improvement.- - - - - - - - - - ranking = 0.2keywords = spinal (Clic here for more details about this article) |
9/114. Postoperative ventriculitis in infants.Eight consecutive cases of ventriculitis detected and treated during a four year period are reviewed. This complication represented 7.9% of all cases of spina bifida treated surgically, and 5.4% of all shunt precedures performed during the study period. Prophylactic antibiotic treatment did not reduce the incidence of ventriculitis, and in fact seemed to produce resistant strains, which led to difficulties in treatment and a worse prognosis. The presence of an intraventricular foreign body precludes permanent sterilization of the ventricular fluid. Finally, in those cases in which the antibiotic of choice penetrates poorly into the cerebrospinal fluid; intraventricular as well as systemic administration of the drug is indicated. With Gram negative organisms, Gentamicin is currently the drug of choice.- - - - - - - - - - ranking = 0.2keywords = spinal (Clic here for more details about this article) |
10/114. The human tail associated with intraspinal lipoma: case report.A case of a tail in a 9-month-old baby is reported. Computed tomography and magnetic resonance imaging clearly demonstrated the presence of spina bifida and lipoma continuous from the tail to the spinal canal. A few medical-historical aspects are discussed. The human tail may be related to spinal dysraphism and requires detailed neuroimaging investigation and microsurgery.- - - - - - - - - - ranking = 1.2072313975952keywords = spinal, spinal canal, canal (Clic here for more details about this article) |
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