1/8. The Chiari/hydrosyringomyelia complex presenting in adults with myelomeningocoele: an indication for early intervention.OBJECTIVE: To determine how adults with myelomeningocoele who develop the Chiari/hydrosyringomyelia complex present, and to determine if surgical intervention influences outcome in these patients. methods: A chart review of the 220 patients who attend a clinic for adults with spina bifida and hydrocephalus (CASBAH), and follow-up of the five cases with myelomeningocoele who had surgical intervention for associated symptomatic Chiari/hydrosyringomyelia complex. RESULTS: Bilateral upper limb weakness and wasting were the commonest presenting symptoms (four patients). Sensory disturbance (three patients) was also common, dysphagia (one) and ataxia (one) occurring less often. The median time to surgical intervention was 36 months. Two patients had a shunting procedure performed in isolation, two foramen magnum decompression in addition to a shunting procedure and one a foramen magnum decompression. Surgical intervention did not completely reverse problems attributed to the Chiari/hydrosyringomyelia complex in any of the cases. One patient died post-operatively. Of the four who survived one had some improvement in function post-operatively, two remained static and one had further mild deterioration. CONCLUSION: All adults with myelomeningocoele should be questioned about changes in upper limb function for early detection of Chiari/hydrosyringomyelia complex. Our results suggest that early intervention is needed if further deterioration is to be avoided, and to improve the chances of neurological and functional recovery.- - - - - - - - - - ranking = 1keywords = hydrosyringomyelia, syringomyelia (Clic here for more details about this article) |
2/8. Treatment of Chiari malformation, syringomyelia and hydrocephalus by neuroendoscopic third ventriculostomy.This short paper illustrates a case with cervical myelomeningocoele, a Chiari malformation (CM), hydrocephalus (HC) and cervical syringomyelia treated by neuroendoscopic third ventriculostomy (NTV) with resolution of both the hydrocephalus and the syrinx. Two similar cases are discussed. The technique is advocated for the treatment of such complex dysraphic conditions.- - - - - - - - - - ranking = 0.00044322918189159keywords = syringomyelia (Clic here for more details about this article) |
3/8. 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 = 0.87508864583638keywords = hydrosyringomyelia, syringomyelia (Clic here for more details about this article) |
4/8. Thoracic meningocele, meningomyelocele or myelocystocele? Diagnostic difficulties, consequent implications and treatment.spina bifida cystica is a closing disorder of the neural tube which infrequently occurs in the thoracic region. A rare lesion called myelocystocele is a variant of spina bifida cystica and is associated with syringomyelia, Chiari type 2 malformation and hydrocephalus. Usually the patient has no neurological deficit, but future deterioration can occur due to posterior tethering of the spinal cord by adhesions. The prenatal diagnosis by ultrasound study can be misleading and in order to attain the correct diagnosis, especially if abortion is considered, a prenatal MRI scan should be done before the parents are counselled, and should be repeated prior to operative treatment. Surgical correction of myelocystocele is not only for cosmetic reasons, but also to untether the spinal cord prophylactically to prevent future neurological deterioration. In this case report, we present a child born with a thoracic myelocystocele, the diagnostic difficulties, consequent implications and surgical treatment.- - - - - - - - - - ranking = 8.8645836378318E-5keywords = syringomyelia (Clic here for more details about this article) |
5/8. Value of dynamometry in assessing upper extremity function in children with myelomeningocele.Children with myelodysplasia frequently have abnormal hand function. Etiologies include brainstem and cervical cord compression due to the Chiari II malformation, syringomyelia, hydrocephalus, and cerebral dysmorphism. Determination of potentially correctable causes of hand dysfunction may be difficult. We describe the use of the Jaymar dynamometer for assessing grip strength as a means of detecting early changes in hand function.- - - - - - - - - - ranking = 8.8645836378318E-5keywords = syringomyelia (Clic here for more details about this article) |
6/8. Symptomatic syringomyelia following surgery to treat retethering of lipomyelomeningoceles.The authors report the cases of three children in whom symptomatic syringomyelia occurred de novo following an operation to relieve retethering of a previously treated lipomyelomeningocele. No patient had a Chiari malformation. In two cases, magnetic resonance imaging performed before the first operation did not show a syrinx. At the time of surgery to relieve retethering, it was discovered that one of these patients had a minor degree of terminal hydromyelia and the other had a prominent central canal within the conus medullaris. The third patient was initially studied by means of myelography, which gave no indication of a syrinx, and one was not found at the time of the surgery to release the retethering. Neurological deficits appeared abruptly within several months of operation in two children, and insidiously after 12 to 18 months in the other symptomatic individual. In all three cases, the syrinx involved the distal spinal cord adjacent to the site of the lipoma. Treatment consisted of syringosubarachnoid shunting, which arrested the progression of deficits but only partially reversed them. The details of each case are presented and the possible mechanism of syrinx formation discussed. Early recognition and treatment of this unusual but important problem are emphasized.- - - - - - - - - - ranking = 0.00044322918189159keywords = syringomyelia (Clic here for more details about this article) |
7/8. Lipomyeloschisis associated with thoracic syringomyelia and Chiari I malformation.Co-existence of Chiari I malformation and myelomeningocele is uncommon. syringomyelia, when associated with a Chiari I malformation, classically involves the cervical spinal cord. Intramedullary extension of lipoma is unusual in lipomyeloschisis. A patient with lumbar lipomyelomeningocele with tethered cord, lower thoracic syringomyelia and Chiari I malformation, shown by MRI is reported.- - - - - - - - - - ranking = 0.00044322918189159keywords = syringomyelia (Clic here for more details about this article) |
8/8. Surgical treatment of syringomyelia associated with spinal dysraphism.Clinical and radiological features of syringomyelia in 15 patients with spinal dysraphism are reported. There were 8 patients with occult spinal dysraphism (lumbosacral lipoma) and 7 with spina bifida aperta (meningomyelocele). syringomyelia with spinal dysraphism can be radiologically divided into two types according to the dysraphic state. The syrinx in the patients with occult spinal dysraphism occurred immediately rostral to the lipoma and was localized to the lower thoracic to lumbar levels, while in the meningomyelocele patients the syrinx extended from the cervical to the thoracic level. Large syrinx formation was recognized in 1 of the 7 occult spinal dysraphism cases and 3 of the 8 meningomyelocele cases. For syringomyelia with occult spinal dysraphism, 4 patients underwent syringo-subarachnoid shunting (S-S shunt, 2 cases) or syringostomy (2 cases) during an untethering operation. In the case of meningomyelocele, S-S shunts were placed in 2 patients. Collapse of the syrinx was achieved in all 6 patients who underwent S-S shunting or syringostomy. Decreased size of the syrinx was also noted in 3 occult spinal dysraphism patients who underwent untethering operations alone. In conclusion, a large syrinx in the case of spinal dysraphism should be surgically treated. S-S shunting is effective in both types of syringomyelia. foramen magnum decompression may be an alternative method of surgical treatment for syringomyelia in patients with meningomyelocele.- - - - - - - - - - ranking = 0.00070916669102654keywords = syringomyelia (Clic here for more details about this article) |