Isolated Horner's syndrome and syringomyelia.
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Although syringomyelia has been associated with Horner's syndrome, it is typically associated with other neurological findings such as upper limb weakness or numbness. A patient is described who had an isolated Horner's syndrome as the only manifestation of syringomyelia. A 76 year old woman was discovered to have right upper lid ptosis and right pupillary miosis. Neurological examination was unremarkable, and pharmacological testing was consistent with localisation of the lesion to a first or second order sympathetic neuron. Neuroimaging disclosed a Chiari I malformation with a syrinx extending to the C2 to C4 level. An isolated Horner's syndrome may be the presenting manifestation of syringomyelia. (+info)
Can hindbrain decompression for syringomyelia lead to regression of scoliosis?
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Scoliosis in childhood develops secondary to syringomyelia in some children. The existing literature does not provide a clear answer as to whether surgical treatment of the syrinx can allow subsequent improvement of the spinal deformity, thus preventing the need for scoliosis surgery. This series comprised 16 patients with syringomyelia who presented with significant scoliosis in the absence of major neurological deficit. All underwent a hindbrain decompression, and follow-up ranged from 1 to 6 years (mean 2.5 years). Subsequent deformity surgery was necessary in eight cases, but the scoliosis was seen to improve or arrest its progression in six (37.5%). Improvement was found to be statistically more likely in children of younger age at the time of syrinx surgery and in those with left thoracic curves. Improvement occurred in 71.4% of those under the age of 10 at the time of hindbrain decompression. (+info)
Spinal dural arteriovenous fistula associated with syringomyelia.
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The previously undescribed association of a spinal dural arteriovenous fistula with syringomyelia was found in a 60-year-old male, who developed increasing paresis, numbness of both lower extremities and sphincteric dysfunction. Symptoms and signs were attributed to a syringomyelia at T5-L1 and an arteriovenous spinal dural fistula at L1. The fistula was successfully immobilised with N-butyl-cyano-acrylate. Six months after the procedure, all abnormalities had nearly disappeared. Whether the relation between the fistula and the syringomyelia was coincidental or causative could not be determined. (+info)
MR findings in spinal hemangioblastoma: correlation with symptoms and with angiographic and surgical findings.
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BACKGROUND AND PURPOSE: To our knowledge, a detailed analysis of MR findings in spinal hemangioblastoma has not been conducted to date. Our purpose was to elucidate the MR features of this disease with special attention to tumor size, correlation with MR findings and clinical symptoms, and any differences between patients with and without von Hippel-Lindau disease (VHLD). METHODS: MR images in five patients with VHLD and seven patients without VHLD were reviewed retrospectively for spinal hemangioblastoma by two neuroradiologists. The MR findings were correlated with clinical symptoms and with angiographic and surgical findings. RESULTS: The MR features depended on the size of the spinal hemangioblastoma. Small (10 mm or less) hemangioblastomas were mostly isointense on T1-weighted images, hyperintense on T2-weighted images, and showed homogeneous enhancement. Larger hemangioblastomas tended to be hypointense or mixed hypo- and isointense on T1-weighted images, heterogeneous on T2-weighted images, and tended to show heterogeneous enhancement. Small hemangioblastomas were located at the surface of the spinal cord, most frequently along its posterior aspect. These were subpial in location at surgery and showed well-demarcated, intense enhancement. Symptomatic small hemangioblastomas had relatively large associated syringes, whereas asymptomatic ones did not. A hemangioblastoma larger than 24 mm was invariably accompanied by vascular flow voids. There was no difference in the MR findings between the two patient groups except for the multiplicity and higher percentage of small tumors in patients with VHLD. CONCLUSION: Knowledge of these MR features helps to differentiate spinal hemangioblastoma from other diseases that show enhancing nodules. (+info)
Surgical management of syringomyelia-Chiari complex.
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Great variety exists in the indications and techniques recommended for the surgical treatment of syringomyelia-Chiari complex. More recently, magnetic resonance (MR) imaging has increased the frequency of diagnosis of this pathology and offered a unique opportunity to visualize cavities inside the spinal cord as well as their relationship to the cranio-cervical junction. This report presents 18 consecutive adult symptomatic syringomyelia patients with Chiari malformation who underwent foramen magnum decompression and syringosubarachnoid shunting. The principal indication for the surgery was significant progressive neurological deterioration. All patients underwent preoperative and postoperative MRI scans and were studied clinically and radiologically to assess the changes in the syrinx and their neurological picture after surgical intervention. All patients have been followed up for at least 36 months. No operative mortality was encountered; 88.9% of the patients showed improvement of neurological deficits together with radiological improvement and 11.1% of them revealed collapse of the syrinx cavity but no change in neurological status. None of the patients showed further deterioration of neurological function. The experience obtained from this study demonstrates that foramen magnum decompression to free the cerebro-spinal fluid (CSF) pathways combined with a syringosubarachnoid shunt performed at the same operation succeeds in effectively decompressing the syrinx cavity, and follow-up MR images reveal that this collapse is maintained. In view of these facts, we strongly recommend this technique, which seems to be the most rational surgical procedure in the treatment of syringomyelia-Chiari complex. (+info)
Natural history of scoliosis in children with syringomyelia.
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We performed a retrospective review of 27 scoliotic patients with syringomyelia using MRI. Their mean age at the first MRI examination was 10.9 years, and at the final review 15.8 years. The mean ratio of the diameter of the syrinx to the cord on the midsagittal MRI (S/C ratio) decreased from 0.49 to 0.24; 14 patients showed a decrease of 50% or more (reduction group). In this reduction group, the cerebellar tonsillar herniation decreased from a mean of 11.3 mm to 6.0 mm, and some improvement in dissociated sensory disturbance was seen in nine of 13 patients. The scoliosis improved by 5 degrees or more in six patients in the reduction group. Our results indicate that spontaneous shrinkage of syringomyelia in children is not unusual and is associated with improvement in the tonsillar herniation, the scoliosis and the neurological deficit. (+info)
Comparison of different operative modalities in post-traumatic syringomyelia: preliminary report.
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Post-traumatic syringomyelia (PTS) is a relatively rare, but potentially disastrous, complication of spinal cord injury. Operative treatment by shunting procedures often shows only a short-term improvement, and the rate of recurrence of syringomyelia is high, so different treatment modalities have been used in the last years. The various results are discussed in this analysis. A prospective clinical study was conducted of 30 patients with PTS treated by shunting procedures or with pseudomeningocele over a period of 9 years, and followed with regular clinical and magnetic resonance imaging examinations. Shunting procedures like syringosubarachnoid and syringopleural or -peritoneal shunting showed good results only at the first follow-ups. In our department, we perform an artificial liquor reservoir at the level of the lesion after opening the spinal pathways and arachnoid adhesions at that level. This procedure was performed in 12 patients. Five of these had been previously operated by shunting procedures; all of them had suffered a recurrence of syringomyelia because of internal occlusion. In the group of patients treated by shunting procedures, a neurological improvement was be recorded in five, and a steady state in eight. Five patients showed a further deterioration. The performance of an artificial liquor reservoir to guarantee a free flow of cerebrospinal fluid around the lesion resulted in a neurological improvement in ten patients, with two maintaining a steady state. Our experience is that shunting procedures often show a neurological improvement only in the short term; the rate of recurrence of typical shunting complications is high. The performance of a pseudomeningocele is an encouraging new step in the treatment of PTS. Further long-term follow-up studies are necessary to assess the benefits of this new method. (+info)
Familial syringomyelia.
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Four cases of syringomyelia in two separate families are reported. (+info)