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A 26-year-old female, the elder of monozygotic twins, presented with slow progressive numbness and pain in her left arm. Magnetic resonance (MR) imaging showed syringomyelia with Chiari malformation. The patient's birth had been difficult with prolonged delivery time, breech delivery, and neonatal asphyxia. MR imaging of the patient's twin sister showed mild tonsillar ectopia, but absence of syringomyelia. This younger sister was born without problems. The patient underwent syringosubarachnoid shunt at the C5-6 level. The syrinx was collapsed promptly, and her symptoms disappeared. This case of syringomyelia with Chiari malformation in one of twins suggests that birth injury is likely to be a cause of the pathogenesis of syringomyelia.
STUDY DESIGN: Review of five cases of post-traumatic syringomyelia originally described between 1898-1920. OBJECTIVES: To describe the earliest presentation of post-traumatic syringomyelia and to compare to modern day views of this condition. SETTING: Historical review. RESULTS: Pathogenesis and interpretation of the findings are discussed in the light of current knowledge. CONCLUSION: Credit for the first description of post-traumatic syringomyelia (in 1898) should go to W Wagner and P Stolper.
A case is reported of a pair of monozygous twins who were discordant for and their syringomyelia, and whose brother also suffered from this disease. On the basis of this extraordinarily rare constellation, the role of heredity and the possible importance of environmental factors in the manifestation of syringomyelia are discussed.
A 37-year-old man had received cervical spinal-cord injury 20 years previously. Recently, he complained of progressive spastic gait and was shown to have fracture dislocation of the C5 vertebral body which compressed the spinal cord posteriorly. He also had a syrinx from the C5 to C2 vertebral level. Postoperatively, this syrinx disappeared after anterior decompression of the C4 and C5 vertebral bodies. Such a case as this has never been reported, and this case may give a suggestion for surgical treatment of syringomyelia, because it is very natural that the most reasonable treatment of any disease is removal of the cause.
A case of platybasia associated with syringomyelia is presented where the syrinx regressed nearly completely after transoral resection of the dens. This course seems to be of considerable interest because it is hardly explainable by the common theories which try to elucidate the pathogenesis of syringomyelic cysts.
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Four normal controls and 26 cases of Arnold-Chiari I malformations and/or syringomyelia were reviewed. The pathologic cases included five isolated Arnold-Chiari I malformations, nine communicating syringomyelia, five idiopathic syringomyelia, four posttraumatic syringomyelia, one syringomyelia with hemangioblastoma, and two postshunt syringomyelia. The objectives of this study were to compare the accuracy of conventional metrizamide myelography with CT metrizamide myelography and to study indirectly the hydrodynamics of CSF flow in syringomyelia by comparing the sequential enhancement patterns of the spinal cords and cord cavities in the different groups of patients. Twenty-five patients underwent conventional metrizamide myelography immediately before CT metrizamide myelography, and one patient underwent CT metrizamide myelography only. Scans were obtained 1-2 hr, 4-8 hr, and 12-24 hr after injection of metrizamide, but not all patients were scanned during all three intervals. CT metrizamide myelography was found to be more sensitive than conventional metrizamide myelography in the diagnosis of both Arnold-Chiari I malformation and syringomyelia. Performing just an immediate and a delayed scan was found to be more cost-effective than doing all three scans. Contrary to previous reports, it was found that delayed (12-24 hr) scans demonstrated more syrinx cavities than intermediate ones. In studying the sequential enhancement patterns of the spinal cords and cord cavities, some interesting trends were observed that tend to support the theories of Aboulker and of Ball and Dayan of transneural passage of CSF into cord cavities in syringomyelia.
Syringomyelia is a condition with many possible causes, the commonest of which seems to be an abnormality at the foramen magnum. Such cases may be grouped under the heading of "Hindbrain related syringomyelia" and the principles of treatment for all such cases are largely similar. The commonest of these foramen magnum region abnormalities is hindbrain herniation which may be associated with a history of birth difficulties, a small posterior fossa, segmentation abnormalities of the cervical vertebrae or the base of the skull, arachnoiditis of the subarachnoid spaces, subarachnoid pouches, hydrocephalus and intracranial tumours or tumours partly blocking the foramen magnum. Other causes of syringomyelia include conditions which could be grouped under the heading of "non-hindbrain related syringomyelia", these mostly produce blockage of the spinal subarachnoid spaces, especially spinal "arachnoiditis" or meningeal fibrosis, including that secondary to traumatic paraplegia. Intraspinal tumours are sometimes cystic and some authors have included this association under the heading of syringomyelia. Syringomyelia of all kinds is almost always a surgical condition, the destructive forces are those of fluid distending the tissues. As a principle, treatment directed against the cause of the accumulation and the intracord propagation of the fluid by normalising the CSF pathways is more likely to be successful than drainage of the cavities. Drainage operations have an inevitable failure rate and a further incidence of complications attends myelotomy and the leaving of any drainage tube within the narrow confines of the spine. Correction of craniospinal pressure dissociation and re-establishment of a cisterna magna appears to be the most successful treatment strategy and is likely to be immediately and permanently successful in correcting not only the pressure problems such as long tract involvement and syringobulbia features but also in producing satisfactory clinical and radiological improvement in the syringomyelia. The recommended technique includes radical means to gain space at the foramen magnum by creating a large artificial cisterna magna, resecting part of the tonsils, preventing the descent of the cerebellum and avoiding the use of space occupying or fibrosis producing dural grafts. Because the pathogenesis of the cavities remains in doubt, the method by which this treatment stratagem is effective is unclear. It may be that change in the closure conditions of parts of the neuraxis, i.e., alteration in the capacitance and consequent change in pulsation characteristics afforded by the decompression may be the factor which predicates success. Surgical management of hindbrain related syringomyelia is not easy, there are hazards associated with operation, hydrocephalus demands priority in it's management.(ABSTRACT TRUNCATED AT 400 WORDS)
BACKGROUND: Most reports regarding pediatric syringomyelia have focused not only on Chiari malformation, but also on spinal dysraphism. However, the clinical characteristics of syringomyelia with spinal dysraphism are quite different from those of syringomyelia due to Chiari Type I malformation. The objectives of this study were to identify clinical characteristics of pediatric syringomyelia and to determine whether surgery prevents or corrects the scoliosis associated with syringomyelia. METHODS: We reviewed the records of 16 pediatric patients with syringomyelia and Chiari Type I malformation. The patients' ages ranged from 3 to 15 years, with mean age of 9.8 years. None of the patients had spinal dysraphism. RESULTS: Nystagmus was observed in 2 of the 16 patients, motor weakness in 8 patients, sensory disturbance in 10 patients, and scoliosis in 13 patients. As the initial surgical procedure, foramen magnum decompression (FMD) was performed in seven patients and syringo-subarachnoid (S-S) shunting in nine patients. The motor function improved in 7 of the 8 affected patients, and the sensory disturbance improved in 9 of the 10 affected patients. The magnetic resonance images obtained after the surgery revealed marked decrease of the syrinx size in all patients. Of the 13 patients with scoliosis, 5 showed improvement, 5 stabilization, and 3 deterioration. CONCLUSIONS: Compared with adolescent and adult syringomyelia, pediatric syringomyelia shows a much lower incidence of sensory disturbance and pain, but quite a high incidence of scoliosis. Surgery is effective in improving or stabilizing scoliosis in these patients.
Twenty-eight years of experience with syringomyelia of various causes in the syringomyelia clinic at the Midland Centre for Neurosurgery and Neurology has provided a database of 723 patients, mostly adults, with either hindbrain herniation, syringomyelia or both. Treatment of syringomyelia by drainage has never been the optimum primary treatment on the basis that the cavity is usually secondary to some other disturbance of the cerebrospinal fluid pathways. Over this period 73 patients had either syrinx drainage (56 syringopleural, 14 syringo-subarachnoid shunts) or other procedure such as myelotomy and cord transection. Ten years after operation only 53.5% and 50% of these two groups respectively continued to remain clinically stable. A complication rate of 15.7% included fatal haemorrhage, infection and displacement of drains. At second operation or necropsy at least 5% of shunts were found to be blocked. All the shunts were inserted without a valve and the lowering of the intrasyrinx pressure has therefore been energetic when the lower end of the drainage tube has been taken to the pleural or peritoneal cavities. This produced collapse of the cord cavities around the tip of the drainage tube and increased the likelihood of blockage. If the mechanisms which were responsible for the syringomyelia were still operative then recurrence was likely to occur alongside the drainage tube leaving the tube immured in the wall of the syrinx cavity. When hydrocephalus was present, in addition treatment of the hydrocephalus by a valved shunt to the peritoneum or to the right atrium was often effective in improving the syringomyelia. This treatment has also been used in patients without hydrocephalus. The mechanisms of improvement were unclear but this treatment stratagem has nevertheless been employed in 45 cases. In 30 out of these 45 cases the drainage of cerebrospinal fluid from outside the syrinx cavities seemed to be worthwhile; 10 patients suffered some form of complication, most of which were reversible; 8 patients were worse following surgery. It is concluded that both drainage of the syringomyelia cavity and also extra-syrinx drainage may have a place in the management of difficult syringomyelia problems although the primary treatment should be to establish the patency of CSF pathways in both hindbrain-related and non-hindbrain-related cases.