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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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The pathophysiology of syringomyelia development is not fully understood. Current prevailing theories suggest that increased pulse pressure in the subarachnoid space forces cerebrospinal fluid (CSF) through the spinal cord into the syrinx. It is generally accepted that the syrinx consists of CSF. The here-proposed intramedullary pulse pressure theory instead suggests that syringomyelia is caused by increased pulse pressure in the spinal cord and that the syrinx consists of extracellular fluid. A new principle is introduced implying that the distending force in the production of syringomyelia is a relative increase in pulse pressure in the spinal cord compared to that in the nearby subarachnoid space. The formation of a syrinx then occurs by the accumulation of extracellular fluid in the distended cord. A previously unrecognized mechanism for syrinx formation, the Bernoulli theorem, is also described. The Bernoulli theorem or the Venturi effect states that the regional increase in fluid velocity in a narrowed flow channel decreases fluid pressure. In Chiari I malformations, the systolic CSF pulse pressure and downward motion of the cerebellar tonsils are significantly increased. This leads to increased spinal CSF velocities and, as a consequence of the Bernoulli theorem, decreased fluid pressure in narrow regions of the spinal CSF pathways. The resulting relatively low CSF pressure in the narrowed CSF pathway causes a suction effect on the spinal cord that distends the cord during each systole. Syringomyelia develops by the accumulation of extracellular fluid in the distended cord. In posttraumatic syringomyelia, the downwards directed systolic CSF pulse pressure is transmitted and reflected into the spinal cord below and above the traumatic subarachnoid blockage, respectively. The ensuing increase in intramedullary pulse pressure distends the spinal cord and causes syringomyelia on both sides of the blockage. The here-proposed concept has the potential to unravel the riddle of syringomyelia and affords explanations to previously unanswered clinical and theoretical problems with syringomyelia. It also explains why syringomyelia associated with Chiari I malformations may develop in any part of the spinal cord including the medullary conus. Syringomyelia thus preferentially develops where the systolic CSF flow causes a suction effect on the spinal cord, i.e., at or immediately caudal to physiological or pathological encroachments of the spinal subarachnoid space.
OBJECTIVE: To discuss the surgical treatment of chiari-I malformation complicated with syringomyelia. METHODS: The surgical treatments of 247 cases were analyzed retrospectively. The indication of operation styles was proposed by various surgical treatment to different MRI (magnetic resonance imaging) findings. MRI findings includes: tonsillar herniation with no or slight syringomyelia (126 cases), tonsillar herniation with syringomyelia above C(2) (second cervical vertebrae) vertebral level (38 cases), serious tonsillar herniation (to C(2) approximately C(3) level) with syringomyelia of isolated spinal segments (67 cases), serious tonsillar herniation (to C(2) approximately C(3) level) with syringomyelia above C(2) vertebral level (16 cases). They were performed by posterior fossa decompression, posterior fossa decompression and incision of the syringomyelia, posterior fossa decompression and resection of the cerebellar tonsils, posterior fossa decompression and incision of the syringomyelia combined with resection of the cerebellar tonsils respectively. RESULTS: The clinical signs and symptoms had been markedly improved or improved in 197 cases (79.8%) until patients were discharged from hospital, unchanged in 39 cases (15.8%), deteriorated in 7 cases (2.8%). there were 4 death in all cases after surgery. 107 cases were followed up from 5 months to 9 years. The postoperative MRI findings in the 107 patients demonstrated that the cavities in spinal cords disappeared completely or nearly in 78 cases, reduced in 14 cases, unchanged in 15 cases. CONCLUSIONS: Posterior fossa decompression, posterior fossa decompression and incision of the syringomyelia, posterior fossa decompression and resection of the cerebellar tonsils, posterior fossa decompression and incision of the syringomyelia combined with resection of the cerebellar tonsils should be an effective method for treatment of chiari-I malformation complicated with syringomyelia. Surgical treatment may fully ameliorate the clinical syndromes.
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)