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Joseph H. Piatt Jr.

visualization of every level of the craniospinal axis. Consequently, investigators at spina bifida programs are seeing fewer new patients but are studying in much greater detail those who do present. Syringomyelia is commonly associated with myelomeningocele, as are such other potentially surgically treatable lesions as Chiari II malformation, diastematomyelia, dermoid and epidermoid cysts, neurenteric cysts, spinal arachnoid cysts, spinal lipomata, and meningeal cicatrix associated with secondary SCT. Each of these entities, however, has a distinct natural history, is

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Joseph H. Piatt Jr.

S yringomyelia is a generally progressive condition characterized by expanding CSF-filled cavities within the substance of the spinal cord. Its developmental process is incompletely understood, but the great majority of cases can be attributed to an underlying disturbance of CSF pulsatility. The Chiari malformations are the most common identifiable cause of syringomyelia, 5, 13, 22 and in this setting the pathophysiological characteristics of syringomyelia have been studied extensively. 5, 16 In both Chiari malformation Types I and II, displaced cerebellar

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Joseph H. Piatt Jr.

the diagnosis of tethering has never been measured, it is likely to be significant. All that can be said is that symptomatic secondary tethering occurs and that selected patients can benefit from surgical treatment. Chiari Malformation and Syringomyelia There is substantial literature describing the presentations of Chiari malformation Type II and associated syringomyelia in childhood, 68 , 70 , 83 although contemporary practice has trended away from earlier emphasis on craniocervical decompression toward confirmation of satisfactory CSF shunt function

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Joseph H. Piatt Jr.

hydrocephalus included shunt insertion, shunt revision, shunt removal, ventriculocisternostomy, and external ventricular drainage (02.2, 02.31–02.35, 02.39, 02.41–02.43, and 54.95). Surgery for degenerative spine disease included cervical and lumbar disc surgery, cervical fusions excluding atlantoaxial fusion, and lumbosacral fusions (03.02, 03.09, 80.50–80.52, 80.59, 81.02, 81.03, 81.06–81.08, 81.32, 81.33, 81.36–81.38, 84.58, and 84.60–84.64). Surgery for spinal cord conditions included surgery for tethering and for syringomyelia (03.4, 03.51, 03.52, 03.59, 03.6, 03.7, 03

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Joseph H. Piatt Jr.

consistent with conventional neurosurgical practice. Review of the details of clinical presentations in Table 1 shows that 60 patients (23% of the study group) presented with headaches alone. In 68 patients (27%) there were brainstem symptoms alone, predominantly dysphagia, vertigo, and difficulty with balance. Such complaints are potentially very disturbing to patients and families, but they are very difficult to measure for the purpose of clinical research. Only 69 patients (27%) had syringomyelia, the most concrete indication for surgical treatment of CM-I. Clearly and

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Tethered hindbrain

Case report

Jennifer C. Kernan, Michael A. Horgan and Joseph H. Piatt

cystoperitoneal shunting. Case report. J Neurosurg 83: 1072–1074, 1996 9. Oakes WJ : Chiari malformations, hydromyelia, syringomyelia , in Wilkins RH , Rengachary SS (eds): Neurosurgery. New York : McGraw-Hill , 1985 , p 2102 Oakes WJ: Chiari malformations, hydromyelia, syringomyelia, in Wilkins RH, Rengachary SS (eds): Neurosurgery. New York: McGraw-Hill, 1985, p 2102 10. Pang D : Split cord malformation: part II: clinical syndrome. Neurosurgery 31 : 481 – 500 , 1992 Pang D: Split cord