Chiari malformation type III and results of surgery: a clinical study: report of eight surgically treated cases and review of the literature.

BACKGROUND: The characteristics of Chiari malformation type III and its treatment are evaluated in this study. Radiological and surgical findings were correlated and the benefits of the therapy are discussed.
METHODS: Eight patients (6 males, 2 females) with Chiari malformation type III were studied. All patients underwent surgery to remove encephalocele along with neural tissue and to repair the dura and the skin. Associated pathologies such as hydrocephalus, tethered cord syndrome and syringomyelia were also surgically treated. Follow-up was based on the evaluation of postoperative motor and mental development, as well as on magnetic resonance imaging findings.
RESULTS: Ventriculoperitoneal shunt malfunction in 2 patients was the only surgical complication. One patient died 10 days after the operation, 7 patients survived, and the outcome of 2 patients was quite poor because of severe mental retardation and neurological deficits at the beginning. In the remaining patients, motor and mental development was normal, and the neurological outcome was satisfactory.
CONCLUSION: Surgery in the newborn period, initial severity of neurological deficits, the presence of intermittent apnoea, delayed treatment of hydrocephalus and the amount of neuronal tissue within the excised encephalocele were determined as unfavourable prognostic factors for the outcome. If the appropriate surgical procedure is done at the right time, the outcome of the Chiari malformation type III patient can be satisfactory with a low mortality rate.