Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome.

The Chiari malformation is a condition characterized by herniation of the posterior fossa contents below the level of the foramen magnum, and is categorized into three types based on the degree of herniation. The authors review their surgical experience between 1975 and 1985 with 50 patients afflicted with symptomatic Chiari malformations. Any patient with associated myelomeningocele, tethered spinal cord, lipoma, or diastematomyelia was excluded from this series. Forty-one patients had Chiari I malformations, seven were classified as having Chiari II, and two as having Chiari III. The presentation of pediatric and adult patients was identical. Treatment was directed at the posterior fossa pathology. Seven patients with accompanying ventral bone compression underwent transoral decompression of the cervicomedullary junction, 42 had posterior decompressive procedures, and six received ventriculoperitoneal shunts. The posterior decompression included opening the outlet foramina of the fourth ventricle, occluding any communication between the spinal cord central canal and the obex, shunting the fourth ventricle, and placing a dural graft. Postoperatively, 20% of the patients are asymptomatic, 66% improved, and 8% stabilized; in 6% the disease has progressed in spite of multiple procedures. Preoperative signs that are predictive of a less favorable outcome include muscle atrophy, symptoms lasting longer than 24 months, ataxia, nystagmus, trigeminal hypesthesia, and dorsal column dysfunction (p less than 0.05, chi-square test). A model based on the presence or absence of atrophy, ataxia, and scoliosis at the time of the preoperative examination has been generated that allows prediction of long-term outcome at the 95% confidence level.