Basichondrocranium anomalies in adult Chiari type I malformation: a morphometric study

The purpose of this study was to determine the frequency of anomalies of the basichondrocranium in a series of 42 patients with Chiari type I malformation compared with a control group of 46 subjects. Sixteen patients also had syringomyelia. Linear, angular and posterior fossa surface area measurements were taken on conventional lateral skull x-rays. Posterior fossa volume was estimated by CT scanning. In patients there was shortening of clivus length, Twining-opisthion distance and Chamberlain's line. Basal and Boogard angles were enlarged. The size of the posterior fossa was smaller in patients than in controls. Only 10 (23.8%) patients had no evidence of occipital dysplasia. When discriminant analysis was applied to the data, the most discriminative variables were posterior fossa area and clivus length which allowed accurate identification of 76% of patients as belonging to the patient group and 79% of controls as belonging to the control group. These findings prove that under-development of the basichondrocranium with a small size of the posterior fossa is an outstanding feature in adult Chiari type I malformation, and support the hypothesis that tonsillar ectopia is secondary to the disproportion between the posterior fossa and the cerebellum, which is forced to grow into the cervical spinal canal.