Posterior fossa volume in children with Chiari malformation Type I

The authors sought to establish whether the volume of the posterior fossa in children suffering from Chiari malformation Type I (CM-I) is smaller than normal, as has been suggested previously. They also investigated the role of syringomyelia in posterior fossa development.

Both posterior fossa volume (PFV) and intracranial volume (ICV) were measured using segmentation techniques on preoperative magnetic resonance images obtained in 42 children who underwent surgery for CM-I (mean age 127 months, range 36-204 months); 25 (59%) of the patients had syringomyelia. The PFV/ICV ratio was calculated to eliminate differential supratentorial growth. Patients who had deformities potentially interfering with skull growth or who had undergone a shunt insertion procedure prior to craniovertebral decompression were excluded. The results were compared with measurements of 51 healthy children using one-way analysis of variance. In patients with CM-I only, the mean PFV and PFV/ICV ratios were not statistically different than those for healthy children. In patients with both CM-I and syringomyelia (CM-S), the mean PFV and PFV/ICV ratios were statistically smaller than those for healthy children. The ICV was 1383 cm3 in the healthy group, 1459 cm3 in the CM-I only group, and 1400 cm3 in the CM-S group (p = 0.363); the PFV was 186 cm3 in the healthy group, 196 cm3 in the CM-I only group, and 171 cm3 in the CM-S group (p = 0.036); the PFV/ICV ratio was 0.135 in the healthy group, 0.134 in the CM-I only group, and 0.122 in the CM-S group (p = 0.004). These differences were more prominent in the first 10 years of life.

Children with isolated CM-I do not have a PFV smaller than normal, whereas children with both CM-I and syringomyelia have a PFV significantly smaller than normal. This result indicates that the two subgroups may represent different phenotypic expression or even a different pathogenesis.