Volumetric analysis of the posterior cranial fossa in a family with four generations of the Chiari malformation Type I

Abstract

Object: Many authors have concluded that the Chiari malformation Type I (CM-I) is due to a smaller than normal posterior cranial fossa. In order to establish this smaller geometry as the cause of hindbrain herniation in a family, the authors of this paper performed volumetric analysis in a family found to have this malformation documented in 4 generations.

Methods: Members from this family found to have a CM-I by imaging underwent volumetric analysis of their posterior cranial fossa using the Cavalieri method.

Results: No member of this family found to have CM-I on preoperative imaging had a posterior fossa that was significantly smaller than that of age-matched controls.

Conclusions: The results of this study demonstrate that not all patients with a CM-I will have a reduced posterior cranial fossa volume. Although the mechanism for the development of hindbrain herniation in this cohort is unknown, this manifestation can be seen in multiple generations of a familial aggregation with normal posterior fossa capacity.