Internal brain herniation in a patient with Apert's syndrome.

Abstract

Patients with Apert's syndrome typically exhibit craniosynostosis, exorbitism, midface hypoplasia, and symmetric syndactyly. There have also been occasional descriptions of the variable dysmorphology of the inner surface of the calvarium. We present a patient with Apert's syndrome who had an intracranial herniation of a portion of the frontal lobe through a ridge of ossified dura. The ridge and the gliotic cortical tissue were removed when the patient underwent repair of the hypertelorism at age six. Bone grafts for this repair were fashioned in part from the resected ridge. We believe this finding may be the result of a small dural tear that occurred during prior surgery.