Correction of clivoaxial angle deformity in the setting of suboccipital craniectomy: technical note.

A subset of patients with Chiari Type I malformation may develop neurological dysfunction secondary to an abnormally obtuse clivoaxial angle (CXA) and clivoaxial deformity causing deformative stress injury to the neural axis. Clivoaxial deformity can occur after initial standard suboccipital craniectomy, duraplasty, and C-1 laminectomy for brainstem compression, or severe clivoaxial deformity may be present in conjunction with a Chiari malformation. Clivoaxial deformity and abnormal CXA can be treated with an occipitocervical fusion (OCF). Performing OCF in the setting of a cranial defect can be challenging with currently available instrumentation. The authors describe their recent experience and outcomes in 3 consecutive pediatric patients using the "inside-out" technique for treating clivoaxial deformity and abnormal CXA in the setting of a craniectomy defect to restore stability to the craniocervical junction, while correcting the CXA.