Basilar impression in osteogenesis imperfecta treated with staged halo traction and posterior decompression with short-segment fusion

Abstract

Basilar impression is a cranial base abnormality associated with osteogenesis imperfecta (OI) with serious neurologic implications but controversial treatment options. Combined anterior and posterior decompression with long-segment posterior fusion is often recommended. We report a patient with OI (Sillence type III) with basilar impression treated with halo traction followed by posterior surgery. The patient was a 12-year-old female with a presentation of hiccups and change in upper extremity function. Diagnostic imaging revealed syringomyelia, compensated hydrocephalus, basilar impression, and Chiari type I malformation. The patient was treated with halo traction followed by posterior decompression fusion from the occipital bone to C2. Bone fusion and improved syrinx were evident on images during the 5 years of follow-up. Five years after surgery, syrinx recurred and the fourth ventricular catheter was revised. The treatment with halo traction followed by posterior-only surgery of basilar impression associated with OI resulted in a good postoperative outcome.

Influence of the Severity of Osteogenesis Imperfecta on Cranial Measurements

“…CCJ alterations have been studied as a cause of death in cases of patients with OI, finding in the case of type III and V OI a high percentage of basilar invagination and respiratory deaths [ 3 , 13 ]. In order to avoid or eliminate the associated neurological symptoms, the literature includes some surgical techniques such as posterior fossa decompression with or without instrumentation, transoral or endonasal decompression with posterior occipitocervical fission, or gravity traction of the halo with posterior instrumentation [ 5 , 14 , 15 ].…”