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

摘要

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.