Management of Syringohydromyelia Associated with Basilar Impression and Osteogenesis Imperfecta

Objective: Syringomyelia associated with osteogenesis imperfecta and basilar impression is a rarely seen pathology. We herein report our management experiences of patients with this rare condition. Methods: We overlook 4 patients with osteogenesis imperfecta, basilar impression and syringohydromyelia. All patients were symptomatic at presentation. One patient received a syringosubarachnoid shunt at the craniocervical junction and 2 patients underwent cranio-cervical decompression with duraplasty. One patient was followed conservatively. Follow up periods of these patients ranged up to 14 years. Results: Two patients that received a cranio-cervical decompression showed complete remission of clinical symptoms through the followup period. One patient who was treated by a syringo-subarachnoid microcatheter shunt had early regression of spinal cord symptoms, but died 6 weeks after surgery. One conservatively treated patient remained neurologically unchanged and therefore surgery was not advocated. Conclusions: Cranio-cervical decompression and duraplasty is a treatment option for symptomatic patients with syringohydromyelia and basilar impression accompanying osteogenesis imperfecta resulting in acceptable perioperative morbidity.