Percutaneous intramedullary fixation of long bone deformity in severe osteogenesis imperfecta.

Abstract

Reported here are seven patients who had severe osteogenesis imperfecta, and who were extremely fragile, with three having cardiac disease. Their age ranged between 8 and 35 months. All seven patients had unbraceable deformities. Twenty five long bones, including 14 tibiae, 10 femora, and 1 ulna underwent percutaneous intramedullary fixation. Four patients had all four lower extremity long bones operated on simultaneously. Followup was 2 to 11 years. There were no neurologic or vascular complications, compartment syndromes, growth plate problems, or transfusion requirements. All bones healed. One patient had migration of a femoral pin into the knee joint. After bracing, all patients were able to sit; five were able to stand and eventually walk. All living patients were able to be sustained until successful definitive long bone fixation could be accomplished, approximately 2 years or longer after this initial procedure. With this safe, reproducible method, early stable fixation can be provided to patients too young and/or too sick for definitive or extensive open surgery. Future treatment regimens are not compromised.