PERCUTANEOUS INTRAMEDULLARY FIXATION OF LONG-BONE DEFORMITY IN SEVEREOSTEOGENESIS IMPERFECTA

Reported here are seven patients who had severe osteogenesis imperfect a, and who were extremely fragile, with three having cardiac disease. Their age ranged between 8 and 35 months. All seven patients had unbra ceable deformities. Twenty five long bones, including 14 tibiae, 10 fe mora, and 1 ulna underwent percutaneous intramedullary fixation. Four patients had all four lower extremity long bones operated on simultane ously, Followup was 2 to 11 years. There were no neurologic or vascula r complications, compartment syndromes, growth plate problems, or tran sfusion requirements. All bones heated. One patient had migration of a femoral pin into the knee joint. After bracing, all patients were abl e to sit; five were able to stand and eventually walk. All living pati ents were able to be sustained until successful definitive long bone f ixation could be accomplished, approximately 2 years or longer after t his initial procedure, With this safe, reproducible method, early stab le fixation can be provided to patients too young and/or too sick for definitive or extensive open surgery. Future treatment regimens are no t compromised.