Title

Percutaneous intramedullary fixation of long bone deformity in severe osteogenesis imperfecta

Document Type

Journal Article

Publication Date

1-1-1994

Journal

Clinical Orthopaedics and Related Research

Issue

305

DOI

10.1097/00003086-199408000-00030

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.

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