title: 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.
authors.affiliation: Orthopaedic Surgery Service, Walter Reed Army Medical Center, Washington, DC 20307-5001.
authors.forename: K A
authors.initials: KA
authors.lastname: McHale
authors.forename: J J
authors.initials: JJ
authors.lastname: Tenuta
authors.forename: L L
authors.initials: LL
authors.lastname: Tosi
authors.forename: D W
authors.initials: DW
authors.lastname: McKay
mesh_terms: D000203:Activities of Daily Living; D001858:Bone Nails; D002675:Child, Preschool; D005269:Femur / Q000601:surgery; D006801:Humans; D007223:Infant; D010013:Osteogenesis Imperfecta / Q000000981:diagnostic imaging / Q000534:rehabilitation / Q000601:surgery*; D011187:Posture; D011859:Radiography; D013977:Tibia / Q000601:surgery; D014457:Ulna / Q000601:surgery