Intramedullary osteosynthesis results in patients with osteogenesis imperfecta

INTRAMEDULLARY OSTEOSYNTHESIS RESULTS IN PATIENTS WITH OSTEOGENESIS IMPERFECTA Osteogenesis Imperfecta is a disorder of connective tissue characterized by long bone fractures and deformities. It's the most common cause of genetic osteoporosis. The basic defect is the lack of qualitative and quantitative type I collagen. The main Orthopaedic aim is the treatment and prevention of deformity and fractures. The main surgical techniques for the treatment of osteogenesis imperfecta essentialy based on "multiple osteotomies and reconstruction of long bones with intramedullary nail fixation " which known as shish-kebab osteotomy. In this retrospective study, 12 surgically treated patients for osteogenesis imperfecta were evaluated between the years 2006-2011. The mean age of the patients was 7 years (Range: 6-16). Total of 29 bones, including the femur and tibia underwent surgical treatment. Only one K-wire was used for the tibia and two K-wires were used for the femur. Mean follow-up period was 3.18 years. The revision rates were 42% for femoral surgery, 62% for tibial surgery and 48% for totally. The complication rates were 39% for femur, 46% for tibia and the overall complication rate was 41%. As a result, we believe that this surgical technique is convenient, accessible, inexpensive, safe and effective method for the treatment of Osteogenesis Imperfecta. Key Words: Osteogenesis Imperfecta, shish kebab osteotomy, fracture, deformity