Our Results of Modified Sofield Procedure in Lower Extremity Deformities with Osteogenesis Imperfecta

Introduction: Osteogenesis Imperfecta (OI) is a connective tissue disorder, which is the most common cause of genetic osteoporosis characterized by long bone deformities and fractures. The key defect is the qualitative and quantitative lack of type I collagen. The primary target in these patients is to fix deformities and to prevent fractures. The surgical application is often a basic surgical technique known as “Shish Kebab” osteotomy and mainly based on the principle of multiple osteotomies, fixation, and intramedullary nailing. In the present study, we present our results of the modified Sofield procedure for osteogenesis imperfecta-related lower extremity deformities. Materials and methods: The study retrospectively examined 12 OI patients who were surgically treated between March 2006 and November 2011. The modified Sofield procedure was applied to a total of 29 lower extremity bones including 21 femurs and eight tibias. Double intramedullary K-wire and single K-wire were used in surgeries for the femur and the tibia, respectively. Results: The median age of the patients was 7 (range: 6 to 16) years. The mean follow-up was 3.18 (range: 1 to 5) years. The revision rate was 42.8% for femoral surgery, 62.5% for tibial surgery, and 48.2% for all surgery. The mean time of union was 47.3 (range: 30 to 60) days including 46.3 (range: 30 to 60) days for femoral, and 49.3 (range: 30 to 60) days for tibial surgeries. The mean time from one surgery to another was 58 (range: 4 to 96) months for femoral revision, and the mean time of tibial revision was 27.7 (range: 9 to 60) months. The mean time required for total revision was 28 (range: 4 to 96) months. The complication rate was 39% and 46% for femur and tibia, respectively. The overall complication rate was 41%. Conclusion: Our study results suggest that this method is an effective and reliable method in the surgical treatment of osteogenesis imperfecta, as it is easy to practice and access as well as being cost-effective. Although surgical treatment does not change the patient ambulation, it maintains mobilization, reducing the incidence of fractures and deformities. The double-rod application used at the femur decreases the number of revisions and prolongs the time of revision.