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Z-osteotomy for uniplanar femoral shaft deformity correction in an adolescent with osteogenesis imperfecta.
Osteogenesis imperfecta (OI) is commonly associated with fragility fractures. It is due to abnormality in the quantity and quality of collagen type 1 caused by mutations in COL1A1 and COL1A2 genes. Patients with OI would also have blue sclera, ligament hyperlaxity, dentinogenesis imperfecta, hearing abnormality, and short stature. Surgical management is preferred to conservative treatment in long bone fractures. For malunited fractures, Sofield-Millar or multiple osteotomies at different sites of deformities are performed with additional intramedullary device to stabilize the bone. This is a case of femur fracture with multilevel CORAs in an adolescent patient with post-trauma OI in which z-osteotomies were performed and stabilized with titanium elastic nails resulting in good outcomes clinically and radiologically.
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