Surgical management of long bone pseudarthrosis with severe limb length discrepancy in osteogenesis imperfecta.

Abstract

We report a three-stage approach in managing an 18-year-old boy with osteogenesis imperfecta who had pseudarthrosis of the left femur with 150° anterior angulation and 9.5 cm shortening. Severe long bone deformity and pseudarthrosis in osteogenesis imperfecta are difficult to manage because of the relative shortening of the surrounding soft tissue and bone fragility. Our staged approach included excision of the pseudarthrosis, gradual soft tissue lengthening by external fixator, and a modified Sofield-Millar operation, to achieve union, deformity, and limb length correction without compromising much of the bone length. The patient was able to walk with support within 3 months and independently at 1 year. In this report, the management of the nonunion in osteogenesis imperfecta is discussed, with a focus on its difficulties and the management principles.