The Hip in Osteogenesis Imperfecta

Osteogenesis imperfecta (OI) can cause fractures and progressive deformity of the bones of the pelvis and hip joint in addition to fractures of the long bones. In children with moderate to severe forms of the disease, coxa vara and acetabular protrusion occur quite commonly as do upper femoral shaft deformities and femoral neck fractures. A small subset of patients with the C-propeptide mutation will have an increased risk of hip dysplasia and dislocation as well. This chapter describes the clinical presentations, pathophysiology and treatment of coxa vara, acetabular protrusion, developmental hip dislocation, proximal femoral fractures and deformity in children with OI.