Surgery in osteogenesis imperfecta

An analysis of 266 operations on 63 patients with osteogenesis imperfecta, mostly type III and IV, showed that about half had corrections of skeletal deformities and internal splinting with intramedullary stainless steel rods. The remaining operations were for other orthopaedic procedures as well as non-orthopaedic procedures such as hernia repairs. There were no major anaesthetic or post-operative complications. Mild pyrexia was common but without the features of malignant hyperpyrexia. Correction of long deformities and internal splintage of the bones with non-expanding rods effectively reduced the fracture rate but deformities and fractures recurred as the epiphyses grew off the ends of the rods. Expanding rods provided continuing splintage during growth but could not be used in children with very narrow or very fragile bones.