Abstract
We performed corrective multiple osteotomy and intramedullary rod fixation on the bilateral femora and tibiae of a 4-year-old boy with osteogenesis imperfecta (OI) who had marked locomotive disturbance resulting from fragility and deformity of the lower limbs. Nailing was performed with a 3.2mm diameter telescoping rod for the right femur, and with a 3mm diameter Kirschner's wire for the left femur and both tibiae. Forward flexure of the right tibia subsequently recurred, resulting in cutting of the tibia by the Kirschner's wire. Reoperation was thus required. One year and 8 months after the reoperation, the patient is able to maintain a standing position and has acquired some locomotive capability.
Children with OI who often have a history of fractures, tend to limit their movement in order to reduce the possibility of sustaining additional fractures. This results in disuse atrophy and fragility of the extremities, and ultimately severe locomotive disturbance. In the present case, invasive treatment reduced the risk of francture. The patient can now perform exercises including kneeling, and these exercises can be expected to further improve his clinical condition.
