Nonoperative Treatment of Osteogenesis Imperfecta

Abstract

The problem of osteoporosis superimposed on the basic collagen defect of osteogenesis imperfecta has been approached by the use of plastic containment orthoses for the lower limbs, in addition to developmentally staged mobility devices that assist early standing and walking. The purpose of forcing early weight-bearing is to provide stress to the lower limb bones in order to minimize osteoporosis, prevent refracture and deformity, and curb subsequent immobilization osteoporosis, thus breaking a vicious cycle. Management goals are based upon adult needs for independence: efficiency in daily living activities and in mobility. These goals were reached in most of our patients via use of plastic orthoses, early weight-bearing, and electrically powered wheelchairs. Manual osteoclasis of the tibia followed by plastic orthoses utilizing principles of fluid compression to support fractured or structurally weak bones appeared successful at the time of follow-up. Intramedullary rodding of the femur was necessary in most of the 12 children with osteogenesis imperfecta congenita. Supplementary plastic orthoses have reduced the refracture rate in both the tibia and the femur. Social integration of the children was reflected by the fact that among the 12 OI congenita cases, ten were attending regular educational institutions. Twelve OI tarda children fared well, all attaining complete independence in daily living, mobility and ambulation. Seven of this group were treated with intramedullary rodding of the femur or tibia and with plastic orthoses. Five patients required no treatment.