To determine the effects of osteogenesis imperfecta and its treatment on
adult life, thirty-one patients with osteogenesis imperfecta were examined
at an average of nineteen years postoperatively. Seventy-two per cent of
the operations that had been performed on these patients in childhood
consisted of multiple osteotomies with intramedullary fixation to reduce
fracture frequency and prevent bowing of the lower limbs. Only eight
patients had remained non-ambulatory, seven of whom had severe long-bone
deformity. Based on the study of these patients, we suggest that the
severity of diaphyseal tapering and of disease are related; that scoliosis
is frequent in adults with osteogenesis imperfecta; that the improvement
gained at operation is maintained and enhances ambulation; and that as
adults, these patients are generally very productive and socially adaptable
individuals.