ABSTRACT
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.
Subject(s)
Fractures, Bone/etiology , Osteogenesis Imperfecta/surgery , Adolescent , Adult , Child , Child, Preschool , Female , Follow-Up Studies , Fracture Fixation, Intramedullary , Fractures, Bone/surgery , Humans , Infant , Infant, Newborn , Male , Osteogenesis Imperfecta/complications , Osteogenesis Imperfecta/diagnostic imaging , Radiography , Sclera/abnormalities , Scoliosis/complications , Scoliosis/diagnostic imaging , Tibia/diagnostic imagingSubject(s)
Disabled Persons , Hospitals, Special/history , Orthopedics/history , Child , History, 20th Century , Humans , North CarolinaABSTRACT
A rare case of intramedullary lipoma involving the distal femur is presented and discussed in relation to previously reported cases. The 26 previously reported extracranial cases of this bone lesion are briefly reviewed. Emphasis is placed upon establishing a definitive pathologic diagnosis by open biopsy when one is dealing with a large bony lesion that has indefinite radiologic features.
