Osteogenesis imperfecta and orthognathic surgery: case report with long-term follow-up

Osteogenesis imperfecta (OI) is the product of the abnormal synthesis and/or production of Type I collagen. Successful surgical management of extremity and spinal skeletal problems secondary to OI is documented in the orthopedic literature. Reports of successful facial skeletal surgery in all types of OI are encouraging. The purpose of this paper is to report on the long-term results of an orthognathic surgery patient successfully treated to correct a severe dentofacial deformity. The patient underwent an uncomplicated Le Fort I osteotomy with homologous interpositional bone grafts to advance and inferiorly reposition the maxilla. Clinically, the patient appeared to heal without difficulty, and a stable Class I skeletal and dental relationship was achieved. Nine years after surgery, the patient has a Class I occlusion, with maintenance of his facial height and skeletal relationship. Craniomaxillofacial surgery can be predictably performed in patients with OI as long as the surgeon maintains strict adherence to proper surgical technique and bears in mind the deficiencies of bone density and other possible medical complications.