Dentofacial rehabilitation by osteodistraction, augmentation and implantation despite osteogenesis imperfecta.

Osteogenesis imperfecta-- a heritable systemic disorder characterized by enhanced bone fragility-- is frequently associated with a Class III malocclusion and distinct dental disorders. This patient, suffering from a late form of osteogenesis imperfecta, displayed early loss of teeth and severe maxillary hypoplasia. Bone grafting of the alveolar ridge was assumed not to guarantee a neutral basal relation before dental implantation. Due to the risk of atypical fractures conventional orthognathic surgery was excluded in the atrophic maxilla. In contrast to a conventional Le Fort I osteotomy, osteodistraction of the maxilla can be performed omitting the precarious down-fracture procedure. Despite a lack of reports on this technique in patients with osteogenesis imperfecta, dysgnathia was corrected by osteodistraction of the upper jaw. The loss of teeth was treated by augmentation of the alveolar crest using autogenous bone from the iliac crest followed by placement of dental implants. Stable normocclusion of the implant-supported overdentures was achieved without any detectable relapse over 4 years. For the first time it has been demonstrated that advanced surgical techniques like osteodistraction, alveolar crest augmentation and dental implantation can successfully be combined for dentofacial rehabilitation even in patients suffering from osteogenesis imperfecta.