ORTHOGNATHIC SURGERY IN OSTEOGENESIS IMPERFECTA - A CASE-REPORT WITH MANAGEMENT CONSIDERATIONS

Osteogenesis imperfecta is a disease of connective tissues with additi onal metabolic defects. It is associated with recognisable facial disp roportion and sometimes warrants surgical intervention for aesthetic, functional and psychological reasons. A severe case of osteogenesis im perfecta type 3 is presented to illustrate the feasibility of bimaxill ary surgery using a mandibular body step osteotomy and maxillary down grafting at the Le Fort I level. The medical, anaesthetic, surgical an d specific maxillofacial implications of surgery in these patients are discussed.