Williams-Beuren Syndrome treated with orthognathic surgery and combined partial glossectomy: case report

Background Williams syndrome, also known as Williams-Beuren syndrome (WBS), is a multi-systems, congenital and rare disorder involving the cardiovascular system, connective tissue, and the central nervous system. It is a genetic disorder caused by a hemizygous microdeletion of chromosome 7q11.23. Here we report a case of WBS treated with bimaxillary osteotomies and glossectomy. Case report Orthognatic surgery was undertaken one year after the first diagnosis and the beginning of the orthodontic treatment. The maxilla was advanced at a Le Fort I of about 4 mm and was fixed with two angled plates, one on each side, applied laterally to the pyriform aperture. The lateral part of the maxilla was stabilized with wires. In addition, bilateral mandibular sagittal osteotomies were carried out together with a midline osteotomy. A partial glossectomy was performed. Intermaxillary adaptation was supported by applying soft elastics according to the concept of semi-rigid bone fixation. Two months post-surgery the occlusion was Angle class I with a well defined overbite and overjet. The healing was uneventful. Functional limitations or nerve disturbances did not occur. The miniplates remained in situ. In the case reported the "keyhole" partial glossectomy was performed in combination with the orthognatic surgery. No complication was recorded in the postoperative period and the patient had a successful outcome.