A Case of Agnathia Treated with Commissuroplasty

Abstract

　　Agnathia is a congenital anomaly of the craniofacial region characterized by mandibular deficiency or dysplasia, tongue deficiency, and microstomia. It is caused by a defect in the development of branchial arches at around 4 weeks of gestation. It is an extremely rare disease and has a poor prognosis. It is sometimes associated with holoprosencephaly and situs inversus. Herein, we describe a case of agnathia without serious complications, in which we treated the microstomia with commissuroplasty with good results. The case was a 6-year-old male. He had agnathia and microstomia from birth. No other external congenital anomalies were found. A commissuroplasty was performed for microstomia, and the width of the mouth commissure was widened from about 13 mm preoperatively to about 30 mm postoperatively. The patient achieved adequate mouth opening and improved oral hygiene. The postoperative course was uneventful. Although functional reconstruction is difficult, a commissuroplasty for microsomia associated with agnathia may be effective in improving oral hygiene and cosmetics.