Abstract
This paper describes a very rare case of congenital aglossia diagnosed as a Hypoglossia-Hypoductylia Syndrome.
The etiology of this anomalie is not clear. However, the intrauterine environmental factors are considered to be more important than the genetical influences. The patient was a 3 year-2 month-old girl with complaints of congenital aglossia and rampant carious teeth who was referred to the Clinic of Pedodontics at Osaka University Dental School Hospital. An evaluation revealed a completely defective tongue, mandibular hypognatia, partial anodontia of permanent teeth, and a high arched palate. The severely retarded development of the mandibular arch did not allow occlusion with the maxillary arch.
Growth of the maxilla seemed to be influenced by the tongue defect. Anomalies of the limbs were not observed, but the patient had complications with a venticular septal defect of the heart and had been admitted for surgery when she was 1 year and 3 months old. She seemed to speak well, appeared intelligent, and adapted well to the prosthesis. In addition, she could distinguish various kinds of tastes.
