Complete and isolated congenital aglossia: case report and treatment of sequelae using rapid prototyping models

Aglossia is a rare anomaly caused by failed embryogenesis of the lateral lingual swellings and tuberculum impar from the fourth to eighth gestational weeks. Most cases of aglossia and hypoglossia reported in the literature were associated with limb deformities, cleft palate, deafness, situs inversus, and several syndromes, such as Moebius, Pierre Robin, and Hanhart. This report describes the case of a 14-year-old girl with complete aglossia. As the tongue plays an important role in facial growth, this patient had dentofacial deformities that affected the mandible in particular. She also had severe malocclusion and agenesis of permanent mandibular incisors. Thyroid dysfunction, recently associated with aglossia, was not observed. The use of rapid prototyping models of the jaws as an aid to osteogenic distraction of the mandibular symphysis is also described.