SEVERE CONGENITAL HYPOPLASIA OF TONGUE AND ABNORMAL SHAPE OF SOFT PALATE AND TONSILLAR PILLARS

This paper describes an 8-year-old boy who presented with the very rar e condition of congenital right-sided aglossia. The left side of tongu e was hypoplastic and tethered to the floor of the mouth. The soft pal ate was short and almost absent on the right side with abnormal positi oning of the right anterior and posterior tonsillar pillars; this situ ation resulted in significant velopharyngeal incompetence (VPI). The l eft mandible was slightly hypoplastic with anodontia posterior to the first premolar tooth. There has been no previous report of a similar d efect. The possible differential diagnoses related to the abnormality are discussed. The correction of the VPI is presented to demonstrate t he versatility of the sphincter pharyngoplasty.