Isolated hypoglossia: Oromandibular Limb Hypogenesis Syndrome Type 1 A - A Rare Case Report.

Abstract

Hypoglossia is a rare developmental anomaly of tongue. It is usually associated with various syndromes and other anomalies. Most common association of hypoglossia is with limb deformity and these disorders are collectively grouped as Oro Mandibular Limb Hypogenesis (OLHS) Syndrome. It represents a spectrum of disorders and cases with deviation from the original classification has been reported. Isolated hypoglossia without limb deformity is very rare and has been classified as OLHS Type 1 A by Hall. Features associated with this disorder is hypoplastic mandible, absence of mandibular incisors, intra oral bands and marked enlargement of sublingual muscular ridges and glands. This article reports a case of 22 years old female patient with isolated hypoglossia. Patient reported with the chief complain of irregularly placed teeth. Extra oral finding revealed a convex profile with retrognathic mandible. Intraoral examination revealed crowding in teeth with a constricted maxillary and mandibular arch. A small rudimentary tongue with reduced range of movement was seen. However no abnormalities of the extremities was seen. Key words:Hanhart syndrome, Hypoplastic mandible, Oligodontia, Small tongue.