Aglossia (aglossia congenita) is a congenital defect resulting in a partial development or complete absence of a tongue. Aglossia is commonly associated with craniofacial and limb defects (Adactylia syndrome) and is thought to belong to a family of oromandibular limb hypogenesis syndrome or OLHS. It is believed to be caused by heat-induced vascular disruption near the fourth week of embryonic development.
Aglossia congenita (AC), congenital absence of the tongue, is a rare and unusual midline developmental anomaly usually associated with craniofacial and limb defects and has overlapping features with other syndromes. All these syndromes are now believed to belong to a family of oromandibular limb hypogenesis syndrome (OLHS). The incidence of OLHS is very low (1/175,000 live births) and most cases are actually hypoglossal rather than true aglossia.[rx] Isolated aglossia without limb defects is even more rare. This is a rare case of isolated AC, unusual median palatal grooving, and remarkable functional adaptability in a young Indian male patient.