Abstract

Background: Macroglossia is a term used to describe a large tongue which protrudes outside of the mouth while in a resting position. It is relatively an uncommon condition in children. True macroglossia; congenital or acquired; includes both muscular hypertrophy and tissue infiltration. Congenital macroglossia commonly presents as part of a syndrome, or idiopathic muscular hypertrophy which is a rare entity of true tongue enlargement without any underlying etiology. It is responsible for many functional and psychological disorders. The current surgical approaches emphasise protecting the neurovascular bundles, retaining sufficient length, and preserving lingual movement. The keyhole technique combines both an anterior wedge resection and a central tongue reduction. This debulks the tongue significantly while protecting the lateral neurovascular bundles.

Case Presentation: A five-year-old boy presented with congenital macroglossia due to idiopathic muscular hypertrophy. Other aetiologies, especially syndromes like Beckwith-Widemann syndrome Trenaunay Klipper syndrome and Down syndrome or vascular anomalies, like venous malformations were excluded. Macroglossia was surgically corrected by the keyhole technique. Functional and aesthetic results were excellent at rest and during mobility of the tongue.

Conclusions: Congenital macroglossia requiring reduction glossoplasty is relatively rare and these procedures are, therefore, uncommonly performed. Regardless of its rarity, it can be life saving for some infants and children, and life altering for the others. Reduction glossectomy can greatly enhance the functional and aesthetic results of macroglossia.