Tongue reduction for macroglossia in Beckwith Wiedemann syndrome: review and application of new technique

Abstract

Beckwith Wiedemann syndrome (BWS) is a rare, congenital overgrowth disorder that is characterized by macroglossia, anterior abdominal wall defects, visceromegaly, gigantism, and neonatal hypoglycaemia. Macroglossia may contribute to anterior open bite malocclusion with prognathism, speech articulation disturbances, drooling and the perception of intellectual disability. It was the purpose of this study to review a series of BWS patients who underwent surgical reduction of the tongue by a modified technique with respect to aesthetic and functional outcomes. Seven BWS patients, age 6 months to 21 months, had a 'stellate/anterior wedge' reduction with an anterior rotation flap and were followed up from 4 months to 9 years postoperatively. Assessment of aesthetics together with tongue morphology and mobility were recorded and a postoperative speech evaluation was performed. Minor contour deformities were present in two patients during function but all parents were satisfied with the results. The speech pathology assessment results indicated positive outcomes for speech, oral structure and function, and feeding for all children assessed. This modified technique allows for an adequate reduction of tongue volume with conservation of motor and sensory function as well as preservation of anatomical contour.