Treatment of the Craniofacial Complications of Beckwith-Wiedemann Syndrome

Variable degrees of macroglossia have been noted in patients with Beckwith-Wiedemann syndrome. Past studies have shown that a major effect of the macroglossia is protrusion of dentoalveolar structures, resulting in an anterior open bite and a prognathic mandibular appearance secondary to an abnormally obtuse gonial angle and increased effective mandibular length. In our series of 11 Beckwith-Wiedemann syndrome patients we have observed that early correction of the macroglossia by means of partial glossectomy has resulted in decreased anterior open bite and mandibular prognathism as compared with patients managed conservatively.