TREATMENT OF THE CRANIOFACIAL COMPLICATIONS OF BECKWITH-WIEDEMANN SYNDROME

Variable degrees of macroglossia have been noted in patients with Beck with-Wiedemann syndrome. Past studies have shown that a major effect o f the macroglossia is protrusion of dentoalveolar structures, resultin g in an anterior open bite and a prognathic mandibular appearance seco ndary to an abnormally obtuse genial angle and increased effective man dibular length. In our series of 11 Beckwith-Wiedemann syndrome patien ts we have observed that early correction of the macroglossia by means of partial glossectomy has resulted in decreased anterior open bite a nd mandibular prognathism as compared with patients managed conservati vely.