Macroglossia as a presentation of the Beckwith-Wiedemann syndrome

Plastic surgeons are not infrequently required to reduce the size of a large tongue in a child. Macroglossia is one of the main presenting features of the Beckwith-Wiedemann syndrome. This comprises a spectrum of disorders that includes a high incidence of malignancy. Patients with this syndrome should be identified and carefully assessed to exclude serious complications. This paper reports a series of 30 patients presenting to the Royal Children's Hospital over an 8-year period. An incidence of malignancy of 10 percent is recorded.