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.
McManamny
D S
DS
Barnett
J S
JS
D001506:Beckwith-Wiedemann Syndrome / Q000150:complications / Q000235:genetics / Q000473:pathology*; D002454:Cell Differentiation; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006984:Hypertrophy / Q000473:pathology; D007223:Infant; D007231:Infant, Newborn; D007668:Kidney / Q000473:pathology; D007680:Kidney Neoplasms / Q000209:etiology; D008260:Macroglossia / Q000150:complications / Q000473:pathology / Q000601:surgery*; D008297:Male; D014059:Tongue / Q000601:surgery*; D009396:Wilms Tumor / Q000209:etiology