Long-term outcomes of surgical tongue reduction in Beckwith-Wiedemann syndrome.
BACKGROUND
Surgical tongue reduction is often performed in Beckwith-Wiedemann syndrome when macroglossia results in abnormal tongue function or cosmesis; however, no published studies have examined the long-term outcomes of this procedure.


METHODS
Patients older than 18 years with Beckwith-Wiedemann syndrome who had previously undergone surgical tongue reduction at the Royal Children's Hospital in Melbourne underwent assessment of speech and tongue function, mobility, sensation, and cosmesis. These assessments were performed by questionnaire on 11 subjects and by formal medical and speech pathology review in four of these 11 subjects.


RESULTS
Eleven patients aged 19 to 31 years completed responses by means of questionnaire. The most common self-reported abnormalities were continued disproportionate tongue bulk (91 percent), abnormal tongue appearance (82 percent), specific speech sound errors (73 percent), and a short tongue tip (55 percent). Formal assessments investigated speech, swallowing, taste, and tongue mobility in four quite different subjects. One of these four subjects had no detectable deficits in tongue function. All 11 patients were of normal intelligence and did not report significant difficulties in their day-to-day life that were attributable to their macroglossia or tongue reduction surgery.


CONCLUSIONS
Pediatric patients with symptomatic macroglossia requiring surgical tongue reduction may not achieve complete normality in tongue function and appearance in adulthood. It is important that surgical tongue reduction addresses the global nature of the macroglossia and aims to retain a tapered tongue tip with length sufficient to permit normal tongue movements.
Tomlinson
Jillian K
JK
Department of Plastic and Maxillofacial Surgery, Royal Children's Hospital, Parkville, Victoria 3052, Australia.
Morse
Sue A
SA
Bernard
Simon P L
SP
Greensmith
Andrew L
AL
Meara
John G
JG
D000293:Adolescent; D000328:Adult; D001506:Beckwith-Wiedemann Syndrome / Q000150:complications*; D005260:Female; D005500:Follow-Up Studies; D005927:Glossectomy; D006801:Humans; D008260:Macroglossia / Q000150:complications / Q000601:surgery*; D008297:Male; D017060:Patient Satisfaction