title: Long-term outcomes of surgical tongue reduction in Beckwith-Wiedemann syndrome.
abstract: 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.
authors.affiliation: Department of Plastic and Maxillofacial Surgery, Royal Children's Hospital, Parkville, Victoria 3052, Australia.
authors.forename: Jillian K
authors.initials: JK
authors.lastname: Tomlinson
authors.forename: Sue A
authors.initials: SA
authors.lastname: Morse
authors.forename: Simon P L
authors.initials: SP
authors.lastname: Bernard
authors.forename: Andrew L
authors.initials: AL
authors.lastname: Greensmith
authors.forename: John G
authors.initials: JG
authors.lastname: Meara
mesh_terms: 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