title: Surgical treatment of macroglossia in patients with Beckwith-Wiedemann syndrome: a 20-year experience and review of the literature.
abstract: Macroglossia is observed in the majority of paediatric patients diagnosed with Beckwith-Wiedemann syndrome and surgical treatment may be indicated. A 20-year retrospective study was performed to evaluate all patients with Beckwith-Wiedemann syndrome who underwent tongue reduction surgery at the authors' institution. A literature review was performed. Surgical treatment was indicated and carried out in 23 patients with a yearly average varying from 0 to 3 subjects. The mean follow-up time was 7 years. Primary indications for surgical treatment of macroglossia included significant tongue protrusion in 30% (n=7), and a combination of clinical problems in 70% (n=16). In all cases, the procedure was performed by the same surgeon using an anterior, V-shaped, wedge resection technique. Two patients had direct postoperative complications. No recurrence of macroglossia was observed in this study. Reported clinical outcome during follow-up demonstrated a satisfactory resting tongue position in all patients. Review of the literature demonstrated variability in surgical indications and techniques, and postoperative outcome. The results of this study indicate that the anterior wedge resection is a simple, effective and safe technique in the surgical treatment of paediatric patients, diagnosed with Beckwith-Wiedemann syndrome, suffering from macroglossia.
authors: Department of Plastic and Reconstructive Surgery, Academic Medical Center, Amsterdam, The Netherlands. d.j.kadouch@amc.uva.nl
authors: D J M
authors: DJ
authors: Kadouch
authors: S M
authors: SM
authors: Maas
authors: L
authors: L
authors: Dubois
authors: C M A M
authors: CM
authors: van der Horst
mesh_terms: D000367:Age Factors; D000402:Airway Obstruction / Q000473:pathology; D001506:Beckwith-Wiedemann Syndrome / Q000601:surgery*; D016063:Blood Loss, Surgical; D002675:Child, Preschool; D003970:Diastema / Q000473:pathology; D055154:Dysphonia / Q000473:pathology; D004435:Eating / Q000502:physiology; D005260:Female; D005500:Follow-Up Studies; D005927:Glossectomy / Q000379:methods; D006801:Humans; D007223:Infant; D008260:Macroglossia / Q000601:surgery*; D008297:Male; D008313:Malocclusion, Angle Class III / Q000473:pathology; D008334:Mandible / Q000254:growth & development; D019647:Oral Surgical Procedures / Q000379:methods; D011183:Postoperative Complications; D012189:Retrospective Studies; D012449:Safety; D012798:Sialorrhea / Q000473:pathology; D012891:Sleep Apnea Syndromes / Q000473:pathology; D013997:Time Factors; D014061:Tongue Habits; D016896:Treatment Outcome