Anterior "W" Tongue Reduction for Macroglossia in Beckwith-Wiedemann Syndrome.

ABSTRACT

INTRODUCTION: Macroglossia occurs in 80% to 99% of patients with Beckwith-Wiedemann syndrome (BWS) and a variety of surgical techniques for tongue reduction are offered by surgeons. The purpose of this study is to evaluate the postoperative outcomes of the anterior "W" tongue reduction technique in patients with BWS. METHODS: A retrospective review was conducted of all patients diagnosed with BWS that underwent an anterior "W" tongue reduction for macroglossia in the past 7 years, performed by 2 surgeons. Demographics, procedural characteristics, perioperative outcomes, and complications were assessed. RESULTS: A total of 19 patients met inclusion criteria consisting of 8 male and 11 female patients. The mean age at the time of surgery was 405 days, mean surgeon operating time was 1.06 h, and mean length of follow-up was 467 days. Postoperative oral competence was observed in 100% of patients. There was no reported history of sleep apnea or airway compromise. Speech delay was seen in 4 patients pre- and postoperatively. Feeding issues decreased from 7 patients preoperatively to 1 patient postoperatively. Preoperative prevalence of class III malocclusion (53%) and isolated anterior open bite (26%) decreased postoperatively to 37% and 16%, respectively. The only reported complications were superficial tip wound dehiscence in 3 patients treated with nystatin antifungal therapy. None of the patients required revisional surgery. CONCLUSION: Patients treated with the anterior "W" tongue reduction technique had low rates of perioperative complications and significant improvements in oral competence. Anterior "W" tongue reduction is safe and effective for the correction of macroglossia in patients with BWS.