Anterior “W” Tongue Reduction for Macroglossia in Beckwith-Wiedemann Syndrome

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.

Reduction Glossectomy for Macroglossia in Beckwith-Wiedemann Syndrome: Is Post-Op Intubation Necessary?

Objective: Macroglossia is a characteristic feature of Beckwith-Wiedemann syndrome (BWS), commonly treated with reduction glossectomy to restore form and function. There exists no consensus on the perioperative management of these patients undergoing tongue reduction surgery, including anecdotal information regarding how long postoperative intubation should be maintained. The aim of this study is to evaluate the necessity of prolonged postoperative intubation in patients receiving tongue reduction surgery via the surgical and anesthetic management methods at our center. Design: Retrospective case series. Setting: Institutional care at Level I Children’s Hospital. Participants: All children less than 18 years old with BWS and congenital macroglossia who underwent tongue reduction surgery over 5 consecutive years at our center (N = 24). Interventions: Tongue reduction surgery via the “W” technique. Main Outcome Measures: Success of immediate postoperative extubation and related surgical complications. Results: Immediate, uncomplicated postoperative extubation was successfully performed in all patients who received tongue reduction surgery for congenital macroglossia. Conclusions: Prolonged postoperative intubation for tongue reduction surgery may not be necessary as immediate, uncomplicated postoperative extubation was achieved in 100% of patients who received tongue reduction surgery at our center.