A Comparison of Surgical Techniques for Macroglossia in Beckwith-Wiedemann Syndrome

Background: Tongue reduction surgery can improve symptomatic macroglossia in Beckwith-Wiedemann syndrome (BWS), but there is no consensus on the optimal surgical technique. This study assesses trends and outcomes of several tongue reduction techniques for treating macroglossia in BWS. Using perioperative and polysomnographic data, we present our institution’s rationale for increasingly adopting the peripheral resection technique. Methods: Medical records of patients with BWS seen from 12/2004 to 6/2024 were reviewed for molecular diagnosis, surgical history, polysomnographic findings, and perioperative complications. Results: One hundred and three patients underwent 114 tongue reductions at a median age of 1.3 (interquartile range: 0.4-2.8) years. Use of the peripheral resection technique increased from 10% of all tongue reductions before 2019 to 76% after 2019 (p<0.05). Compared to the W-plasty with keyhole approach, the peripheral resection had shorter operative times (29.0 (24.0-34.0) minutes vs. 35.0 (28.0-39.0) minutes, p<0.05), higher incidence of immediate extubation (51.7% vs. 25.0%), shorter intubation periods (0 (0-2.02) days vs. 5.0 (0-6.0) days, p<0.01), shorter pediatric intensive care unit admissions (3.0 (1.0-10.0) days vs. 7.0 (3.0-20.0) days, p=0.042), and shorter hospital stays (4.0 (2.0-10.0) vs 11.0 (5.0-19.0) days, p=0.005). Improvements in polysomnography and volume of resected tissue were similar between the peripheral and W-plasty approaches (p>0.05). The anterior wedge (22.2%) and W-plasty (15.6%) had more complications than the peripheral resection (6.6%; p=0.351). Conclusion: The peripheral resection appears non-inferior to the W-plasty and anterior wedge approaches in treating symptomatic macroglossia in Beckwith-Wiedemann syndrome, and this study documents our institutional transition towards increasingly adopting this technique.