Tongue Reduction Surgery Improves Mandibular Prognathism in Beckwith-Wiedemann Syndrome Without Compromising Tongue Function

Objectives. This study evaluated the surgical outcomes of patients with Beckwith-Wiedemann syndrome who underwent tongue-reduction surgery and analyzed whether the malocclusion and mandibular prognathism caused by macroglos-sia could be improved. Methods. A retrospective medical record review was performed for 11 patients with Beckwith-Wiedemann syndrome whose macroglossia was surgically treated. Demographic data, symptoms and signs, and intraoperative and postoperative surgical outcomes were evaluated. Surgery was performed by a single surgeon using the “keyhole” technique, involv-ing midline elliptical excision and anterior wedge resection. Preoperative and postoperative plain skull lateral X-rays were evaluated to assess prognathism improvement. Results. The median age at the time of surgery was 35.09 months, and the ratio of males to females was 4:7. The median surgical time was 98±31.45 minutes, and the median duration of the postoperative intensive care unit stay was 3.81±2.4 days. There were no airway complications. Two patients (18.2%) had postoperative wound dehiscence; however, there was no nerve damage, recurrence, or other complications. Among the five patients who underwent postoperative speech evaluation, all showed normal speech development, except one patient who had brain dysfunc-tion and developmental delay. Measurements of the A point-nasion-B point (ANB) angles and sella-nasion-B point (SNB) angles (point A is the most concave point of the anterior maxilla; point B is the most concave point on the mandibular symphysis) on plain X-rays showed a significant decrease in the postoperative SNB angle (P<0.001) and a significant increase in the ANB angle (P<0.011). Conclusion. Tongue-reduction surgery is an effective and safe technique for severe forms of macroglossia associated with Beckwith-Wiedemann syndrome. In addition, it improves mandibular prognathism in young Beckwith-Wiedemann syndrome patients with macroglossia.