Tongue Reduction Surgery Improves Mandibular Prognathism in Beckwith-Wiedemann Syndrome Without Compromising Tongue Function
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Objectives. This study evaluated the surgical outcomes of patients with Beckwith-Wiedemann syndrome who underwenttongue-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 whosemacroglossia was surgically treated. Demographic data, symptoms and signs, and intraoperative and postoperativesurgical 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-rayswere 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 mediansurgical time was 98±31.45 minutes, and the median duration of the postoperative intensive care unit stay was3.81±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 underwentpostoperative 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 themandibular symphysis) on plain X-rays showed a significant decrease in the postoperative SNB angle (P <0.001) anda 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 withBeckwith-Wiedemann syndrome. In addition, it improves mandibular prognathism in young Beckwith-Wiedemannsyndrome patients with macroglossia.
Abstract
Objectives. This study evaluated the surgical outcomes of patients with Beckwith-Wiedemann syndrome who underwenttongue-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 whosemacroglossia was surgically treated. Demographic data, symptoms and signs, and intraoperative and postoperativesurgical 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-rayswere 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 mediansurgical time was 98±31.45 minutes, and the median duration of the postoperative intensive care unit stay was3.81±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 underwentpostoperative 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 themandibular symphysis) on plain X-rays showed a significant decrease in the postoperative SNB angle (P <0.001) anda 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 withBeckwith-Wiedemann syndrome. In addition, it improves mandibular prognathism in young Beckwith-Wiedemannsyndrome patients with macroglossia.
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