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

김도원; 권성근; 김정규; 허진; 이도영

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자료유형: 학술저널

저자정보: 김도원 (Seoul National University Hospital Seoul Korea) 권성근 (Seoul National University Hospital Seoul Korea / Seoul National University Boramae Medical Center Seoul Korea) 김정규 (Seoul National University Hospital Seoul Korea) 허진 (Seoul National University Hospital Seoul Korea) 이도영 (Seoul National University Boramae Medical Center Seoul Korea)

저널정보: 대한이비인후과학회 Clinical and Experimental Otorhinolaryngology Clinical and Experimental Otorhinolaryngology 제16권 제1호

발행연도: 2023.2

수록면: 67 - 74 (8page)

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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.

#Beckwith-Wiedemann Syndrome #Macroglossia #Prognathism

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