前“W”舌缩小治疗Beckwith-Wiedemann综合征大舌缺失。

Bar Y Ainuz, Emily L Geisler, Rami R Hallac, Jeyna K Perez, James R Seaward, Alex A Kane
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引用次数: 2

摘要

导语:80% - 99%的贝克威氏综合征(BWS)患者会出现大舌缺失,外科医生可提供多种舌缩手术技术。本研究的目的是评估前路“W”舌复位技术在BWS患者中的术后效果。方法:回顾性分析2位外科医生在过去7年中为治疗大舌失联而行前“W”舌复位的所有BWS患者。评估人口统计学、手术特点、围手术期结局和并发症。结果:19例患者符合纳入标准,其中男性8例,女性11例。手术时平均年龄405天,平均手术时间1.06 h,平均随访时间467天。100%的患者术后口腔功能正常。无睡眠呼吸暂停或气道损害史。4例患者术前和术后出现语言迟缓。进食问题从术前的7例减少到术后的1例。术前III类错牙合发生率(53%)和孤立前开咬发生率(26%)在术后分别降至37%和16%。3例患者经制霉菌素抗真菌治疗后,仅报告有浅表尖端创面裂开。没有患者需要翻修手术。结论:采用前路“W”形舌复位术患者围手术期并发症发生率低,口腔功能明显改善。前路舌形“W”形矫治BWS患者巨舌畸形安全有效。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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.

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