在贝克维思-维德曼综合征中保守地管理巨
Dominic J Romeo1, Connor S Wagner1, Manisha Banala1
1Division of Plastic, Reconstructive, and Oral Surgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Pediatrics
|March 11, 2025
概括
在贝克威特-维德曼综合征 (BWS) 中轻度至中度巨的非手术治疗显示出良好的结果. 阻塞性睡眠呼吸暂停和舌头大小通常会随着孩子的成长自然而然地改善.
科学领域:
- 遗传学和发育生物学
- 儿科医学 儿科医学
- 耳鼻喉科 耳鼻喉科 耳鼻喉科
背景情况:
- 贝克维特-维德曼综合征 (BWS) 经常出现巨,影响生活质量.
- 虽然严重的大光症往往需要手术,但轻度至中度病例的自然史在非手术治疗中得到了解较少.
- 保守的管理是BWS中较不严重的光症的常见情况,但结果需要进一步的表征.
研究的目的:
- 描述BWS患者在没有早期手术干预的情况下管理的巨的自然史.
- 评估BWS.中的轻度至中度巨的保守管理的有效性.
- 为了将临床严重程度,睡眠呼吸暂停和BWS患者的治疗结果相关联起来.
主要方法:
- 追溯对BWS患有巨症的患者的回顾 (2004-2024年).
- 保守的管理定义为在生命的前三年内不进行手术.
- 使用BWS指数的宏光度 (BIG) 尺度评估的表型严重程度;分析了多睡眠学数据.
主要成果:
- 在320名BWS患者中,231名 (72.2%) 接受了保守的治疗.
- 增加BIG分数与增加阻塞性睡眠呼吸暂停严重程度和手术发生率相关.
- 保守管理的患者在2.2-2.6岁时,阻塞性呼吸暂停-缺氧指数和BIG得分显著改善.
结论:
- 非手术治疗在头三年内与BWS中轻度和中度巨的良好结果有关.
- 巨和阻塞性睡眠呼吸暂停通常会随着面部骨架的生长而消失或改善.
- 在BWS中,应考虑对轻度至中度的光症进行保守的治疗,以便自然改善.
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