Bariatric Surgery in Patients With Prader-Willi Syndrome.

Maximilian Herbert Dressler, Jong-Ho Choi, Kyoyoung Park, Seong-Ho Kong, Do Joong Park, Hyuk-Joon Lee
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Abstract

Prader-Willi Syndrome (PWS) is a genetic disorder characterized by insatiable hyperphagia, resulting in severe, early-onset obesity that is often refractory to conventional management. The associated comorbidities and reduced life expectancy in PWS present a significant therapeutic challenge. This review synthesizes the existing literature on the controversial role, outcomes, and complexities of bariatric surgery in patients with PWS. In recent decades, bariatric surgical techniques have evolved from malabsorptive or restrictive operations to modern procedures such as sleeve gastrectomy (SG) and Roux-en-Y gastric bypass (RYGB). We present a 21-year-old male's SG case to illustrate key themes. Published series from specialized, high-volume centers demonstrate that SG and RYGB yield significant short- to mid-term weight loss and metabolic improvement with an acceptable perioperative risk profile. However, the fundamental challenge in PWS remains the persistent, inherent hyperphagia. As exemplified by the presented case and supported by the broader literature, this insatiable hunger severely limits long-term surgical success and frequently leads to substantial weight regain. Maintaining durable success requires rigorous, lifelong external dietary supervision and intensive multidisciplinary support. Moreover, ethical considerations, particularly regarding informed consent and procedural choice, complicate the application of bariatric surgery in this population. Bariatric surgery should therefore be considered an adjunctive tool rather than a definitive cure for PWS-related obesity. Its use must be reserved for carefully selected individuals who have severe comorbidities and robust, long-term support structures in place. This perspective highlights the critical need for ongoing research into optimal patient selection, procedural approaches, and adjuvant therapies to improve durable outcomes.

Abstract Image

Abstract Image

Abstract Image

Prader-Willi综合征患者的减肥手术。
普瑞德-威利综合征(PWS)是一种以贪得无厌的贪食为特征的遗传性疾病,导致严重的早发性肥胖,通常难以常规治疗。PWS的相关合并症和预期寿命缩短对治疗提出了重大挑战。这篇综述综合了关于PWS患者减肥手术的争议性作用、结果和复杂性的现有文献。近几十年来,减肥手术技术已经从吸收不良或限制性手术发展到现代手术,如袖式胃切除术(SG)和Roux-en-Y胃旁路术(RYGB)。我们提出一个21岁男性的SG病例来说明关键主题。来自专业、高容量中心的已发表的系列研究表明,SG和RYGB可显著减轻中短期体重和改善代谢,且围手术期风险可接受。然而,PWS的根本挑战仍然是持续的、固有的贪食。正如本例所示和广泛文献所支持的那样,这种无法满足的饥饿严重限制了手术的长期成功,并经常导致大量体重反弹。维持持久的成功需要严格的、终生的外部饮食监督和密集的多学科支持。此外,伦理方面的考虑,特别是关于知情同意和程序选择,使减肥手术在这一人群中的应用复杂化。因此,减肥手术应被视为一种辅助工具,而不是治疗pws相关肥胖的最终方法。它的使用必须谨慎选择那些有严重的合并症和健全的长期支持结构的个体。这一观点强调了对最佳患者选择、手术方法和辅助治疗的持续研究的迫切需要,以改善持久的结果。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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