腺扁桃体切除术成功治疗普瑞德-威利综合征儿童阻塞性睡眠呼吸暂停

IF 1.2 4区医学 Q3 OTORHINOLARYNGOLOGY

International journal of pediatric otorhinolaryngology Pub Date : 2025-03-13 DOI:10.1016/j.ijporl.2025.112305

Ross Rosen , Jamil Hayden , Abdul Saltagi , Chelsea Cleveland , Todd Otteson , Tekin Baglam

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引用次数: 0

摘要

普瑞德-威利综合征（PWS）是一种罕见的遗传性疾病，可增加儿童阻塞性睡眠呼吸暂停（OSA）的风险，由分泌物粘度增加、颅面异常、张力过低和肥胖共同引起。虽然儿童OSA的一线治疗通常是腺扁桃体切除术，但PWS患者OSA的复杂病理生理可能导致这种治疗的成功率较低。方法根据PWS和OSA的诊断以及腺扁桃体切除术、扁桃体切除术和腺样体切除术的手术干预措施，查询TriNetX数据库中18岁及以下的患者。主要终点是术后6个月排除OSA诊断。没有PWS的儿科患者作为对照。次要终点是对PWS患儿进行常见医疗干预后发生OSA的风险。结果共2163例PWS患者，其中1035例（47%）诊断为OSA。接受手术的PWS患者的总成功率为39.0%，而对照组为79.6% (p <；0.001)。与人口统计学和肥胖状况相匹配的对照组相比，这些手术的总成功率也显著降低(36.8%对82.1%,p <；0.001)。生长激素的使用(RR 1.43, p <；0.001)和睾酮(RR 1.39, p <；0.001)均与OSA风险增加相关。结论硬膜扁桃体切除术治疗PWS患儿阻塞性睡眠呼吸暂停的成功率明显降低。这些患者可能受益于多学科治疗，以治疗他们的阻塞性睡眠呼吸暂停，减轻未经治疗的疾病的影响，进一步的研究确定护理这些患者的最佳做法是必要的。

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Adenotonsillectomy success for treating obstructive sleep apnea in children with Prader-Willi syndrome

Background

Prader-Willi syndrome (PWS) is a rare genetic disorder that can increase risk of pediatric obstructive sleep apnea (OSA), caused by the combination of increased viscosity of secretions, craniofacial abnormalities, hypotonia, and obesity. While first-line treatment of pediatric OSA is typically adenotonsillectomy, the complex pathophysiology of OSA in PWS patients may lead to less success with this therapy.

Methods

The TriNetX database was queried for patients 18 years old or younger based on the diagnoses of PWS and OSA and the surgical interventions of adenotonsillectomy, tonsillectomy, and adenoidectomy. The primary endpoint was the removal of the diagnosis of OSA 6 months postoperatively. Pediatric patients without PWS were used as a control. Secondary endpoints were the risk of OSA with common medical interventions for children with PWS.

Results

A total of 2163 patients were found to have PWS, with 1035 (47 %) diagnosed with OSA. PWS patients undergoing surgery had a total success rate of 39.0 %, compared to 79.6 % in controls (p < 0.001). Total success for these surgeries was also significantly lower compared to controls matched by demographics and obesity status (36.8 % versus 82.1 %, p < 0.001). Use of growth hormone (RR 1.43, p < 0.001) and testosterone (RR 1.39, p < 0.001) were both associated with increased risk of OSA.

Conclusions

Adenotonsillectomy has significantly lower rates of success at treating pediatric OSA in patients with PWS. These patients would likely benefit from multidisciplinary care to treat their OSA and mitigate the effects of untreated disease, and further studies determining best practices for caring for these patients are necessary.

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来源期刊

International journal of pediatric otorhinolaryngology 医学-耳鼻喉科学

CiteScore

3.20

自引率

6.70%

发文量

276

审稿时长

62 days

期刊介绍： The purpose of the International Journal of Pediatric Otorhinolaryngology is to concentrate and disseminate information concerning prevention, cure and care of otorhinolaryngological disorders in infants and children due to developmental, degenerative, infectious, neoplastic, traumatic, social, psychiatric and economic causes. The Journal provides a medium for clinical and basic contributions in all of the areas of pediatric otorhinolaryngology. This includes medical and surgical otology, bronchoesophagology, laryngology, rhinology, diseases of the head and neck, and disorders of communication, including voice, speech and language disorders.