Chopra Meera, Siu Jennifer M, Sell Erick, Summers Jane, Chiang Jackie, Propst Evan J, Pankiv Evelina, Wolter Nikolaus E
Department of Otolaryngology - Head and Neck Surgery, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
Division of Neurology, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada.
J Otolaryngol Head Neck Surg. 2025 Jan-Dec;54:19160216251364759. doi: 10.1177/19160216251364759. Epub 2025 Aug 8.
ImportanceAngelman syndrome is a rare genetic disorder characterized by developmental delay, sleep disturbances, and a happy demeanor. Tonsillectomies are common procedures for individuals with Angelman syndrome, and their postoperative recovery may be complicated by challenging pain assessments, respiratory complications, or feeding difficulties. Inappropriate laughing may mask perioperative pain and anxiety.ObjectiveThe objective of this study is to review postoperative outcomes and complications of children with Angelman syndrome undergoing tonsillectomy.Methods (Design, Setting, Participants, Intervention, Measures)We conducted a retrospective review of patients with Angelman syndrome undergoing tonsillectomies from 2000 to 2024 in a quaternary pediatric hospital. Demographic, clinical, and surgical outcome variables were collected.ResultsTwelve children with Angelman syndrome underwent tonsillectomy: 7 for sleep-disordered breathing, 4 for sialorrhea, and 1 for recurrent tonsillitis. Median (IQR) duration of stay was 4.0 (3.0-5.3) days. The most common reason for prolonged hospital stay was inadequate oral intake. Nine (75.0%) children experienced postoperative complications, most frequently pooling of secretions and oxygen desaturations. Three children (25.0%) experienced severe postoperative complications, including 1 opioid overdose, 1 respiratory distress, and 1 aspiration pneumonia. Two patients were readmitted to the hospital: 1 for irregular breathing and poor pain control, and 1 for epistaxis.ConclusionThe postoperative course following tonsillectomy in children with Angelman syndrome can be complicated by a prolonged recovery, inadequate pain control, opioid toxicity, respiratory complications, and poor oral intake. Caregiver input on pain behavior is critical to develop an effective postoperative management strategy.RelevanceBased on our results and a literature review, we have created recommendations for post-tonsillectomy care in children with Angelman syndrome.
重要性
安吉尔曼综合征是一种罕见的遗传性疾病，其特征为发育迟缓、睡眠障碍和愉悦的举止。扁桃体切除术是安吉尔曼综合征患者常见的手术，其术后恢复可能因疼痛评估困难、呼吸并发症或喂养困难而变得复杂。不适当的笑可能掩盖围手术期的疼痛和焦虑。
目的
本研究的目的是回顾接受扁桃体切除术的安吉尔曼综合征患儿的术后结局和并发症。
方法（设计、设置、参与者、干预、测量）
我们对2000年至2024年在一家四级儿科医院接受扁桃体切除术的安吉尔曼综合征患者进行了回顾性研究。收集了人口统计学、临床和手术结局变量。
结果
12名安吉尔曼综合征患儿接受了扁桃体切除术：7例因睡眠呼吸障碍，4例因流涎，1例因复发性扁桃体炎。中位（四分位间距）住院时间为4.0（3.0 - 5.3）天。住院时间延长的最常见原因是经口摄入量不足。9名（75.0%）患儿出现术后并发症，最常见的是分泌物积聚和氧饱和度下降。3名（25.0%）患儿出现严重术后并发症，包括1例阿片类药物过量、1例呼吸窘迫和1例吸入性肺炎。2例患者再次入院：1例因呼吸不规则和疼痛控制不佳，1例因鼻出血。
结论
安吉尔曼综合征患儿扁桃体切除术后的病程可能因恢复时间延长、疼痛控制不佳、阿片类药物毒性、呼吸并发症和经口摄入量不足而变得复杂。护理人员对疼痛行为的反馈对于制定有效的术后管理策略至关重要。
相关性
基于我们的研究结果和文献综述，我们为安吉尔曼综合征患儿扁桃体切除术后的护理制定了建议。
