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病例报告:经口内镜下肌切开术治疗儿童急性全自主神经功能不全相关的远端食管痉挛

Case report: Peroral endoscopic myotomy for acute pandysautonomia-associated distal esophageal spasm in a child.

作者信息

Zhang Hanhua, Chi Biyun, Wang Fengfan, Shao Pei, Liu Huanyu, Fang Ying

机构信息

Department of Gastroenterology, Xi'an Children's Hospital, Xi 'an, China.

Xi'an Medical University, Xi'an, China.

出版信息

Front Pediatr. 2023 Jan 17;10:935915. doi: 10.3389/fped.2022.935915. eCollection 2022.

Abstract

Acute pandysautonomia-associated distal esophageal spasm is a rare disease with an unclear etiology. Here, we describe a 12-year-old boy with an acute pandysautonomia-associated distal esophageal spasm who was treated using a peroral endoscopic myotomy (POEM). The patient's clinical features included recurrent dysphagia, nausea, vomiting, growth retardation, and signs of autonomic nerve dysfunction (e.g., a decreased production of tears and sweat, and an increased production of saliva). Signs of the distal esophageal spasm were visible in upper gastrointestinal radiography, endoscopy, and high-resolution esophageal manometry. After the POEM, the patient exhibited improvements in nausea and vomiting, and his dysphagia symptoms were relieved by the 6-month follow-up visit. However, the patient's neurological problems persisted. The satisfactory short-term clinical responses in our patient suggest that POEM is feasible, safe, and effective for the treatment of acute pandysautonomia-associated distal esophageal spasms in children.

摘要

急性全自主神经功能不全相关的远端食管痉挛是一种病因不明的罕见疾病。在此,我们描述一名12岁患有急性全自主神经功能不全相关远端食管痉挛的男孩,他接受了经口内镜下肌切开术(POEM)治疗。患者的临床特征包括反复吞咽困难、恶心、呕吐、生长发育迟缓以及自主神经功能障碍的体征(如泪液和汗液分泌减少、唾液分泌增加)。在上消化道造影、内镜检查和高分辨率食管测压中可见远端食管痉挛的体征。POEM术后,患者的恶心和呕吐症状有所改善,吞咽困难症状在6个月的随访中得到缓解。然而,患者的神经问题仍然存在。我们患者令人满意的短期临床反应表明,POEM对于治疗儿童急性全自主神经功能不全相关远端食管痉挛是可行、安全且有效的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea5a/9887313/db50f0b5cf1d/fped-10-935915-g001.jpg

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