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儿童贲门失弛缓症——临床表现、诊断、长期治疗结果及生活质量

Achalasia in Children-Clinical Presentation, Diagnosis, Long-Term Treatment Outcomes, and Quality of Life.

作者信息

Jarzębicka Dorota, Czubkowski Piotr, Sieczkowska-Gołub Joanna, Kierkuś Jarosław, Kowalski Adam, Stefanowicz Marek, Oracz Grzegorz

机构信息

Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, The Children's Memorial Health Institute, 04-730 Warsaw, Poland.

Department of Pediatric Surgery and Organ Transplantation, The Children's Memorial Health Institute, 04-730 Warsaw, Poland.

出版信息

J Clin Med. 2021 Aug 30;10(17):3917. doi: 10.3390/jcm10173917.

DOI:10.3390/jcm10173917
PMID:34501361
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8432175/
Abstract

BACKGROUND

In spite of the introduction of peroral endoscopic myotomy (POEM), Heller myotomy (HM) remains the mainstay of treatment and the role of pneumatic dilatation (PD) is being debated. The aim of this study was to present a single-center experience in the diagnostic approach and treatment of esophageal achalasia (EA), including the long-term assessment of the QoL.

METHODS

Data collection was based on the retrospective analysis of clinical notes and prospective interviews with patients and their parents.

RESULTS

The study group consisted of 60 patients with EA (F: 26, M: 34), with a median age of 12.0 (1-17) years at diagnosis. The time from the first symptoms until the diagnosis was 1.0 year (0.5-2.0) and the most common were: regurgitation (91.3%), dysphagia (84.8%), and chest pain (47.8%). The diagnostic approach showed a high sensitivity for barium X-ray follow through, esophageal manometry, and endoscopy. Overall, a long-term good outcome of HM was achieved in 27 out of 37 patients (73%) and it was negatively affected by the time between the first symptoms and the diagnosis. Out of the 16 patients who underwent PD before HM, a good outcome was achieved in 14 patients (87.5%), compared to 13 out of 21 patients (62%) who only underwent HM ( = 0.22). Concomitant fundoplication was routinely performed, and 18% required post-operative endoscopic dilatation. At the end of the 12.1 (0.7-26.6)-year follow up, most patients had a good QoL, which significantly corresponded with the treatment outcomes.

CONCLUSIONS

Patients suspected of EA should undergo a thorough clinical evaluation including a manometry, a barium X-ray, and an endoscopy. HM is a safe and effective treatment for achalasia and the outcome is not worsened by a preceding endoscopic PD. In most patients, HM alleviates symptoms, although an impaired QoL is common in long-term follow ups.

摘要

背景

尽管经口内镜下肌切开术(POEM)已被引入,但 heller 肌切开术(HM)仍是主要的治疗方法,而气囊扩张术(PD)的作用仍存在争议。本研究的目的是介绍单中心在食管贲门失弛缓症(EA)诊断方法和治疗方面的经验,包括对生活质量的长期评估。

方法

数据收集基于对临床记录的回顾性分析以及对患者及其父母的前瞻性访谈。

结果

研究组由 60 例 EA 患者组成(女性 26 例,男性 34 例),诊断时的中位年龄为 12.0(1 - 17)岁。从首次出现症状到确诊的时间为 1.0 年(0.5 - 2.0),最常见的症状为:反流(91.3%)、吞咽困难(84.8%)和胸痛(47.8%)。诊断方法对钡餐 X 线透视、食管测压和内镜检查显示出高敏感性。总体而言,37 例患者中有 27 例(73%)HM 取得了长期良好效果,且首次症状与诊断之间的时间对其有负面影响。在 HM 前接受 PD 的 16 例患者中,14 例(87.5%)取得了良好效果,而仅接受 HM 的 21 例患者中有 13 例(62%)取得良好效果(P = 0.22)。常规进行了同期胃底折叠术,18%的患者术后需要内镜扩张。在 12.1(0.7 - 26.6)年的随访结束时,大多数患者生活质量良好,这与治疗结果显著相关。

结论

疑似 EA 的患者应接受包括测压、钡餐 X 线和内镜检查在内的全面临床评估。HM 是治疗贲门失弛缓症的一种安全有效的方法,先行内镜下 PD 不会使治疗结果恶化。在大多数患者中,HM 可缓解症状,尽管在长期随访中生活质量受损较为常见。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/530a/8432175/c1eec8cf0f47/jcm-10-03917-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/530a/8432175/c1eec8cf0f47/jcm-10-03917-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/530a/8432175/c1eec8cf0f47/jcm-10-03917-g001.jpg

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