Zaninotto G, Bennett C, Boeckxstaens G, Costantini M, Ferguson M K, Pandolfino J E, Patti M G, Ribeiro U, Richter J, Swanstrom L, Tack J, Triadafilopoulos G, Markar S R, Salvador R, Faccio L, Andreollo N A, Cecconello I, Costamagna G, da Rocha J R M, Hungness E S, Fisichella P M, Fuchs K H, Gockel I, Gurski R, Gyawali C P, Herbella F A M, Holloway R H, Hongo M, Jobe B A, Kahrilas P J, Katzka D A, Dua K S, Liu D, Moonen A, Nasi A, Pasricha P J, Penagini R, Perretta S, Sallum R A A, Sarnelli G, Savarino E, Schlottmann F, Sifrim D, Soper N, Tatum R P, Vaezi M F, van Herwaarden-Lindeboom M, Vanuytsel T, Vela M F, Watson D I, Zerbib F, Gittens S, Pontillo C, Vermigli S, Inama D, Low D E
Department of Surgery and Cancer, Imperial College, London, UK.
Office of Research and Innovation, Royal College of Surgeons in Ireland, Ireland.
Dis Esophagus. 2018 Sep 1;31(9). doi: 10.1093/dote/doy071.
Achalasia is a relatively rare primary motor esophageal disorder, characterized by absence of relaxations of the lower esophageal sphincter and of peristalsis along the esophageal body. As a result, patients typically present with dysphagia, regurgitation and occasionally chest pain, pulmonary complication and malnutrition. New diagnostic methodologies and therapeutic techniques have been recently added to the armamentarium for treating achalasia. With the aim to offer clinicians and patients an up-to-date framework for making informed decisions on the management of this disease, the International Society for Diseases of the Esophagus Guidelines proposed and endorsed the Esophageal Achalasia Guidelines (I-GOAL). The guidelines were prepared according the Appraisal of Guidelines for Research and Evaluation (AGREE-REX) tool, accredited for guideline production by NICE UK. A systematic literature search was performed and the quality of evidence and the strength of recommendations were graded according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE). Given the relative rarity of this disease and the paucity of high-level evidence in the literature, this process was integrated with a three-step process of anonymous voting on each statement (DELPHI). Only statements with an approval rate >80% were accepted in the guidelines. Fifty-one experts from 11 countries and 3 representatives from patient support associations participated to the preparations of the guidelines. These guidelines deal specifically with the following achalasia issues: Diagnostic workup, Definition of the disease, Severity of presentation, Medical treatment, Botulinum Toxin injection, Pneumatic dilatation, POEM, Other endoscopic treatments, Laparoscopic myotomy, Definition of recurrence, Follow up and risk of cancer, Management of end stage achalasia, Treatment options for failure, Achalasia in children, Achalasia secondary to Chagas' disease.
贲门失弛缓症是一种相对罕见的原发性食管运动障碍性疾病，其特征为食管下括约肌松弛缺失以及食管体部蠕动缺失。因此，患者通常表现为吞咽困难、反流，偶尔出现胸痛、肺部并发症和营养不良。最近，新的诊断方法和治疗技术已被纳入治疗贲门失弛缓症的手段之中。为了为临床医生和患者提供一个最新的框架，以便就该疾病的管理做出明智的决策，国际食管疾病学会指南提出并认可了《食管贲门失弛缓症指南》（I-GOAL）。这些指南是根据《研究与评价指南评估》（AGREE-REX）工具编写的，该工具已获得英国国家卫生与临床优化研究所（NICE）认可用于指南制定。进行了系统的文献检索，并根据《推荐分级的评估、制定和评价》（GRADE）对证据质量和推荐强度进行分级。鉴于该疾病相对罕见且文献中高水平证据匮乏，这一过程与对每条陈述进行匿名投票的三步过程（德尔菲法）相结合。指南中仅接受批准率>80%的陈述。来自11个国家的51名专家和患者支持协会的3名代表参与了指南的编写工作。这些指南专门涉及以下贲门失弛缓症问题：诊断检查、疾病定义、临床表现严重程度、药物治疗、肉毒杆菌毒素注射、气囊扩张、经口内镜下肌切开术（POEM）、其他内镜治疗、腹腔镜肌切开术、复发定义、随访与癌症风险、终末期贲门失弛缓症的管理、治疗失败的选择、儿童贲门失弛缓症、恰加斯病继发的贲门失弛缓症。
