The 2018 ISDE achalasia guidelines

Abstract

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.

Japanese guidelines for peroral endoscopic myotomy: 1st edition

“…Medium‐term outcomes of POEM, with as many as 5 years of data, are now available in the literature, further supporting its safety and efficacy . A systematic review and meta‐analysis documented that the effectiveness of treatment between POEM and Heller myotomy was similar . In contrast, there has been a nominal amount of comparable information between POEM and balloon dilatation.…”

“…most of whom are committee members for the development of clinical practice guidelines for POEM . Three guidelines have been published for esophageal achalasia, including the Esophageal Achalasia Guidelines (I‐GOAL) proposed and endorsed by the International Society for Diseases of the Esophagus (ISDE) . The current Japanese achalasia guidelines are specific to POEM, and offer endoscopists and surgeons a useful and up‐to‐date resource for applying the best principles of this innovative and potentially disruptive endoscopic technique to manage achalasia.…”

“…In Japan, the Ministry of Health, Labor, and Welfare requires institutional criteria to carry out POEM, whereas in the USA, the Natural Orifice Surgery Consortium for Assessment and Research POEM White Paper Committee proposed that the POEM clinical team be composed of gastroenterologists and gastroenterological surgeons, and that knowledge and training be shared with nurses and other clinical staff . Thus, there has been no global consensus regarding institutional authorization criteria or processes.…”

“…Since 2008, the introduction of a new diagnosis tool, high‐resolution manometry (HRM) has caused predictable evolution in the diagnosis of achalasia . However, upper gastrointestinal endoscopy and barium are still listed as essential diagnostic tools of achalasia, whereas HRM has been the mainstay of diagnostic work‐up particularly for the stratification of the clinical phenotypes of achalasia.…”

“…By using integrated relaxation pressure, specific metrics of propagation and pressurization patterns, the current Chicago classification version provides a systemic stratification scheme that can define this disease into distinct subtypes I, II and III. Type II achalasia has the best prognosis, whereas type III has the worst prognosis . Using HRM to inform surgeons of the proximal location of the diseased segment resulted in longer myotomies that spanned the entire affected segment in type III achalasia .…”