Netherlands trial register (NTR37) and Current Controlled Trials registry (ISRCTN56304564).

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.

Achalasia is the best characterized primary esophageal motility disorder of the esophagus and typically presents with absent peristalsis of the esophageal body and a failure of the lower esophageal sphincter to relax upon swallowing on manometry, associated with progressively severe dysphagia, regurgitation, aspiration, chest pain, and weight loss. The diagnosis is suggested by barium swallow and endoscopy, and confirmed by manometry. As there is no curative treatment for achalasia, treatment is confined to disruption of the lower esophageal sphincter to improve bolus passage. Treatment modalities available for this purpose include pneumatic dilation, laparoscopic Heller myotomy and since recently peroral endoscopic myotomy or POEM. In this review, we will discuss the current diagnosis, management, and treatment options of achalasia.

Background: The COVID-19 pandemic, declared by WHO on March 13 th 2020, had a major global impact on the health care system and services. In the acute phase, the presence of the SARS-CoV-2 virus in the aerodigestive tract limited activities in the gastroenterology clinic and procedures to emergencies only. Motility and function testing was interrupted and as we enter the recovery phase, restarting these procedures requires a safety-focused approach with adequate infection prevention for patients and healthcare professionals.Methods: We summarized knowledge on the presence of the SARS-CoV-2 virus in the aerodigestive tract and the risk of spread with motility and functional testing. We surveyed 39 European centers documenting how the pandemic affected activities and which measures they are considering for restarting these measurements. We propose recommendations based on current knowledge as applied in our center.Results: Positioning of catheters for gastrointestinal motility tests carries a concern for aerosol-borne infection of health care workers. The risk is low with breath tests.The surveyed centers stopped almost all motility and function tests from the second half of March. The speed of restarting and the safety measures taken varied highly.Conclusions and Inferences: Based on these findings, we provided recommendations and practical relevant information for motility and function test procedures in the COVID-19 pandemic era, to guarantee a high-quality patient care with adequate infection prevention.

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Achalasia is a primary esophageal motor disorder of the esophagus that is characterized by the absence of esophageal peristalsis and a failure of the lower esophageal sphincter (LES) to relax upon swallowing. The defective relaxation leads to symptoms of dysphagia for solids and liquids, regurgitation, aspiration, chest pain, and weight loss. Achalasia is believed to result from a selective loss of enteric inhibitory neurons, most likely due to an autoimmune phenomenon in genetic susceptible individuals. As there is no curative treatment for achalasia, treatment is confined to disruption of the LES to improve bolus passage. The two most commonly used treatment modalities available are the endoscopic pneumodilation (PD) and the surgical laparoscopic Heller myotomy (LHM). A recent European randomized controlled trial showed that both treatment modalities have comparable success rates after a follow-up of at least 5 years. In view of these data, both treatments can be used as an initial therapy in achalasia and the choice should be based on the expertise available. Recently, a new endoscopic technique, peroral endoscopic myotomy (POEM), has been introduced with excellent short-term success rates. However, longer follow-up and data from randomized controlled trials are needed before accepting this technique as a new treatment option for achalasia in clinical practice.

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In conclusion, the strategy of treating achalasia starting with PD appears the most economic approach, independent of the health system.