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Achalasia and Esophageal Outlet Obstruction

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Gastrointestinal Motility Disorders

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Abstract

Achalasia is an uncommon motility disorder of the esophagus that is characterized by impaired lower esophageal sphincter (LES) relaxation and, in many cases, failure of peristalsis. Although the cause is unknown, it is thought to arise from the loss of myenteric neurons in the esophagus from an aberrant immune response in a genetically susceptible host. The resultant stasis of ingested food results in dysphagia, chest pain, and weight loss. The Chicago Classification for High Resolution Manometry (HRM) has categorized patients with achalasia into three diagnostic categories. The key finding is impaired LES relaxation as indicated by raised “integrated relaxation pressure (IRP > 15 mm Hg). Type I “classic” achalasia, often with luminal dilatation and little pressure activity in the esophageal body, Type II displays “pan-esophageal pressurization” on swallowing, and Type III “vigorous” achalasia is accompanied by spasm.

There is no cure for achalasia. Thus, treatment aims to disrupt the muscle fibers of the LES to improve esophagogastric junction (EGJ) opening and bolus passage. Pneumatic dilation and surgical myotomy combined with an antireflux procedure are established treatments that have shown comparable treatment outcomes in clinical studies. In recent years, per-oral endoscopic myotomy (POEM) has been introduced and has shown promising short-term results.

Esophagogastric junction outflow obstruction (EGJOO) is a recently described clinical entity categorized as impaired EGJ function with, at least in part, preserved esophageal contractility (i.e., without typical features of achalasia). The sensitivity of HRM for EGJOO is increased by inclusion of adjunctive tests (e.g., solid test meal). Technology to assess reduced EGJ distensibility (e.g., EndoFLIP) at endoscopy has also been described. After exclusion of a structural obstruction (e.g., eosinophilic esophagitis, tight fundoplication), treatment for functional EGJOO is similar to achalasia although large-scale clinical studies and long-term outcome data on EGJOO are awaited.

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Authors and Affiliations

  1. Department of Gastroenterology, Changi General Hospital, 2 Simei Street 3, Singapore, 529889, Singapore

    Daphne Ang M.B.B.S., M.R.C.P., F.R.C.P.

  2. Abdominal Center: Gastroenterology, St. Claraspital, Kleinriehenstrasse 30, Basel, Switzerland

    Mark Fox M.D., M.A.

  3. Neurogastroenterology and Motility Research Group, University Hospital Zürich, Zürich, Switzerland

    Mark Fox M.D., M.A.

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  1. Daphne Ang M.B.B.S., M.R.C.P., F.R.C.P.
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  2. Mark Fox M.D., M.A.
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Correspondence to Mark Fox M.D., M.A. .

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Editors and Affiliations

  1. Institute of Gastroenterology, Sheba medical Center, Tel Aviv University, Ramat Aviv, Tel Aviv, Israel

    Eytan Bardan

  2. Division of Gastroenterology and Hepatology, Medical College of Wisconsin, Milwaukee, Wisconsin, USA

    Reza Shaker

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Ang, D., Fox, M. (2018). Achalasia and Esophageal Outlet Obstruction. In: Bardan, E., Shaker, R. (eds) Gastrointestinal Motility Disorders . Springer, Cham. https://doi.org/10.1007/978-3-319-59352-4_1

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