Elsevier

Gastrointestinal Endoscopy

Volume 91, Issue 2, February 2020, Pages 213-227.e6
Gastrointestinal Endoscopy

Guideline
ASGE guideline on the management of achalasia

https://doi.org/10.1016/j.gie.2019.04.231Get rights and content

Achalasia is a primary esophageal motor disorder of unknown etiology characterized by degeneration of the myenteric plexus, which results in impaired relaxation of the esophagogastric junction (EGJ), along with the loss of organized peristalsis in the esophageal body. The criterion standard for diagnosing achalasia is high-resolution esophageal manometry showing incomplete relaxation of the EGJ coupled with the absence of organized peristalsis. Three achalasia subtypes have been defined based on high-resolution manometry findings in the esophageal body. Treatment of patients with achalasia has evolved in recent years with the introduction of peroral endoscopic myotomy. Other treatment options include botulinum toxin injection, pneumatic dilation, and Heller myotomy. This American Society for Gastrointestinal Endoscopy Standards of Practice Guideline provides evidence-based recommendations for the treatment of achalasia, based on an updated assessment of the individual and comparative effectiveness, adverse effects, and cost of the 4 aforementioned achalasia therapies.

Introduction

Achalasia is a primary esophageal motor disorder of unknown etiology characterized by degeneration of the myenteric plexus, which results in impaired relaxation of the esophagogastric junction (EGJ), along with the loss of organized peristalsis in the esophageal body. These abnormalities typically lead to dysphagia and regurgitation.1 Achalasia occurs equally in males and females. Achalasia has traditionally been viewed as a rare disease, with a globally reported incidence varying from .03 to 1.63 per 100,000 persons per year.2 However, most estimates of incidence have been derived from retrospective searches of hospital discharge databases, with the diagnosis based on older diagnostic techniques such as conventional manometry or barium esophagram. More recent studies incorporating state-of-the-art high-resolution manometry and data derived from motility laboratory databases suggest a higher incidence of 2.92 of 100,000 in Central Chicago2 and 2.3 to 2.8 of 100,000 in South Australia.3

Section snippets

Diagnosis of achalasia

Esophageal motor abnormalities in achalasia lead to symptoms of dysphagia for solids and liquids without oropharyngeal transfer difficulties in roughly 90% of patients, regurgitation in 75%, weight loss in 60%, chest pain in 50%, and heartburn in 40%.4 In patients with a clinical presentation suggestive of achalasia, endoscopy is mandatory to exclude pseudoachalasia or other forms of mechanical obstruction at the EGJ.1 Although endoscopy may often reveal esophageal dilation, retention of food

Aim and scope

In the last decade, there have been considerable advances in the evaluation and management of achalasia. From a diagnostic perspective, high-resolution manometry has become the criterion standard, leading to the definition of 3 achalasia subtypes that have confirmed implications for response to and choice of therapeutic modality. Furthermore, EndoFLIP is emerging as a useful technique for diagnosis and objective assessment after therapy. Although botulinum toxin injection, pneumatic dilation,

Overview

This document was prepared by a working group of the Standards of Practice Committee of the American Society for Gastrointestinal Endoscopy (ASGE). It includes a systematic review of available literature along with guidelines for the role of endoscopy in management of achalasia using criteria highlighted in Table 1.15 After evidence synthesis, recommendations were drafted by the full panel during a face-to-face meeting on March 16, 2018, and approved by the Standards of Practice committee

Treatment of achalasia

Although up to 5% of achalasia patients may require esophagectomy for end-stage achalasia,4 this document focuses on the treatment modalities currently used for managing most patients with achalasia: botulinum toxin injection, pneumatic dilation, laparoscopic Heller myotomy, and POEM. It is important to note that when assessing the literature that describes the effectiveness of achalasia treatments, widely varying definitions of therapeutic success are encountered across different studies. For

Future directions

Final results from multiple randomized trials, including trials comparing POEM versus laparoscopic Heller myotomy and anterior versus posterior POEM, are awaited. Although existing results are very encouraging, POEM remains an intricate endoscopic procedure that requires advanced endoscopic skills, knowledge of surgical anatomy, and expertise in submucosal endoscopy and management of adverse events, such as bleeding, perforation, and leakage.114 Multiple studies have evaluated the learning

Conclusions

Pneumatic dilation and laparoscopic Heller myotomy are effective and established treatment options in the management of achalasia patients. Since the introduction of POEM in 2008, this procedure has gained worldwide acceptance as a primary treatment for patients with achalasia and other esophageal motility disorders. Multiple studies and meta-analyses have reported its excellent efficacy and safety during the short- and medium-term follow-up, and recent literature suggest long-term efficacy as

Disclosure

The following authors disclosed financial relationships relevant to this publication: M. A. Khashab: Consultant for Boston Scientific, Olympus, and Medtronic; medical advisory board for Boston Scientific and Olympus; received royalties from UpToDate. M. F. Vela: Consultant for Medtronic; research support from Diversatek. N. Thosani: Consultant for Boston Scientific, Medtronic, and Pentax America; speaker for AbbVie; received royalties from UpToDate. J. L. Buxbaum: Consultant for Olympus. D. S.

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    This document was reviewed and approved by the Governing Board of the American Society for Gastrointestinal Endoscopy (ASGE)

    Endorsed by the American Neurogastroenterology and Motility Society and the Society of American Gastrointestinal and Endoscopic Surgeons (SAGES)

    Drs Khashab, Vela, and Thosani contributed equally to this article.

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