Last updated March 14, 2022

Diagnosis and Management of Achalasia

Recommendations

Diagnosis and assessment

We recommend that patients who are initially suspected of having GERD but do not respond to acid-suppressive therapy should be evaluated for achalasia. (Strong  “We recommend”, Very low)
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We recommend using esophageal pressure topography over conventional line tracing for the diagnosis of achalasia. (Strong  “We recommend”, High)
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We suggest that classifying achalasia subtypes by the Chicago Classification may help inform both prognosis and treatment choice. (Conditional (weak)  “We suggest”, Low)
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Initial treatment (medical therapy, pneumatic dilation, surgical myotomy, and POEM) 

In patients with achalasia who are candidates for definite therapy:
  • PD, LHM, and POEM are comparable effective therapies for type I or type II achalasia.
  • POEM would be a better treatment option in those with type III achalasia.
  • Botulinum toxin injection is reserved for those who cannot undergo the above definitive therapies.
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We suggest that POEM or PD result in comparable symptomatic improvement in patients with types I or II achalasia. (Conditional (weak)  “We suggest”, Low)
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We recommend that POEM and LHM result in comparable symptomatic improvement in patients with achalasia. (Strong  “We recommend”, Moderate)
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We recommend tailored POEM or LHM for type III achalasia as a more efficacious alternative disruptive therapy at the LES compared to PD. (Strong  “We recommend”, Moderate)
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We recommend that PD is superior to medical therapy in relieving symptoms and physiologic parameters of esophageal emptying. (Strong  “We recommend”, Very low)
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We recommend that PD or LHM are both effective and equivalent short- and long-term procedures for patients with achalasia who are candidates to undergo definitive therapy. (Strong  “We recommend”, High)
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We recommend LHM over botulinum toxin injection in patients with achalasia fit for surgery. (Strong  “We recommend”, High)
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We recommend botulinum toxin injection as first-line therapy for patients with achalasia who are unfit for definitive therapies compared with other less effective pharmacological therapies. (Strong  “We recommend”, Moderate)
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We suggest that previous treatment with botulinum toxin injection does not significantly affect performance and outcomes of myotomy. (Conditional (weak)  “We suggest”, Low)
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We recommend that myotomy with fundoplication is superior to myotomy without fundoplication in controlling distal esophageal acid exposure. (Strong  “We recommend”, Moderate)
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We suggest either Dor or Toupet fundoplication to control esophageal acid exposure in patients with achalasia undergoing surgical myotomy. (Conditional (weak)  “We suggest”, Moderate)
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We recommend against stent placement for management of long-term dysphagia in patients with achalasia. (Strong  “We recommend”, Low)
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Post-therapy assessment

We recommend against obtaining routine gastrograffin esophagram after dilation. This test should be reserved for patients with a clinical suspicion for perforation after dilation. (Strong  “We recommend”, Low)
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We suggest that ES or HRM alone not be used to define treatment failure in evaluating continued or recurrent symptoms after definitive therapy for achalasia. (Strong  “We recommend”, Very low)
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We recommend using TBE as the first-line test in evaluating continued or recurrent symptoms after definitive therapy for achalasia. (Strong  “We recommend”, Very low)
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We suggest that in patients with achalasia, POEM compared with LHM with fundoplication or PD is associated with a higher incidence of GERD. (Strong  “We recommend”, Moderate)
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Post-failed initial therapies

We recommend that PD is an appropriate and safe treatment option for patients with achalasia post-initial surgical myotomy or POEM in need of retreatment. (Strong  “We recommend”, Moderate)
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We suggest that POEM is a safe option in patients with achalasia who have previously undergone PD or LHM. (Strong  “We recommend”, Low)
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We suggest that Heller myotomy be considered before esophagectomy in patients who have failed PD and POEM if the anatomy is conducive, and there is evidence of incomplete myotomy. (Strong  “We recommend”, Very low)
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We recommend esophagectomy in surgically-fit patients with megaesophagus who have failed other interventions. (Strong  “We recommend”, Low)
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Cancer surveillance

We suggest against routine endoscopic surveillance for esophageal carcinoma in patients with achalasia. (Strong  “We recommend”, Low)
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ES, Eckardt score; GERD, gastroesophageal reflux disease; GRADE, Grading of Recommendations Assessment, Development and Evaluation; HRM, high resolution manometry; LES, lower esophageal sphincter; LHM, laparoscopic Heller myotomy; PD, pneumatic dilation; POEM, peroral endoscopic myotomy; TBE, timed barium esophagram.

Recommendation Grading

Overview

Title

Diagnosis and Management of Achalasia

Authoring Organization

Publication Month/Year

August 12, 2020

Document Type

Guideline

External Publication Status

Published

Country of Publication

US

Inclusion Criteria

Female, Male, Adult, Older adult

Health Care Settings

Ambulatory, Outpatient

Scope

Diagnosis, Management

Diseases/Conditions (MeSH)

D004931 - Esophageal Achalasia

Keywords

Esophageal Achalasia, achalasia, motility disorders

Source Citation

Vaezi, Michael F. MD, PhD, MSc, FACG; Pandolfino, John E. MD, MS, FACG; Yadlapati, Rena H. MD, MHS (GRADE Methodologist); Greer, Katarina B. MD, MS; Kavitt, Robert T. MD, MPH ACG Clinical Guidelines: Diagnosis and Management of Achalasia, The American Journal of Gastroenterology: September 2020 - Volume 115 - Issue 9 - p 1393-1411 doi: 10.14309/ajg.0000000000000731