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Achalasia

What's new

Updated 2024 AGA guidelines for peroral endoscopic myotomy in achalasia .

Background

Overview

Definition
Achalasia is an esophageal motility disorder defined by loss of esophageal peristalsis and incomplete relaxation of lower esophageal sphincter.
1
Pathophysiology
Proposed causes of achalasia include GEJ obstruction, neuronal degeneration, viral infection, genetic inheritance, and autoimmune disease.
2
Disease course
Inflammatory neurodegenerative insult with possible viral involvement within the esophagus results in achalasia, which causes the clinical manifestation of progressive dysphagia, regurgitation, chest pain, heartburn, nocturnal cough, aspiration, and weight loss. The progression of the disease may result in aspiration-pneumonia, Barrett's esophagus, and esophageal cancer.
3
Prognosis and risk of recurrence
Achalasia is not associated with an increase in mortality.
4

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of achalasia are prepared by our editorial team based on guidelines from the American Society for Gastrointestinal Endoscopy (ASGE 2025,2020,2014), the European Society for Neurogastroenterology and Motility (ESNM/UEG 2025), the American Gastroenterological Association (AGA 2024), the European Society of Gastrointestinal Endoscopy (ESGE 2020), the International Society for Diseases of the Esophagus ...
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Screening and diagnosis

Diagnosis: as per ACG 2013 guidelines, suspect achalasia in patients with dysphagia to solids and liquids, and in patients with regurgitation unresponsive to an adequate trial of PPI therapy.
B
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Classification and risk stratification

Disease classification: as per ISDE 2018 guidelines, use the Chicago classification of esophageal motility disorders to assign patients with achalasia to one of three disease subclasses:
Situation
Guidance
Type I (classic)
Elevated median integrated relaxation pressure (> 15 mmHg)
100% failed peristalsis
Distal contractile integral < 100 mmHg
Premature contractions with distal contractile integral < 450 mmHg cm/sec satisfy criteria for failed peristalsis
Type II (with esophageal compression)
Elevated median integrated relaxation pressure (> 15 mmHg)
100% failed peristalsis
Panesophageal pressurization with ≥ 20% of swallows
Contractions may be masked by esophageal pressurization and distal contractile integral should not be calculated
Type III (spastic)
Elevated median integrated relaxation pressure (> 15 mmHg)
No normal peristalsis
Premature (spastic) contractions with distal contractile integral > 450 mmHg cm/sec with ≥ 20% of swallows
May be mixed with panesophageal pressurization
E

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  • Severity grading

Diagnostic investigations

Esophageal manometry
As per ESNM/UEG 2025 guidelines:
Obtain testing for esophageal motility disorders with esophageal manometry in patients presenting with chronic nausea and vomiting only if esophageal symptoms (regurgitation, dysphagia) are present and structural disease has been excluded.
B
Obtain high-resolution manometry when an esophageal motility disorder is suspected in patients with regurgitation and/or vomiting, after excluding mechanical obstruction. Consider obtaining complementary tests, such as high-resolution impedance manometry, barium oesophagogram, or endoscopic impedance planimetry functional luminal imaging probe, in complex cases.
A

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  • Barium esophagram

  • Upper gastrointestinal endoscopy

Medical management

Pharmacologic therapy: as per ISDE 2018 guidelines, avoid using nitrates, calcium blockers or phosphodiesterase inhibitors for symptomatic relief in patients with achalasia.
D

Therapeutic procedures

Choice of initial therapy
As per AGA 2024 guidelines:
Offer POEM, laparoscopic Heller myotomy, or pneumatic dilation in patients with type I or type II achalasia, with the choice based on shared decision-making, taking into account patient and disease characteristics, patient preferences, and local expertise. Consider offering POEM as the preferred treatment for type III achalasia.
E
Recognize that POEM may be superior to pneumatic dilation in patients with failed initial POEM or laparoscopic Heller myotomy, but choose between treatment modalities based on shared decision-making, taking into account risk of postprocedural reflux, need for repeat interventions, patient preferences, and local expertise.
E

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  • Pneumatic dilatation

  • POEM (indications)

  • POEM (technical considerations)

  • POEM (post-myotomy GERD)

  • Botulinum toxin injection

  • Other endoscopic therapies

Perioperative care

Preoperative evaluation
As per AGA 2024 guidelines:
Obtain a comprehensive diagnostic workup including clinical history, review of medications, upper endoscopy, timed barium esophagram, and high-resolution manometry in the evaluation for POEM. Consider obtaining endoscopic functional luminal impedance planimetry as an adjunct test, particularly when the diagnosis is equivocal.
E
Obtain a comprehensive evaluation with correlation of symptoms in patients with esophagogastric outflow obstruction alone and/or nonachalasia spastic disorders on manometry. Consider performing POEM on a case-by-case basis only after exhausting other less invasive approaches, as the evidence for POEM for these manometric findings is limited.
E

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  • Perioperative antibiotic prophylaxis

  • Postoperative care

Surgical interventions

Surgical myotomy: as per ISDE 2018 guidelines, perform laparoscopic Heller myotomy for symptom control in patients with Chicago type I and type II achalasia.
B
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  • Esophagectomy

Specific circumstances

Pediatric patients: as per ISDE 2018 guidelines, obtain the same work-up in pediatric patients with a provisional diagnosis of achalasia as in adult patients.
E
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  • Patients with Chagas disease esophagopathy

  • Patients with other spastic motility disorders

Patient education

Preoperative counseling: as per ASGE 2020 guidelines, consider counseling patients undergoing POEM regarding the increased risk of postprocedural reflux compared with pneumatic dilation and laparoscopic Heller myotomy.
C

Follow-up and surveillance

Post-treatment follow-up
As per AGA 2024 guidelines:
Monitor for GERD in all patients following POEM. Obtain additional testing in patients with persistent esophagitis and/or reflux-like symptoms despite PPI use to evaluate for other etiologies beyond pathologic acid exposure and to optimize reflux control.
E
Encourage long-term postprocedure surveillance to monitor for disease progression and complications of GERD.
E

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  • Management of disease recurrence

  • Surveillance for esophageal cancer

Quality improvement

Health professional training: as per ISDE 2018 guidelines, ensure appropriate training with in vivo/in vitro animal model and adequate proctorship before starting a clinical program of POEM.
E