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Achalasia

Clinical Subject Page

Achalasia

Achalasia is an esophageal motility disorder characterized by impaired relaxation of the lower
esophageal sphincter (LES) due to degeneration of inhibitory neurons within the esophageal wall.

Also called

Barrett's oesophagus (British spelling)

ICD-10

K22.0

Specialty

Gastroenterology

Onset

Chronic

Reviewed

July 2026

On This Page

Overview

Achalasia causes failure of the LES to relax and impaired esophageal motility, resulting in
dysphagia, regurgitation, chest pain, and weight loss. High-resolution esophageal manometry is the
preferred test to confirm the diagnosis. Definitive treatment includes pneumatic dilation,
laparoscopic Heller myotomy, or peroral endoscopic myotomy (POEM).

Etiology & Risk Factors

Etiology

Most cases are idiopathic, caused by degeneration of inhibitory neurons in the myenteric (Auerbach) plexus, resulting in impaired relaxation of the lower esophageal sphincter (LES) and absent esophageal peristalsis.

Less common causes include:

  • Chagas disease (Trypanosoma cruzi infection)

  • Autoimmune-mediated neuronal injury

  • Genetic predisposition (rare)

  • Malignancy causing pseudoachalasia (e.g., gastroesophageal junction cancer)

  • Infiltrative disorders (rare)

Risk Factors

  • Age 25–60 years (can occur at any age)

  • Family history (rare)

  • Autoimmune diseases

  • Chagas disease (endemic regions of Latin America)

  • Esophagogastric junction malignancy (pseudoachalasia)

  • Rare genetic syndromes (e.g., Allgrove/Triple A syndrome)

Pathophysiology

1. Degeneration of inhibitory neurons in the esophageal wall.
2. Impaired relaxation of the lower esophageal sphincter (LES).
3. Impaired esophageal emptying.
4. Progressive esophageal dilation with food retention.
5. Symptoms develop due to obstruction of food passage.

Clinical Presentation

  • Common symptoms:
    • Dysphagia to both solids and liquids (most common)
    • Regurgitation
    • Retrosternal (chest) pain
    • Weight loss

History Taking

  • Ask about:
    • Dysphagia to solids and liquids
    • Regurgitation of undigested food
    • Retrosternal chest pain
    • Weight loss
    • Duration and progression of symptoms
    • Previous treatments
    • Symptoms suggesting malignancy (to assess for pseudoachalasia)

Physical Examination

  • Physical examination is often normal.
  • Assess nutritional status and evidence of weight loss.

Investigations

  • Initial Tests

    • Complete blood count (CBC)

    • Electrolytes and renal function tests

    • Nutritional assessment (if significant weight loss)

    Diagnostic Investigations

    Barium Swallow (Esophagram)

    • First-line investigation

    • Shows:

      • Bird’s beak narrowing at the gastroesophageal junction

      • Dilated esophagus

      • Delayed esophageal emptying

    Upper Gastrointestinal Endoscopy (EGD)

    • Excludes mechanical obstruction or malignancy (pseudoachalasia)

    • May show retained food, saliva, and a dilated esophagus

    High-Resolution Esophageal Manometry (Gold Standard)

    Findings:

    • Impaired relaxation of the lower esophageal sphincter (LES)

    • Absent normal esophageal peristalsis

    • Classifies achalasia into Type I, II, and III

    Additional Investigations

    • CT chest or abdomen if pseudoachalasia or malignancy is suspected

    • Endoscopic ultrasound (EUS) if gastroesophageal junction cancer is suspected

Diagnosis

  • Diagnosis is usually based on:
    • Clinical features
    • High-resolution esophageal manometry (gold-standard confirmatory test)
    • Upper endoscopy to rule out pseudoachalasia
    • Barium esophagram as an adjunct when indicated

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Management

  • 1. Definitive Therapy

    Pneumatic Balloon Dilation (PD)

    • First-line option for many adults

    • Endoscopic balloon dilates the LES

    • May require repeat procedures

    Laparoscopic Heller Myotomy (LHM)

    • Surgical division of the LES muscle

    • Usually combined with partial fundoplication to reduce postoperative reflux

    • Provides durable symptom relief

    Peroral Endoscopic Myotomy (POEM)

    • Minimally invasive endoscopic myotomy

    • Particularly effective for type III (spastic) achalasia

    • Higher risk of postoperative GERD than Heller myotomy

    2. Medical Therapy

    Reserved for patients who are not candidates for definitive treatment.

    Options include:

    • Nitrates (e.g., isosorbide dinitrate)

    • Calcium channel blockers (e.g., nifedipine)

    Note: Symptom relief is usually temporary and less effective than procedural treatments.

    3. Botulinum Toxin Injection

    • Endoscopic injection into the LES

    • Suitable for:

      • Elderly patients

      • Frail patients

      • Poor surgical candidates

    • Benefits are temporary and repeat injections are often required.

    4. Nutritional Support

    • Eat slowly and chew food thoroughly

    • Drink water with meals

    • Eat smaller, frequent meals

    • Nutritional assessment if weight loss is significant

    5. Follow-Up

    • Monitor symptom recurrence

    • Assess for GERD after myotomy or POEM

    • Repeat endoscopy if symptoms recur or complications are suspected

    • Long-standing achalasia may require surveillance for esophageal cancer in selected patients

Complications

• Progressive esophageal dilation
• Persistent dysphagia
• Weight loss
• Malnutrition

Prognosis

  • Most patients experience symptomatic improvement with definitive treatment.
  • Treatment choice
    depends on the achalasia subtype, and recurrent symptoms may require reassessment.

Key Points / Clinical Pearls

• Achalasia is caused by degeneration of inhibitory neurons in the esophageal wall.
• It results in impaired LES relaxation.
• Dysphagia to both solids and liquids is the hallmark symptom.
• High-resolution esophageal manometry is the preferred diagnostic test.
• Upper endoscopy is required to exclude pseudoachalasia.
• Barium esophagram supports diagnosis and follow-up.
• Definitive treatments are POEM, laparoscopic Heller myotomy, and pneumatic dilation.
• Botulinum toxin is used when definitive therapy is not feasible.
• Nifedipine is considered only as a last-resort medical option.

  • Puri R, Tian C. National Center for Biotechnology Information (NIH). Achalasia, StatPearls.
  • Yadlapati R, Kahrilas PJ, Fox MR, et al. Esophageal Motility Disorders on High-Resolution Manometry: Chicago Classification Version 4.0. Neurogastroenterol Motil. 2021;33:e14058. PMID: 34490927.
  • Vaezi MF, Pandolfino JE, Yadlapati RH, Greer KB, Kavitt RT. ACG Clinical Guidelines: Diagnosis and Management of Achalasia. Am J Gastroenterol. 2020;115:1393-1411. PMC9896940.
  • MedlinePlus, National Library of Medicine (NIH). Achalasia: Medical Encyclopedia.
  • National Center for Biotechnology Information (NIH). Esophageal Manometry, StatPearls.