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For further information, see CMDT Part 15-20: Esophageal Motility Disorders

Key Features

Essentials of Diagnosis

  • Gradual, progressive dysphagia for solids and liquids

  • Regurgitation of undigested food

  • Barium esophagogram shows "bird's beak" distal esophagus

  • Esophageal manometry confirms diagnosis

General Considerations

  • Idiopathic motility disorder characterized by loss of peristalsis in the distal two-thirds (smooth muscle) of the esophagus and impaired relaxation of the lower esophageal sphincter (LES)

  • Cause unknown

  • Three achalasia subtypes are recognized based on esophageal contractility and pressure patterns:

    • Types I and II (both with 100% failed peristalsis)

    • Type III (failed peristalsis with ≥ 20% distal premature spastic contractions)


  • Increased incidence with advancing age

  • However, can occur in persons as young as 25 years

Clinical Findings

Symptoms and Signs

  • Gradual dysphagia for solid foods and, in the majority, liquids also

  • Symptoms persist for months to years

  • Substernal chest pain, discomfort, or fullness after eating

  • Regurgitation of undigested food

  • Nocturnal regurgitation

  • Coughing or aspiration

  • Substernal chest pain unrelated to eating in up to 50% of patients

  • Weight loss is common

  • Physical examination unhelpful

Differential Diagnosis

  • Chagas disease

  • Primary or metastatic tumors at the gastroesophageal junction

  • Paraneoplastic syndrome

  • Esophagogastric junction outflow obstruction

  • Esophageal spasm

  • Hypercontractile esophagus

  • Scleroderma esophagus with peptic stricture


Imaging Studies

  • Chest radiographs: air-fluid level in an enlarged, fluid-filled esophagus

  • Barium esophagography

    • Esophageal dilation

    • Loss of esophageal peristalsis

    • Poor esophageal emptying

    • A smooth, symmetric "bird's beak" tapering of the distal esophagus

    • Five minutes after ingestion of 8 oz of barium, a column height of > 2 cm has a sensitivity and specificity of > 85% in differentiating achalasia from other causes of dysphagia

Diagnostic Procedures

  • Endoscopy to exclude a distal stricture or carcinoma

  • Esophageal manometry confirms the diagnosis; characteristic features include

    • Complete absence of normal peristalsis

    • Incomplete LES relaxation with swallowing



  • Calcium channel blockers (nifedipine) may provide temporary symptomatic improvement for some esophageal disorders


  • Modified Heller cardiomyotomy of the LES and cardia

    • Performed by a laparoscopic approach

    • Results in symptomatic improvement in over 90% of patients

    • Because gastroesophageal reflux develops in up to 20% of patients after myotomy, most surgeons also perform an antireflux procedure (fundoplication)

    • Most patients are prescribed a once-daily proton pump inhibitor

    • Symptoms recur following cardiomyotomy in 5–15% within 10 years but usually respond to pneumatic dilation

  • Per oral endoscopic myotomy (POEM)

    • Success rates of over 90% are reported, including in patients with type III achalasia

    • Long-term proton pump inhibitor therapy is required in many patients with gastroesophageal reflux ...

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