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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

  • Cause unknown

  • Type III is a spastic variant with less favorable treatment outcomes (66%) than types I (81%) or II (96%)

Demographics

  • Increased incidence with advancing age

  • Can be seen in persons as young as 25 years

Clinical Findings

Symptoms and Signs

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

  • Symptoms persist for months to years

  • Substernal chest pain, discomfort, or fullness

  • Regurgitation of undigested food

  • Nocturnal regurgitation

  • Coughing or aspiration

  • Weight loss is common

  • Physical examination unhelpful

Differential Diagnosis

  • Chagas disease

  • Primary or metastatic tumors at the gastroesophageal junction

  • Diffuse esophageal spasm

  • Scleroderma esophagus

  • Peptic stricture

Diagnosis

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

Diagnostic Procedures

  • Endoscopy to exclude a distal stricture or carcinoma

  • Esophageal manometry confirms the diagnosis; characteristic features include

    • Complete absence of normal peristalsis

    • Incomplete lower esophageal sphincteric relaxation with swallowing

Treatment

Medications

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

Surgery

  • Modified Heller cardiomyotomy of the lower esophageal sphincter and cardia

    • Performed by a laparoscopic approach and preferred to an open surgical approach

    • Results in good to excellent 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)

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

    • Symptoms recur following cardiomyotomy in > 25% within 10 years but usually respond to pneumatic dilation

    • Pneumatic dilation may be less effective in young males (age < 45 years), thus surgical myotomy may be preferred for them

    • Surgical myotomy may also be preferred for patients with the type III variant

  • 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 because a fundoplication is not performed

    • In expert centers, POEM may be the preferred treatment modality for type III achalasia (where a longer myotomy of the distal esophagus is indicated) and an appropriate option in patients with types I and II achalasia who do not wish to undergo laparoscopic surgery

  • Complete esophagectomy or percutaneous ...

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