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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

  • 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 radiograph: 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

  • High-resolution esophageal manometry confirms the diagnosis; characteristic features include

    • Absence of normal peristalsis

    • Impaired esophagogastric junction relaxation with swallowing

Treatment

  • Several effective treatment options are available, all of which promote improved esophageal emptying by lowering distal esophageal pressure either through

    • Endoscopic injection with botulinum toxin

    • Disruption of the lower esophageal sphincter (LES) by pneumatic balloon dilation

    • Cardioesophageal myotomy (surgical or endoscopic)

  • Botulinum toxin injection

    • Endoscopically guided injection of botulinum toxin directly into the LES results in a marked reduction in LES pressure with initial improvement in symptoms in patients

    • However, symptom relapse occurs in over 50% of patients within 6–9 months and in all patients within 2 years

    • It is inferior to pneumatic dilation therapy and surgery in producing sustained symptomatic relief; this therapy is most appropriate for patients with comorbidities who are poor candidates for more invasive procedures

  • Pneumatic dilation

    • Over 80% of patients derive good to excellent relief of dysphagia after one to three sessions of pneumatic dilation of the LES

    • Dilation is less effective in patients who

      • Are younger than age 45

      • Have the type III variant

      • Have a dilated esophagus

  • A modified Heller cardiomyotomy of the ...

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