Achalasia

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)

Demographics

• Increased incidence with advancing age

• However, can occur in persons as young as age 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

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

  • 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

• Endoscopic ultrasonography and chest CT may be required to examine the distal esophagus if tumor at the gastroesophageal junction is suspected

Diagnostic Procedures

• Endoscopy to exclude a distal stricture or submucosal infiltrating carcinoma

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

  • Complete absence of normal peristalsis

  • Incomplete LES relaxation with swallowing

• An integrated post-swallow relaxation pressure > 15 mm Hg has a diagnostic sensitivity of 97%

TREATMENT

Medications

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

Surgery

• Modified Heller cardiomyotomy of the LES and cardia

  • Performed with a laparoscopic approach

  • Results in symptomatic improvement in ∼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 (PPI)

  • Symptoms recur following cardiomyotomy in 5–15% within 10 ...