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 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

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

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 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 ...