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1. ACHALASIA

ESSENTIALS OF DIAGNOSIS

  • Gradual, progressive dysphagia for solids and liquids.

  • Regurgitation of undigested food.

  • Barium esophagogram with “bird’s beak” distal esophagus.

  • Esophageal manometry confirms diagnosis.

General Considerations

Achalasia is an idiopathic motility disorder characterized by loss of peristalsis in the distal two-thirds (smooth muscle) of the esophagus and impaired relaxation of the LES. There appears to be denervation of the esophagus resulting primarily from loss of nitric oxide–producing inhibitory neurons in the myenteric plexus. The cause of the neuronal degeneration is unknown.

Clinical Findings

A. Symptoms and Signs

There is a steady increase in the incidence of achalasia with age; however, it can be seen in individuals as young as 25 years. Patients complain of the gradual onset of dysphagia for solid foods and, in the majority, of liquids also. Symptoms at presentation may have persisted for months to years. Substernal discomfort or fullness may be noted after eating. Many patients eat more slowly and adopt specific maneuvers such as lifting the neck or throwing the shoulders back to enhance esophageal emptying. Regurgitation of undigested food is common and may occur during meals or up to several hours later. Nocturnal regurgitation can provoke coughing or aspiration. Up to 50% of patients report substernal chest pain that is unrelated to meals or exercise and may last up to hours. Weight loss is common. Physical examination is unhelpful.

B. Imaging

Chest radiographs may show an air-fluid level in the enlarged, fluid-filled esophagus. Barium esophagography discloses characteristic findings, including esophageal dilation, loss of esophageal peristalsis, poor esophageal emptying, and a smooth, symmetric “bird’s beak” tapering of the distal esophagus (eFigure 15–47). Five minutes after ingestion of 8 oz of barium, a column height of more than 2 cm has a sensitivity and specificity of greater than 85% in differentiating achalasia from other causes of dysphagia. Without treatment, the esophagus may become markedly dilated (“sigmoid esophagus”).

eFigure 15–47.

Achalasia of the esophagus. Note dilation of the body of the esophagus, with retained barium, and marked narrowing of the distal esophagus (so-called bird’s beak). (Reproduced, with permission, from Doherty GM (editor). Current Diagnosis & Treatment: Surgery, 14th edition. McGraw-Hill, 2015.)

C. Special Examinations

After esophagography, endoscopy is always performed to evaluate the distal esophagus and gastroesophageal junction to exclude a distal stricture or a submucosal infiltrating carcinoma. The diagnosis is confirmed by esophageal manometry. The manometric features are complete absence of normal peristalsis and incomplete lower esophageal sphincteric relaxation with swallowing. Using high-resolution esophageal topographic tracings, three achalasia subtypes are recognized. Type III is a spastic variant with less favorable treatment outcomes (66%) than types I (81%) or II (96%).

Differential Diagnosis

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