Achalasia in a 48-Year-Old Man

A 48-year-old man complained of difficulty in swallowing both solid food and liquids. The dysphagia began several years earlier and had become increasingly severe and more frequent over the past 2 to 3 years. Vague heartburn without reflux and frequent regurgitation were also troublesome.

The patient denied smoking and any decrease in appetite but had lost a significant amount of weight during the past couple of months. Laboratory test results were unremarkable. A chest film revealed a retrocardiac air fluid level; the lung fields and the cardiac shadow were within normal limits (A).

Esophageal abnormality was suspected. A barium esophagogram demonstrated a markedly dilated esophagus with slow emptying and a severely constricted, narrow lower segment with a classic “bird beak” appearance (B). Endoscopic examination of the upper gastrointestinal tract disclosed normal mucosa and no fixed stricture, but increased resistance to the passage of the endoscope into the stomach was noted. The diagnosis of achalasia was confirmed by esophageal manometry.

Drs Navin Verma, Terence M. Brady, and Sonia Arunabh of Queens, NY, comment that achalasia is characterized by the absence of primary esophageal peristalsis and a hypertensive lower esophageal sphincter (LES) that fails to relax appropriately in response to swallowing. Persons with achalasia may present with chest pain in addition to the gastrointestinal symptoms seen in this patient.

Treatment is directed at relieving the dysphagia. This patient was managed surgically and then given calcium channel blockers. These agents can decrease the outflow resistance caused by the dysfunctional LES. Invasive endoscopic or surgical dilatation of the narrowed segment of the esophagus may be considered. Heller's myotomy, which has been associated with perforation, reflux, and other complications, is reserved for patients whose symptoms are unresponsive to other modalities.

Intrasphincteric administration of botulinum toxin to decrease the amount of acetylcholine available at the sphincter has been tried with some success. However, this procedure is still investigational.