Achalasia results in proximal dilatation of the esophagus secondary to a functional obstruction of the distal esophagus with failure of the lower esophageal sphincter to relax and of the smooth muscle to contract with normal peristalsis. In primary achalasia, the cause of dysfunction is unknown, but most think that there is a defect in the cholinergic innervation of the distal esophagus with a general paucity or absence of ganglion cells of the myenteric plexuses. Classic achalasia usually occurs in young patients in their 2nd through 4th decade who present with dysphagia and even episodes of regurgitation.

Radiographic findings on barium examinations include conical smooth narrowing of the distal esophagus in a “bird-beak” or “rat-tail” configuration with most pronounced narrowing at the gastroesophageal junction as this case demonstrates. Breakup of the primary stripping wave is noted below the level of the cricopharyngeus muscle and there is proximal dilatation of the esophagus. A food/debris level can be noted as ingested material is often retained within the esophagus which the patient may be unaware of which can even be a chest radiograph finding. When early achalasia is suspected the examination should also be performed in a recumbent position so that the effects of gravity are removed (which could potentially mask the disease).

Achalasia may be primary in which the cause is idiopathic or secondary in which there is carcinomatous invasion of the myenteric plexus or even infectious (Chaga’s disease). Endoscopy is frequently required to make a visual and pathologic diagnosis (biopsy).