Diagnosis

Primary achalasia

Findings

Oblique image from a barium swallow examination, as well as lateral image of the chest obtained following the procedure. Images demonstrated dilatation of virtually the entirety of the esophagus. In the area of the GE junction, there is a rather smoothly tapering stricturing, resembling a bird's beak. The mucosa appears to be intact on these images.

Discussion

Primary achalasia is a motility disorder thought to be related to a decrease in ganglion cells within the distal 2/3 of the esophagus. This, combined with failure of relaxation of the lower esophageal sphincter, results in the appearance of achalasia. Symptoms in patients with primary achalasia are generally due to dysphagia with both solids and liquids, which tend to be slowly progressive over a long period of time. Differentiation must be made with secondary achalasia, due to an obstructing mass. In these patients, a history can often be obtained of initial dysphagia to solid foods, followed by difficulty swallowing liquids. Fluoroscopically, barium swallow is utilized to demonstrate achalasia. This demonstrates a dilated, at times tortuous appearance to the esophagus with absence of peristalsis in the lower esophagus. Disordered, tertiary contractions can be visualized within the mid esophagus. The smoothly tapered narrowing at the distal esophagus is characteristic of primary achalasia. This can often be differentiated from secondary achalasia, which demonstrates mucosal irregularity. Differentiation may also be possible in terms of age (carcinoma most commonly age >50) and in terms of the duration of patient's symptoms (tends to be less than 6 months in secondary achalasia). Other entities in the differential would be peptic stricture, scleroderma with a reflux stricture and Chagas disease.

Provenzale, Nelson, Duke Radiology Case Review. Lippincott-Raven, Philadelphia, 1998. Eisenberg, Gastrointestinal Radiology Companion. Lippincott Williams & Wilkins, Baltimore, 1999