Archived case 226 · July 31-August 7, 2017
Achalasia
Describe the abnormality
The question posed to readers
What is in your differential diagnosis?
Images
Imaging findings
Achalasia refers to a failure of organized esophageal peristalsis with an impaired relaxation of the lower esophageal sphincter, often resulting in marked dilatation of the esophagus and food stasis. Answer Diagnosis: Achalasia
Diagnosis
Achalasia
Peristalsis in the distal smooth muscle segment may be lost due to an abnormality of the Auerbach plexus (responsible for smooth muscle relaxation), resulting in weak, uncoordinated contractions that are non-propulsive. The abnormality may also occur in the vagus nerve. In most cases, achalasia is idiopathic; however, an identical appearance is seen in patients with Chagas disease . Primary achalasia is most frequently seen in middle and late adulthood (age 30 to 70) with no gender predilection.
Chest x-ray findings include: convex opacity behind the right cardiac border; occasionally left convex opacity if the thoracic aorta is tortuous , air-fluid levels over the mediastinum, and a small or absent gastric air bubble.
On fluoroscopy, findings include: the “bird beak” sign (abrupt narrowing of the lower esophagus), esophageal dilatation, pooling or stasis of barium, and uncoordinated non-propulsive tertiary contractions.
Treatment is aimed at allowing adequate drainage of the esophagus into the stomach. Options include lifestyle changes, surgical dilatation, BOTOX injections, and surgical myotomy (Heller myotomy) which may be combined with a fundoplication to prevent GE reflux.(Click for a larger image.)
Original case written by its authors at Virginia Commonwealth University and published at this address as part of a weekly teaching collection. Reproduced here as an archive.