Achalasia

Achalasia is caused by degeneration of the
myenteric plexus with loss of the normal inhibitory
neural structure of the lower esophageal sphincter (LES). There is failure of the LES to relax
and decreased peristalsis. The LES is usually contracted to prevent the acidic gastric contents
from refluxing backward into the esophagus.


The vast majority of cases are of unknown etiology. A very small number can be from Chagas
disease, gastric carcinoma, or a disease that can infiltrate into the area such as lymphoma.
Clinical Presentation. Achalasia presents with progressive dysphagia to both solids and
liquids simultaneously and can have regurgitation several hours after eating. The patient
complains of esophageal dysphagia with possible weight loss. Achalasia has no relationship
with alcohol or tobacco use. This is different from esophageal cancer, which not only usually
presents with dysphagia to solid foods and progresses to difficulty swallowing liquids, but also
is more common in older patients with a long history of alcohol and tobacco use.
Diagnosis. Heme-positive stools, >6-month duration of symptoms, and weight loss will
confirm diagnosis. Barium esophagography is very accurate and shows dilation of the esophagus,
which narrows into a “bird’s beak” at the distal end. The most accurate test overall (gold
standard) is esophageal manometry, which shows increased lower esophageal (LES) resting
pressure and absence of peristalsis.