What is the pathophysiology of achalasia?

Updated: Dec 29, 2017
  • Author: Eric A Gaumnitz, MD; Chief Editor: Praveen K Roy, MD, AGAF  more...
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Answer

Achalasia is the best defined primary motility disorder and the only one with an established pathology. The predominant neuropathologic process of achalasia involves the loss of ganglion cells from the wall of the esophagus, starting at the LES and developing proximally. The degree of ganglion cell loss parallels the disease duration such that, at 10 years, ganglion cells are likely completely absent. At the LES, the loss of inhibitory nerves is demonstrated by loss of nitric oxide synthase and vasoactive intestinal peptide (VIP) immunohistochemistry staining. Variable amounts of inflammatory cells have been described within the myenteric plexus along with the disappearing nerves. In the peristaltic esophageal body, achalasia is characterized by a loss of intrinsic acetylcholine-containing nerves.


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