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Achalasia pp 9-14 | Cite as

Pathophysiology of Achalasia

  • Wai-Kit Lo
  • Hiroshi Mashimo

Abstract

Achalasia is a motility disorder of the esophagus, most commonly presenting as dysphagia to solids and liquids, with gradual symptom progression. Pathophysiology arises from degeneration of inhibitory neurons of the lower esophageal sphincter (LES), resulting in unopposed tonicity and poor relaxation ability of the LES. The etiology of primary achalasia is unknown, but may involve viral, genetic, autoimmune, or neurodegenerative causes. Secondary achalasia, or pseudoachalasia, may arise from Chagas disease, malignancy associated with invasive or paraneoplastic disease, or other rare syndromes. Diagnosis is made using esophagogastroduodenoscopy, barium swallow radiography, and esophageal manometry, in order to demonstrate the effects of the underlying pathophysiologic changes and to allow for manometric subtyping which predicts treatment response. Treatment includes mechanical and biochemical methods to disrupt the pathophysiologic process. Complications of untreated disease may include overlying infectious esophagitis, esophageal diverticulosis, megaesophagus, and a slight increase in esophageal cancer risk.

Keywords

Primary achalasia Lower esophageal sphincter Neuronal degeneration Nitric oxide Vasoactive inhibitory peptide Pseudoachalasia Megaesophagus Aperistalsis 

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Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  1. 1.Division of GastroenterologyVA Boston Healthcare System, Harvard Medical SchoolBostonUSA
  2. 2.Division of GastroenterologyVA Boston Healthcare System, Harvard Medical SchoolBostonUSA

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