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Pathophysiology of Achalasia

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Achalasia

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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.

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Author information

Authors and Affiliations

  1. Division of Gastroenterology, VA Boston Healthcare System, Harvard Medical School, 150 South Huntington Avenue, Boston, MA, 02130, USA

    Wai-Kit Lo MD, MPH

  2. Division of Gastroenterology, VA Boston Healthcare System, Harvard Medical School, Boston, MA, USA

    Hiroshi Mashimo MD, PhD

Authors
  1. Wai-Kit Lo MD, MPH
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  2. Hiroshi Mashimo MD, PhD
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Correspondence to Wai-Kit Lo MD, MPH .

Editor information

Editors and Affiliations

  1. Brigham and Women’s Hospital Harvard Medical School, West Roxbury, Massachusetts, USA

    P. Marco Fisichella

  2. Surgery, São Paulo Medical School, São Paulo, São Paulo, Brazil

    Fernando A. M. Herbella

  3. Surgery, University of Chicago, Chicago, Illinois, USA

    Marco G. Patti

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© 2016 Springer International Publishing Switzerland

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Lo, WK., Mashimo, H. (2016). Pathophysiology of Achalasia. In: Fisichella, P., Herbella, F., Patti, M. (eds) Achalasia. Springer, Cham. https://doi.org/10.1007/978-3-319-13569-4_2

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  • DOI: https://doi.org/10.1007/978-3-319-13569-4_2

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-13568-7

  • Online ISBN: 978-3-319-13569-4

  • eBook Packages: MedicineMedicine (R0)

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