Esophagus
Nonneoplastic disorders
Achalasia

Author: Elliot Weisenberg, M.D.(see Authors page)

Revised: 12 January 2018, last major update July 2012

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Esophageal achalasia[TI] pathology

Cite this page: Weisenberg, E. Achalasia. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/esophagusachalasia.html. Accessed August 19th, 2018.
Definition / general
  • Esophageal motor disorder characterized by lack of progressive peristalsis and partial / incomplete relaxation of lower esophageal sphincter (LES), preventing passage of food into stomach
  • Preferentially involves circular layer of muscularis propria, which is hypertrophied
  • Patients with achalasia may also have GERD (Eur J Gastroenterol Hepatol 2006;18:369)
  • 5% risk (33x normal) of esophageal squamous cell carcinoma, at mean 21 - 28 years after diagnosis of achalasia (Anticancer Res 2000;20:3717)
  • Also increased risk of aspiration, Barrett esophagus, Candida infection, gastroesophageal reflux, lower esophageal diverticula, peptic ulceration, stricture (Ann Surg 2006;243:196)
Terminology
  • Also called cardiospasm, megaesophagus
Pathophysiology
Etiology
  • Secondary causes: Allgrove syndrome (World J Gastroenterol 2006;12:4764), amyloidosis, Chagas disease (Trypanosoma cruzi, common in South America, destroys myenteric plexus of esophagus, duodenum, colon, ureter), diabetic autonomic neuropathy, polio, sarcoidosis, surgical ablation of dorsal motor nuclei, thyroid disease (World J Gastroenterol 2007;13:594), tumor
Diagrams / tables

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Possible pathogenesis of achalasia cardia

Clinical features
  • Most cases are primary, i.e. idiopathic, usually young adults with progressive dysphagia, nocturnal regurgitation and aspiration of undigested food
  • Can occur in children
Radiology images

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CXR shows achalasia

Gross description
  • Progressive dilation of esophagus above LES, variable wall thickness
Gross images

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Dilated esophagus

Dilated esophagus

Massive dilation

Microscopic (histologic) description
  • Early: Auerbach / myenteric plexus has lymphocytic inflammation (cytotoxic T cells, eosinophils) with germinal centers and submucosal glandular atrophy
  • Late: marked depletion / absence of ganglion cells in myenteric plexus (middle of esophagus, may be normal at LES) and replacement of nerves by collagen with muscular hypertrophy; squamous mucosa markedly hyperplastic with papillomatosis and basal cell hyperplasia resembling GERD (J Gastroenterol Hepatol 2006;21:727)
Positive stains
Electron microscopy description
  • Smooth muscle cells have nuclear and cytoplasmic inclusions, marked loss of small nerve fibers, paucity of granules in nerve fibers; also nonspecific filament disarray, mottling of myocyte fiber density, thick and long cytoplasmic dense bodies, long dense plaques (Am J Clin Pathol 1983;79:319)
Videos

Laparoscopic surgery for achalasia cardia

Differential diagnosis