Esophagus
Non-neoplastic disorders
Achalasia

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

Revised: 11 April 2016, last major update July 2012

Copyright: (c) 2003-2016, PathologyOutlines.com, Inc.

Cite this page: Esophagus - Achalasia. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/esophagusachalasia.html. Accessed December 8th, 2016.
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's esophagus, Candida infection, gastroesophageal reflux, lower esophageal diverticula, peptic ulceration, stricture (Ann Surg 2006;243:196)
Diagrams / Tables

Possible pathogenesis of achalasia cardia

Terminology
  • Also called cardiospasm, megaesophagus
Pathophysiology
Etiology
  • Secondary causes: Allgrove's 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
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

A CXR showing achalasia
( arrows point to the outline of the massively dilated esophagus )

Treatment
Gross Description
  • Progressive dilation of esophagus above LES, variable wall thickness
Gross Images

Dilated esophagus

Dilated esophagus

Massive dilation

Micro 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