Achalasia and esophagogastric junction outflow obstruction

Anand S Jain, John E. Pandolfino

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

The physiologic hallmark of achalasia is impaired lower esophageal sphincter relaxation with absent peristalsis in the esophageal body, in the absence of a mechanical esophageal obstruction at the esophagogastric junction. The primary mechanism of achalasia is thought to be myenteric plexus inflammation with subsequent dysfunction and degeneration of inhibitory postganglionic neurons in the lower esophageal sphincter and/or distal esophagus. Three distinct subtypes of achalasia are quantitatively defined using high-resolution esophageal manometry. There is a prognostic value of the achalasia subtypes: (1) type II patients have the best prognosis with lower esophageal sphincter opening therapy; (2) the treatment response of type I patients is less robust (and reduced further as the degree of esophageal dilatation increases); and (3) type III patients have a worse prognosis with sphincter opening therapy alone and generally require additional myotomy of the associated distal esophageal body spasm. Esophagogastric junction outflow obstruction is a motor pattern defined by impaired lower esophageal relaxation but with some intact esophageal peristalsis. The mechanism of esophagogastric junction outflow obstruction is unknown; but in its idiopathic form, it is thought to be an early variant of achalasia. Definitive therapy of achalasia is mechanical disruption of the lower esophageal sphincter via Heller myotomy, per-oral endoscopic myotomy, or pneumatic dilation. Medical therapy should be reserved for those patients deemed poor candidates for a procedure due to other comorbidities or patient preferences. Medical therapy and Botulinum toxin are reasonable first-line therapeutic options for idiopathic esophagogastric junction outflow obstruction.

Original languageEnglish (US)
Title of host publicationHandbook of Gastrointestinal Motility and Disorders of Gut-Brain Interactions, Second Edition
PublisherElsevier
Pages33-45
Number of pages13
ISBN (Electronic)9780443139116
ISBN (Print)9780443139109
DOIs
StatePublished - Jan 1 2023

Keywords

  • Achalasia
  • Esophagogastric
  • Junction outflow
  • Myenteric
  • Obstruction
  • Peristalsis
  • Physiologic
  • Plexus
  • Sphincter

ASJC Scopus subject areas

  • General Agricultural and Biological Sciences
  • General Biochemistry, Genetics and Molecular Biology

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