Epidemiology


Etiology


Pathophysiology


  • Atrophy of inhibitory neurons in the Auerbach plexus → lack of inhibitory neurotransmitters (e.g., NO, VIP) → inability to relax and increased resting pressure of the LES, as well as dysfunctional peristalsis → esophageal dilation proximal to LES
  • The myenteric plexus is a collection of enteric ganglia located between the inner circular and outer longitudinal muscle layers.

Clinical features


Diagnostics


  • Esophageal barium swallow: supportive and/or confirmatory test
    • Bird-beak sign: dilation of the proximal esophagus with stenosis of the gastroesophageal junction
    • Delayed barium emptying or barium retention

Treatment