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Introduction
  • motor disorder of the distal esophagus caused by degeneration of Auerbach's (myenteric) plexus 
    • the most common motility disorder
  • Pathophysiology
    • autoimmune process leads to  loss of nitric oxide (NO) producing neurons which normally relax the sphincter muscles
      • there is an association with HLA-DQw1 
    • leads to failure of the LES to relax during swallowing
    • result is loss of peristalsis
  • Associated conditions
    • Chagas' disease
      • amastigotes destroy ganglion cells
    • scleroderma (present in 70% of patients with scleroderma)
Presentation
  • Symptoms
    • dysphagia for solids and liquids
    • nocturnal regurgitation of food
    • weight loss
Evaluation
  • Barium swallow
    • will show
      • narrowing of the distal esophagus
        • "bird's beak" appearance
      • loss of peristalsis in the distal two thirds
      • dilated proximal esophagus
  • Manometry studies 
    • will show
      • increased LES pressure
      • diffuse esophageal spasm
Differential
  •  Esophageal dysmotility
    • also seen in Systemic sclerosis or CREST syndrome 
Treatment
  • Pharmacologic
    • medications that reduce LES tone
      • medicaitons include
        • nifedipine
        • long acting nitrates
        • botulinum toxin injection
  • Surgical
    • endoscopic balloon dilation of LES
Complications
  • Esophageal carcinoma
    • ↑ risk of esophageal squamous cell carcinoma secondary to chronic irritation from food stasis
 

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