Achalasia

Presentation

• Dysphagia to solids AND liquids
  
• Chest Pain after eating (unsynchronized peristalsis)
  
• Vomiting of undigested food

Definition

• Chronic, incurable disease
  
• Characterized by incomplete or absent relaxation of the lower esophageal sphincter and a peristalsis of the esophageal body affects the swallowing of both LIQUIDS AND SOLIDS (if just solids, think strictures or tumors, both, think motility disorders) results in a DILATED ESOPHAGUS
  
• DEFICIENCY IN POST-GANGLIONIC NEURONS OF THE MYENTERIC PLEXUS

Pathophysiology

• Destruction of ganglion cells of the esophagus (myenteric plexus) and LES which impairs relaxation of LES and stops motility of esophagus
• AUTOIMMUNE DESTRUCTION OF MYENTERIC NERVES IN THE LES (myenteric nerves are postganglionic)
  
• Inflammatory response causes damages to ganglion cells suggesting autoimmune disorder

Risk

• Aspiration pneumonia is common

Diagnoses

• Barium swallow
  • 95% of pts will have positive barium swallow
    
  • “birds beak”
• Esophageal manometry
  • absence of esophageal peristalsis
    
  • failure of LES to relax

Treatment

• Main goal: relax LES
• Nitrates and Ca+ channel blockers
  • Induce LES relaxation but no long term benefit
• Botulism Toxin (BT):
  • works by inhibiting the release of ACh in muscle synapses and thereby inhibits contraction of LES
  • 90% effective at one month, 35% effective at one year
• Pneumatic dilation:
  • goal is to disrupt the muscle fibers of the LES without causing full-thickness perforation
  • multiple dilations needed in first year
  • less prone to recur than BT (30% vs 65% at one year)
• Combined BT and dilation:
  • not better than dilation alone
• Esophageal myotomy:
  • Laparoscopic Heller Myotomy
  • Increases risk of reflux therefore a fundoplication (anti-reflux procedure) is also done
  • 90% long term effective
  • Treatment of choice for pts who are GOOD SURGICAL RISK