II. Epidemiology

  1. Rare condition with Incidence <5% of cases presenting to specialty centers
  2. More common in older adults than younger patients

III. Pathophysiology

  1. Chronic progressive degenerative disorder often with delayed diagnosis
    1. Delayed diagnosis results in esophageal dilitation, resulting in cases refractory to surgical management
  2. Chronic esophageal Smooth Muscle denervation
    1. Deficient distal esophagus Cholinergic innervation due to loss of myenteric plexus (Auerbach's plexus)
    2. Decreased tissue nitric oxide
    3. Inhibitory Neuron loss
  3. Results
    1. Loss of normal esophageal peristalsis
    2. Lower Esophageal Sphincter (LES) dysfunction, preventing LES relaxation
      1. LES Incoordinate contraction
      2. LES tonic contractions
      3. Constriction of LES in response to Swallowing

IV. Types

  1. Achalasia Type 1 (Classic Achalasia)
    1. No contractility or peristalsis
    2. Lower esophageal sphincter fails to relax (all Achalasia types)
    3. Responds to Laparoscopic Heller Myotomy
  2. Achalasia Type 2 (with esophageal compression)
    1. No normal peristalsis (but some pressurizations)
    2. Lower esophageal sphincter fails to relax (all Achalasia types)
    3. Responds to all treatment options
  3. Achalasia Type 3 (Spastic Achalasia)
    1. No normal peristalsis
    2. Spastic contractions in distal Esophagus (>20% of swallows)
    3. Lower esophageal sphincter fails to relax (all Achalasia types)
    4. Responds poorly to treatment

V. Symptoms

  1. Dysphagia
  2. Chest Pain
  3. Regurgitation of food, Saliva, esophageal secretions
  4. Weight loss

VI. Diagnostics

  1. Upper Endoscopy Achalasia findings
    1. Food retained in Esophagus
    2. Increased resistance across esophagogastric junction
  2. High-Resolution Esophageal Manometry
    1. Required for Achalasia diagnosis
    2. Performed with Nasogastric Tube with closely positioned pressure sensors measure intraluminal pressure
    3. Esophageal pressures are measured as the patient swallows various foods and liquids
    4. Lower esophageal sphincter pressure is also measured before Swallowing and during relaxation
  3. Upper GI (Barium Esophagram)
    1. Proximal esophageal dilatation
    2. Beadlike or "bird beak" narrowing of distal segment at LES
    3. Spasm of lower esophageal sphincter

VII. Differential Diagnosis

VIII. Management

  1. See Esophageal Dysmotility for general measures
  2. Myotomy (definitive therapy)
    1. Laparoscopic Heller Myotomy
      1. Incises Muscles of the distal Esophagus, lower esophageal sphincter and gastric cardia
    2. Peroral Endoscopic Myotomy
      1. Newer, more technically challenging, but less invasive procedure than the laparoscopic Heller myotomy
      2. Incises the same Muscles as the Heller procedure
  3. Pneumatic dilation (by endoscopy)
    1. Disrupts lower esophageal sphincter
    2. Not as effective or longlasting as myotomy (dilation may need to be repeated)
  4. Onabotulinumtoxin A
    1. Endoscopic injection into lower esophageal sphincter
    2. Unknown efficacy, but may be used in patients at too high risk for surgery

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