Harvey's Garden

Achalasia

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aliases

2 min read

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

  • Initial Test: Barium esophagram
    • Findings: Smooth, symmetric tapering at the gastroesophageal junction (“bird’s beak” sign), dilated proximal esophagus, pooling/stasis of contrast, loss of peristalsis.
  • Mandatory Next Step: Upper Endoscopy (EGD) c
    • Essential to exclude pseudoachalasia (malignancy).
    • Primary achalasia: Normal mucosa, dilated esophagus with retained food; endoscope passes through LES with gentle pressure (“pop”).
    • Malignancy: Rigid, non-negotiable LES, nodular/friable mucosa, unable to pass endoscope easily.
  • Confirmatory / Gold Standard: High-Resolution Esophageal Manometry (HRM)
    • Findings:
      1. Incomplete/absent LES relaxation (elevated integrated relaxation pressure [IRP]).
      2. Aperistalsis in the distal two-thirds of the esophageal body.
      3. Elevated resting LES pressure (>45 mm Hg) often present.
  • Biopsy: Done during EGD only if mucosal irregularity or mass is visualized.

Treatment

  • Low Surgical Risk Patients (First-line / Definitive):
    • Laparoscopic Heller Myotomy: Surgical division of LES muscle fibers; typically combined with partial fundoplication (Dor or Toupet) to prevent post-op GERD. Preferred in young patients (<40–45 yrs).
    • Pneumatic Balloon Dilation: Disruption of circular muscle fibers by balloon expansion. High efficacy; risk of esophageal perforation (~2–3%).
    • Peroral Endoscopic Myotomy (POEM): Endoscopic submucosal tunneling technique; comparable efficacy to Heller myotomy.
  • High Surgical Risk / Poor Procedural Candidates (Second-line):
    • Endoscopic Botulinum Toxin Injection: Inhibits acetylcholine release at the LES. Effective short-term (~6–12 months); requires repeat injections. Induces scarring that complicates future myotomy.
  • Refractory / Medical Therapy (Last resort / Bridge):
    • Oral smooth muscle relaxants before meals: Calcium channel blockers (e.g., nifedipine) or long-acting nitrates (e.g., isosorbide dinitrate). Limited efficacy; high rates of hypotension and headache.
  • End-stage Achalasia (Megaesophagus/Sigmoid Esophagus):
    • Esophagectomy.

Graph View

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