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Updated: Mar 6 2023


  • Snapshot
    • A 41-year-old man presents to the clinic complaning of substernal chest pain and regurgitation of undigested food. He also reports having difficulty swallowing both solid and liquids. A barium esophgram demonstrates a dilated proximal esophagus with a narrow tapering.
  • Introduction
    • Motor disorder of the distal esophagus secondary to progressive degeneration of the Aurbach plexus (ganglion cells in the myenteric plexus)
    • Epidemiology
      • incidence of 1.6 cases per 100,000 individuals
      • demographics
        • occurs equally among men and women
        • diagnosis occurs between ages of 25 and 60 years
      • risk factors
        • Chagas disease
        • other diseases such as scleroderma (see etiology)
    • Etiology
      • the etiology of primary/idiopathic achalasia is unknown
      • secondary achalasia occurs due to diseases that cause esophageal motor abnormalities
        • Chagas disease: protozoan parasite Trypanosoma cruzi destroys intramural ganglion cells
        • other diseases include amyloidosis, sarcoidosis, scleroderma, neurofibromatosis, Fabry disease, and eosinophilic esophagitis.
    • Pathogenesis
      • inflammation and degeneration of neurons of the Aurbach’s plexus
        • the cause of the degeneration is unknown but may be autoimmune as suggested by the association with variants in the HLA-DQ regions in affected patients and the presence of antibodies to enteric neurons
      • primarily leads to loss of nitric oxide-producing, inhibitory neurons that affect the relaxation of esophageal smooth muscle
        • results in loss of normal relaxation of the lower esophageal sphincter (LES) and rise in basal sphincter pressure
        • results in aperistalsis
    • Prognosis
      • disease is progressive without treatment that ultimately leads to end-stage achalasia characterized by esophageal tortuosity, angulation, and megaesophagus (diameter >6 cm)
  • Presentation
    • Symptoms
      • dysphagia for solids and liquids
      • regurgitation
      • difficulty belching
      • vomit
      • heartburn/substernal chest pain
      • weight loss
  • Imaging
    • Radiography
      • may demonstrate mediastinal widening
    • Barium esophagram
      • not a sensitive test for achalasia, as it may be interpreted as normal in up to 1/3 of patients
      • positive findings include 1) dilation of the proximal esophagus 2) “bird-beak” appearance at the esophageal sphincter 3) aperistalsis 4) delayed emptying of barium
    • Upper endoscopy
      • may reveal dilated esophagus that contains residual material
      • esophageal mucosa usually appears normal
      • often performed after esophageal manometry to rule out malignancy
    • Esophageal manometry
      • gold standard - required to establish diagnosis
      • high-resolution manometry (vs. conventional manometry) allows for categorization of the achalasia subtype, which can guide management
      • findings include increased LES pressure, inability of the LES to relax, decreased peristalsis, and diffuse esophageal spasm
  • Differential
    • Gastroesophageal reflux disease (GERD)
      • distinguishing factor
        • regurgitated food is typically sour tasting in GERD due to the presence of gastric acid
        • will have nonspecific findings on manometry
    • Pseudoachalasia due to malignancy
      • distinguishing factor
        • may have the same manometry findings but can be differentiated from achalasia via upper endoscopy
    • Cardiovascular dysphagia
      • distinguishing factor
        • compression of the anterior esophagus by enlarged left atrium of the heart
        • normal manometry findings
  • Treatment
    • Medical management
      • Botulism toxin injections
        • High initial success but have more frequent relapses and a shorter time to relapse compared to operative treatments
        • often second-line therapy offered to patients who are high risk for complications
      • pharmacological treatments (e.g., nifedipine, nitrates, or calcium channel blockers) are often ineffective and are limited by side effects
        • indicated in patients who fail treatment with botulism toxin
    • Operative
      • preferred option for patients who have average surgical risk though the efficacy of treatments decreases over time
        • 1/3 to ½ of patients will require repeat treatment within 10 years
      • endoscopic balloon dilation of LES
        • Cure rate of 80%
        • Complication of perforation in <3% of patients
      • myotomy with fundoplication
        • Similar outcomes to that of dilation
      • peroral endoscopic myotomy (POEM)
        • new endoscopic technique that allows for myotomy of more proximal esophageal muscle
  • Complications
    • Esophageal carcinoma
      • ↑ risk of esophageal squamous cell carcinoma secondary to chronic irritation from food stasis
    • ulceration and bleeding
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