ZSFG AM Report Pearls 6/3/2016: Achalasia and Pseudo-Achalasia

Achalasia

  • Epi: Rare! Incidence 1.6/100,000 people/year. Age of onset 25-60.
    • Cello Pearl: new onset achalasia in a person >70 is almost always pseudoachalasia, often from extrinsic esophageal compression
  • Definition: true achalasia is an idiopathic loss of peristalsis in the distal esophagus + failure of Lower Esophageal Sphincter Relaxation
  • Classic presentation: dysphagia (solids>liquids), difficulty belching, chest pain, and regurgitation is delayed.
  • Diagnosis: made with esophageal manometry though EGD, barium swallow or CT chest/abdomen can support findings.
    • Rule out pseudoachalasia due to malignancy in people >60, with significant weight loss, or atypical manometry finding. This diagnosis can be tricky!
  • Treatment: options include balloon dilation, surgical myotomy and/or esophageal botox injections.
  • Bonus pearl: ppl w primary achalasia have 16 fold lifetime increased risk of esophageal cancer

Pseudo-achalasia

  • Definition: disorders that mimic achalasia either due to esophageal compression or infiltration of the esophageal nervous system
    • Esophageal compression: malignancy, often gastric or peridiaphragmatic mets
    • Nervous system infiltration: Chagas, amyloidosis, sarcoidosis, neurofribromatosis, eosinophilic gastroenteritis,
    • Chronic idiopathic intestinal pseudo-obstruction
  • Cello pearl: Thoracic aortitis can rarely cause pseudo-achalasia. In the era of untreated tertiary syphilis, syphilitic aortitis was notorious for it.
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