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Achalasia

Rare disease>Achalasia
Achalasia or acalasia is a failure of a ring of muscle (as a sphincter) to relax (completely).
The esophageal achalasia, also called simply achalasia, is a neuromuscular disorder of the esophagus characterized by the reduced ability to move food down the esophagus (peristalsis). In addition, the inability of the cardia ( also called lower esophageal sphincter)- to relax in response to swallowing (there is increased LES pressure- spasms).

Synonyms : achalasia cardiae, cardiospasm, dyssynergia esophagus, esophageal aperistalsis.

ICD code : 530.0

Table of contents

Signs and symptoms

Diagnosis

Due to the similarity of symptoms, achalasia can be misdiagnosed as other disorders, such as gastroesophageal reflux disease (GERD) and Chagas disease

  • X-ray with a barium swallow or esophagography . Shows narrowing at the level of the gastroesophageal junction ("bird beak"), and various degrees of megaesophagus.
  • Endoscopy, which provides a view from within the esophagus.
  • Manometry, the key test for establishing the diagnosis. A thin tube will be passed through the patientīs into the stomach, which measures pressure of peristalsis and LES resting pressure.
  • CT scan, which provides further visual evidence.

Complications

  1. Gastroesophageal reflux disease-GERD or heartburn.
  2. Barret esophagus or Barret mucosa: in a 10% of the patients.
  3. There is two kinds of esophageal cancer: carcinom[?] and adenocarcinom[?].There are predisposing conditions that, if present for a long time, may lead to esophagus adenocarcinom cancer, like achalasia (upto a 5% of Barrett esophagus lead to esophagus adenocarcinom).

Treatment and new Expectations

  • Balloon (pneumatic) dilation. The muscle fibers will be stretched. Gastroesophageal reflux (GERD) occurs after dilatation in 25% to 35% of patients is a risk to later Heller myotomy.
  • Medication:
    • Intra[?]-sphincteric injection of botulinum toxin (or botox), to paralyze cardia and prevent spasms. It is transitory and symptoms will return in the majority of patients within a year.
    • Drugs that reduce LES pressure such as nifedipine and nitroglycerin may be useful.
  • Heller laparoscopic surgery.
  • Transplant and artificial cardia.

See also : endoscopy, esophageal motility disorder.

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