Rare disease>Achalasia
Achalasia or acalasia is a failure of a ring of muscle (as a sphincter) to relax (completely).
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
1 Signs and symptoms
2 Diagnosis
3 Complications
4 Treatment and new Expectations
5 External links

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. Measures the pressure induced in different parts of the esophagus and stomach during the act of swallowing.
  • CT scan, which provides further visual evidence.

Complications

  1. Gastroesophageal reflux disease-GERD or heartburn.
  2. Barrett's esophagus or Barrett's mucosa: in 10% of patients.
  3. There are two kinds of esophageal cancer: squamous cell carcinoma and adenocarcinoma. There are predisposing conditions that, if present for a long time, may lead to esophageal adenocarcinoma, like achalasia (in up to 5% of cases, Barrett's esophagus leads to esophageal adenocarcinoma).

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 myotomy laparoscopic surgery.
  • Transplant and artificial cardia.

See also : endoscopy, esophageal motility disorder.

External links