Achalasia

Rare disease>Achalasia

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Achalasia or acalasia is a failure of a ring of muscle (as a sphincter) to relax (completely).

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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 showTocToggle("show","hide") 1 Signs and symptoms 2 Diagnosis 3 Complications 4 Treatment and new Expectations 5 External links

Signs and symptoms

• Dysphagia
• Regurgitation of digested food
• Heartburn
• Chest pains : increases after eating and may radiate to the back, neck, and arms.
• Weight loss
• Coughing, especially at night or when lying down

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.

External links

• International Rare Disease Support Network Email List Group for Achalasia (http://groups.yahoo.com/group/achalasia).
• Anatomy and surgeries (http://www.besthealth.com/surgery/Achalasia_1.html).
• Medilineplus (http://www.nlm.nih.gov/medlineplus/ency/article/000267.htm).
• All about achalasia (http://donn.lbl.gov/achalasia).
• U.S. National Guideline about achalasia (http://www.guideline.gov/VIEWS/summary.asp?guideline=1423).
• Emedicine (http://www.emedicine.com/MED/topic16.htm).
• Intelihealth (http://www.intelihealth.com/IH/ihtIH/WSIHW000/9339/9405.html).
• Yahoo health encyclopedia (http://health.yahoo.com/health/encyclopedia/000267/0.html).