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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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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

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 (esophageal dilation).
• 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.