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Achalasia

Achalasia

Achalasia, also known as esophageal achalasia, achalasia cardiae, cardiospasm, dyssynergia esophagus, and esophageal aperistalsis, is an esophageal motility disorder.[1] In this disorder, the smooth muscle layer of the esophagus has impaired peristalsis (muscular ability to move food down the esophagus), and the lower esophageal sphincter (LES) fails to relax properly in response to swallowing.[2] The most common form is primary achalasia, which has no known underlying cause. However, a small proportion occurs as a secondary result of other conditions, such as esophageal cancer or (in South America) Chagas disease.

Signs and symptoms
  • Dysphagia, or difficulty swallowing
  • Regurgitation of undigested food
  • Coughing, especially when reclining or lying down
  • Weight loss due to inadequate nutrient intake
  • Non-cardiac chest pains (NCCP), which may radiate to the back, jaw, neck, and arms
  • Some patients also experience heartburn, difficulty belching, or frequent hiccups
Diagnosis

Due to the similarity of symptoms, achalasia can be misdiagnosed as other disorders, such as gastroesophageal reflux disease (GERD), hiatus hernia, and even psychosomatic disorders.

Investigations for achalasia include

  • X-ray with a barium swallow, or esophagography. The patient swallows a barium solution, which fails to pass smoothly through the lower esophageal sphincter. An air-fluid margin is seen over the barium column due to the lack of peristalsis. Narrowing is observed at the level of the gastroesophageal junction ("bird's beak" or "rat tail" appearance of the lower esophagus). Esophageal dilation is present in varying degrees as the esophagus is gradually stretched by retained food. A five-minute timed barium swallow is useful to measure the effectiveness of treatment.
  • Manometry, the key test for establishing the diagnosis. A probe measures the pressure waves in different parts of the esophagus and stomach during the act of swallowing. A thin tube is inserted through the nose, and the patient is instructed to swallow several times.
  • Endoscopy, which provides a view inside the esophagus and stomach. A small camera is inserted through the mouth while the patient is under sedation. The endoscopist observes a "pop" as the scope passes through the non-relaxing lower esophageal sphincter.
  • CT scan may be used to exclude pseudoachalasia, or achalasia symptoms resulting from a different cause, usually esophageal cancer.

Pathological examination reveals a defect in the nerves that control the motility of the esophagus (the myenteric plexus). The esophagus is dilated and hypertrophied. In Chagas disease, the ganglion cells are destroyed by Trypanosoma cruzi, the causative parasite.[3]

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