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Achalasia

Achalasia or acalasia is a failure of a ring of muscle (as a sphincter) to relax (completely). It refers most commonly to esophageal achalasia, which 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). more...

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

Signs and symptoms

  • Dysphagia
  • Regurgitation of undigested 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.

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Esophageal pouches
From Gale Encyclopedia of Medicine, 4/6/01 by J. Ricker Polsdorfer

Definition

Esophageal pouches, also known as esophageal diverticula, are pocket-like structures formed when the interior space of the esophagus, the tube that connects the mouth to the stomach, protrudes into the walls that surround it.

Description

The esophagus is a muscular tube that propels food into the stomach. A defect in the wall of the esophagus may allow the lining to herniate, creating a space where food can be caught. Pouches can appear anywhere between the throat and the stomach. They occur primarily in men and usually later in life.

Different names for the condition apply to different locations along the esophagus:

  • Zenker's diverticula are pharyngeal pouches, or ones that occur in the upper neck area at the top of the esophagus.
  • Traction diverticula are a type of mid-esophageal pouch.
  • Epiphrenic diverticula occur at the bottom of the esophagus near where it enters the stomach.

Causes & symptoms

To propel food into the stomach (or out of it during vomiting) the esophagus generates internal pressure just like the bowel. Under certain circumstances, that pressure can herniate the esophageal lining through a weakness in the wall, creating a pouch (a balloon squeezed in the hand will herniate through the fingers in the same way). Pouches are more common in people who have motility disorders of the esophagus, swallowing that is not well coordinated and may be spastic. A traction diverticulum can develop from a scar that pulls the esophagus out of shape. Food and saliva can collect in all of these pouches.

Pouches in the neck usually cause bad breath (halitosis) and the regurgitation of swallowed food and saliva. Some patients with Zenker's diverticula can push on their neck and make old food appear in their mouth. Pouches near the stomach may cause swallowing problems, conditions known as achalasia or dysphagia. Mid-esophageal pouches usually cause no symptoms.

In the most serious cases, a person may be unable to swallow because the esophagus is obstructed, or the esphagus may rupture, spilling its contents into the chest or neck.

Diagnosis

Difficulty swallowing, bad breath, or food reappearing in the back of the mouth are among the signs physicians look for when diagnosing this condition. Sometimes the patient may also experience pain in the chest resembling a heart attack. A series of x rays taken while swallowing a contrast agent usually demonstrates the diverticulum clearly. An esophagoscopy may also be needed to gather more detail. Manometry, measuring pressures inside the esophagus using a balloon that is passed down it, may help determine the cause of the diverticula.

Treatment

Treatment for this condition is primarily aimed at alleviating symptoms. Physicians direct the patient to eat a bland diet, to chew his or her food thoroughly, and to drink water after eating to clean out the pouches. If the condition is severe, several types of surgery are available to remove the pouches and repair the defects. If a pouch is due to a stenosis (narrowing) in the esophagus it may be possible to relieve it by passing a dilator through it, a process called bougeinage.

Prognosis

The two complications that can render these nuisances dangerous, obstruction and rupture, are emergencies. Both require immediate medical attention. Other than that, diverticula will usually grow slowly over the years, gradually increasing the symptoms they cause.

Key Terms

Achalasia
Failure of the lower end of the esophagus (or another tubular valve) to open, resulting in obstruction, either partial or complete.
Contrast agent
A substance that produces shadows on an x ray so that hollow structures can be more easily seen.
Dysphagia
Difficult swallowing.
Esophagoscopy
Looking down the esophagus with a flexible viewing instrument.
Herniate
To protrude beyond usual limits.
Manometry
Pressure measurement.

Further Reading

For Your Information

    Books

  • Cecil Textbook of Medicine, edited by J. Claude Bennett and Fred Plum. Philadelphia: W. B. Saunders, 1996.
  • Current Medical Diagnosis and Treatment, edited by Lawrence M. Tierney, Jr., et al. Stamford, CT: Appleton & Lange, 1996.
  • Goyal, Raj K. "Diseases of the Esophagus." Harrison's Principles of Internal Medicine, edited by Kurt Isselbacher et al. New York: McGraw-Hill, August 1997.

Gale Encyclopedia of Medicine. Gale Research, 1999.

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