Achalasia (patient information)

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Achalasia
Overview
What are the symptoms?
What are the causes?
Diagnosis
When to seek urgent medical care?
Treatment options
Where to find medical care for Achalasia?
Prevention
What to expect (Outlook/Prognosis)?
Possible complications
Achalasia On the Web
Ongoing Trials at Clinical Trials.gov
Images of Achalasia
Videos on Achalasia
FDA on Achalasia
CDC on Achalasia
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Blogs on Achalasia
Directions to Hospitals Treating Achalasia
Risk calculators and risk factors for Achalasia

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Jinhui Wu, M.D. Assistant Editor-In-Chief: Meagan E. Doherty, B.S.

Overview

Achalasia is a disorder of the tube that carries food from the mouth to the stomach (esophagus), which affects the ability of the esophagus to move food toward the stomach.

What are the symptoms of achalasia?

• Backflow (regurgitation) of food
• Chest pain, which may increase after eating or may radiate to the back, neck, and arms
• Cough
• Difficulty swallowing liquids and solids
• Heartburn
• Unintentional weight loss

What are the causes of achalasia?

The main problem in achalasia is a failure of the a muscular ring where the esophagus and stomach come together (lower esophageal sphincter) to relax during swallowing.

Another part of the disorder is a lack of nerve stimulation to the muscles of the esophagus. Causes include:

• Cancers
• Damage to the nerves of the esophagus
• Infection with a parasite
• Inherited factors

As a result, the wave-like contractions of smooth muscles that normally force food through the esophagus and other parts of the digestive tract do not work as well. (These contractions are called peristalsis.)

Achalasia is a rare disorder. It may occur at any age, but is most common in middle-aged or older adults.

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.
• Fluoroscopy can be used to demonstrate the lack of peristaltic waves in the smooth-muscle portion of the esophagus. It may also reveal ‘vigorous’ achalasia, which is characterized by random spastic contractions in the esophagus.
• 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.
• Most patients should get and EGD – primarily in order to rule out malignancy (esophageal and gastric).
• Cholecystokinin (CCK) stimulation test: CCK causes mild contraction of the LES and a more pronounced release of inhibitory neurotransmitters in the wall of the esophagus. In normal people, LES tone will decrease due to the predominant effect of the inhibitory neurotransmitters. In patients with achalasia, however, the stimulatory effect on the LES is unopposed, and LES pressure increases.
• 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. '

When to seek urgent medical care?

Call your health care provider if you have difficulty swallowing or painful swallowing, or if your symptoms continue despite treatment for achalasia.

Treatment options

The approach to treatment is to reduce the pressure at the lower esophageal sphincter. Therapy may involve:

• Injection with botulinum toxin (Botox). This may help relax the sphincter muscles, but any benefit wears off within a matter of weeks or months.
• Medications, such as long-acting nitrates or calcium channel blockers, which can be used to lower the pressure at the lower esophagus sphincter
• Surgery (called an esophagomyotomy), which may be needed to decrease the pressure in the lower sphincter

Your doctor can help you decide which treatment is best for your situation.

Medications to avoid

Patients diagnosed with achalasia should avoid using the following medications:

• Alendronate
• Etidronic acid
• Hyoscyamine
• Orphenadrine
  If you have been diagnosed with achalasia, consult your physician before starting or stopping any of these medications.

Diseases with similar symptoms

• Acid reflux
• Esophageal Cancer
• Gastroesophageal Reflux Disease

Where to find medical care for achalasia?

Directions to hospitals treating achalasia

Prevention of achalasia

Many of the causes of achalasia are not preventable. However, treatment of the disorder may help to prevent complications.

What to expect (Outlook/Prognosis)?

The outcomes of surgery and nonsurgical treatments are similar. Sometimes more than one treatment is necessary.

Possible complications

• Breathing food contents into the lungs, which can cause pneumonia
• Regurgitation of acid or food from the stomach into the esophagus (reflux)
• Tearing (perforation) of the esophagus

Sources

• http://www.nlm.nih.gov/medlineplus/ency/article/000267.htm