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Achalasia (cont.)

How is achalasia treated?

Treatments for achalasia include oral medications, dilation or stretching of the lower esophageal sphincter (dilation), surgery to cut the sphincter (esophagomyotomy), and the injection of botulinum toxin (Botox) into the sphincter. All four treatments reduce the pressure within the lower esophageal sphincter to allow easier passage of food from the esophagus into the stomach.

Oral medications

Oral medications that help to relax the lower esophageal sphincter include groups of drugs called nitrates, e.g., isosorbide dinitrate (Isordil) and calcium-channel blockers, e.g., nifedipine (Procardia) and verapamil (Calan). Although some patients with achalasia, particularly early in the disease, have improvement of symptoms with medications, most do not. By themselves, oral medications are likely to provide only short-term and not long-term relief of the symptoms of achalasia, and many patients experience side-effects from the medications.

Dilation

The lower esophageal sphincter also may be treated directly by forceful dilation. Dilation of the lower esophageal sphincter is done by having the patient swallow a tube with a balloon on the end. The balloon is placed across the lower sphincter with the help of x-ray, and the balloon is blown up suddenly. The goal is to stretch--actually to tear--the sphincter. The success of forceful dilation has been reported to be between 60 and 95%. Patients in whom dilation is not successful can undergo further dilations, but the rate of success decreases with each additional dilation. If dilation is not successful, the sphincter may still be treated surgically. The main complication of forceful dilation is rupture of the esophagus, which occurs 5% of the time. Half of the ruptures heal without surgery, though patients with rupture who do not require surgery still must be followed closely and treated with antibiotics. The other half of ruptures require surgery. (Although surgery carries additional risk for the patient, surgery can repair the rupture as well as permanently treat the achalasia with esophagomyotomy.) Death following forceful dilation is rare. Dilation is fast, inexpensive compared with surgery, and requires only a short hospital stay.

Esophagomyotomy

The sphincter also can be cut surgically, a procedure called esophagomyotomy. The surgery can be done using a large abdominal incision or laparoscopically through small punctures in the abdomen. In general, the laparoscopic approach is used with uncomplicated achalasia. Alternatively, the surgery can be done with a large incision or laparoscopically through the chest. Esophagomyotomy is more successful than forceful dilation, probably because the pressure in the lower sphincter is reduced to a greater extent and more reliably; 80-90% of patients have good results. With prolonged follow-up, however, some patients develop recurrent dysphagia. Thus, esophagomyotomy does not guarantee a permanent cure. The most important side effect from the more reliable and greater reduction in pressure with esophagomyotomy, is reflux of acid (gastroesophageal reflux disease or GERD). In order to prevent this, the esophagomyotomy may be modified so that it doesn't completely cut the sphincter or the esophagomyotomy may be combined with anti-reflux surgery (fundoplication). Whichever surgical procedure is done, some physicians recommend life-long treatment with oral medications for acid reflux. Others recommend 24 hour esophageal acid testing with lifelong medication only if acid reflux is found.

Botulinum toxin

The newest treatment for achalasia is the endoscopic injection of botulinum toxin into the lower sphincter to weaken it. Injection is fast, nonsurgical, and requires no hospitalization. Treatment with botulinum toxin is safe, but the effects on the sphincter often last only for months, and additional injections with botulinum toxin may be necessary. Injection is a good option for patients who are very elderly or are at high risk for surgery, e.g., patients with severe heart or lung disease. It also allows patients who have lost substantial weight to eat and improve their nutritional status prior to "permanent" treatment with surgery. This may reduce post-surgical complications.

Achalasia At A Glance
  • Achalasia is a rare disease of the muscle of the lower esophageal body and the lower esophageal sphincter.
  • The cause of achalasia is unknown; however, there is degeneration of the esophageal muscle and, more importantly, the nerves that control the muscle.
  • Common symptoms of achalasia include difficulty swallowing, chest pain, and regurgitation of food and liquid.
  • Complications of achalasia include lung problems and loss of weight.
  • Achalasia may increase the risk of cancer of the esophagus, but this is not clear.
  • Achalasia can be diagnosed by x-ray, endoscopy, or esophageal manometry.
  • Treatments for achalasia include oral medications, dilation or stretching of the esophagus, surgery, and injection of muscle-relaxing medicines (botulinum toxin) directly into the esophagus.

Last Editorial Review: 2/11/2005


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