Achalasia: New Guidelines Address Diagnosis, Treatment

Larry Hand

August 01, 2013

Clinicians have new guidelines for the diagnosis and treatment of achalasia, a primary motor disorder of the esophagus. The guidelines were published online July 23 and in the August issue of the American Journal of Gastroenterology.

Michael E. Vaezi, MD, PhD, from the Division of Gastroenterology, Hepatology, and Nutrition, Vanderbilt University Medical Center, Nashville, Tennessee, and colleagues compiled the guidelines after a review and grading of available evidence and obtaining expert opinion.

Achalasia, an incurable disease, occurs equally among men and women (incidence, 1 in 100,000 people). It is characterized by insufficient relaxation of the lower esophageal sphincter and difficulty in swallowing and is often misdiagnosed as gastroesophageal reflux disease (GERD), the authors write.

"Achalasia must be suspected in those with dysphagia to solids and liquids and in those with regurgitation unresponsive to an adequate trial of proton pump inhibitor (PPI) therapy," they note. Symptoms that may contribute to misdiagnosis include substernal chest pain during meals, unintended weight loss, and heartburn.


The new guidelines for proper diagnosis include:

  • Performing an esophageal motility test on all patients suspected of having achalasia;

  • using esophagram findings to support a diagnosis;

  • using barium esophagram, as recommended for patients with equivocal motility testing; and

  • endoscopic assessment of the gastroesophageal junction and gastric cardia, as recommended, to rule out pseudoachalasia.

The manometric finding of irregular muscle contractions and incomplete lower esophageal sphincter relaxation without mechanical obstruction "solidifies the diagnosis of achalasia in the appropriate setting," the authors write.


Treatment recommendations offer a tailored approach:

  • initial therapy should be either graded pneumatic dilation (PD) or laparoscopic surgical myotomy with a partial fundoplication in patients fit to undergo surgery;

  • procedures should be performed in high-volume centers of excellence;

  • initial therapy choice should be based on patient age, sex, preference, and local institutional expertise;

  • botulinum toxin therapy is recommended for patients not suited to PD or surgery; and

  • pharmacologic therapy can be used for patients not undergoing PD or myotomy and who have failed botulinum toxin therapy (nitrates and calcium channel blockers most common).

"Surgical myotomy has shown excellent results in most patients and remains the surgery of choice, with more being done laparoscopically. The benefit of adding a fundoplication was demonstrated in a double-blind randomized trial comparing myotomy with versus without fundoplication," Dr. Vaezi said in a news release.

"A subsequent cost–utility analysis based on the results of this trial found that myotomy plus Dor fundoplication was more cost effective than myotomy alone because of the costs of treating GERD."

Retreatment of "a good proportion" of patients is likely to be required within 5 years, and that retreatment also should be individualized and based on local expertise, the authors write.

The guidelines also call for follow-up for symptom relief and esophagus emptying results through use of barium esophagram. Endoscopy surveillance for esophageal cancer is not recommended.

The authors have disclosed no relevant financial relationships.

Am J Gastroenterol. Published online July 23, 2013. Abstract


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