Achalasia

ACG Releases Achalasia Guidelines

The American College of Gastroenterology (ACG) recently released guidelines on the diagnosis and treatment of achalasia, including recommendations, summaries of the evidence supporting those recommendations, and key concepts.

The ACG guideline estimates that only 20,000 to 40,000 patients in the United States are affected by achalasia, which occurs with equal prevalence in men and women and across all racial and ethnic groups. Patients most often present with dysphagia and regurgitation. As many as 42% of patients with achalasia also report heartburn, which often leads to a misdiagnosis of gastroesophageal reflux disease (GERD).

The guideline authors report, “Endoscopy, barium esophagram, and esophageal manometry are 3 well-established and often complementary tests in establishing the diagnosis of achalasia. The diagnosis of achalasia is confirmed with high-resolution manometry (HRM), which is the current gold-standard test.”

Through the use of HRM, 3 subtypes of achalasia have been identified. Achalasia type I is characterized by 100% failed peristalsis with the absence of panesophageal pressurization to more than 30 mm Hg. This is the second most common type of this disorder, representing 20% to 40% of cases. Achalasia type II—the most common form of the disorder, to which 50% to 70% of cases is attributed—is distinguished by 100% failed peristalsis with panesophageal pressurization to greater than 30 mm Hg. In the least common form of achalasia, type III (5% of cases), patients present with spastic contractions with or without periods of panesophageal pressurization.

“Endoscopic findings of retained saliva with puckering of the gastroesophageal junction or esophagram findings of a dilated esophagus with bird beaking are important diagnostic clues,” the guideline authors state.

Among the recommendations and key concepts the guidelines contain regarding diagnosing achalasia are:

  • Patients initially thought to have GERD but who do not respond to proton pump inhibitors of other GERD therapy should be evaluated for achalasia.
  • Patients should be screened for pseudoachalasia caused by masses or other obstructions before treatment for achalasia is initiated.
  • Esophageal pressure topography over conventional line tracing is preferred for the diagnosis of achalasia.
  • Using the Chicago Classification to classify subtypes of achalasia can enhance the prognosis of patients and help specify the best treatment choices to optimize outcomes.
  • The most effective nonsurgical treatment for achalasia is serial pneumatic dilation.
  • Botulinum toxin is the most effective pharmacological treatment for achalasia, but its efficacy is of limited duration.
  • Pharmacologic therapy should be reserved for those patients who are unable to undergo surgery and have failed treatment with botulinum toxin.

“Symptomatic patients with suspected achalasia should undergo upper endoscopy to ensure no other pathology and to rule out pseudoachalasia,” the authors state. “High resolution manometry and timed barium swallow should be used to confirm the diagnosis. The choice between the therapeutic modalities depends on manometric subtypes of achalasia, patient preference, and institutional expertise.”

 

—Rebecca Mashaw

 

Reference:

Vaezi MF, Pandolfino JE, Yadlapati RH, Greer KB, Kavitt RT. ACG clinical guidelines: diagnosis and management of achalasia. Am J Gastroenterol. 2020;115(9):1393-1411.  doi:10.14309/ajg.0000000000000731