Designed and created by Guideline Central in participation with the American College of Gastroenterology
Diagnosis and Management of Achalasia
Patient Guideline Summary
Publication Date: August 12, 2020
Last Updated: March 3, 2023
This patient summary means to discuss key recommendations from the American College of Gastroenterology (ACG) for diagnosis and management of achalasia. It is limited to adults 18 years of age and older and should not be used as a reference for children.
- Achalasia is an esophageal motility disorder. The muscular valve between the esophagus and the stomach is too tight, preventing food from entering the stomach. It is rare.
- Symptoms include progressive difficulty in swallowing of solids and liquids, heartburn, chest pain, regurgitation (acid return), and weight loss or nutritional deficiencies.
- Because heartburn presents in many patients with achalasia, patients are frequently misdiagnosed as having gastroesophageal reflux disease (GERD) and are treated with proton pump inhibitors (PPI).
- This patient summary focuses on diagnosis, treatment, and overall management of adult patients with achalasia.
- If you are initially suspected of having GERD but do not respond to acid-suppressive therapy, your doctor will probably evaluate you for achalasia.
- ACG recommends a test called esophageal pressure topography over a test called conventional line tracing for the diagnosis of achalasia.
- The Chicago Classification, which classifies achalasia subtypes by severity, may help inform prognosis and treatment choice. Types I and II have very good outcomes, whereas type III patients require a more extensive myotomy (a cut in the muscle that loosens it, allowing food to pass into the stomach).
- The goals of treatment include reducing symptoms, improving esophageal emptying, and preventing further dilation of the esophagus due to accumulation of food.
- Initial treatment includes:
- Medical therapy
- Pneumatic dilation (PD) [a procedure where an air-filled balloon stretches the lower esophageal sphincter muscle, which is too tight in achalasia.]
- Surgical myotomy
- Peroral endoscopic myotomy [POEM])
- Medication is the least effective option in achalasia. Calcium channel blockers (nifedipine 10–30 mg sublingual before meals) and nitrates (sublingual isosorbide dinitrate 5mg before meals) are the two most commonly used medications in treating achalasia.
- If you have achalasia and are candidates for definitive therapy:
- PD, laparoscopic Heller myotomy (LHM), and POEM are comparable effective therapies for type I and type II achalasia.
- POEM would be a better treatment option if you have type III achalasia.
- Botulinum toxin injection is reserved for those who cannot undergo the above definitive therapies.
- ACG suggests that POEM or PD result in comparable symptomatic improvement if you have types I or II achalasia.
- ACG recommends that POEM and LHM result in comparable symptomatic improvement.
- ACG recommends tailored (longer incision) POEM or LHM for type III achalasia as a more effective alternative disruptive therapy at the lower esophageal sphincter (LES) compared to PD.
- ACG recommends that PD is superior to medical therapy in relieving symptoms and physiologic parameters of esophageal emptying.
- ACG recommends LHM over botulinum toxin injection if you have achalasia suitable for surgery.
- Other treatments are sometimes used in special circumstances.
- ACG recommends against routinely obtaining a gastrograffin (iodinated contrast agent used instead of barium with x-ray) esophagram after dilation. This test is recommended if there is a clinical suspicion for perforation after dilation.
- ACG suggests that Eckardt score (ES) or high-resolution manometry (HRM) alone not be used to define treatment failure in evaluating continued or recurrent symptoms after definitive therapy for achalasia. ES and HRM are tests for achalasia. ES evaluates the success of treatment. HRM diagnoses achalasia.
- ACG recommends using a timed barium esophagram (TBE) (x-ray) as the first-line test in evaluating continued or recurrent symptoms after definitive therapy for achalasia.
- ACG suggests that in patients with achalasia, POEM compared with LHM with fundoplication (a surgical procedure that alters the junction between the esophagus and the stomach) or PD is associated with a higher incidence of GERD.
- ACG recommends that PD is an appropriate and safe treatment option if you have achalasia post-initial surgical myotomy or POEM in need of retreatment.
- ACG suggests that POEM is a safe option if you have achalasia and have previously undergone PD or LHM.
- ACG suggests that Heller myotomy be considered before esophagectomy if you have failed PD, and POEM be considered if the anatomy is conducive and there is evidence of incomplete myotomy.
- ACG recommends esophagectomy if you are surgically fit, have megaesophagus and have failed other interventions.
- ACG suggests against routine endoscopic surveillance for esophageal carcinoma if you have achalasia.
- ACG: American College Of Gastroenterology
- ES: Eckardt Score
- GERD: Gastro-esophageal Reflux Disease
- HRM: High-resolution Manometry
- LES: Lower Esophageal Sphincter
- LHM: Laparoscopic Heller Myotomy
- PD: Pneumatic Dilation
- POEM: Peroral Endoscopic Myotomy
- PPI: Proton Pump Inhibitor
- TBE: Timed Barium Esophagram
Vaezi, Michael F. MD, PhD, MSc, FACG; Pandolfino, John E. MD, MS, FACG; Yadlapati, Rena H. MD, MHS (GRADE Methodologist); Greer, Katarina B. MD, MS; Kavitt, Robert T. MD, MPH ACG Clinical Guidelines: Diagnosis and Management of Achalasia, The American Journal of Gastroenterology: September 2020 - Volume 115 - Issue 9 - p 1393-1411 doi: 10.14309/ajg.0000000000000731
The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.