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Overview

  • How common is achalasia?

    Achalasia is an esophageal motility disorder with reported global incidence and prevalence ranging from 0.03 to 1.63 per 100,000 persons per year and 1.8 to 12.6 per 100,000 persons per year, respectively. Achalasia is a rare diagnosis with only 20,000–40,000 affected patients in the United States.

    • What is achalasia?

      Achalasia is an esophageal motility disorder characterized by aberrant peristalsis and insufficient relaxation of the lower esophageal sphincter.

      • What are the symptoms of achalasia?

        Patients often present with progressive dysphagia to solids and liquids, heartburn, chest pain, regurgitation, and varying degrees of weight loss or nutritional deficiencies.

      Causes

      • What causes achalasia?

        Achalasia is an incurable disease, and the underlying etiology remains unknown. The primary etiology of achalasia is believed to be selective loss of inhibitory neurons in the myenteric plexus of the distal esophagus and lower esophageal sphincter (LES), resulting in a neuronal imbalance of excitatory and inhibitory activity.

      Treatment Options

      • What are the treatments for achalasia?

        Oral medications, botulinum toxin injection, pneumatic dilation (PD), laparoscopic Heller myotomy (LHM), per oral endoscopic myotomy (POEM) or Esophagectomy.

        Botulinium toxin is a medication that is injected in the lower esophageal sphincter during endoscopy to impair contraction of the muscle which helps swallowing.

        Pneumatic dilation is an endoscopic procedure that using a large balloon to disrupt the lower esophageal sphincter muscle.

        Laparoscopic Heller Myotomy (LHM) is a surgical procedure using small incisions to cut the lower esophageal sphincter muscle.

        Per oral endoscopic myotomy (POEM) is an endoscopic procedure in which a tunnel is made to access and cut the lower esophageal sphincter muscle.

        • Can surgery be needed in the treatment of achalasia?

          Laparoscopic heller myotomy is a primary treatment for achalasia and Esophagectomy is a salvage procedure for patient with megaesophagus that is refractory to other treatments.

          Laparoscopic Heller Myotomy (LHM) is a surgical procedure using small incisions to cut the lower esophageal sphincter muscle.

          Esophagectomy is a surgery to remove the esophagus. This is only done in severe cases of achalasia which have failed other treatments and are suffering from megaesophagus, where the food pipe is severely dilated and has food debris recurrently stick in it.

          • Effectiveness of Therapies for achalasia

            The effective and durability of achalasia treatments vary based on the specific therapy. Medications can range in effectiveness from 0-87%. Botulium toxin injection can range in effectiveness from 46.6-78.7%. Pneumatic dilation 50-93%. Surgery 60-94%. POEM >90%.

          Diagnosis

          • What is a Gastroenterologist?

            A gastroenterologist is a physician who specializes in disorders and conditions of the gastrointestinal tract. Most gastroenterologists are board-certified in this subspecialty. After completing the same training as all other physicians, they first complete at least two years of additional training in order to attain board certification in internal medicine, then gastroenterologists study for an additional 2-3 years to train specifically in conditions of the gastrointestinal tract.

            • What types of tests are needed to evaluate achalasia?

              Endoscopy, barium esophagram, and esophageal manometry are 3 well established and often complementary tests in establishing the diagnosis of achalasia.

              Endoscopy

              This test involves passing a small lighted flexible tube through the mouth into the esophagus

              Barium Esophagram

              You will be asked to swallow a liquid barium mixture. The radiologist uses a fluoroscope to watch the barium as it travels down your esophagus and into the stomach.

              You may be asked to move into various positions on the X-ray table while the radiologist watches the GI tract. Permanent pictures (X-ray films) will be made as needed.

              Esophageal Manometry or Esophageal pH

              This test involves passing a small flexible tube through the nose into the esophagus and stomach in order to measure pressures and function of the esophagus.

            Risk/Complications

            • Risks/Complications

              • Achalasia can lead to decreased quality of life, poor nutrition and damage to the esophagus.

              • Patients with long standing achalasia can have complications of megaesophagus and squamous cell carcinoma

            Key Points

            • Some Key Points to Remember about achalasia

              Achalasia is a rare disorder that presents with a constellation of common symptoms.

              Multiple medications or diagnostic tests are performed before arriving at the diagnosis of achalasia.

              Achalasia treatments are for symptom relief and not reversal of the underlying disease process.

            Self-Test

            • Do you have achalasia?

              Do you have trouble swallowing to solids and liquids that is worsening?

              Do you have heartburn, chest pain, or regurgitation?

              Are you losing weight or having nutritional deficiencies?

              Do you have suspected GERD but are not responding to acid suppression?

          Author(s) and Publication Date(s)

          Drew J. Triplett, Dayton Gastroenterology, Inc., Beavercreek, OH - Updated April 2021

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