Achalasia
Achalasia occurs when nerves in the esophagus become damaged. As a result, the esophagus becomes paralyzed and dilated over time and eventually loses the ability to squeeze food down into the stomach. Food then collects in the esophagus, sometimes fermenting and washing back up into the mouth, which can taste bitter. Some people mistake this for gastroesophageal reflux disease (GERD). However, in achalasia the food is coming from the esophagus, whereas in GERD the material comes from the stomach.
There’s no cure for achalasia. Once the esophagus is paralyzed, the muscle cannot work properly again. But symptoms can usually be managed with endoscopy, minimally invasive therapy or surgery.
Symptoms
Achalasia symptoms generally appear gradually and worsen over time. Signs and symptoms may include:
- Inability to swallow (dysphagia), which may feel like food or drink is stuck in your throat
- Regurgitating food or saliva
- Heartburn
- Belching
- Chest pain that comes and goes
- Coughing at night
- Pneumonia (from aspiration of food into the lungs)
- Weight loss
- Vomiting
Causes
The exact cause of achalasia is poorly understood. Researchers suspect it may be caused by a loss of nerve cells in the esophagus. There are theories about what causes this, but viral infection or autoimmune responses have been suspected. Very rarely, achalasia may be caused by an inherited genetic disorder or infection.
Diagnosis
- Barium swallow: For this test, you’ll swallow a barium preparation (liquid or other form) and its movement through your esophagus is evaluated using X-rays. The barium swallow will show a narrowing of the esophagus at the LES.
- Upper endoscopy: In this test, a flexible, narrow tube with a camera on it – called an endoscope – is passed down your esophagus. The camera projects images of the inside of your esophagus onto a screen for evaluation. This test helps rule out cancerous (malignant) lesions as well as assess for achalasia.
- Manometry: This test measures the timing and strength of your esophageal muscle contractions and relaxation of the lower esophageal sphincter (LES). Failure of the LES to relax in response to swallowing and lack of muscle contractions along the walls of the esophagus is a positive test for achalasia. This is the “gold standard” test for diagnosing achalasia.
MANAGEMENT AND TREATMENT
Treatment
Nonsurgical treatment
- Pneumatic dilation. A balloon is inserted by endoscopy into the center of the esophageal sphincter and inflated to enlarge the opening. This outpatient procedure may need to be repeated if the esophageal sphincter doesn’t stay open. Nearly one-third of people treated with balloon dilation need repeat treatment within five years. This procedure requires sedation.
- Botox (botulinum toxin type A). This muscle relaxant can be injected directly into the esophageal sphincter with an endoscopic needle. The injections may need to be repeated, and repeat injections may make it more difficult to perform surgery later if needed.Botox is generally recommended only for people who aren’t good candidates for pneumatic dilation or surgery due to age or overall health. Botox injections typically do not last more than six months. A strong improvement from injection of Botox may help confirm a diagnosis of achalasia.
- Medication. Your doctor might suggest muscle relaxants such as nitroglycerin (Nitrostat) or nifedipine (Procardia) before eating. These medications have limited treatment effect and severe side effects. Medications are generally considered only if you’re not a candidate for pneumatic dilation or surgery, and Botox hasn’t helped. This type of therapy is rarely indicated.
Surgery
- Heller myotomy. The surgeon cuts the muscle at the lower end of the esophageal sphincter to allow food to pass more easily into the stomach. The procedure can be done noninvasively (laparoscopic Heller myotomy). Some people who have a Heller myotomy may later develop gastroesophageal reflux disease (GERD).To avoid future problems with GERD, a procedure known as fundoplication might be performed at the same time as a Heller myotomy. In fundoplication, the surgeon wraps the top of your stomach around the lower esophagus to create an anti-reflux valve, preventing acid from coming back (GERD) into the esophagus. Fundoplication is usually done with a minimally invasive (laparoscopic) procedure.
- Peroral endoscopic myotomy (POEM). In the POEM procedure, the surgeon uses an endoscope inserted through your mouth and down your throat to create an incision in the inside lining of your esophagus. Then, as in a Heller myotomy, the surgeon cuts the muscle at the lower end of the esophageal sphincter.POEM may also be combined with or followed by later fundoplication to help prevent GERD. Some patients who have a POEM and develop GERD after the procedure are treated with daily oral medication.