Achalasia

Achalasia is a rare swallowing disorder characterized by two problems with the esophagus:

• A lack of peristalsis, which is the involuntary process of propelling food from your mouth to your stomach.
• A failure of the lower esophageal sphincter (LES) to relax. Achalasia literally means “failure to relax.”

The causes of achalasia are not fully understood. It occurs mostly in men and women between the ages of 25 and 60, and affects both sexes equally. The incidence of achalasia is about one in 100,000 people per year.

Symptoms of Achalasia

Symptoms of achalasia may include:

• Difficulty with burping or swallowing
• Heartburn or chest pain
• Hiccups
• Regurgitation (reflux)
• Weight loss

How Is Achalasia Diagnosed?

Diagnosis is usually made as a result of one or more of the following tests:

• Endoscopy. In this procedure, you receive an intravenous (IV) sedative and pain medication. The physician examines the area using an endoscope—a lighted tube with a small camera at the end. The physician is able to view any abnormalities and take a tissue samples (biopsies) if necessary.
• Esophageal Manometry. This test measures the pressure inside the lower esophageal sphincter (LES). A thin tube is inserted through your mouth or nose and into your stomach. Once it is in place, the physician gently pulls the tube back into the esophagus and asks you to swallow. As you swallow, the pressure and coordination of your muscle contractions are measured. If the pressure is low or your LES is not relaxing properly, achalasia may be present. Low pressure and lack of relaxation may also indicate gastroesophageal reflux disease (GERD).
• Esophagram. An esophagram, or contrast esophagram (also called a barium swallow), is a series of x-rays of your esophagus. For an esophagram, you drink a barium sulfate liquid while x-rays are taken of the swallowing process. As the liquid moves from the mouth down your esophagus, the physician assesses for any narrowing, enlargement, or abnormalities. You will most likely be asked not to eat or drink for 8 to 10 hours prior to this test.

Treatment of Achalasia

Treating achalasia requires an interdisciplinary approach that draws on various medical specialties. At Boston Medical Center (BMC), physicians in our Department of Gastroenterology and Division of Thoracic Surgery work closely with teams in medical and radiation oncology, pathology and laboratory medicine, pulmonary medicine, and radiology to provide comprehensive, quality care.

Achalasia can be treated with:

• Medication. Nitrates and calcium channel blockers are sometimes able to relax the lower esophageal sphincter (LES) muscles enough to ease achalasia. Recent research has suggested that injections of botulinum toxin (Botox) can also relax the LES by temporarily paralyzing the hyperactive cells that cause contraction.
• Pneumatic (Balloon) Dilation. Used in patients with Barrett’s esophagus, pneumatic dilation is the process of placing a specialized balloon into the esophagus and then inflating it to create a larger opening in the esophagus.
• Myotomy. One of the most effective long-term therapies for esophageal conditions is myotomy, which is a generic term for a surgery in which a muscle is cut. In esophageal conditions, the surgeon typically cuts the muscle fibers in the lower esophageal sphincter (LES) to correct the condition. There are several ways to perform a myotomy:
  • Open surgery by means of thoracotomy or incision. When used to treat achalasia, this is called a Heller myotomy.
  • Laparoscopic surgery performed through tiny incisions in the abdomen so that it is minimally invasive. A thin, lighted tube called a laparoscope, and other surgical tools, are inserted through the incisions to allow the surgeon to work on the esophagus.
    • In robotic-assisted myotomy, a surgeon guides robotic instruments in the abdomen using a computer console. The surgeon’s natural hand movements are conveyed to the robotic instruments, which mimic the same movements that would be used if larger incisions (big enough for the surgeon’s hands) were to be used. BMC was the first medical center in New England to perform thoracic procedures—such as Heller myotomy—using robotics.
  • Peroral endoscopic myotomy (POEM), in which a flexible tube called an endoscope is inserted through the mouth and used to cut the muscles. This is the least invasive type of myotomy and doesn’t involve any incisions.