What is achalasia?
Achalasia is a disorder of the esophagus—the tube that carries food from the mouth to the stomach. Achalasia makes it hard to pass food and liquids through the esophagus. The muscles of the esophagus don’t contract the way they should to move its contents. In addition, the muscle at the bottom—the lower esophageal sphincter (LES)—doesn’t relax to empty contents into the stomach. This results in retaining undigested food and liquid inside the esophagus. Other names for this condition include esophageal achalasia and cardiospasm.
Achalasia is a rare disorder. It typically begins in adults before the age of 60, but can also affect children and older people. Most people notice achalasia symptoms coming on slowly and getting progressively worse with time. The swallowing difficulty can lead to regurgitation, chest pain, and weight loss.
Treatment of achalasia aims to relax and open the LES to ease the passage of food and liquids into the stomach. Most people get relief from a procedure to dilate—or force open—the LES. However, repeat treatments are often necessary. Achalasia surgery (myotomy) is another option. Medications are helpful in some cases when other treatments are not options. Without treatment, achalasia will keep progressing and cause complications, such as pneumonia from inhaling food or liquid. See your doctor promptly is you have swallowing problems that persist or worsen.
What are the symptoms of achalasia?
Achalasia symptoms start gradually. The main one is difficulty swallowing food and even liquids or saliva. This gets progressively worse over the months or years following its onset.
Other symptoms of achalasia
Other signs and symptoms of achalasia can include:
- Anemia and other signs of malnutrition
- Chest pain that starts out mild and may come and go. Some people have more severe chest pain. The pain can worsen after eating and may also affect the back, neck or arms.
- Dry mouth and dry eyes
- Nighttime cough
- Retention of food or liquid in the esophagus leading to regurgitation of these contents. It is possible to inhale these contents into the lungs.
- Unintentional weight loss that can be significant
These symptoms can also be present with other more common swallowing disorders. Seeing your doctor is the only way to find out for sure what is causing your problems. Regardless of the cause, getting an early diagnosis generally leads to better outcomes.
What causes achalasia?
Doctors believe achalasia occurs when the nerves supplying the esophageal muscles are damaged. The damaged nerves can’t send stimulating signals to the esophageal muscles to tell them to contract. This makes swallowing difficult. As more damage occurs, the symptoms of achalasia get worse. Doctors and researchers don’t know exactly how this nerve damage occurs. Two likely explanations are a viral infection and an autoimmune process where the body’s immune system begins attacking itself. Sometimes, achalasia is the result of a tumor.
What are the risk factors for achalasia?
Anyone can get achalasia, but it tends to affect adults between the ages of 20 and 60. It affects men and women equally. However, there seems to be an inherited form of the disease that affects men twice as often as women.
Reducing your risk of achalasia
You can’t control the risk factors for achalasia, so it is not possible to prevent it. But by seeing your doctor regularly, you may be able to find achalasia early in the disease. If you have a family history of achalasia, talk with your doctor about your risk of developing the disorder.
How is achalasia treated?
The goal of achalasia treatment is to relax the lower esophageal sphincter and open the esophagus to allow food and liquids to pass more easily into the stomach. There are two main ways to accomplish this goal:
- Pneumatic dilation or balloon dilation involves inserting a balloon inside the LES and inflating it. This stretches the sphincter and makes the opening larger. Dilation is effective for most people who have it. But it’s common to need a repeat procedure at some point. Doctors use bigger balloons with repeat procedures. If dilation fails to relieve your symptoms, your doctor may recommend surgery.
- Surgical myotomy involves cutting the muscle of the LES. It is minimally invasive surgery that uses a laparoscope. The main problem with this surgery is the development of GERD (gastroesophageal reflux disease). About 15 to 20% of people will end up having GERD after this achalasia surgery. Some surgeons will perform a fundoplication to try to prevent this complication. It provides enough tightening to control reflux. Doctors may use dilation for people who continue to have achalasia symptoms after surgery.
According to the American College of Gastroenterology, balloon dilation and myotomy are equally effective. So, the choice of which one to use depends on your preference and your doctor’s experience. However, not all people will be candidates for these procedures. Usually, it’s because they are high risk for anesthesia complications. If this is the case, your doctor may recommend Botox injection of the LES or medications, such as nitrates or calcium channel blockers.
What are the potential complications of achalasia?
One of the main complications of achalasia is aspiration. This occurs when you inhale the esophageal contents into the lungs. It is more likely to happen in people for whom regurgitation is a symptom. This is especially true for people who have regurgitation and coughing during the night. The result can be pneumonia and even death in some cases.
Another potential complication of achalasia is esophageal cancer. The incidence of this cancer is considerably higher in people with achalasia. Some experts recommend screening exams if you have had achalasia for a long time—10 to 15 years. This may involve an endoscopic exam or an imaging exam.
Finally, achalasia can progress to an end-stage disease called megaesophagus. It occurs when the esophagus progressively widens to the point that it becomes quite large—6 to 8 cm in diameter. This makes it nearly impossible to clear the esophagus of its contents because it can’t generate enough pressure to move contents into the stomach. About 10 to 15% of people with achalasia will develop this condition. It requires esophagectomy to remove a section of the esophagus. The surgeon will reconstruct the section of the esophagus using part of the stomach or large intestine.