Achalasia is a rare but serious condition that affects your esophagus, the tube that carries food from your throat to your stomach.
With achalasia, your lower esophageal sphincter (LES) fails to open up during swallowing. This muscular ring closes off your esophagus from your stomach most of the time, but it opens when you swallow so food can pass through. When it doesn’t open, food can back up within your esophagus.
Symptoms of this condition tend to come on gradually, and they can get worse as time goes on. Eventually, it can become difficult to swallow liquids or food, but treatment can help.
Who gets achalasia?
Achalasia is a fairly rare condition. According to
- in the United States, about 1 in every 100,000 people develop the condition each year
- elsewhere in the world, between 0.1 to 1 in every 100,000 people develop the condition each year
This condition appears to affect people of all genders at roughly the same rates. It’s less common in children:
While adults of any age can get achalasia, it most commonly develops after age 30 and before age 60.
Is achalasia serious?
Without treatment, achalasia can cause serious health complications, including:
- Megaesophagus. This refers to an enlarged and weakened esophagus.
- Esophagitis. This refers to irritation and inflammation in your esophagus.
- Esophageal perforation. A hole can form in the walls of your esophagus if they become too weak from backed-up food. If this happens, you’ll need medical treatment right away to prevent infection.
- Aspiration pneumonia. This happens when the particles of food and liquid trapped in your esophagus enter your lungs
Achalasia can also increase your chances of developing esophageal cancer.
There’s no cure for achalasia, so even with treatment, your symptoms may not go away entirely. You may need to have multiple procedures and make permanent lifestyle changes, including:
- eating smaller meals
- avoiding any foods that cause heartburn
- quitting smoking, if you smoke
- sleeping propped up instead of lying flat
Experts don’t know exactly what causes achalasia, though many believe it’s caused by a combination of factors, including:
- genetics, or family history
- an autoimmune condition, where your body’s immune system mistakenly attacks healthy cells in your body. The degeneration of nerves in your esophagus often contributes to the advanced symptoms of achalasia.
- damage to the nerves in your esophagus or LES
Chagas disease, a rare parasitic infection that mostly affects people in Mexico, South America, and Central America, has also been linked to the development of achalasia.
People with achalasia typically experience dysphagia, or trouble swallowing and feeling as if food is stuck in their esophagus. Dysphagia can cause coughing and raise your risk of inhaling and choking on food.
Other possible symptoms include:
- pain or discomfort in your chest
- unexplained weight loss
- intense pain or discomfort after eating
- dry mouth
- dry eyes
You might also have regurgitation or backflow. These symptoms can happen with other gastrointestinal conditions, such as acid reflux. In fact, people with achalasia sometimes first get an incorrect diagnosis of gastroesophageal reflux disease (GERD).
Achalasia’s rarity can complicate diagnosis of the condition, since some doctors may not immediately recognize the signs.
A doctor or other healthcare professional (HCP) might suspect you have achalasia if you:
- have trouble swallowing both solids and liquids and this difficulty worsens over time
- experience regurgitation of food
- have heartburn, chest pain, or both
They may use a few different approaches to help diagnose the condition:
- Endoscopy. In this procedure, a gastroenterologist will insert a tube with a small camera on the end into your esophagus to look for signs of achalasia. This test only leads to diagnosis in
about a thirdof achalasia cases, but an endoscopy can help rule out other conditions, like stomach or esophageal cancer.
- X-ray. An X-ray of your chest can show whether your esophagus is enlarged and keeping food trapped inside. A doctor or other HCP may also recommend a barium swallow for the x-ray. Taking liquid barium before your X-ray makes it possible for them to track how the liquid moves down your esophagus.
- Esophageal manometry (motility study). For this test, a gastroenterologist will pass a narrow tube into your esophagus through your nose. The tube will measure pressure as you swallow, revealing how the muscles of your esophagus work and whether any pressure has built up at the LES.
The order of these diagnostic tests may depend on your specific symptoms and family history, but doctors often recommend an endoscopy first.
Achalasia treatment can’t completely cure the condition, but it can help:
- improve your ability to swallow by opening the LES
- reduce other symptoms, like pain and regurgitation
- lower the chances of an abnormally enlarged esophagus
Possible treatments include:
This nonsurgical treatment involves passing a special balloon into the lower part of your esophagus and then inflating it. The balloon helps stretch out the muscles of your LES, expanding the opening so food can pass through more easily.
This procedure isn’t without risk, though. Dilation can sometimes lead to esophageal perforation, a fairly uncommon but serious complication. A perforation can be repaired, but if this happens, you’ll need surgery right away.
You’re more likely to need repeat treatments if you:
- were assigned male at birth
- are younger than 40 years old
- have respiratory concerns
- have already had the procedure at least once
Another nonsurgical option, this procedure involves injections of botulinum toxin (Botox) into your esophagus during an endoscopy. A doctor or other HCP may recommend this treatment if other treatments don’t help or you prefer to avoid surgery.
Botox blocks the nerves that typically signal your muscles to contract, so it can help relax the LES so it opens and allows food to pass through. These injections can improve symptoms quickly. The effects aren’t permanent, though, so you’ll need to have the treatment repeated within about 6 months to a year.
Potential downsides include the cost of repeated treatments, plus the fact that repeated Botox injections could
Laparoscopic Heller myotomy
In a myotomy, a surgeon will cut the LES muscle fibers to help relax it so food can pass into your stomach more easily.
Surgeons can use laparoscopic or robotic techniques to perform this surgery less invasively, through five small incisions to your abdomen. You’ll typically need anesthesia and an overnight stay in the hospital.
This surgery has a high success rate, but symptoms of GERD can develop as a possible complication. The surgeon will likely also perform a procedure to help prevent reflux, such as a partial fundoplication.
Peroral endoscopic myotomy
This newer procedure is very similar to a Heller myotomy, but the use of an endoscope makes it less invasive.
The endoscopic approach does have a drawback, though: It prevents the surgeon from doing a partial fundoplication at the same time.
In other words, you have a high risk of experiencing GERD symptoms after the procedure and may need another treatment for GERD later on.
If you can’t get surgery right away, or prefer to avoid it if at all possible, certain medications can offer some relief from your symptoms.
Medication options include:
- nitrates, which help promote relaxation of the smooth muscle making up the lower part of your esophagus
- calcium channel blockers, which can help lower LES pressure by keeping calcium from entering cells and disrupting muscle contractions
- sildenafil, a phosphodiesterase-5 inhibitor that can help lower pressure in the LES, relaxing it enough so food can pass through
These medications may involve some side effects, including:
Medications generally won’t completely improve your symptoms, either, so a doctor or other HCP will typically only recommend them as a short-term treatment.
Because of achalasia’s rarity, experts don’t fully understand how or why it occurs, or who might have a greater risk of developing the condition.
A few potential risk factors include:
- having a spinal cord injury
- getting endoscopic sclerotherapy to treat bleeding or enlarged veins
- having a viral infection
- having an autoimmune disease
- age — it’s more common in middle age and older adulthood
Future research on achalasia may help experts learn more about possible factors contributing to its development, along with strategies that might help prevent the condition.
The outlook for this condition varies. Getting a diagnosis sooner rather than later can help you get treatment to improve your symptoms before they become severe.
You may need multiple treatments before your symptoms improve. Keep in mind, though, that if one treatment doesn’t work, you do have other options to consider. A doctor or other HCP might, for example, recommend surgery if a dilation procedure doesn’t work.
Older research suggests that while achalasia can cause health complications, it doesn’t appear to have a significant impact on life expectancy.