Achalasia is a disorder of the esophagus, or food pipe, which causes the cells and muscles to lose function. This can lead to difficulties with swallowing, chest pain, and regurgitation. Food may also enter the lungs, causing coughing and breathing problems.
Achalasia can affect any part of the digestive tract, including the intestines. Hirschsprung’s disease is a type of achalasia.
People usually receive a diagnosis of oesophageal achalasia between 25–60 years of age. According to the American Journal of Gastroenterology, it affects around 1 in every 100,000 people and occurs equally in men and women.
Doctors do not know what causes achalasia, and there is currently no cure. However, treatment can relieve symptoms.
Esophageal achalasia is a chronic disease of the esophagus, which causes a slow deterioration of nerve function.
The esophagus is the tube that connects the throat with the stomach. It sits between the windpipe and the spine and continues down the neck where it joins the upper, or cardiac, end of the stomach.
When a person swallows, the muscles in the esophagus walls contract and push the food or liquid into the stomach. Glands within the esophagus produce mucus, which supports the motion of swallowing.
In esophageal achalasia, the esophagus does not open to let food pass through. This is due to a weakness of the smooth muscle in the lower part of the esophagus.
When this smooth muscle cannot move food down, this is known as aperistalsis of the esophagus.
The cause remains unknown, but according to the Society of Thoracic Surgeons, recent studies suggest it may be an autoimmune disease in which the immune system attacks nerve cells in the esophagus muscles.
A parasite in South America that leads to Chagas disease can also cause types of achalasia.
The disorder does not run in families, and the risk is equal across all ethnic groups.
At first, symptoms may be mild and easy to ignore. Eventually, however, achalasia progresses, making it harder for a person to swallow food and liquid.
The person may notice:
- dysphagia, or difficulty in swallowing food
- bringing food and liquid back up after swallowing
- coughing, especially when lying down
- chest pain, similar to heartburn, which may resemble a heart attack
- breathing difficulties when a person inhales food, liquid, and saliva into the lungs
The person may also lose weight, have difficulty burping, and feel as though they have a lump in their throat.
People may try to compensate by eating more slowly, lifting their neck, or throwing their shoulders back to assist swallowing.
However, symptoms often get worse.
The doctor may order the following diagnostic tests to rule out other conditions.
X-ray and barium swallow test: An individual swallows a white liquid solution, known as barium sulfate. Barium sulfate is visible on X-rays. As the person swallows the suspension, the solution coats the esophagus. This shows up the hollow structure of the esophagus in X-ray images.
Esophageal manometry: This measures muscle pressure and movements in the esophagus. A specialist in digestive disorders, or gastroenterologist, inserts a manometer, which is a thin tube, through the individual’s nose.
The person with suspected achalasia will need to swallow several times.
The device measures muscle contractions in various parts of the esophagus. This procedure helps the doctor determine whether the lower esophageal sphincter is relaxing properly while the person swallows.
It can also assess the function of the smooth muscle, as well as ruling out cancer.
Endoscopy: This involves using a camera on a thin, lighted tube. A gastroenterologist passes the tube down the esophagus while an individual is under sedation.
This allows the doctor to see inside the esophagus and stomach. It can show signs of achalasia or any inflammation, ulcers, or tumors that could also be causing symptoms.
During endoscopy, the doctor may also take a biopsy to check for any cancers that may be causing digestive difficulties. This involves collecting a sample of tissue and sending it to a laboratory for analysis under a microscope.
Treatment cannot cure esophageal achalasia or restore nerve function completely. However, there are ways to reduce the severity of symptoms.
Medications: If a doctor diagnoses the disorder early in its progression, medications can help dilate the narrowed part of the esophagus so that that food can pass through.
After a few months, some medications may stop working. If this occurs, a person might need to seek different treatments.
Pneumatic balloon dilation: Surgeons inflate a balloon to widen the space by tearing the muscle in the lower esophageal sphincter.
For about 70% of people, balloon treatment will relieve symptoms. This procedure may need to take place more than once. According to the American Journal of Gastroenterology, about 30% of people who undergo a pneumatic balloon dilation may require a follow-up procedure.
Adverse effects include chest pain immediately after the procedure and a small risk of perforating the esophagus. If perforation occurs, a person will need further treatment.
Balloon dilation also leads to GERD in about 15–35% of patients.
Myotomy: This is an operation to cut the muscle. It usually helps prevent obstruction.
The American Journal of Gastroenterology state that surgical myotomy has a success rate of 60–94%. However, up to 31% of people may develop GERD after a myotomy, depending on the type of procedure they have.
There is a range of different approaches to myotomy, including transabdominal myotomy, thoracoscopic myotomy, laparoscopic myotomy, and Heller myotomy.
Peroral endoscopy myotomy (POEM): The surgeon passes an electrical scalpel through an endoscope. They make an incision in the lining of the esophagus and create a tunnel within the esophageal wall.
This procedure appears to be safe and effective. However, it is a relatively new procedure, and its long term effects are unknown.
Botox: A person can receive injections of the botulinum toxin, or Botox. This can relax the muscles at the lower end of the esophagus.
Botox injections can help those who are unable or unfit to undergo surgery. A single injection provides relief for up to 6 months for about 50% of people. Many people need repeat injections after the effect of the first wears off.
Following noninvasive surgery, a person can expect to spend 24–48 hours in the hospital. They will usually be able to return to normal activities after 2 weeks.
A person who undergoes open surgery will probably need a more extended hospital stay but can resume an active lifestyle in 2–4 weeks.
After surgery or some procedures, a doctor might prescribe a medication known as a proton pump inhibitor (PPI). This can help reduce the amount of acid involved in digestion and the risk of acid reflux.
Since there is no cure for esophageal achalasia, people should seek regular follow-up appointments to detect and treat any complications in the early stages.
Acid reflux, severe enlargement of the esophagus, and squamous cell esophageal cancer are all possible complications.
The American College of Gastroenterology do not recommend regular screening by endoscopy for esophageal cancer, as studies do not suggest that this reduces the risk of progression to cancer.
However, some doctors recommend screening every 3 years in people who have had a diagnosis of esophageal achalasia for 10–15 years. Rather than identifying cancer, this is more helpful for diagnosing complications, such as an enlarged esophagus, or megaesophagus.
Megaesophagus and esophageal cancer could make it necessary for a surgeon to remove the entire esophagus. However, early detection and treatment may prevent this.
The person will probably need a liquid diet for the first few days after treatment. When swallowing becomes easier, they can move onto a solid diet.
People with achalasia should eat slowly, chew their food thoroughly, and drink plenty of water during meals. They should avoid eating meals near bedtime.
Sleeping with the head slightly raised can help gravity empty the esophagus and reduce the risk of sticking of regurgitation.
Foods to avoid include:
- citrus fruits
These may encourage reflux. Fried and spicy foods can also irritate the digestive system and make symptoms worse.
There is no specific diet for people with achalasia. However, a 2017 review suggests that a low fiber diet could reduce the bulk of food and allow it to pass more easily through the esophagus.
While researchers have much left to learn about achalasia, according to the Genetic and Rare Diseases Information Center, around 90% of people see a long term improvement in symptoms after treatment.
Sometimes, a surgeon might have to remove the entire esophagus. This occurs in approximately 10-15% of individuals.
If people start to experience swallowing difficulties, they should seek consultation as early as possible to improve their outlook.