Esophageal achalasia is an esophageal motility disorder. Achalasia can affect any part of the digestive tract, including the intestines. Hirschsprung's disease is a type of achalasia.
The cause is unknown and there is no cure, but treatment can bring relief.
What is esophageal achalasia?
In achalasia, food does not get pushed down into the stomach.
The esophagus, or gullet, is the tube that connects the throat, or pharynx, with the stomach. It is located between the windpipe, or trachea, and the spine. It goes down the neck and joins the upper, or cardiac, end of the stomach.
An adult esophagus is approximately 10 inches, or 25 centimeters, long.
When we swallow, the muscles within the esophagus walls contract and push the food or liquid down into the stomach. Glands within the esophagus produce mucus, and this helps us to swallow.
In esophageal achalasia, the esophagus does not open to let food pass through, because there is a weakness in the smooth muscle of lower part of the esophagus, and the lower esophageal sphincter. The inability of the smooth muscle inside the esophagus to move food down is known as aperistalsis of the esophagus.
Achalasia is a chronic disease in which the nerve function deteriorates over time.
The cause remains unknown, but according to Society of Thoracic Surgeons, recent studies suggest it may be an autoimmune disease. In this kind of disease, a person's immune system mistakenly attacks healthy cells in the nervous system of the esophagus. A parasite in South America that leads to Chagas disease can also cause it.
It does not run in families.
At first, symptoms may be minor and easy to ignore, but eventually, it becomes harder to swallow food and liquid.
The person may notice:
- Dysphagia, or difficulty in swallowing food
- Regurgitation of indigested food, and later, liquid
- Coughing, especially when lying down
- Chest pain, similar to heartburn, which can be confused with a heart attack
- Aspiration, when food, liquid, and saliva are inhaled into the lungs
The person may also lose weight, have difficulty burping and feel as if they have a lump in their throat.
Symptoms are usually mild and ignored at first, and people may try to compensate by eating more slowly or by lifting the neck or throwing the shoulders back to help them swallow.
However, symptoms typically get progressively worse.
The doctor may order the following diagnostic tests:
X-ray and barium swallow test: The patient swallows a white liquid solution, known as barium sulfate. Barium sulfate is visible to x-rays. As the patient swallows the suspension, the esophagus becomes coated with a thin layer of barium, enabling the hollow structure to be imaged.
Esophageal manometry: This measures muscle pressure and movements in the esophagus. A pressuring device called a monometer is used. A thin tube goes through the patient's nose, and they have 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 patient swallows. It can also tell how well the smooth muscle is working. It can also rule out cancer.
Endoscopy involves a camera on a thin, lighted tube, that is passed down the digestive channel, while the patient is sedated. This allows the doctor to see inside the esophagus and stomach. It can show signs of achalasia, but it can also reveal any inflammation, ulcers, or tumors.
During endoscopy, a biopsy may be taken.
Treatment cannot cure achalasia or restore the nerve function, but it can reduce symptoms.
Nerve damage means the muscles do not push the food through the digestive system.
Medications: If diagnosis is made early, medications can help dilate the narrowed part of the esophagus so that food can pass through properly. Examples include calcium channel blockers and nitrates. Some patients may experience headaches and swollen feet, and after some months, some medications stop working.
Balloon, or pneumatic, dilation: A small balloon is passed into the narrowed section and inflated to widen the space by tearing the muscle in the lower esophageal sphincter. This may need to be done more than once. For about 60 percent of patients, one balloon treatment is sufficient for a year, and for around 25 percent, the effects last for 5 years.
Complications include chest pain immediately after the procedure, and a small risk of perforating the esophagus, which will need further treatment. Balloon dilation also leads to GERD in about 2 percent of patients.
Myotomy: An operation to cut the muscle usually helps prevent obstruction. It has a success rate of between 70 percent and 90 percent. Symptom relief will last for 10 years in 85 percent of cases, and 65 percent of people will have relief for 20 years.
Peroral endoscopy myotomy (POEM): The surgeon passes an electrical scalpel through an endoscope, makes an incision in the lining of the esophagus and creates a tunnel within the esophageal wall. This procedure appears to be safe and effective, but its long-term effects are unknown, as it is a relatively new procedure.
Botox: This can be given as injections to relax muscles on the lower part of the esophagus. Botox injections can help those who are unable or unfit to undergo surgery. A single injection provides relief for 3 months in 65 to 90 percent of patients, but then it must be repeated.
Following noninvasive surgery, the patient can expect to spend between 24 hours and 48 hours in the hospital and to return to normal activities after 2 weeks. A person who undergoes open surgery will probably need a longer hospital stay, but they will be up and about in 2 to 4 weeks.
After surgery or some procedures, proton pump inhibitors (PPIs) may help prevent gastric acid secretion, and this can prevent reflux.
Since achalasia cannot be cured, patients should seek regular followup to detect and treat any complications in the early stages.
Acid reflux, severe enlargement of the esophagus, known as mega-esophagus, and squamous-cell esophageal cancer are all possible complications.
Some experts suggest using endoscopy to screen for these complications once every 3 years in people who have had achalasia for at least 10 to 15 years.
However, the American College of Gastroenterology does not recommend regular screening by endoscopy for esophageal cancer.
Mega-esophagus and cancer could make it necessary to remove the entire esophagus, but early detection and treatment may prevent this.
The patient will probably need a liquid diet for the first few days after treatment. When swallowing gets easier, they can move onto a solid diet.
Yogurt is easy to swallow and may help minimize stomach acid.
Patients should eat slowly, chew their food thoroughly, and drink plenty of water during meals.
Meals should not be consumed near bedtime. Sleeping with the head slightly raised can help gravity to empty the esophagus so that food does not regurgitate or stay stuck.
Foods to avoid include citrus fruits, alcohol, caffeine, chocolate, and ketchup, as these encourage reflux. Fried and spicy foods can also irritate the digestive system and worsen symptoms.
Anecdotal evidence suggests that the following foods may help:
- Softer foods, such as soups, mashed vegetables, or porridge
- Ginger may aid digestion and prevent heartburn
- Peppermint in teas and yogurts can help reduce gastric secretion
- At least 10 glasses of water daily, to keep hydrated
It is important to maintain a balanced diet that contains all the nutrients and fiber needed for healthy living.