Achalasia is a type of motility disorder that affects the oesophagus. It is a rare condition occurring equally in men and women with an annual incidence of 1 in 100,000. It can occur at any age, but the peak incidence occurs between the ages of 30 and 60 (middle age and in the elderly).
The oesophagus (also called as food pipe) is a hollow muscular tube that moves the food from the mouth to the stomach. If the muscles in the oesophagus don’t contract properly, it will become difficult for the food and liquids to reach the stomach. Such conditions are called as oesophageal motility disorders.
Achalasia is one such condition, where the valve at the lower end of the food pipe (called as lower oesophageal sphincter) doesn’t relax completely, and there is a loss of peristalsis of the oesophagus.
There is a ring of muscle called as lower oesophagal sphincter (LES) at the junction where the oesophagus and the stomach meet. As food passes through the food pipe, the muscular activity of the oesophagus (peristalsis) pushes the food below and the LES relaxes to it to enter into the stomach. In those individuals with achalasia, the LES does not relax as much as it should, and the peristalsis of the oesophagus is decreased. This problem is caused due to degeneration of the nerves supplying the oesophagus.
The exact cause of the degeneration of the nerves in the oesophagus has not been determined. It may be related to heredity, or an auto-immune condition (where the body’s immune system mistakenly attacks healthy cells).
Sometimes, other conditions can cause similar problems such as cancer of the oesophagus or upper stomach and a parasitic infection called Chagas disease (seen in South America).
- Difficulty in swallowing. The main symptom of achalasia is difficulty in swallowing, is seen with both solid and liquid foods.
- Regurgitation of food. The food that is retained in the lower part of the oesophagus can cause backflow to the mouth (regurgitation). If this happens in the night, it can be dangerous as it may enter the windpipe and block the lungs (called as aspiration).
- Chest discomfort. The food retained in the oesophagus can dilate the oesophagus, cause heartburns and chest pain. Some patients confuse the pain to be a heart attack.
- Coughing may be a symptom, especially in the night and when lying down.
- Loss of weight may be seen due to decreased intake of food.
Your doctor will diagnose achalasia based on your symptoms and certain tests. The following diagnostic tests are commonly used:
- Barium swallow (Upper GI X-ray). This investigation is an x-ray imaging test used to visualise the structure of the oesophagus. The patient swallows liquid barium while series of X-ray images are obtained. The X-ray films show the movement of food in the oesophagus and any structural or function defects of the oesophagus.
- Oesophageal manometry. A device called manometer is used to measure the pressure and movements in different parts of the oesophagus. In achalasia, the pressure in the lower oesophagus and the relaxation of the lower oesophageal sphincter is measured when the patient swallows.
- Upper GI endoscopy (esophagogastroduodenoscopy or EGD): A narrow, flexible tube with a camera is inserted to examine the lining of the oesophagus, stomach and the duodenum.
Achalasia is a chronic condition that has no cure. The aim of the treatment is to decrease the pressure in the lower oesophageal sphincter muscle to allow the passage of food and liquids easily into the stomach. The treatment methods available include:
- Balloon dilation of the oesophagus (pneumatic dilation). A small balloon is inserted via an endoscope into the lower oesophagus and inflated to widen the oesophagus at the location where it is narrowed. Satisfactory results are seen in 40-85% of the patients, but repeated dilations with increasing diameter of the balloon may be needed (graded dilation).
- Myotomy Surgery. In this procedure, the affected muscular fibres in the LES are cut, so that food can easily pass into the stomach. This procedure is combined with a partial fundoplication, where a partial valve or wrap is created using stomach to reduce the risk of gastroesophageal reflux (food returning from stomach to oesophagus).
- Botulinum toxin injection. In those who cannot tolerate surgery or dilation procedures, botulinum toxin is injected to lower oesophagus through endoscopy. Botulinum toxin blocks the nerves in the lower end of the oesophagus and causes relaxation of the sphincter to allow food to pass. However, in most patients (60-90%), the results may last only 3-12 months.
- Medications. Some medications such as nitrates and calcium channel blockers can be used to relax the lower oesophageal sphincter, but they work in a very small proportion of patients and used in only those who cannot tolerate other treatments.
- Per-oral Esophageal Myotomy (POEM). Using endoscopy, a hole is made in the oesophagus to create a tunnel, and the muscles of the oesophagus are separated from the stomach muscles. The hole is then closed with a clip or suture. This technique is still under evaluation.
- Esophagectomy. In very severe or late-stage of achalasia when the oesophagus is severely dilated or tortuous, surgery may be recommended to remove the oesophagus.
- Weight loss. Due to reduced food intake by the patient.
- Aspiration. The food that is brought up can enter the lungs and cause infection in the lungs (aspiration pneumonia), which his a tough condition to treat.
- Oesophagitis. As food and fluids collect in the oesophagus, they can irritate the lining and cause inflammation which is called oesophagitis.
- Increased risk of cancer. Achalasia may increase the risk of cancer in the oesophagus, but this is not well established.
- Complications of surgery and dilation. Treatment of achalasia may cause complications such as perforation of the oesophagus and gastro-oesophageal reflux disease.
If you have symptoms of difficulty in swallowing, chest pain/discomfort and regurgitation (bringing back of food) consult a doctor for further evaluation.
Symptoms of difficulty in swallowing, chest pain, heartburn, regurgitation, hoarseness of voice, cough and weight loss can also be seen in oesophageal cancer (which is a rare condition). Consult your doctor immediately if you experience such symptoms, especially if you are an elderly person.
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- Vaezi MF, Pandolfino JE, Vela MF. ACG clinical guideline: diagnosis and management of achalasia. Am J Gastroenterol. 2013 Aug;108(8):1238-49.
- The Society of Thoracic Surgeons. Achalasia and Esophageal Motility Disorders. Accessed at http://ctsurgerypatients.org/lung-esophageal-and-other-chest-diseases/achalasia-and-esophageal-motility-disorders on 20 June 2016.
- Medline Plus. Medical Encyclopedia – Achalasia. Accessed at https://www.nlm.nih.gov/medlineplus/ency/article/000267.htm on 21 June 2016.