Achalasia: Types, Symptoms, Causes, Treatment, Diet, Diagnosis, Prevention

The tube that connects the throat with the stomach is called esophagus. Achalasia is a condition when the esophagus is severely affected. The lower esophageal sphincter or LES is a valve that opens up when food is swallowed, but in patients with achalasia, LES fails to open up during swallowing. This makes the swallowed food to dump in the esophagus region. This situation may be due to damage of nerves in the esophagus region thereby hampering the normal activity of the LES. As a result, the LES muscle fibers can’t get relevant signal through the nerves to move in time.

According to the manometry test, achalasia has 3 types:

• Achalasia Type I: This type shows no motility in the esophagus and the presence of a high-pressure band near the lower esophageal sphincter level.
• Achalasia Type II: Type II reveals the lower esophageal sphincter as always non-relaxed and simultaneous contractions while a person swallows.
• Achalasia Type III: The 3rd type of Achalasia is typically denoted by high-pressure spams in the esophagus.

People with Achalasia often have symptoms of trouble in swallowing or feel like food is stuck in their esophagus. This symptom can cause frequent coughing while taking food and raises the risk of choking on food, inhalation of food and aspiration.

Some other common symptoms of Achalasia are as follows:

• Discomfort or pain in your chest
• Heartburn
• A feeling of discomfort or pain right after eating
• Loss of weight in a steady rate.

Patients might also have regurgitation or backflow of eaten food and sometimes such regurgitation may be accompanied with blood.

Normally, LES relaxes when food is swallowed to allow food to pass into the stomach. People affected with achalasia are unable to relax the LES, as well as fail to perform normal muscular activity of the esophagus called peristalsis. Achalasia is generally caused due to damage in the nerves of the esophagus.

A few other illnesses, such as esophageal cancer or cancer of upper stomach, and Chagas disease, can cause similar problem in the esophagus. Only, correct diagnosis can differentiate achalasia from these diseases.

Achalasia is a rare illness, which mostly occurs in older people and in some cases researchers have also detected that it’s inherited, though its genetic relation is not established yet.

The above stated primary symptoms of achalasia are not sufficient to diagnose the disease precisely, but some other tests are needed to confirm the disease. The tests include:

• Chest x-rays: A chest x-ray may expose an enlarged esophagus and some abnormalities in the stomach. However, it is not sufficient for a diagnosis of achalasia and hence, some more intense testing is required.
• Barium Swallow Test: This test is a common screening test for achalasia. The barium X-ray clearly shows the outline of the esophagus and LES. The characteristic findings of achalasia in barium testing include a persistently tapering part at the end of the esophagus where LES is located, with a dilated esophagus above the narrowed region.
• Esophageal Manometry: Manometry is a special test that measures the changes in pressures in the esophagus caused by the contraction and rarefaction of the muscles adhered to the esophagus. In this test, a thin tube is introduced in the esophagus which contains a number of pressure sensors. Patients are given water to sip while the tube is placed inside to measure the changes in pressure. Esophageal Manometry is used to confirm the diagnosis of achalasia.
• Endoscopy: This test makes the inside of esophagus, LES, and stomach visible using a specially designed lighted, and flexible tube. This test is frequently recommended by the medical practitioners for the patients with suspected achalasia and also useful for detecting other conditions that have similar symptoms like achalasia.

Several treatment options are available for achalasia, but these treatments can’t reverse the damages that already took place in the nerves or loss of nerve cells in the esophagus. The treatments are applied to improve the symptoms of Achalasia. The available treatments can only restore peristaltic movements in the esophagus of the patients with achalasia. Through these treatment procedures, the LES muscle is weakened so that they no longer create any barrier to the passage of swallowed foods to the stomach. Here are some commonly adopted treatment procedures for Achalasia-

• Drug Therapy for Achalasia: Mainly two types of drugs composed of nitrates and calcium channel blockers are recommended 10 -30 minutes before taking a meal to relax the LES muscle. These drugs can decrease symptoms in people with achalasia. Drug therapy is the most common form of treatment for achalasia. However, the effectiveness of the drug therapy diminishes with time and also shows some side effects, such as headache and low BP. Drug therapy is prescribed initially for those patients who are reluctant, panicked or not healthy enough to take mechanical treatments, such as balloon (pneumatic) dilation and myotomy.
• Balloon (Pneumatic) Dilatation: In this process at first a collapsed balloon is positioned in the LES, it is then inflated rapidly to split the muscle of the LES. This procedure relieves the swallowing trouble in the patients with achalasia. Many patients require more than one balloon dilation for getting sufficient relief. The success rate of balloon dilation is quite fair. With single balloon dilation 60% patients stay fit even after one year and 25% patients stay fit even after 5 years. About 15% patients experience severe chest pain and fever after a session of balloon dilation.
• Surgery to Treat Achalasia: The surgery involved in achalasia is called Myotomy, in this process the muscle fibers attached with LES are cut to make the LES easy or less obstructive. In one such surgical technique called Heller myotomy, the muscle fibers attached with the LES at the link of the esophagus and the stomach are operated through laparoscopic procedure. Patients are given local anesthesia, and need to stay in hospitals for a maximum period of two days. The success rate of myotomy is remarkable, on an average 80% patients get relief from the symptoms of achalasia and 85% of patients are reported to be leading normal life even after 10 years and 65% are reported to be in fine condition 20 years after the operation.
• Peroral Endoscopic Myotomy: The most modern introduction in the treatment procedure of achalasia is Peroral endoscopic myotomy or POEM. In this process, an electrical scalpel is passed through the endoscope to make a slit in the inside layer of the esophagus to create a tunnel within the wall of the esophagus. The endoscope is then advanced through the tunnel, and the esophageal muscle fibers are cut using the electrical scalpel device that is passed along with the endoscope. This process is comparatively a very modern introduction in the treatment of achalasia and too few medical experts are accustomed and trained in this process, hence the treatment is not available everywhere.
• Botulinum toxin Injection: Botulinum toxin injections are used to temporarily relax or paralyze the adjoining nerves in the LES so that the signals that are making LES to contract are not available to the nerve cells in that region. It helps to relieve the obstruction temporarily, thus enabling the patient to eat food without any visible obstruction. A single dose of botulinum toxin injection relieves most of the symptoms of achalasia in 70 to 90 percent of the patients for a period of three months to one year.

The primary causes of achalasia cannot be prevented and researchers are still trying to find the reasons behind the occurrence of such malfunctioning of LES muscle. However, the treatment procedures can mitigate the symptoms to a large extent.

The management of the patient detected with achalasia and nutritional problems they are facing is quite similar to that of patients with dysphagia and esophagogastric cancer. Oral feeding is the most relevant solution which needs to be continued as much as possible. For patients with mild to moderate achalasia, normal diet is mildly affected and, the immediate family members should encourage the patients to follow dietary modifications, so the patients don’t face malnutrition or weight loss. Food should be completely individualized for the Achalasia patients. Food needs to be finely chopped, or puréed and fluids may be thickened as much as possible to make the meals easily consumable. One can also start have more fluids along with the food for easy movement of the food through the esophagus.

If Achalasia patient can’t eat and drink in sufficient quantities, then tube feeding is the best option for providing sufficient quantities of fluids and nutrients. Some long term complications may occur from the tube feeding that include infections in the esophagus and tube obstruction. Patients who are fed through gastric tube, there remain a possibility of pulmonary aspiration and in such situations intrajejunal feeding procedure is recommended for these cases.

Achalasia is a typical disease, and its occurrence is rare too. In USA, almost 3000 new cases of achalasia are reported every year. Due to insufficient data and information from different parts of the world, the prognosis of Achalasia is not so obvious to the medical fraternity. Some modern scientific treatment processes and oral medicines have been found to be providing sufficient relief from the symptoms, but here again the positive effect of these treatment procedures vary from patient to patient.