Diseases

Achalasia – Causes, Symptoms & Treatment

Achalasia

Achalasia is a very rare disease that can cause some symptoms to the affected person Physicians speak of achalasia when there is a malfunction in hollow organs with a sealing function.

What is achalasia?

The term achalasia refers to a food passage disorder of the esophagus. The disease manifests itself primarily through swallowing disorders, which usually worsens as the disease progresses. For this reason, achalasia is divided into three stages: stage I (hypermotile form), stage II (hypomotile form) and stage III (amotile form).

In its frequency, the disease occurs very rarely. Only 1 in 100,000 people develop Achalsia. It is not determined by age and gender. Any person between the ages of 20 and 40 can theoretically suffer from this disease.

Causes

The causes of the disease cannot be named at this time. Medicine divides the disease into primary and secondary achalasia. However, it is suspected that it is an autoimmune disease . Certain antibodies that have a negative effect on the autonomic nervous system could be detected in some patients with primary achalasia .

It is also suspected that pathogens such as the measles virus , varicella-zoster virus or human papillomavirus can be the cause of the disease. Hereditary and psychological factors could also play a role. In general, the degeneration of nerve cells of the myenteric plexus Auerbach is responsible for the dysfunction of the esophagus. Inflammatory reactions often support degeneration.

Secondary achalasia can result from either carcinoma or Chagas disease, which is common in the tropics. Gastroenteritis and various other diseases related to the digestive tract can also be the cause of secondary achalasia.

Symptoms, Ailments & Signs

Esophageal dysfunction can cause a variety of symptoms. Dysphagia and pain in the esophagus are typical. Sometimes the pain radiates to the chest and causes increasing discomfort. Achalasia also causes belching of undigested food associated with heartburn and nausea .

At the beginning, the disease initially causes slight difficulty in swallowing , which occurs mainly with solid food: the affected person has the feeling that the food is stuck in the throat. As the disease progresses, those affected find it difficult to absorb liquid food. This can lead to deficiency symptoms, which manifest themselves in the form of dizziness and tiredness .

In addition, leftover food can get into the respiratory tract and cause other symptoms such as pneumonia . A functional disorder of the esophagus is often expressed by involuntary, mostly cramping pain behind the breastbone, which resembles heart problems . Over time, achalasia leads to weight loss .

Externally, the disease manifests itself primarily in the characteristic symptoms, i.e. pale skin, sunken eye sockets and increased sweating. The voice of those affected is usually hoarse and brittle, which can give the impression of a cold . In severe cases, there may be a temporary loss of voice.

Diagnosis & History

The diagnosis of achalasia is usually carried out in the early stages with a pressure measurement (manometry), which is carried out in several sections of the esophagus. Insufficient opening (relaxation) of the lower sphincter of the esophagus can be determined.

In addition, there is usually a disruption in the pumping function of the esophagus. In the late stages of the disease, the patient is given a barium meal (contrast meal). The findings can then be made by means of an X-ray examination . Routine diagnostics also include endoscopy of the upper digestive tract or a gastroscopy so that other causes of the disease can be ruled out.

The course of the disease usually goes through all three stages. The hypermotile form of achalasia is characterized by the fact that the esophagus is still functional. However, it tries to pump against the high pressure in the lower sphincter – the entrance to the stomach – and works overactively. As the disease progresses to the hypomotile form, the esophageal muscles are about to become sluggish due to esophageal dilatation. In the amotile form, the end stage of the disease, the esophagus is defective and hangs like a slack muscle in the upper chest.

In the affected patients, a sometimes massive weight loss is often recognizable in the further course of the disease due to the considerable swallowing disorders. The general course of achalasia is usually very different and can be very rapid or gradual. Treatment is important in both cases, otherwise the situation will deteriorate.

Complications

Achalasia can cause various complications. If there is no treatment or if it is too late, the esophagus will first expand. In extreme cases, this can lead to a so-called megaesophagus. The chyme can no longer be transported to the stomach and inflammation and other complications occur.

Typical of a megaesophagus is increased belching, dry cough , hoarseness, and later pneumonia. Achalasia itself also increases the risk of esophageal cancer and its individual consequences. Comprehensive treatment can greatly reduce the risk of such complications, but the swallowing difficulties often persist for years.

This puts a considerable strain on the entire throat area, which can lead to infections and pain when eating, but also to an overload of the immune system. In less severe cases, an overburdened immune system can lead to fatigue and lethargy; in severe cases, chronic diseases and permanent damage to the internal organs develop. Allergic reactions, inflammation and other typical complications are possible when treating achalasia, but the symptoms are usually alleviated without complications.

When should you go to the doctor?

If achalasia is suspected, medical advice is always required. A visit to the doctor is particularly urgent if the typical symptoms and signs persist over a longer period of time and increase in intensity and duration over time. So, with constant weight loss or constant belching, a doctor should be consulted quickly. If symptoms such as dry cough, hoarseness and sore throat are added, there may already be a so-called megaesophagus, which must be treated immediately.

Pain when eating and infections in the mouth and throat indicate that achalasia is already at an advanced stage. In this case, a medical examination should be carried out as soon as possible in order to avoid a chronic development of the disease. In general, achalasia must always be medically clarified and treated. A doctor must therefore be consulted at the first suspicion of a serious illness. Patients at risk should have any noticeable symptoms clarified immediately by a specialist.

Treatment & Therapy

Achalasia is always treated with the aim of expanding the gastric entrance gate. Conservative, interventional and surgical therapies are possible for this.

In conservative treatment with medication, agents are used that reduce the muscle tension in the esophagus. However, since the use of medication only has a minor influence on the symptoms of the disease, the conservative therapy method is usually only chosen in stage I of the disease and only if the affected patients cannot be treated interventionally or surgically.

Interventional therapy methods include balloon dilatation and botulinum toxin injection (BTX). Both treatment methods are carried out by means of a gastroscopy and therefore do not require an operation. In balloon dilatation, a balloon is inserted up to the narrowed entrance to the stomach and “blown up” there, so to speak. However, with this treatment method there is also the risk that many or even all layers of the esophagus will be destroyed. This procedure can also cause life-threatening infections. The treatment usually has to be repeated after about one to five years.

Botulinum toxin injection has now become a standard treatment. Here, an agent is injected into the gastric entrance gate, through which the muscle relaxes. In the short and medium term, the therapeutic successes are positive, but not yet in the long term.

Muscle splitting, Heller’s myotomy, has proven itself as a surgical treatment method. As a rule, the so-called “open” surgical procedure, i.e. the abdominal incision, is used. With an abdominal incision, the long-term results are usually better than with the more gentle laparoscopic technique. An operation on the muscles of the gastrointestinal tract is usually the most successful treatment method in the long term.

Outlook & Forecast

Achalasia is a chronic disease in which spontaneous healing can be ruled out. If the disease is not treated, the typical swallowing difficulties usually continue to increase. In the later course of the disease, there is an increasing enlargement of the esophagus , which can ultimately lead to megaesophagus and severe lung and esophagus diseases.

However, if achalasia is diagnosed and treated in the early stages, the prognosis is positive. With the help of modern medicines and therapeutic measures, the symptoms can usually be sufficiently alleviated. An operation can counteract the relaxation of the esophagus and relieve the symptoms acutely. In the best case, achalasia does not progress after successful treatment or can at least be well controlled with the help of therapeutic measures and various drugs.

Since genetic factors are responsible for the development of achalasia, a prenatal examination is sometimes an option. This means that achalasia can be treated shortly after birth . Affected infants and young children have the prospect of esophageal surgery and, in most cases, make a full recovery.

Prevention

Since the exact cause of achalasia has not yet been found, no effective prevention is known. By renouncing nicotine and alcohol , however, side effects such as esophagitis can be avoided. In general, doctors also advise that after successful treatment of achalasia, regular follow-up care should be carried out by endoscopy. This is the only way that possible late effects such as esophageal cancer can be recognized in the early stages.

Aftercare

As a rule, aftercare is not possible for achalasia. The disease must be treated by a doctor, as it does not heal itself and usually worsens the symptoms. The patient is usually dependent on taking medication.

These should be taken regularly, although interactions with other medications should also be taken into account. The affected person should also refrain from taking nicotine and alcohol if possible. In general, a healthy lifestyle with a healthy diet has a positive effect on the further course of the disease.

It is not uncommon for achalasia to be treated by surgery. The person concerned should rest after the procedure and protect the body. Strenuous activities or sporting activities should be avoided. The patient’s stomach should also be protected.

You should avoid greasy food. Since achalasia can also lead to cancer in the esophagus in some cases, the affected person should have regular examinations. Furthermore, contact with other people affected by achalasia can also be useful, where stimulating information can be discussed.

You can do that yourself

Patients affected by achalasia suffer from a variety of health problems, which mainly manifest themselves in food intake and digestion. Typical difficulties with eating, such as difficulty swallowing and frequent belching, can be alleviated with medication.

The patient takes this, for example, before a meal, whereby regular intake of the medication must be coordinated with the responsible specialist in any case. It is also often helpful if the patient pays full attention to eating and swallowing while eating. This makes it easier to manage swallowing difficulties and may reduce the risk of complications such as heavy choking.

The feeling of fullness typical of achalasia, especially after eating, is partially reduced by the selection of suitable foods. In this context, however, it is possible for patients to achieve a healthier diet at the same time . In addition, the affected person reduces foods that often promote a feeling of fullness after eating, such as very greasy foods.

On the other hand, with achalasia there is a risk of unintentionally losing body weight due to impaired food intake. In this case, the patient follows a meal plan prepared by the doctor or a professional nutritionist to ensure adequate energy intake.

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Hello! I am Lisa Newlon, and I am a medical writer and researcher with over 10 years of experience in the healthcare industry. I have a Master’s degree in Medicine, and my deep understanding of medical terminology, practices, and procedures has made me a trusted source of information in the medical world.