Achalasia is a very rare disease, which can cause the person concerned quite a few complaints. The physician then speaks of achalasia when there is a disorder in hollow organs with occlusive function.

What is achalasia?

Slanted illustration of the anatomy of achalasia. Click to enlarge.

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

The frequency of the disease is very rare. Only 1 out of every 100, 000 people contracted Achalsie. It is not intended for age and gender. Anyone between the ages of 20 and 40 can theoretically suffer from this condition.

causes

The causes of the disease can not yet be named. The medicine divides the disease into primary and secondary achalasia. However, it is believed that it is an autoimmune disease. For example, in some patients with primary achalasia, certain antibodies could be detected that have a negative effect on the autonomic nervous system.

In addition, it is suspected that pathogens such as measles viruses, varicella-zoster viruses or human papillomavirus come as the cause of a disease in question. Hereditary and psychological factors could also play a role. In general, the degeneration of neurons of the plexus myenterious Auerbach is responsible for the dysfunction of the esophagus. Inflammatory reactions often support degeneration.

Secondary achalasia can be caused by either carcinoma or Chagas disease, which is common in the tropics. Also, gastroenteritis and various other diseases around the digestive tract may be the cause of secondary achalasia.

Symptoms, complaints & signs

A dysfunction of the esophagus can cause various symptoms. Typical are swallowing disorders and pain in the area of ​​the esophagus. Sometimes, the pain radiates to the chest and cause an increased malaise. Achalasia also leads to the regurgitation of undigested food, combined with heartburn and nausea.

Initially, the disease causes mild dysphagia, especially in solid foods: the person has the feeling that the food is stuck in the throat. In the further course, it is difficult for those affected to absorb liquid food. This can lead to deficiency symptoms, which manifest themselves in the form of dizziness and fatigue.

In addition, food debris can enter the respiratory tract and cause other complaints such as pneumonia. Often a malfunction of the esophagus is expressed by involuntary, usually spasmodic pain behind the sternum, which is similar to heart trouble. In the long term, achalasia leads to weight loss.

Externally, the disease is mainly due to the characteristic signs of disease, so pale skin, sunken eye sockets and increased perspiration. The affected person's voice is usually hoarse and fragile, giving the impression of having a cold. In severe cases, a temporary loss of voice may occur.

Diagnosis & History

The diagnosis of achalasia in the early stages is usually carried out with a pressure measurement (manometry), which takes place in several esophageal sections. In this case, the insufficient opening (relaxation) of the lower sphincter of the esophagus can be detected.

In addition, there is usually a disturbance of the pumping function of the esophagus. In the late stage of the disease, the patient is administered a barium porridge (contrasting gruel). The findings can then be made by X-ray examination. Routine diagnostics also include endoscopy of the upper digestive tract or gastroscopy, so that other causes of illness can be ruled out.

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, she tries to pump against the high pressure in the lower sphincter - the stomach entrance - and works overactive. When the disease becomes hypomotile, the muscles of the esophagus are about to become sluggish due to the expansion of the esophagus. In the amotile form, the end stage of the disease, the esophagus is broken, so to speak, and hangs like a limp muscle in the upper thorax.

Often, in the affected patients due to the significant dysphagia in the further course of disease, a partial massive weight loss can be seen. The general course of achalasia is usually very different and can go very fast or creeping. But in both cases treatment is important, otherwise the situation worsens.

complications

Achalasia can cause various complications. If the treatment stops or occurs too late, the esophagus widened first. In extreme cases, this can lead to a so-called megaesophagus. The chyme can no longer be transported to the stomach and it comes to inflammation and other complications.

Typical of a megaesophagus is increased eructation, irritable coughing hoarseness, and in the further course of 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 dysphagia often persists for years.

As a result, the entire pharynx is heavily loaded, which can lead to infections and pain during food intake, but also to an overload of the immune system. In less severe cases, an overloaded immune system can lead to fatigue and fatigue; in severe cases, chronic diseases and lasting damage to the internal organs develop. In the treatment of achalasia allergic reactions, inflammation and other typical complications are possible, but usually there is a complication-free relief of the symptoms.

When should you go to the doctor?

Any suspected achalasia requires medical advice in any case. The 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 a constant weight loss or constant regurgitation, a doctor should be consulted quickly. If symptoms such as irritating cough, hoarseness and sore throat are added, there may already be a so-called mega-esophagus, which must be treated promptly.

Pain in food intake 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. Generally speaking, achalasia must always be medically clarified and treated. For a first suspicion of a serious illness, therefore, a doctor must be consulted. High-risk patients should have obvious symptoms promptly checked out by a specialist.

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Treatment & Therapy

A treatment of achalasia is always with the aim to expand the Mageneingangspförtner. For this purpose, conservative, interventional and surgical therapies are possible.

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

Interventional therapy methods include balloon dilatation and botulinum toxin injection (BTX). Both treatments are performed by gastroscopy and thus require no surgery. In the balloon dilatation, a balloon is introduced to the narrowed stomach entrance and there, so to speak, "blown up". However, there is a risk with this method of treatment that many or even all layers of the esophagus are destroyed. Even life-threatening inflammations can arise through this intervention. Mostly the treatment has to be done again after about one to five years.

Injection of botulinum toxin has become a standard treatment. Here, a means is injected into the Mageneingangspförtner through which the muscle relaxes. In the short and medium term, the therapeutic successes are quite positive, but not yet in the long run.

As an operative treatment, muscle splitting, the Heller myotomy, has proven itself. As a rule, the so-called "open" surgical procedure, ie the abdominal incision, is used. In the abdominal section, the long-term results are usually better than with the gentler laparoscopic technique. An operation on the musculature of the stomach entrance therapist is usually the most successful treatment method in the long run.

Outlook & Forecast

Achalasia is a chronic condition in which spontaneous healing can be ruled out. If the disease is not treated, the typical dysphagia usually continues to increase. In the later course of the disease, an esophagus eventually enlarges, leading to megaesophagus and severe lung and esophageal diseases.

However, if a diagnosis and treatment is made in the early stages of achalasia, 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 by therapeutic measures and various drugs.

Since genetic factors are responsible for the development of achalasia, sometimes a prenatal examination is possible. As a result, achalasia can be treated shortly after birth. Diseased infants and toddlers have the prospect of esophageal surgery and, in most cases, a full recovery.

prevention

Since so far the exact cause of achalasia could not be found, no effective prevention is known. By abstaining from nicotine and alcohol, however, accompanying symptoms such as esophagitis can be avoided. In general, physicians are also advised to undergo follow-up care after successful treatment of achalasia regularly by endoscopy. Only in this way can possible long-term consequences such as esophageal cancer be recognized at an early stage.

aftercare

As a rule, no aftercare is possible with achalasia. The disease must be treated by a doctor, since it does not lead to a self-healing and usually to a worsening of the symptoms. The patient is usually dependent on the intake of medication.

These should be taken regularly, although interactions with other medications should also be considered. The person should also abstain 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.

Not infrequently, the achalasia is also treated by surgical procedures. The affected person should rest after the procedure and protect the body. Here, strenuous activities or sports activities should be avoided. Furthermore, the stomach of the patient should be spared.

It is necessary to abstain from fatty food. Since achalasia can also lead to cancer in the esophagus in some cases, the affected person should be examined regularly. Furthermore, the contact with other afflicted achalasia may be useful in which stimulating information can be discussed.

You can do that yourself

Patients with achalasia suffer from a variety of health conditions, mainly food intake and digestion. Typical eating difficulties such as difficulty swallowing and frequent regurgitation can be alleviated by medical means.

This takes the patient, for example, before the meal, with a regular intake of the medication in each case with the appropriate specialist. Often it is also helpful if the patient focuses his entire attention on food intake and swallowing during the meal. This makes it easier to manage swallowing difficulties, and the risk of complications such as severe ingestion may be reduced.

The feeling of fullness typical of achalasia, especially after eating, is reduced in part by the selection of suitable foods. In this context, however, it is possible for patients to simultaneously achieve a healthier nutritional style. In addition, the sufferer reduces foods that often favor a feeling of fullness after eating, such as greasy foods.

On the other hand, in the case of achalasia, there is a risk of unwanted body weight being lost as a result of impaired food intake. In this case, the patient adheres to a diet prepared by the physician or a professional nutritionist to ensure adequate energy intake.

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