Achalasia

What is it?

Achalasia cardia, or esophageal achalasia, is a condition in which the lower esophagus does not relax enough during the process of moving food. It makes it difficult for food to enter the stomach. The disorder is neurogenic and manifests itself as swallowing difficulties, throwing food debris into the oral cavity, and pain in the upper abdominal area. Treatment is conservative or operative.

Disease

Achalasia cardia is one of the most common diseases of the esophagus. Among the diseases that cause an esophageal obstruction, it ranks third after malignant tumors and scars after burns. The pathology is diagnosed with equal frequency in men and women. The most dangerous age is the period from 20 to 40 years.

Classification

Doctors distinguish two types of achalasia based on the results of an X-ray examination. The pathology of the first type is characterized by moderate narrowing of the terminal esophagus and mild enlargement of the overlying areas. Circular muscles are simultaneously subjected to hyper- and dystrophy. The second type has a marked narrowing of the lower part, with expansion and S-shaped deformation of the overlying areas.

When choosing a tactic for treatment, doctors also take into account the stage of development of the achalasia, which is determined by the degree of narrowing of the esophagus and is accompanied by the appearance of specific symptoms:

  • Stage I is manifested by periodic spasms of the esophagus sphincter; there are no anatomical changes;
  • Stage II is characterized by a stable but insignificant expansion of the adjacent part of the esophagus;
  • Stage III: the lower part of the esophagus has severe narrowing and a cicatricial malformation, overlying parts have enlarged significantly;
  • Stage IV: changes in the esophageal anatomy reach a critical stage, the mucosa is inflamed, ulcers and areas of necrosis (gangrene) are found on its surface.

Symptoms of achalasia

The most typical symptom of the disease, observed in almost all patients, is swallowing difficulties. In addition, in achalasia, food debris is thrown back into the oral cavity due to stagnation in the esophagus caused by the malfunctioning of the esophageal sphincter.

In the early stages, swallowing difficulties (dysphagia) only occur when taking solid food, as the disease progresses, swallowing difficulties are also observed when taking liquid food.

Patients often wake up at night because of a choking sensation or a strong cough when throwing up food. 

Also, symptoms such as heartburn and chest pain may occur in achalasia cardia. Pain sensations are concentrated at the back of the sternum, have a squeezing or pinching nature, and may be referred to the back, lower jaw, or neck pain.

Causes of achalasia

The exact causes of this disease have not been determined. It is based on a disruption of the nervous system of the esophagus, which can be associated with various diseases:

  • congenital diseases of the nervous plexus;
  • inflammatory processes that affect nerve fibers;
  • vitamin B deficiency;
  • malignant tumors of the stomach, lungs, lymph nodes, etc.;
  • myasthenia gravis;
  • systemic lupus erythematosus;
  • thyroid hormone deficiency, etc.

Diagnosis

When the first symptoms of the disease appear, you will need to contact a gastroenterologist and undergo an examination. The doctor will do a questionnaire and assessment of the patient and then order the necessary tests. The symptoms of achalasia of the esophagus are similar to some other diseases, such as esophageal hernia, which can make diagnosis difficult.

The patient will be prescribed a series of instrumental studies aiming to visualize the organ and evaluate its function.

  • Radiography using a contrast. The essential part of the examination is that the patient drinks a barium sulfate solution, which covers the esophagus with a thin layer. Thanks to this, it is possible to obtain a clear picture of the structure of the organs and identify any pathological changes.
  • Endoscopic examination (esophagoscopy). It is performed using a special device, an endoscope, with a camera located in a thin tube. This tube is moved through the digestive tract, and the doctor sees any pathological changes in the esophagus or stomach in real-time. During the endoscopy, a biopsy may also be performed (if indicated).
  • Manometry of the esophagus. This procedure is used to measure muscle pressure. A special device (pressure gauge) measures muscle contractions in different parts of the esophagus, which allows the specialist to find out how well the sphincter of the esophagus is working.
  • Tests designed to detect nervous disorders (administration of medicines that increase muscle tone).

These examinations allow us to make an accurate diagnosis of achalasia and select tactics for treatment.

Treatment

Treatment aims to stop the spasm of the lower esophageal sphincter. To achieve this goal, conservative or surgical methods may be used.

Conservative methods include pneumodilation. It is the gradual dilation of the lower esophageal sphincter, which is performed using special gas cylinders of different diameters that gradually increase in pressure.

As a result, the sphincter overstretches, leading to a decrease in tone and a return to normal function. Potential complications of this procedure include fissures and tears of the esophagus, the formation of scars on the sphincter, and the development of reflux esophagitis.

Surgical treatment

Minimally invasive methods of treating achalasia include esophageal dilation under the supervision of an endoscope and the introduction of botulinum toxin into the esophageal sphincter to relax it.

Endoscopic dilation should be applied in the first three stages of the disease, especially if there is no effect fromconservative treatment. Up to three extension courses are allowed. If no result is achieved, the procedure is considered ineffective.

Intersphincter administration of botulinum toxin type A is performed under endoscopic guidance. The procedure can be used for first-line treatment and in patients with a fourth stage, who have contraindications. Botulinum-therapy is indicated for relapse of the disease after pneumodilation and in patients over the age of 70.

Surgical treatment for achalasia consists of removing the enlarged esophagus, followed by replacing it with other organs. The procedure is used in the fourth stage of the pathological process.

Recently, minimally invasive endoscopic surgery has been actively developing, allowing timely pathogenetically sound treatment of the disease and preventing its progression with the development of complications. The core procedure is to dissect the sphincter and dissect it in the submucosal layer. In modern conditions, the operation is performed through the natural openings of the gastrointestinal tract, while the duct is formed in the submucosal layer through a small incision in the mucosa. With the help of miniature instruments, it is easier to access the areas that are in a state of muscle spasm. The endoscopic myotomy method to treat achalasia can be used not only in adults but also in children over three years of age.

All these surgical procedures are performed in more than 750 hospitals all over the world (https://doctor.global/results/diseases/achalasia). For example, laparoscopic myotomy for achalasia is performed in 13 clinics of Israel with an approximate treatment cost of $9.3 K. (https://doctor.global/results/asia/israel/all-cities/all-specializations/procedures/laparoscopic-myotomy-for-achalasia).

Prevention

There is no specific prevention of the disease. Preventive measures are limited to preventing complications.

The patient is recommended to:

  • normalize a diet – patient must eat often (5-6 times a day), but in small portions.
  • follow a special diet – do not eat fast food, spicy, salty, fatty, fried foods;
  • chew food properly before swallowing;
  • give up bad habits (alcohol abuse, smoking);
  • engage in physical activity;
  • take more walks in the fresh air;
  • the management of work and rest;
  • avoid physical and mental stress.

Rehabilitation 

After surgery on the esophagus, the patient is prescribed a gentle diet. Only fluid is allowed for the first few days, then gradually the diet is expanded. Until the stitches are completely healed, it is necessary to use processed food, for example, mashed potatoes and purees. The food should not be too hot, cold, or spicy. As the condition stabilizes, the patient returns to their everyday life. It is necessary to visit the gastroenterologist regularly and follow all preventive measures.

Achalasia cardia: from symptoms to management FAQ

Yes, if the disease was treated conservatively (pneumodilatiion of esophagus).
Drug treatment only brings temporary relief. It is suggested to relieve uncomfortable symptoms while waiting for surgery or pneumodilation.
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