What is Achalasia? Symptoms and Treatment

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Achalasia, the most well-known treatment of the esophagus, is motor dysfunction.

Achalasia literally means difficulty in relaxation, and the disease has been given this name because the valve at the lower end of the esophagus, where it joins the stomach, cannot relax during swallowing (See. Anatomy of the digestive system).

The first case of achalasia was published by Sir Thomas Willis in 1674, and the patient was treated with dilatation using whalebone.

Although it is rare, it can be said that almost every gastroenterologist or surgeon sees between 2 and 5 achalasia patients per year.

Frequency and Etiopathogenesis

Achalasia is seen with the same frequency in men and women, mostly in the 30-60 age range. Its incidence is 0.5-1/100.000 and its prevalence is around 7-10/100.000. (Incidence is the number of newly occurring cases and prevalence is the number of all existing cases.)

The main lesion is degeneration of the neurons of the esophagus (neuron = nerve cell) and consequent loss of innervation in the esophageal body and lower esophageal sphincter (LES). As a result, peristaltic activity is lost in the esophagus (aperistalsis).

The LES resting pressure increases and sphincter relaxation becomes difficult during swallowing. Immunohistochemical studies have shown that in patients with achalasia, there is a decrease in inhibitory neuron activity in which substances such as vasoactive intestinal peptide (VIP) and nitric oxide (NO), which normally provide LES relaxation, act as mediators.

Although the exact cause of myenteric neuron loss or damage in achalasia is not known, antimyenteric antibodies against neurons (30-90% in the patient group, 0-43% in the normal people) and inflammatory cell infiltration in the neurons (90% in the patient group, 90% in the normal people) are observed in the serum of these patients. 0) and the fact that some tissue groups are more common in these patients (HLA DQw1) (>80%) highlights the role of autoimmunity in the etiopathogenesis of the disease.

Clinical Finding İn Achalasia

The most distinctive clinical feature of achalasia is dysphagia (dysphagia) and is found in 95% of patients.

Difficulty in swallowing may start suddenly, but it is usually chronic and recurrent, and becomes continuous in the later stages of the disease.

Some patients may develop maneuvers that facilitate swallowing, such as back banging and straining. Food coming back into the mouth (regurgitation) is seen in ¾ of the cases.

There may be bad breath. Although patients have a good appetite, they become reluctant to eat, and this causes weight loss. Partial weight loss is a finding seen in approximately 60% of patients with achalasia.

Almost half of the patients have intermittent pain behind the sternum, and a smaller number of patients have a burning and burning sensation.

How İs Achalasia Diagnosed?

As it should be done in every patient presenting with chronic dysphagia, the first examination to be done in patients with achalasia is barium esophageal film (esophageal passage graphy).

Delay in the passage of barium to the stomach in barium X-ray, enlargement of the esophagus, air-liquid level in the esophagus, the appearance of falling snow caused by swallowed barium in the liquid accumulated in the esophagus, loss of peristaltic activity in the esophagus, narrowing in the form of a bird’s beak at the lower end of the esophagus (Bird beak deformity) and disappearance in the stomach air pocket are radiological findings that can be seen in patients with achalasia.

In advanced cases, the over-enlarged esophagus becomes wide and curved, resembling an enlarged stocking (sigmoid esophagus). In some patients, enlargement of the esophagus shadow and air-fluid level can be seen on chest X-ray.

Endoscopy is a useful method in the differential diagnosis of other diseases (pseudoachalasia – false achalasia) that may cause achalasia-like symptoms such as malignancy and peptic stricture.

In patients over 40 years of age and/or with short-term symptoms of the disease, who have lost a lot of weight in a short time, in patients with a family history of esophageal or stomach cancer, in patients who consume large amounts of cigarettes and alcohol, in patients with gastroesophageal reflux disease for a long time, and suspicious findings on barium X-ray Upper gastrointestinal system endoscopy must be performed in patients with diabetes mellitus (See. Gastroscopy).

The absence of enlargement and peristalsis in the esophagus, difficulty in passing from the junction of the stomach and esophagus, and the presence of food residues in the esophagus are endoscopic findings that may help in the diagnosis of achalasia. Endosonography and computed tomography are other examination methods that can be used in differential diagnosis when necessary.

How İs Achalasia Diagnosed?

Cancers involving the area where the esophagus and stomach meet, and tumors of neighboring organs that press on this area from the outside can mimic achalasia (See. Anatomy of the digestive system).

This condition is called secondary achalasia or pseudoachalasia. This should be considered especially in patients who are elderly and whose disease symptoms appear in a short time.

Pseudoachalasia is seen in 3% of patients presenting with achalasia clinic and this rate may increase up to 10% over 60 years of age.

Chagas disease is a disease that is mostly seen in South America and is caused by a protozoan called Trypanosoma cruzi (a kind of parasite), causing chronic damage to the nervous network in the urinary, gastrointestinal and respiratory systems.

Infiltrative diseases such as amyloidosis, sarcoidosis, familial or sporadic visceral neuropathy, diabetes, intestinal pseudoobstruction, multiple endocrine neoplasms, scleroderma, Sjögren’s syndrome and other pseudochalasia that may cause familial adrenal insufficiency (Triple A syndrome or Allgrove syndrome) with absence of tears (alacrima) can be counted among them.

How İs Achalasia Treated?

Endoscopic balloon dilatation (Pneumatic dilatation) is based on the rupture of the muscles in this region (myotomy) by inflating the dilatation balloons with a diameter of 30-40 mm placed at the level of the LES in endoscopy.

The success rate in the first year after one or two pneumatic dilatations is around 70-80%. A second dilatation is required in 30-50% of patients.

A good thing about the method is that it can be sent to the patient’s home on the same day. Bleeding, perforation (2-8%) and reflux esophagitis (3%) that may develop after dilatation are the main complications of pneumatic dilatation.

Balloon dilatation gives better results in patients over 40 years of age. In cases where achalasia symptoms recur after two dilatations, alternative treatment methods should be considered. In young patients, surgical treatment can be considered when adequate improvement is not achieved after the first dilatation.

A new achalasia or endoscopic method that has been applied in the treatment of achalasia in recent years is the method called POEM (Peroral endoscopic myotomy). In this method, the lower esophageal sphincter region is reached through a tunnel opened under the mucosa in the esophagus and the muscles there are cut with the help of cautery (myotomy).

Patients can usually go home after achalasia treatment. Although the method was reported to be a method with a high chance of success in the first years following when it was first applied (90%), the evaluations made after 5 years show that the long-term success rate is lower.

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