Achalasia is a pathological condition causing dysphagia, reflux, and regurgitation. Overall, less than 5% of patients with symptoms present under the age of 15.  Achalasia develops in about 1 in every 1,000,000 children in the United States each year.  There is no race or ethnic group that is more affected than others and the condition does not run in families. 

The causes of achalasia are unknown, There are several theories that exist. One is related to the degeneration of the nerve cells located between the layers of esophageal muscles. These nerve cells enable the esophagus to push food toward and into the stomach.

There are studies that suggest there maybe a relationship between achalasia and parasitic or viral infections. People with achalasia may be more likely to show evidence of previous infections, such as antibodies to the herpes simplex virus, human papillomavirus, measles virus and others.

Achalasia has also been attributed to an inflammatory autoimmune disorder, which means it could be caused by the body attacking itself. Moreover, patients with achalasia are 3.6 times more likely to have an autoimmune disorder such as uveitis, type I diabetes, rheumatoid arthritis, systemic lupus erythematosus and Sjögren’s syndrome. 

In children, Achalasia has been associated with Trisomy 21, congenital hypoventilation syndrome, glucocorticoid insufficiency, eosinophilic esophagitis, familial dysautonomia, Chagas’ disease, and achalasia, alacrima, and ACTH insensitivity (AAA) syndrome

Some achalasia symptoms are caused by food piling up in the esophagus and being unable to pass into the stomach. As the esophagus fills up, it widens and can become twisted. Other achalasia symptoms are related to the abnormal contractions of the esophageal muscles.

Symptoms of achalasia occur during or after eating. They include:

  • The feeling that food or liquid are hard to swallow and are getting caught in the esophagus or “sticking” on the way down to the stomach (this is the most common symptom of achalasia)
  • Regurgitation (food and liquid backing up into the mouth after being swallowed)
  • Chest pain, which can be severe and awaken the person from sleep
  • Heartburn
  • Coughing, especially at night
  • Choking,or inhalation of food or liquid into the airways (aspiration)

Both chest pain and regurgitation, some of the first symptoms a person with achalasia might experience, are also associated with gastroesophageal reflux disorder (GERD). However, the two conditions are different. GERD is caused by a lower esophageal sphincter that is too loose, allowing stomach acid to enter the esophagus. With achalasia, the sphincter is too tight.

Achalasia symptoms can become serious. Severe achalasia may lead to significant chest pain, fatigue, malnutrition and weight loss. If food particles enter the airways due to coughing and choking while eating, it can lead to pneumonia — inflammation in the airways that can become life threatening.

The way muscles in the esophagus malfunction in people with achalasia varies. In all cases of achalasia, the lower esophageal sphincter that controls the passage between the esophagus and the stomach fails to relax at the right time. Based on other problems that happen at the same time, doctors identified three types of achalasia:

  • Type 1 achalasia is sometimes called classic achalasia. With this type, the esophagus muscles barely contract, so food moves down because of gravity alone.
  • Type 2 achalasia, pressure builds up in the esophagus, causing it to become compressed. This is the most common type of achalasia and it often causes more severe symptoms than type I.
  • Type III achalasia is sometimes called spastic achalasia because there are abnormal contractions at the bottom of the esophagus where it meets the stomach. This is the most severe type of achalasia. The contractions can cause chest pain that can awaken a person from sleep and imitate the symptoms of a heart attack.

These tests are commonly used to diagnose achalasia. 

1. Barium swallow

Your child will swallow a barium solution (liquid or another form) and its movement through the esophagus is evaluated using X-rays.  The barium swallow will show a narrowing of the esophagus at the lower esophageal sphincter (LES) where the esophagus empties into the stomach.

2. Upper endoscopy

In this test, a flexible, narrow tube with a camera on it (called an endoscope) is passed down the esophagus.   The camera projects images of the inside of the esophagus onto a screen for your doctor to evaluate.  This procedure would be done while your child is asleep. 

 3. Manometry

This test measures the timing and strength of the esophageal muscle contractions and relaxation of the lower esophageal sphincter (LES).  If the LES does not relax in response to swallowing and there is a lack of muscle contractions along the walls of the esophagus, it is a positive test for achalasia.

4. EndoFLIP

This is a balloon catheter inserted into the esophagus (while asleep under anesthesia), at the time of POEM or laparoscopic Heller myotomy, which measures the degree of narrowing at the LES and the distensibility of the esophagus.  The success of the surgery to cut the LES muscle is assessed during the procedure by using the EndoFLIP both before and after the myotomy

Two surgical procedures that are commonly performed in children are Laparoscopic Heller Myotomy with or without Fundoplication and Per Oral Endoscopic Myotomy

Achalasia is a lifelong condition, with symptom management, you can avoid the most serious complications of achalasia, such as aspiration, choking, and malnutrition.

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