Esophageal achalasia

Achalasia is a condition that affects the ability of the esophagus to deliver food towards the stomach.

In addition, the sphincter between the esophagus and stomach (lower esophageal sphincter) does not relax sufficiently when swallowing the food to enter the stomach.

What is achalasia?

In the esophagus there are muscles and nerves to facilitate the movement of food.
The nerves coordinate the relaxation and opening of the sphincters and the peristaltic waves in the esophagus.
Achalasia affects both the muscles and nerves of the esophagus, especially the nerves that relax the sphincter between the esophagus and stomach.
The muscles do not contract sufficiently, thus complicating peristalsis (rhythmic muscle movement of the digestive organs), which allows food to pass through the esophagus.
However, the effects on the nerves are considered more serious.

At the beginning of achalasia under the microscope, inflammation can be seen in the muscle of the lower esophagus.
As the disease progresses, the nerves begin to degenerate and eventually disappear, especially the nerves responsible for relaxing the lower esophageal sphincter.
Over time, the muscle cells begin to degenerate as a result of nerve damage.

The result of these changes is the inability to relax the lower sphincter and muscles in the lower part of the esophagus.
If the sphincter is not sufficiently relaxed, the food cannot enter the stomach unhindered. This makes digestion more difficult.
Over time, the lower esophagus stretches and expands.

What are the causes of esophageal achalasia?

The causes of achalasia are unknown. There are theories about the risk factors: infection, heredity, or an autoimmune disease that causes damage to the lower esophagus.

Scientists believe that this may be related to a virus and some new studies show that achalasia may be caused by nerve cells of the involuntary nervous system located inside the muscle layers of the esophagus. These cells are attacked by the patient’s immune system and slowly degenerate for reasons that are still unclear.

Risk factors of esophageal achalasia

Achalasia is a rare condition that can occur at any age, but it is most common in middle-aged or elderly people.

What are the symptoms of esophageal achalasia?

Symptoms of achalasia can begin in children or develop gradually in childhood or adulthood. The most common symptoms are:

• Difficulty swallowing liquids or solid foods
• Chest pain
• Belch
• Heartburn
• Lump feeling in the throat
• Cough
• Weight loss

Possible complications of esophageal achalasia

• reflux (regurgitation) of stomach acid or solid food from the stomach into the esophagus,
• inhalation of food particles that can enter the lungs and thus lead to aspiration pneumonia,
• Perforation of the esophagus

Diagnosis of achalasia

The symptoms of achalasia are similar to those of gastroesophageal reflux, hiatal hernia and some psychosomatic disorders. This disease is often confused with other diseases.

The doctor may prescribe the following diagnostic examinations:

X-ray with barium – the patient must swallow a white liquid known as barium sulfate. Barium sulfate is visible under X-rays. As soon as the patient swallows this solution, the esophagus lines itself with a thin layer of barium, which makes the hollow structure visible in the X-ray.

Esophageal manometry – this is done to measure the pressure and movements in the esophagus. For this purpose, a device called a “pressure gauge” is used, with which the pressure is measured.

The doctor can perform this examination to rule out cancer.
A fine tube is inserted through the patient’s nose and the patient is asked to swallow several times.

The device measures muscle contraction in different areas of the esophagus.
This procedure allows the doctor to determine whether the sphincter of the lower esophagus is relaxing correctly while the patient swallows. He can determine how the smooth muscles work.

Biopsy – this is not commonly used to diagnose achalasia. A tissue sample is taken during a gastroscopy.

Treatment of esophageal achalasia

Achalasia therapy focuses on relaxing and closing the valve between the esophagus and the stomach (lower esophageal sphincter) so that the food and liquids can more easily reach the digestive tract.
The exact treatment depends on the age and severity of the disease.

The possibilities include:

Surgery. The surgeons use a minimally invasive procedure, the so-called extramucosal myotomy according to Heller, to separate the muscle at the lower end of the esophageal sphincter.
People who undergo this procedure may subsequently develop gastroesophageal reflux.
To avoid reflux problems, during the operation, in which the surgeon severs the muscle of the esophageal sphincter, surgeons can use a second procedure called fundoplication (see hiatal hernia page).
Surgery may be the preferred option for young people, although research shows that it can also be effective for the elderly.

Balloon dilatation. A small balloon is inserted into the esophageal sphincter and inflated to widen the opening. This outpatient procedure can also be repeated if the esophageal sphincter does not remain open. Some people can cope with it for up to 10 or 15 years before balloon dilatation has to be repeated.

Botox (botulinum toxin). This muscle relaxant can be injected directly into the esophageal sphincter via an endoscope.
Studies indicate that the injections sometimes have to be repeated, which can also make it more difficult to have an operation that may be required later.

The guidelines recommend using Botox only for the elderly and for those who are not good candidates for surgery.

What is the long-term prognosis in children with esophageal achalasia?

Even though there is no actual treatment for this condition, surgery can allow most individuals to lead a normal life.

After surgery
There are some things you can do after dilation or surgery to reduce symptoms:

• Chew the food well
• Take the necessary time to eat
• Drink plenty of fluids to eat
• Always eat sitting and in an upright posture
• Use several pillows or raise the headboard of the bed. This will keep you sleeping in a sufficiently upright posture and prevent the acid from flowing into the esophagus, leading to heartburn.

• Hiatal hernia or diaphragmatic hernia
• Home remedies for heartburn
• Reflux esophagitis or eosinophilic esophagitis