What is Achalasia?

Achalasia is defined as a rare esophageal motility disorder that involves the smooth muscle layer of the esophagus (the muscular tube that transports food from your mouth to your stomach) and lower oesophagal sphincter. In patients with achalasia, the esophagus muscles do not contract properly which prevents foods and drinks from passing into the stomach, which can get stuck and be brought back up leading to vomiting or choking.

Which age and gender are more affected by this condition?

This disorder typically affects people between the second to the fifth decade of life with a peak incidence between the ages of 30 to 60 years.

Men and women are found to be equally affected. Symptoms may also be found in children after the age of 10 years.

No particular race or ethnic group is more affected than others and familial predisposition is rarely seen.

What are the causes leading to Achalasia?

It is a rare disorder but the following factors have been found to cause it-

• Damage to the esophageal nerves.
• Cancer of the esophagus or upper stomach
• Genetic predisposition.
• Family history.
• Any autoimmune condition.

Can achalasia lead to malnutrition?

Achalasia can be a serious health issue if not treated and may lead to severe weight loss and malnutrition in later stages.

It can also cause chest pain, fatigue, and compromised immune symptoms leading to infections like pneumonia.

What are the signs and symptoms commonly seen in this condition?

People with achalasia typically experience dysphagia with other common symptoms like-

• Regurgitation of the undigested food
• Pain in the chest
• Difficulty in swallowing liquids and solids
• Heartburn
• Cough triggered when lying in a horizontal position.
• At times food and liquids are retained in the esophagus and may be inhaled through the lungs.

How to diagnose Achalasia?

Diagnosis of achalasia is multifactorial.

First, a physical examination might be performed. However, physical examination is not enough. Few tests should be performed to understand and diagnose this condition.

It can be done by:

• X-ray imaging: To evaluate the structure of the esophagus.

• Barium swallow: Barium solution is swallowed, and its path is evaluated along the esophagus using X-rays.

• Esophageal manometry: This test measures the force, timing, and efficiency of muscular contractions of the esophagus and the lower esophagus sphincter. The absence of these contractions is taken as a positive sign of achalasia cardia.

 – Lower oesophagal sphincter (LES) fails to relax upon wet swallow (<75% relaxation)

   – Pressure of LES <26 mm Hg is normal,>100 is considered achalasia, > 200 is nutcracker achalasia.

 – Aperistalsis in the oesophagal body.

 – Relative increase in intra-esophageal pressure as compared with intra-gastric pressure.

• Upper gastrointestinal endoscopy: This test gives a clear view of the inner esophageal wall. In this test, a narrow tube with a camera called an endoscope, is inserted down the esophagus, and the images are seen on the screen.
• Biopsy: A small sample of tissue may be acquired from the lower part of the esophagus to look for cancer cells.
• Wireless pH testing or 24-hour pH impedance testing: To evaluate the acidity in the esophagus during an extended period and rule out other conditions such as GERD.

What other diseases look like Achalasia?

Certain diseases that might mimic symptoms of achalasia are-

•  Gastroesophageal reflux disease
•  A hiatus hernia
•  Psychosomatic disorders

GERD: In GERD, the LES doesn’t close after emptying of esophagus and results in reflux of stomach contents back to the esophagus.

A hiatus hernia: In hiatus hernia, the top of your stomach pushes up through an opening in your diaphragm into the chest.

Psychosomatic disorders: It is a physical condition resulting from mental stress and distress. Stress can affect the body in many ways. In psychosomatic disorders, the main line of treatment should be stress management.

What can be the complications of achalasia?

Commonly occurring complications of this condition are-

These are the complications of achalasia cardia: 

• Aspiration pneumonia
• GERD
• Esophagitis with fungal infection
• Perforation of the esophagus
• Oesophagal cancer
• Malnutrition

1. Aspiration Pneumonia

It is the most severe and rare complication of achalasia cardia. Due to the sudden regurgitation process, the gastric contents from the stomach enter the lungs, leading to bronchopneumonia. In this complication, the patient may feel a choking sensation, sudden breathlessness, vomiting, and respiratory distress.

   2. GERD:

The long-term immobility of the esophagus causes the weakening of the lower esophageal sphincter that lead to frequent regurgitation of the food contents to the mouth that causes acid reflux. The patient may present with epigastric pain, fullness after meals, water brash and indigestion.

   3. Esophagitis:

There is a constant collection of food and gastric contents in the oesophagus that can irritate the mucosal lining which causes the inflammation of the oesophagus known as esophagitis. There is a fungal infection in the oesophagus. 

  4. Perforation of the oesophagus:

Chronic inflammation and irritation in the oesophagus can cause ulceration which leads to the formation of a hole in the oesophagus called an oesophageal perforation. 

  5. Oesophageal cancer:

There is not much significant evidence about the medical correlation of oesophageal cancer with achalasia cardia. Though some patients have been diagnosed with adenocarcinoma of the oesophagus have a dilatation procedure for achalasia cardia. 

  6. Malnutrition:

There is prolonged weight loss due to the inability to swallow liquids and solids that lead to significant weight loss and weakness which affects the nutritional status. 

Which precautions can be helpful in Achalasia?

Some precautions that patients with achalasia can take to improve the prognosis of their condition are-

• Eat the food slowly and chew very well.
• Drink plenty of fluids when eating.
• Avoid eating solid food for three to four hours before going to bed.
• Raise the bed head or sleep with the wedge pillow to avoid flare-up of symptoms at night.
• Food that aggravates the reflux needs to be avoided.
• If there is a symptom of dysphagia or regurgitation, then stop smoking. Smoking can weaken the lower esophageal sphincter.

Is this treatable?

Yes, this condition is treatable. The main goal is to relieve the suffering so that the food and drinks can be swallowed and passed through the esophagus to the stomach. There are some surgical and some non-surgical treatments available:

Surgical:

• Balloon dilatation- In this procedure, a specifically designed balloon is inserted in the lower esophageal sphincter and inflated. It makes the muscular fibers relax so that the food passes to the stomach.
• Laparoscopic Heller Myotomy (LHM)- In this procedure, the muscle fibers of the lower esophageal sphincter are cut. An additional procedure called fundoplication is performed to deal with the side effects of LHM i.e., GERD.
• Peroral Endoscopy Myotomy (POEM)- In this procedure, the muscles around the esophagus, lower esophageal sphincter, and fundus are cut with a knife. This causes the relaxation of muscle fibers, and the food enters the stomach directly.

Non-surgical:

If surgery is not an option, the botulinum toxin injections can relax muscles. These injections need to be repeated to maintain the control of symptoms.