Achalasia Cardia: Symptoms, Causes, Diagnosis, & Treatment

Achalasia cardia, often referred to as achalasia, affects the esophagus (the tube that carries swallowed food from the throat down into the stomach).

A ring of muscle called the lower esophageal sphincter encircles the lower esophagus just above the stomach. This sphincter muscle when contracts, closes the esophagus. When the sphincter is closed, the contents of the stomach cannot flow back into the esophagus. The backward flow of stomach contents (reflux) can irritate and inflame the esophagus, causing symptoms such as heartburn. The act of swallowing causes a wave of esophageal contraction called peristalsis, which pushes the food along the esophagus down into the stomach.

Normally, during peristalsis, the esophageal sphincter relaxes to allow the food into the stomach. In achalasia, which means ❝failure to relax❞, is a rare esophageal motility disorder in which the normal esophageal peristalsis and the lower esophageal sphincter relaxation are impaired. 

Achalasia Cardia is a rare motility disorder that affects about 12-16 people per 1,00,000 population. The condition affects the nerves and muscles of the esophagus, causing difficulty swallowing food and liquid. As the disease progresses, increased motor dysphagia causes paralysis of the esophagus. While there is no cure for the condition, Achalasia Cardia can be managed, and the progression is slowed with therapy. The prevalence of Achalasia is estimated to be 10 in 1,00,000.

Types of Achalasia Cardia

Doctors and researchers classify Achalasia Cardia into three distinct types -

• Type 1 (Classic) - The esophagus has low contractility in this type of Achalasia. It means that the esophagus grows progressively rigid.
• Type II - The most common type of Achalasia is Type II, which causes intermittent periods during which pressure builds up in the esophageal body.
• Type III (Spastic) - Type III Achalasia causes abnormal contractions in the distal or bottom part of the esophagus. It disallows the food to pass on to the stomach causing severe chest pains and reflux.

Achalasia Cardia Causes

It has been over three centuries since the symptoms of Achalasia Cardia were initially described and the condition was identified. Despite this, the exact cause of the condition remains unknown. Several researchers have suggested that the condition's cause may result from a previous infection. Others have pointed to autoimmune or genetic causes for the condition.

What is known to us is that Achalasia Cardia causes the degeneration of the nerve cells located between the muscle bands of the Esophageal Sphincter, causing an inability to relax.

What are the Symptoms of Achalasia Cardia? 

Achalasia Cardia is a progressive condition. Some of the symptoms include -

• The inability to swallow food or drink is referred to as dysphagia. This feels like a block in the food pipe or could feel like food getting stuck in the throat.
• Regurgitation or backing up of food that has piled in the esophagus. This is often mistaken for GERD in the early stages.
• Chest pain, heartburn, and belching - are also symptoms that mimic GERD making Achalasia difficult to diagnose early.
• Coughing at night
• Aspiration of food or drink into the lungs, leading to pneumonia
• Vomiting
• Weight loss is caused by an inability to eat food properly.

How to Diagnose Achalasia Cardia?

In the early stages, diagnosing Achalasia Cardia often requires the elimination of other diseases with similar symptoms and thorough testing. Achalasia is often mistaken for GERD or even heart attack. Your doctor at Max Healthcare will take a thorough medical history and understand the symptoms before establishing a diagnosis. These are some of the tests your doctor may recommend -

• Endoscopy- Endoscopy involves introducing a thin tube with a camera down the mouth, esophagus, into the stomach. This helps the doctor visualise the upper GI tract and take samples of abnormal tissue if any. These can help differentiate between Achalasia from cancer or carcinoma of the esophagus.
• Esophageal Manometry - Pharyngeal and esophageal manometry can help your doctor measure the change in pressure in your throat and esophagus as you swallow. This can help determine any rigidity or weakness in the esophageal tract and is one of the most reliable tests for Achalasia.
• X-Ray – An X-Ray of the chest combined with a barium contrast can help determine abnormalities such as the narrowing of the esophagus. This is a tell-tale sign of Achalasia, often called Bird Beak.

What are the Treatment Options for Achalasia Cardia?

Achalasia has no known cure. Therapy aims to help relax and dilate the esophageal sphincter to allow food and liquid to pass into the stomach. Depending on the severity of the condition, your doctor may recommend -

Non-surgical Treatment

• Pneumatic Dilation - Pneumatic Dilation involves the insertion of an air-filled balloon through an endoscopic catheter to help keep the esophageal sphincter open. This procedure may need to be repeated subsequently.
• Botox (Botulinum Toxin Type A) - Botox (Botulinum Toxin Type A) is a muscle relaxant and when injected directly into the esophageal sphincter. This, again, is a therapy offering temporary relief.
• Medicines - Medicines such as nitro-glycerine or nifedipine may be recommended to ease the symptoms, but these have strong side effects.

Surgical Treatment

• Heller Myotomy - Heller Myotomy may be performed with or without fundoplication. This surgery cuts the esophageal muscles near the sphincter allowing food to pass into the stomach. This is often done laparoscopically and is minimally invasive.
• Peroral endoscopic myotomy (POEM) - Peroral endoscopic myotomy (POEM) has shown much promise in treating patients with Achalasia Cardia. It is less invasive than Heller myotomy and uses an endoscope to loosen the muscles in the esophagus by cutting them.

Complications of Achalasia Cardia

The greatest challenge faced by Achalasia patients involves early diagnosis. Achalasia symptoms are often mistaken for GERD or other gastrointestinal conditions. Sometimes, the symptoms can cause serious damage. As the condition progresses, it can cause worsening chest pain. Food accumulation in the esophageal tract can cause pain and even lead to serious infections. Dietary insufficiency due to the condition can cause malnutrition and weight loss. The risk of choking due to food or drink entering the airway is significant.

This can also cause pneumonia due to Regurgitation and Aspiration of food. Apart from this, Achalasia is not known to cause heart disease, but the condition increases the risk of developing esophageal cancer.

Diet Plan to Manage Achalasia Cardia

Achalasia Cardia is not curable, but lifestyle and dietary changes can help improve the patient's quality of life. Achalasia cannot be cured or treated by dietary changes, but certain soft foods are easier to swallow and cause less esophageal irritation or symptoms like heartburn. If the condition has caused a lot of weight loss, you may need to consume a high-calorie diet. With the progress of the disease, you may want to avoid certain foods like:

• Tough proteins like meats or legumes
• Caffeine or alcohol
• Very spicy or fatty foods

It is important to cut the food into small pieces and chew well before swallowing to help the food pass into the stomach. It is a good idea to avoid consuming very dry foods.

We at Max Super Speciality Hospital are one of the few centers where manometry is being performed and we offer incisionless endoscopic surgery called POEM (Per oral endoscopic myotomy) for achalasia. POEM procedure for achalasia is a safe procedure offering excellent long-term results, and also not requiring long hospital stays. After 24 hours of the POEM procedure, a liquid diet is started.

At Max hospital, Saket, our gastroenterology team with cumulative experience of more than 150,000 endoscopic procedures, is providing cutting-edge treatment for patients with complex GI disorders. Our team, under the leadership of Dr. Vikas Singla, has one of the highest experiences in the treatment of achalasia cardia. The team has already performed more than 400 cases of per oral endoscopy myotomy in patients of achalasia cardia with excellent results.

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