Achalasia Cardia: Symptoms, Causes & Modern Treatment Options

Achalasia Cardia is a rare disorder of the esophagus or food pipe, characterized by difficulty in swallowing food. In normal conditions, swallowed food passes through the food pipe into the stomach. This requires coordinated action of the esophagus known as peristalsis and relaxation of the lower esophageal sphincter known as the lower esophageal sphincter or LES. In patients with achalasia cardia, both the contraction of the esophagus muscle and relaxation of the LES are defective, leading to difficulty in swallowing food. Patients usually complain of a choking sensation after swallowing food or liquids.

Causes of Achalasia Cardia

The exact etiology of achalasia cardia is unknown, however various viral infections have been implicated in causing achalasia cardia.

Symptoms of Achalasia Cardia

Difficulty Swallowing Food

Difficulty in swallowing food, both solids and liquids. Many times patients modify their diet, take more water to assist swallowing or perform various manoeuvres such as raising shoulders to assist swallowing. Many times patients take longer to finish food. In social gatherings, patients are not able to take adequate food, leading to dissatisfaction. Many patients tend to avoid social meetings to avoid such circumstances.

Regurgitation of Food

Regurgitation of food, liquid food coming back from mouth or nose specially during night. Food and liquids swallowed during the day are not able to move down in the stomach and get accumulated in the food pipe. When a patient lies down, the liquid food may come back through the mouth or nose and will cause awakening from sleep.

Recurrent Chest Infections

Food from the esophagus may move upward and pass into the food pipe and lungs. This condition may lead to recurrent episodes of chest infections.

Weight Loss

Weight loss due to poor oral intake. If the condition is untreated, due to poor oral intake, patients may suffer from weight loss.

Diagnosis of Achalasia Cardia

Because of less awareness in the medical community, there is often delay in diagnosis of achalasia cardia.

Upper GI Endoscopy

GI endoscopy shows slight resistance at the lower part of the esophagus known as the lower esophageal sphincter or LES, without any evidence of cancer, tumor, ulcer or stricture. Many patients may have fear of pain during endoscopy. It is always good to perform endoscopy after 8 hours of fasting with proper sedation.

Esophageal Manometry

If achalasia cardia is suspected during endoscopy, the next test should be esophageal manometry. Esophageal manometry should be performed in a conscious state as the patient has to swallow water during manometry. During manometry, a catheter is passed through the nose into the esophagus. Initially baseline pressures are recorded, after that patients take multiple swallows of water. After swallowing, pressures in the food pipe and lower esophagus sphincter are measured. Based on Chicago 4 classification, diagnosis of achalasia cardia is confirmed. Achalasia cardia has three types, which can be easily diagnosed on manometry.

Barium Swallow

During barium swallow, patients swallow small amounts of barium and movement of barium is tracked in the lower esophagus. Barium may reveal dilated esophagus and resistance of passage to barium in the lower esophagus across the LES.

Treatment Options for Achalasia Cardia

Medical Management

Medical management has limited efficacy for patients with achalasia cardia. Medicines have to be swallowed before meals and may relax the lower esophageal sphincter. Medicines have limited efficacy for treatment of achalasia cardia.

Per Oral Endoscopic Myotomy (POEM)

POEM is a minimally invasive endoscopic procedure designed to address the motility issues associated with achalasia cardia. It is performed entirely through the mouth using an endoscope.

Procedure

• Once diagnosis is confirmed, the patient is admitted for 48 to 72 hours for performing this procedure. The patient is first evaluated for fitness of procedure by anaesthesia team. After fitness for the procedure, POEM is performed under general anesthesia, no pain occurs while performing the procedure.
• Endoscope insertion: The endoscope is introduced through the patient's mouth and guided down into the esophagus. Using the endoscope, a small incision and tunnel is created in the esophageal wall, followed by cutting the muscles of the tight esophageal sphincter. The last step is closure of the initial incision with the help of clips. Clips do not cause any pain or difficulty in swallowing and may fall off spontaneously in most patients in the next 3 to 6 months.
• POEM is a safe procedure and thousands of procedures have been performed across the globe safely.

Post Operative Care

• Patients are usually kept in the hospital for 24 to 48 hours after the procedure for monitoring.
• X-ray along with ingestion of contrast is performed next week, following which a liquid or soft diet is recommended initially and gradually transitioning to regular foods.
• Follow-up appointments and tests are scheduled as per requirement.

Efficacy of POEM

After POEM, 90 to 95 percent of patients can overcome the difficulty in swallowing food and the problem of regurgitation of food.

Gastroesophageal Reflux Symptoms After POEM

Five to ten percent of patients may feel reflux symptoms of heartburn and regurgitation. Recent studies have shown that long-term clinical symptoms of GERD are equal after POEM or surgery.

Pneumatic Balloon Dilatation

During pneumatic balloon dilation, the balloon is placed across the lower esophageal sphincter. The balloon is inflated with air and forceful dilation with the balloon leads to rupture of muscles in the lower esophageal sphincter leading to reduction in pressure. Repeated balloon dilatation may be required. Recent evidence has shown that POEM is more effective than balloon dilatation. Balloon dilatation may lead to excessive rupture of muscle and can lead to perforation which can be grave complications.

Botulinum Toxin Injection

With the help of endoscopy, botulinum is injected in a tight esophageal sphincter. Injection has to be repeated every 3 months and is performed in patients who have high risk for complications and cannot withstand longer procedures.

Surgical Option

Muscle cutting of a tight lower esophageal sphincter can also be performed with laparoscopic surgery. During laparoscopic surgery, a small incision is made in the skin and cutting of tight sphincter muscle is performed. A recent study published in a medical journal has shown equal efficacy and clinical reflux problems after POEM and laparoscopic surgery.

Conclusion

Achalasia cardia is a complex condition that requires timely diagnosis and expert management. With modern endoscopic and surgical techniques, most patients achieve excellent long term relief. Early evaluation and appropriate treatment help prevent complications and significantly improve quality of life.