Achalasia cardia has three main features;

• The lower esophageal sphincter (LES, the ring of muscle at the lower end of the food pipe) does not relax.
• Aperistalsis (the food pipe does not push food down).
• High pressure in the LES

Introduction

 Achalasia cardia is a problem with the movement of the food pipe (esophagus). The LES does not relax. So food cannot pass easily into the stomach. The food pipe also loses peristalsis. This is the wave of muscle movement that pushes food down. It happens in the whole digestive tract (GIT). In the stomach, it mixes food. In the intestine, it moves digested food forward.

Pathophysiology of Achalasia cardia

Here, the inhibitory nerve cells (nerves that tell muscles to relax) break down. They sit in the myenteric plexus. Doctors also call it the Auerbach plexus.

 Myenteric plexus

It is a group of nerves in the wall of the gut. It lies between two layers of smooth muscle. This plexus controls peristalsis. But when it fails, the LES cannot relax.

Degenerated pre and post ganglionic nerve cell on electron microscopy.

Etiology/ causes of Achalasia:

There are two types. They are primary and secondary achalasia.

Primary achalasia

Primary achalasia is idiopathic. This means the cause is unknown. But these causes may play a role:

• For instance, an autoimmune attack may damage the nerves. In this case, the body’s immune system hurts its own healthy cells.
• Also, viruses may trigger it. But experts have not found one sure viral cause.
• In particular, herpes simplex virus type 1 (HSV-1) is the virus most often linked to it.

secondary achalasia.

Also, doctors call it pseudoachalasia. It looks like the primary type. But another disease causes it. Most often, a blockage such as esophageal cancer or stomach cancer is the cause.

 Chagas Disease 

Specifically, a tropical parasite called Trypanosoma cruzi causes it. A bug bite spreads the disease. Then the parasite damages the automatic nerves of the food pipe. As a result, the LES cannot relax. This causes secondary achalasia.

Chicago classification

To decide the type, doctors use manometry. It is a test that measures;

• Pressure inside the food pipe.
• How well the LES relaxes.
• How hard the LES squeezes.

Also, it is the gold standard test (the best test) for this disease. It also helps doctors pick the right treatment for each type.

 Procedure of Esophageal Manometry

During the test, the physician passes a pressure-sensitive catheter through the nose into the esophagus. As the patient swallows small sips of water, the machine records esophageal pressure and LES relaxation. 

Type 1 Achalasia 

Doctors also call it the classic type. The test shows;

• No squeeze of the food pipe muscle
• Very low pressure inside the food pipe

Type 2 Achalasia

The test shows high pressure in the whole food pipe at the same time during swallowing. Doctors call this pan-esophageal pressurization;

• There is no peristalsis
• Also, it responds best to treatment

 Type 3 Achalasia 

Doctors also call this type spastic. The food pipe has sudden, tight spasms, while the LES does not relax. As a result, patients feel severe chest pain.

Clinical Features of Achalasia Cardia

 Dysphagia

         Notably, this is the most common feature. Dysphagia means trouble swallowing. With a physical blockage, patients find only solid food hard to swallow. But in this disease, both solids and liquids are hard to swallow.

Regurgitation

        Typically, undigested food comes back into the mouth. It mostly happens at night, while the patient sleeps.

Chest Pain

       In fact, many patients report chest pain. It can be so severe that they eat less and lose weight. It is most common in type 3 because of sudden spasms of the food pipe muscle.

 Weight Loss

      As a result, patients lose weight because they eat less.

Heartburn

          Heartburn may occur because food stays in the food pipe. Also, irritation can cause it. After treatment, acid reflux (acid coming back up) may cause it too.

• Cough at night
• Wheezing sound
• Aspiration Pneumonia

 How To Diagnose  Achalasia Cardia?

Barium Swallow

       Essentially, this X-ray test shows the food pipe and how swallowing works.

 Procedure

First, the patient drinks a white liquid called barium. It coats the food pipe. As a result, the coating shows clearly on the X-ray. Then the X-ray often shows;

• A wide food pipe at the top (dilated esophagus)
• A narrow LES
• A bird beak shape at the lower end

Upper GI endoscopy for Achalasia Cardia

In this test, the doctor passes a thin camera tube from the mouth to the stomach. It helps tell primary achalasia from cancer. In primary achalasia, the scope passes through the LES with gentle pressure. But it is hard to pass in cancer. The scope also shows;

• Leftover food in the food pipe, even after fasting
• A tight or closed LES

Finally, high-resolution manometry is the gold standard test. So it confirms;

• Poor LES relaxation
• High integrated relaxation pressure (IRP)
• No peristalsis

CT- scan

It also helps find cancer or other blockages that look like it.

High Resolution Manometry of Achalasia Cardia

It measures the pressure of the food pipe and the LES. So it is the best test to confirm the diagnosis.

 Differential Diagnosis

• Cancer (carcinoma)
• GERD (acid reflux disease)
• Diffuse esophageal spasm (sudden tight squeezes)
• Esophageal stricture (a narrow food pipe)

How to Manage Achalasia Cardia

      But doctors cannot repair the nerve damage. Still, several treatments can relieve symptoms.

Medical Treatment of Achalasia Cardia

Medicines give only short relief. So doctors give them to patients who cannot have surgery.

• Nitrates (isosorbide dinitrate) relax the LES.
• Calcium channel blockers (nifedipine) also lower the pressure in the LES.

Pneumatic Dilatation for Achalasia Cardia

•       Doctors use a balloon to stretch a tight LES. First, the patient gets light anesthesia (medicine for sleep). Then the doctor passes a camera tube from the mouth into the food pipe. Next, the doctor passes a guide wire through the tube. After that, the doctor removes the tube and leaves the wire in place. Then the doctor slides a balloon over the wire and places it across the LES. Finally, the doctor inflates the balloon with air or contrast fluid for 30 to 60 seconds.
• This method has a small risk of a tear (perforation) in the food pipe. So doctors watch the patient closely afterward.

Botulinum toxin injection

Also, doctors inject botox into the LES. It blocks acetylcholine (a chemical that makes muscles squeeze). But the benefit lasts only a short time. So doctors use it in older patients who are at high risk.

Heller Myotomy

Heller myotomy is an operation that cuts the tight LES muscle. Surgeons use a camera through small cuts in the belly (laparoscopy). Also, they often add a fundoplication. This means they wrap the top of the stomach around the lower food pipe to stop acid reflux. As a result, it gives long-term relief.

POEM

Notably, POEM is a modern treatment.

• Per oral means through the mouth
• Endoscopic means using a camera tube
• Myotomy means cutting muscle

First, the doctor passes a camera tube through the mouth. Next, the doctor makes a small cut in the inner lining of the food pipe. Then the doctor cuts the tight muscle fibers. Finally, the doctor closes the entry site with clips.

     

Advantages Of POEM

First, there is no cut on the skin. Second, recovery is fast. Also, it works very well. It is especially good for type 3.

Disadvantage of POEM

But the risk of GERD (acid reflux disease) is higher. This is because POEM does not include a fundoplication.

Complications of Achalasia cardia

First, ulcers may form in the food pipe. Also, the risk of cancer rises if the disease stays untreated for a long time. Next, leftover food can cause esophagitis (swelling of the food pipe). Then regurgitation can cause aspiration pneumonia. In some cases, the food pipe becomes very wide (megaesophagus). As a result, malnutrition (poor nutrition) can occur. Rarely, a tear (perforation) may occur after treatment.

FAQs

Medical Disclaimer

This article is for education only. It is not medical advice, a diagnosis, or a treatment plan. This condition needs a proper check-up. Taking medicines without a doctor’s advice can cause harm. So if you have trouble swallowing or chest pain, see a doctor right away.

About the Author

Dr. Sufiyan Awan (MBBS) is a medical doctor and health writer. He worked as a House Officer at Nishtar Hospital, Multan. There he treated many patients. He also took training from the World Health Organization (WHO). He writes clear, evidence-based articles. They help people understand health topics easily.