Achalasia Cardia: Uncommon but an important cause for difficulty in swallowing

Posted by Dr. Chirag Thakkar on February 26th, 2020

Achalasia Cardia is a rare problem affecting swallowing. It needs special tests for its diagnosis and treatment by an expert doctor. Although not a life-threatening problem, early diagnosis and proper treatment are needed for achieving a good quality of life. 

What is Achalasia Cardia?

Achalasia is a rare swallowing disorder, that makes it difficult for food and liquid to pass into your stomach. Most people are diagnosed between the age of 25 and 60 years. But it is rarely seen is children also. In achalasia, nerve cells in the esophagus (or what we call a food pipe) degenerate for reasons that are not known. This leads to distrubance in the swallowing function of food pipe  Food pipe is the tube that carries food from the mouth to the stomach.

Normal swallowing

To understand what happens in Achalasia Cardia, let us first understand the normal process of swallowing. Normally, when we swallow food, food is pushed down the food pipe by rhythmic contractions of the muscles of the food pipe. These contractions are called peristalsis. Also, as we swallow food, the LES valve relaxes and opens up to allow the food to pass to the stomach. The LES valve or the Lower Esophageal Sphincter is located at the lower end of the food pipe relaxes. And once the food reaches the stomach, the valve closes tightly. Thus it also prevents the reflux of food and acid from the stomach back to the food pipe. Acid reflux causes ulcers in the food pipe and leads to heartburn. Thus proper functioning of this valve is important

What happens to the food pipe in Achalasia cardia

In people with achalasia, the loss of nerve cells in the esophagus that is the food pipe, causes two major problems that interfere with swallowing:

First, the muscles that line the food pipe do not contract properly. So the swallowed food is not propelled from the food pipe into the stomach in a normal manner.

Second, the lower esophageal sphincter that is the LES valve, fails to relax normally with swallowing. This creates a barrier that prevents food and liquids from passing into the stomach.

Over a period of time, the food pipe above the LES valve dilates, and large volumes of food and saliva can accumulate in the dilated food pipe.

Symptoms of Achalasia Cardia: What happens to the patient in Achalasia Cardia

The most common symptom of achalasia is difficulty swallowing. Patients often experience the sensation that swallowed material, both solids, and liquids get stuck in the chest. This may cause chest pain while eating. The food and liquid that is stuck in the chest many a time flows back to the mouth when patients lie down. Occasionally this may cause cough or water coming out of the nose during sleep. Residual food remaining in the food pipe can cause ulceration and heartburn. Weight loss is also seen in most of the patients diagnosed as Achalasia.

Patients of Achalasia also has a higher chance of developing cancer of the esophagus in the future, hence timely treatment and good follow up is important

This problem often begins slowly and progresses gradually. Many people do not seek help until symptoms are advanced. Some people compensate by eating more slowly and by using maneuvers, such as lifting the neck or throwing the shoulders back, to improve the emptying of the esophagus.

Diagnosis of Achalasia Cardia

Achalasia can be overlooked or misdiagnosed because it has symptoms similar to other digestive disorders. The initial test for any patient complaining of a problem with swallowing is endoscopy and a dye test.

• Upper endoscopy

  Your doctor inserts a thin, flexible tube equipped with a light and camera called an endoscope, down your throat, to examine the inside of your esophagus and stomach. In Achalasia patients food residue is commonly seen in the food pipe. Also, the valve does not open up easily on the passage of the endoscope, as would happen in a normal person. The main role of endoscopy is to rule out other reasons for difficulty in swallowing like tumors, narrowing (strictures), severe ulcers and foreign bodies.

• Barium/Dye swallow test

  X-rays are taken after you drink a chalky liquid called barium or a thin watery dye, that coats and fills the inside lining of your food pipe. A radiologist can view the passage of this dye through your food pipe into your stomach on an X-ray machine. In persons having Achalasia, the x-ray images will show hold up of dye in your food pipe, dilatation of your food pipe and narrowing at the lower end suggesting non-opening of the valve. Although quite accurate, these findings are sometimes absent in the initial phase of the disease, when your food pipe is not much dilated.

• Esophageal manometry

  Manometry is the final confirmatory test and is a must to confirm the diagnosis of achalasia. This test measures the rhythmic muscle contractions in your esophagus when you swallow, the coordination and force exerted by the esophagus muscles, and how well your lower esophageal sphincter relaxes or opens during a swallow. In early phase of Achalasia, when barium and endoscopy can be normal, manometry can be the only test that will diagnose Achalasia. It also helps in deciding the types of Achalasia Cardia. The outcome various treatment depends on the type of Achalasia, thus manometry also helps in predicting the outcome and deciding the treatment.
• Read more about esophageal manometry and upper endoscopy.
• To know Why and when Manometry is needed, How Manometry is done, watch these videos

Treatment of Achalasia Cardia

Regarding treatment, we must understand that none of the treatments can stop or reverse the underlying loss of nerve cells in the esophagus. Also, none of the available treatments are expected to restore normal peristaltic contractions in the food pipe of patients with achalasia.

All the treatments aim to weaken the lower esophageal sphincter that is LES valve, to the point that it no longer poses a barrier to the passage of food.

Thus the food will pass down the food pipe with the help of gravity. And without the resistance of the LES valve, it will pass into the stomach easily. Overall, surgical treatments are usually effective for improving symptoms.

Let’s first talk about the Nonsurgical options

  The non-surgical option includes medication, endoscopic botox injection and endoscopic balloon dilatation. But we are not discussing medications and a Botox injection in detail as they do not have good results and are not commonly advised to patients.

Thus the non-surgical option available for all practical purposes is Pneumatic dilation.

• It is an endoscopic procedure, where a balloon is inserted into the food pipe and inflated at the LES valve to tear the sphincter muscle and keep it open. It is a crude and inaccurate method of opening of the valve and hence has inferior results compared to the surgical treatment. This procedure may need to be repeated if the esophageal sphincter doesn’t stay open. Nearly one-third of people treated with balloon dilation need repeat treatment. There is also a small chance of perforation or creating a hole in the food pipe during the procedure. The chance of this complication is about 2-4 % and is likely to need emergency surgery to correct it. As balloon dilatation does not include anti-reflux measures, the chances of acid reflux after pneumatic balloon dilatation are also very high.

Surgery for Achalasia Cardia

Surgery is a more effective treatment with very good long term results. Here the muscle of the LES valve is systematically divided under the vision to keep the valve open. Surgical options include laparoscopic and endoscopic surgery. Let’s discuss one by one

Laparoscopic surgery for Achalasia Cardia: is called Heller Cardiomyotomy with Dor/Toupet Fundoplication

• This is the Gold standard treatment of Achalasia cardia. Laparoscopy means a camera and instruments are inserted in your tummy through small cuts and surgery is performed.The surgeon cuts the muscle, which is the LES valve at the lower end of the food pipe to keep it open. This allows the food to pass more easily into the stomach. As this is done under vision, the division of muscles is accurate. In cases where the division of the muscles leads to creating a hole in the inner layer of the food pipe, it is closed and repaired then and then only without affecting the outcome. Endoscopy is done during the surgery itself to confirm the valve has been opened adequately and that no hole has occurred. Once the valve is opened, the surgeon wraps the top part of your stomach and fixes it to the cut end of muscles of the valve and the hiatus. This is called a partial or Dor fundoplication. It is done to prevent the reflux of acid from the stomach to the food pipe, which commonly occurs once the valve is open.

Pre and post surgery course for Laparoscopic surgery

Usually, patient is admitted on the day of surgery or previous evening. After surgery liquids are started on the same day. This is followed by a soft diet once liquids are tolerated. A normal diet can be resumed within about a week’s time. Patients are out of bed and can move around normally few hours after surgery. Most patients are discharged the next day of surgery. In case the hole in the food pipe has been repaired during surgery, diet and discharged can be delayed by one or two days.

Outcomes of Laparoscopic surgery for Achalasia Cardia

This surgery gives excellent long term outcomes. 70-90% of patients have good symptomatic relief over the long term. The outcome depends on the type of Achalasia as defined by the manometry and on the amount of dilatation of the food pipe as seen on a dye swallow test. Patients going for surgery early have better outcomes compared to the ignored and neglected cases where the food pipe is too much dilated. Also with the addition of fundoplication, the chances of acid reflux are reduced significantly.

Endoscopic surgery: Peroral endoscopic myotomy (POEM)

This surgery is done by inserting the endoscope and the instrument through your mouth. The surgeon uses an endoscope inserted through your mouth and down your throat to create an incision or a cut in the inside lining of your esophagus. Then, as in a Heller myotomy, the surgeon cuts the muscle at the lower end of the esophageal sphincter under vision. The cut on the inner lining is finally closed with endoscopic clips. The resolution of symptoms is comparable to laparoscopic surgery, but it is a newer procedure and hence long term results need to be seen.

Pre and post surgery course after POEM

Usually patient is admitted on the day of surgery or previous night. As a part of preparation for surgery, patients are advised to take clear liquids diet for 2 days before surgery. This is necessary as the surgery mandates a cut in the inner layer of food pipe. Due to his reason, your surgeon would want your food pipe to be clean at the time of surgery, and not filled with food particles. Patient are out of bed and can move around few hours after surgery. Liquid diet is started about 2 days after surgery followed by soft diet after few more days. Initial restriction in diet is advised to give rest to the repair of the cut in the inner layer of your foodpipe.  

Outcomes of POEM

The improvemnt in swallowing after POEM is very good. Almost equal to that seen with a laparoscopic surgery. The main disadvantage of this procedure is that it doesn’t include an anti-reflux procedure meaning a fundoplication. And hence the chances of acid reflux are very high, in the range of 40-60%. Patients who develop acid reflux after surgery would need to take antacid medications for a long period.

 Achalasia Cardia follow up

Since none of the treatments for achalasia cure the underlying disease, and because there has a higher chance of developing esophagus cancer, regular follow-up is needed.

The goal is to recognize and treat recurrent symptoms or complications, especially GERD.

Hope this information has helped you understand your problem. For further queries or clarification, you call us, WhatsApp or email your reports to us on 8146078064 /079-29703438 or drchiragthakkar1307@gmail.com.

Watch these videos to listen to the experience of patients with Achalasia Cardia