Achalasia Treatment

Achalasia is a rare but serious motility disorder characterized by the inability of the muscles at the lower end of the esophagus to relax and by impaired esophageal movements. This condition causes symptoms such as difficulty swallowing and food getting stuck in the esophagus.

The question of what the symptoms of achalasia are is important for the early diagnosis of the disease. Difficulty swallowing, especially the sensation of sticking with solids and liquids, chest pain, regurgitation (food coming back up), and weight loss are the most common findings. Symptoms may progress over time.

The question of how achalasia is diagnosed forms the basis of the diagnostic process. A definitive diagnosis is made with advanced imaging and functional tests such as an esophageal transit graph, endoscopy, and manometry. Manometry in particular is critical in evaluating lower esophageal sphincter pressure and peristalsis.

The question of how achalasia treatment is performed varies according to the patient’s symptoms and the stage of the disease. Surgical and endoscopic methods such as balloon dilation, botulinum toxin injection, POEM (peroral endoscopic myotomy), and Heller myotomy may be used. The aim is to facilitate food passage by reducing sphincter pressure.

What Is Achalasia?

Achalasia is a rare motility disorder that occurs as a result of the inability of the lower esophageal sphincter at the lower end of the esophagus to relax and the disruption of esophageal muscle movements. It progresses with difficulty swallowing, chest pain, weight loss, and the return of undigested food. Diagnosis is made with manometry, endoscopy, and radiological examinations. Balloon dilation, botulinum toxin injection, or surgical myotomy may be applied in treatment.

What does achalasia disease change in our body?

A normal swallowing process is a much more organized and complex operation than you think. The moment you swallow a bite, rhythmic waves descending from top to bottom begin in your esophagus. We call this peristalsis. These waves push the food downward like squeezing a tube of toothpaste. When the bite reaches the entrance to the stomach, the valve system located here, called the “lower esophageal sphincter,” which is normally closed, automatically relaxes, opens the door, and allows the food to enter the stomach. As soon as the process is over, it closes tightly again to prevent stomach acid from escaping upward.

In patients with achalasia, this perfectly functioning system collapses at two points. First, the esophagus loses that rhythmic pushing force. In other words, the motor that will push the bite downward is broken. The second and main problem is that the door at the stomach entrance does not receive the “open” command. The patient swallows, the esophagus starts to fill, but the valve below stubbornly remains closed. As a result, food accumulates in the esophagus until it is forced downward by the effect of gravity. This situation causes the esophagus to widen and its form to deteriorate after a while.

What causes achalasia and in whom is it seen?

This is one of the questions for which we are still looking for a clear answer in the medical world. Our patients often ask, “Did I cause this? Did I eat wrong?” The answer is no, this is not your fault. Achalasia is a neurodegenerative process involving the loss of cells in the nerve network (ganglion cells) that control the esophageal muscles. In other words, the problem is not so much with the muscle itself as with damage to the nerves that give commands to that muscle.

Although we do not have definitive evidence as to why this nerve damage begins, we have strong theories. It is thought that some people have a genetic predisposition. It is emphasized that viral infections added on top of this may be triggering factors. In addition, autoimmune mechanisms in which the body’s immune system becomes confused and attacks its own nerve cells are also among the blamed factors. Whatever the cause, with our current medical knowledge it is not possible to reverse this nerve damage or revive the dead cells. Therefore, our treatment strategy is based not on correcting the cause but on managing the result. In other words, we aim to return life to normal by mechanically opening that door that does not open.

What are the symptoms of achalasia?

The disease usually begins insidiously. Since complaints increase gradually, our patients may normalize the situation or dismiss it by saying, “It happened because I didn’t chew well.” However, over time the picture becomes clearer. The most typical symptom is difficulty swallowing, which begins with solid foods and over time is also reflected in liquids. Not only the sensation of sticking, but also the pressure and pain felt behind the rib cage are common.

Common symptoms seen in patients with achalasia are:

• Difficulty swallowing solid foods
• Difficulty swallowing liquids
• Pain in the middle of the chest after eating
• Food coming back into the mouth
• Unintentional weight loss
• Cough that wakes one from sleep at night
• Wetness or stains on the pillow
• Frequent recurring throat infections
• Bad breath
• A sensation of blockage while eating

Especially the condition we call “regurgitation,” in which undigested food comes back into the mouth, is very socially challenging for patients. Unlike nausea or vomiting, because the food never reaches the stomach, it comes back into the mouth spontaneously without effort.

What is the relationship between night cough and achalasia?

This issue is very critical in terms of understanding the seriousness of the disease. Many patients cannot establish a connection between difficulty swallowing and night cough, and for years try to be treated for asthma or allergies in pulmonary disease outpatient clinics. However, the mechanism is very simple. The foods you eat during the day and that accumulate in the esophagus flow backward when you lie down in bed at night because the effect of gravity disappears.

These food residues and saliva overflowing from the esophagus may enter your windpipe. We call this “aspiration.” This condition causes you to wake up suddenly at night with a feeling of choking and severe coughing attacks. More dangerously, these food particles can go down into your lungs and cause pneumonia or chronic lung damage. If you have an unexplained persistent cough and difficulty swallowing, the source of the problem may not be your lungs but your esophagus.

How is achalasia diagnosed?

In a patient presenting with a complaint of difficulty swallowing, we need to perform a series of tests to confirm the diagnosis and exclude other possible diseases. Because similar complaints can also be seen in reflux disease or esophageal tumors. For this reason, we approach with suspicion and act with evidence.

The methods used in the diagnostic process are:

• Endoscopy
• Barium esophagography
• High-resolution manometry
• Computed tomography

Endoscopy is our first-step procedure. By seeing the inside of the esophagus with a lighted camera, we check whether there is a mechanical obstruction, tumor, or irritation. In patients with achalasia, seeing food residues inside the esophagus and noticing that the entrance to the stomach is closed gives us the first clue. In a barium study, an X-ray is taken while the patient drinks a contrast liquid. The thinning of the lower end of the esophagus into a “bird’s beak” appearance is very typical for achalasia.

However, the test we consider the “gold standard” for a definitive diagnosis today is High-Resolution Manometry (HRM). In this test, a thin catheter is passed through your nose and the pressures inside the esophagus are measured. This test tells with millimetric precision whether the valve relaxes during swallowing and whether there is contraction in the body of the esophagus. It not only makes the diagnosis, but also allows us to determine which subtype of the disease it is (Type 1, 2, or 3), which is of vital importance for predicting treatment success.

Is non-surgical achalasia treatment possible?

The most natural reaction of our patients is to avoid the operating table and seek a solution with simpler methods. There are some non-surgical methods that have been applied in the past and still today, but there are serious limitations regarding their permanence and effectiveness:

Drug therapy is generally the first route tried. Drugs such as nitrates or calcium channel blockers can provide temporary relief by relaxing the valve muscles. However, the effect of these drugs is very short-lived, they need to be taken before every meal, and side effects such as headache and low blood pressure often overshadow the benefit they provide.

Another method is Botulinum toxin injection. Botulinum toxin is applied to the muscles endoscopically, and relaxation is achieved by causing paralysis of the muscles. Although it seems like an effective method, its effect lasts an average of 6 months. Repeated injections, on the other hand, harden the tissue in that area (fibrosis), making the permanent operation that will need to be performed in the future much more difficult and risky.

Balloon dilation is another option. A balloon is inflated endoscopically and the muscle fibers are attempted to be torn. However, this procedure is a “blind” one. You cannot fully control how much tearing the balloon will create. It may be insufficient or, worse, it may tear the esophagus full-thickness (perforation), leading to a life-threatening condition requiring emergency surgery. Statistics show that the vast majority of patients who undergo balloon treatment eventually need surgery anyway. For this reason, today we do not recommend these temporary methods as a first choice in candidates suitable for surgery.

How is laparoscopic Heller myotomy surgery performed?

At the point modern medicine has reached, the most effective, safest, and most satisfactory treatment for achalasia is Laparoscopic Heller Myotomy (LHM) surgery. This surgery is a mechanical solution that perfectly corrects not the cause of the disease but its result.

The operation is performed under general anesthesia. There is no need to open the abdomen from top to bottom as in the past. The camera and fine surgical instruments are inserted through 5 tiny holes (ports) opened in the abdominal wall. The laparoscopic method, that is, closed surgery, keeps the patient’s comfort at the highest level. The surgeon clearly visualizes the region where the esophagus meets the stomach. Here, the muscle layer that remains contracted and does not allow food passage is carefully cut without damaging the mucosal layer above it (the inner lining of the esophagus). This procedure is like cutting and loosening the neck of a tight sweater. When the muscles are cut, that tight knot is resolved and the path for food passage is opened wide.

The success rate of this operation is quite high. More than 90% of patients are completely relieved of difficulty swallowing after surgery. The hospital stay is usually 2-3 days, and patients can return to their normal lives in a short time. Since there are no large incisions in the abdominal area, postoperative pain is minimal and cosmetic results are excellent.

Why is an anti-reflux procedure necessary?

This is the point to which we as surgeons show the greatest sensitivity and which our patients most need to know. In achalasia surgery, when we cut those tight muscles, we are actually disabling the body’s natural “doorkeeper.” Yes, we opened the door and food now goes down to the stomach comfortably. However, because the door remains constantly open, it is only a matter of time before acid in the stomach escapes upward into the esophagus (reflux).

If we only cut the muscles and leave it at that, the patient gets rid of the difficulty swallowing but this time is left alone with severe reflux that poisons life. Reflux does not only create a burning sensation; in the long term it irritates the lower end of the esophagus, paving the way for wounds, strictures, and even cancer risk. That is why, as an indispensable part of the Heller Myotomy procedure, we add what we call “Dor Fundoplication.”

In this procedure, we lay the upper part of the stomach (fundus) over the esophageal area we have cut like a patch and suture it. This creates a loose but functional new valve mechanism. While this method does not prevent foods from going down, it prevents stomach acid from escaping upward. This strategy both solves the swallowing problem and protects the long-term health of the esophagus.

Is the POEM method or surgery better?

In recent years, with technological developments, a new method called POEM (Peroral Endoscopic Myotomy) has become popular. In this procedure, the muscles are cut from the inside by entering only through the mouth with an endoscope, without any skin incision. Although it sounds very attractive, just as every method has its advantages and disadvantages, POEM also has aspects that need to be considered:

The biggest advantage of POEM is that it leaves no scar on the skin and is especially very effective in patients who have previously undergone unsuccessful surgeries or in spastic type achalasia (Type 3). However, the biggest disadvantage of POEM is that the anti-reflux mechanism (fundoplication) we just mentioned cannot be performed with this method. The valve muscles are cut, but no new barrier can be put in place.

Research and clinical experience show that postoperative reflux rates are significantly higher in patients who undergo POEM compared to laparoscopic surgery. Reflux is a serious risk, especially for young patients with a long life expectancy. As thoracic surgeons, we see the combination of LHM and Dor Fundoplication as a more balanced and protective approach that both solves the swallowing problem and keeps reflux under control. POEM, on the other hand, remains in our hands as a strong “rescue” option, generally in cases recurring after surgery or in special situations not suitable for surgery.

How is advanced-stage achalasia (Sigmoid) treated?

If the disease is left untreated or neglected for many years, the esophagus becomes excessively widened and elongated due to food accumulation. It reaches such a point that it is no longer a straight tube but takes the shape of a curved “S” letter. We call this “Sigmoid Achalasia” or end-stage disease. At this stage, the esophagus has virtually turned into a bag and has completely lost its ability to contract.

In these patients, a standard myotomy (muscle-cutting) procedure may sometimes be insufficient. Because even if you open the lower valve, the esophagus is so misshapen and so curved against gravity that food cannot flow into the stomach under its own weight. In this case, complete removal of the esophagus (esophagectomy) may be recommended in some centers. However, this is a very major and risky operation.

Our approach is to preserve the organ as much as possible. In these advanced-stage patients, we may apply a special technique called “Esophagogastrostomy.” In this method, we open a wide side-to-side window between the lower part of the esophagus and the stomach. Thus, by bypassing the narrowed area, we allow foods to pass directly into the stomach through a wide opening. This procedure saves the patient from losing the esophagus and seriously increases quality of life. It is a vital “rescue surgery” for these advanced-stage patients.

How should nutrition be after surgery?

Undergoing surgery is only one part of the process. Afterwards, getting used to a new diet reinforces the success of the treatment. Immediately after surgery, we usually start with liquid foods and gradually move to soft foods and finally to solid foods. However, there are some golden rules that should be observed for life:

The nutritional rules our patients should pay attention to are:

• Eating meals very slowly
• Chewing bites very well
• Consuming water during meals
• Avoiding eating while standing
• Eating while sitting upright
• Stopping eating at least 3 hours before bedtime

In addition, it is necessary to stay away as much as possible from foods that may trigger reflux. Although these foods may vary from person to person, there are generally known triggers:

Foods that should be avoided or consumed carefully are:

• Very fatty foods
• Fried foods
• Hot and intense spices
• Chocolate
• Mint and mint products
• Carbonated drinks
• Alcohol
• Caffeinated beverages
• Citrus fruits
• Foods with tomato sauce

Do not let this list scare you; over time you will get to know your body and discover for yourself what is good for you and what is not. The important thing is to accept that eating is no longer a race but a process to be savored.

Does achalasia increase cancer risk?

Although this question worries our patients, it is an issue that must be discussed honestly. Yes, in patients with untreated achalasia, the risk of esophageal cancer is slightly higher than in the general population. The reason for this is the chronic inflammation and irritation created by food accumulating in the esophagus for years. In addition, uncontrolled reflux that may develop after treatment can also affect this risk.

However, there is no need to panic immediately. This increase in risk exists proportionally, but the absolute number is still low. The presence of risk should not frighten us but rather push us to be cautious. For this reason, we surgeons do not cut our ties with our patients no matter how successful the surgery may be.

The things that should be done in long-term follow-up are:

• Annual doctor check-up
• Endoscopy at certain intervals
• Immediate consultation if complaints recur
• Monitoring reflux symptoms

Regular endoscopic check-ups even 10-15 years after surgery make it possible to catch a possible cellular change at a very early stage. Early diagnosis saves lives. Our main philosophy in achalasia treatment is not only to solve the swallowing difficulty of that day, but also to secure our patient’s esophageal health decades later.

Frequently Asked Questions