Achalasia is a disorder caused by the failure of the lower esophageal sphincter, which allows food to pass from the esophagus into the stomach, to relax, and is characterized by difficulty swallowing. The disease is caused by the loss of nerve cells.

What is Achalasia?

Achalasia is a disease that affects the esophagus (food pipe). Due to a disorder in the relaxation of the physiological structure called the lower esophageal sphincter, which is made up of muscles on the stomach side of the esophagus, solid and liquid foods cannot pass easily into the stomach, resulting in difficulty swallowing. The pathology here is the loss of function or absence of nerve cells.

Children with achalasia cannot adequately pass the food they eat from the esophagus to the stomach, causing the esophagus to widen. Since the muscles at the lower end of the esophagus cannot relax, food accumulates in this area. Children with achalasia experience nutritional deficiencies, chest pain, and breathing problems.

 

What causes achalasia?

The cause is not known for certain. However, it is thought that certain parasites and viruses may cause it, or that it may be caused by autoimmunity (when our immune system perceives our own tissues and cells as enemies and attacks them).

Achalasia is a rare disease. One in every 100,000 people has this disease. Achalasia can occur at any age. It can even occur in newborn babies. However, it has been observed that the incidence of the disease increases with age.

Are There Different Types of Achalasia?

Using high-resolution manometry, achalasia is classified into three types.

• Type I: Classic Achalasia
• Type II: Achalasia with Compression
• Type III: Spastic Achalasia. Most cases in children fall under this type.

Determining the type of the disease is important for monitoring and treating the condition (response to treatment).

What are the symptoms of achalasia?

The symptoms of achalasia develop gradually. Therefore, diagnosis may be difficult and delayed. The symptoms of achalasia may include the following complaints:

• Difficulty swallowing; unable to swallow both solids and liquids.
• Food coming back into the mouth
• Chest pain or burning sensation
• Coughing after eating
• Weight loss
• Wheezing
• Belching
• Feeling of fullness in the chest

How is Achalasia Diagnosed?

The diagnosis is made based on medical history and physical examination and confirmed by certain tests.

1. Esophagogram (barium esophagus X-ray): This method is used to visualize the anatomy and movements of the esophagus. The lower end of the esophagus has a bird's beak appearance. However, since the esophagus does not dilate in the early stages of the disease, the diagnosis may be missed. This X-ray may show dilation of the esophagus. In adults, the disease is divided into three stages based on this dilation:

• Stage I (minimal): Esophageal diameter <4 cm
• Stage II (moderate): Esophageal diameter 4-6 cm
• Stage III (advanced): Esophageal diameter >6 cm

 

1. Endoscopy
   It is the direct examination of the valve that opens to the esophagus and stomach with the help of a flexible device with a camera at its tip. Endoscopy findings; dilated esophagus, food residues and esophagitis findings at the lower end of the esophagus, closed lower end of the esophagus, but the endoscope passes easily from here to the stomach without getting stuck.

   

    

2. Manometry
   It is a method that measures the pressure in the esophagus. It helps measure the amount of pressure the esophagus exerts on liquid or solid foods. Manometry is sent to the esophagus and from there to the stomach. This test can show the increase in pressure from the contractions of the relevant muscles. The main findings related to achalasia;

• Lower esophageal pressure increase
• Lower esophageal relaxation inadequacy
• Lack of contraction movements due to contraction in the esophagus

 

How is Achalasia Treated?

Treatment of achalasia disease includes different approaches depending on the patient's age, clinic, and degree of the disease. The disease is likely to recur after dilation (expansion).

1. Medical Treatment; It can be applied in the early stages of the disease. Nitrate group and calcium channel blockers can be preferred. However, the success rate is low and the recurrence rate is high. It can be used in patients who cannot undergo surgery or in whom the disease has recurred.

2. Pneumatic dilatation: A balloon is sent into the esophagus by the physician through an endoscope, passed through the valve between the esophagus and stomach, and then inflated.

 

Endoscopic dilatation is performed with balloons of 30-40 mm diameter at the lower end of the esophagus. The success rate is approximately 70%. In case of failure, the procedure can be repeated. Surgery is performed in patients in whom dilatation is unsuccessful.

Are There Complications of Balloon Dilatation?

Yes, there are. Main complications;

• Esophageal perforation
• Gastro Esophageal Reflux; This complication is more common in surgical procedures.
• Failure of the procedure

Injection: Botox injection is performed endoscopically to prevent the contraction of the muscles at the lower end of the esophagus. This method is preferred in patients with early stages of the disease and recurrence after surgery, and the probability of recurrence is high. The effect of the injection lasts for 6-12 months and then recurs.

The success rate of injection and dilatation is lower than surgery. In addition, inflammation and fibrosis at the lower end of the esophagus make the surgical procedure to be performed later difficult.

 

Surgery (Heller Myotomy); Surgery performed to widen and relax the valve between the stomach and esophagus is called myotomy. In myotomy, some muscles of this valve are cut. This type of surgical procedure usually provides long-term relief from achalasia symptoms. The success rate is high. The surgery can be performed open or closed (laparoscopic).

In children, 1-2 myotomies are performed on the distal esophageal and stomach entrance, extending up to 2.5-5 cm above the esophagus, and an anti-reflux surgery called funduplication is also performed to prevent GER.

 

Can Heller Myotomy Be Performed Laparoscopically?

Yes, it can be done. However, when performed by inexperienced individuals, the surgery time may be longer and mortality and morbidity may be high.

Does Laparoscopic (Closed) Surgery Have Advantages Over Open Surgery?

Yes, there are, the main advantages are listed below;

• Less pain
• Less bleeding
• Less infection
• Less surgical scars or even no scars
• Shorter hospital stay
• Faster and easier recovery
• Quicker return to daily activities

How is Laparoscopic (Closed) Heller Myotomy Surgery Performed?

Carbon dioxide (CO2) gas is injected into the abdomen through the tiny holes opened in the abdomen to create a large area in the abdomen and the abdomen is inflated. New tubes are placed by making 3 0.3-1 cm wide incisions in the appropriate places on the skin, one of which is from the belly button. The planned surgery is performed by placing “long surgical instruments” through these newly added tubes.

However, it should not be forgotten that Laparoscopy can cause more serious complications when performed by inactive people.

What is Done Before Heller Myotomy Surgery?

First of all, a good history should be taken and a general physical examination should be performed. Then, the family (mother and father) should be well informed about the process, that is, before the surgery, during the surgery and after. If the child is older, the child is also included in this. A number of tests are performed, these tests are not general and are determined according to the patient. The patient is also seen by the anesthesiologist, the family and the child are informed about the anesthesia process that will be experienced. The patient is fasted for 4 hours before the surgery.

 

What are the Complications of Heller Myotomy?

• Esophagus and stomach perforation
• Bleeding
• Gastroesophageal Reflux
• Recurrence of the disease
• Infection are the most common complications.

Is Anti-Reflu Surgery Necessary Along with Open or Laparoscopic Myotomy Surgery?

Yes, it is necessary. Because Gastroesophageal reflux is seen at a high rate after surgery in these patients. However, the right anti-reflux surgery should be chosen. Parial Funduplications and methods that do not close the myotomy area have a high success rate and a low complication rate.

 

What are the Effective Methods in Achalasia Treatment?

The 2 most effective treatment methods are balloon dilatation and surgery (laparoscopic Heller myotomy and partial funduplication). The highest success rates are achieved in surgical treatment. With surgical treatment, 85-100% of swallowing difficulties completely disappear.

What Happens If Achalasia Is Not Treated?

• The patient's inability to gain weight and lose weight
• Malnutrition due to inadequate nutrition
• Fatigue and reluctance to perform daily activities due to inadequate energy intake, weakness and fatigue
• Complications due to inadequate intake of vitamins and minerals
• Frequent lung infection due to vomiting

Does Cancer Develop If Achalasia Is Not Treated?

In long-term (adult) follow-ups, it has been shown that the lower end of the esophagus cancer is higher than in normal individuals.

What is POEM (Per-Oral Endoscopic Myotomy) and How is it Performed?

It is the non-surgical cutting of the lower end of the esophagus and stomach muscles by entering through the mouth endoscopically. It is a method that has been put into practice in recent years.

The initial results are successful, but the long-term results are unknown. Since antireflux is not applied, the probability of reflux is high. In addition, the knowledge and experience in children is insufficient.

 

*** The information provided here and the content of the website are designed to inform visitors. No information should be considered as advice by visitors and should not lead to any decision or action. Families should definitely have their patients examined by a pediatric surgeon, consult with him/her and make a decision based on his/her personal knowledge.