What is oesophageal (oesophageal) atresia?

Before defining oesophageal atresia, it is necessary to define oesophagus. The oesophagus is an organ located between the pharynx and the stomach and is predominantly composed of muscle structure. It is approximately 20-25 cm in length in adults and provides the transfer of food to the stomach. It also prevents the transfer of food from the stomach back to the oesophagus with the anatomical structures formed by the stomach at the point where it meets the stomach. The oesophagus starts after the structure we call the pharynx, which is the continuation of our mouth, and passes through the neck and chest cavity and merges with the stomach in the abdomen. In the meantime, it is in close neighbourhood with many anatomical structures, especially the trachea.

Atresia means congenital underdevelopment of any organ. Oesophageal atresia means that part of the oesophagus does not develop. We can also call this partial absence of the oesophagus. It is a congenital defect that occurs in the oesophagus of the baby while the baby is in the womb.

In the early stage of life in the womb, the oesophagus and windpipe begin to develop as a single structure that connects the mouth and stomach as a long and continuous tube. In babies with oesophageal atresia, the oesophagus develops as 2 tubes that do not join with each other. The ends of these two tubes are closed and associated with the windpipe. In the later stages, the trachea and oesophagus are completely separated from each other and become a hollow luminal structure. Defects in this developmental stage due to unexplained reasons cause oesophageal atresia.

How does oesophageal atresia occur?

During pregnancy, the baby's oesophagus is formed from the first part of the foregut, the foregut. Between the 4th and 5th week of pregnancy, the tissue called lung bud starts to develop in the baby from the front part of the tube-like tissue. This bud tissue follows a downward development course. In the meantime, a curtain tissue is formed and this curtain separates the foregut into two different parts. One of these two parts forms the breathing channels and lung, while the other part forms the oesophagus. As a result of the curtain tissue reaching the throat, two different entrances, the oesophagus and the trachea, are formed. With the continuation of development in a short time, these two tissues, which take the shape of a tube, are completely separated. During this separation, oesophageal atresia develops with the formation of a deficiency in the oesophagus part of the foregut tissue. Briefly, it can be said that it occurs as a result of the airways not being completely separated from the foregut.

Does oesophageal atresia accompany other congenital organ anomalies?

Along with this disease, other birth defects can be seen in other systems. The rate of anomalies accompanying oesophageal atresia is approximately 30-60%. Cardiovascular anomalies, gastrointestinal anomalies, kidney anomalies and skeletal system anomalies are most common. Therefore, all systems should be evaluated for anomalies in a baby with oesophageal atresia.

How often is oesophageal atresia seen?

Oesophageal atresia is a very rare condition. Approximately every 3000 to 5000 births have oesophageal atresia. It is more common in male babies.

Is oesophageal atresia genetic or familial?

There is no genetic basis for oesophageal atresia. On the other hand, it cannot be called familial. However, if one of the mother or father has oesophageal atresia, the incidence in children is 3.9%, if one of the children has oesophageal atresia, the incidence in others is 0.5-2%, and if there are two children in the family, the risk of having the third is 20%. Esophageal atresia is also more common in twins.

What is the cause of oesophageal atresia?

It is not known for certain. However, it is thought that some hormones, chemicals and vitamin deficiencies and blood supply problems of the organs in the womb may be the cause.

Are there any types of oesophageal atresia?

There are many types of oesophageal atresia, but the most common are 5 different types of oesophageal atresia. The types of this developmental abnormality are made according to the fact that a canal-shaped connection remains when the oesophagus is formed and according to its relationship with the trachea.

What are the symptoms of oesophageal atresia and how is it diagnosed?

The diagnosis is usually made with a good anamnesis (history) and physical examination. Firstly, some findings in ultrasonography performed in the womb suggest oesophageal atresia.

These are

• Increased maternal fluid (polyhydramnios),
• Normanden small and hollow stomach.
• The enlargement of the upper part of the oesophagus suggests that the baby may have oesophageal atresia. These findings may vary depending on the type of atresia.

The first clinical finding after birth is that if there is prenatal (in the womb) suspicion, NGT (nasogastric probe) cannot be inserted and the probe is inserted around 10 cm and does not progress.

Also;

1. Foaming in the mouth and excess saliva secretio

The first sign of oesophageal atresia in infants is the accumulation of excess saliva in the baby's mouth. At the same time, saliva secretion can also be seen as foaming. This is because babies cannot swallow the saliva secreted in their mouths. However, as this secretion continues to be secreted in the mouth, it starts to flow outwards. As babies breathe in and out, foaming occurs in the saliva secretion. Sometimes mothers do not take the baby to the doctor for examination because they do not suspect this condition. If the diagnosis is made later, milk comes back from the mouth and nose of the breastfed baby. This can cause respiratory distress in babies and can be life-threatening. If any of the symptoms are observed, a doctor should be consulted immediately.

2. Cough and Bruising

In the case of oesophageal atresia, usually type H, coughing and bruising are more common symptoms. This is because these people have fewer problems with feeding. This is why bruising occurs due to coughing and respiratory failure during coughing. This is because the food taken during feeding escapes into the trachea through a fistula. This causes severe coughing and bruising in infants. As the fluid escapes into the trachea, it causes fluid accumulation in the lungs. However, infection develops in the lungs. In some cases, the fluid escaping into the trachea can first pass into the oesophagus and then into the stomach. When this problem occurs, a swelling appears just above the navel of the babies.

If there is an atresia without fistula, the abdomen is sunken and the abdomen appears gasless (ie white) on the direct X-ray film.

What are the necessary tests to determine the type of oesophageal atresia before surgery?

• Some Blood tests
• Lung and Standing Direct Abdominal Graphy
• Abdominal Ultrasound (Additional anomaly investigated)
• Echocardiography (Heart pathologies are investigated)
• Contrast radiography
• Endoscopy and Bronchoscopy (for fistula investigation)

What is the treatment of oesophageal atresia?

The first treatment is to prevent the baby's saliva and formula from escaping into the lungs. Fluid therapy and antibiotic treatment are then started. The most basic principle in the treatment of oesophageal atresia is to eliminate the missing part of the oesophagus and thus to ensure the continuity of the oesophagus. Depending on which type of oesophageal atresia develops, the treatment may also vary. If there is a problem caused by the ends of the oesophagus and trachea being too close to each other, these ends can be separated from each other with a simple intervention. Surgery may also be preferred in the treatment of oesophageal atresia. Especially in the most common type, surgical intervention may be mandatory. With the surgery, the tissues are separated by applying an incision to the adhered part of the trachea. At the same time, the opening in the trachea caused by this incision is repaired. The suturing method ensures continuity in the trachea and oesophagus.

However, this may sometimes not be possible and then alternative treatments are applied. Sometimes the upper end of the oesophagus is opened out of the neck to prevent saliva from escaping into the lungs. If there is a fistula, it is connected. The stomach is nourished through the anterior abdominal wall. After reaching a certain age, permanent operations are performed.

Is Thoroscopic (Closed) Oesophageal Atresia Surgery Performed?

Yes, it can be done.

How is oesophageal atresia performed thoroscopically?

In closed surgery methods, a large incision between the ribs is not made as in open surgery. It does not have to be opened by placing a retractor. Instruments with a diameter of 5 - 10 mm are inserted into the thoracic wall (chest wall) through 2 or 3 holes and surgeries are performed through these holes. Closed surgeries provide a wider viewing angle than open surgeries. 

What is the treatment of oesophageal atresia?

Yes, there are many advantages such as less pain, shorter recovery time and more aesthetic. The thorocoscopic method provides a wider viewing angle than open surgery

It should not be forgotten that thoroscopic (closed) surgeries require serious experience, knowledge and skill along with advanced technology. Otherwise, it should not be forgotten that serious complications may be caused.

What happens after oesophageal atresia surgery

Immediately after the operation, the baby should stay in intensive care. During the operation, a chest tube is inserted into the baby's stomach with a probe. This tube should remain fixed and should not be removed for a while. The baby is usually fed with a chest tube for 3 to 4 days. Afterwards, breast milk or formula is given and the baby is fed naturally. After 5 to 7 days following the operation, this probe placed in the stomach is removed. However, before it is removed, a medication is administered, a film is taken and it is checked whether the appearance targeted by the surgery has been achieved.

After the surgery, a number of temporary and permanent complications, both early and late, may be encountered.

Early Complications;

• Lung problems, sometimes it may be necessary to connect to the device.
• Anastomase leakage - as it will close spontaneously, 2nd - 3rd operations may be required.

Late Complications;

• The most common problems are stenosis in the anastomosis area
• Gstroesophageal reflux.
• In addition, temporary and permanent hoarseness, i.e.
• wheezing can be seen.

When stenosis develops, dilatation (expansion) is performed at certain intervals, and for reflux, primarily medication and positioning treatment is applied, but 2nd surgery may be required for this.

Are there risk factors that determine the prognosis of a baby with oesophageal atresia?

Yes, there are. How many months and how much weight the baby was born, whether the baby has lung problems, the presence of additional anomalies and the weight, if any, are the main factors that determine the prognosis.

What is done if the ends of the oesophagus cannot be brought together?

In such cases, some methods to lengthen the oesophagus can be tried. If these methods are not successful, as mentioned above, oesophagostomy (externalisation of the oesophagus to the neck) and gastrostomy (temporary feeding of the baby is provided by a tube inserted into the stomach through the abdominal wall). After the patient reaches a certain age, which is usually around 2 years of age after starting to walk, the oesophagus is made from the large intestine.

 

 

Schematic oesophagostomy and gastrostomy.

 

 

What are the complications (problems) of making an oesophagus from the large intestine (colon)?

First of all, you should know that the best organ is the original organ of the person. Although the colon is the patient's own organ, since it is not the original oesophagus, some early and late problems may be encountered after surgery.

Early Problems;

• Lung infection,
• Anastomotic leakage - some of these will pass on their own, some of them require a second operation -,
• Very rarely complete decay of the large intestine.
• Anastomotic stenosis

Late problems;

• Anostomotic stenosis,
• Breathing difficulties and nutritional problems due to excessive expansion of the clone in the chest - Reflux - which may require a second operation.

*** The information given here, the content of the website has been organised for the purpose of informing the visitor, especially the families. No information should be considered as advice by visitors and should not lead to any decision or action. Families should definitely have their patient examined by a paediatric surgery specialist, consult with him/her and make a decision by consulting his/her knowledge.