Atresia of the esophagus

Atresia of the esophagus is not a disease, but a developmental disorder of an innate nature, so patients are children almost from the first days of life. The esophagus looks like a short tube with a blind upper end, and the bottom can be connected to the trachea.

Equally common in boys and girls. The prevalence is 3 cases per 10 thousand born, for other authors 1 case for 3-5 thousand newborns.5% is usually combined with the pathology of chromosomes. Atresia of the esophagus in children accompanies other congenital disorders of development( heart, vessels, urogenital organs, intestines and stomach).

In the International Statistical Classification, "atresia of the esophagus without fistula" is taken into account separately under code Q39.0 and "esophageal atresia with tracheal-esophageal fistula" - Q39.1( cases of bronchial-esophageal connection are included here).

Causes and mechanism of development of

The formation of a defect occurs in the early stages of fetal development. In the second month of pregnancy, the trachea and esophagus form from the embryo from the head of the anterior bowel. They communicate with each other. By the fifth week should occur under normal flow separation.

If the speed and direction of growth of these organs do not correspond, then by 20-40 day atresia of the esophagus appears. At the same time, other defects are formed in the fetus in 50-70%:

• defects of the musculoskeletal system( 30%);
• heart defects( up to 37%);
• stenosis of the pyloric part of the stomach, atresia of the jejunum and biliary tract( 20-21%);
• defects of the genito-urinary organs( 10%);
• infection of the nasal cavity due to atresia of the hoan, other defects of the facial part of the skull( 4%).

Up to 7% of children born have chromosomal abnormalities in the form of hydrocephalus, microcephaly due to trisomy in 13, 18 and 21 pairs of chromosomes. An analysis of the course of pregnancy of women showed that the child's vice is more often in cases of polyhydramnios, with the threat of interruption in the first trimester.

Anatomical variants of atresia

Atresia of the esophagus in newborns is possible both without a connection to the trachea, and in the most common form - with tracheoesophageal fistula( 80-90% of cases).

Dimensions of the fistulous stroke can be different

Fistula is located at the level of the last cervical vertebra, upper thoracic vertebrae and below. When the fistulous course is formed, the end of the esophageal tube remains at the level of the thoracic vertebrae( II-III), the lower part is connected along the posterior or lateral surface with the trachea( bronchus).

The lowest variant is the most common. Usually the upper segment of the esophagus is wider and more than the lower one. Before the mouth, there is not enough length of the esophageal tube in 8-12 cm.

The blind end in the histology of the tissue shows hypertrophy, and in the other, the thinning of the wall is observed. Tracheal-esophageal fistula is an unusual canal lined from the inside with epithelium or granulation tissue. Possible the formation of a double fistulous course.

Classification by type

Of all types of atresia of different parts of the digestive system, esophageal flora occurs most often. Depending on the anatomical shape and location, it is common to distinguish six types of pathology:

• esophagus is completely absent, it is replaced by a cord from connective tissue;
• from one esophagus tube are formed 2 separate blind bags;
• , the upper part of the tube terminates blindly, the lower part is connected by a course with the trachea at a level above the bifurcation;
• is the same, but the lower esophageal fistula is located in the bifurcation zone;
• fistula is located in the upper end of the esophagus, the lower one is blind;
• both segments( both upper and lower) are connected to the trachea.

Another classification has a modified interpretation and provides for 5 types that occur at different frequencies:

• 85% of cases occur in the variant with the blind upper end and tracheoesophageal fistula from below;
• 8% - "pure" atresia without fistula formation;
• 4% - "type in the form of H" - with a passable esophagus;
• 1% - atresia with closed upper end and fistulous course at the bottom;
• 1% - atresia with two fistulas.

The course of the pathology of

Symptoms of blemish appear almost immediately after birth, even before the first feeding. They are quite typical, which makes it possible to quickly organize treatment. The newborn has a permanent discharge from the mouth and nose of viscous foamy mucus( a symptom of "false hypersalivation"), attacks of suffocation with cyanosis of the face and body due to aspiration( ingestion of mucus in the trachea).

Improvement occurs temporarily after the removal of mucus, then everything repeats. At the beginning of the first breast-feeding the fluid immediately regurgitates, and in the presence of tracheoesophageal fistula enters the respiratory tract, stimulates coughing, respiratory failure, cyanosis. The reverse movement of the fluid( regurgitation), unlike the true vomiting, appears immediately after two sips.

Respiratory distress causes effects in the body in the form of respiratory acidosis( deviation of the acid-base balance towards acidification), increased red blood cells and hematocrit.

Condition deteriorates rapidly. Complications of respiratory failure are:

• aspiration pneumonia;
• depletion;
• dehydration.

With atresia, the doctor can not hold a catheter further than 10-12 cm from the gums because of abutment in the blind end.

. A child's examination reveals:

• is a deviation or bulging in the upper half of the abdomen;
• rapid breathing with a disturbed rhythm;
• temporary, and then complete cyanosis of the body;
• wet wheezing in the lungs.

These disorders increase when trying to feed. Marked departure of meconium with a stool after birth in the first day, then defecation does not occur.

Diagnostics

Experienced obstetricians and neonatology doctors know that when the first symptoms appear, it is required to immediately check the permeability of the esophagus by inserting it through the nose of the catheter.

If there is a fistula, it is impossible to get into the stomach. Probing of the esophagus is recommended to do to all newborns with a foamy discharge from the mouth, as well as with respiratory disorders immediately after birth.

It is recommended that a sample be taken by the Elephant: a small amount of air is inserted into the catheter inserted into the esophagus until it stops, using a syringe. With the available atresia, he immediately with a noise comes out of the nasopharynx and the mouth of the patient.

For final diagnosis, an X-ray examination with the introduction of contrast agent Jodolipol in the esophagus should not exceed 2 ml. To prevent aspiration, the child is kept in an upright position.

After completion of the study, Iodolipol needs to be sucked. The barium mixture is not used precisely because of the density and difficulty of removal. The picture shows the filling of the esophagus by contrast medium, its penetration through the tracheal fistula into the lung tissue, the blind end of the upper segment.

If there is tracheoesophageal communication in the lower part, the stomach and intestines are full of air. Absence of air filling is a sign of a nonspecific type of esophagus atresia.

Chest examinations and images in the direct and lateral projection

Is it possible to diagnose a baby in the womb?

Diagnosis of pathology in the fetus is called prenatal. It can be based on the following indirect signs, which are detected by ultrasound:

• polyhydramnios - with a significant increase in the weight of the pregnant, in this case it is associated with a reduced turnover of amniotic fluid due to the inability of the fetus to swallow the amniotic fluid;
• no image of the contours of the stomach or its too small dimensions in the dynamics under observation.

The probability of confirming the pathology by these features reaches 50%.In the II and III trimesters, an experienced physician observes periods of filling and emptying the blind end of the esophagus. There are reports of the importance of determining the content of the enzyme acetylcholinesterase in the amniotic fluid of the fetus, if during pregnancy there is a suspicion of congenital pathology.

Treatment

The operation is necessary for a child for life reasons, therefore, training begins immediately after birth in the department of newborns, without waiting for transfer to a specialized surgical clinic. The baby is placed in a kuvez with an elevated position of the upper parts of the trunk, where it can be warmed, oxygen is constantly supplied for breathing.

The child needs to ensure a lying position on the abdomen with a raised head at an angle of 30 degrees and at the right half of the body. Similarly, it is possible to improve gastric emptying, to reduce the possibility of aspiration of acid secretion through the fistulous course.

Babe is under constant supervision of specialists

Every quarter of an hour, the contents of the mouth and nose are sucked. Feeding through the mouth is prohibited. The child is injected with liquids, nutrient mixtures, antibacterial agents with a broad spectrum of action, based on weight.

Increasing respiratory failure is an indication for transferring a patient to an artificial way of ventilation. The trachea is intubated and the ventilator is connected. In this case, it is necessary to take into account the possibility of an intensive discharge of air through the fistula into the stomach.

Then there is a rerun of the stomach and intestines, limiting the movement of the diaphragm. As a result, respiratory failure is even more progressive, there is the possibility of stomach rupture and cardiac arrest. Resuscitators under such conditions try to deploy or deepen the intubation tube. These measures help to reduce the discharge of air through the fistula.

Operational risk is lower in term infants, without a combination of atresia of the esophagus with other vices that have not undergone birth trauma. The procedure of the operation is selected depending on the type of atresia, the height of the location and the presence of the fistulous course.

The essence of the intervention in the first stage: the patient's chest is opened, the fistula is bandaged between the trachea and esophagus, both ends of the esophagus are connected by an anastomosis. If it is impossible to connect the ends of the esophagus, put the stoma on the neck( exit from the esophagus) and in the upper part of the abdomen( from the stomach).In patients without fistula, the operation is less severe.

At high operational risk( children premature, less than 2 kg in weight, with other defects), a double gastrostomy is established in the first stage: one for feeding after surgery with a probe in the duodenum, the second with the stomach to reduce compression and aspiration. The next stage of the operation( separation of the trachea and esophagus) is performed after 2-4 days.

Children with congenital atresia of the esophagus live with postoperative esophagus and gastrostomy, which can prevent severe complications, and carry out feeding. The final stage - the plasty of the esophagus - is performed depending on the condition of the baby between the ages of three months and three years. The material for plastics is your own transplant from the colon.

How is the postoperative period?

After the operation, the child is in the intensive care unit. Intensive care continues. A solution of glucose, electrolytes is injected into the vein. Antibiotics of a wide spectrum of action, Metronidazole are applied.

Feeding is carried out through a probe or gastrostomy while restoring the intestinal peristalsis

Doctors try to transfer the baby to their own breathing as soon as possible, since a prolonged intubation tube in the trachea can cause tracheomalacia and edema, then urgent tracheostomy is required.

In the first 7 days after the operation, the neck of the child can not be unbent. This movement stretches the esophageal anastomosis and can cause divergence of the sutures. On the 6-7th day, it is necessary to check the anastomosis with an X-ray method with the introduction of Jodlipol. The permeability of the esophageal tube is checked, there is no leakage.

If the plastic is not planned, the baby can be fed through the mouth. Food starts with special mixtures( Human AR, Frisov, Antireflux, Nutrizone), prokinetics preparations( Domperidone) are added to ensure peristalsis of the stomach and intestines.

During 2-3 weeks, esophagogastroscopy is performed, the zone of anastomosis, the nearest parts of the stomach is visually assessed. When a narrowing occurs( occurs in 30-40% of patients), bougie is added to the treatment. Dense rubber bougie( size 22-24) inserted into the esophagus to develop a narrowed canal.

Postoperative complications of

In the near future after the operation, one should expect:

• failure of anastomotic sutures;
• gastroesophageal reflux;
• pneumonia;
• mediastenitis;
• laryngotracherapy;
• bleeding and anemia.

Within a year after the operation, there may be:

• swallowing disorders( anastomosis obstruction);
• hoarseness of the voice( trauma of the recurrent nerve);
• night regurgitation due to gastroesophageal reflux;
• repeated pneumonia.

Gastrostomy allows the baby to be fed until the esophagus is completely restored

Forecast

If the operation is performed for isolated esophageal atresia without other vices, then a complete cure comes in almost 100% of cases. When combined with other vices and chromosomal abnormalities, the indicator is reduced to 30-50%.The absence of complications creates a favorable prognosis for life and development of the child.

Be sure to observe the patient pediatrician and surgeon. Parents control the regime and nutrition, maintain immunity. Even the performed operation leaves an increased risk of contracting respiratory infections, the development of pneumonia, bronchial asthma.