Esophageal atresia

The frequency of esophageal atresia is 1: 1000-5000 newborns ^[2] . Isolated congenital tracheoesophageal fistula occurs in 3-4% of all esophageal abnormalities. The combination of male and female sex is 1: 1. Often, atresia of the esophagus is combined with other malformations - congenital malformations of the heart , gastrointestinal tract , genitourinary system and others. In 5% of cases, esophageal atresia occurs in chromosomal diseases ^[3] .

The development of the defect is associated with disorders in the early stages of embryogenesis . It is known that the trachea and esophagus arise from one rudiment - the head end of the anterior intestine. In the earliest stages, the trachea is widely associated with the esophagus. Their separation occurs at 4-5 weeks of embryogenesis. If the direction and growth rate of the trachea and esophagus, as well as the vacuolization processes in the solid stage, which the esophagus passes along with other formations of the intestinal tube in a period of 20 to 40 days, do not coincide, esophageal atresia may develop. Of the history of pregnancy, polyhydramnios and the risk of miscarriage in the first trimester are typical.

This defect is one of the most severe, incompatible with life without early surgical intervention. Like most congenital malformations of internal organs, atresia is often combined with congenital malformations of other organs and systems.

V. A. Klimansky, V. D. Tikhomirova et al. Indicate a significant percentage of detection (up to 80-90%) of the tracheoesophageal fistula associated with this defect. RJ Bereton, H. Linda describe the combination of esophageal atresia with choanal atresia, pyloric stenosis of the stomach, biliary agenesis, congenital heart defects, and jejunum atresia. In 4-6.9% of children with esophageal atresia, signs of micrognathia , hypertelorism , microcephaly , hydrocephalus , hypoplasia of the facial part of the skull that reflect chromosomal abnormalities (trisomy chromosomes 21, 18, 13-15) were revealed.

Based on the anatomical options, six types of esophageal atresia are distinguished:

• complete absence of the esophagus; instead, there is a connective tissue cord;
• the esophagus forms two isolated blind bags;
• the upper segment of the esophagus ends blindly, the lower is connected by a fistulous passage with the trachea above its bifurcation.

Anatomical forms of atresia of the esophagus occur both without communication with the trachea (complete absence of clearance, aplasia of the esophagus), and with a tracheoesophageal fistula. The sizes of the fistulous course are various. In the second variant of atresia, the upper segment of the esophagus is located at the level of the II – III thoracic vertebra, and the lower segment is connected by a fistulous passage to the posterior or lateral wall of the trachea or bronchus. Both segments are from each other at a distance of several millimeters to several centimeters. The level of communication with the respiratory tube is different: above the I thoracic vertebra, at the level of II – III thoracic vertebrae, below the III thoracic vertebra (the latter is the most common). Double fistula cases are known. As a rule, the diameter of the upper segment of the esophagus is larger than the lower. The blind ends of the segments can be in contact with each other or go one after another ^[3] .

Signs of atresia of the esophagus are already visible in the first minutes and hours of a newborn’s life.

A characteristic feature is the continuous abundant secretion of foamy saliva from the mouth and nose . When aspirating saliva into the lumen of the trachea , symptoms of respiratory distress are noted: wheezing, cough , tachypnea , shortness of breath , asphyxiation . When mucus is sucked out of the nasopharynx and trachea, a temporary improvement occurs, but soon the symptoms recur.

During the first feeding, the newborn chokes, while cyanosis appears, and then vomiting with unchanged milk. In the presence of a tracheoesophageal fistula , gastric contents are refluxed into the trachea and distress syndrome occurs, in addition, bloating is observed due to air from the trachea into the stomach . ^[2]

If you suspect atresia of the esophagus, the first diagnostic tests are carried out in the delivery room:

• Elephant’s test - through a probe installed in the blind end of the esophagus, air is supplied with a syringe, it comes out of the nose with noise;
• the introduction through the nose into the esophagus of a radiopaque probe with an atraumatic end; with atresia of the esophagus, the catheter meets an obstacle at the level of the blind end of the esophagus and, wrapping up, appears in the mouth of the newborn.

Instrumental diagnostics

X-ray examination of the chest and abdominal organs:

• no contrast agent. In the presence of atresia of the esophagus, a convoluted radiopaque probe is detected;
• with a contrast agent.

If a tracheoesophageal fistula is suspected, tracheobronchoscopy is recommended. To exclude concomitant anomalies, an ultrasound of the urinary system , neurosonography, ECHO-KG is necessary ^[2] .

The treatment of atresia of the esophagus is exclusively operational, can be performed in two ways:

• open surgery - thoracotomy ;
• thoracoscopic one-stage surgery (performed if there is technical feasibility, preparedness of staff and the absence of indications for staged treatment) ^[2] .

With isolated atresia of the esophagus, the survival rate is 90-100%, with severe combined anomalies 30-50%. With timely diagnosis and treatment, the prognosis is favorable ^[2] .

• Atresia // Brockhaus and Efron Encyclopedic Dictionary : in 86 volumes (82 volumes and 4 additional). - SPb. , 1890-1907.
• Esophageal malformations, instructional video
• Modern views on esophageal atresia