What Is Esophageal Atresia?

Esophageal atresia is a congenital malformation of the esophagus, with an incidence rate of about 1/3000 to 1/4000. The reason is that the embryo develops from the 3rd to the 6th week and develops abnormally. Bronchial communication forms an esophageal-bronchial fistula. Symptoms include immediate vomiting or severe coughing, cyanosis, or suffocation. The diagnosis can be confirmed by placing a catheter through the esophagus and injecting X-ray examination with lipiodol. The principle of treatment is to first save the patient's life, then rebuild the continuity of the digestive tract, and use the esophagus as much as possible.

Esophageal atresia is a congenital malformation of the esophagus, with an incidence rate of about 1/3000 to 1/4000. The reason is that the embryo develops from the 3rd to the 6th week and develops abnormally. Bronchial communication forms an esophageal-bronchial fistula. Symptoms include immediate vomiting or severe coughing, cyanosis, or suffocation. The diagnosis can be confirmed by placing a catheter through the esophagus and injecting X-ray examination with lipiodol. The principle of treatment is to first save the patient's life, then rebuild the continuity of the digestive tract, and use the esophagus as much as possible.

Chinese name

Esophageal atresia

Foreign name

atresia of esophagus, esophageal atresia

Esophageal atresia I. Etiology and related diseases

The etiology of esophageal atresia may be related to genetic factors, inflammation, or vascular dysplasia, but the specific cause is unknown.

1.Genetic inheritance

Genetics have not been fully confirmed, but some clinical data suggest that the offspring of children with esophageal atresia may have the same deformity. There have been many clinical reports on chromosomal abnormalities found in esophageal atresia cases. This may be the esophageal atresia gene, and its change regions are: 3pter p21, 4pter p15, 4q31 qter, 5q31 qter, 6q13 q15 , 14q32 qter, 18p, and 18q (p is the short arm of the chromosome, q is the long arm, and ter is the terminal end). The incidence of twins in esophageal atresia is about 6%, while the incidence of twins in the general population is about 1%. About 6.6% of children with esophageal atresia have chromosomal abnormalities, mainly including trisomy 18 syndrome.

Environmental teratogenic factors

Environmental teratogenic factors may be related to their occurrence, and related to the mother's long-term use of contraceptives or estrogen or androgens during pregnancy. It has also been reported that esophageal atresia can occur in children with diabetic mothers or in children who have stopped responding (thalidomide).

3. Cell- to-cell material migration

There may also be problems with cell-to-cell material migration in esophageal atresia. At the molecular level, the shh gene can affect the vertical development of vertebrate organs and play an essential role in the development and differentiation of the gut.

The pathological classification commonly used in clinical esophageal atresia is the Gross5 type classification method. Type I: the upper and lower ends of the esophagus are closed, and there is no fistula between the esophagus and trachea, accounting for about 6%; type II: the lower end of the fistula is formed between the upper end of the esophagus and the trachea. Atresia, about 2%; Type III (clinical most common): The upper end of the esophagus is closed, and the lower end communicates with the trachea to form a fistula, which accounts for about 85%. For the distance between the ends of the esophagus, the distance between the blind ends is greater than 2cm, and the distance is less than 2cm, which is b. Type IV: The upper and lower ends of the esophagus communicate with the trachea to form a fistula, accounting for about 1%; Type V: The esophagus is not blocked, only the trachea and esophageal fistula form an H-type fistula, accounting for about 6%.

Esophageal atresia 2. Differential diagnosis

1.Prenatal diagnosis

(1) Too much amniotic fluid

Children with esophageal atresia cannot swallow amniotic fluid, so the mother often has a history of excessive amniotic fluid. It has been reported that the fetus was found to have oligohydramnios in the mother before antenatal examination 30 weeks ago, and the central nervous system deformity was more common; and after 30 weeks, too much amniotic fluid was found to be esophageal atresia, etc.

Gastrointestinal malformations are more common. Polyhydramnios is associated with congenital atresia of the esophagus and the type of esophageal and tracheal fistula.

(2) Upper Neck Blind Pouch

Although the increase of amniotic fluid and the disappearance of the small stomach (or gastric vesicles) are important basis for finding esophageal atresia, its positive diagnostic value is not high. A more reliable basis for prenatal B-ultrasound diagnosis of esophageal atresia is "upper neck blindfold". It can be seen in the B-ultrasound that with the fetal swallowing activity, there is a cystic blind bag in the esophagus area, which appears to be "filled" or "emptied."

(3) Magnetic resonance imaging

In T2 weighted MRI of children with congenital esophageal atresia, the proximal esophagus is dilated and the distal esophagus disappears. Studies have shown the sensitivity and specificity of MRI in diagnosing esophageal atresia, and its sensitivity and specificity are considered to reach 100% and 80%. The sensitivity of ultrasound for congenital atresia is 24% to 30%. Some children with esophageal atresia do not necessarily show too much amniotic fluid. Therefore, prenatal B-ultrasound is easily missed; and for fetuses who have not found blind bag syndrome on B-ultrasound, MRI can also diagnose esophageal atresia. At the same time, magnetic resonance imaging is non-invasive and safe, and generally does not affect fetal development and organ function. Therefore, the use of MRI in suspected esophageal atresia or high-risk fetuses is advocated to improve prenatal diagnosis.

2. Postpartum diagnosis

(1) Clinical manifestations

Patients often show excessive saliva from 1 to 2 days after birth. Foamy fluid overflows from the mouth and nasal cavity, and cough, shortness of breath, and temporary cyanosis sometimes occur. The typical symptoms appear after the first feeding, and the baby starts coughing after sucking a mouthful or two of breast milk. The breast milk then overflows from the nostrils or mouth, and it becomes difficult to breathe and looks cyanotic. This is because the food quickly fills the blind bag near the esophagus. Afterwards, the results returned to the trachea and bronchi.

(2) Stomach tube failure

A small catheter was inserted from the nostril or oral cavity, and it was obstructed and turned back. It should be noted that the catheter curled in the blind bag of the esophagus and caused the illusion of entering the stomach.

(3) B-ultrasound

"Upper Neck Blind Pouch" is displayed. It is non-invasive and rapid.

(4) Esophageal angiography

A 25% water-soluble iodine or 0.5 to 1 ml of air is dripped through the catheter, and the blind end of the esophagus can be found on the chest radiograph. In recent years, some scholars oppose the use of contrast agents, because 80% of patients with pneumonia after using contrast agents, and only 44% of those who do not use contrast agents with pneumonia. Therefore, when suspicious V-esophageal atresia is suspected, esophageal angiography should be used cautiously. Meilan can be injected through the trachea for fiber esophagoscopy. If blue is found in the esophagus, it is helpful for diagnosis.

(5) CT examination

CT can provide sagittal, coronal, and three-dimensional reconstructed images to help find esophageal atresia and associated fistulas. Three-dimensional CT can accurately provide information such as the location of the fistula and the distance of the blind end.

(6) Bronchoscope and virtual bronchoscope

Bronchoscopy is often used to find the location of the fistula. For traumatic procedures, general anesthesia is required, which often brings complications such as hypoxia, laryngospasm, pneumothorax, airway edema, and bleeding. It is not recommended for use in newborns. Virtual bronchoscope, which uses 3D CT to reconstruct trachea, carina and main bronchus. Doctors who are familiar with bronchoscopy can find that reading virtual pictures is consistent with the speculum, and that virtual bronchoscopy can see the narrow distal end beyond the narrow part of the operation, and can accurately locate from the picture. For patients with fistula recurrence after esophageal atresia Children are especially suitable.

Esophageal atresia III. Principles of treatment

1. Surgical treatment

(1) surgical methods

According to Gross classification, there are differences in the surgical methods of congenital esophageal atresia. The most common type is type III esophageal atresia with esophageal and tracheal fistula. The fistula is close to the tracheal prosthesis. Surgery is the most important treatment. Generally speaking, one-stage esophageal anastomosis is not suitable for a distance of more than 3 cm at the stump, and type and esophageal atresia is often more than 3.5 cm away from the blind end of the proximal and distal esophageal atresia. Delayed esophageal anastomosis can be considered for esophageal reconstruction Or esophageal replacement. The operation can be performed in children 8 to 12 weeks. At this time, the body weight of the child is doubled, and the distance between the two blind ends is often less than 2 cm. End-to-end anastomosis is used; if the distance between the proximal and distal ends of the esophagus is greater than 2 vertebrae (That is, long-section esophageal atresia). Generally, staged surgery is used. The first time the esophageal stoma, tracheal fistula repair and abdominal gastrostomy are performed on the neck. After 6 months, esophageal or colon esophageal surgery will be performed.

(2) Minimally invasive surgery

Thoracoscopic neonatal type IIIb esophageal atresia fistula ligation esophageal anastomosis is a desirable surgical approach, which has a small impact on the child during the operation, light chest wall damage, rapid postoperative recovery, minimally invasive effects, and whether satisfactory exposure can be obtained during the operation. The distance between the two ends of the esophagus is an important factor affecting the operation. Minimally invasive surgery is smaller and more beautiful than thoracotomy, but postoperative esophageal stricture and anastomotic fistula may still occur.