I. Definition
Congenital esophageal atresia is one of the serious congenital anomalies in newborns due to abnormal development of the esophagus and trachea during embryonic period without forming a complete esophagus, and is often combined with thoracic and pulmonary anomalies and various other malformations.
Etiology
The etiology is unknown, but may be related to the common origin of the esophagus and trachea. Both the esophagus and trachea occur from the primitive foregut in the early embryonic stage. At 3 weeks of embryonic life, two longitudinal ridges appear in the primitive foregut from each side of the wall. By 5-6 weeks of embryonic life, the longitudinal sulcus deepens and the longitudinal ridges become closer together, finally fusing into a septum that divides the foregut into two ducts, the ventral side forming the trachea and the dorsal side forming the esophagus. The esophagus undergoes a stage of parenchymal transformation in which the epithelial cells within the tube multiply and proliferate, causing the esophagus to become occluded, and later the vacuoles within the tube appear and fuse with each other, re-crossing the esophagus and forming a hollow tube. During the 8 weeks of embryonic life, if the separation or vacuolation is incomplete, it can cause different types of malformations. It is thought to be related to vascular anomalies, and the reduced blood supply to the foregut can lead to atresia.
III. Diagnosis
1. Clinical manifestations
(1) Pendant fluid cannot be swallowed, salivation and white foam after birth, and saliva often overflows around the lips.
(2) Immediately after the first feeding, spitting up, violent choking and coughing may occur, and even asphyxia.
(3) Due to the inability of food to enter the gastrointestinal tract, it presents water loss and wasting.
(4) The catheter placed through the mouth or nasal cavity is blocked at the blind end of the upper esophagus and cannot be passed into the stomach.
2.Auxiliary examination
(1) X-ray plain film: a frontal lateral film of the chest can be seen with an inflated blind pouch of the esophagus in the flat T4 of the posterior superior mediastinum. However, the abdomen should be included.
(2) Transnasal insertion of the catheter to the subpharynx is obstructed or returned. Length is approximately 8-10 cm. A silicone tube that is impervious to X-rays is seen to return from the blind end of the esophagus on X-rays. (T4)
(3) Esophagogram technique: inject 0.5 ml of aqueous contrast agent (barium sulfate is contraindicated) through a nasal cannula, then follow up with 1 ml of gas and take frontal and lateral chest films.
(4) X-ray and ultrasound can basically meet the requirements for examination of cardiac and vascular, urological malformations, and spinal malformations.
IV. Differential diagnosis
Congenital esophageal stricture can be single or multiple strictures. In severe strictures, there can be dysphagia, vomiting or choking after swallowing soon after birth, but in mild cases, there can be no symptoms. Gastric tube can be placed experimentally with resistance or difficulty in placement, and esophagogram with a small amount of iodine oil injected through the tube can show the stenotic segment with mild dilatation of the esophagus above it. Esophagoscopy can show the stenotic segment. In mild cases, esophageal dilatation is feasible, while in severe cases, the stenotic segment needs to be removed and esophagoplasty performed.
Cardia insipidus is caused by recurrent vomiting due to the absence or degeneration of the interosseous ganglion cells in the lower esophagus and cardia, resulting in flaccid insufficiency. It is not uncommon in newborns. The vomiting appears within the first few months of life and gradually worsens, but is generally good and can cause pneumonia and malnutrition in severe cases. Esophagogram shows marked narrowing of the lower esophagus and cardia and extreme dilatation of the upper segment with a funnel shape. It can be treated with postural treatment, and in severe cases, esophageal dilatation surgery is performed.
V. Treatment
1, the disease is untreated death a few days after birth, so after a clear diagnosis should be performed as soon as possible surgery to correct the deformity.
2, preoperative should pay attention to the appropriate amount of rehydration, correct dehydration and electrolyte imbalance. Prevent and treat aspiration pneumonia. Give antibiotic drugs, maintain normal body temperature, etc.
3.Good development, birth weight over 2.5kg, good general condition, no other congenital malformation or moderate pneumonia or above can be performed a stage of esophageal anastomosis.
4, weight less than 2kg, general condition is not good or with other congenital malformations, need to perform the surgery in stages. The first stage of surgery is to sever and suture the esophagotracheal fistula. A gastrostomy is performed through the abdomen to supply nutrition. A cervical esophagostomy is performed via the neck to prevent aspiration pneumonia. After a few weeks, the second stage of surgery will be performed when the body weight has increased to about 3 kg, and the upper and lower esophagus will be anastomosed. This type of case also requires staged surgery. In the first stage, the proximal esophagus is drained through a cervical incision and the blind end of the proximal esophagus is incised to drain the pendant fluid. A gastrostomy is also performed through an abdominal incision to provide food and drink. A colonic pouch esophagus will be performed when the child reaches 3-4 years of age.
VI. Grading and treatment guidelines
Classification.
Type I. The upper and lower ends of the esophagus are not connected, and each end is blind, and the distance between the two ends without fistula is far, accounting for 4%-8%.
Type II The proximal esophagus is connected with the trachea by a fistula, and the distal end is blinded and distant, accounting for 0.5%-1.0%.
Type III The upper part of the esophagus is blind and the lower part is connected to the trachea, and the distance between the two ends is less than 2 cm for type IIIb and more than 2 cm for type IIIa, accounting for 85%-90%.
Type IV The upper and lower segments of the esophagus are connected to the trachea respectively, accounting for 1%.
Type V: No esophageal atresia, but fistula with trachea, accounting for 2-5%.
Guidelines for diagnosis and treatment.
In cases with weight less than 2 kg, poor general condition or other congenital malformations, surgery is required in stages. In the first stage, the esophagotracheal fistula is cut and sutured. A transabdominal gastrostomy is performed to provide nutrition. A cervical esophagostomy is performed via the neck to prevent aspiration pneumonia. After a few weeks, the second stage of surgery will be performed when the body weight has increased to about 3 kg, and the upper and lower esophagus will be anastomosed. This type of case also requires staged surgery. In the first stage, the proximal esophagus is drained through a cervical incision and the blind end of the proximal esophagus is incised to drain the pendant fluid. A gastrostomy is also performed through an abdominal incision to provide food and drink. A colonic pouch esophagus will be performed when the child reaches 3-4 years of age.
VII. Admission criteria
Once diagnosed, pendant fluid cannot be swallowed, salivation and white foam after birth, saliva often spills around the lips; immediately after the first feeding, vomiting, violent choking and coughing, or even asphyxiation may occur; the catheter placed through the mouth or nasal cavity is obstructed at the blind end of the upper esophagus and cannot be passed into the stomach; then the child can be admitted.
VIII. Critical value report
None
IX. Guidelines for admission to ICU
1. shortness of breath, dyspnea; 2. violent choking and coughing, cyanosis.
X. Consultation criteria
1. inability to swallow, salivation and white foam after birth, frequent saliva spillage around the lips; 2. spitting up immediately after the first feeding, violent choking and coughing, or even asphyxia; 3. the catheter placed through the mouth or nasal cavity is blocked at the blind end of the upper esophagus and cannot be passed into the stomach
XI. Complications and treatment
1. pneumonia, esophageal anastomotic fistula strengthen anti-infection treatment, closed drainage of the chest cavity, and if necessary, perform open-chest repair again;
2.Esophageal stenosis Repeated postoperative esophageal dilatation, if the effect is not good, feasible substitution of esophageal surgery;
3.Recurrence of esophageal tracheal fistula Re-surgical ligation.
12. Discharge criteria
1. The child is in good general condition and can eat formula or breast milk normally;
2. stable breathing, no dyspnea, shortness of breath, and no large volume of both lungs
3.Esophagogram indicates that the esophagus is open and no fistula exists.
XIII. Follow up guidance
1. Follow-up visit of esophagogram in 1 month, 3 months and 6 months after discharge from the hospital to understand the esophageal stricture;
2. Early esophageal dilatation should be performed according to the presence of choking and recurrent pneumonia infection after the child has eaten a liquid diet;