The incidence of congenital esophageal atresia (EA) is approximately 1 in 2500-1 in 4000 newborns, with a slightly higher incidence in twins. About 50% of esophageal atresia is associated with congenital malformations of other organs. Treatment of esophageal atresia has improved considerably in the last 20 years, with an overall survival rate of more than 90%.
However, the treatment of esophageal atresia still faces many problems, and low body mass, combined with complex malformations and long segmental esophageal atresia are still important factors affecting the prognosis, especially the treatment of long segmental esophageal atresia, although there are several surgical options, there is still no ideal treatment. Based on expert experience, the current situation in China and recent literature reports, the Neonatal Surgery Group of the Pediatric Surgery Branch of the Chinese Medical Association has reached the following consensus on the diagnosis and treatment of congenital esophageal atresia for colleagues’ reference.
I. Diagnosis of congenital esophageal atresia
Prenatal diagnosis of esophageal atresia is still difficult, and only a small number of children can be diagnosed prenatally. Ultrasonography from 16 to 20 weeks of gestation should suspect esophageal atresia if there is excessive amniotic fluid with small gastric alveoli or if it is absent, but the sensitivity and specificity of the diagnosis is low, and the positive rate of this sign can reach 75%-90% for type I esophageal atresia. The upper esophageal blind pouch sign detected by B-mode ultrasonography at 32 weeks of gestation is a more reliable sign for prenatal diagnosis of esophageal atresia. In children with congenital esophageal atresia, the proximal esophagus is dilated and the distal esophagus is absent on T2-weighted MRI, which is more sensitive, but the false-positive rate for esophageal atresia is higher with MRI alone.
Recommendation: Prenatal B-mode ultrasound reveals signs such as blind pouch sign, gastric vesicles not showing, and excess amniotic fluid, which can diagnose partial esophageal atresia prenatally, and fetal MRI screening is recommended.
2. Postnatal diagnosis After birth, the child shows excessive salivation, choking and coughing when drinking milk, cyanosis, and inability to insert or fold back the gastric tube. The diagnosis relies on X-ray and 0.5-1ml of non-ionic contrast agent is injected through the catheter, and the proximal blind end of the esophagus can be detected on the frontal and lateral chest films. CT esophageal 3D reconstruction can be performed to clarify the location of the distal esophagotracheal fistula if the contrast shows a high proximal esophageal blind end.
CT is useful to determine the location of the fistula and the distance to the blind end, and is mainly used for esophageal atresia with distal and proximal esophagus or with multiple malformations. Preoperative bronchoscopy is routinely performed in more than 60% of children’s medical centers abroad and can detect and determine the location of fistulas as well as identify specific types of fistulas.
Recommendation: Clinical signs and failure to insert a gastric tube after birth are highly suggestive of a diagnosis of esophageal atresia; the common types of type III and I esophageal atresia are diagnosed on chest and abdominal plain radiographs and conventional esophagograms. CT esophageal 3D reconstruction is feasible in cases where preoperative imaging shows a high proximal esophageal blind end. For rare types of esophageal atresia such as type II, type IV, and type V esophageal atresia, esophagoscopy and tracheobronchoscopy should be combined to clarify the fistula and its location and guide the surgery.
More than 50% of children with esophageal atresia have other congenital anomalies, and some of them have two or more anomalies (VACTERL syndrome), the most common of which are cardiovascular anomalies (23%), limb and bone anomalies (18%), anorectal and gastrointestinal anomalies (16%), urinary anomalies (15%), head and neck anomalies (10%), mediastinal anomalies (8%), chromosomal anomalies (5.5%), and chromosomal anomalies (5.5%). The deformity of colored body is 5.5%.
Recommendation: A comprehensive physical examination should be performed to determine whether there are combined limb, skeletal, head and neck, and rectoanal malformations, and cardiac ultrasound and urinary ultrasound should be performed routinely before surgery to determine cardiac and urinary malformations, especially complex cardiac malformations, and to change the surgical approach if there is a right aortic arch.
Preoperative risk classification of esophageal atresia
The preoperative risk classification of children is helpful for clinical formulation of reasonable treatment plan and prognosis, and the Montreal classification considers whether to rely on mechanical ventilation and combined malformations as important factors for prognosis.
The Spitz classification focuses on the effects of combined congenital heart disease, and
Class I: body mass >1,500 g without significant cardiac anomalies, with a survival rate of 96%.
Class II: body mass <1,500 g or significant cardiac anomalies, with a survival rate of about 60%; Class III: body mass <1,500 g or significant cardiac anomalies, with a survival rate of about 60%.
Grade III: body mass < 1,500 g with significant cardiac malformation, survival rate only 18%.
Recommendation: The Montreal and Spitz classifications are useful for preoperative evaluation and can better predict the prognosis of the child. In China, the severity of cardiac malformations, such as the combination of complex cardiac malformations represented by tetralogy of Fallot and transposition of the great arteries, as well as the severity of perioperative combined pneumonia and pulmonary dysplasia, are important factors affecting the prognosis of children with esophageal atresia.
Surgical approach
Surgery is usually performed 24-72 h after birth. The surgery is divided into open surgery and thoracoscopic surgery.
1. Open surgery for esophageal atresia Open surgery generally chooses an extrapleural approach, which has less impact on lung function. The left-sided position is chosen for surgery, or the right-sided position if the right aortic arch deformity is found by preoperative B-mode ultrasound. Most of the surgical incisions are chosen as posterior lateral incisions between 4-5 ribs at the subscapular angle. Cutting the odd vein arch helps to expose the tracheoesophageal fistula, and the fistula is cut after suturing close to the trachea. The proximal blind end of the esophagus can be identified by inserting a gastric tube, and the proximal esophagus can be separated above the level of the thoracic inlet, which requires careful inspection for fistulae between the proximal blind end of the esophagus and the trachea.
The distal end is not easily separated too much to avoid affecting the blood supply to the esophagus. The esophageal anastomosis is interrupted with 6-0 or 5-0 single-strand absorbable sutures, and the posterior wall of the esophagus can be anastomosed with the help of an assistant who can insert a gastric tube before anastomosing the anterior wall. The extrapleural approach can be performed without drains, but if transthoracic operation requires continuous negative pressure drainage of the thoracic cavity, the placement of drains is more beneficial for the observation and treatment of postoperative anastomotic fistula. Recommendation: The extrapleural approach has less impact on the lung function of the child and the postoperative recovery is faster. Cutting the odd vein helps to expose the tracheoesophageal fistula, and placing a continuous negative pressure drain in the chest is more favorable for the observation and treatment of the postoperative anastomotic fistula.
2.Thoracoscopic esophageal atresia surgery Thoracoscopic esophageal atresia surgery can shorten the postoperative recovery time and reduce the incidence of postoperative pain and postoperative thoracic deformity, but it requires the surgeon to have rich experience in the specialty and good lumpectomy skills, as well as the effective cooperation of anesthesiologists. About half of the specialists in developed countries have started to use thoracoscopic surgery for esophageal atresia, and large pediatric centers in China have been performing thoracoscopic surgery in recent years.
Most scholars believe that thoracoscopic surgery should not be an option for children with severe congenital heart disease, low body mass, long-segment esophageal atresia, and poor general condition. Thoracoscopic surgery requires attention to the occurrence of intraoperative hypercapnia and acidosis.
Recommendation: The operating hospital should have extensive experience in neonatal and thoracoscopic anesthesia and a high level of NICU care. The surgeon should be certified (many years of experience in open esophageal atresia and good lumpectomy skills or training in lumpectomy techniques). In China, we recommend that beginners of thoracoscopic esophageal atresia surgery should be selected from children with a body mass of 2.5 kg or more, without severe congenital heart disease or severe pneumonia, and with a blind esophageal distance of 2 cm or less.
Preoperatively, the location of the fistula should be determined on the basis of the imaging and tracheoscopic findings, and the surgical approach should be chosen. The fistula is usually short and the tracheoesophageal wall is tightly packed in a “)(” pattern, which should be carefully separated during surgery to avoid tracheal or esophageal injury. Accurate identification of the fistula is the key to successful surgery, and there are reports of the use of combined gastroscopy and bronchoscopy with the aid of a guide wire drawn from the airway to the esophagus to accurately locate the fistula and facilitate intraoperative identification and accurate repair of the fistula.
After the fistula is ligated, the sternocleidomastoid muscle flap or subglottis muscle flap can be freed to fill the tracheal and esophageal gap to prevent fistula recurrence. Recommendation: The location of the fistula should be determined preoperatively based on esophagogram and esophagoscopy or tracheobronchoscopy. A cervical approach can be chosen for fistula locations above the level of the second rib, and a thoracic approach can be chosen for locations below the level of the second rib.
Treatment strategy for long gap esophageal atresia
Long gap esophageal atresia refers to a situation where the proximal and distal ends of the esophagus are more than 2 vertebrae apart (approximately 2 cm). The treatment of long-segment esophageal atresia remains a challenge, and no ideal treatment has been found. Generally speaking, if the distance between the proximal and distal esophagus is more than 3 cm, there is a technical difficulty in the surgery for phase I anastomosis. The common practice is to perform gastrostomy for gastrointestinal nutrition in the neonatal period first, to prevent saliva aspiration by suction or drainage at the proximal end, and to perform transthoracic fistula ligation if there is esophagotracheal fistula at the distal end to avoid aspiration pneumonia due to gastroesophageal reflux.
1. Surgical approaches to esophageal lengthening It is generally believed that repair of the child’s own esophageal lengthening is preferable to substitution of esophageal surgery, and therefore various surgical approaches to esophageal lengthening have arisen. A circumferential esophageal plasma myotomy (Livaditis procedure) can effectively lengthen the esophagus by 5-10 mm, but there is a risk of proximal esophageal end necrosis resulting in anastomotic fistula and esophageal diverticulum. In general, proximal and distal esophageal distances of more than 3 cm make one-stage anastomosis technically difficult.
Recommendation: If the anastomosis is still under large tension after adequate freeing of the proximal and distal esophagus, esophageal lengthening can be chosen.
2. One-stage anastomosis of the extended esophagus Currently, the concept of treatment of long-segment esophageal atresia has changed: even without external traction, the rate of esophageal lengthening and dilation is much faster than the growth of the body, and the fastest period of esophageal growth is 8-12 weeks, depending on the stimulation of swallowing reflex and gastric reflux. Accordingly, some international scholars believe that the proximal and distal esophagus is less than 2 vertebrae away from the esophagus and that a one-stage end-to-end esophageal anastomosis should be performed.
The proximal and distal esophagus is located between 2 and 6 vertebrae, and a postponement of the esophageal anastomosis is used; the proximal and distal esophagus is greater than 6 vertebrae, and a second-stage esophageal repair or esophageal replacement is used. The Foker’s procedure, which uses external pulling force to accelerate the growth of the esophagus and postpone the first-stage anastomosis, is a more feasible procedure with satisfactory long-term follow-up results.
Recommendation: If the medical unit has a high level of NICU monitoring and care, has some experience in delayed esophageal surgery, the proximal and distal esophageal distance is between 2-6 vertebrae, and the parents can afford better, delayed one-stage anastomosis can be performed on a trial basis.
Since there are still many complications associated with Foker’s operation or delayed one-stage anastomosis, there are still more specialists who choose various alternative esophageal surgeries. There are also a few reports of gastroesophageal anastomosis performed in the neonatal period in one stage, which is relatively risky and needs to be evaluated effectively.
(1) Gastric substitution of the esophagus Gastric substitution of the esophagus is a common choice for long-segment esophageal atresia because of the well-developed gastric musculature and abundant blood supply. In total gastric substitution surgery, the bulk of the stomach is in the mediastinum and chest, and the dilated stomach is more obviously compressing to the heart and lungs, and the gastric emptying is also slower and prone to reflux, while gastric tube substitution esophagus can effectively reduce the volume of the stomach in the chest cavity, and it has become a common procedure in pediatric surgical esophageal substitution surgery in recent years. Gastric tube substitution is performed by cutting the proximal side of the gastric body and cardia into a 2 cm diameter gastric tube, lifting it to the neck and anastomosing it with the esophagus, keeping the distal 1/4 of the gastric body in the abdominal cavity, which can reduce reflux and vomiting.
(2) Colonic interposition surgery The physiological functions of the colon and esophagus differ greatly, and the incidence of postoperative functional obstruction and anastomotic stricture is also higher. Compared with gastric substitution, colonic interposition has more gastrointestinal complications (40.3% vs. 35.4%) and fewer respiratory complications (7.0% vs. 10.8%).
(3) Interjejunal surgery Interjejunal surgery in pediatric patients has been reported less frequently. The advantage of interjejunal surgery is the appropriate caliber and good peristaltic function, but the instability of blood supply is the problem it faces, and the incidence of postoperative respiratory complications varies widely from report to report.
Recommendation: There is no ideal treatment for long-segment esophageal atresia. Gastric substitution esophagotomy is the commonly used option, and colonic interposition surgery colonic substitution esophagus currently has the highest number of cases in the literature, and the clinical results are still satisfactory. The jejunal interposition surgery has even less experience. Only a few children’s medical centers in China have carried out the above procedures, and experience needs to be accumulated.
(4) Alternative esophageal routes There are posterior sternal and posterior mediastinal esophageal routes. The posterior mediastinal route is more common, and the abdominal incision is made through the posterior mediastinal space with blunt separation of the tunnel to the neck, but about 47% of children require additional thoracic incisions if fibrous adhesions are caused in the first stage of surgery. The posterior sternal approach avoids the adhesions caused by stage I surgery and reduces surgical trauma without opening the chest, but may cause compression of the heart.
In recent years, laparoscopic gastric substitution of the esophagus has also been used, with pyloroplasty followed by lifting of the stomach from the posterior mediastinum to a cervical anastomosis, and thoracoscopic surgery can be used in combination if the thoracic adhesions are severe. Recommendation: The posterior mediastinal route is the original esophageal route, with a short path and less pressure on the heart and lungs, which is more desirable, but the blunt separation of the tunnel requires great care and high operational skills. If it is difficult to form the posterior mediastinal tunnel, it is recommended to change to the posterior sternal route to reduce the risk of surgery.
V. Surgical strategy for esophageal atresia combined with other congenital gastrointestinal tracts
Children with esophageal atresia are often combined with other gastrointestinal anomalies, the more common one being congenital anal atresia. However, for children with significant abdominal distension that seriously affects breathing, colostomy or anoplasty can be performed first, followed by esophageal anastomosis or gastrostomy.
For children with combined cricothyroid pancreatic and duodenal atresia, if the diagnosis is clear before esophageal atresia, they should be treated together with surgery. If recurrent vomiting and gastroesophageal reflux occur after esophageal atresia, duodenal obstruction or pyloric stenosis should be ruled out, and gastrointestinal imaging or B-mode ultrasonography can clarify the diagnosis. Recommendation: If esophageal atresia is combined with gastrointestinal malformation, surgery can be performed at the same time; if combined with anal atresia, if it is not determined to be low atresia, colostomy should be performed in one stage.
Perioperative treatment
In addition to the general preoperative management of neonatal surgery (including warming, rehydration, anti-inflammation and maintenance of general condition), the key is to prevent aspiration and reflux pneumonia: (1) sputum should be continuously aspirated before surgery to avoid choking caused by oral secretions; (2) semi-recumbent position to reduce gastroesophageal reflux. The ventilator should be used routinely for 24-48 h after surgery, and the ventilator should be withdrawn only after stable spontaneous breathing. Upper gastrointestinal tract angiography is feasible 1 week after surgery to understand the healing of the anastomosis. The child with a gastrostomy can be fed via nasogastric tube for 3-5 days after surgery and through the stoma tube for 48 hours after surgery.
Recommendations: Continuous aspiration before surgery to reduce the occurrence of aspiration pneumonia; microfeeding through a gastric tube 3-5 days after surgery; gastrointestinal imaging 7-10 days after surgery to check the healing of the anastomosis.
VII. Treatment of postoperative complications
1. Anastomotic leak The occurrence of postoperative anastomotic leak is related to large anastomotic tension, excessive esophageal separation leading to blood flow disorders, gastroesophageal reflux and anastomotic technique. In case of anastomotic leak, adequate drainage should be continued and nutritional therapy should be strengthened. If gastroesophageal reflux is suspected, tube feeding can be suspended or tube feeding can be lowered below the duodenum. Simple anastomotic leaks can heal in 2-4 weeks with conservative treatment. Recurrent tracheoesophageal fistulas often require reoperation. In recent years, there has been a significant increase in recurrent tracheoesophageal fistulas in China, associated with the popularity of esophageal atresia surgery and the initial development of thoracoscopic surgery, which should be noted because of the difficulty of reoperation.
2. Anastomotic stenosis The incidence of anastomotic stenosis is 34.9% -49%, and the occurrence of stenosis is related to factors such as anastomotic tension, anastomotic leakage, suture type and gastroesophageal reflux. Mild stenosis can be gradually improved by swallowing activities and can be observed on follow-up. When symptoms such as dysphagia, esophageal foreign body and recurrent pneumonia appear, esophagogram or gastroscopy should be performed to clarify the degree and length of esophageal stricture. For simple and limited strictures, dilation is an effective treatment, and balloon dilation is safer and more effective than probe dilation.
An interval of 2 weeks to 1 month between dilation sessions is appropriate. Postoperative dilatation for esophageal strictures can be performed 1-15 times, and most symptoms improve within 6 months of dilatation, with a success rate of 58%-96%. The number of dilatations and the interval between dilatations should be designed individually according to the child’s symptoms. For complex stenosis with a stenosis of more than 2 cm and distorted esophagus, surgical resection may be considered if the child still has difficulty in eating and growth retardation after multiple dilation treatments.
3. Gastroesophageal reflux About 50% of children with esophageal atresia have different degrees of gastroesophageal reflux after surgery, especially in long-segment esophageal atresia. Children may present with recurrent vomiting, food refusal, irritability, cough, recurrent pneumonia, and low body mass. The preferred diagnostic method is upper gastrointestinal angiography.
4. Long-term complications Difficulty in swallowing is a common symptom after esophageal atresia, and esophageal manometry shows that about 70% of children have esophageal motility disorders, but about 1/3 of them have no clinical symptoms. Some children have growth retardation. The incidence of respiratory diseases such as bronchitis, chronic cough, pneumonia, and asthma is also higher in children after esophageal atresia surgery, and the incidence of respiratory diseases in adolescence can reach about 40%.