Esophageal Atresia

What is esophageal atresia?

The esophagus (feeding tube) is the tube that connects the mouth with the stomach. The esophagus is usually present in a fetus at 30 days of gestation. Esophageal atresia is an abnormality in which the middle portion of the esophagus is absent. The esophagus may end blindly into a pouch or may connect to the trachea. Esophageal atresia occurs about 1 in 4000 live births annually.

Children born with esophageal atresia may have other abnormal connections between the esophagus and the trachea (breathing tube). Esophageal atresia is classified by the length of the esophagus as well as by the connection between the esophagus and the trachea.

The most common type is proximal atresia and distal tracheal fistula (connection). The type and timing of surgery will depend on the anatomy of your child's esophagus and trachea. Children born with a long gap between the two ends of the esophagus may have to wait for surgical repair to allow for growth of the esophagus.

Approximately 50 % of children born with esophageal atresia have other associated problems that may be present. Therefore soon after birth, a series of ultrasounds and X-rays will be performed to identify any of these problems. These associated problems may need immediate treatment or may require no treatment at all. In the hospital, you may hear the physicians discussing a "VACTERL workup". This is a term that is used to define the possible problems associated with esophageal atresia.

V = verebral this involves the bones in the spine being formed abnormally. The most common is hemivertebrae.

A = anorectal this involves formation and blockages in the rest of the GI tract. The most common is imperforate anus.

C = this involves the heart. The most common is a patent ductus arteriosus.

TE = this is the tracheal and esophageal abnormalities. The most common is the proximal esophageal atresia with distal tracheal fistula.

R = this involves the kidneys and the tubes connecting them to the bladder. The most common is ureteral reflux.

L = this involves the limbs. The most common is absence of the radial bone in the arm.

How is esophageal atresia diagnosed?

Parents of children with esophageal atresia often are alerted to a problem while still pregnant. Often a prenatal ultrasound will show extra amiontic fluid. The medical term for this is polyhydramnios. If this occurs, your OB doctor will probably monitor your pregnancy more closely. Also a meeting with a pediatric surgeon prior to delivery may be arranged to discuss the special care the infant will need after being born.

The infant has excessive drooling after birth and may choke and cough with feedings. The infant may also turn "blue" (develop cyanosis) with feeding. These problems are related to the saliva in the mouth having no way to reach the stomach. The salvia collects in the blind pouch of the esophagus. This may cause problems with airway obstruction and aspiration pneumonia. To protect the infant's airway, a nasogastric or orogastric tube will be placed. It is usually placed in the blind pouch and the salvia is then suctioned out.

Patients with a tracheoesophageal fistula, but no esophageal atresia, (4% of all children) often present with episodes of gastric distension (stomach bloating) during crying and choking and cyanotic spells during feeding. Diagnosis is best made by bronchoscopy and esophagoscopy which demonstrates the "H-type fistula" between the trachea and esophagus. Occasionally, a barium swallow is necessary for documentation of the fistula.

The simplest way to diagnose esophageal atresia is to attempt pass a nasogastric or orogastric tube. If you meet resistance or find the tube curling back out of the infant's mouth a X-ray will show the tube in the upper pouch. Further X-ray studies involve careful administration of contrast into the upper pouch with the patient sitting upright in order to avoid aspiration. This dye will be seen on X-ray and will define the presence of esophageal atresia and or the rarely present proximal tracheo-esophageal fistula. An abdominal X-ray will document the presence of air within the G.I. tract which also helps distinguish esophageal atresia with a distal tracheoesophageal fistula (85%) from a pure esophageal atresia (7%). Children with pure esophageal atresia will have no air in the stomach as is not connected to the mouth.

Preoperative Management

The goal of before surgery is prevent aspiration of stomach contents or salvia into the lungs. A nasogastric or orogastric tube will be placed into the proximal esophageal (blind pouch) on continuous suction to prevent aspiration of secretions. The infant with a tracheoesophageal fistula usually has the head of the bed in order to prevent stomach secretions from refluxing into lungs. Intravenous antibiotics should be administered to help prevent the development of pneumonia. The "VACTERL" workup is completed prior to surgical repair.

Surgery

The goal of operative therapy is to correct the problem if possible with one operation. In general, patients with esophageal atresia and a distal tracheesophageal fistula have adequate esophageal length to allow primary reconstruction. Currently, this procedure is being performed using a thoracoscopic approach in many centers. Surgery involves stretching the 2 ends of the esophagus and sewing them together. The may be mild to moderate levels of tension at this site after surgery. If the child also has a tracheal connection this is divided and sewed also. The infant will have a chest tube after surgery to allow for drainage of secretions if a leak occurs at the surgical site.

However in newborns with prematurity, substantial respiratory difficulties, and/or associated anomalies and esophageal atresia and tracheoesophageal fistula complete surgical repair is not done with one operation. These factors greatly increase the risks associated with complete surgical repair. While waiting for the infant to stabilize, a gastrostomy tube may be placed to initiate feedings while the blind pouch continues to have suction via nasogastric tube. When the child has grown and has been stable for a period of time the operation will be performed. This usually involves closure of the TEF with reconstruction of the esophagus. The gastostomy tube is most often left in place and can be removed on an elective basis after the child has recovered.

Infants with esophageal atresia and no tracheoesophageal fistula often have a very short esophagus. Thus surgical repair is done in stages over a series of months. Generally, a gastrostomy tube is placed to initiate feedings and to help increase the size of the stomach. Most children also have a nasogastric tube to suction in the blind pouch help the infant deal with oral secretions.

After the gastrostomy tube is place and the child is growing in first few months of life, the upper pouch is dilated daily. This is done in an attempt to stretch the esophagus.

At that point, a decision must be made whether to attempt to salvage the native esophagus. Options include: 1. After 8 to 12 weeks, growth of the proximal and distal esophageal segments will typically allow an esophagoesophagostomy to be performed; or 2) Creation of a cervical esophagostomy with gastrostomy tube placement in the newborn which will allow stable management until reconstruction of the esophagus with stomach, colon, or small bowel may be performed at one year of life.

Postoperatively patients may be extubated as tolerated. Any attempt at reintubation should be performed carefully in order to avoid accidental and catastrophic intubation of the esophagus. The head is flexed slightly forward in order to decrease tension on the esophagoesophagostomy. Oral suctioning is performed only to the level of the pharynx and certainly not distally into the esophagus. Endotracheal tube suctioning must be performed carefully in order to avoid trauma or perforation at the site of the TEF closure. A gastrostomy tube is placed at the time of esophageal repair only if it is anticipated that one will be needed for patient management in the future. Otherwise, a nasogastric tube passed through the anastomosis at the time of esophagoesophagostomy will suffice. A hypaque swallow is performed at seven days after operation. If the anastomosis appears intact, then feedings are initiated, antibiotics discontinued, and the retropleural chest tube removed.

Current overall survival rates are generally in the 85% - 90% range. Mortality is usually secondary to associated anomalies. Immediate postoperative complications include anastomotic leak in 15% of cases. Almost all leaks will spontaneously resolve with continuation of IV antibiotics and chest tube drainage. Stricture formation occurs in approximately 15% of cases and is often associated with an anastamotic leak. Most strictures are responsive to repeated dilatations. Recurrent tracheoesophageal fistula occurs in 5% of cases and requires reoperation with division and ligation of the fistula. The most common long term problems associated with esophageal atresia include gastroesophageal reflux which occurs in up to 70% and tracheomalacia which is observed in approximately 25% of patients.

This information is provided by the University of Michigan Department of Surgery, Section of Pediatric Surgery and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. For additional health information, please contact your health care provider or our offices.