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.

More technical medical information is available in our Referring Physicians section.

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.