Our Care of Esophageal Atresia

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Esophageal atresia (EA) is a condition present at birth in which a baby’s esophagus (the tube that carries food from the mouth to the stomach) does not form properly. Instead of being one continuous tube, it ends in a pouch and does not connect normally to the stomach.

Because of this, milk or saliva cannot pass from the mouth into the stomach the usual way.

A tracheoesophageal fistula (TEF) is an abnormal connection between the esophagus and the trachea (windpipe). Some babies with esophageal atresia also have a TEF. When this happens, air can pass from the windpipe into the stomach, and food or saliva can enter the lungs, which can cause coughing or breathing problems.

(Pic: child with esophageal atresia with distal tracheoesophageal fistula)

Multidisciplinary Evaluation: meet with MFM specialist, pediatric surgeon, neonatologist, and other experts who will care for your baby

Comprehensive Imaging: detailed ultrasounds and other tests when indicated to assess your baby's condition and help plan treatment

Coordinated Delivery Planning: our team works to ensure your baby receives immediate specialized care at birth

Babies with a known EA are cared for in a neonatal intensive care unit (NICU) that specializes in the care of these infants and where a pediatric surgeon can immediately evaluate your child.  Most cases are diagnosed after birth requiring the child be transferred in to Primary Children's Hospital for care. The neonatologist is a specially trained pediatrician that will manage your baby’s medications, feeding, and daily needs while in the NICU. 

Once the child is born, a neonatologist will evaluate the baby and make sure the heart and lungs are working appropriately. A tube placed through the mouth (oral gastric or OG) into the upper esophagus to suck out saliva and oral secretions to prevent your baby from choking contents into the lungs.   

Your baby will receive fluids and antibiotics through a special IV called a PICC line, initially through the umbilical cord, then placed in one of the limbs.  Because the child will not be initially allowed to eat, they will also receive nutrition through the PICC line called TPN, or total parenteral nutrition. TPN contains protein, fat, sugar, vitamins, and minerals and will meet all your baby’s nutritional needs

The child undergoes an esophagram, or special x-ray study 5 - 7 days after repair to look for a "leak" which can occur in about 10 - 15% of cases. If no leak is present, then feedings are initiated. Most leaks that occur are contained, and will resolve on their own, typically within another 1 - 2 weeks.

A child with EA is typically in the hospital for 4 - 6 weeks, although this may be longer if other conditions are also present, such as a cardiac defect

EA requires surgery, usually within the first few days of life, where:

• The surgeon reconnects the esophagus.
• If a TEF is present, the abnormal connection to the windpipe is closed.
• In some cases, the gap between the two ends is too long, and repair will require staged procedures, including the placement of a gastrostomy feeding tube (G-tube).

This can be done either through an open incision (thoracotomy) or through multiple small incisions (thoracoscopy). Both result in excellent outcomes. The surgeon will discuss these two options prior to the procedure.

The team at Primary Children's Hospital treats some of the highest volumes of EA/TEF in the US, between 30 - 40 new cases annually

(Pic: surgical team performing thoracoscopic surgery)

Children with a esophageal atresia (with or without TEF) may also undergo repair through three small incisions, called a thoracoscopy. The surgeon will discuss this option at the time of surgery

(Pic. Infant undergoing thoracoscopy)

These children typically go to the operating room within the first few days of life, not to repair the EA, but to place a gastrostomy tube, also known as a g-tube. The child is then fed through the g-tube, allowing them to grow. This also allows the stomach and lower esophagus to grow as well.

Around 4 weeks after surgery, the child will go to the operating room again and undergo a calibration study, also referred to as a gap study, where a tube is placed into the upper esophagus through the mouth, and the lower esophagus through the g-tube site. An X-ray then helps measure the distance (or gap) between the upper and lower esophagus.

If the gap is less than 2 cm, then surgery is performed, usually through a thoracotomy, to sew the two ends of the esophagus together. If the gap is still longer than 2 cm, then the child continues feedings through the g-tube for another 4 weeks, when another gap study and possible repair are repeated

(Pic. Infant with long-gap esophageal atresia and gastrostomy tube)