How do doctors diagnose esophageal atresia?

Some cases of esophageal atresia can be diagnosed prenatally (during pregnancy) through an ultrasound. Mothers who are pregnant with babies who have esophageal atresia commonly experience polyhydramnios during pregnancy. Polyhydramnios is when there is excess amniotic fluid in the uterus. Polyhydramnios can occur for many reasons, but it may be a sign of esophageal atresia if the baby’s stomach is unusually small or their fetal stomach bubble cannot be found during an ultrasound. The fetal stomach bubble is what shows up as your baby’s stomach on an ultrasound. It typically looks like a black bubble in your baby’s abdomen. 

Usually, doctors can diagnose your baby with esophageal atresia shortly after your baby is born. The doctor does this by passing a feeding tube through your baby’s mouth or nose. An x-ray will show that the tube does not go beyond the upper portion of the esophagus.

How is esophageal atresia treated?

Babies with esophageal atresia or tracheoesophageal fistula will need surgery soon after birth. Without early treatment, esophageal atresia could cause aspiration (when stomach contents are accidentally breathed into the lungs).

Typically, babies who have esophageal atresia and/or a tracheoesophageal fistula will not breastfeed or eat by mouth right away. For this reason, your baby will receive nutrition through an intravenous catheter (IV) while waiting to have surgery.

What are the types of surgery available at MGHfC for esophageal atresia and/or tracheoesophageal fistula?

At MGHfC, the care team provides a well-rounded, multidisciplinary approach to caring for children with esophageal atresia. Our surgeons are experienced and trained with multiple surgical techniques, including both open and thoracoscopic repair of esophageal atresia and/or tracheoesophageal fistula. Our surgeons work closely with children and their families to personalize their surgical care. Often, a minimally invasive approach is possible, meaning smaller incisions and, potentially, a faster recovery.

Open repair esophageal atresia and/or tracheoesophageal fistula surgery is when surgeons make large incisions in your child’s abdomen and/or neck to repair the esophageal atresia and/or tracheoesophageal fistula. Time to operate and recovery for this surgery are much longer than thoracoscopic surgery.

Thoracoscopic surgery is when a surgeon inserts a thoracoscope through a small incision through the skin. The thoracoscope is a thin, flexible tube with a small camera on the end that allows surgeons to operate without making large incisions. Thoracoscopic repair of esophageal atresia was first performed in 2000 and remains one of the most effective treatments currently available.

What can I expect for my child’s thoracoscopic repair surgery?

First, your child will receive general anesthesia (medicine to help your child sleep safely and soundly during surgery). Then, surgeons make three incisions around the areas affected by esophageal atresia and/or tracheoesophageal fistula. One incision is for the camera and two are for instruments. From there, surgeons will connect the upper and lower sections of the separated esophagus. They will also close the tracheoesophageal fistula. Lastly, surgeons will insert a chest tube to help remove excess air from space around the lungs. This allows the esophagus and/or tracheoesophageal fistula to heal. It also helps your child breathe easier after surgery. Surgeons will also remove the thoracoscope and close the incisions with dissolvable sutures (stitches that dissolve on their own over time).

What can I expect for my child’s recovery after thoracoscopic repair surgery?

The outcomes between open and thoracoscopic repairs are similar. On average, children who have thoracoscopic surgery spend fewer days on narcotics (prescription pain medicines) and feeding tubes. They also wait fewer days before being extubated (having a breathing tube removed). Additionally, they experience fewer strictures (narrowing of the esophagus) and fewer leaks in the esophagus and/or trachea.

MGHfC is one of several children’s hospitals around the country that offers thoracoscopic repair of esophageal atresia and/or tracheoesophageal fistula. Cornelia Griggs, MD, of Pediatric Surgery at MGHfC, learned how to perform this treatment from the creator of the technique.

What are the possible risks associated with thoracoscopic surgery?

Most babies experience some esophageal dysmotility following surgery. This means they may have trouble keeping food and liquid down. Possible risks associated with surgery include:

  • Gastroesophageal reflux disease (GERD): A condition in which stomach acid travels up esophagus and causes heartburn-like symptoms, such as sore throat, a burning feeling in the chest or trouble swallowing
  • Breathing difficulties like tracheomalacia (when the windpipe walls are weak and cause wheezing or a high-pitched sound when inhaling)
  • Stricture (narrowing) of the esophagus, which can be caused by scar tissue

What is the outcome for my child as they grow up?

Esophageal atresia treatment methods have greatly improved over the past several decades. Now, babies born with esophageal atresia are expected to live well into adulthood if their condition continues to be managed properly. Postoperative (after surgery) complications, like GERD, could become long-term health conditions. Additional surgeries and medications may be needed.

The Pediatric Aerodigestive Center Team at MGHfC

The Pediatric Foregut Program works closely with the Pediatric Aerodigestive Center. As a treatment program shared between MGHFC and the Massachusetts Eye and Ear Infirmary, the Pediatric Aerodigestive Center brings together dedicated practitioners who specialize in caring for children with compromised abilities to eat, breathe or speak. The collaboration with the Pediatric Aerodigestive Center allows us to care for children with both short-term and ongoing aerodigestive difficulties with a multidisciplinary approach. By creating a team of providers from many different backgrounds and skillsets, the care team develops individualized care plans to provide every child with the best care possible.