April 29, 2021

Innovative techniques help treat boy’s rare condition

Carter Ray has never been inside his family home. As a matter of fact, since he was born on Nov. 3, 2019, he has only lived in a hospital.

Lacey Ray plays with her son, Carter, at Monroe Carell Jr. Children’s Hospital at Vanderbilt. Carter, who has a rare esophageal condition, has spent his entire life in hospitals.
Lacey Ray and son, Carter, pose for a photo on April 22, 2021. Photos by Donn Jones/Vanderbilt University Medical Center.

by Jessica Pasley

Carter Ray has never been inside his family home.

As a matter of fact, since he was born on Nov. 3, 2019, he has only lived in a hospital.

But several innovative, minimally invasive techniques used over the past 12 months to improve Carter’s health — including a first-of-its-kind procedure performed in the United States — means he can finally go home.

Carter, nearly 18 months old, was born with a very rare form of esophageal atresia (EA), transesophageal fistula (TEF) Type B and tracheobronchomalacia (TBM), conditions that ultimately required reconstruction of the esophagus.

Seen in 1 in 4,000 U.S. births, EA is a rare birth defect where part of the esophagus isn’t developed properly and sometimes has a connection to the airway. TEF caused secretions to collect into Carter’s airway and lungs and led to several bouts of pneumonia, while TBM resulted in excessive weakness and near collapse of his windpipe wall. These conditions interfere with breathing and can cause significant lung issues.

The complexity of the case required the specialized care of the surgical team at Monroe Carell Jr. Children’s Hospital at Vanderbilt, led by Irving Zamora, MD, MPH, assistant professor of Pediatric Surgery and director of Advanced Minimally Invasive Surgery, who put together a team of experts including surgery partners Harold Lovvorn, MD, Nathan Novotny, MD, as well as ENT colleagues, Christopher Wooten, MD, and Lyndy Wilcox, MD. Together they focused on repairing the esophagus and airway with the ultimate goal of getting Carter home.

After performing an anterior aortopexy and a thoracoscopic posterior tracheopexy to repair Carter’s airway, which collapsed when he breathed, the multidisciplinary team repaired the airway in a two-stage, minimally invasive approach several weeks apart.

Irving Zamora, MD, MPH, holds patient Carter Ray before his trip home. (photo by Donn Jones)

They first suspended the upper part of the tracheal wall to the aorta through his left chest. In a second operation, through his right chest, they attached the lower portion of the airway to his spine and brought the two ends of the esophagus closer together.

“This is a novel approach that essentially allows for the opening of a collapsed airway,” said Zamora. “Not many institutions are doing this, and even fewer are using a minimally invasive surgery approach to perform these innovative procedures.”

Once the airway was opened, the biggest challenge was reconstructing the esophagus, which was still not connected to allow secretions and nutrients to reach Carter’s stomach.

Zamora and his team took months preparing for the first-ever use of the Connect EA – Magnamosis device in the United States.

This is a procedure that uses magnets to connect the two ends of a congenitally separated esophagus in patients with complex TEF/EA. He collaborated with Michael Harrison and team at UCSF (creators of the magnet device) and Oliver Muensterer in Munich, Germany (the only other surgeon to use the magnets).

Given the novel nature of this approach, the procedure required a compassionate use approval from the FDA. It was accomplished using minimally invasive techniques.

“While this was a milestone for the institution and others interested in such innovations, most importantly, we saved this patient’s life,” said Zamora. “We were able to allow the family hope for going home. That is huge. We are humbled and honored to be a part of Carter’s care.

“This intercontinental, multi-institutional effort that layered science, technology and the guidance of experts across the country and world was absolutely amazing.”

What would have been a potentially daylong, high-risk, open-chest and abdominal surgery was cut to a 38-minute endoscopic procedure.

“Harrison and his team deserve the credit for refining this technology for nearly 20 years. We are fortunate enough to be the first to use it in this country,” Zamora said

The collaborative endeavor required a live interoperative Zoom conference with Harrison and Muensterer.

“Carter was a very, very sick baby when he first came to us, and over the course of the past year we were able to rescue an incredibly complex patient and save his life. We are grateful to his parents for entrusting us with his care,” Zamora said.

The team was able to place magnets in close proximity to each other on the ends of the esophagus. As the magnets were drawn to each other, the tissue between the magnets began to disintegrate or die, allowing the ends of the esophagus to grow together and create a connection (an anastomosis). After 15 days, the magnets were removed.

“The use of the magnets was a minimally invasive way of being able to create that connection without having to perform a complex and high-risk open surgery,” said Zamora. “He has tested us to our limits, and we answered the call at every turn.”

On April 27, Carter Ray was finally able to leave the hospital for the first time since he was born 17 months ago. Here, Children’s Hospital personnel celebrate the milestone.
On April 27, Carter Ray was finally able to leave the hospital for the first time since he was born 17 months ago. Here, Children’s Hospital personnel celebrate the milestone. (photo by Donn Jones)

Although tracheostomy- and ventilator-dependent, Carter no longer requires tubes coming from his mouth and nose to suction secretions and help him breathe.

With the help of physical and occupational therapy, the team is hopeful that he will learn to swallow, walk and begin experiencing many firsts.

“Carter was born 17 months ago, and we haven’t gotten him home yet,” said his mother, Lacey Ray. “Now there is hope that we will all be home together because of the work of Dr. Zamora and his team. I’m glad there are teaching hospitals like this. It’s why they can try new things that will hopefully help others in the future who will need this kind of procedure.

“It’s not like this has been easy, and we have all had to take risks, but without them, we wouldn’t have learned how to repair his esophagus. Sometimes you have to take chances to make improvements, and you have to have someone willing to be the first.

“Since arriving at Vanderbilt, Zamora’s team has focused on how to fix Carter and get him home. He has made him a priority and not an afterthought. His care is genuine,” she said.

Carter was finally able to leave the hospital for the first time on April 27 to join the family at their home in Lynchburg, Tennessee, where his older brother, Jackson, 3, can begin developing a sibling bond.

“My biggest wish now is to see my kids be brothers,” Lacey Ray said. “He is a miracle, and we are looking forward to our future together.”