treating severe CDH with FETO: expert care + support from before birth and into toddlerhood

special editorial submitted by friends steward partner, Cincinnati Children's Fetal Care Center

TTrey is a typical toddler, happily crawling and walking everywhere. His development before birth was anything but usual.

When mom Nora was 24 weeks pregnant, her obstetrician noticed something unusual during an ultrasound. Trey’s heart was pushed to the side, and there was excess fluid in his chest.

The doctor referred Nora and her husband Tim to a maternalfetal medicine (MFM) specialist near their home in Indianapolis. Trey was diagnosed with a severe right-sided congenital diaphragmatic hernia (CDH).

CDH is a rare condition that occurs when a baby’s diaphragm—the layer of muscle between the abdominal and chest cavities—doesn’t close properly. Right-sided CDH is rarer than leftsided CDH.

In Trey’s case, a large portion of his liver and intestines pushed through the opening in his diaphragm and into the chest cavity. The overcrowding organs in his chest put pressure on his heart and lungs, preventing them from developing normally.

understanding options: reasons for hope

The MFM specialist sent Nora and Tim to Cincinnati Children’s Fetal Care Center for a consultation. During a twoday visit, they learned that Trey’s condition was more severe than they realized. Nora and Tim met with fetal care experts, including neonatologists, MFM doctors, fetal nurses and Foong-Yen Lim, MD, surgical director of the Fetal Care Center.

Dr. Lim and the team talked with Nora and Tim about:

• Treatment options

• How Trey might be affected

• Challenges and roadblocks Trey might face after birth

Cincinnati Children’s offers a specialized treatment for CDH called fetoscopic endoluminal tracheal occlusion (FETO). This procedure places an inflated balloon in the trachea (windpipe), blocking the airway. This promotes lung growth by building up fluid and pressure in the developing lung.

The team was transparent with Nora and Tim, explaining that FETO may not work for Trey, Dr. Lim says. “We wanted them to feel supported, regardless of how they decided to proceed.”

Nora says Dr. Lim and other specialists “went out of their way to make sure that we had choices and we were an equal part of the team.”

The couple’s biggest questions for Dr. Lim dealt with CDH specifics, along with the FETO procedure and how Trey might be affected. “We really wanted to understand what that could possibly look like, Nora says. “They answered all of our questions.”

After the first day of appointments, Tim says he and Nora felt a sense of peace thanks to several factors, including the collective expertise of the fetal care team and the resources available to them across numerous divisions and departments at Cincinnati Children’s.

“We both got in the car after the appointment and knew we were going to Cincinnati Children’s,” Nora says. “In order to feel like we did everything to help Trey survive and thrive, we knew it was where we needed to be.”

Carefully Managed Care

Cincinnati Children’s is highly skilled in treating CDH and in using the FETO procedure for children with severe CDH. The fetal care team strives to improve the care environment for babies with the most severe CDH, Dr. Lim says. Tracheal occlusion improves the management of severe CDH patients “from the moment they are born,” Dr. Lim says. “Hopefully, they will be slightly different babies compared to if they did not have the FETO intervention.”

Tracheal occlusion accelerates lung growth that otherwise would not be possible in a baby with a severe hernia.

“We still don’t fully understand how it works or why some patients have a great response while others have minimal to no response,” Dr. Lim says.

Because there is no way to identify which patients will or will not respond well to the therapy, FETO is only offered to patients with the most severe CDH. In most cases, Cincinnati Children’s can successfully manage less severe cases of CDH with other treatments.

“For those who historically we struggle to get them through all the hurdles, we hope that this can have a positive impact on the outcome,” Dr. Lim says.

FETO is not a guaranteed cure. Currently, 80% of patients who receive FETO for severe CDH survive and are discharged from the hospital; while survival for mild CDH approaches 100%.

the FETO process

Tim and Nora chose to move forward with the FETO procedure for Trey. Families that choose the FETO treatment at Cincinnati Children’s commit to living in Cincinnati for months before and after their child is born.

The decision shows a family’s passion to do all they can for their child’s care. Dr. Lim hopes that Tim, Nora and Trey’s story inspires other families and shows them it is possible to go through the process of managing a challenging diagnosis with care and support.

“It’s a difficult journey, and the outcome isn’t always rosy,” Dr. Lim says. “But there are things we can do for patients despite some severe conditions.”

Dr. Lim, Jose Peiro, MD, Kara Markham, MD, and David McKinney, MD, performed Trey’s surgery. Tim received regular updates from a nurse in person and by text message. The fetal team takes it a step further with updates after each stage of the procedure.

“They texted me play-by-play updates. It was awesome,” Tim says. “My wife and my baby were in there—my whole world—so it was good to have those updates to know what was going on.”

After the procedure, the care team provided Tim with a full recap. Once Nora came out of sedation a few hours later, the team gave her a full update.

Families whose babies receive the FETO procedure must stay close to Cincinnati Children’s in case mom goes into labor before the balloon in the trachea is removed. If labor occurs while the baby’s airway is still obstructed, specialists perform a c-section and remove the balloon right away, Dr. Lim says.

Babies whose tracheal balloon is removed before birth (usually at 34 weeks) can safely be born vaginally.

Tim and Nora stayed in Cincinnati. Trey’s tracheal balloon was removed a few weeks after the original FETO procedure. The couple continued their Cincinnati stay so that Trey could receive the best possible care until his birth.

CDH babies treated with FETO are less likely to need a heart-lung bypass machine or extracorporeal membrane oxygenation (ECMO) after birth.

“If we can avoid ECMO in some of these babies, that is a good response,” Dr. Lim says. “It doesn’t guarantee survival, but so far, all babies who did not need heartlung bypass have survived.”

A Psychosocial Journey

Trey had a good response to the FETO procedure. Born in June 2021, he spent three months in the newborn intensive care unit (NICU). He was on ECMO for 18 days. During that time, Beth Rymeski, DO, and Dr. Lim repaired his large right-sided diaphragmatic hernia.

It was a difficult time for them. Every family who receives the FETO procedure undergoes careful diagnosis and counseling. There are risks with the procedure. Most babies are also born early, which comes with additional risks.

“This is not trivial,” Dr. Lim says. “The family goes through all kinds of thinking processes, not knowing if this will work for their baby.”

Families eligible for FETO must meet selection criteria set by the Food and Drug Administration. They must have a severe CDH diagnosis. Their babies cannot have any major conditions that could significantly impact the procedure outcome, such as other significant congenital anomalies or genetic conditions.

Not every family offered FETO as an option chooses the treatment, Dr. Lim says.

Once babies are born, hernia repair surgery takes place as soon as the infant is stable. For babies with extremely severe CDH who cannot come off ECMO without the hernia repair, doctors make the correction within a few days of birth, Dr. Lim says.

family engagement key to baby’s recovery and development

typically remain in the NICU for a while after their hernia repair, as Trey did.

That time gives the family many opportunities to learn about their baby and the care they need. Parents of fetal patients absorb so much information that by the time the baby is born, they are more knowledgeable about the baby than some of the providers caring for them, Dr. Lim says.

Indeed, Tim and Nora say they learned a great deal during daily meetings with the team.

“We had a crowd every day for rounds,” Tim says. “They were all great.”

Dr. Lim and the Fetal Care Center are “still actively studying the severe CDH patient population to see how they can improve care in the future.”

Children treated at Cincinnati Children’s are doing well. Dr. Lim recalls a patient who is now 6, and another who is 4. In total, 50 FETO procedures have been completed worldwide by the surgeons at Cincinnati Children’s Fetal Care Center.

Trey, who is now more than a year old, continues to progress. He’s an active toddler. He’s off oxygen, is growing and reaching developmental milestones like crawling and eating.

Dr. Lim says a big reason for Trey’s success is his family’s engagement in his care. Family involvement helps a lot of these patients realize their full potential.

Of course, Nora and Tim will do whatever Trey needs to live his best life.

“We knew we had to be strong for him,” Nora says. “In our minds, we’re focused on going forward.”

That’s what Dr. Lim likes to hear—that patients and families have options and hope.

cincinnati children's fetal care center

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