Beyond Congenital Pulmonary Airway Malformation (CPAM): Baby Elisabeth's Story

Anna and Jared walked into the Fetal Care Center for their intake on a Wednesday. By the time they walked out, they had surgery scheduled for Friday.

This experience is common at Connecticut Children’s Fetal Care Center, but it’s a rarity in the world of fetal care. We are one of only 9 centers in the U.S. who provide the full range of fetal interventions offered here at Connecticut Children’s. Only a handful — including Connecticut Children’s — can do the full range of what’s possible, up to and including open surgery for spina bifida. This fact makes Connecticut Children’s Fetal Care Center the only one of its kind in the Northeast.

It was lucky for Anna and Jared, who needed surgery as soon possible.

The baby’s cyst was known as a congenital pulmonary airway malformation (CPAM). To relieve the pressure it was putting on her heart, Dr. Crombleholme’s team planned to place a tiny shunt that would drain any fluid from the CPAM to outside the baby’s chest, safely into the amniotic fluid.

On the Friday of surgery, Dr. Crombleholme guided a fiber-optic tube called a fetoscope — just 3 millimeters in diameter, roughly the size of a matchhead — through Anna’s abdominal wall and uterus, into the baby’s left chest and the CPAM. Known as a fetal thoracoscopy, this technique allowed Dr. Crombleholme to actually see inside the cyst and be sure that, once he placed the shunt, all parts of it would decompress.

That’s exactly what happened. As soon as the shunt was in, the CPAM shrank by 70%, and the baby’s heart shifted back to its normal position. “It worked immediately,” says Anna. The signs of heart failure gradually resolved over the next week.

Nurse Jenn Humphrey remembers hearing the good news. “Not long ago, no one had even conceived of fetal surgery,” she says. “That we can provide this service right here in Connecticut is incredible.”
 

Baby Elisabeth was born at 29 weeks at Hartford Hospital, weighing three pounds and one ounce. Members of her Connecticut Children’s neonatal care team were in the room. They’d been preparing for this moment, both in the labor and delivery room and down the hall in Connecticut Children’s Level IV NICU. They intubated Elisabeth to help her breathe, and inserted a tube into her chest to replace the shunt, keeping the deflated cyst from filling up with air and causing the same dangerous mass effect.

To survive on her own, Elisabeth needed to have that cyst removed entirely, along with part of the lung it was attached to. First, though, she needed to get bigger and stronger. For about six weeks, Anna and Jared traded off sitting beside her incubator in the NICU with being at home for their other two children, ages 1 and 3. It was heart-wrenching to only be in one place at a time, but their family and friends rallied around them. So did Elisabeth’s care team.

“All the nurses at the NICU were wonderful. They kept sending us pictures of Elisabeth, and did really cute things that showed that they cared about her. That they cared about us, too,” says Anna.

Finally, Elisabeth was strong enough for her second surgery with Dr. Crombleholme — who, beyond fetal surgery, is a board-certified pediatric surgeon. Over a few hours, the team successfully removed the left lower lobe containing the CPAM.

It was the moment everyone had been working toward. After the procedure, Elisabeth was able to breathe on her own. She started eating more. She grew healthier and healthier. And then, after 77 days in the NICU, she got to go home.

Her sister, Lydia, and brother, Silas, were waiting to shower her with affection. Thoroughly.

“They love her. Sometimes, they love her half to death. I’m like, ‘Step back!’” says Anna, laughing.
 

Today, at 5 months old, Elisabeth is healthy and thriving. Over the next few years, her body will compensate for the bit of lung that was removed, and by the time she’s racing around the playground with her siblings, she’ll be indistinguishable from any other kid her age. The diagnosis that would have taken her life is now just a story her parents tell.

She is part of a generation of youngsters saved by recent advances in fetal care. Each year, Dr. Crombleholme adds to the pile of graduation and holiday cards he receives from grateful families, marking milestones that this care made possible.

“The most gratifying aspect of my career is to see these interventions not just save a child’s life, but to see the events of a lifetime that are made possible,” says Dr. Crombleholme. “There are a number of conditions that, if we’re able to intervene in utero, we’re able to change the course of the condition. That list gets longer every year. When parents receive a diagnosis, I want them to be aware that we can help.”

Before she was pregnant with Elisabeth, Anna had never heard of fetal surgery, or the emerging field of care surrounding it. It seemed beyond imagination. Now, it has given her and Jared a third child.

That’s her biggest takeaway for other families.

“What do I want other parents to know?” says Anna. “That there is hope.”