Open Fetal Surgery Corrects a Malformation in the Lungs

Kimberly Thorpe’s pregnancy with her first child was going along smoothly until an ultrasound at her 20-week checkup showed an abnormality. Kimberly was sent to a specialist who said that her baby − later named Ruthie − had a mass on her lungs that would need to be monitored.

Kimberly and her husband, Jimmy, would soon learn a lot about that mass, called a CPAM, or congenital pulmonary airway malformation. That’s its new name. It was previously termed a CCAM, or congenital cystic adenomatoid malformation. The new name defines more specifically where in the body the malformation occurs − in the lungs.

 “CPAM is a developmental malformation in how the lower respiratory tract and the fetal pulmonary beds are formed,” says Amy Ross, RN, MSN, CPN, a Cincinnati Children's Fetal Care Center nurse coordinator. “In Kimberly’s baby, they didn’t grow normally in the fetal chest. What it can do is take the form of a mass of solid or liquid tissue that doesn’t let anything move through it.” The result can be a cancerous or noncancerous mass, usually on one or more lobes of the lung.

A Challenging Journey

In many of these cases, the mass is small enough that the pregnancy can continue to term with no complications. Sometimes a CPAM will even shrink or disappear during the third trimester. But in Ruthie’s case, the mass grew to be so large that it was creating serious health complications. The mass was pushing other organs and tissues to the side, compressing blood vessels and making it harder for the heart to circulate blood, and causing the potential for heart failure, known as hydrops. Left untreated, Ruthie’s health would have been severely compromised.

Kimberly’s journey to a healthy delivery was a difficult one. Ruthie’s CPAM caused a dangerous excess of amniotic fluid (called polyhydramnios) so Kimberly underwent an amnioreduction to reduce the fluid. Ruthie also needed a shunt − a small, flexible, plastic tube that is surgically placed into the fluid in the baby’s lung and into the amniotic fluid in the uterus. This catheter-like device works to continuously drain the excess fluid from the mass to relieve the pressure on the heart. On top of that, Kimberly took steroids in hopes of shrinking the tumor and developing Ruthie’s lung tissue.

At 34 weeks pregnant on Oct. 4, 2011, Kimberly’s surgeon reassessed the large size of Ruthie’s mass and decided to refer the family to the Fetal Care Center. “I was ready to go anywhere at that point,” says Kimberly, who lives in Livingston, TN.

Ross, the Thorpes’ nurse coordinator, called Kimberly to schedule her appointment. Ross told her, “Pack like you’ll be staying through delivery.” Kimberly was relieved. “The last thing I wanted was to go into labor in Tennessee and have to be flown to Cincinnati,” she recalls. “I was happy to be going to a place where my baby would have a safe delivery.”

Treating CPAM

The Thorpes were at the Fetal Care Center the next day. Kimberly underwent a series of tests, including MRI, ultrasound, and fetal echocardiogram. She and Jimmy met with their entire care team: Ross, maternal fetal medicine specialists James Van Hook, MD, and Ronald Jaekle, MD, and surgeons Foong-Yen Lim, MD, and Sundeep Keswani, MD. They told the Thorpes that Ruthie’s best option was to undergo what’s called an EXIT procedure.

An EXIT procedure involves opening the mother’s abdomen and exposing only the part of the baby that needs surgery, while keeping the baby attached to the placenta. The surgery is performed, and then the baby is delivered as it would be during a routine C-section. The EXIT procedure is done to allow the baby to continue receiving its blood and oxygen supply from the umbilical cord, giving the surgeons more time to safely complete the procedure with better outcomes for the baby.

Kimberly’s procedure was an EXIT-to-resection, meaning that the surgeons resected − or cut out and removed − the mass from Ruthie’s lung prior to delivery. To do this, the surgeons opened Kimberly’s abdomen while she was sedated, then exposed Ruthie’s head and right arm. They made an incision from her right nipple around her side and to the middle of her back, allowing them to remove the lung mass. They also inserted a breathing tube (called intubation) that would let Ruthie breathe more easily upon delivery while the doctors evaluated her status. Ruthie’s incision was closed and then she was delivered. All in all, the surgery took about 2½ hours.

Finding Comfort in Confidence

“When I first heard about the procedure, I thought it was pretty amazing,” Kimberly says. “It sounded like it was from a science fiction movie. But my care team explained it with such confidence that I was pretty comfortable with it.”

Since 2004, the Fetal Care Center has evaluated more than 140 patients for fetal CPAM. The condition is rare, and typically the size of the lung mass is relatively small. “Ruthie’s mass was one of the biggest we’d ever seen,” says Ross. CPAM size is evaluated with a volume ratio. Ross says the ratio number becomes alarming when it is greater than 1.6. Ruthie’s was 4.33.

“Generally babies don’t suffer long-term effects from this condition,” Ross says. Ruthie lost the right upper and right middle lobes of her lung during the resection, but children’s lungs regenerate until they’re about 8 or 9 years old. So, Ross says, Ruthie’s left lung will compensate and get bigger.

Originally the Thorpes were told to expect Ruthie to be in the neonatal intensive care unit (NICU) at Cincinnati Children’s for anywhere from six weeks to six months while she developed enough to be cared for at home. They were beyond thrilled when Ruthie was doing so well that she was released on Nov. 14, 2011, at just 5 weeks old.

Happy Ending

Ruthie is now a happy seven year old with a big heart. She is a cheerleader, dancer, reader, and friend. At her last check up with Dr. Lim in 2014 her lungs were healing nicely. She runs, jumps, plays, and dances, and her pediatrician says she is a picture of health.

Kimberly is pleased with the high-quality care her entire family received and would highly recommend Cincinnati Children’s to anyone. “Literally everyone, from the surgical director to the billing department, was amazing. That has not been our experience at other hospitals."

“I felt like we weren’t just going through this by ourselves. We had a whole team of people who were invested in what was going on with Ruthie. I can’t imagine a better team to walk with us through this experience.”

Updated: February 2019