Kelly and Dan — both registered nurses — researched the condition online and found a lot of information from Children’s Hospital of Philadelphia’s Center for Fetal Diagnosis & Treatment (CFDT). Even the educational materials provided by their obstetrician at the hospital near their home in Cleveland had been created by the CFDT.
Their OB’s plan was to monitor the tumor’s growth for the rest of the pregnancy and have a pediatric surgeon remove the tumor after birth. But the tumor grew rapidly — from 3 centimeters in diameter to 12 centimeters in diameter in just four weeks — and was taking blood from the fetus’ circulation, causing heart failure (known as hydrops). Waiting was no longer an option.
Given how rare this situation was, their home hospital wasn’t equipped to offer fetal surgery, so Kelly and Dan turned to a place they knew could — the team that had shown up again and again in all their early research. The CFDT is led by N. Scott Adzick, MD, MMM, a world-renowned pioneer in the diagnosis and treatment of birth defects, who performed the world’s first successful open fetal surgery for SCT in 1996. Since then, only 15 open fetal surgeries for SCT have been performed worldwide, nine of which have been performed by Dr. Adzick.
The couple requested a referral, arranged for family members to look after the twins, and flew out to Philadelphia the next day. After a comprehensive all-day evaluation — including fetal MRI, fetal echocardiography and fetal ultrasound — Adzick, maternal-fetal medicine specialist Julie Moldenhauer, MD, and other members of the CFDT team met with Kelly and Dan to discuss their options, which were few.
Acting fast to help both mom and baby
Kelly had begun to exhibit signs of maternal mirror syndrome, taking on symptoms of the sick fetus’ heart failure such as the accumulation of excess fluid in her tissues. The declining health of both mother and fetus made the family candidates for open fetal surgery, in which the uterus is opened, the tumor removed and the fetus put back in the womb to continue the pregnancy for as long as possible.
Repairing Birth Defects in the Womb
Adzick and his team’s experience with these cases told them they needed to move quickly in order to save the baby, so they recommended the fetal surgery take place first thing the next day. Their instincts were right. During the operation they discovered the tumor had ruptured in two places sometime overnight and as a consequence, the fetus had already lost a lot of blood and was very anemic.
“Had Kelly not been the first surgery of the day, the baby most likely wouldn’t have made it,” Dan says.
That wasn’t the only complication the clinical team experienced during the surgery. In order to remove the massive tumor, the team had to make a larger-than-usual incision in Kelly’s uterus. As a result, the amniotic membranes separated from the uterine wall. Adzick repaired them using a special technique, but it put Kelly at increased risk for preterm delivery.
Then, after successfully removing the tumor — which was nearly the size of the fetus — the fetal heart stopped. Through the umbilical cord, the team administered emergency medicine and blood transfusions, and Dr. Adzick did cardiac compressions under fetal echocardiographic monitoring, but the team began to worry that the fetus would have to be delivered right then and there — at just 25 weeks. Fortunately, the team was able to stabilize the fetus and close the uterus, preventing an emergency delivery.
“It was a very, very, very difficult operation; about as tough of an operation as I’ve done,” recalls Adzick.
Despite the difficulties during surgery, the procedure’s success was immediately evident. A fetal echocardiogram later that day showed the baby’s heart was already recovering so that the signs of heart failure could resolve. And despite such a traumatic surgery and cardiac arrest, a fetal MRI two weeks post-op showed no evidence of brain injury.
Waiting for Maeve
Because of the membrane separation, Kelly remained on bed rest in CHOP’s Garbose Family Special Delivery Unit for seven weeks, under the care of Moldenhauer and team. Maeve was born at 32 weeks’ gestation weighing 4 lbs., 4 ounces.
A week and a half after birth Maeve had another surgery to remove the remaining pelvic component of the tumor and reconstruct her bottom. She spent nearly five weeks in the Newborn/Infant Intensive Care Unit (N/IICU) recovering. Thanks to an organization called Hosts for Hospitals, Kelly and Dan were able to stay in the home of a wonderful family nearby in New Jersey and be by Maeve’s side every day. Hosts for Hospitals provides families who have come to Philadelphia for medical care free or low-cost lodging at the homes of volunteer host families.
After returning home, Maeve had regular follow-up appointments at a hospital there. So far, no news is good news. Her appointments have been uneventful, and Maeve is expected to have a happy, healthy childhood.
“It is miraculous,” says Adzick.
Stubborn and silly
These days, Maeve is busy developing her own personality. She loves her older brothers, dogs, Bubble Guppies, and going to the library.
"She is both stubborn and silly," says Kelly. "She is running, singing, saying her ABCs, and her language has just exploded over the past two months. We will be registering her for preschool for the 2018 school year in January, which is kind of hard to believe!"
While Maeve continues to hit one milestone after another, the family is looking forward to the annual Fetal Family Reunion and the chance to reunite with the clinical team that saved Maeve’s life.
Originally posted: September 2015
Updated: December 2017