A little more than year ago, Janet Bessette had never heard of a sacrococcygeal teratoma (SCT). But then her baby was diagnosed with the tumor 20 weeks into Janet’s first pregnancy. Now she easily ticks off facts about the condition, mentioning how common it is, the open fetal surgery to correct it, and the “mirror syndrome” that could have threatened Janet’s health.
Janet and her husband, M.J., are from Shawano, WI, more than 500 miles away from Cincinnati. In late December of 2010 after her 20-week ultrasound, Janet visited a specialist in Wisconsin for a closer look at a mass at the base of her unborn child’s tailbone. The specialist assessed the growth and called the Cincinnati Children's Fetal Care Center for consultation.
New Year in a New City
“That was on New Year’s Eve,” Janet says. As it turns out, she and M.J. would start the new year by traveling to Cincinnati for evaluation at the Fetal Care Center.
Upon arriving at the center, Janet had several tests including an MRI and ultrasound. For every patient the Fetal Care Center sees, a care team is formed, each member lending expertise in their specialty to result in the best possible outcome for the mother and baby. In the Bessettes’ case, their care team included a nurse coordinator, surgeon, maternal fetal medicine specialist, and cardiologist.
After Janet’s testing, she and M.J. met with their care team and were presented with the findings from the tests. They learned that a sacrococcygeal teratoma is a tumor made up of different types of tissues that form around the lower back and buttocks of a fetus. These tumors occur in one of about every 35,000-40,000 births. Oftentimes they aren’t diagnosed until after birth. But occasionally a fetus will experience rapid growth of the tumor prior to birth, which can cause complications for both the mother and baby. This was the case with Janet and M.J.’s baby, Kristina.
Watch and Wait
During their team meeting, the Bessettes also learned their treatment options. Open fetal surgery was a possibility for Janet and Kristina, but first their doctors wanted to keep an eye on the tumor to see how quickly it was growing. Its rapid growth can lead to heart failure for the baby, called hydrops, as the heart works increasingly harder to supply blood to the tumor. It also poses a risk for the mother’s health through what is called a maternal mirror syndrome, where the mother begins exhibiting symptoms similar to those of the sick fetus. If this sets in, the mother’s health can be in grave danger.
While Janet’s condition continued to be watched closely, she stayed in Cincinnati at the nearby Ronald McDonald House. “I had to have an ultrasound every day for a week, then every other day,” Janet says. After four weeks of being monitored, Kristina’s heart rate soared, and it was determined that the best option for the health of both Janet and Kristina was immediate surgery.
A Complex Surgery
On Jan. 25, 2011, at almost 25 weeks gestation, Janet went in for what is technically called an open fetal surgery for debulking of an SCT. The term “debulking” means to surgically remove part of a tumor. During an open fetal surgery, an incision is made in the mother’s abdomen similar to a Cesarean section. The uterus is then brought out, and an incision is made where the surgeons expose only the portion of the baby needing surgery. In Kristina’s case, that meant lifting out just her legs and the tumor. The rest of the baby stays in the uterus. That’s so the baby has the least amount of exposure possible, the Fetal Care Center says, which is safer for the baby.
Then the doctors perform the debulking, where they carefully resect — or remove — the tumor that is present on the outside of the baby. Kristina’s was three-fourths her size. After the tumor removal was complete, Kristina was placed back into her mother’s womb, the uterus was sewn up, and Janet’s incision was closed. The surgery is performed this way to allow the baby to grow and develop in utero for as long as possible. Janet immediately went on bed rest at University of Cincinnati Medical Center for the remainder of her pregnancy.
Welcome to the World!
Six weeks later, Kristina was born. She was ten weeks premature. The Fetal Care Center says that additional six weeks that Kristina was in the womb were crucial. The extra time allowed her to develop further and she was more stable at delivery.
Because she was premature, Kristina was put on a ventilator to help support her breathing, and then was sent to the neonatal intensive care unit (NICU) at Cincinnati Children’s, another of the three area hospitals providing services for the Fetal Care Center. During her time there, Kristina underwent another surgery to remove the portion of the tumor that was inside her body.
Sacrococcygeal teratomas can be malignant, so affected children are followed closely for the first three years of their lives. They undergo regular blood tests to monitor protein levels that can indicate the presence of cancer. The Bessettes return to Cincinnati Children’s once a year to check for regrowth of the tumor. Kristina will also be monitored for possible bladder or bowel obstruction, which is common in children who have had these tumors due to their location on the body. After three years, if Kristina has no signs of malignancy or regrowth, she will be considered a survivor.
Home, Sweet Home
After 58 days in the NICU, Kristina was finally strong enough to be cared for at home. The family headed back to Wisconsin. “We actually went home before my original due date of May 16,” Janet says.
Kristina is now an active 1-year-old, showing signs of walking, growing some hair, and giggling often. “Everyone at the Cincinnati Fetal Care Center was incredible,” Janet says. “Going through this has opened my eyes to how strong a baby can be. I can’t believe all Kristina has been through. It’s awe-inspiring.”