EXIT Procedure Allows Will to Breathe
When Kristin Hileman was 29 weeks pregnant, a routine prenatal checkup found that her gestational measurement was larger than it should have been. An ultrasound showed a mass on her baby’s neck the size of a small orange. Kristin was immediately referred to a specialist who diagnosed the mass as a cervical teratoma.
A cervical teratoma is a tumor that grows on the neck of a baby in utero. It can grow so large that it obstructs the airway of the baby, making it impossible for the child to breathe at birth. This type of tumor is rare, affecting approximately one in 16,000 babies.
Kristin and her husband, Bill, of Columbus, Oh, soon learned that the best chance for their baby’s survival would be for Kristin to undergo what is called an EXIT procedure (ex-utero intrapartum treatment). This procedure combines a surgery on the baby’s airway with a C-section delivery. They were referred to the Cincinnati Children's Fetal Care Center and quickly made arrangements for what turned out to be a lengthy stay.
Help From Afar
The Hilemans were able to take advantage of a concierge service through Guest Services at Cincinnati Children’s, one of three local hospitals that makes up the Fetal Care Center. This service helps out-of-town families make travel arrangements such as lodging and transportation. That freed up the Hilemans to coordinate care for daughter Brynn, who was 3 at the time.
Upon arriving at the Fetal Care Center on Jan. 17, 2010, Kristin underwent standard prenatal tests including an MRI and ultrasound. She and Bill later met with their care team, consisting of a maternal fetal medicine specialist, fetal surgeon, nurse coordinator, and others who would be involved in the care of Kristin and her baby. They were presented with all of the facts of their case and learned what to expect over the coming weeks until the baby’s delivery.
“We had several team meetings with our doctors,” Kristin says. “It was very helpful. Any time we had an MRI they’d go over all of the results with us.” They not only learned what to expect before their baby’s birth, but also what they would face after delivery and the special needs their baby might have due to complications from the tumor.
“It’s scary to go through something like this,” Kristin says. “There’s a risk for the mom as well as the baby. They really made us feel at home and took great care of us.”
A Complex Procedure
On Feb. 10, at 33 weeks pregnant, Kristin went in for the surgery and delivery. Her procedure, called an EXIT-to-airway, involved putting Kristin under general anesthesia to relax her uterus. Then the surgeons made an incision in Kristin’s abdomen as is done with a standard C-section. Next they lifted out the baby’s head and shoulders while the baby remained attached to Kristin through the umbilical cord. This allows the doctors more time to work on the airway, since the baby is still being supported by the mother.
In Kristin’s case, the doctors needed to create an artificial airway for her baby because the tumor was compressing the natural airway. This involved intubating the baby − inserting a tube into the throat − so that he could breathe once he was fully delivered. The tumor would later be removed after birth during a separate surgery. After they intubated him, the doctors delivered him as during a typical C-section.
A Long Road Ahead
The procedure was a success, and the Hilemans welcomed baby William into their family. The surgery allowed him to breathe through an endotracheal tube. But he had a long way to go. He was so weak, in fact, that Bill’s family drove through a snow storm from Michigan to meet baby Will because they weren’t sure he would make it.
“He was so very sick,” Kristin says of Will, who had immediately been taken to the Newborn Intensive Care Unit (NICU) after his birth. “He needed surgery to remove the tumor from his neck. The operating room was right across from his pod in the NICU, but we didn’t think he could even be moved there because he was so fragile.” Finally, there was a six-hour period of time where Will looked well enough to tolerate the surgery. “It was the longest wait of my life,” says Kristin.
Will proved strong, surviving the surgery and continuing to grow and develop during his stay in the NICU. But his list of challenges was lengthy. Tests showed his tumor was cancerous. His prematurity and prenatal condition combined to present medical challenges including a brain hemorrhage, a diseased thyroid that had to be removed, and a pyloric stenosis, where the contents of his stomach were blocked from emptying into his small intestine.
But Will persevered, and almost three months after he was born, he was finally well enough to be transferred to Nationwide Children’s in the Hilemans’ hometown of Columbus. He stayed there for another month to complete his transition to home care.
Progress amidst Struggles
By age two, Will had become an active toddler. In a world full of feeding tubes, breathing tubes, home nursing care, and endless doctor visits, Kristin and Bill saw the potential in their son. He loved to explore, read, and play with toys. He could drink from a cup and follow directions. Every time they were at a checkup, the doctors would say, “Look how much he can do!”
Will was soon weaned from his ventilator, and occupational therapy and physical therapy continued to challenge and strengthen him. One of the Hileman’s biggest celebrations was in July of 2016 when Will was decannulated. It was not certain whether he would be able to have his tracheostomy reversed, but after much prayer and patience, the day came, and the process was successful. His stoma is now completely closed.
Still Going Strong
Today, as the Hileman’s celebrate Will’s ninth birthday, they are inspired by his tenacity to keep moving forward and accomplish his goals. Will has made great progress with drinking water, and now he is attempting to learn to eat by mouth.
Will is non-verbal, but he does have some spoken words and can use some sign language. He is becoming skilled at expressing his wants through a communication device on his iPad, or often he just does things for himself. “He is very independent,” Kristin says, “which I love.”
Full of energy, Will participates in cub scouts and enjoys doing outdoor activities. He also participates in the Miracle League baseball organization. “We are so grateful,” Kristin says, “for the medical advancements and care that have helped Will be the strong boy he is today!”
Kristin feels strongly that Will would not have survived birth without the EXIT procedure. And she and Bill are thankful that they found the Fetal Care Center. “The care we got from the Fetal Care Center was above and beyond our expectations,” Kristin recalls. “We are so happy to have our little boy here with us. We would be lost without him.”
Updated: May 2019