At a 20-week ultrasound Lindsey and Jeremy Walley were excited to learn the gender of their first-born child.
Their elation that it was a girl was met by a sudden quiet and serious look from the ultrasound technician, who then whisked them to another room for a meeting with their obstetrician-gynecologist. The couple knew something must be wrong.
That’s when they learned about the omphalocele — a birth defect in which the intestines and other abdominal organs grow outside the abdomen through a hole in the belly. The organs are covered only by a thin, translucent membrane. The Walley’s baby had a giant omphalocele containing her liver and intestines. A unique collaboration between an orthotics company and a dedicated doctor gave the little girl and her parents a reason to smile.
“We had never heard of it,” Lindsey said.
“At the time we were blown away. No one in our family had ever had anything like this, and we were the first ones to have a baby who had a birth defect.”
About 775 babies in the United States are born each year with an omphalocele, accounting for about one out of every 5,386 births, according to the Centers for Disease Control and Prevention.
The Walleys, who live in Cookeville, Tennessee, were referred to Monroe Carell Jr. Children’s Hospital at Vanderbilt where Lindsey underwent detailed anatomic ultrasounds and scans of the baby, whom they named Hailey, to help define the extent of the omphalocele.
At 31 weeks into her pregnancy, Lindsey started having contractions. Hailey had dropped into the birth canal. After a weekend in the hospital and medicine to stop the contractions, Lindsey went home, only to return 24 hours later when her water broke.
Because of the risk to the omphalocele, Hailey was delivered via cesarean section Jan. 11, 2017, at 32 weeks and quickly taken away to the Neonatal Intensive Care Unit.
Jeremy and Lindsey had to wait four days before they could finally hold her for the first time.
“I was very scared to hold her,” Lindsey said. “I was scared to see the omphalocele when she was born and to see her on the ventilator and with IVs. That’s not how you picture seeing your first-born child.”
Due to her underdeveloped lungs and added pressure from the omphalocele, Hailey was put on a ventilator and eventually graduated to oxygen through a continuous positive airway pressure (CPAP) machine for preemies.
“That was one of our largest problems — the underdevelopment of her lungs due to prematurity and being on the ventilator and the CPAP. Besides the omphalocele, that was one of our major obstacles to overcome,” Jeremy said.
To help protect the omphalocele after birth and prepare for her eventual surgery, Harold “Bo” Lovvorn III, MD, associate professor of Pediatrics and Pediatric Surgery, painted the thin sack with a cream called silvadene, which is typically used to help burn victims heal. Over time, Hailey’s skin would begin to grow and as she grew, doctors could start gently pushing the sack into the abdominal domain.
“If we as physicians don’t take a proactive approach to contain the sack, it will mushroom, and there is no way to put it back in there,” Lovvorn said.
After 64 days in the NICU, Hailey was finally able to go home on oxygen support. The Walleys had wondered if there was a way to cast the ‘O,’ as her parents dubbed it. They didn’t know how they would transport Hailey in a car seat without a seat belt rubbing against the sack. Hailey began in a special recombinant car seat then graduated to a Jefferson car seat, which reroutes the straps around her belly.
The ‘O’ protector
While omphaloceles are rarely fatal, they often require multiple surgeries as the baby grows to properly place the organs back into the abdominal cavity. Worried about risk of infection and possible rupture of the “O,” the new parents wondered about a way to protect it and allow Hailey to be a typical baby.
Lovvorn, in his research, came across a new innovative and experimental orthotic device, a brace of sorts that covers the omphalocele and compresses it over time so that only one repair surgery is required. The Walley’s journey had already been a long haul, as they worried about the omphalocele but also lung development issues Hailey had due to prematurity.
“We had become aware of a prosthetic device made in Boston that would keep the sack from growing larger, with the concept that the baby will grow around the sack, but also that you can gradually compress and reduce the contents back inside,” said Lovvorn.
The Walleys agreed to try the device for their little girl.
On a follow-up visit with Lovvorn and after consults with Kaitlynn Pung, a resident at Nashville-based Bulow Orthotics and Prosthetic Solutions, the Walleys received a custom orthosis for Hailey when she was about 4 months old. Because Boston Orthotic and Prosthetic fabricated the original brace for a previous omphalocele patient, they fabricated a custom device for Hailey based on measurements and a CAD scan obtained by Pung.
“We got in touch with Boston O and P and the person who handled that case, and they were eager to help,” said Pung. “I had to make major brace design adjustments in the first couple of months as the ‘O’ began to shrink. I also attended her bimonthly follow-up appointments with Dr. Lovvorn at which time I took progress measurements and determined if we were still moving in the right direction. This case really highlights how important it is to have interdisciplinary care and to work with a patient’s physician to ensure the patient receives the best care.”
The Walleys felt more comfortable once Hailey had the brace. They started seeing immediate results.
“It was amazing. The ‘O’ changed shape. Every time I took it off it was a different shape,” Lindsey said. “It started as an oval shape from head to toe and this device was trying to compress it in. (At visits) Lovvorn would come in and manipulate it and press it back down to the abdomen to see if she could handle it.”
Hailey could tolerate more tummy time, aided by a special O-shaped pillow her mom created out of memory foam so it had a hole for where her brace would sit. Throughout the process, Pung continued to adjust the device to keep the sack compressed.
“Hailey needed a device that could compress her omphalocele while allowing her to just be a baby without limitations,” Pung said.
In November 2017, Lovvorn performed a successful repair surgery on Hailey, putting her organs back into her abdominal cavity and closing it up.
Jeremy said that initially they thought it would be 18-24 months before surgery could be done, but the device allowed the surgery to happen sooner. “The device helped us to do this in a one-and-done surgery versus multiple surgeries,” he said. “We were very happy and have been impressed with the care along the way. We are extremely thankful for the people here —everyone from the nurses who took care of her, to the physicians, to the surgeon. We couldn’t have had a better surgeon for her.”
At a recent visit to Children’s Hospital, Hailey waves around a Mickey Mouse doll, putting it in her mouth as she squirms in the lap of her dad. She’s clearly eager to be on the go, and finds her way to her mother.
Hailey, wearing a blue printed dress and white leggings, has had a long journey in the first year and a half of life.
Lovvorn says Children’s Hospital is uniquely positioned to walk this journey with families and handle complex case due to the specialty care it offers and he hopes Hailey’s successful outcome can help other children born with omphaloceles.
“We have expertise in prenatal care, both diagnostic and therapeutic, and our postnatal care is excellent in neonatology and all the supportive services. We have a group of critically-thinking surgeons who are experts and well-read and aware of innovative techniques out on the horizon and who are trying to stay current with progress. This brace is not a widely known technique. It’s new and emerging, but if one reads and stays current on the literature, eventually you’ll find something that works.”