It’s a good thing Rhett Bausmith was child No. 4.
It meant his mom, Crystal Bausmith, didn’t panic when she found out something was really wrong with her son. Bausmith recalls that they had no warning there were going to be problems. She was in labor for 10 hours.
“We knew it wasn’t right, but we weren’t quite sure what.”
After the birth, a neurologist came to consult with Crystal and Timothy Bausmith to explain what was wrong. Their son’s head was misshapen because he had craniosynostosis, a birth defect in which the bones in a baby’s skull join together too early. He would need to be referred to the Medical University of South Carolina.
MUSC's Ramin Eskandari, M.D., a pediatric neurosurgeon, and Jason Ulm, M.D., a plastic surgeon, joined forces to figure out the best way to help Rhett. Imaging showed his skull had not one, but two sutures (fibrous joints on babies' skulls that turn into bone) that had prematurely closed.
Often in these cases it’s just one suture that closes, but Rhett’s case was rare, Ulm says. The two sutures that had fused were affecting brain growth and the formation of Rhett’s face. Already one section of the skull that encases the eye, the orbital rim, had pulled back. Ulm discussed options with colleagues in Boston. “We usually don’t see something this severe.”
Ulm and Eskandari requested MUSC’s bioprinting labs to take Rhett’s scans and make 3-D skulls. Ulm says only a handful of medical centers in the country are using bioprinting technology to create replicas of patients’ skulls to better plan surgical procedures. It also opens a new frontier in the training of residents and in creating a powerful tool to educate families about what can be very complex surgical cases.
Eskandari, a new father himself, says he understands how hard it is for parents to get bad news. In severe cases, it can be hard for families, who often are in shock, to understand the complexity of the surgery. “Even with Rhett, where you could see something really was wrong, it’s not until now, when there’s been a significant correction, where his mom says, ‘Oh my God, my baby was messed up.’”
It also lets him do a better job of obtaining informed consent, which prepares families for the upcoming treatment and its possible risks. “No matter how much you talk to a family about what you’re going to do as a surgeon, if you ask them even hours later, they have very little idea,” he says.
“Real-life visuals help with the process of getting better informed consent and lead to better questions from the patient or parent.”
Prepping for Surgery
The parents, especially when they heard how many cuts would need to be made into their son’s skull, had lots of questions.
Rhett, born May 1, 2015, was slotted for surgery that August. Ulm and Eskandari decided to do a two-stage repair. Doctors like to wait longer to do surgery to allow for more bone growth in the skull so there’s enough bone to perform reconstruction, but Rhett’s condition was progressing quickly. They needed to relieve pressure on his brain so they opted to go ahead with the first surgery.
Eskandari holds up the 3-D skull. “On this model, you can see where this side is open and this side has been fused. In this case when things fuse like this too early, as the brain is trying to grow and the skull tries to expand, the side that is fused can’t grow. As the brain continues to try to grow, everything gets pulled over to one side.”
Rhett’s eye had started to pull up – a condition called a harlequin eye – and a part of the forehead area was pushing forward. The first step was to take away bone from the sutures that had prematurely fused. “That’s when this comes really in handy,” he says, turning the 3-D model in his hand. “We used these to plan the cuts.”
Since Rhett’s brain needed room to expand, the surgeons also did a technique called a barrel stave osteotomy. “The barrel stave sort of describes it. You make slits in the bone – it’s like the Blooming Onion from Outback,” he said, explaining a crisscrossing of cuts in the skull.
“The cuts give it the extra ability to grow faster. By relieving the pressure from the bone by doing the barrel staves, it forces the brain to grow in the direction you want it to grow faster.”
Following surgery, the doctors also opted to do helmet therapy to really force growth in the directions they wanted. “He’s gotten a good correction,” says Eskandari. “His brain growth is significant in the early stages and the cuts and the helmet all worked together to get a good reconstruction.”
When Rhett is about 18 months old, they’ll perform a second surgery to remove the frontal bone and reconstruct the forehead and eye area. Because the orbits or eye sockets have really been affected, they will do an orbital osteotomy. Ulm, a father of three, is glad to see the progress Rhett already has made. It’s one of the parts of his job he enjoys the most.
“It’s amazing the difference you can make in kids and see the immediate and profound change that you can make in their appearance and development at that age.”
Training of the future
Ulm says he and Eskandari often work complicated cases such as this as a team. “We’re both young, fresh and eager to improve the methods by which we treat babies with these conditions to make surgery safer and more effective.”
Eskandari agrees, adding that Ulm is so laid back, it’s hard to get a rise out of him. “He’s so even keel and he’s so cerebral. He’s very meticulous and very thoughtful.”
Their differing backgrounds as a neurosurgeon and a plastic surgeon allow them different perspectives. They capitalize on each other’s expertise and both embrace using the new 3-D bioprinting technology, Eskandari says.
Ulm agrees, adding that the technology helps plastic surgeons better plan where to make the cuts. “We can see what parts of the skull we want to move and how we can move those pieces around, sort of like a jigsaw puzzle, to reshape the head into a more normal position. We can draw on it and then simulate the cuts like you would in the operating room. The idea is to decrease the operative time because you have already simulated the operation before in the lab.”
Eskandari says the next step with the 3-D skulls is to get drills that can be used with them to better train residents.
“That way all the residents can go through the operation and really see where the burr holes, which are the initial holes to enter the skull, go. Knowing that stuff beforehand is very beneficial. It allows residents to do almost the same surgery – the dimensions are the same.”
A long-term goal is to not only print the skull but the dura or soft tissue underneath as well. Eventually they could replicate the whole brain including the blood vessels and mimic an aneurysm, for example. “So literally, you could do the whole approach on an adult skull to treat an aneurysm. You literally have a mock version of your surgery with absolutely no risk.”
The advantage of that is like comparing a scratch-and-sniff version of a muffin to eating a muffin, he says.
“From a surgeon’s standpoint to be able to manually do that the night before – not from a 3-D scan on a computer – but really manually do it -- that muscle memory is very important in surgery. The potential benefit from a training standpoint and in terms of what that does for patient safety is huge.”
Neurosurgery is very difficult to simulate so the technology advances are key in his field, he says. “The brain is so complicated, and there are so many parts and angles to it, and the folds and the blood vessels, it’s just such a complex thing. And the spine is just a completely different beast.”
The bottom line is better patient care, Eskandari says.
“When you see the results years later, and the child just looks so much better and you know what you’ve done is not just cosmetic, it’s really a life changer. Some of these kids would look extremely abnormal as children and young adults. That already puts them in a very precarious situation in this day and age with so much emphasis on looks and image.”
The Bausmiths already are fans of the new technology.
Rhett’s mom, Crystal, says having the 3-D skull was very helpful, especially in understanding the eyes and how the orbits were misshapen. It also helped when the doctors were explaining what they were going to do. “We could hold it and look at it and see it,” she says. “I kept showing the kids and my family. It was his little head. It’s really cute. Especially if you’re a visual person, you’ve got to see it.”
Her husband, Timothy, an engineer, also liked having it. “When you have the skull, it helps you get your head wrapped around it.”
Fortunately, Rhett is doing well, and they’ve noticed no developmental delays so far. His mom says the difference the one surgery made has been amazing. “It’s very reassuring. As messed up as his head was, we were surprised how well he’s doing.”
Rhett’s dad agrees, adding that they were excited to be able to take one of the skulls home.
“Hopefully all he will have of this one day is a cool scar. That will be the only thing and maybe a 3-D model skull.”