Chattanooga surgeon pioneers new procedure to help infant stricken with rare condition

Chattanooga surgeon pioneers new procedure to help infant stricken with rare condition

May 20th, 2014 by Kate Harrison Belz in Local Regional News

WHERE TO WATCH

"The Doctors" episode featuring Chattanooga craniofacial surgeon Dr. Larry Sargent will air Wednesday, May 21 on WTVC, Channel 9 at at 10 a.m. ET.

Dr. Larry Sargent holds a 3D model of Layla Shook's clover-shaped skull in his office. The model helped him decide how to proceed with Layla's surgery, which took place when she was just 24 days old.

Photo by Kate Harrison Belz/Times Free Press.

Layla before surgery.

Photo by Contributed Photo/Times Free Press.

Layla after surgery.

Photo by Contributed Photo/Times Free Press.

Dr. Larry Sargent and a team with the Tennessee Craniofacial Foundation perform an operation on Layla.

Photo by Contributed Photo/Times Free Press.

Dr. Larry Sargent shows two 3D models of Layla Shook's skull at different stages. The one to the left is a model made from a scan of her clover-shaped skull just a few days after her birth. The one to the right is of the baby's skull several months after reconstructive surgery.

Photo by Kate Harrison Belz/Times Free Press.

Peering into the ultrasound screen four months into pregnancy, parents can just begin to detect their little one's tiny facial features. A little nose. Eye sockets. the curve of a lip, if you look closely enough.

But when first-time Ringgold, Ga., parents Brian and Lisa Shook saw their daughter on the screen four months in, they didn't need a magnifying glass to see that something was wrong.

"You could see her skull wasn't a round shape," the mother remembers. "It was actually more like a clover."

As the pregnancy progressed, the Shooks knew that their baby was going to need significant surgery. But it wasn't until shortly after Baby Layla was born in 2011 - when reconstructive surgeon Dr. Larry Sargent visited them in the neonatal intensive care unit - that they realized just what they were in for.

The doctor said surgery needed to be done within a few weeks, and the severity of the baby's condition meant that he was going to try some surgical techniques that had never been used before.

Sargent's pioneering work in Layla's case has since attracted national attention, and Wednesday morning their story will be featured on the Emmy Award-winning ABC medical talk show "The Doctors."

"She had a very unusual and rare deformity," explains Sargent, who is the founder of the Tennessee Craniofacial Center at Children's Hospital at Erlanger. "Left untreated, it results in death."

Only 130 cases of "cloverleaf skull," as Layla's condition is called, have been reported in medical literature, Sargent said. Layla's condition is associated with a diagnosis called Apert's syndrome, which is characterized by bones that fuse too early.

As growth plates in the baby's head are constricted, the head bulges out in three distinct lobes, much like a cloverleaf. As the baby's brain and eyes grow, the pressure builds.

Sargent had encountered cases of cloverleaf skull before, but because Layla was so young and tiny, he knew he needed to be innovative.

Beforehand, he took Layla's cranial scans and a 3D printer to create to-scale models of Layla's skull. Using those, he strategized about how to reconstruct "a perfect fit," and to devise a method to safely stretch the infant's skull as she grew.

Layla was just 24 days old when she went into surgery. For at least four hours, Sargent worked with neurosurgeon Dr. Tim Strait to reshape the front of the baby's head. Sargent used plates from Layla's clover-shaped skull to fashion an entirely new forehead and brow.

The team then affixed small metal screwlike rods, called "distractors," on the sides of the skull. By slightly turning tiny knobs that protruded from the skin, Layla's parents could help to gently stretch the baby's skull a millimeter a day.

When her parents finally got to see her after surgery, she looked like a completely different baby.

"I loved Layla the way she looked before, for those 24 days I got to know her," her mom says. "But I knew there was no way to keep her like that."

The distractors ended up gradually stretching Layla's skull 2 and a half inches - "an enormous amount for an infant," explains Sargent. Because of the amount of space created, Layla was spared at least three additional operations, he says.

Sargent's innovation soon gained wide attention, becoming the cover story for a peer-reviewed journal of plastic surgery.

Using methods like the ones he used for Layla, he said, more children could safely undergo similar procedures at earlier ages - improving their outlook for brain development.

It wasn't long before Sargent got a call from "The Doctors." They wanted him on the show to hear about his new approach to the difficult procedure.

A crew came to Chattanooga to film a surgery Sargent performed on Layla, then flew Sargent out to Los Angeles this March to interview him in front of a live audience.

"You keep your descriptions on a level that's understandable," said Sargent. "It's a really educational show and a great way for the public to be informed about medical advances."

Though Layla - now 2 and a half years old - has needed more procedures to expand the back of her skull, she has shown remarkable progress, her mom says.

She's been on a feeding tube, but she's now trying new foods. She's learning to use a little walker. The once-silent toddler now jabbers nonstop. She'll start school next year.

The family calls Sargent "our hero," and is excited to share his work and their journey with a national audience.

"At first, we didn't want anybody to be with us - to see the fear we had," Shook says. "But now we want to share her and her story. We want parents who are in this kind of situation to see that they can hang in there. We love Layla the way she is. She is beautiful."

Contact staff writer Kate Harrison at kharrison @timesfreepress.com or 423-757-6673.