Encephalocele (US)

Sierra Yoder was having a normal pregnancy, but the 20-week prenatal ultrasound seemed to tell another story. The Yoders learned that their child — a boy to be named Bentley — had something called an encephalocele. Brain tissue was bulging out of an abnormal opening in his skull, unprotected by bone.

“They said he had zero chance of survival — ‘incompatible with life,’ they told us,” recalls Sierra. “I specifically remember asking is there any chance he could survive? They said no, that in the best-case scenario, he’s going to be a vegetable. They made it out like I was going to lose him at any point.”

With that knowledge, the Yoders decided to end the pregnancy. But at the 11th hour, Sierra changed her mind. It didn’t feel right — Bentley was moving and kicking and had a strong heartbeat.

So they kept going.

“All the way through, I just kept having the feeling that it’s going to be OK, it’s going be all right,” says Dustin, Bentley’s father.

Bentley Yoder encephaloceleThe rest of the pregnancy was uneventful. Sierra went into labor on Bentley’s due date — Halloween — and he was born on November 1. “They thought we might get an hour with him,” recalls Sierra. “We bought him one outfit.”

The hospital staff arranged hospice care. Still, Bentley survived — even thrived. At home, he drank his bottle, cooed, took a binkie and, at six months, blew kisses. “I couldn’t make him out any different from my other son,” says Sierra. “He was just a normal baby with something on the top of his head.”

Bentley's second chance part 1

A second opinion

But clearly they couldn’t continue this way. The encephalocele was covered only by skin and a thin membrane, and could easily get bumped or become infected. The weight of it was holding Bentley’s development back. And it was steadily growing, filling with fluid.

The neurosurgeon at their children’s hospital in Ohio was confident he could remove the encephalocele, seeing it as completely damaged tissue that could just be cut away. But Sierra had her doubts. “We said, what if he’s using what’s up there?”

At the Cleveland Clinic, where they got a second opinion, neurosurgeon Dr. Violette Recinos agreed there could possibly be living, working brain tissue in the encephalocele. She wasn’t fully comfortable with operating on Bentley. But she knew surgeons at Boston Children’s Hospital who might be.

‘This kid has a chance’

In Boston, the Yoders met with plastic surgeon Dr. John Meara, of the Cleft and Craniofacial Center.

Meara brought in neurosurgeon and Dr. Mark Proctor, his partner on encephalocele cases. Both thought they could safely operate on Bentley. But rather than cut the encephalocele off, they would preserve what they saw as functioning tissue. They planned to drain the fluid, expand Bentley’s skull, and gently put the brain back in.

“That was like a breath of fresh air, that they weren’t thinking, “this kid isn’t going to make it,’” says Sierra. “They were actually saying, ‘this kid has a chance.’”

It would be a delicate operation. “We’ve operated on a lot of encephaloceles in the past, but we’d never had one where there was so much brain tissue we felt was functional and had to be protected, put back in the cranium and covered up,” says Proctor. “It really presented a unique challenge.”

A major part of the challenge was that Bentley’s skull was very small, since most of his brain was outside it. It would need to be expanded.

Simulating Bentley’s surgery

Bentley's second chance part 2

Proctor and Meara turned to Boston Children’s Simulator Program (SIMPeds), which has begun offering customized, 3D-printed models of patients’ anatomy to clinicians around the hospital, based on patients’ actual CT and MRI scans.

For Bentley, SIMPeds produced several models for the surgeons to practice on, including models of his skull and the encephalocele, showing the contours of the brain tissue and the fluid surrounding it. Since the encephalocele kept growing, SIMPeds provided final models just a week before surgery.

Using the models, Meara and Proctor practiced making a series of vertical cuts in the skull, creating slats like those circling a barrel, all joined at the bottom. This would create more room for Bentley’s brain tissue.

“Young kids like this will generally fill in the bone gaps that are left,” explains Meara. “Having the models beforehand, we knew where to make the cuts before we got in there.”

The kindest cuts

It took about an hour for the anesthesia team to carefully prepare Bentley for surgery. The team had made extensive plans for keeping him stable throughout the operation. His head and the large encephalocele had to be positioned in such a way that he could be intubated to protect his airway and keep him ventilated throughout surgery. The team also took measures to limit blood loss.

“Bentley is a cute, social baby and came happily with me into the operating room,” says attending anesthesiologist Dr. Susan Goobie. “I sang Bentley to sleep with a lullaby, as I often do, while we gave him a strawberry-flavored anesthesia mask.”

Not counting the anesthesia time, Bentley’s operation lasted less than five hours. He remained stable throughout.

“We felt well practiced and prepared for all of the eventualities that we faced,” says Proctor. “We were able to save the vast majority of the brain and achieved an excellent closure.”

Bentley bounced back quickly once the anesthesia wore off. But two days later, he was limp, lethargic and uninterested in his binkie, wanting only to sleep. Not unexpectedly, fluid had accumulated in his brain, raising pressure inside his head.

The surgeons, prepared for this possibility, inserted a drain and later a permanent shunt to divert the brain fluid to his abdomen. Bentley perked up almost immediately.

“The more the shunt was working, the better he was starting to get,” says Sierra. “He looks at us way more now and is starting to be more interactive. He fights tooth and nail to stay awake now — he doesn’t want to miss anything.”

The road home

Bentley post op

Bentley’s future development is uncertain, since his brain tissue has always been structured very abnormally.

“We think it is unlikely that he’s going to develop as a perfectly normal child, but we feel that we have given him the best chance,” says Proctor. “I am certainly optimistic that he could have a rewarding life.”

His parents feel prepared whatever happens. Bentley is on some post-op medications but otherwise needs no special care and will be followed by neurologists in Ohio. Meara thinks he may not need further skull reconstruction, though that is a possibility.

“It’s two weeks after surgery and we’re going home and we’re comfortable going home,” says Sierra. “We’re waiting to see what he’ll give us, and we’ll be happy with anything he does give us. I’m more overwhelmed now than I was in the beginning, because everything has worked out so well.”

“It’s like winning the lottery but better than that,” says Dustin. “It’s an unbelievable feeling.”