Dustin and Sierra Yoder, from Ohio, US, knew that their baby, named Bentley, had a rare condition in which his brain was growing outside of his skull.

Sierra Yoder said doctors told them that their son would not live long past his birth. If he didn't die, she said doctors warned, he would live with no cognitive function.

She said she and her husband were urged to consider abortion - and they did - but the night before the procedure, they chose to continue the pregnancy.

Baby Bentley wasn't expected to live long after birth. Photo / YouTube / Boston Children's Hospital
Baby Bentley wasn't expected to live long after birth. Photo / YouTube / Boston Children's Hospital

"We were excited to meet him, even if it was only for an hour," Sierra Yoder told The Washington Post. "We were just relieved he made it that far and we would get to meet him, living and breathing."


Now 7 months old, he is alive and alert after surgeons at Boston Children's Hospital put together a plan for what one called the "granddaddy" of cases to place his brain back into his cranium.

"It was a life-saving procedure," Mark Proctor, neurosurgeon in chief at Boston Children's, told The Post. "But it will not restore a normal life."

Indeed, Bentley will have struggles.

Encephalocele is a rare congenital disorder in which a baby's brain herniates from the skull in the womb and the bones do not properly form around it. The portion of the brain that sits outside the skull is usually covered by thin skin or membranes, according to the National Organization of Rare Disorders.

The Centers for Disease Control and Prevention states that each year in the United States, about 375 babies - or one out of every 10,000 - are born with the malformation, which can cause mental and physical issues, vision problems, seizures and muscle weakness in the arms and legs. The cause is not known.

The family had been told by doctors that Bentley would have no brain function, but he quickly proved them wrong. Photo / YouTube / Boston Children's Hospital
The family had been told by doctors that Bentley would have no brain function, but he quickly proved them wrong. Photo / YouTube / Boston Children's Hospital

Like most neurological conditions, however, experts say there's a spectrum from mild to moderate to severe cases such as Bentley's.

Alan Cohen, head of pediatric neurosurgery at Johns Hopkins Hospital, said the herniated sac may contain brain, brain membranes and brain fluid. When the sac is filled with non-functional brain tissue, he said, a surgeon typically removes it. But when it contains vital brain structures, he said, the surgeon must find a way to put the brain back into the skull.

A complicated surgery

At about 4 weeks, Bentley saw a specialist at Nationwide Children's Hospital in Columbus. After reviewing his MRI, Sierra Yoder said, a neurosurgeon told her that Bentley's brain - both the portion inside his skull and out - was too damaged and he would not survive, "so at that point, we had no confidence."

That's when the family went to Boston Children's Hospital. Dustin and Sierra took Bentley to meet the surgical team, which sees a few severe encephalocele cases each year. Proctor, the neurosurgeon, and John Meara, Boston Children's plastic surgeon in chief, formed a surgical strategy.

The baby, 5 months old at the time, had a pouch protruding from his skull that held a significant portion of his brain - the part that controls motor function and problem solving, and the part that controls vision. Unlike many encephalocele cases, Bentley's mass, which was now buried beneath a blanket of golden curls, could not be removed; he was using it.

His Boston Children's surgical team used 3D-printed models to plan out and practice their maneuvers.

On May 24, the team went to work. They shaved his curls and cut back the skin and membranes covering his brain. The portion outside his skull included a smaller part of the right frontal lobe and a larger part of the right occipital lobe, the neurosurgeon said.

The doctors drained cerebrospinal fluid from Bentley's brain. Then they made the cuts in the cranium and eased the brain back into his head, Meara said, adding that the team took leftover bone from the cuts and criss-crossed them over the top of the head to close the gap.

Five hours later, Bentley was in recovery.

In the waiting room, his family - parents, grandparents and big brother, Beau - had locked eyes on the surgeons who were walking their way. "When we saw them, our hearts dropped," Sierra Yoder said, explaining that they had been told the surgery would take much longer. "We panicked."

They went to see a bandaged-up Bentley, clad in a tiny hospital gown covered in cartoon tigers and lying in a hospital crib. "He was awake; he was looking at us, " Sierra Yoder said. "He wasn't cranky. He was just lying there, taking it all in."

Almost a month since Bentley brain surgery, his mother said, he is now able to hold up his head. He's eating. He's smiling. He's jabbering.

"His hair is growing back in," Sierra Yoder said. "He looks like his brother now."

No one quite knows what Bentley's future will look like but, his mother said, but the Yoders have hope again. "Because of how different his brain really is, they have no one to compare him to," Sierra Yoder said, adding that the doctors think "he will have a rewarding life. We just have to take it step by step.