Girl, 2, survives 'drastic' surgery to reconstruct her skull

When a two-year-old girl arrived in Quebec with a skull nearly the size of a basketball, doctors knew they had a rare case on their hands.

Doctors don't often seesuch extreme cases of hydrocephalusin North America, where health practitioners typically intervene earlier.

In the case of this little girl, so much fluid had accumulated in her brain that it had forcedhercranium to become deformed and grow abnormally large.Her head was so heavy that she was unable to lift it on her own. There was a risk of her neck snappingunder the weight. And there was pressure building up in her cranium.

Using a3D-printedmodel, some 210 screws and a black pen, surgeons undertook a risky 12-hour operation to reduce the size of the toddler's skull and allow her brain to grow.

The child, whom CBC News has agreed not to name to protect the family's privacy,wasborn in a country inNorth Africaandarrived in Quebec last year.

The damage to her brain is certainly major.- Dr. AlexanderWeil, pediatric neurosurgeon

Montreal surgeons examined her and discovered her cranium was filled with about three litres of fluid (roughly 20 times the amount in a healthy adult) and had grown to 71 centimetres in circumference.

Pediatric neurosurgeon Dr. Alexander Weil placed an internal shunt in her brainto help drain the excess fluid.

But the effect was minimal.

"It was like sticking a straw in a pool," said Weil, of Montreal's Sainte-Justine Hospital.

In addition to her physical limitations, the two-year-old had developmental delays because her brain had atrophied under the pressure of the excess liquid, to the point its volume was 80 per cent less than a normal one for her age.

"The damage to her brain is certainly major," Weil said.

While no surgery would be able to fully restorethe child's neurological functions, Weilwas sure he could give her a better life.

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But it would take what he called a "drastic procedure" to reduce the size of her skull.

The plan was to take apart her skull and then piece it back together like a puzzle to make it smaller.

CBC/Radio-Canada's French-language TV programDcouvertehad exclusive access to the operating roomduring the procedure.

No guarantees

Weil teamed up with plastic surgeon Dr. Daniel Borsuk, as well as engineers in a lab in Michigan, to prepare for the challenging procedure.

Before entering the operating room, the team created a 3D virtual mock-up of how the skull could bereconstructed.

"We took the skull of the patient, we put it in [virtual ] pieces, and we found the best way to reduce the volume and the shape of the skull using those pieces," Borsuk explained.

The surgeons' plan was to reduce the size of the girl's cranium by 60 per cent.

"It would be impossible to make the shape of the skull normal. That's not our goal. Our goal is to give this child the best chance at having as normal a life as possible," Weil said.

"Will the operation guarantee that? Definitely not. But it will definitely increase her chances."

No room for error

On November5 at 11:05 a.m. Borsuk prepared for the first incision.Despite all the preparations, there were still plenty of risks.

The first step was to make an incision from one ear to the other, to expose the skull.

When they cut through the child's skull, the surgeons had to be careful to not hit blood vessels directly beneath.Any obstruction or damage to the brain's superior sagittal sinus which allows the brain's hemispheres to drain could cause cerebral hemorrhaging.

Once the skin was removedand the skull exposed, something that looked like a plastic helmet was brought over to the operating table.

It wasthe key to the entire procedure a cutting guide, created on a computer several weeks earlier by the American engineers, and printed in 3D.

The surgeons used the guide and a black pen to trace the path they wouldcut, dividing the top half of child's skull into 12 pieces.As each piece was cut, it wasplaced on an adjacent table, where all 12 pieces would later be reassembledinto a new, smaller skull.

After several hours, the girl's brain was fully exposed.

That's when the riskiest part of the procedure began: draining the fluid.

Weil removed the liquid slowly, at a speed ofabout 10 millilitres per minute, or about half a litre per hour. Moving any faster would have posed unnecessary risk.

Get a peek inside the operating room

6 years ago

Duration 0:56

CBC/Radio-Canada's French-language TV program Dcouverte got exclusive access.

Weilsaid he was able to remove about half of the liquid that had accumulated, aboutone and a half litres.

"For us, the worry is that if we take away too much liquid all at once, it will greatly increase the complications and risk of complications," he said.

The final step was to place the reconstructed skull back on the girl's brain.To fix it in place, the team used210 screws, which will degrade in the span of a year and be absorbed by the body.

The operation lasted 12 hours, and the child was taken to intensive care to recover.

Two weeks later, an MRI confirmed the surgery was a success: her brain hadnearly doubled in volume.

Now that the girl's brain was unburdened from the excess fluid, her surgeons have hope the child's neural networks will reorganize themselves, and that she'll some day be able to play like other kids her age.