National Post (National Edition)
‘This small little body with this giant head’
In a 12-hour operation in November, surgeons at CHU Sainte-justine Hospital in Montreal removed a two-year-old’s massive skull and put the pieces back together like a jigsaw puzzle, giving her a smaller head — and hope at a normal life
MONTREAL• Alexander Weilwa sat home the night the emergency room called. “I have something here I have never seen before,” said the doctor on the other end of the line. “You need to come.” It was 9:30 p.m. The emergency room doctor texted a photo to Weil, a neurosurgeon. It showed a ninemonth-old baby girl with soft tufts of curly black hair, and a head almost as big as her body.
The baby’s parents had immigrated to Montreal from Northern Africa just weeks earlier, in the spring of 2017. On this May evening they had arrived in the emergency room at Montreal’s CHU Sainte-justine hospital with their baby’s head wrapped in a scarf, and carrying big brown manila envelopes filled with copies of scans of her brain.
She had been born with hydrocephalus, a disorder that causes an abnormal accumulation of fluid in the brain. Untreated, it can be deadly.
When Weil arrived later that night in emergency room #7, the child was in a car seat. He asked the mother to please unwrap the scarf. The mother lifted the child into her arms; Weil could see how much she was struggling. When the scarf was removed, he saw a head bulging with fluid, the veins visible. The bones that made up the baby’s scalp had spread and split apart. Her body should normally have contained a little over half a cup of cerebral spinal fluid, the clear, water-like liquid that delivers nutrients to the brain and keeps it buoyant. Instead, more than three litres of the fluid had amassed in and around the child’s brain, a massive weight anchoring her down like a bowling ball to wherever she was laid. She could move her arms and legs, and twist her body slightly. But she couldn’t roll over, crawl or sit up because of the sheer weight of her head. It often took two people to carry her — one supporting her neck and head, the other supporting her body.
At 76 cm in circumference, her head was nearly twice the normal size for a baby her age. “You just see this small little body with this giant head,” Weil said.
The neurosurgeon’s thoughts raced to Port-au-prince, where he volunteers with Healthy Kids Haiti caring for children with hydrocephalus, one of the most common treatable causes of neurological disability and death in the developing world.
Doctors in rich countries like Canada routinely measure babies’ heads, checking for signs of the condition. If detected, it is most often treated with a shunt, a rubber drainage tube that diverts the extra fluid out of the cranial cavity and into another part of the body. With proper treatment, children usually go on to lead entirely normal lives.
In Africa, however, doctors had told this baby’s parents — who asked that the family not be identified in this story — nothing could be done for her. They simply didn’t have access to the surgery she needed.
“Of course it’s tragic to see a case like this,” Weil said. “Had we met her at birth, we could have intervened sooner, and certainly prevented it from getting to that state.”
The baby’s hydrocephalus was caused by a congenital abnormality, a birth defect that was causing cerebral spinal fluid to pool in her brain. Over the next 18 months, Weil would perform two surgical procedures to try to siphon off that excess fluid, and bypass the obstruction that was keeping it from being reabsorbed. But still, her head remained massive.
Then, this November, a pediatric team led by Weil and Dr. Daniel Borsuk, Sainte-justine’s chief of plastic surgery, took a radical, almost desperate approach: In a 12-hour operation, they cut into the now-26-monthold child’s scalp from ear to ear, removed her skull in pieces, drained nearly two litres of fluid from her brain and then pieced her skull back together, like a child’s wooden jigsaw puzzle, reconstructing her head to make it smaller.
Said Borsuk, the surgeon who in May led Canada’s first face transplant: “We created a skull that will fit her brain.”
Hydrocephalus was once commonly known as “water on the brain” — a misnomer, since the liquid builds up not on the organ but inside it.
The brain makes cerebral spinal fluid at a relatively constant rate, about the equivalent each day of a can and a half of Coke. The fluid normally flows through four chambers in the brain called ventricles, bathing the brain and spinal cord and then washing up and over the surface of the organ, where it’s reabsorbed by the veins into the bloodstream.
If the fluid’s passage is blocked and it can’t escape the ventricles they can become engorged, stretching like balloons. A baby’s skull and head can increase in size indefinitely, because during the first two years of life, when the brain grows and develops rapidly, the six bones in the skull are like continents. Instead of being fused as they are in an adult skull, they are held together by stretchy tissue called sutures that expands as the brain develops. As the brain grows, the skull grows with it and slowly the bones fuse and harden.
“But if you have something going on inside the brain that occupies space and increases pressure,” said Weil, a pediatric neurosurgeon and assistant professor at the Université de Montréal, “the brain stretches, as does the skull and skin.” The bones never fuse — they just keep expanding.
About one to two of every 1,000 babies are born with hydrocephalus. The most obvious sign is an unusual and frequently rapid growth, which is why pediatricians routinely measure a baby’s head circumference and plot it against normal growth charts. The circumference is like a vital sign for the brain, an indication something might be going wrong inside. One of the first symptoms of hydrocephalus can be a bulge in the fontanelle, the soft spot just above a baby’s forehead. There can also be vomiting, sleepiness, poor feeding, downward pointing eyes known as “sunset eyes” and irritability.
The mother who arrived at Sainte-justine in May 2017 had been four months pregnant when a routine ultrasound showed the baby’s skull wasn’t normal, and that fluid was building up in it. “The obstetrician said that sometimes the fluid goes away on its own, but it just kept getting worse,” she said. As the pregnancy progressed, doctors warned the parents their baby might have trouble eating or walking, might be severely handicapped or might die soon after birth.
When they searched the internet for information on the condition, “a lot of things scared me,” the mother said.
The fetal skull kept growing. Five weeks before the baby was due, she was delivered by caesarean section, because her head was simply too big to risk a vaginal birth.
The family had long planned to immigrate to Canada, even before the baby was born. Two weeks after arriving in Montreal, they took the baby to a walk-in clinic. There, an alarmed doctor told them to get to an emergency room immediately.
Once at Sainte-justine, scans showed the baby’s brain was stretched out and thin. The normal circumference of a ninemonth-old’s head is 42 cm. An adult head normally has a circumference of around 55 or 56 cm. At 76 cm, the size of this baby’s head was not only off the charts, “it was off the page,” Weil said. Doctors in Canada rarely, if ever, see a case so severe.
SHE COULD MOVE HER ARMS AND LEGS. BUT SHE COULDN’T ROLL OVER, CRAWL OR SIT UP BECAUSE OF THE SHEER WEIGHT OF HER HEAD. IT OFTEN TOOK TWO PEOPLE TO CARRY HER — ONE SUPPORTING HER NECK AND HEAD, THE OTHER SUPPORTING HER BODY.
From the moment he first examined her, Weil knew they had to move quickly. Her brain tissue was atrophying, literally wasting away from the pressure. Hospital administrators helped fast-track the family’s provincial medicare coverage. Once it was in place, the surgeons went to work, at first burning the structures that produce cerebral spinal fluid to try to decrease the flow. Weil also made an opening in the floor of one of the ventricles, hoping to let the fluid bypass the blockage. It wasn’t enough.
In a second surgery, a permanent shunt — a flexible plastic tube — was inserted into the base of her skull to drain the built-up fluid and divert it into her belly.
It worked, in the sense the shunt was functional. The child’s head stopped growing. It stabilized. But it didn’t decrease in size. It was like putting a straw into a swimming pool, Weil said. “You’re going to drain fluid out, but it’s such a huge reservoir of fluid.” And, despite the surgeries, she still had a massive head. The treatments didn’t address the problem caused by the hydrocephalus — macrocrania, from the Greek “makros,” meaning large, and “kranio,” skull.
“And so we talked to the parents,” said Weil. “We said, ‘We have a solution. It’s not perfect. But we have something that we think could help her — help her develop and function and live in society.’”
They needed to carefully consider the risks, including the risk of massive bleeding, stroke or infections. “The parents said, ‘If you think this is going to help our child, and you are confident you can do it well, we’ ll do it.’ ”
Surgery started the morning of Monday, Nov. 5. The mother was holding the baby in her arms outside the operating room. “They were worried and nervous, as any parent would be,” Weil said. “I reassured them that we would give her the best care possible and that we would treat their daughter as if she was one of our own.” Two nurses lifted the baby out of the mother’s arms, and carried her into the operating room.
Thirteen medical staff were waiting — neurosurgeons, plastic surgeons, a craniofacial surgeon, anesthesiologists, nurses and orderlies. Music played in the background — Hotel California and other soft classic rock. The mood was focused.
They placed the child’s head on a padded horseshoe-shaped headrest. They needed to expose the entire skull, but it had to be sturdy. “We fiddled a lot with the positioning. Just the sheer volume and size of the head compared to her body made positioning challenging,” Weil said.
The surgery had been mapped out in advance, using a special computer software that allowed them to create 3D printed models of the child’s skull based on CT scans of her head. In the operating room, two 3D replica skulls — the one she had, and the one they wanted to create — rested on sterile blue surgical cloths.
They first made a scalp incision from ear-to-ear, peeling back the scalp — one flap in front, the other in the back — to expose the entire skull.
Next, Borsuk clamped a 3D cutting guide over the dome, tracing the pieces to cut. Once the wedges of bone were lettered, Weil went in with his instruments and saws and cut out the pieces, careful to preserve important veins.
When the skull was removed, they saw the dura mater — the tough, leathery outer membrane that surrounds the brain. They were shocked by its sheer size, a large sack of fluid and brain, almost like a giant water balloon.
They installed an external drain, slowly draining the fluid down to less than a litre, careful not to tear a vein and cause bleeding. Drain, and stop. Drain, and stop.
As the fluid drained and the brain began to shrink, Weil had to shrink the dura mater. It was a crucial part of the surgery. The music was turned off.
Assisted by neurosurgeon Dominic Venne, Weil first began burning pieces of dura mater, but it wasn’t enough — so he began cutting the tissue in places and stitching it back together. At one point, the brain started poking out because there was still so much pressure from the fluid. As a neurosurgeon, Weil’s job is to protect the brain, to make sure it gets through the surgery. “And here we are, we’re draining all this fluid, we’re taking the skull out, we’re decreasing the size of the dura mater, and we’re putting the skull bones back and a smaller skull, all in a handful of hours. It’s like a storm. You have to try to do it in the most controlled way possible.”
As Weil and Venne worked on the dura mater, Borsuk worked at a side table, reassembling the skull. He placed the lettered pieces into a cutting bowl and attached them with absorbable plates and screws. Once done, “we just flipped it over and put it onto the patient’s head and fixed it into place.”
Towards the end of the surgery, the superior sagittal sinus, one of the major veins that carries blood from the brain back to the heart, started kinking and oozing blood. It had been so stretched by the excess fluid that it was now looser. The risk was a massive bleed and stroke. Weil knew it was time to start closing.
During a visit last week at Sainte-justine, the mother leaned down into a hospital crib and scooped the child into her arms, supporting her head with its arc of stitches. She propped her up in a brightly coloured toddler car seat. “Put your hands up. Cross your arms,” she said. “Bravo, bravo!”
The girl has brown eyes and long lashes, and as Weil bent down to nuzzle her cheek, she reached for his red beard. “She’s more active, she’s more dynamic,” said her mother. “She’s moving with more ease.”
Without treatment, the child’s skull could have grown steadily in size, causing more brain damage. “She would never have been able to develop the strength to support her own head,” Borsuk said.
The child spent 24 hours in a drug-induced coma after surgery. She is at home now. Her head is dramatically smaller, though still larger and wider than normal. It will grow smaller with time, though it could take years. The scars have healed and are undetectable. The surgeons couldn’t remodel the base of her skull, which was also stretched wide, because it would have meant more cutting, and lower down, meaning more bleeding. Her body’s entire volume of blood had to be replaced twice during surgery, and they couldn’t risk further damage to her brain tissue. She will need rehabilitation and physiotherapy to strengthen her neck muscles. “But she’s starting to do things that we expect all kids to be able to do,” Weil said. She’s playing and smiling, and her brain is starting to grow. It’s much less compressed, and is now about twice as thick as it was before surgery.
“This family is incredibly resilient,” Weil said. “That was their reality, and they were dealing with it. And now they’re here. They don’t have much, but they’re trying to start a new life. They take the bus and find ways to travel and bring their daughter around. They’re good, solid people.”
Even now, there are unknowns, he said. “But without this surgery, she probably never would have walked, maybe never have sat up. She would always have had to be carried and her development would certainly have stayed delayed.
“There would have been no way she could have lived a normal life. At least now, there’s hope,” he said. “She’s a beautiful little girl.”
AND HERE WE ARE, WE’RE DRAINING ALL THIS FLUID, WE’RE TAKING THE SKULL OUT, WE’RE DECREASING THE SIZE OF THE DURA MATER, AND WE’RE PUTTING THE SKULL BONES BACK AND A SMALLER SKULL, ALL IN A HANDFUL OF HOURS. IT’S LIKE A STORM — ALEXANDER WEIL