Saving Cyla

By KELLY GRANT and PATRICK DELL
The Globe and Mail
Cyla Daniels agreed to let The Globe and Mail into the operating room as doctors opened her skull and burrowed into her brain in search of a deep-seated tumour, waking her up to test her speech, reading and writing skills as they went. Warning: This multivideo feature has graphic content (Read a text-only version)

A frightening discovery

Daniels’s seizures, which started when she was 19, were increasing in frequency and intensity – threatening the Oshawa, Ont. student’s dream of studying law. A scan revealed something deep in her brain

Video: Three days before her surgery, Cyla Daniels, 23, remembers feeling scared and emotional. Her tumour had grown from a speck to the size of a date, prompting the decision to remove it. “I don’t know how I’m gonna look after, or be,” she says.

Every brain is different: All human brains have a similar landscape, but the elements differ from person to person, a bit like a park with same-size gardens and different plants.

Dr. Sunit Das, a neurosurgeon at St. Michael’s Hospital in Toronto, favoured an awake craniotomy to remove Daniels’s tumour, a procedure that would involve waking her and asking her to perform a series of tests while he stimulated her exposed brain. The goal: To cut a safe path through the vegetation and pluck out the weed – the tumour – all while preserving the species that make it possible for Daniels to speak, read, write and move.

Finding this safe path to her tumour was the best-case scenario, Das predicted. In a less ideal case, the surgical team would open her skull and discover the tumour was fenced in by crucial brain matter, beyond safe reach. Or, through a biopsy, they would find that – against the odds – the tumour was malignant, meaning Daniels would need chemotherapy or radiation.

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The medicine

An American neurosurgeon and two Italian anesthesiologists at St. Michael’s have developed a new protocol – which includes novel use of a drug and a refined scalp nerve block – to make longer awake brain surgery for high-risk patients possible

Photo: Daniels listens to music on her phone while anesthesiologists Dr. Andrea Rigamonti (left) and Dr. Marco Garavaglia (right) prepare her for an awake craniotomy. The anesthesiologists are part of the team that refined the new anesthesia protocol.

Evolution of awake brain surgery: Awake brain procedures are not new to this country. Canadians with even a fleeting knowledge of medical history will recognize the story of Dr. Wilder Penfield, the path-breaking neurologist and neurosurgeon who in the 1930s helped perfect the “Montreal Procedure,” a method that allowed him to electronically stimulate the brains of hundreds of epileptic patients as they lay awake and, eventually, to create the first functional maps of the surface of the human brain. His work was cemented in the popular imagination with a Canadian Heritage Minute that featured a woman on an operating table exclaiming: “Dr. Penfield, I can smell burnt toast!”

After Penfield’s breakthroughs, however, operating on awake patients fell out of fashion.

“I think a lot of people thought that awake brain surgery was barbaric,” said Dr. Mark Bernstein, a neurosurgeon at Toronto Western Hospital who has performed more than 1,000 awake craniotomies since 1991, when he and an American colleague helped revive the practice for the purpose of safely extracting tumours.

The St. Michael’s medical team that would operate on Daniels has added its own small footnote to the legacy of surgeons like doctors Penfield and Bernstein. The team helped refine an anesthesia protocol, using a recently approved sedative called dexmedetomidine, that allows doctors to safely perform the longest awake craniotomies ever recorded in the medical literature – including on patients for whom the procedure would normally be considered too risky, such as the old, the obese, and those afflicted with tough-to-extract brain tumours.

“The advantage here is if you’ve got a brain tumour near an area of your brain that’s very important to your function – speech, movement or language – you want to maximize the resection of that tumour without impacting the normal functions for your life.”— Dr. Gregory Hare, anesthesiologist at St. Michael’s Hospital

The team’s first surgery on an awake patient lasted an astonishing nine hours and allowed them to wake the patient multiple times for testing.

So far, Das and anesthesiologists Dr. Marco Garavaglia and Dr. Andrea Rigamonti have used the protocol in more than 30 cases, the first 10 of which they recounted in a clinical report published in the Journal of Neurosurgical Anesthesiology in July. Now they use it on all their patients, even otherwise healthy ones like Daniels whose surgery is not expected to last longer than five hours.

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Getting into Cyla’s head

As Daniels was rolled into the operating room, ear buds connected to her iPhone filled her mind with music – a distraction that, along with some last-minute anti-anxiety medication, was meant to smooth her frayed nerves

Video: Daniels will be asleep while doctors put her head in a clamp, cut through her skin and open her skull. The clamp will ensure her head stays in the same position throughout the surgery.

A controversial approach: Lengthy awake craniotomies are somewhat contentious. Patients with their heads locked in a clamp can become uncomfortable and claustrophobic as the operation drags on.

“I’m very sensitive about time. Anything that I think will go longer than four hours I don’t book for an awake craniotomy,” said Bernstein, the veteran neurosurgeon at Toronto Western.

Still, he called it a “terrific operation,” and praised the dexmedetomidine protocol, which provides patients with sedation and anxiety relief without requiring a breathing tube or laryngeal mask, thereby allowing them to speak.

Toronto Western is in the midst of a clinical trial comparing the new sedative to a more traditional alternative, propofol, which – like dexmedetomidine – keeps the airway clear, but doesn’t appear to protect it quite as well. St. Michael’s, meanwhile, has just launched a different sort of study of the dexmedetomidine protocol, one that aims to measure the short- and long- term psychological effects of being awake during brain surgery.

The St. Michael’s team is also test-driving a tablet for neurological testing that is safe to use inside the giant magnet of an MRI machine and on the operating table while patients are awake, a tool developed by scientists at Toronto’s Sunnybrook Research Institute.

But before doctors could use the new tablet in Daniels’s case, they had to get into her head. After they turned her on her side, secured her skull in a brace and draped her body in sterile sheets, they sliced a reverse-question mark incision above her left ear and exposed her brain.

The way to the brain

graphic

The way to the brain

Graphic: After the incision is made through the scalp, holes are drilled in the exposed skull using a perforator. Cuts are then made with the craniotome from hole to hole until a section of the bone is free and can be removed. Once the bone is removed, the anesthesiologist lightens the sedation and wakes up the patient. In the meantime, surgeons make an incision in the dura, the thick membrane that surrounds the brain and spinal cord, and the final layer that contains pain fibres.

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Waking Cyla

With her skull opened and the electric prod and tablet ready, testing could begin. The goal? To determine exactly which areas on the path to the tumour control speech, language and other critical functions

Video: Once Daniels wakes up, the electric prod is used to stimulate the brain while she performs a series of tests, including counting and writing. When the stimulation is applied, it inhibits her ability to complete the tests. In this way the surgeons can find a safe corridor to the tumour.

Testing the brain: It took more than 20 minutes to rouse Daniels. A microphone amplified her voice and a video streaming onto a screen let Das watch her face as he worked. More than a dozen times he asked Daniels to count to 10 – shifting his wand, prodding her brain as she spoke.

Sometimes she plowed through the kindergarten chant with ease; other times her voice dropped off halfway through and picked up again after Das removed the electric charge.

After the counting test, Das asked her to read words flashed on a screen and to write on the tablet designed by Simon Graham, a senior scientist and engineer at the Sunnybrook Research Institute. (Daniels had used the tablet to perform similar tasks inside an MRI machine before her surgery, allowing the doctors to make a rudimentary map of the area around her tumour.)

Graphic: The first diagram illustrates the anatomically defined functional areas of the brain, including motor and language. The second diagram illustrates the word generation areas that lit up when Daniels was tested with the tablet in the MRI scanner. These are the areas surgeons would have to avoid on their way to the tumour. (Source: Melanie Morrison & Dr. Simon Graham, Sunnybrook Research Institute)

When the neurological tests ended, an emotionally raw Daniels apologized for “failing” – not realizing that for the tests to succeed, she would have to fail to determine which parts of her brain couldn’t be cut into without compromising speech, writing, language and other core functions.

Das reassured her she had done well. She turned on her iPhone and fell back to sleep. She had been awake for a little more than 25 minutes.

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Mining for a tumour

With a safe path determined, surgeons shift their focus to sampling and removing the growth that’s been triggering Daniels’s seizures

Video: Once Daniels is back asleep, surgeons begin burrowing into her brain. It takes them approximately one hour to reach the tumour.

Rush to pathology: When Das reached the lesion in Daniels’s brain, he removed a tiny sample and sent it off for initial review at a small lab inside the hospital. Pathology staff slipped the specimen onto a glass slide and peered at it through a microscope, allowing them the first in-depth look at the tumour that had been causing Daniels’s seizures.

The growth was consistent with a low-grade ganglioglioma – a benign, slow-growing tumour that often affects children and young adults. Later, further pathology testing on Daniels’s tumour confirmed the finding. An MRI reading after the surgery yielded even better news: Her scan was clear.

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After the surgery

Daniels and her family get the good news. Following a three-month recovery period, Daniels will be able to hit the books and chase her dream of becoming a lawyer – seizure-free

Video: Three weeks after her surgery, Daniels remembers worrying she might never be able to go back to school. “Before they told me it was going to be an awake brain surgery I was nervous... what if they hit something and I can’t talk?”

Feeling strong: Three weeks to the day after the operation, the awake portion of the surgery felt like a hallucination Daniels could barely recall. A few dream-like snatches stayed with her, but she had no memory of the performance anxiety she expressed at the end of the neurological testing.

“I think I remember me doing well,” she said. “I think so. I can’t exactly remember. I feel like I did well on it, but I’m not exactly sure.”

Daniels had not seen the video of her surgery. Told she had apologized to the doctors for struggling with the tests, Daniels admitted to an inner insecurity that the drugs had apparently laid bare. But surviving her seizures and surgery has boosted her confidence.

“Now I actually want more of [my hair] shaved on the side to kind of show off my scar,” she said. “I’m just proud of what I went through and how strong I was.”

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