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Disarming the Bomb In Hunter's Head & Other Tales of the King of Pediatric Neurosurgery By
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CHRISTOPHER
JOHNSTON
Fortunately, the tiny bomb lurking in Hunter Severns' head has not escaped detection. Diagnostic scans on two light screens on the operating room wall reveal its shadowy outlines insinuating throughout his brain, stealing blood from its rightful recipients. It's bruit (pron. "brewy"), or whooshing sound from the fast-rushing fluid, rumbles through a stethoscope pressed to his shaved scalp. His bomb has a medical name: arteriovenous fistula (AVF) or vascular malformation. In plain English, this knot of errant veins and arteries threatens to explode into seizures or a stroke. Upon detonation, the well-concealed mind mine would probably prohibit Hunter from growing beyond his three and a half years. Today, though, the Danville, Ohio, boy has entered. the realm of Big Al Cohen, King of Pediatric Neurosurgery: pressure of working in complete silence "Our goal today is to reduce the flow OR #28 at Rainbow Babies and and tension erupting into the angry in this fistula," Big AI explains. "We Childrens Hospital. Dr. Cohen will be exchanges he often witnessed in his resiprobably won't be able to completely eliminate it, but we're going to cut it directing the day-long drama about to dency years in New York and Boston. down significantly. The risk is that he unfold, orchestrating the intricate dance It's now 9:00 am. Fully anesthetized, can bleed explosively again, but it's a of an elite squad of neurosurgeons, nursHunter's bald pate is elevated from the controlled situation, so we're giving him es, anesthesiologistsand neuroradiologists. table, pinned into a graphite head-holder this one shot." The music? that is radiolucent, which means it will "You need the King for tough surgallow X-rays to pass through should the Hunter has many things going for eries," Big AI says,referring not to himsurgeons need to check their progress him today, including the fact that the pediatric brain is more resilient than the .,.;s:,;;e~lf:>~b:;:..u';;;,t:c..,;:.to:;:.,.;t;,;.h;,:;e~se~l~e.:::c~ti:.:::o:.:;n~s...:fi;;;;r;;:;o~m~E~1:.;v~is;:..'_..;,W1.:;.;¡;;:;th~a~n~angiog!"am. The arcing baseball adult brain. Platinum Collection CD, a gift from the stitch in his scalp reveals an angry remnurses, blaring from a boombox in the nant of an operation performed by Dr. "In pediatric neurosurgery, we deal corner. Cohen when Hunter was just two with the evolving child, so it's a chance "The whole OR works better if weeks old to staunch a nearly fatal hemto have a profound effect on someone's everybody's relaxed," he adds, outlining orrhage. The doctors knew then they entire life," Big AI says later. He also his theory of different music for different would have to revisit the "big pipe-like enjoys the opportunity to confront a stages of the operation. Brain surgery is feeding vessels," five times their normal whole spectrum of neurosurgerical probtense enough without the additional size, at a later date. lems in the pediatric brain, whereas
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roughly 80 percent of adult neurosurgeries are spinal operations. As the team swirls around the child, covering every inch of his naked body with blue, sterile surgical drapes, including several stapled to his scalp to reduce the risk of infection in the soon-to-be wide-open head, Hunter lies quietly, vulnerable, trusting in the sweet brutality of late 20th-century medical science to save his life - and all of his neurological functions, personality, emotions. First, they must first sever, drill and saw off a third of his skull. Due to the complexity of the operation, two senior residents will assist. At 9:S5, under Dr. Cohen's watchful guidance, doctors Christopher Taylor and Skekar Kurpad initiate the craniotomy that will expose the pulsing, bloodied gray peaks and valleys of Hunter's abnormal brain. "Fish hooks" attached to his scalp hold back the loose flap of skin. As they begin to drill burr holes through his skull at points drawn in purple marker, a faint whiff redolent of the dentist's drill entering enamel hits the air. Bright red blood drips into the plastic irrigation bag attached to Hunter's head to keep fluids from lubricating the floor. The team displays an easy camaraderie, joking about the competition between the two senior residents. Dr. Cohen deadpans that they are prima donnas comparable to Major League Baseball pitchers Roger Clemens and David Wells. Dr. Taylor counters that they drew short straws the night before to determine who would have to work with Big AI. "We spend years of training and practice, and everyone here is highly skilled, so when we're doing a standard opening, it's like changing a carburetor," Big Al offers. "So we can be a little more relaxed." Things get tense fairly quickly, however, as they penetrate the leather-like protective lining of the brain, the dura mater, literally "hard mother" or colloquially "tough bitch," and at 10:29 one of the vesselsstuck to the lining ruptures, spouting blood several inches into the air. Using bi-polar forceps to cauterize the vessel, they work quickly to get the bleeder under control. "That was impressive," assessesBig
AI. Noticing that the CD has ended, he calls for the circulating nurse. "Kathy? What's missing from this room?" She pops in a Vince Gill CD from the pile on the speaker marked "To Be Played." The procedure is about one quarter complete. Many hours will be spent slowly dissecting Hunter's brain to reach the torturous tangle of veins and arteries so they can be clamped off. "It takes an hour for us to move a millimeter here, but that's the way it works in the brain," Dr. Cohen says.No one expected the job to be easy. After all, this IS brain surgery. The doctor who would be King, Alan R. Cohen, MD, FACS, FAAP, chief of pediatric neurosurgery, University Hospitals Health System, Rainbow Babies & Childrens Hospital, acquired his honorary title through the power vested in him by his loyal subjects: patients newborn to age 18. In their minds he is King solely because
they still have minds, brains, spines, nerves that function healthily after tumors, vascular malformations, aneurysms and other foreign invaders were repelled by Cohen and his Court, the hospital's medical staff. In his office, Big Al's majestic designation residesin the crayoned thank-you notes framed on his walls, including one from a lS-year-old girl grateful for his removing the bullet from her frontal lobe after she lost big at Russian roulette. Or the letter from the 9-year-old subject who, thinking her King should have an autographed Jay Leno head shot to counteract the "dorky Pat Sajak" picture in his office, wrote The Tonight Show host. Leno agreed. The true seat of his kingly powers, however, arises from the regal, pink Elvis-in-Full-Vegas lamp on his desk. And the black velvet Elvis painting on his wall. And the Elvis doll on his bookshelf Etceteras. Actually Big AI, who's
"There's nothing more devastating than losing a child," he says."When you have a child with a brain tumor, the whole familyjust fallsapart." Thus, the Elvis paraphernalia and the lS0-pound fish tank become imbued with a loftier purpose. "These objects sort of diffuse things, especially for the parents," saysBig Al.
47, admits, "I'm not that crazy about the King," but someone gave him the lamp as a gag gift and a pediatric legend was born. The kids figured if their man likes the King of Rock, then, hey, why not crown him the King of Pediatric Neurosurgery? Actually, Big Al's not that big, either. But his well-defined hands seem too large for his average stature. The hands of a surgeon. The hands of a magician. Sure, magician works metaphorically for this story, but the man is actually trained in slight of hand. Seeking a diversion
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while doing a residency at Bellevue Hospital in New York, he studied with Tony Slydini, a septuagenarian Italian magician. Until he got too busy, Cohen used to unicycle into the kids' ward and entertain them with tricks. Not every operation ends with a miraculous vanishing disorder, though. On the bulletin boards laden with snapshots sent to him by his subjectsand their families,Big Al can point out a few with malignant tumors who never had a chance or died severalyears after he operated from a recurrence of their cancer.
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DAVID ROBINSON TREASURES his first exposure to Big Al's own private Graceland, just a few days after his surgery. "They wheeled me into his office before I went home, and I still couldn't think straight," he recalls. "I saw the Elvis lamp and all this Elvis stuff, and I didn't know if it was just me that was weird or if it was Al." David's father, Michael, laughs when he remembers his first audience with the King, too. Though he wasn't laughing on that hot May night in 1997, as the Akron attorney tried to wrap his own mind around the word "ganglioglioneurocytoma," a tumor that had felled his 17-year-old son, who just 12 hours earlier awoke a perfectly healthy kid, except for a headache. The Bath, Ohio, Robinson family had ventured to Athens to visit Ohio University, where David was headed in the fall. During the day, he had grown increasingly ill, to the point where he couldn't hold a cold can of pop his father offered, thinking his son had the flu from which his brother and stepmother had just recovered. A frantic trip to a Parkersburg, West Virginia, hospital ensued. There, after reviewing a CAT scan, the neurosurgeon, who had
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interned at University Hospitals, recommended Rainbow. For this particular disorder, the Robinsons' family doctor also endorsed Rainbow. Still, after his son was life-flighted to UH and he raced at 100 mph to Cleveland, Michael Robinson was not convinced. Then the friendly doctor who happened to be on call that night also recommended Rainbow to remove the rare tumor, which had somehow grown to the size of a softball without any symptoms and was hemorrhaging. "It's kind of comical now," Michael chuckles. "I was standing there talking to the best doctor I could find, but I'm thinking he's just some guy they sent in on Saturday night who doesn't know anything, and I want my kid out of there." He knew he was talking to a Dr. Cohen, but he didn't know until 12 hours later that he was dealing with the King of Pediatric Neurosurgery. "Everyone wants to think they've accomplished something by going to the Mayo Clinic, but we couldn't have ended up with anybody better, no matter where we would have gone," he continues. "Al has just been an all-around nice guy since then. He never just passed us off to other people in his department, and he stays in touch with us." "Big Al's an awesome guy," claims David, who recovered so quickly he was able to attend his senior prom and graduation just a few weeks later. After a rough freshman year, recovering emotionally from his experience, he has gone
Though he retired from neurosurgery last year after 38 years behind the scalpel, Dr. White continues to teach at Case Western Reserve University's medical school and travel the world lecturing on "the most magnificent structure ever created." Through the Brain Research Laboratories he established at CWRU and Metro General Hospital in the early '60s, Dr. White's research team became the first to isolate the brain in a monkey, removing it and keeping it alive with machines, then later transplanting the monkey's brain. Their National Institutes of Health-funded work also pioneered cooling techniques to enhance brain surgery procedures and limit injuries in stroke and head-trauma patients. Although
Dr. White
believes
his
chosen field has benefited from advances in anesthetics, optics and radiology, he's less optimistic about progressions in neurosurgery itself For instance, one of the brain's most primal adversaries, malignant cancer, remains a vexing challenge. "If I were going to operate on a malignant tumor, I don't think I could do any better job today than I could have done 30 years ago," he says. Nevertheless, Dr. White rates Cleveland a good place to seek neurosurgical succor. Referring to the popular
on to earn a 3.3 GPA at OU, where he
but flawed annual rankings of healthcare institutions in U.S. News & World Report, Dr. White says, "We have been improperly rated as a neurosurgical complex. The Mayo Clinic is at the top, and it's hard to beat all of the resources of
performs with the marching band. "There's really nothing I can't do now,"
New York, Chicago, Boston or Los Angeles, but I would say overall, with
he says. He has an MRI every six months to ensure his ongoing health. So, in one cerebral hemisphere, President Bush, who labeled the '90s the
the Cleveland Clinic, University . Hospitals and MetroHealth System, we're 9th or 10th in the country." As we round out the Decade of the Brain, our King of Pediatric Neurosurgery, whom Dr. White ranks as "excellent," is proactively pursuing
Decade of the Brain, would be pleased to know great strides have been made in mitigating certain life-threatening, neurological disorders. In the other hemisphere, though, essential advances appear out of sight, far into the next century. "We know less about the brain than we think we do," posits Cleveland's dean of neurosurgery, Robert J. White, M.D., Ph.D. "It's hard to give it a percentage, but we only know a fraction of the brain's capabilities."
advances in neuroendoscopic surgery or minimally invasive techniques to treat some tumors and hydrocephalus, water on the brain, the most common pediatric neurosurgical problem. The intent is to replace the severely invasive craniotomy procedure whenever possible and allow the patient to go home the next day with "just a Band-Aid on their head."
In the era of cybersurgery, neurosurgeons have taken the endoscope, a small tube employed for laproscopic procedures to remove gall bladders without exenterating the abdomen, and modified it to enter the skull through a dime-sized opening. A fiberoptic camera projects onto a TV screen, allowing surgeons to maneuver inside the head with miniaturized lasers, grasping forceps, scissors, etc. This approach also relies on stereotactic surgery, a computer-guidance system which translates the two-dimensional
images from a CAT scan or MRI into a three-dimensional plane, enabling surgeons to place their instruments with less than a millimeter accuracy to reach any place in the brain safely. In early October, Big AI will once again lead a course sanctioned by the American Association of Neurological Surgeons in these video-centered procedures he refers to as "Nintendo neurosurgery." Picture a group of some of the finest neurosurgeons in the world hunkered over pumpkins, trying to extract a
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seed to test their eye-hand coordination with the endoscopic instruments before moving on to cadavers. "We teach that the goal of the surgeon is to follow the bank robber's credo: Get in without anybody knowing about it, get the money, get out," Big AI quips. Ever the optimist, Big Al cites advances that have resulted in curing ailments fatal just a decade ago. Hunter's case, for example, a disorder that accounts for less than one percent of pediatric neurological procedures, became treatable in the '90s. One of the advances making that possible is the ability to perform both neuroradiological therapies as well as intraoperative diagnosis. In other words, as in Hunter's case, a team of neuroradiologists first spent 12 hours the day before his surgery inserting tiny coils through a catheter in his thigh to occlude or close as much of the vascular malformation as they could reach. Then during his surgery, they brought in a portable angiogram machine several times to provide new scans that would tell the surgical team how successful their efforts were in closing the remaining aberrant vessels.
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In fact, at 5:15 that Friday, though everyone is tired and itching to close, Dr. Cohen requests a fourth angiogram. "If we've really occluded the malformation, then we're done, and Hunter won't need to get a post-operative angiogram and go through the general anesthesia again," he says. "Anything that's still there, we're going to clip, then get out.. .. I just feel obliged to take one more look." The angiogram confirms that the
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1999
bomb has been defused. For now. One tiny irregular spot remains deep inside Hunter's head, which will have to be monitored regularly. Another day, another brain saved. It's after 8:00 pm and time for Big Al to treat his two senior residents to a cele-
obliterated. By 6:10, Hunter's skull has been reattached with delicate, titanium burr covers and screws, and doctors Taylor and Kurpad are closing to the strains of "Wipeout" by The Safaris. That is until Big Al dedicates a song to them, slipping in a tape of "Stand By Your Man" as sung by ... Big Al. An hour later, the team awakes a groggy Hunter to make sure he can move all of his limbs. "Hi, Hunter. The operation's over. Would you like to see your mommy?' Dr. Cohen says, before trekking off to Rainbow's waiting room to inform the frazzled parents their son is okay. The
Come see what's brewin' a( our house!
bratory drink. Then back to the office first thing Saturday for a meeting. When told both the Severns and the Robinsons expressed amazement at the long hours kept by the King, who's been known to return calls or e-mails at all hours, he replies: "I always remind myself of what those people are going through on the other side of the desk, and it makes it easier to do whatever is necessary." When pressed as to whether he toils 24 hours a day, though, Big Al merely allows, "Only 23 and a half I'm
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ThursdaylFriday Saturday
After hanging in the operating room all day with the King, Christopher Johnston wants to release a Big Al compilation CD.
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