disarming the bomb in hunter's head & other tales of the king of pediatric neurosurgery

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Disarming the Bomb Hunter's Head In & Other Tales of the King of Pediatric Neurosurgery By 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: arteri- ovenous fistula (AVF) or vascular mal- formation. 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 halfyears. Today, though, the Danville, Ohio, boy has entered. the realm of Big Al Cohen, King of Pediatric Neurosurgery: pressure of working in complete silence OR #28 at Rainbow Babies and and tension erupting into the angry Childrens Hospital. Dr. Cohen will be exchanges he often witnessed in his resi- directing the day-long drama about to dency years in New York and Boston. unfold, orchestrating the intricate dance It's now 9:00 am. Fully anesthetized, of an elite squad of neurosurgeons, nurs- Hunter's bald pate is elevated from the es,anesthesiologistsand neuroradiologists. table, pinned into a graphite head-holder The music? that is radiolucent, which means it will "You need the King for tough surg- allow X-rays to pass through should the eries," Big AI says,referring not to him- surgeons need to check their progress r+'__ .,.;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 Platinum Collection CD, a gift from the stitch in his scalp reveals an angry rem- nurses, blaring from a boombox in the nant of an operation performed by Dr. corner. Cohen when Hunter was just two "The whole OR works better if weeks old to staunch a nearly fatal hem- everybody's relaxed," he adds, outlining orrhage. The doctors knew then they his theory of different music for different would have to revisit the "big pipe-like stages of the operation. Brain surgery is feeding vessels," five times their normal tense enough without the additional size, at a later date. "Our goal today is to reduce the flow in this fistula," Big AI explains. "We probably won't be able to completely eliminate it, but we're going to cut it down significantly. The risk is that he can bleed explosively again, but it's a controlled situation, so we're giving him this one shot." Hunter has many things going for him today, including the fact that the pediatric brain is more resilient than the adult brain. "In pediatric neurosurgery, we deal with the evolving child, so it's a chance to have a profound effect on someone's entire life," Big AI says later. He also enjoys the opportunity to confront a whole spectrum of neurosurgerical prob- lems in the pediatric brain, whereas 133

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Christopher Johnston wrote this article for Northern Ohio Live, where it was published in September 1999.

TRANSCRIPT

Disarming the BombHunter's Head

•In& Other Tales

of the King of Pediatric Neurosurgery

By CHRISTOPHER JOHNSTON

Fortunately, the tiny bomb lurking inHunter Severns' head has not escapeddetection. Diagnostic scans on two lightscreens on the operating room wallreveal its shadowy outlines insinuatingthroughout his brain, stealing bloodfrom its rightful recipients. It's bruit(pron. "brewy"), or whooshing soundfrom the fast-rushing fluid, rumblesthrough a stethoscope pressed to hisshaved scalp.

His bomb has a medical name: arteri-ovenous fistula (AVF) or vascular mal-formation. In plain English, this knot oferrant veins and arteries threatens toexplode into seizures or a stroke. Upondetonation, the well-concealed mindmine would probably prohibit Hunterfrom growing beyond his three and ahalfyears.

Today, though, the Danville, Ohio,boy has entered. the realm of Big AlCohen, King of Pediatric Neurosurgery: pressure of working in complete silenceOR #28 at Rainbow Babies and and tension erupting into the angryChildrens Hospital. Dr. Cohen will be exchanges he often witnessed in his resi-directing the day-long drama about to dency years in New York and Boston.unfold, orchestrating the intricate dance It's now 9:00 am. Fully anesthetized,of an elite squad of neurosurgeons, nurs- Hunter's bald pate is elevated from thees, anesthesiologistsand neuroradiologists. table, pinned into a graphite head-holder

The music? that is radiolucent, which means it will"You need the King for tough surg- allow X-rays to pass through should the

eries," Big AI says,referring not to him- surgeons need to check their progressr+'__ .,.;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

Platinum Collection CD, a gift from the stitch in his scalp reveals an angry rem-nurses, blaring from a boombox in the nant of an operation performed by Dr.corner. Cohen when Hunter was just two

"The whole OR works better if weeks old to staunch a nearly fatal hem-everybody's relaxed," he adds, outlining orrhage. The doctors knew then theyhis theory of different music for different would have to revisit the "big pipe-likestages of the operation. Brain surgery is feeding vessels," five times their normaltense enough without the additional size, at a later date.

"Our goal today is to reduce the flowin this fistula," Big AI explains. "Weprobably won't be able to completelyeliminate it, but we're going to cut itdown significantly. The risk is that hecan bleed explosively again, but it's acontrolled situation, so we're giving himthis one shot."

Hunter has many things going forhim today, including the fact that thepediatric brain is more resilient than theadult brain.

"In pediatric neurosurgery, we dealwith the evolving child, so it's a chanceto have a profound effect on someone'sentire life," Big AI says later. He alsoenjoys the opportunity to confront awhole spectrum of neurosurgerical prob-lems in the pediatric brain, whereas

133

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roughly 80 percent of adult neurosurg-eries are spinal operations.

As the team swirls around the child,covering every inch of his naked bodywith blue, sterile surgical drapes, includ-ing several stapled to his scalp to reducethe risk of infection in the soon-to-bewide-open head, Hunter lies quietly,vulnerable, trusting in the sweet brutalityof late 20th-century medical science tosave his life - and all of his neurologicalfunctions, personality, emotions. First,they must first sever, drill and saw off athird of his skull.

Due to the complexity of the opera-tion, two senior residents will assist.At9:S5, under Dr. Cohen's watchful guid-ance, doctors Christopher Taylor andSkekar Kurpad initiate the craniotomythat will expose the pulsing, bloodiedgray peaks and valleys of Hunter'sabnormal brain. "Fish hooks" attachedto his scalp hold back the loose flap ofskin. As they begin to drill burr holesthrough his skull at points drawn in pur-ple marker, a faint whiff redolent of thedentist's drill entering enamel hits the air.Bright red blood drips into the plasticirrigation bag attached to Hunter's headto keep fluids from lubricating the floor.

The team displays an easy cama-raderie, joking about the competitionbetween the two senior residents. Dr.Cohen deadpans that they are primadonnas comparable to Major LeagueBaseball pitchers Roger Clemens andDavid Wells. Dr. Taylor counters thatthey drew short straws the night beforeto determine who would have to workwith Big AI.

"We spend years of training andpractice, and everyone here is highlyskilled, so when we're doing a standardopening, it's like changing a carburetor,"Big Al offers. "So we can be a littlemore relaxed."

Things get tense fairly quickly, how-ever, as they penetrate the leather-likeprotective lining of the brain, the duramater, literally "hard mother" or collo-quially "tough bitch," and at 10:29 oneof the vesselsstuck to the lining ruptures,spouting blood several inches into theair. Using bi-polar forceps to cauterizethe vessel, they work quickly to get thebleeder under control.

"That was impressive," assessesBig

AI. Noticing that the CD has ended, hecalls for the circulating nurse. "Kathy?What's missing from this room?" Shepops in a Vince Gill CD from the pileon the speaker marked "To Be Played."

The procedure is about one quartercomplete. Many hours will be spentslowly dissectingHunter's brain to reachthe torturous tangle of veins and arteriesso they can be clamped off.

"It takes an hour for us to move amillimeter here, but that's the way itworks in the brain," Dr. Cohen says.Noone expected the job to be easy. Afterall, 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 thepower vested in him by his loyal sub-jects: patients newborn to age 18. Intheir minds he is King solely because

they still have minds, brains, spines,nerves that function healthily aftertumors, vascular malformations,aneurysms and other foreign invaderswere repelled by Cohen and his Court,the hospital'smedical staff.

In his office, Big Al's majestic desig-nation residesin the crayoned thank-younotes framed on his walls, including onefrom a lS-year-old girl grateful for hisremoving the bullet from her frontallobe after she lost big at Russian roulette.Or the letter from the 9-year-old subjectwho, thinking her King should have anautographed Jay Leno head shot tocounteract the "dorky Pat Sajak" picturein his office, wrote The Tonight Showhost. Leno agreed.

The true seat of his kingly powers,however, arises from the regal, pinkElvis-in-Full-Vegas lamp on his desk.And the black velvet Elvis painting onhis wall. And the Elvis doll on his book-shelf Etceteras. Actually Big AI, who's

47, admits, "I'm not that crazy about theKing," but someone gave him the lampas a gag gift and a pediatric legend wasborn. The kids figured if their man likesthe King of Rock, then, hey, why notcrown him the King of PediatricNeurosurgery?

Actually, Big Al's not that big, either.But his well-defined hands seem toolarge for his average stature. The handsof a surgeon. The hands of a magician.Sure, magician works metaphorically forthis story, but the man is actually trainedin slight of hand. Seeking a diversion

while doing a residency at BellevueHospital in New York, he studied withTony Slydini, a septuagenarian Italianmagician. Until he got too busy, Cohenused to unicycle into the kids' ward andentertain them with tricks.

Not every operation ends with amiraculous vanishing disorder, though.On the bulletin boards laden with snap-shots sent to him by his subjectsand theirfamilies,Big Al can point out a few withmalignant tumors who never had achance or died severalyearsafter he oper-ated from a recurrence of their cancer.

•"There's nothing more devastatingthan losing a child," he says."When youhave a child with a brain tumor, thewhole familyjust fallsapart."

Thus, the Elvis paraphernalia and thelS0-pound fish tank become imbuedwith a loftier purpose. "These objectssort of diffuse things, especially for theparents," saysBig Al.

DAVID ROBINSON TREASURES his first expo-sure to Big Al's own private Graceland,just a few daysafter his surgery.

"They wheeled me into his officebefore I went home, and I still couldn'tthink straight," he recalls. "I saw theElvis lamp and all this Elvis stuff, and Ididn't know if it was just me that wasweird or if it was Al."

David's father, Michael, laughs whenhe remembers his first audience with theKing, too. Though he wasn't laughingon that hot May night in 1997, as theAkron attorney tried to wrap his ownmind around the word "ganglioglioneu-rocytoma," a tumor that had felled his17-year-old son, who just 12 hours ear-lier awoke a perfectly healthy kid, exceptfor a headache.

The Bath, Ohio, Robinson familyhad ventured to Athens to visit OhioUniversity, where David was headed inthe fall. During the day, he had grownincreasingly ill, to the point where hecouldn't hold a cold can of pop hisfather offered, thinking his son had theflu from which his brother and step-mother had just recovered. A frantic tripto a Parkersburg, West Virginia, hospitalensued. There, after reviewing a CATscan, the neurosurgeon, who had

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136 NorthernOhioLive ; September 1999

interned at University Hospitals, recom-mended Rainbow. For this particulardisorder, the Robinsons' family doctoralso endorsed Rainbow.

Still, after his son was life-flighted toUH and he raced at 100 mph toCleveland, Michael Robinson was notconvinced. Then the friendly doctorwho happened to be on call that nightalso recommended Rainbow to removethe rare tumor, which had somehowgrown to the size of a softball withoutany symptoms and was hemorrhaging.

"It's kind of comical now," Michaelchuckles. "I was standing there talking tothe best doctor I could find, but I'mthinking he's just some guy they sent inon Saturday night who doesn't knowanything, and I want my kid out ofthere." He knew he was talking to a Dr.Cohen, but he didn't know until 12hours later that he was dealing with theKing of Pediatric Neurosurgery.

"Everyone wants to think they'veaccomplished something by going to theMayo Clinic, but we couldn't haveended up with anybody better, no matterwhere we would have gone," he contin-ues. "Al has just been an all-around niceguy since then. He never just passed usoff to other people in his department,and he stays in touch with us."

"Big Al's an awesome guy," claimsDavid, who recovered so quickly he wasable to attend his senior prom and grad-uation just a few weeks later. After arough freshman year, recovering emo-tionally from his experience, he has goneon to earn a 3.3 GPA at OU, where heperforms with the marching band."There's really nothing I can't do now,"he says. He has an MRI every sixmonths to ensure his ongoing health.

So, in one cerebral hemisphere,President Bush, who labeled the '90s theDecade of the Brain, would be pleasedto know great strides have been made inmitigating certain life-threatening, neu-rological disorders. In the other hemi-sphere, though, essential advances appearout of sight, far into the next century.

"We know less about the brain thanwe think we do," posits Cleveland'sdean of neurosurgery, Robert J. White,M.D., Ph.D. "It's hard to give it a per-centage, but we only know a fraction ofthe brain's capabilities."

Though he retired from neurosurgerylast year after 38 years behind the scalpel,Dr. White continues to teach at CaseWestern Reserve University's medicalschool and travel the world lecturing on"the most magnificent structure evercreated." Through the Brain ResearchLaboratories he established at CWRUand Metro General Hospital in the early'60s, Dr. White's research team becamethe first to isolate the brain in a monkey,removing it and keeping it alive withmachines, then later transplanting themonkey's brain. Their NationalInstitutes of Health-funded work alsopioneered cooling techniques toenhance brain surgery procedures andlimit injuries in stroke and head-traumapatients.

Although Dr. White believes hischosen field has benefited from advancesin anesthetics, optics and radiology, he'sless optimistic about progressions in neu-rosurgery itself For instance, one of thebrain's most primal adversaries, malig-nant cancer, remains a vexing challenge.

"If I were going to operate on amalignant tumor, I don't think I coulddo any better job today than I couldhave done 30 years ago," he says.

Nevertheless, Dr. White ratesCleveland a good place to seek neuro-surgical succor. Referring to the popularbut flawed annual rankings of health-care institutions in U.S. News & WorldReport, Dr. White says, "We have beenimproperly rated as a neurosurgical com-plex. The Mayo Clinic is at the top, andit's hard to beat all of the resources ofNew York, Chicago, Boston or LosAngeles, but I would say overall, withthe Cleveland Clinic, University

. Hospitals and MetroHealth System,we're 9th or 10th in the country."

As we round out the Decade of theBrain, our King of PediatricNeurosurgery, whom Dr. White ranks as"excellent," is proactively pursuingadvances in neuroendoscopic surgery orminimally invasive techniques to treatsome tumors and hydrocephalus, wateron the brain, the most common pediatricneurosurgical problem. The intent is toreplace the severely invasive craniotomyprocedure whenever possible and allowthe patient to go home the next day with"just a Band-Aid on their head."

In the era of cybersurgery, neurosur-geons have taken the endoscope, a smalltube employed for laproscopic proce-dures to remove gall bladders withoutexenterating the abdomen, and modifiedit to enter the skull through a dime-sizedopening. A fiberoptic camera projectsonto a TV screen, allowing surgeons tomaneuver inside the head with miniatur-ized lasers, grasping forceps, scissors, etc.This approach also relies on stereotacticsurgery, a computer-guidance systemwhich translates the two-dimensional

images from a CAT scan or MRI into athree-dimensional plane, enabling sur-geons to place their instruments with lessthan a millimeter accuracy to reach anyplace in the brain safely.

In early October, Big AI will onceagain lead a course sanctioned by theAmerican Association of NeurologicalSurgeons in these video-centered proce-dures he refers to as "Nintendo neuro-surgery." Picture a group of some of thefinest neurosurgeons in the world hun-kered over pumpkins, trying to extract a

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seed to test their eye-hand coordina-tion with the endoscopic instru-ments before moving on to cadavers.

"We teach that the goal of thesurgeon is to follow the bank rob-ber's credo: Get in without anybodyknowing about it, get the money,get out," Big AI quips.

Ever the optimist, Big Al citesadvances that have resulted in curingailments fatal just a decade ago.Hunter's case, for example, a disor-der that accounts for less than onepercent of pediatric neurological pro-cedures, became treatable in the '90s.

One of the advances making thatpossible is the ability to performboth neuroradiological therapies aswell as intraoperative diagnosis. Inother words, as in Hunter's case, ateam of neuroradiologists first spent12 hours the day before his surgeryinserting tiny coils through acatheter in his thigh to occlude orclose as much of the vascular mal-formation as they could reach. Thenduring his surgery, they brought ina portable angiogram machine sev-eral times to provide new scans thatwould tell the surgical team howsuccessful their efforts were in clos-ing the remaining aberrant vessels.

In fact, at 5:15 that Friday,though everyone is tired and itchingto close, Dr. Cohen requests afourth angiogram. "If we've reallyoccluded the malformation, thenwe're done, and Hunter won't needto get a post-operative angiogramand go through the general anesthe-sia again," he says. "Anything that'sstill there, we're going to clip, thenget out.. .. I just feel obliged to takeone more look."

The angiogram confirms that thebulk of the malformation has been

138 NorthernOhioLive : September 1999

obliterated. By 6:10, Hunter's skull hasbeen reattached with delicate, titaniumburr covers and screws, and doctorsTaylor and Kurpad are closing to thestrains of "Wipeout" by The Safaris.That is until Big Al dedicates a song tothem, slipping in a tape of "Stand ByYour Man" as sung by ... Big Al.

An hour later, the team awakes agroggy Hunter to make sure he canmove all of his limbs.

"Hi, Hunter. The operation's over.Would you like to see your mommy?'Dr. Cohen says, before trekking off toRainbow's waiting room to inform thefrazzled parents their son is okay. The

bomb has been defused. For now. Onetiny irregular spot remains deep insideHunter's head, which will have to bemonitored regularly.

Another day, another brain saved. It'safter 8:00 pm and time for Big Al totreat his two senior residents to a cele-bratory drink. Then back to the officefirst thing Saturday for a meeting.

When told both the Severns and theRobinsons expressed amazement at thelong hours kept by the King, who'sbeen known to return calls or e-mails atall hours, he replies: "I always remindmyself of what those people are goingthrough on the other side of the desk,and it makes it easier to do whatever isnecessary."

When pressed as to whether hetoils 24 hours a day, though, Big Almerely allows, "Only 23 and a half I'mnot crazy."

Long live the king.

After hanging in the operating room all day withthe King, Christopher Johnston wants to releasea Big Al compilation CD.

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