When the Air Hits Your Brain: Tales from Neurosurgery (17 page)

I followed the patient to the holding area outside the operating suite. She wept quietly into a handful of tissues.

“Is there anyone I can call to be with you?” I asked.

“My dad died two years ago and my mum’s got a bad heart. I’d rather not tell her anything right now.”

I couldn’t begin to imagine her despair as she faced this, crisis alone. After several minutes of waiting, an OR nurse whisked her into the room and an anesthesiologist ended her suffering with a whiff of gas.

Little more than fifty minutes had passed since she had arrived in the casualty department. British medicine at its finest. Although mired in red tape, the U.K. system avoids the legal wrangling that hamstrings American medicine. In the United States, taking a woman to the OR without objective studies, such as MRI or CT scans, invites malpractice action should anything go wrong. True, Gary had cut Billy’s spine without a myelogram, but such fortitude is rare. Back home, anesthesia would balk at doing her so soon after she had eaten breakfast. And the operating rooms would have been overbooked. In London, the mighty Mr. Cunningham spoke and things happened.

Mr. Cunningham and his registrar positioned Alice in the head clamp, then scrubbed her mouth and nose with soap. The pituitary, an embryonic relic of the nasal passages, lies buried in the hard palate, just above the uvula. The neurosurgeon approaches the gland through the nose, with the aid of a microscope and some truly long instruments. To enter the hypophysis (the correct name for the gland), the surgeon traverses the sphenoid sinus; hence the operation’s tongue-twisting name—transsphenoidal hypophysectomy.

First used by Cushing in the early twentieth century, the
transsphenoidal procedure is arguably the most bizarre operation in the neurosurgeon’s repertoire. To enter the nose, the surgeon cuts under the upper lip and peels the face up and away from the “nares,” the openings of the nasal passages. Cracking the nasal septum to one side, the surgeon advances a large speculum to the base of the skull, the sphenoid sinus is removed, and the floor of the pituitary chamber is chiseled away.

Cushing had little luck with this approach and abandoned it, preferring to take the tumors out through the head rather down through the nose. Superb a technician though he was, Cushing lacked a microscope, and the fiber-optic lighting, needed to do the operation safely. Jules Hardy and his colleagues in Canada resurrected the procedure in the 1960s, with superb results, and the nasal approach soon became standard. Hardy’s use of the microscope and intraoperative X rays overcame Cushing’s difficulties.

Mr. Cunningham deftly dissected the pretty woman’s face and exposed her pituitary gland in less than an hour. The gland looked blue and taut on the video screen. With a swift poke of his pointed microblade, thick clot and purulent yellow tumor gushed from the gland and into Mr. Cunningham’s thin suction tip. He spent several minutes rummaging around with small currettes, until satisfied that all tumor and blood clot were evacuated. The task of reassembling the face fell to the registrar.

Alice’s broad smile in the recovery room told more about her eyes than any formal vision test could do. A few hours after surgery, her vision returned to normal. The nasal packing was removed on the third postoperative day and she went home on the fifth—pretty as ever. Her mother never learned of Alice’s brief flirtation with disaster.

The day of her apoplexy, as I was fetching Mr. Cunningham
from his office, I noticed a wooden plaque on the surgeon’s wall. It read prophetically:
SOMETIMES SURGERY DOES HELP

I returned to the United States shortly after Alice went home. My first assignment was Children’s Hospital, where surgery often did not help at all.

O
n my return from England, the department assigned me to the division of pediatric neurosurgery at the local children’s hospital. The pediatric rotation was unpopular among the neurosurgery residents. Drawing blood from a wailing infant in the middle of the night while the mother screams “You’re murdering my baby” was just one reason. The place did have one selling point: it
wasn’t
the pain service.

The neurosurgical floor in Children’s Hospital was more of a Sesame Street advertisement than a clinical ward. Bert and Ernie stared down from every wall like Muppet versions of Big Brother. In the middle of the ward was the treatment room,
where minor procedures were performed. The very name of that place suggested an interrogation chamber. Muppet stuffed animals littered the place, as if they could soothe a child lying strapped to a table awaiting the cold steel of a needle. Once, as I was having a particularly difficult time getting blood from an infant, I kept smacking my head against a Muppet mobile which was dangling from the light fixture. Driven nearly mad by the infant’s screaming, I pulled the contraption from the light, heaved it into the corner of the room, and shouted “Fuck you, Cookie Monster!”

The infamous “swinging chairs,” in which infants rocked for hours on end, lined the halls. Nurses had the audacity to call me when one of the pendular infants puked. “Let me put you in a swinging chair for a couple of hours,” I would sneer, “and see how much of your lunchtime hoagie
you
keep down.”

A child’s cry pierces to the bone and aggravates the soul far out of proportion to its decibel level. The crying of a sick child is far worse. Eric put crying into perspective for me: “When you go to draw blood on a two-month-old, remember, he doesn’t know it’s just a simple procedure. He’s think’s you’re trying to
kill
him! He’s going to peg the meter, pull out all the organ stops.”

Three weeks into my tour of pediatric duty, I met Rebecca, the only child of a rural couple from out of state. A free clinic had transferred the six-week-old infant to our emergency room that morning for evaluation of her lethargy, vomiting, and failure to gain weight. A CT scan, ordered by the ER pediatrician, revealed a tumor in Rebecca’s small brain. As the neurosurgical resident on call, I was summoned to admit her for further treatment.

I found Rebecca with her parents in the ER’s cast room,
a cluttered cubicle used for setting bones and placing plaster casts. The cast room also doubled as a holding area for ER patients awaiting hospital admission. The infant, wearing only a plastic diaper and soiled T-shirt, squirmed in her mother’s lap and gummed a pink pacifier.

Both parents sported worn denim clothes and looked to be no older than twenty. Father paced the small room puffing a cigarette, while mother stared quietly at the floor, her pale face framed by straight, bleached hair.

Rebecca’s appearance startled me. Her bulbous, oversized head teetered unsteadily atop a tiny body, the scalp stretched to a porcelain sheen and laced with delicate blue veins. An intravenous line, anchored by a piece of yellow Big Bird tape, dangled from a scalp vein. Her rib cage bulged beneath the parchment skin of her chest. Her eyes deviated so far downward that only small crescents of her watery-blue irises were visible. The exposed white of her eyes, together with the gaunt, corrugated chest and large head, endowed Rebecca with a pitiful, buglike aspect—typical of untreated infantile hydrocephalus.

Hydrocephalus, Greek for “water brain,” results from a blockage of normal cerebrospinal-fluid flow within the brain. CSF moistens and cushions the gelatinous nervous tissue. One pint of CSF is made in the brain daily, percolating through small chambers and tunnels within the head and spine before flowing back to the brain’s surface, where it is absorped by large veins.

CSF production is relentless, a faucet with no “off” position. Any obstacle to CSF flow causes fluid accumulation, increasing the pressure on the brain. A variety of diseases cause hydrocephalus. The viscous pus of meningitis, for example, plugs the tiny CSF channels like grease clogging a kitchen trap.
Intrauterine infections, including cytomegalovirus and toxoplasmosis. scar the fetal brain’s inner cavities and produce a form of congenital hydrocephalus.

Rebecca’s hydrocephalus stemmed from a blockage of the fourth ventricle, the main drainage pathway within the brain. A tumor in her cerebellum, the crinkled hindbrain, was the culprit.

Because the mature skull is solid bone, adult-onset hydrocephalus slowly crushes the brain between pressurized fluid and the skull. The infant skull, on the other hand, is pliable, consisting of eggshell-thin plates of bone linked by fibrous fontanelles, or “soft spots.” Designed to expand slowly during normal brain growth, the baby’s skull offers little resistance to unchecked accumulation of CSF. If untreated, infantile hydrocephalus inflates a head to freakish proportions, transforming the brain into a translucent water balloon.

While hydrocephalus remains common in children, modern treatments using surgically implanted plastic shunts have reduced monstrous heads to textbook oddities, although in some rural areas, where access to medical care is limited, advanced cases of untreated hydrocephalus still exist. About once a year, a child with a grotesque head is trundled into our medical center, carried on a wagon or cart like a human watermelon. Children with uncontrolled hydrocephalus look more like Hollywood-created space aliens than human beings, with massive foreheads bulging out over tiny faces.

Hydrocephalus damages brain mechanisms controlling eye movements, deviating the eyes downward. The exaggerated downward gaze is called “sunsetting,” since only the top portions of the irises are visible. The brain’s nausea center, when compressed by hydrocephalus, signals frequent, forceful vomiting, with subsequent dehydration of the patient. In Rebecca’s
case, her inability to hold down food spurred her parents to seek medical attention.

Rebecca was not irreversibly deformed, not yet anyway. But she was in serious trouble. I approached her parents, the Hobsons, with my best professional demeanor.

“I’m Dr. Vertosick, Mr. and Mrs. Hobson, from neurosurgery,” I said as I strode into the room, Rebecca’s X-ray jacket under my arm. This introduction garnered only quizzical stares.

“Neurosurgery?” asked Mr. Hobson.

“We’re the brain surgeons,” I continued, hoping to clarify the obviously unfamilar term “neurosurgeon.” I disliked the label “brain surgeon.” It evoked silly images of Jethro Boudine, bearer of a sixth-grade education.

“Brain surgeon! What do we need a brain surgeon for?” cried the mother. She held Rebecca closer, as if to keep her out of my clutches. This family had no clue—no one had bothered to tell them about the scan.

“Your daughter—Rebecca, is it?—her scan shows a growth, a growth in her brain. That’s why she’s been spitting up all the time.” “Growth” is a good word, much better than “tumor,” or “cancer.”

Dad ground out his cigarette under the heel of a weathered boot. “What sort of growth?”

“A lump, growing in the back of the brain—here,” I explained, touching the velvety nape of Rebecca’s tiny neck, “about the size of a grape.”

Foods are the traditional yardstick for tumors anywhere in the body. “A tumor the size of” a grape, walnut, egg, melon, orange. Although a macabre practice, food comparisons allow a visceral feel for prognosis—someone with a cancerous cantelope in their chest isn’t going to live as long as someone with a cancerous pea. Although a “grape-sized” brain tumor doesn’t seem threatening, it’s plenty big enough to kill an infant.

“It may be benign,” I went on, “something we could successfully remove, or it might be something that we…uh, we can’t remove completely.” I can’t bring myself to use the word “malignant.” At least not during the initial confrontation with patients or their parents. People faint from hearing such words. My calm assurances were deceitful. I knew that infants almost never have benign brain tumors.

I coaxed Mrs. Hobson into telling me her baby’s brief history. Born after an uneventful pregnancy, Rebecca was fine for about a week before her vomiting began. A clinic pediatrician in their home state advised changing formulas, thinking the child was developing a food intolerance. This strategy worked for one or two days, but the vomiting soon resumed.

After one month of life, Rebecca weighed less than she did at birth. Her head grew large as her body withered. Since Rebecca’s father was unemployed and without health care coverage, they avoided further medical care and tried to correct the problem themselves, feeding the infant herbal teas, whiskey and water, orange drink, ginger ale—anything they thought she might hold down. Nothing worked. When Rebecca grew stuporous from dehydration, the parents finally agreed to come to us for help.

After examining the baby, I mumbled some additional words of encouragement to her shaking parents and left the room to call Dr. Wilson, the attending neurosurgeon on the case.

“It’s a cerebellar mass, lateral, enhancing, about two centimeters,” I told Dr. Wilson over the phone, holding the scan films over my head to illuminate them with the ceiling lights. “Big-time hydrocephalus, huge vents, sunsetting, the works. She’ll need to be done soon.”

“Yeah, sounds like it,” he replied. I could hear him shuffling through some papers. “I have to give a deposition tomorrow,
so put her on for Wednesday. We may need the microscope, maybe not…and schedule some brain-stem evoked potentials. How old is she?…Six weeks? Not good, not good. Must be a PNET.” PNET stood for primitive neuroectodermal tumor, a tumor of the embryonic tissue which gives rise to the neurons, or nerve cells, of the brain.

I returned to the cast room. Rebecca was crying: not the penetrating wail of a healthy baby, but the weak, catlike meowing of a brain-impaired newborn. Her mother’s eyes were puffy and red as well. Her quaking fingers fumbled to replace Rebecca’s now discarded binky. She must have spent most of the last six weeks trying to settle her starving baby in this way, and the strain of her sleeplessness was palpable.

“I’ll take you up to the neuro floor. Dr. Wilson will be your staff surgeon and he’ll talk with you later. We don’t plan to do anything more today, other than give her IV fluids and some steroid medication to make her feel better.”

I glanced at my watch: lunchtime. There wasn’t much more I could, or would, tell them at that time. I left them there, alone. Two adolescent parents and their dying child.

The goals of Rebecca’s surgery
were to relieve her hydrocephalus by removing the obstructing tumor and to confirm the diagnosis of brain cancer. If the hydrocephalus persisted after tumor removal, a permanent shunt would be necessary. The shunt consisted of a thin plastic tube, inserted just beneath the skin, to redirect excess CSF to the abdominal cavity.

Surgery on an infant brain can be a nightmarish affair. While the adult brain is soft as warm gelatin, the infant brain is even softer. What little rigidity the brain has derives from the tough insulating fat known as myelin. Myelin first appears during the third or fourth month of life, and continues forming until the nervous system reaches full maturity at age twenty-five.
A neuroanatomy professor once lamented to me that twentyone was too young an age to vote, given that the brain wasn’t even “done” yet.

At six weeks of age, the unmyelinated brain is thick soup which can be inadvertently vacuumed away by operative suctions. Moreover, nerves the thickness of pencil lead in adults are little more than a spider’s web in a baby.

Infant surgery poses other problems. The loss of a thimbleful of blood, not enough to make a decent stain on a gauze sponge, sends an infant into shock. Worse still, infants are prone to fatal hypothermia. The operating room must be kept very warm. Even the IV fluids must be at the proper temperature to avoid cooling the infant excessively. The exquisite difficulties of infant surgery have a Darwinian explanation: nature doesn’t want sick babies to have surgery, but to be buried. Civilization no longer cares about survival of the fittest, however. We want all our babies to live.

Dr. Wilson and I took Rebecca to the operating room on Wednesday, as planned. After she was anesthetized and a breathing tube was inserted into her throat, she was wrapped in foil to retain her body heat. These foil wraps gave us our affectionate nickname for sick infants: hoagies.

Since her tumor was in the rear portion of her brain, we flipped Rebecca into the prone position, with her face resting on a padded “horseshoe” head holder. In adults, we would use a pin-and-clamp head holder known as the Mayfield device to suspend the head in midair. The horseshoe, on the other hand, is just a modified pillow. In long operations, pressure from the horseshoe breaks down the skin, causing blisters, pressure sores, even permanent facial scarring. The thin infant skull cannot support the Mayfield clamp, however, making the risks of the horseshoe unavoidable.

Skin ulceration during prolonged operations is just one
example of how anesthesia differs from sleep. During sleep, we change positions every hour, protecting each body area from prolonged pressure. When injured by a contorted position, a limb becomes painful and numb, forcing us to move or even awaken. During brain operations, such sudden movements could cause the surgeon to slip, with devastating results. For safety, the patient must be kept absolutely motionless for hours.

For long operations, we position patients with extreme care, filling eyes with ointment, padding nerves, covering intravenous tubes with soft foam.

After the baby was positioned to our satisfaction, I began shaving her downy hair, using a straight razor. This resident’s job took as much skill as the surgery itself. Rebecca’s scalp was just a few millimeters thick—a careless swipe and the blade could cut to the bone.

Dr. Wilson counted any nicks made during shaving. He charged us a quarter a nick, paid as one lump sum into the residents’ research fund at the end of our rotation. Rebecca’s head shave cost me a dollar, but I made no major razor blunders.