07/10/14
BrainVeins

Veins of the Brain

BrainVeins

A Neurosurgeon’s Perspective on the Veins of the Brain

The most curious thing about the veins of the brain is how seemingly innocuous they are, until you mess with them. They can get you in trouble in a hurry. Thin walled, they can be very stubborn and persistent in their oozing (and oozing is what they do, this is largely a low pressure system). They don’t hold stitches well, so often if damaged they have to be sacrificed. That may or may not turn out well. In general, if a vein has a name (vein of Labbe, vein of Trolard), it was named for a reason. Probably somebody in the past messed with it, usually with poor consequences.

For instance, the vein of Labbe is the largest vein draining the temporal lobe into the transverse or sigmoid sinus. Sacrificing it often results in a stoke of the temporal lobe—so much so that one rarely violated rule is ‘don’t fuck with Labbe!’ The vein is Trolard is a less constant vein, usually draining the parietal lobe up to the superior sagittal sinus.

Sinuses differ from veins in that they are designed not to collapse. They carry great volumes of blood under low pressure, but they don’t like to be occluded (stopped up). They bleed copiously and when stoppered, even for a few seconds, the brain behind them swells dangerously. Not good.

Click to see JUICING OUT on Amazon.

Click to see JUICING OUT on Amazon.

In my stories, I have written several fictional scenes of a neurosurgeon dealing with venous bleeding. Here’s one, from my novella JUICING OUT, which is just 99¢ for the Amazon Kindle (though the price is going up soon):

He looked pale. Like instead of a bullet to the brain he’d taken a vampire to the neck. His blood pressure was ninety over thirty one moment and sixty over fuck the next. If he had been awake and not anesthetized, he’d have passed out. He would have fainted and never awakened. Yes sir, he looked like a forty percenter to me.

Bobby, he drinks some but he ain’t no bad guy you know. He hardly ever do me when I don’t want it. I thought Bobby’s days of doing her were just about over.

I stole a glance at the scrub tech. She was frantically trying to get the instruments laid out and ready. I glanced at the table, saw a scalpel and a mess of hemostats, scissors, clamps, and sponges. I thought it probably wouldn’t be enough. I thought it had to be enough. Jesus Mother Mary. You wait any longer, I told myself, you’ll be operating on a cadaver.

“I can’t wait,” I said. “Help me with this, Joe.”

Together we unwrapped the diaper and the crown of his head came into view. He was mostly bald, middle-aged or better I thought. I hadn’t asked his age in the confusion, not that it mattered much now. His age would be on his tombstone after all. No confusion about that.

The skin at the back of his head was torn and bruised. I saw that much. Then Joe pulled the diaper off the hole in Bobby’s skull and the damn burst wide. A sea of dark blue, almost black, blood. For an  instant, it poured out like somebody had diverted Niagara freakin’ Falls into that broken head. Acting with twenty years of experience behind me and without really thinking, I stuffed a wad of cotton into that opening. It might make things worse, might even kill him on the spot if there was a clot inside half as big as the wad of cotton outside. That clot would press the brainstem and then, in the words of Josie’s grammie, he’d go to ground quick. But I didn’t think that would happen. Blood finds it’s own level, and, like water, is always looking for a way out, any way out. I hadn’t seen a clot on the scan (which at twenty minutes old was, admittedly, now ancient history) but the man had looked too good right up until a minute before. So I pressed that wad of cotton against the hole in his skull, against his brain, and bought us a few more precious minutes. “Get that blood, goddammit.” I hadn’t even had time to wash my hands or put on a gown.

“Getting frequent PVC’s over here.” Back flips again. The beeps filled the room, going up and down like a radio signal you couldn’t quite tune in. Bobby’s heart was losing the race. “We need blood for christ’s sake.”

The door opened just then and a pimply faced kid came in carrying a picnic cooler. If he had a clue he was carrying the man’s life in his hands, he didn’t show it. “Hey who do I give this to?”

The blood was hanging within one minute. One of the anesthesia folks (I couldn’t keep straight who was doing what and didn’t try) was squeezing the blood bag between his hands. When the first was finished, he squeezed in a second. He was on his third bag before I pulled away the cotton.

A large piece of lead floated out of Bobby head, followed by dark chunks of what could only be pieces of Bobby’s brain.

The torrent started up again. It flowed steady rather than pulsed with his heart. I knew from that, and from the amount of blood, that it was that mofo vein bleeding. And probably more than a small tear if the amount of blood was telling. I thought there had to be a hole the size of Montana in that thing. “Jesus Mother Mary” I said, then “Stitch!”

The scrub tech slapped a needle holder into my palm, a curved needle and silk stitch clamped into the end of it. I might have closed my eyes—I’ve been told I do that sometimes in surgery when I’m trying to visualize something—though if so I don’t remember doing it. I took that needle and aimed it into the pool of blood. “Suck here Joe, right here,” I said, and when I thought I could see something, something gray and not black red, I plunged the pointy end of the needle through whatever the visible tissue was and looped it out again. I cinched it down and tied it quick, then repeated the maneuver again after adjusting slightly for lighting, sweating, my own bounding heartbeat, and the regret I wasn’t wearing my own diaper. We’re losing.

An image of Josie came to me then. Josie in her Howard Johnson’s maid’s outfit, her weight in the upper limits of the couch potato zone, her unhealthy ruddy complexion. She sucked a cigarette and smelled of pinesol from the toilets she cleaned. The blue beneath her eye had coalesced somehow and now she had a decidedly black eye. Bobby, he drinks some but he ain’t no bad guy you know. He hardly ever do me when I don’t want it. I know he love me.

I fished out another piece of lead. Either the blood was slowing or whatever was left in his veins was thinning, I couldn’t say which at that moment. But I thought maybe I could see better, that Joe was doing a pretty damn good job moving that sucker here and there, sucking away the blood and oozing brain so I could work. Looking at that broken mess, looking through that thinning blood, I suddenly saw what needed doing, how there was not but one thing to do.

I saw that if I oversewed that mofo vein, it would probably kill him within a few minutes. That’d be like plugging a hose at its business end while water still flowed in from the faucet. In a few seconds that hose would rupture at its weakest point. Bobby’s weakest point was somewhere deep inside his skull, somewhere I hadn’t a prayer of getting to, and when it burst—game freaking over.

But I had no other choice. He was like a pig on a stick otherwise. I could watch him bleed out right now, or I could oversew that mofo vein and wait a few minutes for his head to explode.

He hardly ever do me when I don’t want it. I know he love me.

I thought, So that’s true love then This is for you, Josie. It’s all I got left to save your ain’t no bad guy. And I began to oversew that mother-fucker. When I was nearly done, I looked up to see Bobby’s color was better and, more important, his heart sounded a steady beep throughout the room. More important still, the puddle of blood at my feet had stopped growing and the flow out of his head had thickened and trickled. A few final throws and it stopped altogether.

“Damn fine job, Sam,” Joe said. “You can cut on me anytime.”

Want more? JUICING OUT, a kindle novella for just 99¢ over at Amazon. Give it a read today. Thanks.

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06/28/14
FirstGSWSpineLogo

The First Clinical Xray

FirstGSWSpineLogo

 

This is an historic image.

On January 1st, 1896, Wilhelm Rontgen presented the first ever xray, an image of his wife Anna’s left hand. Later that year, a young doctor by the name of Harvey Cushing—who would go on to become one of the most prolific surgeons in history and the father of modern neurosurgery, produced this image, the first clinical x-ray (they called them rontgenograms back then)—a gunshot wound to the neck and spine.

There are actually two images here, one from the front (on top) and one from the side (on bottom). Each image took an incredible thirty-five minutes to expose.

The xrays show the bullet frgament (the dark blob) either within or overlying the C6 vertebra. So which is it—within the bone or in front of it?

It’s worth recalling that x-rays are 2-D representations of 3-D space. Consider a regular photograph. Everything visible in the frame of a regular photo will be condensed to a single plane, with only the items in the forefront visible. Everything behind will be hidden. With an x-ray however, everything in the frame will be condensed to a single plane including the items in the background. What?

If I take a picture of my hand over my face, my face is hidden by my hand. You can’t see how big my nose is or how many teeth are missing, because they are in hidden by my hand. But if I take an xray of my hand over my face, you’ll see not only my hand bones, but all the bones, teeth, and various soft tissues of my both my hand and face. In fact, that’s the hallmark of an xray—it looks through what’s on top to show what’s behind.

Put another way, in a photo only the visible foreground information is used to generate the image. By contrast, in an x-ray, all of the information in the frame (hidden or not to the naked eye) is used to generate the image. 

So how do we know if the bullet is in the bone or in front of it? The answer, of course, is we have to have two images in different orientations. Fortunately, Harvey Cushing intuited this even back in 1896. The image on top is an AP image—an exposure taken from the front. The dark spot overlying the C6 vertebra is the bullet. C is the skull and D is the ribs and lungs. The image is severely underexposed, so the soft tissue does not show well (including the lungs). Of course, since it was the first x-ray of its kind, we can forgive the underexposure.

The lower image is a side view. It shows the bullet overlying the C6 vertebral body as well. Since both the AP and side (lateral) xrays show the bullet overlying the bone, it must in fact be within the C6 vertebral bone itself—and not just in the soft tissue in front of or alongside the bone.

FirstGSWSpineDetail

On the lateral image, A is the spinal canal (where the spinal cord itself lives, here it has been colored in for clarity). B is the so-called pre-vertebral space, the soft tissue in front of the spinal column. There isn’t much detail here compared to today’s imaging, but there’s enough for us to say there is no fracture of the bones despite the presence of the bullet in the body of the C6 vertebra. The body of any vertebrae lies in front of the spinal cord, and very often a bullet may be lodged there without damage to the cord itself (especially if it is a low velocity missile image, as it likely was here). 

At other times, severe spinal cord injury (paralysis) may result from a gunshot wound to the spine, even one that completely missed the spinal cord itself. This is caused by blast effect, a sort of shock wave that goes through the tissue as a result of the kinetic energy of the gunshot wound as it passes through and disrupts the anatomy. Think of it as the ripples emanating from a pebble thrown into a quiet pond of water.

Of course, it’s possible the bullet passed through the spinal cord before it came to rest in the C6 body. That’s doubtful here though, since there’s no apparent bony fracture and such a path would very likely have disrupted bone. More likely, this bullet entered the front or side of the neck and came to rest just in front of the spinal cord, within the C6 vertebral body. From a stability standpoint, such an injury is very stable (that is, it does not compromise the spine’s ability to hold the head up—the patient would not need to be externally braced). We would not operate to remove such a bullet today, unless CT showed a blood clot compressing the spinal cord. Even then, the primary goal would be to remove the blood clot; retrieving the bullet would be secondary. Of course, there might be a non-neurosurgical reason to operate, such as damage to the trachea, etc.

Occasionally, serial imaging will show the bullet’s position is not stable, that is, that the bullet is migrating around. In such a case, removal may become necessary. There is a famous case in the neurosurgical literature of a very heavy bullet migrating through brain tissue. Imagine the damage that could cause!

Was this patient paralyzed? No way to tell from the images and his or her clincial fate is lost to us.

What about Harvey Cushing, the enterprising young doctor who made the x-rays? As noted, he went on to become the father of modern brain surgery. Almost single handedly he reduced mortality in brain surgery from a wopping 70-90% to much more tolerable though still astounding 10% (today it’s well below 1%).

Harvey Cushing operated on over 2,000 brain tumors. After he lost a patient in surgery as a medical student (he was responsible for anesthetizing the patient while the professor repaired a hernia during a lecture—when the patient died, the operation proceeded anyway!), he developed the anesthetic record, being the first to use serial recordings of heart rate and breathing. Blood pressure was added later after he discovered the BP cuff on a trip to Italy and brought it back to the United States with him (standard of care is to record all of these vitals and much more in modern day surgical anesthesia).

He made numerous advances in our understanding of the pituitary gland (Cushing’s disease, a dysfunction of the pituitary gland, is named after him).

Along with William Bovie, he developed electrocautery, which is used in 99% of operations today to minimize bleeding. Without it, modern surgery would not be possible.

He also won the 1926 Pulitzer Prize for his biography of Sir William Osler (considered by many the father of modern medicine, and one of the founders of Johns Hopkins Hospital). By the way, Cushing, who manned a military hospital in France during the last year of WWI and made many contributions to military medicine and surgery as well, was present when Osler’s son Revere died of his war wounds in France.

Cushing, a chain smoker, died of heart disease in 1939 at age 70. An autopsy showed he had a benign form of brain growth called a colloid cyst—which might well have been operated on using today’s standards.

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06/19/14
NOATW Sketch

Not One Among Them Whole—Intense & Engaging

For these men & their charges, laudable pus will be the least of their worries.

An intense and audacious tale of battlefield surgery, distressed surgeons,
and the insanity of life & death in the Civil War.

“Engaging, heart-breaking, & absolutely fantastic. A terrific book.”
—D. Buxman, a top 1000 reviewer & Vine Voice at Amazon

NOATW Sketch

It is the summer of 1863, and the greatest battle ever fought on American soil is in full tilt. Southern Pennsylvania has become one great grinding stone and thousands of dead or dying are its grist. In this tilted landscape, reputations are made, careers are ruined, and men and women are driven to the brink in the wake of two armies intent on killing one another. Yet opportunity is everywhere…

For the privates and officers who fight the battle, it’s a kill or be killed world, with salvation or damnation just a bullet away…

For one undertaker in particular, the dead are a canvas, and his ability to make a body reflect the living individual is nothing short of uncanny. For Jupiter Jones, the burgeoning dead themselves are the opportunity…

And finally, for one teenage former slave, alive only because his father had the courage to bury him, opportunity comes in the form of a ten-year-old boy with a creel and only one shoe, who may or may not be a ghost…

In the summer of 1863, humanity itself is under siege. What happens amid the carnage and human flotsam of Gettysburg, Pennsylvania, will be unholy, unnerving, and all but unbearable, with only this certain: not one among them will escape unscathed.

Here, hell is in session.

And it’s the devil’s own day.

 

“McDaniels’ fine Civil War novel is not the world of Robert E. Lee or Ulysses S. Grant or even Abraham Lincoln. McDaniels’ Gettysburg is a microcosm, a seething world of its own from which no player escapes.”
— P.B. Sharp, an Amazon Top 500 Reviewer

 

~ THE CHARACTERS ~

Ezra Coffin, a severely wounded Union soldier, has never seen his infant son, and perhaps never will…

Major Tom Jersey, a Confederate officer, awakens terribly mangled in the aftermath of combat, his only companions: the wounded enemy, without whom he can’t survive, and the ghost of his son—who may be his only means of escape…

Major Solomon Hardy, chief surgeon, who stands at the tables until his health fails—then watches over his own dying son…

Major Josiah Boyd, a gifted surgeon but a flawed man. His time behind the knife may cost him everything—and his patients even more…

Captain Tobias Ellis, his courage under fire makes him a hero, but he may just be the most flawed of all—and the most dangerous…

Liza Coffin, who isn’t eighteen, but already has been a homeless orphan, a mother, and perhaps now a widow too…

Jupiter Jones, showman extraordinaire, itinerant undertaker, and reader of the dead. His healing Oil, acquired from the equatorial coast of West Africa, may be the real thing…

And finally Cuuda Monk, a teenage boy and former slave, alive today only because his father had the courage to bury him when the end came. His visions of the boy with the creel may make him the sanest man in the land—and just may be the means to all their salvation…

It is the summer of 1863 and humanity is under siege. What happens next amid the carnage and human flotsam will be unholy, unnerving, and all but unbearable.

It is the summer of 1863 and everything is about to change.

Everything.

Forever.

Not One Among Them Whole, A Novel of Gettysburg, by Edison McDaniels

Click the book cover to buy the Kindle version from Amazon for just $6.99!

 ~An Excerpt~

Surgeon Josiah Boyd leaned toward the corner, pressed his tongue against his lips, and spat. Reflex mostly, something he did without thinking whenever the need presented, which was often. The gob splattered against the wall, joining the smear of juice already there. Assistant Surgeon Tobias Ellis gave little thought to the smear of tobacco, keeping his mind on the task at hand. Like Boyd, his hands and forearms were streaked with the blood of scores of men. They looked raw, almost skinned.

“Artery clamp.” Boyd stretched a palm out in waiting. 

Tiny, the surgeon’s helper, was a heavyset kid in his early twenties, though he looked younger. His experience put the lie to his appearance. He’d spent the better part of two years—the worst part of a lifetime—with the field hospital. He rummaged in the dirty water of the basin even as he held a chloroformed mask over the patient’s face. His fingers made quick work in the cool, blood pink water, finding the instrument by feel. He slammed the clamp into Boyd’s hand with a sharp smack and Boyd squeezed the clamp around the femoral artery as Ellis lifted the great vessel out of its bed, the thrum of the pulse fighting back from within. The clamp clicked as it locked, and the artery beyond the obstruction went limp. “Ligature,” Boyd said with a practiced calm.

Major Josiah Boyd was old for his thirty-eight years. His hair had thinned up top and he hadn’t shaved in days. He was of lanky build and sallow complexion, possessed of a long drawn-out face almost ghoulish in its particulars, with prominent cheekbones high under his eyes. His lower jaw had been twice broken (once by a horse, once by a man) and poorly set. It jutted obtrusively and his teeth came together at an angle somewhat off the expected, so that the whole of his face looked skewed. His hands were large and his fingers long and spindly like the legs of a great spider. They were economical in their wanderings across the surgical field however, with no waste of motion. 

The soldier on the table lay on his back. He was insensible to the workings both around and upon him, heavy under the influence of a chloroform-soaked towel. Boyd and Ellis worked quickly at their labors, their movements looking somewhat frantic at times. They had about them a look of resigned experience, showing both intolerable exhaustion and inordinate energy at the same time. They’d been working feverishly at one task or another since first light a dozen hours before. Their efforts had made not a dent in the line of men awaiting their services. At times, they worked so fast and the wounded spent so little time before them, it seemed they were cutting the same man over and over again.

Tiny passed the silk ligature and Ellis encircled the artery twice with it, just above the clamp. His fingers blurred with movement as he tied the thread and occluded the artery. He repeated the exercise on the thinner-walled vein beside it. Tiny retrieved a pair of scissors from the basin and slapped them into Ellis’s hand even before the man could ask for them. The assistant surgeon divided the vessels—artery and vein—below the ligatures and removed the clamp.

They had cut away the soldier’s trousers and filleted his thigh to the bone midway between hip and knee ten minutes before. Now, with the last of the muscle and flesh parted and only the bare thigh bone joining the lower leg to the upper, the amputation was complete in all but fact. Only the saw cut remained.

“Capital saw,” Ellis said, and out of his side vision he caught Boyd turning to spit again. This time he had time to consider the action, something he’d seen Boyd do a thousand times in a dozen hovels just like this one. In the instant before the handle of the bone saw struck his palm, Ellis wondered at the incongruities of the man who was his direct senior. He had ‘good hands’. Goddammit that ain’t true, Ellis thought, he’s more than that, a genuine honest-to-God born surgeon. Ellis had seen a lot of men work the tables in his almost two years as an assistant surgeon and Boyd was, hands down, the best cutter out there. But it was also true the man had odd ways. Like his want to chew during surgery, which perhaps wasn’t all that bad, except it meant he was always spitting. And there was his habit of spacing out in the middle of an operation. He’d suddenly walk away from the table, turn his back to the room or go behind a wall, then reappear before too long as if nothing had happened. Except something had happened, Ellis would always think. At such times Boyd looked different. Certainly not better, and not worse (or probably not worse, he’d had occasion to think a few times—and how curious was that?). Just different. It showed most in his hands, which looked somehow, he tried to think of the correct word, revitalized? Was that it? Upon returning to the table, those hands, which had seemed worn and tired, would now be spirited and quick to perform. But it was Boyd’s eyes that bothered. Once the surgeon reappeared, Ellis always found those eyes…unsettling. As if Boyd’s eyes had developed an unpleasant ‘lag,’ a sort of disparity with his hands. As if the one had given to the other, Ellis suddenly thought. He swallowed hard then, tried to put that absurd notion out of his head. A fevered product of his own exhausted mind, he decided. After all, once a battle was joined and the bloodletting began, there was never enough rest.

Tiny put the saw in Ellis’s palm and he came back to the moment. He curled his fingers around it—they seemed to have conformed to it over the endless months of the war—and went to task on the soldier’s femur. Boyd held the meat of the leg out of the way as Ellis laid the business side of the saw against the lower end of the bare thighbone and began to run it back and forth. The blade’s teeth bit at the glistening bone with a gritty feel and flecks of ivory dust and crimson blood peppered the air as he worked. The sawing took more force than Ellis supposed it should and he made a mental note to have Tiny replace the blade before the next patient. When he’d about sawn through the whole of the femur, the remnant snapped with the pop of a dry twig and the leg fell away. Tiny stuffed a wad of lint against the bleeding stump as Boyd removed the now useless limb.

“Bone file.”

Tiny anticipated the request and passed it without hesitation. Ellis grasped the narrow, five-inch flat metal file and worked the roughened side against the sharp edges of the bony stump. When he was satisfied with its appearance and feel, no sharp edges to work through the skin later, he nodded at Boyd, who took a quick feel as well. “That’ll do,” the senior man said. Ellis handed the file back to Tiny and Boyd took up an amputation knife—its long, sharp edge might easily slice a ham—and carved away a bit of remaining muscle and flesh on the back of the thigh, until he was satisfied with the look and feel of the flap to lay over the stub of bone.

They continued to work largely in silence, with no idle chit-chat. Ellis removed the lint from the end of the sawn bone. Satisfied the wound was not oozing too much blood, the surgeons flapped the skin up and approximated the edges with several silk stitches placed an inch apart. Ellis dressed the incision with a plaster cap and Tiny fanned the man to purge the chloroform from his system. A quick whiff of liquor of ammonia served finally to bring him back to consciousness, where upon he began to groan. Judging the man was safely over the effects of the chloroform, Boyd dribbled a few drops of laudanum, a sweet concoction of opium and alcohol, on the soldier’s tongue to dull him to the agony of the hours to come. Ellis had seen its effect on men time and again. Some the laudanum slept, others it simply relaxed. Ellis himself had taken it once or twice to kill a headache. Its effect had felt something akin to salvation—removing him from the horrors of the field hospital and the tussle of war, albeit transiently. Good stuff, he thought, and dangerous. Too much of that could turn a man out.

A pair of stretcher bearers stepped forward and lifted the patient with only a bare afterthought of gentleness, grabbing him under his butt and armpits. The man’s grunts as they carried him outside to be deposited alongside the other unfortunates were lost in the chaos of the battlefield hospital. Boyd stood off to one side of the room, several men interposed between he and Ellis. Ellis watched as the surgeon put his hands out, looking at the trembling, blood soaked palms as if they might suddenly fall off. Another of Boyd’s odd behaviors. There followed a curious moment in which Boyd looked about, spotted Ellis, and slipped awkwardly out the back of the building. 

Another soldier was placed on the table. As Tiny pressed a cloth over the man’s nose and mouth and chloroformed him, Ellis probed the wound in his calf with a stiff finger, feeling for splinters of bone and the ball that had done the damage. The soldier winced, not quite under yet. As Ellis pulled his finger from the man’s innards, Boyd appeared at the side of the assistant surgeon and chuckled in an odd, not at all funny way. “The truth of the flesh,” he said, or something very much like it. Ellis couldn’t be sure. It was a small thing, but as Boyd called for a scalpel, it disturbed Ellis nonetheless and he had no idea why.

 

If you liked the excerpt, you aren’t alone… 

NOT ONE AMONG THEM WHOLE is available for the Amazon Kindle & in trade paperback. Also available in other eBook formats. Now featured at the National Park Service bookstore at Gettysburg itself.

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06/7/14
BrainSqueeze

Brain Squeeze

 

Brain Squeeze: The Intracranial Pressure Monster
by Edison McDaniels, MD

The skull is a closed box. Think about it. They don’t call it the cranial vault for nothing. Once past the age of a toddler, the skull is fused—it continues to grow towards the normal adult size, but the bones of the skull are knitted together and there is no mechanism whereby they can open again (short of the surgeon’s knife, or a terrible fracture) to rapidly expand the volume of the vault.

The box is closed and the volume within is, for all intents and purposes, fixed.

This, of course, presents a curious problem. There’s only so much room inside a closed box. What happens when that room is exhausted, that is, over subscribed? What happens when there’s more stuff filling the space than it was designed for?

Huh? What?

To understand this further, imagine a shoe box with an expanding balloon inside. With the lid off the box, the balloon simply expands outward, eventually expanding out of the box entirely. But with the lid on, the expanding balloon eventually destroys the box. Now imagine the box is not empty to begin with, that when the balloon is placed inside it, the box is already 90% full. What happens then? In that case, it’s the stuff filling the box that gets damaged first as the ballon expands to squeeze the contents of the box.

Now, imagine that box is the skull, already filled to near capacity by the brain. Picture the expanding balloon to be a tumor, or perhaps trapped spinal fluid (hydrocephalus), or maybe even a brain swelling out of control after a head injury.

Ouch. Not a pretty picture. Let’s look at this box and its contents more closely.

For starters, it turns out that for all practical purposes there are only three things inside this skull box: blood, brain, and spinal fluid (CSF).

CSF is a non-compressible, usually straw colored fluid that is largely water. It contains no cells under normal circumstances. It bathes the brain and spinal cord, providing a cushion in which these elements float.

Yes, the brain floats.

This is good and bad. It’s good in that under normal circumstances there is nothing pushing against the brain. It’s bad in that with a rapid acceleration or deceleration, like a fall or a blow to the head (think of a baseball bat, or a fist), the skull stops moving before the floating brain does. Or the skull starts moving while the brain is still standing still. Either way, the brain slams into the skull. As they say, it’s not the fall that gets you, nor even the sudden stop. It’s that you don’t stop all at once. Ouch again. 

Back to the CSF. The liquor cerebri, as it has been called, is produced at a constant rate of about 500 cc per day come hell or high water. Under normal circumstances it is reabsorbed into the venous system through the large vein at the top of the brain. The production and reabsorption of CSF are independent of each other however, and uncoupling of this fine balance can lead to life threatening problems within a matter of hours. This buildup of CSF is called hydrocephalus.

You might think the brain itself is a fixed volume, but you’d be wrong. The substance of the brain is composed of cells interspersed in a watery milieu, the so-called interstitial fluid that exists between the tightly packed cells. Both the cells themselves and the interstitial space they sit in are capable of changes in volume relative to a given physiological stress.

What kind of stresses? Simple breathing for one. The volume of the brain is reduced slightly with hyperventilation, i.e., rapid breathing. As a caveat, hypoventilation (very slow breathing) can cause an increase in the volume of the brain. This happens every night when we sleep (sleeping produces a relative hypoventilation—shallow breathing—leading to a slight increase in brain volume. Not enough to cause a problem in the normal circumstance, but if there is something else taking up space inside the head (hydrocephalus, a tumor, or even a slowly expanding hemorrhage—the chronic subdural of the aged), this slight change in brain volume while sleeping can cause symptoms (this is partly why patients with brain tumors tend to have a headache in the morning when they first get up—later in the process they often vomit in the mornings as well, after which they feel better because the act of vomiting has pushed a good deal of CSF out of the head and into the spine, relieving pressure within the skull).

Gravity is another stress. The volume of the brain (as well as the amount of CSF within the head itself) tends to be lower during the day when we are upright. Most of us sleep lying flat, however, and lying flat takes gravity out of the picture and fluid tends to flow back into the head during the sleeping hours. Patients with untreated brain tumors or chronic hydrocephalus learn that sleeping upright feels better and causes less trouble. Neurosurgeons often elevate the head of their patients after surgery for the same reason.

A much more significant stress is head injury. Physicians often refer to closed head injury, CHI. CHI is generally a blunt force trauma, like a fall or striking the head in an auto accident. Being hit by a thrown ball is another example. Gunshot wounds and stabbings are examples of penetrating head injuries. All of these cause swelling in various degrees, i.e., an increase in the amount of water in the brain tissue (not to mention hemorrhage—this is the definition of contusion, which is bruising).

Uncontrolled brain swelling can cause shifts of brain tissue within the skull. It turns out the intracranial space is not a simple box with a brain within. Rather, it is a complicated 3-D space with various shelves, nooks, and crannies. These divide the space into various compartments, each called a fossa (anterior, middle, and posterior—front, middle, and back), as well a right and left half with a large shelf of tissue between. Neurosurgeons spend years learning how to get in and out of these various fossas with as little injury as possible.

Swelling represents an increase in the local pressure within the brain. Simple physics dictates that material moves from an area of higher pressure to an area of lower pressure. The brain is no different. With brain swelling, injured tissue has a higher pressure than surrounding normal brain. These gradients are generally gradual and so the shift is small at first. But when the gradient increases (the swelling rises), shifts of significant magnitude can occur between and across the various shelves and compartments. This not only squeezes and distorts good brain, it also squeezes blood vessels, which may lead to stroke. All of these things can potentially turn good brain into bad brain. Bad brain swells, and the situation is potentiated until either the surgeon intervenes successfully or the patient dies. This is malignant cerebral edema (AKA malignant brain swelling).

So that’s CSF and brain. What about the third substance normally present within the skull, blood? Does the volume of blood change? Yes. In fact, the hyperventilation/hypoventilation discussed above also affects the amount of blood in the cerebral vessels. Hyperventilation reduces any pooling of blood, which is a very quick and effective way of reducing increased pressure in the head related either to brain swelling or tumors. This effect is short lived, hours to a day or so, but is quite potent and is used often in emergencies to temporize on the way to the operating room. It is one of the reasons that head injured patients are intubated so quickly.

The caveat of the above paragraph is that hypoventilation (under ventilation or shallow breathing) can kill. Hypoventilation allows pooling of blood in the head and, in the setting of brain swelling, that is very dangerous. One place this can occur is in the CT scanner in the first moments evaluating a head injured patient. This is another reason such patients are intubated early in their care, not so much because hyperventilation is necessary, but because hypoventilation is so bad.

So, the three most frequent occasions when there is a problem with the volume of brain tissue itself are tumors, brain swelling from head injury, and aging.

A brain tumor may be thought of in terms of an expansion of brain volume or brain tissue. As the tumor enlarges, it takes up valuable space inside the so-called cranial vault, space normally occupied by the normal brain. As the tumor grows, it displaces normal brain (and spinal fluid). If the tumor gets large enough, this displacement leads to shifts in brain matter (for the same reasons as above). These shifts are known as herniation. Herniation is a situation in which brain tissue is displaced out of it’s normal position. Sometimes this herniation is relatively benign and serves as a marker that there is a problem. Though the herniation itself might be relatively benign, if allowed to continue it may lead to either stroke or death—so not so benign afterall. Other types of herniation are even more significant, leading to pressure on the centers of the brain controlling heart rate, blood pressure, and breathing. Obviously, such herniations are immediately life threatening. These are sometimes irreversible.

Another situation in which blood becomes a problem inside the head is with hemorrhage (intracranial bleeding). An expanding intracranial hematoma can rapidly become lethal. This may take the form of a post-traumatic hemorrhage (epidural or sudural hematoma, hemorrhages outside the brain proper) or a spontaneous hemorrhage, which is usually within the substance of the brain itself. This latter is a form of stroke and is caused by the rupture of a small vessel within the brain substance. Sometimes such a hematoma will be amenable to removal, sometimes it occurs in an area where it cannot be reached without leaving a person devastated from a brain function perspective (in such a case, surgery is usually deferred).

Not only is the size of an expanding mass inside the skull important, but the rate of expansion of the mass is equally important. I have seen a slow growing benign tumor the size of a softball cause little in the way of problems (the young woman presented with a lump on her head she was curious about, no headaches or other symptoms!). Benign tumors can grow very, very slowly, taking many years to achieve a clinically significant size. For this reason, when such a tumor is discovered in an elderly person (whose brains have lost volume due to aging and so have extra room already), neurosurgeons often chose not to remove the tumor unless it shows evidence of significant growth over time. Depending on a person’s age, a slow growing tumor may not grow fast enough to become a problem during a person’s life.

On the other hand, an expanding epidural hematoma after a fall or other head injury may cause life-threatening problems at a relatively small size of just 25-30 cc. Sometimes a lucid interval occurs after a head injury, often seemingly minor, during which blood is accumulating in the skull until it reaches a symptomatic size, by which time it may be too late. The classic case is an epidural hematoma (bleeding outside the brain and just under the skull). Natasha Richardson, the actress, was in a lucid interval when she declined medical aid after suffering what was thought to be a minor head injury while skiing. Somewhat later, perhaps an hour or two, she collapsed when the hematoma finally reached a size where it caused one of the above noted herniations.

So, to recap. First, the skull is a closed box. Second, there are only three things inside the box: blood, brain, and CSF. Third, an increase in the volume of any one of these three substances (blood, brain, CSF) leads to a compensatory decrease in the volume of the other two. And finally, once these compensatory decreases are exhausted, the intracranial pressure rises and shift and herniation is the result. If this badness cycle isn’t interrupted at some point, death or stroke is the result.

The last paragraph is a statement of the most fundamental doctrine in all of neurosurgery, the Munroe-Kellie Doctrine. This is learned by every neurosurgery resident during the first week of training and never forgotten. In fact, there is nothing a neurosurgeon does inside the head where he or she does not have to take the Monroe-Kellie Doctrine into account.

Stated another way, as a neurosurgeon, with every intervention I make involving the brain, I have to consider the effect on intracranial pressure. Not to do so invites catastrophe, and preventing catastrophe is what treating brain squeeze is all about.

Oh, one more thing. I am often asked what happens after we lose CSF in surgery. The short answer is, nothing. As I noted above, CSF is produced at a constant rate of about 500 cc a day, which is about the volume of CSF in the head. Thus, anything lost at surgery is replenished in a matter of hours. Until then, the brain sort of sits like a lump inside the head. This might well cause a headache, though it doesn’t seem to be too bad in most patients. Sometimes nausea as well, though it’s difficult to say if this comes from the anesthesia meds, the removal of the tumor itself, or the loss of spinal fluid. Either way, the situation resolves itself before the patient goes home. This is one reason most patients don’t go dancing the night of surgery, though many are able to walk the halls of the hospital by the morning after surgery.

If you found this article interesting, please leave a comment.

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06/6/14
JoanBooks

The Anna Donovan Novels

THE ANNA DONOVAN NOVELS
by Joan La Blanc

JoanBooks

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The Anna Donovan novels are Joan La Blanc’s linked four-volume series of carefully researched and beautifully written historical romances about young nurse Anna Donovan’s life before, during, and after World War Two.

INNOCENCE OF ANGELS – The first in the series.  In spring, 1941, as war looms, Anna Moss’s life seems on course for a secure, comfortable future. Not only does she have a good job as a nurse in a naval hospital, she’s engaged to a promising young writer who shares her values of diligence, thrift, and moderation in all things. But then she meets Dan Donovan, a new patient on her ward. Intrigued by the handsome but brash young submarine officer, she’s challenged to experience more of life than she’s previously dared. Eventually unable to resist, she’s caught up in a wartime world where the romance is heady, the risks great, and the realities stark. Yet even as she faces loss, sorrow, and an uncertain future, she’s comforted by the knowledge that she’s followed her dreams into a love deeper and richer than she ever imagined. Available in ebooks on KindleNook, and Kobo, and as a quality trade paperback . . .ask at any bookstore (or order direct here for only $13.95 us).

MINISTRY OF ANGELS – In October, 1943, after the loss of both her Navy officer husband and the baby they were expecting, 25-year-old widow Anna Donovan leaves her parents’ New Hampshire home to work as resident nurse on a remote, isolated island off the coast of Maine. Hoping to find new purpose for her life, she moves into an hold hotel operated by another young woman with her own sense of loss. Their friendship is tested when a German U-boat blows up offshore, and Jean becomes involved with a fugitive enemy sailor. Meanwhile, Anna is increasingly attracted to Dr. Jim Millett, the crippled doctor she works for, but with no expectation he’ll ever share her passion. Tragedy draws them together briefly, but soon dashes her hopes of ever winning his love. Or maybe not . . . Available as an ebook in Kindle,  Nook, andKobo . . . and soon to be a quality trade paperback as well.

ODYSSEY OF ANGELS – To the World War Two battlefield casualties evacuated from bloody Pacific beachheads to the clean white beds of hospital ships, the nurses who care for them are angels. For young Ensign Anna Donovan, however, the stresses chip away at her morale, revealing a side of her that’s anything but angelic. Far from family and friends aboard USS Compassion, she’s horrified to find herself drawn to a charismatic but married Navy chaplain. As the war reaches its bloody climax, Anna soothes her conscience by rationalizing that all that really matters is that she performed her duty. But is her plan to return to her interrupted civilian life, with the man she really loves, just a delusion? And when she leaves the ship, the friends she served with, and the man who shared her illicit passion, what will happen in that bright postwar future so many died to win for her? Available in ebooks on Kindle,  Nook, and Kobo and as a quality trade paperback . . .ask at any bookstore(or order direct here for only 15.95).

ORDINARY ANGELS – Coming home at the end of World War Two, Anna Donovan jumps into marriage to Dr. Jim Millett, to whom she was engaged before she joined the Navy. But she’s still caught up in recollections of her wartime hospital-ship romance with the Rev. Mark Whitmore.  Even as her fondest plans come to fruition, the shadows of her past darken her wonderful new life. Forced at last to confront her ghosts, Anna suffers a breakdown that threatens to rob her of everything she holds dear . . . unless she can must the courage to confess.  Available in ebooks on Kindle and Nook (And out soon as a quality trade paperback . . . watch this site!).

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06/6/14
LowerEastSide

Autobiography of the Lower East Side by Rashidah Ismaili

Ismaili is an internationally-renowned poet, and her mastery of language shows!

LowerEastSide

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“This well established poet makes a brilliant debut in fiction with these complex, poetically detailed, interrelated stories of Blacks from Africa, the Caribbean and the USA who converge and form an artistic community in the early 1960s in the most easterly regions of Alphabet City .”
–David Henderson, author of ‘Scuse Me While I Kiss the Sky

Autobiography of the Lower East Side is a  novel in short stories, set in New York during the late nineteen-fifties and the turbulent decade that followed.  Inhale the exotic spices from tenement hallways, smell the sweat and garbage in the streets, feel the sweltering heat of summer in the City. Taste the texture and densities of African dishes: the rice and pepper sauce, stewed fruits, tagine, okra soup, bread and fish. Walk the alphabet streets in the daytime, weaving among pushcarts, or at night in the biting winds of winter, footsteps too close at your back. Sway to the cool jazz. Groove to the lilt of African voices reciting poetry, intoning prayers. Follow a junkie riding out a Jones, an anarchist handing out pamphlets, a pacifist leading a draft resister on the Underground route from New York City to Canada. 

Ismaili is an internationally-renowned poet, and her mastery of language shows! Her richly-evoked setting presents characters learning to survive in the jazz scene, the theater, and the arts while dealing with interracial relationships, abuse, addiction, and the toll of the Vietnam draft.

Available now in ebook on Kindle, Nook, & Kobo, as well as trade paperback.

 

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06/5/14
Harlen_HopeYouGuess

Heather Harlen: Hope You Guess My Name

Harlen_HopeYouGuess

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Visit Heather Harlen at www.heatherharlen.com

“Ms. Harlen captures the heartbeat (and cholesterol) of Northeastern Pennsylvania in her expertly plotted thriller. I loved the reluctant gumshoe—spunky and fallible Marina Konyeshna—with her weakness for pocketbooks and shoes, and went to sleep wondering what on earth she could get herself—and her family and friends—into next.”
— Sara Pritchard, author of HELP WANTED, FEMALE

There’s no place like home…especially when it’s the center of a horrific criminal enterprise.

Event planner Marina Konyeshna is a tomboy in peep toe pumps who can plan both elegant soirees and adrenaline-pumping skydiving birthday parties. Unfortunately, her quarter-life crisis is in full swing: she’s crashing on the sofa bed in her mom’s basement, her career at Prestige Events is veering toward disaster and her Adderall prescription needs to be refilled but she’s low on cash. 

To make matters worse, Marina witnesses a terrifying assault and discovers the body of a young girl on the banks of the Susquehanna River. It’s soon clear the two events are related, but she’s forced into silence by the thugs responsible. 

Enter a mysterious and gorgeous client from Turkey. Arman Ocalan, a wealthy construction company owner, takes Marina out on a date and sparks fly; but Marina’s boss’s boyfriend warns her to stay away from Arman and his “connections,” leaving her confused.  When Arman invites Marina to form a team for an elite geocaching event, she can’t pass up the prize money.  As their team’s adventures in Northeastern Pennsylvania unfold, neither Arman or the competition are what they seem and the fates of six strangers depend on Marina, her two best friends and Arman coming in first.

If plucky heroines had their own secret society, Marina Konyeshna, Stephanie Plum and Bridget Jones would all know the handshake. Ranked five stars on both Amazon and Barnes & Noble!  Only 5.99 from Kindle and Nook, with a trade paper edition planned for this fall.

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06/5/14

John Koloski: Empyres Bloodblind

Voted the Best Paranormal Book of 2013 by Paranormal Cravings!

Koloski-Empyres

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Click here to visit John Koloski’s website.

 

If Richard Matheson and H. P. Lovecraft had ever collaborated on a modern horrornovel, they might have produced one like this:
    Driving through the night at 95 miles an hour, 32-year-old artist Adam Morrow has a lot to live for. Yve, a gorgeous girlfriend who loves him; a powerful sports car; and Patrick, a buddy who kills for him without question or hesitation, in the back seat.
    But every blessing hides a curse. The Corvette is stolen. Adam is blind. A crash years ago killed his fiancée, Leiko Hunter. The crash was caused by empathic vampires, who wanted him dead. Empyre leader Sterling Richards meant to kill Adam and take Leiko as his bride. But the plan went horribly wrong.
    Yve, beside Adam in the Corvette, is also an Empyre. She’s pregnant with Adam’s child. And Patrick, an ex-con and serial killer, has sworn to obey him only until they reach Philadelphia. Will Patrick kill him then?
    Not likely, because Adam’s already been dead for a week.
    Now, pursued both by police and Richards’s murderous Empyres, the three of them race toward Philadelphia, where a hidden cure might restore Yve’s life and his own. Amrita, an Empyre elixir, promises Adam, Yve and their baby a normal future. It makes the living immortal, and even brings the dead back to life. The key to finding it lies in Adam’s painfully fragmented recollections of the accident that blinded him and killed Leiko. Reliving those memories might be worse than dying.
But the real struggle isn’t to save Adam, or even Yve: it’s really a fight to save their unborn child . . . the already-conscious, half-human fetus, gifted with unimaginable power, that they’ve begun to know as ‘Shassa.
    Beautifully crafted, unfailingly imaginative, and spellbindingly tense from beginning to end, EMPYRES: Bloodblind marks the debut of a stunning new talent in fantastic horror. Voted the Best Paranormal Book of 2013 by Paranormal Cravings! 
Available as an ebook from NookKindle, and Kobo, $3.99 US.  Also available as a handsome trade paperback at $15.99 US from Northampton House Press.  Order through any bookstore

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06/2/14
baby smile HealthTap

I’m now on HealthTap.com

What has 55,000 doctors and no waiting room?

baby smile HealthTap

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55,000 physicians with a combined total of 28,000,000 years of experience at your fingertips.

An interactive format in which you can actually get answers to your most important questions—usually within just a few minutes—and direction on where to go, who to see, and how urgent the matter at hand truly is.

Health tips and other useful information.

Doctor recommended health & wellness apps.

Oh, and 14,062 lives saved at last count.

And it’s all free.

 

Yep, you read that right. FREE.

The future of medicine is here. This stunning interactive website not only represents the best of humanity working together with modern technology, it is quite simply the holy grail of 21st century medical care: people helping people to live better, longer, smarter, healthier, and more compassionate lives. In a world where, in the not too distant future, nearly every person on earth will be part of the online community, HealthTap.com provides a service so intuitive—and so indispensable—it beggars the mind to figure out why it hasn’t evolved before.

The model is simple. They connect patients who have questions with health care experts—board certified physicians of every specialty—who have answers. In doing so, they get patients talking to doctors again. And without the intervening layers that usually insulate physicians from patients. Things like fees. And insurance paperwork. And office overhead.

This is affordable care at a cost that can’t be beat.

Wondering about that rash on little Jonny’s rear? Text a few words their way and before long, less than an hour anyway, you’ll have a direction to go. They might not be able to tell you exactly what it is, but they will be able to say if it’s worrisome or urgent, needs immediate attention, or is something to discuss with your pediatrician next week.

Wake up with a headache this am? Thinking it’s a brain tumor? Get your fears alleviated here. And if it does sound serious, they can tell you how to go about getting it checked out—what tests are usual, what questions are important, and what specialist to see.

Wondering if you can get HIV from drinking from the same glass as someone who is positive? Need to know if it’s ok to do gymnastics with a ventriculoperitoneal shunt? How long do you have to wait after a heart attack before having sex? What is erysipelas? Is blood in the urine normal three days after your period? How come I can’t see out of my left eye sometimes? Maybe you just want to know what side of your belly your liver is on. Or perhaps you need some clarification on something your family doctor told you this am in her office? Or some terminology you saw on your CT report.

All these questions and many, many more are not only answered—they are answered in a timely manner and often by multiple docs. This means you get multiple opinions. And here’s something else worth knowing: when the docs aren’t answering your questions, they’re critiquing the answers of other docs. That’s peer review folks, and it keeps doctors on their toes. That means you get the best advice out there.

Fast, free, and from anywhere you can connect to the internet.

55,000 doctors. 28,000,000 years of experience. Sign up today at HealthTap.com and get informed.

One more thing and full disclosure. If you’ve got a neurosurgical question you’d like me to answer, contact me over at http://www.healthtap.com/surgeonwriter. I’ll be happy to chat with you. My advice will be generic, but damn interesting. 

Click here to ask me a clinical question at HealthTap.com. 

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05/29/14
SONY DSC

A Most Striking Image

SONY DSC

 

A Most Striking Image
by Edison McDaniels MD

He lies on a bed, listless and naked as the day is long, the lone hand of a kind woman (a nurse? or perhaps his mother?) supporting his feeble, overly large head. Despite the sepia hue of the old photo, it is not too much to imagine his skin a sickly milk pale, his complexion sallow. He is too quiet, does not coo, and his cry is mostly a mewl. A kitten bleats louder. We can almost hear an old time gramophone scratching out a tinny version of Brahm’s lullaby in the background. If so, he gives no indication he hears it.

His scalp is thin and veiny. The spidery vessels are visible throughout, delicate streams of blue under the all but translucent skin. His hair has yet to grow, so there is nothing to obstruct the view of his bossy forehead and overgrown braincase. We can almost see the separation of the underlying skull plates, the too large and too taunt spaces between them. Indeed, the skull is overinflated, some would say grotesquely so, with the forehead protuberant and the tiny face an afterthought the way it occupies only the lowest fraction of the front side. The ears appear set too low, the crown too high.

But it is the eyes that are the most part of him. They are, quite simply, stunning. Our own eyes are drawn to them as if tugged by some unseen hand of the Almighty. There is just a hint of how big and black the pupils are, despite being buried in the lower eyelids so that the meaty whites are too visible, too obvious. Like mad doll’s eyes are those eyes. It is obvious they are forcibly deviated downward. This sunsetting is the most striking quality of the image, and conveys life to what is otherwise an unbidden stillness.

These eyes are distinctive to the point of menacing. They telegraph danger. This is a child in dire trouble, a child with infantile hydrocephalus.

At the time this photograph was taken, very likely sometime in the first or second decade of the twentieth century, hydrocephalus was akin to a death sentence. Hydrocephalus is an over accumulation of cerebrospinal fluid (CSF) in the head. The problem is one of simple plumbing. 

CSF is 98% water. It was first described by Hippocrates in ancient times, who recognized its presence but generally attributed it to disease. Even such later luminaries as Galen, Leonardo da Vinci, and Vesalius were not aware it was normally present in and around the central nervous system. A true knowledge of its significant position in neuroanatomy and physiology had to wait until the late 18th and early 19th centuries. 

CSF is normally produced within fluid filled voids within the brain, the so-called ventricles. The CSF circulates outward to bathe the brain, and is reabsorbed into the venous system over the top of the brain. Unfortunately, a number of choke points within this system serve as places where the normal circulation of CSF can be obstructed. Like water trying to flow through a crimped hose, the fluid cannot easily pass and the resultant buildup of CSF behind the obstruction (CSF is continuously produced at a steady rate independent of its reabsorption) balloons the ventricles and distorts and disturbs the brain.

The pathologic buildup of CSF is common to all forms of hydrocephalus. In the adult, this buildup can prove rapidly fatal since the skull is a solid box and cannot expand. The building fluid rapidly squeezes the brain against the skull, a fatal circumstance if not relieved.

But in infantile hydrocephalus, the infant skull is not fixed in volume because the skull plates—collapsible in order to pass through the birth canal—have not yet fused solid. Such fusion normally happens between 14 and 18 months of age. Until then, accumulating CSF distorts both the brain and the skull. The immature skull is capable of expanding to truly massive proportions, several feet in diameter in the worst cases. 

Untreated infantile hydrocephalus does not kill immediately. The expanding head becomes like a giant bag of water, one which can be trans-illuminated with a flashlight in many instances. The brain is squeezed and thinned and squeezed and thinned until just a ribbon of brain is left against the bony confines of the enlarged skull. As if the brain were nothing so much as dough and the building CSF a kind of rolling pin flattening it out. This is the very definition of trouble.

Such a dire situation causes unfathomable damage over time, of course. These children become listless and dull, unable to keep food and water down. They show an apathy to their environment, with little or no response to sounds or visual cues in the later stages. The increasing pressure inside the head presses down on the upper brainstem, against an area which controls eye movements. This leads first to an unyoking of the eyes (they look off in different directions; if the child could talk he’d perhaps complain of double vision). Later this pressure produces the characteristic downward deviation of the eyes as seen here. This is an ominous finding, suggesting the hydrocephalus is far advanced.

By this time there is another complication. The building pressure has also damaged the vision severely. Looking into these eyes with an ophthalmoscope, the doctor would see a pale, dusky, atrophic optic nerve—like a dried and shriveled prune. The optic nerve is an extension of the brain (the only part of the brain normally visible from the outside!) and its decayed condition is further evidence of the damage within. Indeed, these eyes don’t react to light, the pupils don’t constrict.

This child is very likely blind.

Hydrocephalus was largely fatal until 1960 or so. Although the problem was well described in the first part of the twentieth century, a definitive treatment proved surprisingly elusive.

The need to divert CSF out of the head for absorption elsewhere in the body was recognized, but where exactly? Just about every part of the body has been involved in one treatment or another for hydrocephalus. Physicians have tried diverting the fluid to the chest, abdomen, pelvis, and heart. One early treatment had the fluid diverted to under the scalp, where it became a bag of water outside the skull (there was not enough absorptive capacity however and the fluid simply accumulated under the skin).

A rather drastic solution, popular in the 1950s but no longer used today, was to divert the fluid to the urinary bladder. This involved removing (and sacrificing) a perfectly good kidney however (not an easy operation in itself) and passing the shunt tubing from the lumbar CSF space into the ureter (the natural tube from kidney to bladder). In addition, patients lost excess salts and water because the CSF was just peed out and not absorbed. Such a treatment would be anathema today.

Another popular treatment was to place the end of the shunt into the heart. This is called a ventriculoatrial shunt and is still used on occasion today. It has several disadvantages, chief among them being it requires periodic lengthening since the shunt does not grow with the child, but is useful in particular circumstances when the belly cannot be used.

It’s also possible to place the shunt into the chest, where the fluid spills out around the lungs. Not ideal, but again useful in certain situations. This is a ventriculopleural shunt.

By far the most common shunt in use today is the ventriculoperitoneal shunt (VP shunt). This shunt runs under the skin from the ventricles of the brain to the belly. Specifically, the end of the shunt floats in the space around the intestines—not inside the intestines or stomach themselves. This is a very useful shunt.

It wasn’t until the 1960′s that the first reasonably acceptable shunts were produced. These remarkable devices incorporate both a valve to prevent over-shunting and a hollow tube of inert material (usually silicone today) the size of a wet spaghetti noodle. The CSF is thus diverted past the obstruction to the space around the intestines, where it is reabsorbed into the blood stream just as it would have been in the head.

Today, treatment of hydrocephalus is straightforward, but requires a lifelong diligence against malfunction and infection. For most individuals these issues are not a significant problem. Most people with a ventriculoperitoneal shunt (VP shunt) live normal lives, with normal brain development.

It is very likely that if you live in the industrialized world, you know or work with somebody who has shunted hydrocephalus, though you may not know it. That’s a striking example of how far neurosurgery has come since the days of this unfortunate child, when hydrocephalus was essentially 100% fatal.

Author’s note: There are still many places in the world today where hydrocephalus is a major health concern, as a simple google search will show. In East Africa for instance, there are more than 6,000 new cases a year, most caused by infection. With about 1 neurosurgeon per 10,000,000 people in East Africa, most cases do not make it to definitive care. Those that do still have to contend with the possibility of a later shunt malfunction or infection. India is another example.

indian-baby-2

Comments appreciated.

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