05/29/14
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A Most Striking Image

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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

 

05/18/14
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Hydrocephalus Owner’s Manual

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Click image to download the entire Hydrocephalus Owner’s Manual as a free pdf.

Hydrocephalus: An Owner’s Manual

by Edison McDaniels II, MD

WHY I WROTE THIS MONOGRAPH

I am a brain surgeon.

Several years ago, I was confronted with a young man in the emergency room who had earlier that morning been found unconscious by his college roommates. In fact, when I met him he was essentially comatose, that is, unresponsive in any meaningful way. Fortunately, one of his roommates recalled something about him having a shunt. With this piece of information, the emergency physician quickly called for a stat head CT and a diagnosis of shunt malfunction was made. I was called, took the patient to surgery for an emergent shunt revision, and he recovered and lived happily ever after.

Well almost. It turned out he was a college student and the ordeal left him rather exhausted, though neurologically normal, and he would spend several months recovering from his near death experience. His mother, who lived in a city 160 miles away, drove over immediately and was waiting for me when I came out of the operating room. I have seldom seen a mother so grateful as that woman—unless it be virtually every other mother I have ever dealt with as a neurosurgeon.

Largely because of their children, parents are special people.

The bond between parent and child is like no other. I have seen octogenarians break down while recalling the death of a forty-year-old son or daughter—never mind that the death occurred fifteen years before. Perhaps the only bond in all of nature that can never be fully broken, it continues beyond divorce, separation, abandonment, illness, and even death. At its best, the parent-child bond drives us to be our best, to meet our full potential. Even when it is missing, entire lives are predicated, even formulated, on the basis of such a loss.

Almost without exception, the parents I meet would gladly exchange places with their child in these moments of extreme stress. These parents feel helpless and at the mercy of the situation. I am often asked What could I have done? Or Is there anything I can do to prevent this from happening again? 

I know the feeling.

I am a brain surgeon. I am also a parent. Several years ago, my oldest son died suddenly. In my years on this earth I have lost people close to me—a brother, a half-brother, both parents, several close friends—but all of their deaths paled in comparison to losing a child of my own. It was and remains the single most difficult event of my life, the defining moment if you will.

A bond which cannot be broken.

Which brings me to this monograph.

Hydrocephalus—loosely defined as a build-up of fluid in the brain—is a life-threatening, fairly common, and relatively easily treated condition. Unfortunately, with existing medical technology, the treatment requires a lifelong diligence on the part of loved ones as well as the patient himself/herself. But that being said, the treatment is not onerous on a daily basis and the benefits are dramatic. Most patients with hydrocephalus live normal lives in virtually every respect. They play sports (even extreme ones), marry, have regular jobs, carry babies through labor and delivery, and die as an old man or woman (or at least we expect they will—the technology is only fifty or so years old and thus people shunted as young children are only now reaching late middle age). With the possible exception of the more remote parts of Alaska, if you live in the United States you almost certainly have at least one friend, acquaintance, student, or co-worker with a vp shunt—though you may not know it.

So why this monograph?

Because, to put it in the simplest terms possible, failure to recognize a shunt malfunction can be fatal. The boy I took care of above had failed to get out of bed for class one morning. When his roommates returned home for lunch, they found him unresponsive and still in bed. They called an ambulance and he was taken to my hospital, where a CT of the brain showed the problem. He received prompt medical attention—but only belatedly and it nearly cost him his life.

Had his roommates known the gravity of his failure to arise that morning, his brush with death would likely have been avoided. His mother recognized this fact. She knew how close to the edge he had come. She was one of those who asked What could I have done? Is there anything I can do to prevent this from happening again?

The advice I gave her became this monograph.

Author’s note: This is not an exhaustive treatment of hydrocephalus. It’s a brief, informative, interesting, and I hope useful summation of some of the more common questions I have been asked repeatedly over the years. There are many longer, much more exhaustive books on hydrocephalus. This, however, will answer most of your questions. And it is written with the lay person in mind.

 

 

About The Author

Edison McDaniels is a writer, wordsmith, novelist, and physician living in the American midwest. His writing tends to involve ordinary people in extraordinary circumstances and is often informed by medicine. His stories showcase historical fiction and the supernatural, especially ghosts. He received honorable mention in The Seventeenth Edition of the Year’s Best Fantasy and Horror (2003), and has been published in Paradox Magazine, The Summerset Review (available online), The Armchair Aesthete, On The Premises Magazine, and others. Several of his short stories can be found online.

He is also a graduate of Stanford University and is a neurosurgeon. He is board certified in the practice of adult and pediatric neurosurgery, with over 6,000 operations to his credit.

He and his wife collect historical etchings and attend at least 1-2 baseball games a week between April and October, more if the Minnesota Twins are in town.

His novels include NOT ONE AMONG THEM WHOLE, THE BURDEN, and the forth coming THE MATRIARCH OF RUINS. His latest novella, BLADE MAN, is available as an eBook.

 

03/12/13
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Announcing The Hydrocephalus Owner’s Manual

Soon available for the Kindle.
It just might be the most important book you read. Ever.

HydroCover0What, exactly, is hydrocephalus? How is it treated? What is a vp shunt? What are the signs and symptoms of vp shunt failure and when do you need to seek help for yourself or a loved one? What are the common sense tips you can do to minimize the chance of your child having a problem when they are away from home–living in a college dorm, for instance?
 
If you or a loved one had hydrocephalus and could sit down with a neurosurgeon, what would you want to know?

This is a 6,000 word monograph on hydrocephalus, written by an experienced neurosurgeon FOR THE LAY PATIENT and his/her family. This is an essential guide for those who have hydrocephalus or deal with loved ones with hydrocephalus—a chronic condition in which spinal fluid builds up in the head and does not circulate property. Although the condition can be life-threatening & requires life-long observation, the treatment is generally straight forward and not onerous on a daily basis. The monograph includes information about vp shunts, as well as a mother’s companion and answers to common questions and scenarios.

You can also read it here for free, but it will be easier to read on the Kindle, and more convenient too. And this is the sort of thing you want to have available in a tough moment, when you might want to refer to it on the spot.

 

03/4/13
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6/6 Hydrocephalus Owner’s Manual

Hydrocephalus: An Owner’s Manual Part 6 of 6

A Mother’s Companion

 

Imaging

Generally, a CT is a better study to look at shunt function than is an MRI.

When evaluating shunt function in the ER, the size and shape of the ventricles on CT are of most significance. Being able to compare a CT obtained in the ER under symptomatic conditions to a past CT obtained for simple surveillance (during a period when the hydrocephalus was asymptomatic and the patient was well) can make all the difference in the world (and possibly prevent unnecessary surgery). Such a comparison will often make the diagnosis of a shunt malfunction much easier.

It is reasonable to obtain a surveillance head CT (without contrast; your doctor will know the meaning of this) every three years during childhood through the growth spurt at puberty. In adults, surveillance CT is obtained following a shunt revision but is rarely necessary otherwise. In reality, shunted patients are occasionally seen for headache in the ER and often a CT scan will be obtained at that time. These studies are often normal and reassuring to the physician and the family.

Most patients with a shunt and a headache will not have a malfunction and do not need a CT scan. This is a clinical decision.

It is worth remembering that a CT is a static image of one moment in time. It does not replace a careful history and physical examination.

A shunt series is often helpful. This is a plain set of regular xrays, including at least two views of the skull, chest, and abdomen. The purpose is to follow the shunt tubing throughout its course, looking for kinks, disconnections, or tubing out of place (such as when the abdominal catheter has pulled up out of the belly and is coiled under the skin—where it won’t work properly).

 

Medic Alert

Once a child starts kindergarten, a medic alert tag is recommended, ex. Obstructive hydrocephalus with VP shunt.

 

Traveling

Carrying a digital copy of the latest surveillance CT when traveling will help in the evaluation of a person with a shunt issue. Check with your local hospital or physician as to how to obtain a copy of the CT scan on CD or flash drive.

 

The Special Case of Infancy

Throughout this monograph, we have assumed the skull is a closed box with a fixed volume. However, this is not the case in infancy, up to about eighteen months of age. During this time, the head is rapidly expanding with normal growth and the skull is composed of several plates of bone, which interact with each other along what are called suture lines. These sutures are very pliable and capable of separating, allowing for expansion of the skull and the underlying brain.

Therefore, the volume of the space inside the skull is not fixed in infants. The box is open.

What this means in practical terms is that hydrocephalus in infants is manifested by an enlarging head circumference, which is why your pediatrician measures the head circumference at the well baby checks.

One can also feel along the sutures, which separate and leave gaps in hydrocephalus.

The anterior fontanelle, the soft spot on the top of a baby’s head, becomes very tense or taut from the underlying pressure.

An important finding at the time the diagnosis is made, as well as in the presence of a shunt malfunction, is that of sunsetting. This occurs when pressure inside the head affects that part of the brain which controls eye movements. The eyes are forced to look down, sometimes very forcibly. This is an ominous finding and requires emergent evaluation and intervention to prevent permanent brain injury or death.

One more thing. Although infants can get pretty sick from hydrocephalus, only in the most extreme cases do they suffer acutely life-threatening problems with a shunt malfunction (because the head just expands a little more; in untreated infantile hydrocephalus, the head is capable of truly monstrous expansion). Adults, on the other hand, with a fixed amount of space, very often have acutely life-threatening issues with shunt malfunction and hydrocephalus. There is little room for compensation in adults, in whom the box is always closed.

 

IIIrd Ventriculostomy

In the presence of obstructive hydrocephalus (but not communicating or non-obstructive hydrocephalus, and not normal pressure hydrocephalus) there is sometimes an alternative to shunting: IIIrd ventriculostomy.

In this case the surgeon creates an artificial opening between the obstructed ventricle and the CSF space at the base of the brain. This effectively bypasses the obstruction to the flow of CSF and allows it to be reabsorbed through regular pathways.

IIIrd ventriculostomy is performed through an endoscope by a qualified neurosurgeon and is only available when the anatomy is favorable. There are potentially significant complications, but the benefit if it works is that lifelong shunting can be avoided. 

 

It would be beneficial to keep the following information in one place.

My Hydrocephalus Log

My neurosurgeon is:

Recommend that every adult family member keep the neurosurgeon’s card in their wallet or purse, or on their iphone. Give the card to college roommates, landlords, and place in employee health records as well.

 

Type of hydrocephalus:

Obstructive (non-communicating)

Non-obstructive (communicating)

Normal pressure hydrocephalus (NPH)

 

The original cause of my hydrocephalus:

Congenital (born with it)

Spina bifida

Aquaductal stenosis

Hemorrhage

Meningitis

Post-trauma

 

Date of most recent shunt:

Type:

VP

VA

Manufacturer:

Programmable:

Current setting:

 

Date of first shunt:

 

Number of revisions:

 

Have I ever had a shunt removed because of infection:

Date:

End of the Hydrocephalus Owner’s Manual Series.

Disclaimer: The information contained in this blog is simply that, information. I am not doling out specific medical advice. Nothing contained herein is meant to replace a complete evaluation by a qualified member of the medical establishment. This page is nonfiction.