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.