by Brian Harvey
Water on the Brain
My father once had a wooden boat that began to leak after we’d had it on dry land to paint the bottom. The planks had shrunk enough for water to fill the bilge in minutes once we’d put the boat back in. He sat in the boat all night, pumping the ocean out and waiting for the wood to swell enough to stop the bleeding. I wonder if, during those long, lonely hours, the analogy with hydrocephalus occurred to him.
I began to visit the medical library more often after my father died (or, as he might have put it, finally fell completely apart). I spent much of one winter there trying to understand the thing that happened when the currents in our inner ocean became backed up or reversed: hydrocephalus. Hippocrates appears to have coined the name. For him, hydrocephalus meant a collection of fluid outside the brain — what we would now call a hematoma. Because autopsies were then considered an impiety, there was never any link made between cerebrospinal fluid (CSF) and the ventricles, those four cavities in the brain in which the CSF pools. The ancient Greeks and Romans knew about the ventricles all right, but for them their function was to house the soul.
Hydrocephalus was well known throughout the Dark Ages, a six-hundred-year stretch in Europe when “Western” science was kept alive by the Arabs and Turks. Avicenna’s The Canon of Medicine was one of many learned treatises from the flowering of the Moslem Empire, and the English version of Avicenna’s book that I found in the Victoria Medical Library was based on Latin ones that were in use all the way to the nineteenth century. What better illustration of how slowly science moved in those times: a text with an eight-hundred-year shelf life!
Islamic cultures shared the distaste for dissection, so Avicenna’s writings are probably based on clinical notes alone, which explains why he could not identify the exact location of the cerebrospinal fluid. Avicenna knew there was no hope for true, intracranial hydrocephalus, the kind I was most interested in. The treatments available to him for any disease seem depressingly wrong-headed: blood-letting, leeches, cupping (in which a candle was burned in an inverted glass, to create a slight vacuum). I thanked my lucky stars and moved on.
Four hundred years after Avicenna, S¸erefeddin Sabuncuog˘lu was a surgeon who wrote one of the earliest Turkish medical texts. Hydrocephalus is largely a disease of children, and it was Sabuncuog˘lu who made the first pediatric neurosurgical drawings. For him as for Avicenna, hydrocephalus seems to have meant the external type of the disease. One of his exquisite miniatures shows a female physician extracting a dead hydrocephalic fetus from a seated mother.
These were Dark Ages indeed. Physicians and frightened parents knew about early childhood hydrocephalus, the most common form of the disease, but they were powerless to cure it. Its appearance in the weeks after birth must have been appalling: the bulging anterior fontanel (soft spot); the splitting cranial sutures; the telltale resonance when the skull was rapped (still called, quaintly, “MacEwan’s crackpot sound”); the downward gaze (“setting sun eyes”) from compression of the bony plates. The infant’s scalp became shiny, the veins bulged, and the rapidly enlarging head was hard to hold up. Something was going horribly wrong, the child’s cranium blowing up like an overfilled wineskin. In these times, the physician was as much a spectator to hydrocephalus as were the parents; his treatments, as with so many in the history of medicine, proved spectacularly irrelevant. In a few cases, the disease arrested spontaneously, but usually the head just kept expanding. Then came the seizures, merciful unconsciousness, death. From the Romans to the Renaissance, a period of recorded medical experience and opinion of more than eight hundred years, this was how it was.
This shouldn’t be a surprise, because medicine has always moved slowly. My father used to relate how, in the mid-1940s, he worked on what he called an “assembly line” for electroshock therapy in a Toronto psychiatric hospital (he was in the Army Medical Corps). When the convulsions had subsided and the patient began to breathe again, “every breath was a screaming gale, and foaming saliva poured from his mouth, and sometimes the foam was mixed with blood. The attendants wheeled him out and brought in another.” That sounded pretty medieval to me.
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Hydrocephalus remained misunderstood and untreatable until the anatomists came along. These men went in with saw and scalpel and prising fingers to see what made the human body tick, falter, collapse into entropy-filled chaos. For the anatomists, if there really was a “spiritus animus,” it had to fit in with the plumbing, the ducts and valves and cavities they sliced open, traced, measured, and drew. It was like going inside the black box.
The shift in treatment of hydrocephalus wasn’t sudden — the disease remained virtually a death sentence until the early twentieth century — but it did follow the leads uncovered on those dissecting tables. The Renaissance anatomist Vesalius was the first to describe internal hydrocephalus in a scientific way. His report on a two-year-old hydrocephalic girl he dissected in Augsburg, Mozart’s birthplace, convinced him finally to break with the Hippocratic concept of the disease: fluid could be found in the ventricles. The poor girl’s brain had become all ventricle; what was left was like the rind of a grapefruit.
But progress was agonizingly slow, as it had to be in a field where experimentation was near-impossible and inferences could be made only after dismantling dead people. So there was still no hope for famous hydrocephalus sufferers like William Henry, Duke of Gloucester, next in line as ruler of Great Britain. Hope for continuing the Stuart dynasty died with William in 1700. He had mild hydrocephalus. At age five, “his hat was big enough for most men.” A generation later, Jonathan Swift may also have suffered from hydrocephalus. By 1740, the author of A Modest Proposal and Gulliver’s Travels had lost most of his memory. He died at age seventy-eight after three days of convulsions; the post-mortem found “much water on the brain.”
After a week or so in the library, I knew this: by the close of the eighteenth century, childhood hydrocephalus was known to involve accumulation of cerebrospinal fluid in the ventricles. It would be another hundred years before physicians could do much about it, but they’d made a start. The turning point came with the dissections of Giovanni Battista Morgagni, the young professor of anatomy at the University of Padua. Morgagni’s work marked the birth of the “anatomical concept,” in which disease and symptoms are correlated with observable changes in an organ — in other words, the beginnings of pathology.
On hydrocephalus, Morgagni writes about the dissection of a baby girl in whom there was “no cerebrum, but only a kind of bladder, in which nothing was contained but a kind of yellowish water.” Another infant’s brain was “hollowed out after the manner of a species of cabbage.” And another where “a portion of its substance remained, so small as scarcely to equal a little egg in bulk.” As to cause, he speculates that the “pituitary gland may be obstructed, or other passages, which serve to carry off the water collected in the brain” — so he wasn’t far off.
But really, the message I took from Morgagni was still the wretched slowness of it all. Another two hundred years after Vesalius, and we’re still describing swollen heads in terms of garden produce? Sitting in the library surrounded by the accumulated medical wisdom of two and a half thousand years, I found it hard not to think about the past — and not just the flash and sizzle of my father’s life and career, no more than a mosquito caught in the bug zapper on a hot summer night, but the drawn-out, deeper drone of ghosts long past.
I found myself conjuring the scene in Morgagni’s time: the anatomists bent over the small body on the table, the saws and knives and bowls, the oil lamp brought closer. For me, his most appalling description is of opening up a living infant in whom the bones of the skull had been “drawn asunder” by the pressure from accumulated cerebrospinal fluid. Morgagni found, with successive cuts (between convulsions), that the brain was “extended almost to the thinness of a membrane.” Medicine advanced; the child died. It was a gory business, and I wondered how my fathe
r had stood it for so long.
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By 1808, the surgeon John Cheyne was able to write his long Essay on Hydrocephalus Acutus, or Dropsy in the Brain. The title alone tells us how far we still had to go: dropsy is the old word for edema, and in those days it meant cathartic medicines were in order. So Cheyne’s poor patients vomited out emetics and voided the ghastly stools he characterized as oily-looking, glazed, tremulous, or glairy (which means, I found out, “covered with glair”). Cheyne ignored intracranial pressure. For him, hydrocephalus had to be dropsy. So his harrowing treatments continued: the leeches, the foul-smelling poultices, the blisters to the stomach and head that only acted, like Vicks VapoRub, as counterirritants.
The last great contribution from the anatomists was by the protean Swede Gustaf Retzius. Until him, people still believed the fluid was only present as a result of hydrocephalus, not in healthy people. His monumental neuroanatomy text of 1875, written with Axel Key, contained many illustrations of the crucial dye-injection experiments that finally mapped the cerebrospinal circulation. Retzius and Key’s exquisite engravings became the road map for neurosurgical advances in the next century.
But the new chart of the cerebrospinal fluid system did not prevent L. Emmett Holt, in his 1897 textbook The Diseases of Infancy and Childhood, from believing it better to refrain from any treatment at all “unless rupture seems likely.” Most of the twentieth-century writers like Holt were practising physicians, and their texts read like how-to books. In the 1912 edition of Cheyne and Burghard’s Manual of Surgical Treatment, I learned how to insert a crude permanent spigot into the lateral ventricle, to drain off excess CSF. This was the first time I came across anything that resembled the modern shunt, the mainstay of treatment until just recently. Sometimes it worked. In a few cases, skull size diminished dramatically, the edges actually overlapping to produce “a most curiously deformed small skull,” a description that brought to my mind a curled-up woodbug.
W.W. Keen, in Surgery, Its Principles and Practice (1919), went one better with a bypass that drained CSF from the lower back to the peritoneal (abdominal) cavity. This required a tricky laminectomy (removing part of a vertebra) to get the fluid out through the lower spine. It was daring surgery, a kind of permanent lumbar puncture or spinal tap.
Once I encountered terms like “laminectomy” and “lumbar puncture,” I began to feel on oddly familiar ground. Not because I knew what the words meant, but because they had been a subliminal part of my upbringing. At the breakfast table, other children might have heard about politics or the weather or perhaps the hockey scores; for us, it was my father’s offhand, “I have to do a laminectomy today,” or my mother’s anxious, “Keep it down. Your father was up all night doing a craniotomy.” I had no idea what these words meant, but I never forgot them, and it was when I began to meet them again, in the library, that I realized I was enjoying myself. Nosing around in the classics of medicine, finally finding out what a ventriculogram was (another of those breakfast-table words), reading about glairy stools and exploding heads — this stuff was interesting.
But I was still stuck in 1919. Neurosurgery, the specialty my father trained so hard for and, at the end, likened to “just being a cobbler,” hadn’t even been invented yet. I needed someone to get me from W.W. Keen to the treatment for hydrocephalus that my father first learned at the University of Chicago in 1949. There was only one person who could do that, and his name was Walter Dandy.
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The history of the treatment of hydrocephalus is all about getting rid of fluid. All those surgeons bent over swollen-headed infants seemed to me like sailors struggling to keep a leaking boat afloat — except that, for the surgeons, the boat was producing its own water. The child was drowning in its own ocean. But the solution was the same: man the pumps.
The terrible prognosis for children with true intracranial hydrocephalus stayed unchanged until the 1940s and the ultra-competitive American surgeon Walter Dandy. Dandy was the authority on hydrocephalus. My father’s copy of Dandy’s 1945 Surgery of the Brain is inscribed “Chicago, August 1947,” where he was completing his Ph.D. in neurosurgery. The pages are shiny and yellowed and well thumbed.
Much of Dandy’s information came from experiments on dogs, and reading his book brought back uncomfortable memories. Even as a child, the little I knew about my father’s research included the use of dogs and monkeys, and it troubled me then. There was no CT or MRI in those days; if you wanted to learn about the cerebral circulation (which was why my father was in Chicago), you did things to monkeys and dogs. I still have a cardboard box in the basement with his collection of monkey skulls; in many, the top half has been neatly severed, like an ostrich egg cup.
Dandy and his colleague Blackfan went through a lot of dogs, and what they did was this: inject the animal with a dye, engineer a blockage in the CSF system, then wait to see where the dye did or didn’t go. Tracing the plumbing, that’s what it was. Long before our trip with Vera, I remembered leafing through Dandy in my living room and listening to the nighttime snuffles and sighs of my own dog. Charley was the perfect size for advancing medical knowledge. His cranium fit nicely into the palm in my hand. But he wouldn’t go quietly: the Colonel Blimp eyebrows would shoot up and the facial fur erect like a bottle brush and Charley would be a salt-and-pepper blur. The doctor would be lucky to escape, lab coat flapping, before Charley got the syringe away from him and jammed it into a calf.
But it was no good romanticizing; I didn’t have a hydrocephalic child. The deaths of Dandy’s dogs, while conclusively mapping out the canals and spaces, still hadn’t offered much in the way of treatment for hydrocephalus. There’s a picture in Dandy’s book of a three-year-old boy lying against the pillow and gazing expectantly at the photographer. He has lovely eyes, a button nose, an alert expression — but above the eyebrows his head balloons like a cartoon Martian’s. I flashed back to my only experience of hydrocephalus, on a clogged, malodorous sidewalk in Bangkok where the competition for alms was so fierce, the beggars outdid each other with sympathy-inducing props. A hydrocephalic kid lay uncovered on the pavement next to someone who was presumably a parent but might just as easily have rented the boy for the day. The skin of the youngster’s head was taut, like a grape on a stick. The boy stared wordlessly at me; I dug in a pocket for damp, soiled baht.
For the hydrocephalic child of Dandy’s time, the surgeons could do little. Repeated ventricular and lumbar punctures, used to reduce pressure since the time of Hippocrates, could still end in meningitis and death. Artificial bypass drains or “shunts” got clogged and infected. So Dandy’s book offered only ten pages on treatment, and he devoted them to the then-unpopular method of puncturing the third ventricle. Here he was ahead of his time, and his operation is only now coming into its own with the advent of endoscopes that actually let you see what you’re perforating.
Dandy’s biggest practical contribution may in fact have been the procedure he pioneered called the ventriculogram. Dandy knew his ventricles, and he developed a way of visualizing them long before we had CAT scans. It was brilliant: he drained the CSF out of the ventricular system and replaced it with air so that an X-ray would show the size, shape, and any defects in those cavities. His procedure became a mainstay of diagnosis.
The cerebral ventriculogram (there is also a cardiac ventriculogram, done on the heart) is important to my father’s story. It was just about the only imaging tool he had. I remember watching him do one. As teenagers, my brother and I once gowned up and followed him into the operating room. Nobody batted an eye; in those days, the neurosurgeon was top dog. When he pierced the ventricle, CSF spurted like water from a squirt gun. Once air was injected and the patient’s head rebandaged, we all rode the freight elevator down to X-ray in the basement of the old Victoria General. The air-filled patient snored lightly on the stretcher.
Now, of course, a neurosurgeon would no more think of doing a ventriculogram than h
e would order up a few leeches to slap on the patient’s head. CT scans and MRIs reveal a galaxy where a ventriculogram might have shown a few dim stars. But in my father’s time, the neurosurgeon confronted by a hydrocephalic child did a ventriculogram. That was all there was.
Regarding the causes of hydrocephalus, what have we learned since Dandy? Most childhood hydrocephalus is caused by impaired CSF absorption or by blockages. Other common causes are cysts, tumours, and meningitis. When there’s no blockage, hydrocephalus is called “communicating,” and its main causes are intraventricular hemorrhage (the most common) and meningitis. Hemorrhage is especially common in premature infants, so prematurity and hydrocephalus often go together, a terrible double whammy. This is also important to my story, because the case for which my father was sued involved a premature infant with hydrocephalus. Nowadays, an ultrasound shortly after birth can tell doctors if there’s been an intraventricular hemorrhage; if there has, there are some newer treatment options to prevent hydrocephalus developing in very small preterm infants, or at least to keep it in check. In my father’s time, hemorrhage couldn’t be detected in the premature infant, so the temporary fixes didn’t exist.
What about modern treatments for hydrocephalus? We still deal only with the symptoms. While advances in medicine might eventually produce a real cure, pediatric neurosurgeons will probably continue to do the plumbing work-around, as they have since the 1960s. In other words, most kids will still be “shunted.” By the early 1990s, the prognosis for most hydrocephalic children was to develop with relatively normal intelligence at the cost of a lifelong shunt. All that matters to my story is that, at the time my father was operating, shunts ruled, and the cascade of events that brought him down all started with an infected shunt. So shunts deserve a little more attention here.
The first shunt was done in 1908, a rubber tube that led from the brain to the peritoneal cavity (the space around the abdominal organs), not very different from today’s preferred route. Many variants were tried over the years. Other overflow destinations included the heart, the jugular vein, the gallbladder, and even the salivary ducts — a destination I imagine is especially unpleasant, the patient having a permanent mouthful of cerebrospinal fluid. The earliest shunts my father did were connected, amazingly, to the middle ear, which in turn drained into the throat.