PART TWO
A Mystery
A patient presents with a new headache that occurs shortly after assuming an upright position and is relieved by lying down. Although such a positional headache pattern is well-known following a diagnostic lumbar puncture, the spontaneous onset of an orthostatic headache is not well recognized and the patient may be diagnosed with migraine, tension headache, viral meningitis, or malingering. This has been a typical scenario for many patients experiencing spontaneous intracranial hypotension . . . [and] an initial misdiagnosis remains the norm. Unfamiliarity with spontaneous intracranial hypotension among physicians in general and the unusually varied spectrum of clinical and radiographic manifestations may all contribute to a delay in diagnosis that often is measured in months or even years and decades.
—Wouter I. Schievink, “Spontaneous Spinal Cerebrospinal Fluid Leaks and Intracranial Hypotension,” JAMA, 2006
7
The days pass by in a series of blinks and I fumble my way through an appointment with a neurosurgeon the ENT suggested I see. My sister drives me, lets me lie down in her car, helps me joke about my weird leaking brain. The neurosurgeon listens to my now rote recitation of my history, thumbs the file from the ENT, and says sure, this probably really is a CSF leak, and says sure, the through-the-face surgery to fix it is probably fine—if that’s really where the leak is. If not, the surgery could actually cause a new leak, and there’s also a significant risk of my losing my ability to smell. But it’s up to me. My call.
I blink and then I am somehow back at the hospital, waiting for the next MRI and CT, a nurse taking pity on me and allowing me to lie in a free bed while I wait for the CT, while they insert the IV I need for the MRI, while they fix it after it fills with blood. I’m given powerful antibiotics in advance of the through-the-face surgery that it is my call to have done. It is some kind of fluoroquinolone, and I take only one pill before I notice my previously injured ankle tendon feeling strange, and then google to see if that could somehow be a kind of side effect and discover a host of warnings about the dangers and risks of fluoroquinolones (including tendon problems), and I stop taking it.
I blink and days later there is a flurry of activity, sudden phone calls and intense consultations, when the CT scan of my neck is shown to have revealed a secret fracture—an old injury to the second cervical vertebra. The first vertebra, C1, is where the skull attaches to the neck. The C2 vertebra, sometimes called the axis vertebra, allows the head to rotate. It is actually connected to C1 by a tooth-like protuberance called the dens, and it is this, the dens, that has appeared on my CT scan as damaged, fractured somehow, long ago. Looking up information about this anatomy, I’m pleased to read that the C2 and its toothy dens has a rare nickname: vertebra dentata. There is concern, however, that this fracture may be the source of my leak, that this vertebra dentata has in fact somehow chewed through my dura. But this seems unlikely to the ENT; he is more concerned with the shadowy spot around my ethmoid sinus. He recommends further CTs and X-rays of this old C2 fracture, a consultation with yet another neurosurgeon, all things that can be done after my surgery.
I show up the day of surgery and go through the motions of filling out forms, being checked in. Gil is there this time, and he handles the things that are too complicated, answering questions for me, doing the paperwork. I’m able to lie flat on a bed in the pre-op area, and this is a relief. I’m told the surgery will involve first pumping some glow-in-the-dark stuff up through my spine, which will evidently make anyplace in my head with a tear or leak light up, and then some kind of mesh being inserted and positioned over that lit-up area and secured in place. Then, after everything is sewn and patched, my intracranial pressure will be monitored to make sure there’s enough of it, and not too much or too little. The whole process will require me to spend four or five days in the hospital. None of this seems like a good idea. But I’m not sure what else to do about this leak in my head, and shouldn’t the doctors know better than I do how to fix it?
I’m waiting and waiting on a bed in the curtained-off room, hooked up to IVs in my hospital gown, and the ENT comes by from time to time, checking in. “Still leaking?” he asks, and I recognize this is supposed to be a joke. “Just checking!” he says, and continues, “You know, if you don’t have a hole in your head before I go in, you’ll definitely have one by the time I’m done. Think about it.” He taps the protective guard rails on the bed and strolls out. I’m concerned. I talk to Gil. None of this seems okay. The next time the ENT strolls by, we ask what the hold-up is, why the wait, and he says it’s almost time, and again introduces some doubt into the process, reminding me that this is my call. I don’t like the feeling I’m getting, like this surgery is a thing I’m forcing him to do. It doesn’t seem right, and I feel less confident that this is the right decision. He and Gil confer, and Gil says they’re waiting for
