Perm; Tracking Variables

The spinal MRI was done on a Sunday morning.  For future reference, asking a 9 year old boy to sit still for 2.5 hours first thing in the morning is a really, really bad idea.  In our defense, we were told they were scheduling 90 minutes of magnet time and could probably do it more quickly since they were doing the cervical, thoracic, and lumbar series back-to-back.  And like a total novice, I figured MRI time was expensive enough that I actually believed the scheduling receptionist.  Ha.  Future MRIs will all be done as the last appointment of the day after as active a day as he can tolerate.  It wasn’t that he was in any way rambunctious, it was just that the tiniest of movements create blurry MRI images, requiring that segment to be repeated.  It’s been 3 months and Christopher is still adamant he won’t ever do a full spine MRI again.  We did, however, discover that there are no syrinx in his spine at this time, although there's roughly 50% odds Chiari patients will develop syringomelia in their lifetime.

The eye appointment was, from my perspective, far worse, though.  His eyes had to be fully dilated so that the ophthalmologist could see as far back along the optic nerve as possible.  She didn’t find any evidence of present or past damage, although she qualified it by saying she can only see so far, and it’s possible for damage to be present posterior to what she can observe.  She felt that the visual disturbances were migraine auras, and felt confident he’d be prescribed a migraine preventative when he saw the headache specialist—but couldn’t herself as she treats eyes, not heads.  Christopher didn’t like the eye exam, but the nightmare started when he walked into bright light and had to ride home.  At one point, he asked me why he was seeing rainbows on his hands.  By the time we got home, the headache was debilitating and he spent the rest of the day curled up on the couch in the dark.  I came very close to taking him to the ER that night, as he had a couple pretty scary moments, but chose instead to see if he could sleep it off.

Come morning, he wasn’t any better and his ears were hurting badly, so off we went to the pediatrician.  She diagnosed him with an ear infection and prescribed antibiotics, but again, couldn’t do anything for the headache.

Kirov; Johns Hopkins Provides a Second Opinion

As much as I innately liked our the pediatric neurosurgeon we already had, I still had in my head my physician friends’ warning that Chiari malformations are often over diagnosed.  At the same time our pediatrician told us he thought Christopher needed to be hospitalized to get a definite diagnosis as soon as possible, he also told us we ought to go to Johns Hopkins University Hospital if the Chiari were confirmed, because Ben Carson had assembled the best team in the world for treating them.  So I made an appointment, and off we went to see Dr. Mari Groves, a pediatric spinal neurosurgeon.  Her PA for the day, Stephanie, took a very detailed history and had Christopher complete his most thorough neurological exam to date.  

Dr. Groves, however, isn’t as warm and appealing as Dr. Moores.  I’d describe her as quietly competent.  She definitely agreed that he has a Chiari I malformation, and she explained that there are several potential complications of the Chiari on the central nervous system, all of which require careful monitoring.  First, Christopher needed to do a full spine MRI to see if CSF was collecting in pockets called syrinx.  Syringomyelia, once it develops, can cause permanent nerve damage, so they operate as soon as it’s diagnosed.  Full spine MRIs are done approximately every 12 months.  Second, Christopher needed to see a pediatric ophthalmologist to have his optic nerves checked.  The occipital lobes are at the back of the brain, so compression at the skull base can also damage the optic nerves, which can cause color blindness and strabismus (lazy eye).  Optic nerve damage is, therefore, also considered an emergent reason to operate.  Optic nerve checks are done every 3-6 months.

Figure 1--Basic diagram of the spinal cord and its relationship to the brain.  The Brain Book, p. 53.

Figure 2-Even though cerebrospinal fluid doesn't flow freely around the back of the brain in Chiari patients, it can get stuck inside the spinal cord.  When this happens, it is called a syrinx.  Image courtesy the mayo Foundation.

At that time, Christopher’s headaches were all over his head and weren’t localized to the base of his skull, so while some of his headaches—like the exertional ones he experienced during swim team—were Chiari in nature, the Johns Hopkins crew were concerned about the others.  Were they migraine?  Were they allergy/sinus?  Were they tension/stress?  Christopher had developed some pretty scary visual disturbances, sometimes seeing everything through an orange or yellow haze, sometimes seeing “steam” in his right peripheral vision.  Were these disturbances related to optic nerve damage?  Or were they migraine auras?  To help elucidate these questions, we were asked to also find a pediatric headache specialist and a cognitive behavioral therapist.

Image 1--This is technically from Christopher's spinal MRI, not his brain MRI, but this  shows the different structures quite nicely and shows that there is clear herniation of the cerebellar tonsils below the foramen magnum.

Leaving that appointment was hard.  Christopher was not thrilled to hear he had more tests and specialists to see, with no one offering anything concrete to make the headaches go away.  I’ve spent enough years performing experiments in a lab that I could appreciate the Hopkins perspective.  They wanted to eliminate distractors and isolate variables.  But how do you explain that to a child who’s been in pain for weeks?  Somehow, the fact that, living near DC, we’re in a specialist hotspot and rarely have to wait more than a few weeks for an appointment was cold comfort.