Novosibirsk; Texas Road Trip 2016: We Escape His Diagnosis

By this point, Christopher needed a break from being poked and prodded.  He’d made good progress on the amitriptyline, but we had always intended to visit my parents’ Texas ranch in May.  He was still hopeful that the medicine, which takes 3-4 weeks to titrate into your system, would eliminate his headache.  He was also really scared of doing the cerebral angiogram if Dr. Ahn ordered it, because it would require an IV and going to sleep (I’m still amazed by the things that scare him, they’re never the things I’d guess).  So we rescheduled his appointment with Dr. Ahn for June 14 so that we could spend some time off the train.

You can’t really completely ignore these medical diagnoses, especially since we were still trying to process whether or not to go ahead with the Chiari surgery.  Once again, I was worried CYA medicine was at play with the AVM diagnosis, and I was seriously contemplating whether it was really capable of harming him or not.  Part of that is that I had pretty well followed Dr. Groves’ recommendation not to Google the condition, so I still knew very little of what it was, or what it could do.  But Dr. Bernier had asked us not to “check in” as part of his headache plan, so I tried to reign in the desire, as a scientist, to document and detail every symptom.  Christopher, for his part, spent as much time at the ranch as he could.  Getting there had been pretty rough on him, and the final day of our road trip had spawned a nasty migraine.  He tired more easily than usual, several very active days would follow several days on the couch, and he still had a very short fuse for frustration, but the scary, is-my-kid-having-a-stroke-or-seizure? headaches were all gone.  In general, though, he spent a lot of time having fun and being a kid, whether he was fishing, petting horses, playing pool, or driving the EZ-Go.  And we did manage to squeeze some touristy stuff into both road trips, visiting both Biltmore Estate and the Kentucky Horse Park on our 5600 mile journey.

Omsk; How and When Should You Fix a Chiari?

By the time we had our follow-up appointment with Dr. Moores, our first neurosurgeon, Christopher was doing remarkably better.  The obvious migraines were gone, and the headache had gone from a daily 8 to a baseline of 3/4, even though it never went away.  This was enough improvement for him to begin doing math and grammar studies again.  We told Dr. Moores what all we’d done at the behest of Johns Hopkins and the information we’d learned since we’d last seen him.  

We also told Dr. Moores we'd sought out a second opinion because we'd been advised by a dear friend who's a pediatric ER doc that Chiari I Malformations are over-diagnosed.  He said he agrees.  In his opinion, there are a whole lot of people in the world with headaches, represented by the red circle.  There are slightly less than 1 in 1000 people with a Chiari I Malformation.  But most of those people are asymptomatic, and only discover they have a Chiari I because a scan of the brain/neck is done for a completely unrelated reason.  So the percentage of people whose Chiari defects actually cause headaches is represented by the overlap of those two circles.

Figure 1--My interpretation of Dr. Moores' Venn Diagram describing the relationship between Chiari and Headache.

The obvious question, which of course we asked, was what category does Christopher fall into?  He feels that the back of Christopher's skull cuts in to the base of his brain so as to inhibit his posterior cerebrospinal fluid flow such that he has zero functional flow as soon as he stands up.  But wait.  Christopher's a kid.  And kids grow.  Can't this all just be a problem where his current brain size is out of sync with his skull size and, if we're patient, he'll grow until they're back in phase again?  After all, when I look at my 12 year old's photos, it was at about age 9 that his face really started changing shape.  Unfortunately, all that skull change is confined to the front of the head.  The back of the head, and particularly the posterior fossa, don't change much after age 4.  The brain itself also reaches 99% of its final size by age 4, even though the connections it forms continue developing in some parts well into the 20s.  Dr. Groves had answered this question in exactly the same way.

Image 1--In this study, it's fairly obvious that there's a nice bright white line of CSF flowing around the top of Christopher's brain, and along the front of his brainstem.  Down the back of his brainstem?  Not so much.  Behind his cerebellum?  Eek.


So.  In Dr. Moores' estimation, Christopher will never be headache free without surgery to decompress the base of his brain.  He wasn't as worried as Dr. Groves about operating without eliminating the other causes of headache.  But he also feels it’s non-emergent right now because there's no syrinx or obvious optic nerve compression.  Scott, who hadn’t yet met Dr. Moores, agreed that his bedside manner is impeccable and his explanations, rooted in physics, just.make.sense.

Both neurosurgeons were in complete agreement as to what they would do to fix it:  A Chiari decompression via craniectomy of the foramen magnum, laminectomy of C1, and a duraplasty.

Figure 2--The opening of the foramen magnum is enlarged by removing small sections of bone, as well as widening the interior diameter of C1 if necessary.  They can't make the posterior fossa larger, but they can remove the pressure it exerts on the back of the brain.  Image courtesy of the Mayfield Clinic.

Figure 3--Posterior diagram showing the new skull configuration after surgery.  Duraplasty is done to enlarge the dural space and reintroduce proper CSF flow.  Image courtesy of the Mayfield Clinic.

Tyumen; We Meet the Pediatric Headache Specialist

Dr. Raquelle Bernier of the Children’s National Headache Clinic is one of my favorite physicians ever.  She has the presence and demeanor of a kindergarten teacher (i.e., everyone wants to be friends with her), but is also one of the most technically competent physicians I’ve ever met.  She made a point of double-checking Christopher’s MRI after we told her about the newly diagnosed arteriovenous malformation (AVM) and agreed that it was there and needed to be checked out.  She also agreed with the ophthalmologist that Christopher was having migraines with auras.  After taking a very thorough history, she told us that headache specialists are starting to see a pattern wherein kids with migraine diagnoses have a history of colic as infants, digestive upset (abdominal migraine), and extreme, early onset motion sickness.  They believe these aren’t separate issues, but rather, an indication that the pediatric brain is very different than the adult brain, and experiences pain much differently.  So it wasn’t that our perfectly normal child suddenly started experiencing blistering headaches at 9 years of age.  It was that he’d been symptomatic from the Chiari (and possibly the AVM) from birth, it was just at age 9 that he started experiencing those symptoms in a way that led to a brain MRI and proper diagnosis.  That, she said, was why we'd noticed the abdominal migraines (vomiting, nausea, diarrhea, stomach cramping) fading away as the more classical migraines with auras kicked in.

More importantly for Christopher, though, she put him on 10mg amitriptyline as a daily headache preventative and wrote out an extensive treatment plan that included dietary, water, exercise, and sleep components to keep the headaches at bay.  Because the AVM presents a stroke risk, NSAIDS and triptans are verboten, but occasional Tylenol is allowed.  She also asked that he stay away from rollercoasters and contact sports, I presume because he has enough issues without adding a concussion into the mix.

For more information on what the medical community now knows about Pediatric Headaches, please follow the link below.  If this had been known a decade ago, Christopher would have had a brain MRI by his first birthday.  Not only was he the colicky baby to end all colicky babies, the colic extended well beyond the normal first three months of his life.  By five months, he was diagnosed as failure to thrive because I couldn't keep milk in him.  No matter how gingerly I raised him to a vertical position after feeding, as soon as he was fully upright, he'd projectile vomit everything in his belly, and continue for hours.  As for motion sickness....I could have set a timer for him puking 20 minutes after getting in a car.  By the time he was 8 months old, the pediatrician recommended I put Baby Einsteins on loop in front of his rear-facing carseat on even a short trip to the grocery store so that I wasn't always driving distracted from the crying.  He had his own Zofran prescription for car, plane, and train rides at his second birthday because Dramamine wasn't effective.  This was the pattern that emerged from Dr. Bernier's questioning, which is corroborated by this summary from the Michigan Headache & Neurological Institute.
  
http://www.mhni.com/headache-pain-faq/pediatric-headaches