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.