Tuesday, January 16, 2007

Peds Neurosurgery

Roughly a month ago, a boy named Jay was born with a prosencephalic defect. (I couldn't get a straight answer from the neurosurgeons, one telling me it was something between prosencephaly and holoprosencephaly, and another that it was sort of like holoprosecephaly. I could try and figure it out, but I'm lazy, and I have to study more neurology. Anyway, to return.) After an insult to his development very early in gestation, his brain stopped developing in the way it is expected to, and began to force his brain outside the confines of his skull, so that, at term, his CT scan looked something like the picture to the right.

Enter Dr. Lest. Aside from being one of the nicest people I've ever met, and remarkably personable and witty, he specializes in pediatric neurosurgery. He combines the personality of a pediatrician with the skills of a surgeon. Enheartening to watch. Exempli gratia, today he was speaking with Jay's family, and one of them expressed surprise that, except for the defect, he looked and acted like a normal newborn. The good doctor's reply: "I know, he's cute as a button, isn't he?"

So today was this child's surgery. If you look at that CT scan from the first paragraph, you can see that the brain is quite thin along the top of the skull. With Jay, the thin part protruded much farther from the skull, and there was actually no bone over the defect. It was essentially a fontanelle about 4cm across. So the plan was to remove some of the skin, drain the excess fluid off the child's brain, and reconstruct the skull to allow more normal development.

The surgery took 5 hours. First the chief resident and Dr. Lest removed a strip of skin over the hole, then they incised the dura over the area, sparking a tiny fountain of CSF for a few seconds. Then they peeled back the dura and looked inside. There, inside a living, breathing body, was visible both right and left thalamus, all the way forward to the optic chiasm. This was only possible because Jay doesn't have a corpus callosum and then, of course, the hole here in the middle of his brain is a bit larger than it should be.

Seeing this ranks as one of the few near-mystical experiences I've had in med school. All of them have involved this closeness to life, this first hand knowledge of these beautiful structures, these elegant machines we struggle to understand, which keep our biological lives in motion. The first was nearly three years ago, in neuroanatomy lab, when I removed and then held in my hand the brain from my cadaver for the first time. Chilling and inspiring and sad and exalting and humiliating all at once. I felt a bit like a stage Hamlet pondering Yorick's skull, only I was wearing scrubs, and I reeked of formaldehyde. I guess satori is independent of the scent of carcinogenic preservatives.

Back in Jay's surgery, several pictures were taken, as this is a "once in a career surgery, for some" in Dr. Lest's words. I certainly won't see it again. Then he and the chief were able to close the hole and reconstruct the bones to partway cover it, and start the kid on the road to recovery.

I was curious, as was the family, what this kid's chances for recovery are. And apparently, no one knows. Only time will tell. But Dr. Lest has "several dozen" former patients who've had this procedure done who are now doing just fine in school, apppropriate to their grade level. And the only way you can tell is, in his words, that they wear glasses. Anything is possible. There's a card taped to Jay's warmer that says "God is with you." Certainly, this is true, and therein lies his greatest hope.

First picture from enotes
Second two from Gray's Anatomy (not the weird medical-ish soap opera, the real one)


Thainamu said...

Your post made me remember this. More than 20 years ago the doctor told us that other parts of the brain would compensate for the part missing, and though she will never be a ballerina, it appears that has indeed happened. We are wonderfully made.

Anonymous said...

porencephalic or pseudoporencephalic? - Nelson chapter 585. Beautifully described intervention, Nathan. Thank you for taking time to write and making your notes available. GTB

Nathan said...

thainamu - the plasticity of the human brain is indeed remarkable.

GTB - you are very welcome, and thank you for the kind words.