A's Update 3 (1/29/14): Surgery Details
So yesterday we got more of the "nitty gritty" from the plastic surgeon on how this surgery will be going down (for those recently having our story shared with you you can do some background reading by starting at the beginning of our story CLICKING HERE).
So to answer that I offer a quick refresher on the issue. An "Encephalocele" is a neural tube defect in which as the skull was forming in utero pieces of the skull failed to fuse together, in A's case, right above her nose bone. This is one of a couple major "fuse points". Therefore her skull was left with a hole and as the rest of her brain started forming it started pushing through the hole. Your brain also has pockets of CSF (Cerebralspinal Fluid) to help "pad" your brain. This hole also allowed CSF fluid to leak into the space and the combination of pushed brain matter (which becomes dysplastic, non-functioning, upon being squeezed through the opening or just because it was formed too chaotically to be functional) and CSF is what makes up the "Encephalocele" that protrudes on her face.
Ok, so now that that's understood there are 2 major components that need to be taken care of: 1) The hole in the skull needs to be closed and 2) The dysplastic brain matter needs to be "trimmed" off. The primary reason for #1 is obvious, and the primary reason for #2 is that not only does the non-functioning brain matter not contribute anything to her but leaving it there poses risk of seizure activity at unpredictable times. Your brain is constantly sending electrical pulses all over the places to communicate with all the necessary parts of the brain for whatever that signal is trying to accomplish and if one of those signals passes across the non-functioning brain matter the signal meets an unexpected end, like a short circuit, and this could trigger seizure activity because the brain was not expecting the signal to be interrupted.
She will have two major incision sites. For the bone repair they will zig-zag cut her from ear to ear across the top of her head. They plan to go across the top of her head because her hair will cover the scar eventually, they plan to zig-zag the incision so it does not affect her hairline too much (if it was a straight cut then potentially she would have a weird, forever part, in her hairline. This large incision will allow them to have access to her skull bone and they will remove the face plate of her skull bone (forehead). With the face plate removed the plastic surgeon will take small pieces of her skull from elsewhere on her skull to fill the void of the hole. He will also sprinkle bone dust (collected during the removal process, as our plastic surgeon put it "We plastic surgeons do not like to throw ANYTHING away") onto the new pieces of bone he's put in place. The purpose of the dust sprinkling is to hopefully provide a "scaffolding" structure to encourage more bone to naturally grow and continue repair in the area naturally.
In addition to the hole repair work the plastic surgeon will also need to repair her nose bone structure, this will be the site of her second major incision. This incision will basically take place right where her encephalocele is and gives both the neurosurgeon and plastic surgeon clear access to the area. The goal is create the incision where it will be a midline scar right in vertical alignment to her nose. Our plastic surgeon has had excellent results with a scar placed in this area in it's long term aesthetic appearance. However, if during surgery he discovers a better place to put the scar, say along a natural "wrinkle" in the face or whatever, he will do it. To best explain what needs repaired here I'd ask you to go ahead and grab your nose bridge between your eyes, go ahead I won't tell anyone and we'll wait for you to do it. Did you do it? A doesn't have that. That structure is incredibly small in babies to begin with and with the hole in her skull bone that bone structure has developed bent outwardly (picture like when a tire explodes and there are shards of tire mesh bent/blown outward). So she needs to have her nose structure rebuilt (again using bone pieces from other parts of her skull. BTW, all these bone pieces they're "stealing" from other parts of her skull will be taken from areas that should be able to handle the temporary defect and heal themselves since her head is still growing rapidly.
So those are the two major components of the plastic surgeons job, as well as any smaller jobs associated with those and adapting on the fly to any changes that happen during surgery.
While the plastic surgeon is doing the aforementioned tasks the neurosurgeon will be trimming off the non-functioning brain matter from the front of her brain. As you might imagine this is a little trickier than busting out the hot pink handled Fiskars and trimming back a little off the top. Our brains have a covering over them called the "dura mater" it is a membrane that keeps our brains contained in a sterile environment as well as helps hold in that CSF I mentioned earlier. So the neurosurgeon will need to carefully excise the non-functioning brain matter and repair the dura to ensure there is still a good seal around the brain. He also obviously won't want to trim back too far into functioning brain matter so keeping a close eye on what's good and what's bad brain matter is key. As an added complication the neurosurgeon will be needing to work around a major artery that carries some 25% of your cardiac output.
Once everyone's done their part they'll put her back together using as much dissoluble parts as possible (screws and plates that will be absorbed into her bone over time as opposed to metal). The plastic surgeon has told us that he almost guarantees she will need future adjustment operations. For example, remember when you pinched your nose bridge earlier? Did you notice how your orbital structure is set deep back into your head, far enough so that there's probably one knuckle length of your finger before your get to the bridge of your nose? This is achieved by some ligaments that help hold that structure back there. She still won't have these ligaments, or at least what she does have will be very very small and weak. So most likely her orbital structure will be relatively flat with the bridge of her nose, or at least not as deep set as is normal. This COULD be addressed right now, but it's been our plastic surgeons experience that trying to "hook it all up" at this age is more of an exercise in frustration because that ligament is so weak in her and at this age that it almost never holds, and he prefers to go back in a year or so and mess with it then when everything's become stronger.
I am pretty sure you'll understand my motivations for brevity on this topic. All risks of surgery and anesthesia apply. Depending on the length of surgery there's a good chance she'll need at least one blood transfusion during surgery as this will certainly be a major draw on her body. Post-surgery the biggest risk will be a CSF leak, remember that dura I mentioned, if it's not healed up properly it will allow CSF to leak out and the bone can't heal in the presence of CSF. Luckily A already has a shunt in her head for her hydrocephalus and the purpose of the shunt is to help drain CSF from her head. This is good because her shunt is "programmable" which means they can alter how much, or how little it drains. So post surgery they can crank that puppy up and have it drain a lot and give the CSF somewhere to go that's NOT the front of her head.
Your prayers, positive thoughts, vibes, tribal dances, are all welcome! Thank you all very much for the support you've shown and we've been receiving on this journey, it truly, truly, makes a world of difference in our state of mind.
"How do you fix this?"
So to answer that I offer a quick refresher on the issue. An "Encephalocele" is a neural tube defect in which as the skull was forming in utero pieces of the skull failed to fuse together, in A's case, right above her nose bone. This is one of a couple major "fuse points". Therefore her skull was left with a hole and as the rest of her brain started forming it started pushing through the hole. Your brain also has pockets of CSF (Cerebralspinal Fluid) to help "pad" your brain. This hole also allowed CSF fluid to leak into the space and the combination of pushed brain matter (which becomes dysplastic, non-functioning, upon being squeezed through the opening or just because it was formed too chaotically to be functional) and CSF is what makes up the "Encephalocele" that protrudes on her face.
Ok, so now that that's understood there are 2 major components that need to be taken care of: 1) The hole in the skull needs to be closed and 2) The dysplastic brain matter needs to be "trimmed" off. The primary reason for #1 is obvious, and the primary reason for #2 is that not only does the non-functioning brain matter not contribute anything to her but leaving it there poses risk of seizure activity at unpredictable times. Your brain is constantly sending electrical pulses all over the places to communicate with all the necessary parts of the brain for whatever that signal is trying to accomplish and if one of those signals passes across the non-functioning brain matter the signal meets an unexpected end, like a short circuit, and this could trigger seizure activity because the brain was not expecting the signal to be interrupted.
How they will fix the hole in her skull bone:
She will have two major incision sites. For the bone repair they will zig-zag cut her from ear to ear across the top of her head. They plan to go across the top of her head because her hair will cover the scar eventually, they plan to zig-zag the incision so it does not affect her hairline too much (if it was a straight cut then potentially she would have a weird, forever part, in her hairline. This large incision will allow them to have access to her skull bone and they will remove the face plate of her skull bone (forehead). With the face plate removed the plastic surgeon will take small pieces of her skull from elsewhere on her skull to fill the void of the hole. He will also sprinkle bone dust (collected during the removal process, as our plastic surgeon put it "We plastic surgeons do not like to throw ANYTHING away") onto the new pieces of bone he's put in place. The purpose of the dust sprinkling is to hopefully provide a "scaffolding" structure to encourage more bone to naturally grow and continue repair in the area naturally.
In addition to the hole repair work the plastic surgeon will also need to repair her nose bone structure, this will be the site of her second major incision. This incision will basically take place right where her encephalocele is and gives both the neurosurgeon and plastic surgeon clear access to the area. The goal is create the incision where it will be a midline scar right in vertical alignment to her nose. Our plastic surgeon has had excellent results with a scar placed in this area in it's long term aesthetic appearance. However, if during surgery he discovers a better place to put the scar, say along a natural "wrinkle" in the face or whatever, he will do it. To best explain what needs repaired here I'd ask you to go ahead and grab your nose bridge between your eyes, go ahead I won't tell anyone and we'll wait for you to do it. Did you do it? A doesn't have that. That structure is incredibly small in babies to begin with and with the hole in her skull bone that bone structure has developed bent outwardly (picture like when a tire explodes and there are shards of tire mesh bent/blown outward). So she needs to have her nose structure rebuilt (again using bone pieces from other parts of her skull. BTW, all these bone pieces they're "stealing" from other parts of her skull will be taken from areas that should be able to handle the temporary defect and heal themselves since her head is still growing rapidly.
So those are the two major components of the plastic surgeons job, as well as any smaller jobs associated with those and adapting on the fly to any changes that happen during surgery.
How will they trim her brain:
While the plastic surgeon is doing the aforementioned tasks the neurosurgeon will be trimming off the non-functioning brain matter from the front of her brain. As you might imagine this is a little trickier than busting out the hot pink handled Fiskars and trimming back a little off the top. Our brains have a covering over them called the "dura mater" it is a membrane that keeps our brains contained in a sterile environment as well as helps hold in that CSF I mentioned earlier. So the neurosurgeon will need to carefully excise the non-functioning brain matter and repair the dura to ensure there is still a good seal around the brain. He also obviously won't want to trim back too far into functioning brain matter so keeping a close eye on what's good and what's bad brain matter is key. As an added complication the neurosurgeon will be needing to work around a major artery that carries some 25% of your cardiac output.
Once everyone's done their part they'll put her back together using as much dissoluble parts as possible (screws and plates that will be absorbed into her bone over time as opposed to metal). The plastic surgeon has told us that he almost guarantees she will need future adjustment operations. For example, remember when you pinched your nose bridge earlier? Did you notice how your orbital structure is set deep back into your head, far enough so that there's probably one knuckle length of your finger before your get to the bridge of your nose? This is achieved by some ligaments that help hold that structure back there. She still won't have these ligaments, or at least what she does have will be very very small and weak. So most likely her orbital structure will be relatively flat with the bridge of her nose, or at least not as deep set as is normal. This COULD be addressed right now, but it's been our plastic surgeons experience that trying to "hook it all up" at this age is more of an exercise in frustration because that ligament is so weak in her and at this age that it almost never holds, and he prefers to go back in a year or so and mess with it then when everything's become stronger.
What kind of risk is A facing:
I am pretty sure you'll understand my motivations for brevity on this topic. All risks of surgery and anesthesia apply. Depending on the length of surgery there's a good chance she'll need at least one blood transfusion during surgery as this will certainly be a major draw on her body. Post-surgery the biggest risk will be a CSF leak, remember that dura I mentioned, if it's not healed up properly it will allow CSF to leak out and the bone can't heal in the presence of CSF. Luckily A already has a shunt in her head for her hydrocephalus and the purpose of the shunt is to help drain CSF from her head. This is good because her shunt is "programmable" which means they can alter how much, or how little it drains. So post surgery they can crank that puppy up and have it drain a lot and give the CSF somewhere to go that's NOT the front of her head.
As for mom and dad, we're doing, OK. Extremely anxious, nervous, pretty much any emotion you can imagine is in the bag. We've been trying to prepare ourselves for this day for almost 8 months now since we first found out about her condition and we're quickly finding that no amount of time truly prepares you. For more on the emotional tolls my wife has been capturing those narratives excellently on her blog so I won't try to restate them, you can read her posts here: http://www.ballerinablock.blogspot.com/
Your prayers, positive thoughts, vibes, tribal dances, are all welcome! Thank you all very much for the support you've shown and we've been receiving on this journey, it truly, truly, makes a world of difference in our state of mind.
Thank you for the wonderful post, I didn't realize surgery is so close. We are praying for all of you in a special way, it makes me tear up to think of it all... We will be checking up on the updates as they come along.
ReplyDeleteThanks so much Mary!
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