Moyamoya Disease Surgery for Children: Pial Synangiosis

Moyamoya Disease Surgery for Children: Pial Synangiosis

>>With Moyamoya syndrome there’s some kind
of progressive narrowing of the two major arteries in the front of your neck, the carotid
arteries. That narrowing gradually constricts the blood flow to the brain.>>The goal of surgery is to provide a separate
avenue to get blood to the brain.>>The child and their family who come to CHOP for surgery because of their blood vessel
disease, their Moyamoya, or any other vascular disease, is going to experience what I feel
is an incomparable team approach. And care that starts from the moment they walk in the
door to not only after they leave the hospital but for years to come, for as long as they
need it.>>Moyamoya is the name that it was given
because of the way it looks on the radiograph, like a puff of smoke. It’s a Japanese term.
Moyamoya is a disease of the blood vessels. Usually you’ll see a narrowing of the carotid
arteries, and then they often end up developing decreased blood flow to the brain, which leads
to less oxygen to the brain, which then can lead to a stroke.>>A stroke is a clinical condition that is caused by a loss of blood flow or bleeding
in a part of the brain.>>It’s certainly something that we see plenty
of. They can be having headaches. They may be having transient weakness. People may not
be thinking is a result from stroke because they don’t think of children as having strokes.>>I thought it was a sinus issues and thought, oh I’m not going to leave the ER until they
do a CAT scan to see what’s going on with her sinuses. It being a stroke, it just took
me totally off guard. What 5-year-old has a stroke? I mean we all know how to handle
an elderly person that has a stroke, but how do you handle a 5-year-old that’s had a stroke.
Within a few hours we were at CHOP, and Dr. Ichord came in. She first gave us the diagnosis,
and was like, “Oh this is Moyamoya disease. Yes it’s very rare, but let me tell you about
it.” And she knew everything about it.>>I think that that level of sort of calmness
that the whole hospital has with new or novel or unusual disease is helpful for the family
because they can just see it as a health problem that we know how to treat.>>We talked more about the Moyamoya disease itself and what it does and what is causes
and that there was surgery available. They talked about the surgery and that they had
done it before. “It’s gonna be this way. And we’re both gonna be working on each side,”
Dr. Storm, Dr. Heurer; it put my mind absolutely at ease.>>The point of the pial synangiosis is to provide another access, another delivery system
of blood to the brain with the goal being to prevent further strokes.>>You can sometimes talk about it in terms of say a traffic jam. And if one road is blocked,
then you have to reroute the traffic through another road. So we have a set of blood vessels
that go to our skull and our scalp and skin, and the surgeon identifies a viable branch
from that external circulation.>>We favor the indirect bypass at CHOP where
you take a superficial temporal artery, which is in the scalp, and then you make an opening
in the skull and literally just lay that artery over the brain. It’s quite loose, so literally
just remove a piece of bone, and then lay that artery that’s quite free, on top of the
brain. And then we put the skull back over top, notching out holes on either side, allowing
it to come in, go down in the brain, and come back out into the scalp. Because the
brain is trying to get arteries growing into it that little branches will start growing
from that artery. And then you can see huge blood flow through the brain from that.
If you put a seed in the ground, and it’s going to start growing roots into it, then
you lay this artery on top because it needs to … that brain wants to be fed oxygen,
it will start growing into it to deliver blood and ultimately oxygen.>>We tell a family it doesn’t usually cause much pain. Hospitalization is about maximum
two to three days, and then they can go home.>>You don’t get a benefit right away. It
often takes three to six months, and then we do a repeat angiogram in a year just to
see how the blood flow is developing. I think one of the big benefits of having
your neurosurgical care at CHOP is that all the other related fields are so strong. Neurosurgery
is rarely a condition where you have one surgery and you’re done. I mean there are those, we
have plenty of those, but in general these are complicated patients who need a lot of
care, either neuro-oncology, neurology, endocrinology, physical therapy, rehabilitation, all that
happens under one umbrella.>>The Pediatric Stroke Program at CHOP has
expertise in caring for children with Moyamoya, including their medical care, surgery, but
also sort of the long-term needs of patients, whether that is physical, psychological, school
related.>>The Pediatric Stroke Program at CHOP was
one of the first established in the nation and is viewed widely as one of the best.>>We see children with all different types of strokes and also other kinds of problems
with the blood vessels in the brain.>>Stroke is just one part of neurovascular
disorders. Many other kinds of abnormalities may affect the blood vessels in the brain.
There are malformations called arteriovascular malformations. Another type is called a cavernous
malformation. These and other kinds of blood vessel diseases can cause injury to the brain
through bleeding, or through a risk of bleeding. And so the Stroke Program in partnership with
many other departments and with the surgery department take care of those kinds of problems
together, as well as we take care of Moyamoya and the more traditional stroke condition.>>We do over a thousand cases a year. One of the busiest if not the busiest pediatric
neurosurgery departments in the country. We also are diverse. We do a lot of different
kinds of operations. But we do enough of them to be familiar, in the hospital, to be familiar
with what we’re doing. So it doesn’t feel like you’re getting sent from one doctor to
another. It feels like it’s a seamless set of doctors who are all talking to each other.>>Every time we came there was either a social worker or the neurologist or someone was always
there.>>Awesome, respectful, go out of their way
if you need anything.>>The most rewarding part is having the kids
succeed, and seeing them come so far and work so hard and surpass, you know, the expectations
that their parents have.>>I can be in the kitchen making cookies
and she can play. She’s OK. I try to live every day and let her be a kid.>>The great thrill to me is just to witness, to bear witness, to how well children recover.
How determined they are. How they find a way to be happy and whole, and that is humbling;
it’s endlessly fascinating; and it’s very rewarding.

2 thoughts on “Moyamoya Disease Surgery for Children: Pial Synangiosis

  1. My son was diagnosed with Moya Moya when he was 8 yrs old back in 1984 and is still going strong. even though he still experiences episodes of mini TEA's his name is Chris Sitler. and he is a 41-year-old man he lives in Bloomsburg,Pa.

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