>> 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.