"He
plays, he's happy, he's ornery like any
2-year-old," says Tammie Jackson of her son's
recovery from his operation, "Dr. Tufaro was a
godsend."
The tumor on
18-month old Maison Jackson's face had ballooned
to the size of a softball. Appearing around the
little boy's first birthday, the mass had been
steadily enlarging week by week, distorting his
mouth, inflating his left cheek and closing his
left nostril. By the time his parents were
referred to Anthony Tufaro, a plastic surgeon
specializing in head and neck surgery, the tumor
had inched dangerously close to Maison's left
eye.
At
first, physicians had told the Jacksons that the
growth was simply part of the port wine stain
Maison had been born with. The reddish facial
birthmark, characterized by an overabundance of
capillaries just beneath the skin's surface,
typically signals the presence of Sturge- Weber
syndrome, a congenital neurological disorder
that can include such symptoms as seizures,
glaucoma and excessive blood vessel growth on
the surface of the brain. When he was 8 months
old, Maison did have two seizures but ever since
then has responded well to medication. And by
the time his tumor became visible, he was
already being treated for glaucoma. Yet, despite
the progressively worsening facial disfigurement
that was also afflicting their son, the,
Jacksons heard only that there was nothing to be
done for it.
"The moment I saw
Maison,” says Tufaro, "I knew he needed an
operation. He had what's called an ossifying
fibroma - a rare tumor that's usually benign but
very aggressive. It was so large that it had
displaced his upper teeth and jaw. He couldn't
eat, he couldn't breathe, he was snoring. He was
so dysfunctional from the tumor, we had to do
something.”
Tufaro knew
immediately that the sheer size of Maison's
fibroma was worrisome-"We weren’t sure it wasn’t
cancer," he says, "and we didn't know if we'd
have to remove his eye and socket!' But what
presented one of the toughest technical
challenges was the fact that the tumor was
sitting amid a bed of dilated blood vessels. Not
only would Tufaro have to completely remove the
boy's upper jaw in order to excise the massive
growth, he would have to do so with the risk
that the child could bleed to death during the
operation.
To solve that
problem, Tufaro turned to interventional
radiologist Sally Mitchell. Mitchell first used
magnetic resonance imaging to make the tangle of
blood vessels visible, then assisted Tufaro
during the four-hour procedure by floating coils
in the vessels to limit blood flow. Even so,
says Tufaro, “I had to go slow. One blood vessel
feeding that tumor was the size of my finger.”
Tufaro was able to remove virtually all of the
5-centimeter mass, which to everyone's relief
was non-cancerous. "Maison will probably need
further reconstructive surgery down the line;'
he says, "but he bounced back one two three.
He’s growing. He’s thriving.”
