A revolutionary new surgery changes the picture for children with Apert syndrome.
A distractor, also called a “halo,” moves the middle of James Weatherwax’s face forward slowly over several weeks. James was born with Apert syndrome,
a genetic disorder that prevents the bones in a child’s skull and face from growing normally, and can make it difficult for kids like him to do everyday
things like breathe, eat, talk or sleep.
If we’re born with the expected
configuration of features, we can
afford to take our faces for granted.
Although we might wish for a smaller
nose or movie-star cheekbones, our
faces will do.
But children born with Apert syndrome don’t have that luxury.
Their faces get in the way of simply
breathing, eating and sleeping.
Apert syndrome is a rare and
extreme form of craniosynostosis,
where one or more of the special joints
between the bones of the skull, called
sutures, fuse before birth – years too
early – deforming the head and
putting pressure on the brain.
Because the bones in the center
of their faces are jammed together like
a compressed jigsaw puzzle, children
with Apert syndrome have concave
faces; bulging eyes; a small, beaked
nose; and an undersized upper jaw.
Their fingers and toes are often fused
together. They likely have severe sleep
apnea, and can suffer hearing loss,
dental problems and developmental
Face is portal to the world
The difficulties of Apert syndrome
go beyond the physical challenges,
says Dr. Richard Hopper, a craniofacial
surgeon who is revolutionizing the
surgical options for kids with the
“We love James
looks like, but I
had to do this
to improve his
quality of life.”
“We interact through our faces; it’s
our portal to the world. Imagine a child
whose face constantly lets them down.
They can’t breathe. Their eyes are
watery. They’re drooling. Their teeth
don’t fit together. They talk funny. Their
face is always in the way of what they
want to do.
“And as humans we instinctively
scan a person’s face when we meet
them. If the proportions and balanceof the face look right, we’re more
likely to interact,” says Hopper. “When
someone meets a child with Apert
syndrome for the first time, it can be
hard for the person to get past the fact
that the child’s face just looks wrong.”
James has been treated at Children’s since he was a baby
and has had more than 15 surgeries. Ongoing relationships like these are a big part of what motivates
Dr. Richard Hopper to continue improving care.
James Weatherwax knows these
challenges all too well. He was born
with Apert syndrome and cleft lipand palate. He and his mom, Kecia
Weatherwax, have been making
the long journey from their island
community in Klawock, Alaska, to
Seattle Children’s since he was a baby.
Now 10, James has already had more
than 15 surgeries on his skull, face,
hands and feet.
Weatherwax says that over the years
she’s worried about how James would
be accepted by others. People in theirhome community know and accept her
son, but this hasn’t always been the case.
“There were times where kids have
been cruel. People can just be really
mean because they don’t understand
him,” she says.
Reassembling the puzzle
Drs. Richard Hopper and Hitesh Kapadia collaborate with each
other and other members of the 18 specialties that make up the Craniofacial Center. The kinds of dramatic improvements Hopper seeks for his patients are only possible in a collaborative
environment like Children’s.
Traditional surgeries for Apert and other
syndromic forms of craniosynostosis
move the facial bones together as one
block, the same distance and in one
direction. While it’s effective in helping
kids breathe, that approach doesn’t
normalize facial proportions. Hopper
felt his patients deserved more.
So, over the past five years, he and
his colleagues in the Craniofacial Center
developed a new approach called
segmental subcranial distraction. It
combines existing techniques in a way
that hasn’t been done before, and
gives patients the benefit of multiple
procedures in one surgery. It also does
more than alleviate breathing and
chewing problems: by normalizing
the features, it gives a child with
Apert syndrome the chance to interact
socially without his face getting in
“You need to move different parts
of the face in different directions, and
different distances,” explains Hopper.
“Essentially, you have to unlock the
compressed jigsaw puzzle and then
During the delicate, daylong
surgery, Hopper and his team separate
the bones of the face from the skull,
releasing the cheekbones and eye
sockets from the middle of the face
and repositioning them with small plates
and screws. Using an implant that he
and his colleagues design specifically
for the child, he brings the forehead
forward and attaches a device known
as a distractor to the central part of
Like James, Dino Velagic, 17, has
Apert syndrome, and struggled to breathe until Hopper and his team performed a
revolutionary new procedure called segmental
subcranial distraction. This
procedure, developed by Hopper, has only been
done seven times – all at Seattle Children’s.
While the child is recovering at
home, a parent turns the distractor a
millimeter a day for about two weeks
to continually move the bones. New
bone grows and fills in the gaps. Once
everything is in the right place, the
distractor stays on for about six more
weeks while the bone solidifies.
Transformative innovations like
segmental subcranial distraction are
only possible, Hopper says, because
of the depth and range of skills in
Children’s Craniofacial Center, the
largest in the United States.
Dr. Hitesh Kapadia, one of just a
few craniofacial surgical orthodontists
in the country, is a key collaborator.
Weeks and even months before the
surgery, he uses computer imaging
and standardized growth predictions
to precisely plan the direction and
magnitude of the movement of the
facial bones. Kapadia must take intoaccount the age of the child at the
time of surgery and accurately predict
where the teeth need to meet, and
how that affects the cheekbones,
nose and the soft tissues of the face.
“You need to
parts of the
face in different
Quality of life
Social worker Cassandra Aspinall collaborates with child life specialists to
help children work through their anxiety. Depending on the child’s age and developmental needs, they may use tools like this doll fitted with
its own custom-made halo.
Weatherwax initially hesitated when
she heard about the new treatment.
“We love James whatever he looks
like, but I had to do this to improve
his quality of life,” she says. Early
in 2013, they made another trip to
“He is doing really well now and is
just being a happy boy who is always
on the go,” says Weatherwax. “He
goes out to play with his friends and
he loves playing basketball and
swimming. He is much more active
and a lot more independent.”
For Hopper, that’s the measure of
success. “For me, it’s never about ‘We
moved that bone into a better position,
wasn’t that a great surgery?’ It’s got to
be, ‘Was that the best way to move that
bone? How does that affect the child at
2 o’clock in the morning? How does it
affect him when he meets a friend for
the first time on the playground?’”
Giving Families Options
After endoscopic surgery, Wesley Matthes
wore a custom helmet for eight months
to reshape his head. “He slept fine with it
from day one,” recalls his mother, Angela
Neeleman. “Helmet therapy was worlds
easier than I expected it to be.”
As Angela Neeleman and Steve
Matthes discovered when their son
Wesley was born, single-suture
craniosynostosis (where just one of
the skull joints fuses prematurely)
can happen in any family. In fact,
one in every 2,000 babies born
each year has the condition.
The standard treatment involves
a surgery with a large incision to
remove the fused suture and reshape
the skull bones; absorbable plates and
screws hold the pieces in place while
Recently, Seattle Children’s has
given families another option: a
camera-assisted operation called
endoscopic strip craniectomy. It uses
two small incisions, takes half as long
as the open surgery, and rarely requires
a blood transfusion (which is almost
guaranteed with the open method).
After the endoscopic surgery the
baby must wear a helmet 23 hours a
day for up to a year to mold the skull,
and the family has to be able to come
back frequently to get the helmet
adjusted. (The helmet is similar to
one originally developed at Children’s
for babies who develop “flat head
syndrome” from lying on their backs.)
“We’re confident both surgeries
work equally well so families can
choose the option that makes the
most sense for them,” says Dr. Amy Lee, the neurosurgeon who brought
the procedure to Children’s.
Neeleman was uneasy with the
open surgery and appreciated having
a choice about Wesley's treatment.
“I’m his mom; my job is to protect him
as best as I can from as many things
as possible. The endoscopic option
allowed me to do that.”
Published in Connection magazine, November 2013