Pfeiffer Sydrome: Treatment
Surgery of the Midface:
There are three operations to bring the mid face forward, the LeFort I,
the LeFort III and the monobloc. The LeFort I brings the lower mid face
forward, from the level of the upper teeth, to the nostrils. The LeFort
III brings the entire mid face forward, from the upper teeth to just above
the cheekbones. The monobloc brings both the entire mid face and the forehead
forward together at the same operation. Your surgeon will discuss what
is he or she thinks is best for your child. In general, the LeFort I is
not performed until children are in their teenage years. This leaves the
LeFort III and the monobloc. Some surgeons believe the monobloc is a very
good operation for Pfeiffer syndrome. Among the advantages of this procedure
is that the forehead and mid face are brought forward at the same time;
saving a child one operation. However, this operation does have a high
risk for very serious infections, which is the reason that many surgeons,
including myself, choose to not perform this procedure (see
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The LeFort III is the most commonly performed operation for treating
the mid face in Pfeiffer syndrome. Of all the operations done for Pfeiffer
syndrome, this procedure has the greatest effect on improving a child's
appearance. If this operation is not being done to treat sleep apnea,
it is usually being performed to improve a child's appearance. Many surgeons
will recommend this surgery when your child is between 6 and 8 years old.
Children do not really begin teasing each other about appearance until
age 6. Depending on how a child with Pfeiffer syndrome is developing and
coping with his or her appearance will help determine the timing for this
procedure. This operation is one of the bigger operations a child will
undergo. The procedure is performed through the same incision, on the
top of the child's head, used for the skull operation. The bones of the
mid face are cut across the top of the nose, along the floor of the orbit
(under the eye), and down the sides of the cheekbones. No scars are put
on the child's face. After the bones are cut, the mid face is moved forward
and held in position with bone grafts (taken from the skull) that are
held in place with plates and screws. In young children, the teeth are
frequently wired together for 4-6 weeks. In older children a LeFort I
may be done at the same time as a LeFort III.
Since 1998, I have been using a halo-distraction technique, which I
developed utilizing a device called the RED system, for the LeFort III
operation (see
[ ], [ ]). The device is not actually red, but is purple, and is gets
its name for being a rigid external distraction device. This device is
used primarily in children, because it does not offer much for teenagers
unless their mid face is really far back. With the RED system, the bones
of the mid face are basically cut as described above, with some small
differences. Then, instead of pulling the mid face forward and filling
in the gaps with skull bone, the skin is closed, and a halo is attached
to the outside of the skull with 8-10 screws. A splint (U-shaped piece
of plastic) is attached to the upper teeth and two wires extend forward
from this splint to attach to the halo. The forward pull of the mid face
comes from the dental splint; I do attach any wires to the bones of the
face, which go through the skin (this would leave facial scars that are
unnecessary). The parents, or the child, turn two screws daily, in order
to slowly (and painlessly!) bring the mid face forward. The children are
allowed to eat soft foods, and may go to school. Eight weeks later the
device is removed with a 15 minute anesthetic. One advantage of using
the RED device is that it is a slightly smaller operation, and is better
tolerated by the children (compared to those who undergo the traditional
technique and need to have their jaws wired shut). The greatest advantage
of the RED device is that it enables surgeons to move the mid-face more
than three times further forward than is achieved on average with the
traditional technique (based on a study done at our center). I am very
excited about this new technique and have made many improvements in the
way this operation is done, since I first developed this technique in
early 1998. So far, I have treated over 60 children with the RED device,
but have continued to perform the standard LeFort III in older children.
Other Pfeiffer Syndrome Treatments:
Skull Surgery
| Treating Sleep Apnea
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Syndrome
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