Apert Sydrome: Treatment
Skull Surgery:
I believe that we have developed some innovative techniques at our center
that have improved both the safety and the results of surgery. All children
undergo evaluation by the appropriate members of the craniofacial team
(craniofacial surgeon, pediatric neurosurgeon, anthropologist, geneticist,
psychologist, neuroradiologist, craniofacial nurse coordinator, a speech
pathologist, and social worker if needed) prior to deciding on surgical
treatment. In my practice, the timing for the skull surgery depends on
the type of skull the child has. If the skull bones are all fused from
birth, the first operation a child with Apert syndrome will undergo is
a procedure to enlarge the skull (also called cranial vault remodeling).
If a child has a split metopic suture (a large soft spot), I tend to delay
surgery a little longer, and will begin with a release of the hands and
feet first. Delaying the initial skull operation, as long as it is safely
possible, reduces the total number of skull procedures required. As the
infant gets bigger, there is a decreasing likelihood that a blood transfusion
will be necessary during the operative procedure; therefore, surgery before
12 weeks of age is uncommon unless the deformity is quite severe. At our
center, we routinely give children erythropoietin before surgery to raise
the child's blood levels sufficient to hopefully prevent the need for
blood transfusions (see [
], About Procrit ). We also use a "cell-saver" to recycle some of the blood
that is lost at surgery, so that it can be given back to the child. Using
this combination of techniques, most children do not require any blood
transfusions.
At our center, operations on the skull are always performed with both
a pediatric neurosurgeon and craniofacial surgeon present for the entire
operation. I believe that having two experienced surgeons present at all
times improves the speed and safety of the operation. In addition, only
pediatric anesthesiologist's with extensive craniofacial surgical experience
are used. The family is given hourly updates as to their child's condition
during the operation. Typically, skull surgery takes about four hours.
We do not shave any hair for surgery, although many centers still prefer
to do so. The skull is reached through an incision that extends from ear
to ear across the top of the head. Many years ago, I changed the typical
straight-line incision to a wavy, "zigzag" incision. I developed this
wavy pattern incision because I noted that when children get their hair
wet, it parts on a straight-line scar (see
[]). Making the incision in a wavy pattern helps to better
hide the scar, and many other craniofacial surgeons have now adopted this
technique.
The purpose of the surgery is to remove the areas of skull that were
affected by the fused suture and reposition the skull to allow the brain
to grow. The techniques used to achieve this goal will vary from surgeon
to surgeon. I base the decision on how to rebuild the skull on a number
of factors: including the CT Scans with 3-dimensional reconstructions,
measurements made by our anthropologist, photographs that are blown up
to life-size (I have used a medical artist who traces over the life-size
photographs and using tenets of art makes suggestion for surgical movements),
and finally, I rely on aesthetic judgment.
Surgeons use various techniques to hold the skull bones in place after
moving them into position. We have previously reported our findings concerning
the use of metal plates and screws to hold the skull bones together at
the end of the operation. We found that when we re-operated on children
in whom other surgeons had placed metal plates and screws, with subsequent
growth of the skull, these plates will end up on the inside of the skull
with the screws poking into the brain (see
[]). While I am not aware of any case in which this has
caused a problem I, nevertheless, have chosen to use dissolving stitches
to put the bones together instead of plates (see
[]).
With this technique, when the child grows up nothing artificial will be
left behind. Fairly recently, dissolving plates and screws have been developed
for use in the skull and facial bones. Although I occasionally find that
dissolving plates are useful, it is my preference not to use them for
a number of reasons, including the finding that they can end up inside
the skull in growing infants. For a short time I evaluated using distraction
devices with skull surgery, with the hope of being able to move the skull
bones further forward. However, I found the results with skull distraction
were not sufficiently positive to compensate for the additional surgery
required with the distraction technique, and the complication rates were
also higher with skull distraction. Therefore, I do not currently recommend
this technique.
At the end of the operation, the scalp is closed with dissolving stitches.
I never use metal staples, as these can hurt when they are removed. I
also do not put any bandages on the child, nor are any drainage tubes
placed. Instead the child is given a shampoo before leaving the operating
room and the hair is combed over the incision. Many surgeons do like to
use head wrap bandages. I do not think that this is wrong; I simply believe
that these bandages are completely unnecessary.
After surgery, children usually will spend one night in the pediatric
intensive care unit and is transferred to the floor the following day.
The child spends a total of 48-72 hours in the hospital, prior to discharge.
The risks of the surgery are very small at experienced centers. In a recent
published report looking at our center and another center's combined experience
(see
[]), no infections were noted in infants undergoing operations
for the first time (however, it is nevertheless possible for a infant
to get an infection).
When this type of operation is performed on older children, they tell
us that there is very little discomfort associated with this operation.
Nevertheless, I have recently instituted the policy of playing soft music
in the child's room after surgery. Soft music has been shown to decrease
the amount of pain medicine that a child requires after an operation.
Children with Apert syndrome usually need two to three operations on
their skull over a lifetime. It is very important that children be followed
closely to monitor for raised intracranial pressure until early teenage
years. In addition, we also monitor for cerebellar tonsillar herniation
and enlarging ventricles.
Other Apert Syndrome Treatments:
Treating Sleep Apnea
| Midface Surgery
| Hands & Feet
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