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 publications [ #19, June '02], 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 publications [ #10, 1994]). 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 publications [#12, 1995]). 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 publications [#20]). 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 publications [#15, 1997]), 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.