Second Craniosynostosis Corrections

How do parents know if their child needs another operation, after they have already undergone one operation that was supposed to have fixed their child’s craniosynostosis? At our center we have found that there are three reasons that children end up having to undergo repeat craniosynostosis corrections:

  1. Signs of raised intracranial pressure. Children may start complaining of more frequent or severe headaches. If children are too young to be able to verbalize that their head hurts, parents might have a sense that their child’s behavior has changed or that their child seems more fussy.
  2. Parents notice that their child’s head does not look normal. The forehead may seem irregular, or the overall shape seems wrong.
  3. There are holes in the skull that are large enough that they may be leaving the brain unprotected. Sometimes parents can see the scalp pulsating with each heartbeat.

The decision as to whether or not a child needs to undergo a second (or even third) operation is not easy one and is best made by those who primarily specialize in treating craniosynostosis and not by surgeons who only occasionally treat this condition. Many years ago it was thought that all children with a single sutural craniosynostosis could be successfully treated with just one single operation. More recently, studies have shown that up to 40% of children with craniosynostosis either end up getting redo operations, or are judged to need a repeat procedure. As our center has gained more experience in reoperating on children who had already undergone craniosynostosis corrections at other hospitals, we have discovered that different surgeons treat this condition in very different ways and no one center seems to correct this condition in exactly the same way. So, if your child needs a repeat operation, what went wrong? There seem to be two basic causes for children requiring redo operations: poor postoperative growth (the surgery was performed at too young an age), or the initial surgery was not done in the best way. In order to better understand why so many children seem to need more than one operation, it is helpful to have a basic understanding of skull growth. Studies performed at our center in Dallas have shown that the growth of the skull is not normal following craniosynostosis corrections, with a tendency for the skull shape to return towards the way it was before the correction. One of the causes for this reduced postoperative growth is the surgery itself. Operating on the skull causes the future skull growth to slow down. Therefore, the sooner the operation is done after a child is born, the more limited the skull’s ability to grow and the more likely it is that a second operation will be necessary as the child gets older. One published study from our center has shown that when craniosynostosis corrections were done closer to a year of age, better long-term growth of the skull was noted compared to those infants operated on before 6 months of age. The other reason some children need to have their craniosynostosis operations redone is that the surgery may not have originally been performed in the best way. One example would be when surgeons perform strip craniectomies (removing skull bone and leaving it out) in older infants. This technique is dependent on bone reforming to fill in the area that was discarded. If the skull does not completely regrow, then there will be holes in the skull that later need to be patched using the child’s own bone. An additional issue with strip craniectomies is that they often do not create an expansion that is sufficient to accommodate the rapidly growing brain. Another example of a suboptimal correction is when surgeons recommend the use of restrictive headbands, or helmets, after surgery. The principle behind using these bands is to limit skull growth in some areas, forcing the growing brain to push out the skull in other areas. We believe that this restriction in growth can result insufficient expansion of the skull, increasing the likelihood that a later expansion might need to be surgically performed. Another potential problem with headbands is that these rely on the capability of the orthotic technician to get the skull molded into the best shape. If this technician is unable to achieve a normal shape, subsequent corrective surgery may be necessary. Probably the most important contributor to the need to repeat a craniosynostosis correction is when the surgeon does not sufficiently over-correct the skull shape (to compensate for future reduced growth) during the initial surgery. So, with the inhibited growth after surgery the skull is more likely to grow back the way it was, making a second correction necessary.
No parent wants their child to look “operated on,” but it can be very challenging for a surgeon to achieve a completely normal appearance after a child has already undergone an earlier operation. Second operations are almost always more complex than first operations. The more operations that have been done, the less normal a parent should expect their child’s skull to appear with redo operations. Families faced with the need for their child to undergo a repeat operations should ideally seek out the most skilled and experienced teams they are able to travel to, in order to maximize the odds of their child achieving a normal appearance that will last them a lifetime, and minimize the chances that yet a 3rd, or even 4th operation might be necessary later on.

For a further discussion on this topic you can email, write or call Dr. Fearon:

Jeffrey A. Fearon, MD
The Craniofacial Center
7777 Forest Lane, Suite C-700
Dallas, Texas 75230