Simplified Explanation
Early fusion of the skull plates. As the brain grows it pushes against the fused plates and causes the head to become misshaped. CT scans and MRI’s are used to confirm suture fusion. Babies born with craniosynostosis almost always needs surgery to release the skull joints to allow room for the developing brain to grow. There are multiple types of craniosynostosis that fall under two general categories syndromic and non-syndromic:
- Syndromic: Syndromic craniosynostosis (such as Pfeiffer syndrome) have additional anomalies that affect other parts of the body. A syndromic diagnosis can be made with a physical evaluation and/or genetic testing. Not always, but most of the time the genetic mutation can be found with syndromic craniosynostosis.
- Non-syndromic:One or more of the cranial sutures are fused at birth or shortly after birth. But, there are no other physical anomalies.
Skull sutures are the joints between the skull bones. Kids born with Pfeiffer syndrome typically have their Coronal sutures fused at birth, in many cases other sutures are fused as well.
(credit NIH)
Treatment
Cranial vault remodeling is the surgical procedure used to separate fused sutures. A zig-zag incision is made along the skull from ear to ear and the bone is cut to release the suture. Screws and may or may not be used.
Endoscopic surgery is a less invasive surgery done by making small incisions and using tools through these openings to release the fused sutures. This surgery is best done early. Following endoscopic surgery the baby will need to wear a helmet for a period of time that helps reposition the plates of the skull.
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