Thursday, November 13, 2008

WHAT???

Following definition courtesy of http://www.cappskids.org/- a great resource for craniosynososis.
Craniosynostosis is a congenital anomaly characterized by the premature closure of one or more cranial sutures (the fibrous joints between the bones of the skull). The disorder results in an abnormal skull and head shape. Most cases are sporadic, although there are several genetic syndromes with Craniosynostosis. Disorders of the fibroblast growth factor receptor molecule have been recently found in some cases of hereditary Craniosynostosis.
Five separate bones make up the cranium of the head: the occipital bone (back portion of the skull); two parietal bones (on either side of the skull); and two frontal bones (front portion of the skull). The bones, which are in place by the 5th month of gestation, are like pieces of a puzzle, and are initially separated by sutures (or fibrous joints). The skull bones, coupled with the sutures, form a protective shield for the brain.
As the brain grows, the sutures allow the skull to expand rapidly. If for any reason one or more of the sutures closes too early, the skull grows in a different direction where the bones are not resisting growth, thereby resulting in skull deformities.
In our childrens case- it is metopic craniosynostosis. This means the frontal bones which come together to form the forehead closed too soon. The metopic suture is usually the first to close in normal development so the appearance is not far from normal aside from the prominent ridge. The incidence of craniosynostosis is 1 in 3,000 live births and of the cases of this only 5-10% are of the metopic suture. So the incidence of metopic craniosynostosis is between 1 in 30,000 to 1 in 60,000. If you are good at math you will find that we have three out of three kids with this condition which is well...uncommon... which brings us to the question of why?

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