Craniosynostosis (premature fusion of the fontanel)

Is premature fusion of the fontanel common in CdLS? What is the recommended treatment?

The entity is called craniosynostosis, or premature fusion of the sutures. Normally, the skull is made up of several separate bones that eventually fuse together at the suture sites, but in the meantime allowing the brain to grow. The fontanel is the center point for these sutures and normally closes by 18 months. Premature fusion of the sutures will lead to abnormal head shape and growth and can limit the brain’s growth. There are a number of causes of this. One cause is poor brain growth itself can lead to early fusion (microcephaly). Other causes include specific syndromes and abnormal positioning of the head in the uterus. The standard treatment is surgical release and usually this is needed for cosmetic reasons as well as allowing the brain to grow. I have noted a few patients with CdLS who reported craniosynostosis repairs but it is not a common malformation. Sometimes you can wait and see if the fusion will progress, but usually if the surgery is recommended by a pediatric neurosurgeon, it is needed. A parent could get a second opinion if very concerned. I believe it’s a relatively safe surgery.

TK 7-13-10

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Answer Published On: October 18th, 2018 9:02 PM