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Fontanel surgery (Craniosynostosis)


Pytanie

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

Odpowiedź naszych ekspertów

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

Zatwierdzone przezClinical Advisory Board (CAB)
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Zalecenie(a)

Autonomic nervous system

R47
Napady padaczkowe u osób z CdLS powinny być leczone zgodnie z ogólnymi schematami postępowania.
R48
Badanie MRI mózgu powinno być rozważane tylko wtedy, gdy u osoby z CdLS występują objawy neurologiczne inne niż mikrocefalia (głowa mniejsza niż normalna).

Zalecenie(a)

Paediatric Medical Care

R47
Napady padaczkowe u osób z CdLS powinny być leczone zgodnie z ogólnymi schematami postępowania.
R48
Badanie MRI mózgu powinno być rozważane tylko wtedy, gdy u osoby z CdLS występują objawy neurologiczne inne niż mikrocefalia (głowa mniejsza niż normalna).

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Please take note that the Ask the Expert service is comprised of volunteer professionals in various areas of focus. Answers are not considered a medical, behavioral, or educational consultation. Ask the Expert is not a substitute for the care and attention your child’s personal physician, psychologist, educational consultant, or social worker can deliver.

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