Craniosynostosis

What is craniosynostosis?
The term craniosynostosis refers to premature fusion or closure of one or multiple cranial sutures. Craniosyostosis is commonly present at birth, but is not usually noticeable.

How does craniosynostosis present?
In the early months of an infant’s life it presents as a skull deformity. The clinical manifestations include facial and cranial deformity and intracranial pressure elevation in early closure of sutures. Children who have elevated intracranial pressure can experience chronic headaches, poor school performance and visual failure.

What testing tool is available to physicians in making a diagnosis of craniosynostosis?
The introduction of computerized tomography (CT) scan in the late 1970’s offered a new tool for visualizing such a deformity. Cranial computed tomography is more accurate than plain radiographs of the skull.

Is this a common condition?
The incidence of craniosynostosis is estimated to be 1 case in 2000 live births.

What is the cause of craniosynostosis?
The cause is not known at the present time. Its association with genetic defects is becoming more widely recognized. A familial occurrence has been noticed in 4-10% of the patients.

What are the social impacts of craniosynostosis on children with this deformity?
As these children grow, abnormal facial appearance has a negative impact on their social integration with an associated effect on personality development.

What is the medical treatment for craniosynostosis?
No medical treatment or medication exists for craniosynostosis. However, surgical treatment is available. Early surgical correction of the deformity by a pediatric neurosurgeon is recommended.

What is the role of helmets in craniosynostosis?
Positional plagiocephaly (PP) results from children lying on their back (supine position). This position is recommended as preferred and safe position for infants during sleep. However, it has also resulted in positional plagiocephaly. When PP is severe, these children can be treated with plastic caps which are fitted externally on the head and can gradually manipulate the shape of the skull. No clear benefit has been identified and tolerance can be poor. Most cases of mild PP do not require treatment.