Craniosynostosis is the improper development of the skull’s bones, which can result in an abnormal head shape. Craniosynostosis refers to the premature fusion of the fibrous joints, or sutures, between certain bones. Primary craniosynostosis is distinguished from secondary craniosynostosis, which occurs due to a failure in brain and may lead to abnormalities in head shape.
While the exact cause of primary craniosynostosis is unknown, the skull abnormalities may result from the abnormal hardening, or ossification, of the cranial sutures. It is usually apparent at birth or within a few months, but mild cases may go undiagnosed until early childhood. In most cases, affected children have normal intelligence and do not suffer from other abnormalities besides head shape. If untreated, this can cause increased pressure, known as intracranial pressure, and can result in cognitive impairment or developmental delays.
Treatment is directed toward specific symptoms. Surgery is the main form of therapy for most, but not all, children and is performed to ensure that there is enough room within the skull for the developing brain to grow, to relieve any intracranial pressure, and to improve the appearance of a child’s head.