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Craniosynostose

  • Article
  • 2021-03-07

Craniosynostosis is a birth defect of the skull characterized by the premature closure of one or more of the fibrous joints between the bones of the skull (called the cranial sutures) before brain growth is complete. Closure of a single suture is most common. Normally the skull expands evenly to accommodate brain growth; premature closure of a single suture limits growth in that part of the skull and promotes growth in other parts of the skull where the sutures remain open. This results in a misshapen skull, but does not prevent the brain from expanding to normal volume. However, when many sutures close prematurely, the skull is unable to expand to accommodate the growing brain, leading to increased pressure in the skull and reduced brain development. Craniosynostosis can be gene-linked or caused by metabolic diseases (such as rickets) or an overactive thyroid gland. Some cases have been associated with other conditions such as microcephaly (abnormally small head) and hydrocephalus (excessive accumulation of cerebrospinal fluid in the brain). The first sign of craniosynostosis is an abnormally shaped skull. Other features may be signs of increased intracranial pressure, developmental delays or decreased cognitive development caused by narrowing of the growing brain. Seizures and blindness can also occur. Other features may be signs of increased intracranial pressure, developmental delays or decreased cognitive development caused by narrowing of the growing brain. Seizures and blindness can also occur. Other features may be signs of increased intracranial pressure, developmental delays or decreased cognitive development caused by narrowing of the growing brain. Seizures and blindness can also occur.

Therapy

Treatment for craniosynostosis usually consists of surgery to improve the symmetry and appearance of the head and to relieve pressure on the brain and cranial nerves. In some children with minor problems, skull shapes can reshape the skull to accommodate brain growth and improve the appearance of the head.

Prognosis

The prognosis for craniosynostosis varies depending on whether there are single or multiple cranial sutures or other abnormalities. The prognosis is better for people with a single attachment and no associated abnormalities.


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