Craniosynostosis
From Holoprosencephaly
Craniosynostosis
This is a birth defect 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. The abnormally shaped skull that results is due to the brain not being able to grow in its natural shape because of the closure. Instead it compensates with growth in areas of the skull where the cranial sutures have not yet closed. The condition can be gene-linked, or caused by metabolic diseases, such as rickets or an overactive thyroid. Some cases are associated with other disorders such as microcephaly (abnormally small head) and hydrocephalus (excessive accumulation of cerebrospinal fluid in the brain).
Sagittal synostosis (scaphocephaly) is the most common type. It affects the main (sagittal) suture on top of the head. The early closing forces the head to grow long and narrow, instead of wide. Babies with this type of craniosynostosis tend to have a broad forehead.
Frontal plagiocephaly is the closure of one side of the suture that runs from ear to ear on the top of the head.
Metopic synostosis is a rare form of craniosynostosis that affects the suture close to the forehead. The child's head shape may be described as trigonocephaly, and the deformity may range from mild to severe.
Signs/ Symptoms
The first sign of craniosynostosis is an abnormally shaped skull. A“ridge” of bone may be felt along the cranial suture lines. Other features can include signs of increased intracranial pressure, developmental delays, or mental retardation, which are caused by constriction of the growing brain. Seizures and blindness may also occur.
Diagnosis
An x-ray, CT scan or MRI scan of the head may be completed
Treatment
Treatment for craniosynostosis generally consists of surgery to relieve pressure on the brain and the cranial nerves. For some children with less severe problems, cranial molds can reshape the skull to accommodate brain growth and improve the appearance of the head.
Prognosis
The prognosis for craniosynostosis varies depending on whether single or multiple cranial sutures are involved or other abnormalities are present. The prognosis is better for those with single suture involvement and no associated abnormalities.
Source: http://www.ninds.nih.gov/disorders/craniosynostosis/craniosynostosis.htm
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