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Craniosynostosis

Craniosynostosis is a birth defect of the brain 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 following Washington University pediatric neurosurgeon and pediatric plastic surgeon treat patients for craniosynostosis at St. Louis Children's Hospital.

Matthew D. Smyth, MD - Neurological Surgery
Craniofacial Surgery Program Co-director at St. Louis Children's Hospital

Albert S. Woo, MD - Plastic and Reconstructive Surgery
Chief of Pediatric Plastic Surgery at St. Louis Children's Hospital

FOR APPOINTMENTS OR INFORMATION CALL 314-454-2811
Monday through Friday from 8:00 am to 5:00 pm (CST).

In contrast to normal skull growth, in which the skull expands uniformly to accommodate the growth of the brain, premature closure of a single suture restricts the growth in that part of the skull and promotes growth in other parts of the skull where sutures remain open. This results in an abnormal shape of the skull, but does not prevent the brain from expanding to a normal volume.

However, when many sutures close prematurely, the skull cannot expand to accommodate the growing brain, which leads to increased pressure within the skull and impaired development of the brain. Craniosynostosis 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). The first sign of craniosynostosis is an abnormally shaped skull.

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.

Craniosynostosis Treatment and Prognosis

Treatment for craniosynostosis generally consists of surgery to improve the symmetry and appearance of the head and to relieve pressure on the brain and the cranial nerves. Surgery should allow the brain adequate space to grow and develop if there is no underlying brain abnormality.

For some children with less severe problems, cranial molds can reshape the skull to accommodate brain growth and improve the appearance of the head.

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. 

Read more about craniosynostosis on the St. Louis Children's Hospital web page.

Other web resources on craniosynotosis:

Information Courtesy of National Institutes for Health web site at Nih.gov
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Copyright 2013 Washington University School of Medicine