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Craniosynostosis is a congenital or birth defect, characterized by the premature closure of one or more sutures, which causes an abnormally shaped skull. The skull is normally composed of bones which are separated by sutures. As an infant’s brain grows, open sutures allow them to expand and develop a relatively normal head shape. When one or more sutures close early, it can cause the skull to expand in the direction of the open sutures. This often results in an abnormal head shape and in severe cases, places excessive and damaging amounts of pressure on a growing brain.

Types of Craniosynostosis
There are different types of craniosynostosis. The most common is scaphocephaly in which the sagittal suture is closed. This particular form of craniosynostosis causes the head to grow long and narrow to accommodate the growing brain. It occurs in approximately 1 in 2000 births. In addition, males are affected about three times as often as females.

Another type of craniosynostosis is metopic hypostasis. It typically manifests itself as a visible ridge down the child's forehead and the eyebrows seem to be pinched on either side. The eyes may also appear to be close together.

A third form of craniosynostosis, known as unilateral coronal synostosis, is the one which affected our son, Jorge Luis. This type affects the coronal suture which extends from ear to ear on the top of the head. Early closure of one side results in the forehead and orbital rim having a flat appearance on that side. The other side is pushed forward (sort of like a bump in the forehead),and also one eyebrow is higher than the other one.

Finally, when both sides are fused it is called bicorporal hypostasis. In these cases, the child may have a very flat and recessed forehead.

 

Cause of Craniosynostosis
The cause of craniosynostosis is unknown. It can be hereditary and caused by a genetic defect, or it can occur sporadically in a family with no other affected relatives, like our case with Jorge Luis.

Diagnosis
In order to be able to diagnose this condition it is necessary to measure the circumference of the infant’s head. If deformities are noted, the doctor should order x-rays and confirm the diagnosis by a physical evaluation. In addition, a CT scan should also be performed to ensure that there are no underlying abnormalities in the brain.


Treatment
The primary treatment for craniosynostosis during infancy is surgery. There are three goals for the surgery. First, relieve intracranial pressure. Second, assure that the skull has the capacity to accommodate the brain’s growth. Third, attempt to cosmetically improve the appearance of the child’s head. While the deformity may vary from mild to severe, the psychological effect of a noticeably misshapen forehead, face and head can cause great problems and is a very important reason for seeking treatment.

Disclaimer:

The preceding information should not be viewed as advise on the treatment and/or diagnosis of craniosynostosis or any other medical condition. Advice on the treatment or care of a child suffering from craniosynostosis should be obtained only through consultation with a physician who has examined that child or is familiar with that child's medical history.


Additional Information
National Institute of Neurological Disorders
Children's Hospital at Montefiore
Children's Craniofacial Association (PDF Document)
Foundation for Faces of Children