craniosynostosis

Craniosynostosis

What causes craniosynostosis?

Whats Craniosynostosis?

Craniosynostosis is most often sporadic (occurs by chance). In some families, craniosynostosis is inherited in one of two ways:

Diagnostic testing

•Autosomal recessive - Autosomal recessive means that two copies of the gene are necessary to express the condition, one inherited from each parent, who are carriers. Carrier parents have a one in four, or 25 percent, chance with each pregnancy, to have a child with craniosynostosis. Males and females are equally affected.

x-rays of the head - a diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues and bones of the head onto film.

Craniosynostosis is a birth defect in which one or more of the joints between the bones of your infant's skull close prematurely,before your infant's brain is fully formed.

What are the different types of craniosynostosis?

computerized tomography scan (Also called a CT or CAT scan.) of the head - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the head. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.

•Autosomal dominant - Autosomal dominant means that one gene is necessary to express the condition, and the gene is passed from parent to child with a 50/50 risk for each pregnancy. Males and females are equally affected.

Plagiocephaly - It involves fusion of either the right or left side of the coronal suture that runs from ear to ear. This is called coronal synostosis and it causes the normal forehead and the brow to stop growing. Therefore, it produces a flattening of the forehead and the brow on the affected side, with the forehead tending to be excessively prominent on the opposite side.

There are numerous types of craniosynostosis. Different names are given to the various types, depending on which suture, or sutures, are involved, including the following:

When your baby has craniosynostosis, his or her brain can't grow in its natural shape and the head is misshapen.

•Trigonocephaly - Trigonocephaly is a fusion of the metopic (forehead) suture. This suture runs from the top of the head down the middle of the forehead, toward the nose. Early closure of this suture may result in a prominent ridge running down the forehead. Sometimes, the forehead looks quite pointed, like a triangle, with closely placed eyes (hypotelorism).

•scaphocephaly - Scaphocephaly is an early closure of fusion of the sagittal suture. This suture runs front to back, down the middle of the top of the head. This fusion causes a long, narrow skull. The skull is long from front to back and narrow from ear to ear.

Surgery

Fun Facts

Surgery is done by a team which includes both a Craniofacial Plastic Surgeon and a Neurosurgeon. For a Cranial Vault Reconstruction (CVR ) surgery, the head is cut in a zig-zag pattern from ear-to-ear (which allows for the scar to be more easily covered with hair in the future).

The skull bone is then cut to release the fused suture. After the suture is released, the skull is then stabilized with dissolvable plates and screws

When you have Craniosynostosis, your an adult infant when your born.