Craniosynostosis

Definition

Craniosynostosis is a congenital (present at birth) defect that causes one or more sutures on a baby's head to close earlier than normal. Sutures are connections that separate each individual skull bones. The early closing of a suture leads to an abnormally shaped head.

Alternative Names

Causes

The cause of craniosynostosis is unknown. Which suture is involved determines the abnormal shape of the head.

A person's genes may play a role in craniosynostosis. The hereditary form often occurs with other defects that can cause seizures, diminished intellectual capacity, and blindness. Genetic disorders commonly associated with craniosynostosis include Crouzon, Apert, Carpenter, Chotzen, and Pfeiffer syndromes.

However, most cases of craniosynostosis occur in a family with no history of the condition and children with craniosynostosis are otherwise healthy and have normal intelligence.

There are different types of craniosynostosis. Sagittal synostosis (scaphocephaly) is the most common type. It affects the main (sagittal) suture on the very 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. It is more common in boys than girls.

Frontal plagiocephaly is the next most common form. It is the closure of one side of the suture that runs from ear to ear on the top of the head. It is more common in girls.

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.

References

Goetz CG. Textbook of Clinical Neurology. 2nd ed. St. Louis, Mo: WB Saunders; 2003: 576-577.

Behrman RE, Kliegman RM, Jenson HB. Nelson Textbook of Pediatrics. 17th ed. St. Louis, Mo: WB Saunders; 2004:1992-1993.

Ridgway EB. Skull deformities. Pediatr Clin North Am. 2004; 51(2): 359-87.