Craniofacial Anomalies

Craniosynostosis Syndromes

Signs and Symptoms
Diagnosis
Treatment

Signs and Symptoms

Craniosynostosis is a term that refers to the early fusing or closing of one or more of the sutures (openings) in the skull during fetal development. The skull is composed of multiple bones separated by sutures. If any of these fuse prematurely, the skull expands in the direction of the open sutures, which results in an abnormally shaped head.

Premature closure of a single suture sometimes is caused by space constraints in utero, which is why it occurs more often in twins. Single suture closure usually is not associated with a syndrome nor is it genetic in nature.

Premature closure of multiple sutures, including facial sutures, is seen in individuals with a craniosynostosis syndrome. The most common craniosynostosis syndromes are Crouzon, Pfeiffer and Apert. In these syndromes, several sutures in the head are fused, which results in abnormal head shapes. Often bones in the face also are fused, making the middle area of the face flat and the eyes protrude. Other characteristics vary by syndrome, for example children with Apert syndrome have syndactyly (webbing) of the hands and feet.

Reviewed by health care specialists at UCSF Children's Hospital.
Last updated May 8, 2007