Craniosynostosis is a condition in which one or more of the fibrous sutures in a young infants skull prematurely fuses by turning into bone (ossification), thereby changing the growth pattern of the skull. Because the skull cannot expand perpendicular to the fused suture, it compensates by growing more in the direction parallel to the closed sutures. Sometimes the resulting growth pattern provides the necessary space for the growing brain, but results in an abnormal head shape and abnormal facial features. In cases in which the compensation does not effectively provide enough space for the growing brain, craniosynostosis results in increased intracranial pressure leading possibly to visual impairment, sleeping impairment, eating difficulties, or an impairment of mental development combined with a significant reduction in IQ.Craniosynostosis occurs in one in 2000 births. Craniosynostosis is part of a syndrome in 15% to 40% of affected patients, but it usually occurs as an isolated condition. The term is from cranio, cranium; + syn, together; + ost, relating to bone; + osis, denoting a condition. Signs and symptoms
Children born with craniosynostosis have a distinct phenotype, i.e., appearance—observable traits caused by the expression of a conditions genes. The features of craniosynostosis particular phenotype are determined by which suture is closed.
The bones are then replaced and fixated to form a cranial vault with a normalized shape.Retrospective analysis has given an indication that the use of total cranial vault remodelling provides children with a better cephalic index than does the extended strip craniectomy.An approach that is currently being evaluated involves the use of springs. This intervention is likely most effective when used in the time frame between three and six months of age. Metopic synostosis/trigonocephaly
The main elements of metopic suture closure involve a low volume of the anterior cranial fossa, the metopic ridging and hypotelorism. These problems are all addressed during the surgical intervention.The volume is increased by placing the frontal bones and the supraorbital rim further forward. This is done by excision of the bones after which they are reshaped with greenstick fracturing. Replacement of the bones provides a possibility for the correction of the hypotelorism at the same time. A bone graft is placed in between the two halves of the supraorbital bars, thereby increasing the width between the orbits. The metopic ridge can then be corrected with a (simple) burring. Unilateral coronal synostosis/anterior plagiocephaly
The treatment of unilateral coronal synostosis is typically performed in two parts: the forward advancement of the supraorbital bar and the correction of the orbital asymmetry.The supraorbital bar is the rim just above the eye socket; as discussed under phenotype, the supraorbital and the frontal bone are typically recessed at the ipsilateral side of the head.