Craniosynostosis and craniofacial dysostosis: diagnosis and surgical management

Abstract

Craniosynostosis is defined as the premature fusion or absence of 1 or more of the cranial vault sutures and is associated with clinically significant neurologic and morphologic consequences. Growth is arrested perpendicular to the fused suture and a compensatory overgrowth occurs across the sutures that remain open. The result is a characteristic craniofacial dysmorphology and lack of cranial vault growth that restricts the growing brain. Management of craniosynostosis requires release of the involved suture(s) combined with the dismantling and reconstruction of the dysmorphic skeletal components. Plagiocephaly resulting from postnatal external deformational forces (benign positional skull molding) is generally not associated with any functional neurologic problems and is not a surgical problem.