Syndromic Craniosynostosis: Unique Management Considerations

Abstract

Craniosynostosis involves the premature fusion of 1 or more cranial sutures and commonly presents as an isolated, nonsyndromic diagnosis. A subset of patients have syndromic craniosynostosis. Several unique considerations must be taken into account when managing patients with syndromic craniosynostosis. A multidisciplinary craniofacial team with a central coordinator is particularly useful for coordinating care among various specialists, and close monitoring is mandatory owing to the increased risk of intracranial hypertension. Surgical management varies among centers, but core options include fronto-orbital advancement with cranial vault remodeling, posterior vault expansion, endoscopic-assisted suturectomy with postoperative orthotic therapy, and midface advancement.

Keywords: Apert; Craniosynostosis; Crouzon; Muenke; Pfeiffer; Saethre–Chotzen; Syndromic.