Open Cranial Vault Remodeling in a Child with Crouzon Syndrome Presenting with Turricephaly

A BSTRACT Craniosynostosis, characterized by the premature fusion of cranial sutures, can lead to abnormal skull development and increased intracranial pressure. Turricephaly, a specific manifestation involving the vertical elongation of the skull, often results from the fusion of multiple sutures and presents functional and esthetic challenges. Crouzon syndrome, an autosomal dominant disorder caused by mutations in the fibroblast growth factor receptor 2 gene, is a common syndromic form of craniosynostosis, frequently involving the coronal and sagittal sutures, leading to complex craniofacial deformities. This case report details the surgical management of a pediatric patient presenting with turricephaly secondary to craniosynostosis. The patient underwent open cranial vault remodeling, a procedure involving strategically removing and reshaping cranial bones to correct deformities and accommodate brain growth. Postoperative outcomes were favorable, with the patient exhibiting improved cranial morphology and no neurological deficits. This case underscores the efficacy of open cranial vault remodeling in addressing complex cranial deformities and highlights the importance of timely intervention to optimize neurodevelopmental outcomes.