Syndromic craniosynostosis: current concepts in intracranial hypertension, cranial base pathology, and surgical management
- Author:
So Young LIM
1
Author Information
- Publication Type:Review Article
- From:Archives of Craniofacial Surgery 2026;27(3):113-119
- CountryRepublic of Korea
- Language:English
- Abstract: Syndromic craniosynostosis is characterized by premature fusion of multiple cranial sutures accompanied by progressive craniofacial dysostosis and neurological complications. Although reduction of intracranial volume (ICV) was historically regarded as the principal mechanism underlying elevated intracranial pressure (ICP), contemporary evidence suggests that intracranial hypertension in syndromic craniosynostosis is multifactorial. Abnormal cerebrospinal fluid circulation, venous hypertension, upper airway obstruction, and premature cranial base fusion appear to contribute substantially to disease progression. A narrative review was performed using previously published clinical studies, radiologic investigations, and institutional experience regarding syndromic craniosynostosis, with emphasis on Crouzon syndrome, cranial base suture fusion, increased ICP, hydrocephalus, and surgical outcomes. Recent radiologic investigations demonstrated that cranial base sutures and synchondroses exhibit characteristic physiologic closure patterns during childhood. In syndromic craniosynostosis, particularly Crouzon syndrome, premature cranial base fusion correlates with recurrent increased ICP and repeated cranial vault remodeling procedures. Elevated ICP cannot be explained solely by reduced ICV because many patients maintain relatively normal ICV through compensatory cranial growth. Clinical manifestations of intracranial hypertension are often nonspecific, whereas papilledema and radiologic changes remain useful surveillance markers. Contemporary surgical management includes fronto-orbital advancement, cranial vault remodeling, distraction osteogenesis, endoscopic strip craniectomy, and long-term multidisciplinary monitoring. Syndromic craniosynostosis represents a progressive disorder involving not only major calvarial sutures but also cranial base structures. Premature cranial base fusion may serve as an important prognostic factor for recurrent intracranial hypertension and disease severity. Improved understanding of cranial base pathology may facilitate individualized treatment strategies and improve long-term neurological outcomes.
