Syndromic vs Non-Syndromic Management of Multisuture Craniosynostosis: A Single-Center Experience

Introduction: Multisuture craniosynostosis (CS) involves the fusion of two or more cranial sutures and can be divided into syndromic and non-syndromic craniosynostosis. The surgical and medical management of children with multisuture CS have evolved over time to improve aesthetic and neurodevelopmental outcomes. We plan to describe and analyze the incidence, management and outcomes of multisuture craniosynostosis in syndromic and non-syndromic patients at our institution over an 11-year period.

Methods: Retrospective study of all pediatric patients with multisuture craniosynostosis that received care at Texas Children’s Hospital (TCH) between September 2009-August 2023. Demographics, clinical data, imaging studies, operative interventions, and clinical follow-up were evaluated. Chi square test was used to compare categorical variables and Kruskal Wallis test was used for continuous variables.

Results: 75 patients with multisuture CS who underwent surgery were identified: 36 were syndromic and 39 children were non-syndromic. Among syndromic patients, there were higher number of male (n = 44) and Hispanic patients (n = 37). The median number of surgeries was 2, and the average age at first-craniosynostosis surgery was 11 months (Table 1). The most common type of procedure was fronto-orbital advancement (FOA) (n = 30) . The most common syndrome was Crouzon (n = 7). Compared to non-syndromic children, syndromic children had a higher median total number of CS surgeries (p = 0.0027) and differed in the types of pre-operative sutures fused before the 1st CS surgery (p=0.001) . The rate of craniocervical pathology, mostly Chiari Type 1, was higher in the syndromic children (38.9%) compared to non-syndromic children (23.1%). One out of three (33.3%) syndromic patients had a shunt placed (p=0.001). Two out of three (66.7%) syndromic children required 2 total number of CS surgeries at their latest follow-up (0.007).

Conclusion : We found that syndromic children had a higher total median number of CS surgery, had first surgery at a younger age, had a higher incidence of shunt placement, and had a higher incidence of Craniocervical pathology.