Progressive Multisutural Craniosynostosis in Syndromic and Nonsyndromic Patients: Identification of Patterns and Risk Factors
Aditi M. Kanth, MD, Brittany Nguyen, MD, Anusha Pasumarthi, BA, Ariana Adamski, BA, Courtney Carpenter, MD, Oluwaseun A. Adetayo, MD, Matthew A. Adamo, MD.
Albany Medical Center, Albany, NY, USA.
Background: Secondary craniosynostosis, or the development of fusion of an initially patent suture, is rare, with poorly understood risk factors. Prior studies have identified elevated risk in patients with craniofacial syndromes. Bicoronal craniosynostosis has also been identified as a risk factor, however its association with syndromic diagnosis is a potential confounder. Recent reports have also identified endoscopic suturectomy with barrel staving as a potentiating factor in nonsyndromic patients. The purpose of this study is to identify potential patterns and risk factors in both the syndromic and nonsyndromic patients who develop secondary craniosynostosis.Methods: A retrospective review was performed of an institutional database of pediatric patients treated for craniosynostosis with at least 2 year follow up. Patients who developed progressive multisutural craniosynostosis (PMSC) were identified and compared to those who did not (control group). Data collection included initial fused suture, operation, time to operation, syndromic diagnosis, additional sutures fused, and time to PMSC. The authors independently examined the syndromic and nonsyndromic cohorts. Statistical analysis was performed with SPSS.Results: 76 patients were included. 8 patients (10.5%) developed PMSC. The mean time to PMSC was 23.3 months. Endoscopic strip craniectomy did not have a significant association with the development of secondary synostosis (p=0.75). 3 of the 8 patients with PMSC (37.5%) and 5 of the 68 patients in the control group (7.4%) were syndromic, with a significant association between syndromic diagnosis and PMSC (p=0.03). In the nonsyndromic cohort, 7.3% developed PMSC. There was no significant difference between the nonsyndromic PMSC group and control group with regard to age at first surgery (p=0.49) or treatment with endoscopic strip craniectomy (p=0.41). In the nonsyndromic PMSC group, the initial suture fused was bicoronal in 2 patients, metopic in 2 patients, and sagittal in 1. Fisher exact test revealed a significantly higher likelihood of developing PMSC if the initial fusion was bicoronal in nonsyndromic patients (p=0.02). The most common secondary synostosis was sagittal. In the syndromic cohort, 37.5% patients developed PMSC. None were treated with endoscopic strip craniectomy. Within the syndromic cohort, no significant findings were found between the PMSC and control groups with regard to suture fusion patterns.Conclusion: We identified bicoronal synostosis as an independent risk factor for PMSC in nonsyndromic patients. Endoscopic suturectomy without barrel staving did not have a significant association with PMSC. These findings add to the literature and confirm the complex interplay between genetics and intervention in the development of PMSC.
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