S12-15 SESSION 12: FACIOCRANIOSYNOSTOSIS – PART II ENDOSCOPIC STRIP CRANIECTOMY IN CHILDREN WITH APERT SYNDROME

Abstract

Introduction: Apert syndrome is a unique craniofacial disorder that is associated with bilateral coronal craniosynostosis. Due to its rarity, little data exist to demonstrate superiority of surgical technique in treating the associated craniosynostosis. Minimally-invasive endoscopic strip craniectomy (ESC) has been used as a primary treatment in some children, but clinical efficacy to open procedures has not been demonstrated in this population. We present a series of 36 patients with Apert syndrome who underwent open correction (Group 1, n=17) or ESC with helmeting (Group 2, n=19) to determine whether ESC is comparable to open procedures in correcting head growth. Methods: We conducted a retrospective cohort study of children with Apert syndrome treated at Boston Children’s Hospital over a 24-year period. Median (IQR) post-operative follow-up was 185 (70, 208) and 12 (7, 25) months, for Groups 1 and 2, respectively. Postoperative head circumference and growth until 24 months of age were compared between groups using generalized estimating equations (GEE) to account for serial measurements within the same patient and WHO normative growth curves.Wilcoxon rank sum test and Fisher’s exact test were used to compare incidence of morbidity between groups. Results: For groups 1 and 2, Median (IQR) age at operation was 11 (9, 14) and 3 (2, 3) months, respectively. Median operative time was 248 minutes (IQR: 162, 311) and 64 minutes (IQR: 59, 75), respectively (P<0.001). Group 1 had significantly higher rates of blood transfusion, ICU admission, and longer hospital length of stay (all P<0.01). There was no statistically significant difference in refusion rates, complications, need for further procedures, complaints of asymmetry, or postoperative head circumference growth rates between the two groups. Overall, children in Group 1 had larger head circumferences at 2 years of age (P<0.001). Compared to WHO age- and gender-based normative growth curves, all patients in both groups were above the 50thpercentile for head circumference at last follow-up. Conclusion: Children with Apert syndrome and bilateral coronal craniosynostosis treated with ESC and helmeting have rates of head growth parallel to children treated with open correction. Following either intervention, children with Apert syndrome adopt similar head circumferences relative to their age and gender matched peers. The increased head size seen in Group 1 may be attributed to the advancement of the bandeau that is more easily achieved in open procedures compared to ESC. Longer-term assessments are needed to determine the natural history of head growth and its correlation with neurocognitive development in this population.