S4A-16 SESSION 4A: GENERALITIES IN CRANIOSYNOSTOSIS – PART I RELEVANT FACTORS FOR REPEAT SURGERY FOR NON-SYNDROMIC CRANIOSYNOSTOSIS: A 21-YEAR EXPERIENCE AT A SINGLE INSTITUTION.

Abstract

Introduction: The need for additional surgery following open repair of single-suture craniosynostosis is a well-recognized issue. However, factors contributing to secondary surgery are poorly understood and infrequently reported in the literature. We have evaluated the prevalence of symptomatic re-synostosis following repair of non-syndromic craniosynostosis at a single institution over two decades and elucidated several involved factors, as well as the likely significance of elevated intracranial pressure (ICP). Methods: All non-syndromic patients undergoing open surgical repair of craniosynostosis at CNMC between 1998–2019 were examined for symptomatic re-synostosis requiring correction in addition to associated factors (gender, age at initial surgery, affected cranial suture[s]). The presentation of elevated ICP was closely scrutinized. Patients requiring reoperation for other cosmetic issues (e.g., persistent cranial defects) were excluded. Results: 312 patients with non-syndromic craniosynostosis underwent initial open surgical repair at a median age of 7.06 months (2.07–98.2 months). Thirty-three (10.6%) required further surgery at 60.6 months (11.47–189.9 months) for a combination of clinical, cosmetic, radiographic, and ophthalmologic evidence of resynostosis. 55% manifested clinical symptoms of elevated ICP, 57% exhibited decelerated head circumference growth, 85% showed CT/MRI findings of elevated ICP and 12% had papilledema. Intraoperative evidence of increased ICP (thinned calvarium/translucent dura), was frequently noted. Rates of secondary surgery were sagittal (7.2%), unicoronal (10.6%), bicoronal (77%), and metopic (7.1%). Length of time between initial procedure and reoperation was 5.05 years (0.96–15.8 years). Non-syndromic patients with bicoronal synostosis were significantly more likely to experience recurrent synostosis (p < 0.005). All patients requiring reoperation had acceptable cosmetic outcomes (Whitaker Class I/II result) with an average follow-up 8.12 years (3.01–16.9 years). Conclusion: This study of recurrence following open surgical repair of non-syndromic craniosynostosis represents the largest to date. Given the prolonged time interval between initial surgery and recurrence, long-term surveillance for symptomatic re-synostosis up to a decade post-operatively should be encouraged. It is also critical to evaluate for any likelihood of increased ICP, as this may present in a subtle and insidious fashion. Additionally, parents of patients with non-syndromic bicoronal synostosis should be informed of the increased risk of symptomatic recurrence.