S12-05 SESSION 12: FACIOCRANIOSYNOSTOSIS – PART II FRONTO-FACIAL MONOBLOC ADVANCEMENT WITH INTERNAL DISTRACTION IN CROUZON SYNDROME: LONG-TERM EFFECTS OF MOLAR DEVELOPMENT AND GROWTH

Abstract

Introduction: Crouzon syndrome is a syndromic faciocraniosynostosis that can be associated with severe fronto-facial retrusion leading to major functional impairments such as ocular proptosis and obstructive sleep apnea. The procedure of choice for the primary correction of this retrusion is fronto-facial monobloc advancement (FFMBA) with internal or external distraction. FFMBA involves pterygomaxillary dysjunction (PMD), using either a superior or an intra-oral approach. This step is at risk of damaging the germs of the decidual and permanent molars. Methods: Here we considered a series of 15 patients with Crouzon syndrome who benefited from FFMBA performed by the same surgeon, using a superior approach through the infra-temporal fossa for PMD. Based on pre-operative, early post-operative and late post-operative CT-scans, we recorded missing teeth, morphological dental anomalies and the Nolla stage for the first and second permanent maxillary molars. Results: We showed that early FFMBA has significant dental consequences, and that the extent of these dental effects is correlated with an early age at surgery. The use of transfacial pins was also correlated with the extent of dental damage. Conclusion: The indications of early FFMBA in Crouzon syndrome with severe functional repercussions are not questionable, but dental outcome should be taken into account when discussing early indications without severe functional impairment. Furthermore, our results still need be compared to dental outcomes of FFMBA performed with an intra-oral PMD.