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ISCFS 2019 Abstract Supplement

S3-18 SESSION 3: FACIOCRANIOSYNOSTOSIS – PART I Secondary facial advancement in syndromic craniosynostoses: a prospective assessment of 109 cases

Haber, S. E.1 2,*; Khonsari, R. H.1,,2; Leikola, J.3; Fauroux, B.4; Morisseau-Durand, M.1,,2; Nowinski, D.5; Arnaud, E.1,,2

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Plastic and Reconstructive Surgery - Global Open: August 2019 - Volume 7 - Issue 8S-2 - p 40-41
doi: 10.1097/01.GOX.0000582960.50147.f4
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Introduction: Secondary Le Fort III (LF3) facial advancement is indicated for the management of persistent obstructive sleep apnea (OSA) and/or midfacial retrusion after a primary fronto-facial monobloc advancement (FFMBA) in faciocraniosynostosis. Here we aimed to provide a quantitative and prospective assessment of the effectiveness of secondary facial advancement on sleep apnea in faciocraniosynostosis.

Methods: We prospectively included all patients managed for faciocraniosynostoses over a period of 14 years and followed-up their apnea-hyponea index (AHI) based on polysomnographic (PSG) studies, before and after fronto-facial monobloc advancement (FFMBA) with internal distraction. Patients who required a secondary LF3 for persisting or relapsing OSA were identified. Polysomnographic, genetic and clinical data were analyzed. Primary outcome was AHI normalization defined as reaching AHI<5 events per hour. Secondary outcome was achieving a more than 50% reduction in AHI. A multivariate logistic regression model was fit to identify factors associated with secondary advancement.

ResultsA total of 108 patients were included and 407 PSG at different time points were collected. Secondary facial advancement was required in 12 patients (11%) to treat respiratory symptoms. None required tracheotomy after primary FFMBA. No major complication occurred. Mean age at primary FFMBA was 29.5 ± 20.2 months. Mean age at secondary LF3 was 75.7 ± 50.7 months. Average time between primary and secondary facial advancement was 46.2 ± 36.0 months. Mean follow-up time was 62.8 ± 45.1 months after secondary LF3. 6 patients (50%) reached the primary and secondary outcomes 19.8 months after secondary LF3. 4 patients required an additional tertiary facial advancement (2 with Crouzon and 2 with Pfeiffer). Multivariate analysis could not detect predictive factors of response after secondary advancement.

Conclusion: Secondary LF3 advancement effectively treats persistent or relapsing OSA after FFMBA in faciocraniosynostosis. Higher baseline AHI and earlier primary FFMBA are predictors of secondary advancement.

Copyright © 2019 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of The American Society of Plastic Surgeons. All rights reserved.