S3-17 SESSION 3: FACIOCRANIOSYNOSTOSIS – PART I FRONTO-FACIAL MONOBLOC ADVANCEMENT WITH DISTRACTION REDUCES SEVERE OBSTRUCTIVE SLEEP APNEA IN FACIOCRANIOSYNOSTOSES: PROSPECTIVE ASSESSMENT OF 109 CASES

Abstract

Introduction: Obstructive sleep apnea syndrome (OSAS) is prevalent in children with faciocraniosynostoses and is associated with poor cognitive outcome and risk for increased intracranial pressure. Fronto-facial monobloc advancement (FFMBA) with distraction advances the midface and is performed for OSAS management. Here we aimed to provide a quantitative and prospective assessment of the effects of FFMBA on OSAS in faciocraniosynostoses. Methods: We prospectively included all patients managed for faciocraniosynostoses over a period of 18 years and followed-up their apnea-hyponea index (AHI) based on polysomnographic (PSG) studies, before and after FFMBA with internal distraction. AHI was assessed using several linear and logistic multivariate models, in order to quantify (1) pre-operative AHI values, (2) absolute decrease in AHI values after FFBMA, (3) AHI normalization (AHI<5) after FFMBA, (4) need for a secondary facial advancement and (5) genotype-phenotype correlations. In the second half of the experience, systematical removal of adenoids and tonsils were performed prior to monobloc surgery Results: A total of 109 patients were included and 407 PSG at different time points were collected. We showed a global and significant decrease in AHI after FFMBA: mean pre-operative AHI was 19.11+/-17.25 (19.31+/-18.31 in Crouzon, 17.00+/-11.87 in Apert and 21.82+/-19.38 in Pfeiffer syndrome). The mean post-operative AHI value for all patients was 12.47+/-13.58, which corresponded to a significant decrease from the mean pre-operative value of 19.11+/-17.25 (p<0.05); the decrease from pre-operative values was significant for all three syndromes (p<0.05). Pre-operative lateral and/or posterior decompression was associated with more AHI decrease in absolute value. FFMBA at older ages, Pfeiffer syndrome and pre-operative tonsillectomy and/or adenoidectomy were associated with more AHI normalization. In this subgroup with premonobloc ENT surgery, the intermediate OSAS (AHI between 10–20) the mean pre-op was 16/h and mean post-op was 11/h. In the severe OSAS patients (pre op AHI >20), the mean pre op was 43/h and mean post op was 15/h. Conclusion: FFMBA is efficient in reducing OSAS in faciocraniosynostoses. Pre-operative adenoido-tonsillectomy and lateral and/or posterior skull decompression are recommended before FFMBA in order to improve the respiratory results of the fronto-facial advancement. Delaying surgery when allowed by the neurosurgical and respiratory medicine teams is also a parameter favoring better respiratory outcomes.