Caroline Driessen1, Bianca F Rijken2, Priya N Doerga2, Marjolein H Dremmen3, Koen F Joosten4, Irene M Mathijssen2. 1. Department of Plastic, Reconstructive and Hand Surgery, Dutch Craniofacial Centre, Rotterdam, The Netherlands. Electronic address: c.driessen@erasmusmc.nl. 2. Department of Plastic, Reconstructive and Hand Surgery, Dutch Craniofacial Centre, Rotterdam, The Netherlands. 3. Department of Radiology, Dutch Craniofacial Centre, Rotterdam, The Netherlands. 4. Intensive Care Unit, Department of Paediatrics and Pediatric Surgery, Dutch Craniofacial Centre, Rotterdam, The Netherlands.
Abstract
INTRODUCTION: The investigators hypothesized that patients with Crouzon syndrome and premature fusion of the spheno-occipital synchondrosis (SOS) more often have, or have more severe midface hypoplasia and obstructive sleep apnea (OSA). METHODS: A prospective cohort study was performed among patients with Crouzon syndrome to analyze SOS closure, midface hypoplasia represented by the sella-nasion angle (SNA) and OSA. RESULTS: Forty patients were included in whom the OSA-prevalence was 65%. Kaplan Meier analyses suggest a trend towards earlier closure of synchondrosis in patients with OSA (p = 0.066). The mean SNA was 74.7°. There was a positive effect of age on the SNA (p = 0.020). There was no difference in SNA for patients with an open SOS as compared to patients with a closed SOS after correction for age. CONCLUSIONS: The longitudinal data are suggestive of a trend towards earlier fusion of the SOS in patients with Crouzon syndrome and OSA as compared to patients with Crouzon syndrome without OSA. Although the SNA increases with age, our results suggest that this increase in independent closure of the SOS.
INTRODUCTION: The investigators hypothesized that patients with Crouzon syndrome and premature fusion of the spheno-occipital synchondrosis (SOS) more often have, or have more severe midface hypoplasia and obstructive sleep apnea (OSA). METHODS: A prospective cohort study was performed among patients with Crouzon syndrome to analyze SOS closure, midface hypoplasia represented by the sella-nasion angle (SNA) and OSA. RESULTS: Forty patients were included in whom the OSA-prevalence was 65%. Kaplan Meier analyses suggest a trend towards earlier closure of synchondrosis in patients with OSA (p = 0.066). The mean SNA was 74.7°. There was a positive effect of age on the SNA (p = 0.020). There was no difference in SNA for patients with an open SOS as compared to patients with a closed SOS after correction for age. CONCLUSIONS: The longitudinal data are suggestive of a trend towards earlier fusion of the SOS in patients with Crouzon syndrome and OSA as compared to patients with Crouzon syndrome without OSA. Although the SNA increases with age, our results suggest that this increase in independent closure of the SOS.
Authors: Xiaona Lu; Antonio Jorge Forte; Rajendra Sawh-Martinez; Sarika Madari; Robin Wu; Raysa Cabrejo; Derek M Steinbacher; Michael Alperovich; Nivaldo Alonso; John A Persing Journal: Plast Reconstr Surg Glob Open Date: 2018-10-01
Authors: Francisco Vale; Inês Francisco; António Lucas; Ana Roseiro; Francisco Caramelo; Adriana Sobral Journal: Int J Environ Res Public Health Date: 2020-11-29 Impact factor: 3.390