P-56250681-142 - Improvement of periorbital appearance in crouzon syndrome after subcranial Le Fort III with facial bipartition and internal distractor device. A retrospective study.

Abstract

<h3>Introduction</h3> Children with crouzon syndrome have a characteristic inversion of the orientation of the palpebral fissures, an increase of the inter-orbital distance, telecanthus, and exorbitism. Here, Le Fort III osteotomy with facial bipartition combined with internal distraction device was evaluated as a tool to improve the position middle and upper third. <h3>Material and Methods</h3> All patients with crouzon syndrome who underwent monoblock osteotomy with subcranial bipartition and distraction osteogenesis using an internal device, between 2013 and 2018, with available preoperative and postoperative frontal photographs, were included into the study. Palpebral fissure inclination was measured. Ratios of the intercanthal distance (ICD) to the outer-canthal distance (OCD) and the interpupillary distance to the OCD were computed. Preoperative and postoperative values were compared. <h3>Results</h3> The authors included 12 patients with crouzon syndrome. The mean age at surgery was 10±3.4 years and the average follow-up was 4.6±1.7 years. We found normalization of the negative inclination of the palpebral fissures (right eye: 10.7±2.4 degrees preoperatively versus 7.0±3.1 degrees postoperatively, P<0.001; left eye: 12.4±3.9 degrees preoperatively versus 8.7±4.1 degrees postoperatively, P=0.01) and a significant reduction of the inter-pupillary distance: OCD ratio (0.717±0.027 preoperatively versus 0.699±0.030 postoperatively, P=0.03). These modifications were stable on the long term. <h3>Conclusions</h3> Monoblock facial advancement with subcranial bipartition and distraction improves the position and orientation of the orbital region in children with crouzon syndrome.