Improvement of Periorbital Appearance in Apert Syndrome After Subcranial Le Fort III With Bipartition and Distraction

Abstract

Children with Apert 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 subcranial bipartition and distraction osteogenesis was evaluated as a tool to improve the position of the palpebral fissures in Apert syndrome. All patients with Apert syndrome who underwent Le Fort 3 osteotomy with subcranial bipartition and distraction osteogenesis using an external device, with canthopexy, between 2009 and 2014, 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 using the Wilcoxon signed-ranks test. The authors included 15 patients with Apert syndrome. The mean age at surgery was 10 ± 3.4 years and the average follow-up was 7.3 ± 2.9 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. There was no significant change of the inter-canthal distance:OCD ratio. Le Fort III facial advancement with subcranial bipartition and distraction improves the position and orientation of the orbital region in children with Apert syndrome.