Improvement of periorbital appearance in Crouzon syndrome after subcranial Le Fort III osteotomy with facial bipartition and an internal distractor device: a retrospective study

Abstract

Purpose Children with Crouzon syndrome have a characteristic inversion of the orientation of the palpebral fissures, an increase of the interorbital distance, telecanthus, and exorbitism. Here, Le Fort III osteotomy with facial bipartition combined with an internal distraction device was evaluated as a tool to improve the middle and upper third positions. Methods All patients with Crouzon syndrome who underwent monoblock osteotomy with facial bipartition and distraction osteogenesis using an internal device, between 2013 and 2019, 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. Results The study included 12 patients with Crouzon syndrome. The mean age at surgery was 10 ± 3.4 years, and the average follow-up period was 4.6 ± 1.7 years. We found normalization of the negative inclination of the palpebral fissures and a significant reduction of the interpupillary distance: OCD ratio (0.717 ± 0.027 preoperatively vs 0.699 ± 0.030 postoperatively; P = .03). These modifications were stable over the long term. There was no significant change of the intercanthal distance. Conclusions Monoblock facial advancement with subcranial bipartition and distraction improves the position and orientation of the orbital region in children with Crouzon syndrome and is a technique that offers predictable results.