Pierre Robin sequence (PRS) is a congenital malformation characterized by micrognathia, glossocoma, and mechanical obstruction of the upper respiratory tract. These deformities impair respiration, sleep, feeding, and swallowing, and can lead to malnutrition, stunted development, and death. Bilateral mandibular distraction osteogenesis, whereby the mandible and tongue root are extended outward, is the standard treatment to relieve upper airway obstruction in severe PRS. Accurate placement of the distraction device is essential but challenging, especially in infants, and requires the pre-operative fabrication of surgical guides based on CT images. Three-dimensional (3D) printing allows for the accurate recreation of objects from digitized models. We compared surgical efficacy and safety of bilateral mandibular distraction osteogenesis using 3D printed or traditionally fabricated surgery guides for treatment of infants with severe PRS. During the period from 2014 to 2016, 22 patients with severe PRS were treated using either traditional or 3D printed surgery guides. We compared outcome measures of operations, including intraoperative bleeding, operation time, and postoperative complications. The 3D printed surgery guide group demonstrated significantly shorter operation time (P <.05) as well as moderately shorter hospital stay and artificial ventilation time (∼1 day less). Furthermore, despite markedly younger average age of the 3D printed group (1.3 vs 3.5 months), there was no increase in postoperative complications using the 3D printed guides. Three-dimensional printed surgery guides were used successfully for bilateral mandibular traction osteogenesis, and according to several outcome, parameters demonstrated superior efficacy and safety compared to traditional guides. Further research is warranted to extend the applications of 3D printed surgical guides for craniofacial surgery.

Background: Airway management is challenging in children with Robin sequence (RS) requiring mandibular distraction osteogenesis (MDO). We derived and validated a prediction rule to identify difficult intubation before MDO for children with RS based on craniofacial computed tomography (CT) images. Method: This was a retrospective study of 69 children with RS requiring MDO from November 2016 to June 2018. Multiple CT imaging parameters and baseline characteristic (sex, age, gestational age, body mass index [BMI]) were compared between children with normal and difficult intubation according to Cormack−Lehane classification. A clinical prediction rule was established to identify difficult intubation using group differences in CT parameters (eleven distances, six angles, one section cross-sectional area, and three segment volumes) and clinicodemographic characteristics. Predictive accuracy was evaluated by receiver operating characteristic (ROC) curve analysis. Results: The overall incidence of difficult intubation was 56.52%, and there was no significant difference in sex ratio, age, weight, height, BMI, or gestational age between groups. The distance between the root of the tongue and posterior pharyngeal wall was significantly shorter, the bilateral mandibular angle shallower, and the cross-sectional area at the epiglottis tip smaller in the difficult intubation group. A clinical prediction rule based on airway cross-sectional area at the tip of the epiglottis was established. Area > 36.97 mm² predicted difficult intubation while area < 36.97 mm² predicted normal intubation with 100% sensitivity, 62.5% specificity, 78.6% positive predictive value, and 100% negative predictive value (area under the ROC curve = 0.8125). Conclusion: Computed tomography measures can objectively evaluate upper airway morphology in patients with RS for prediction of difficult intubation. If validated in a larger series, the measures identified could be incorporated into airway assessment tools to guide treatment decisions. This was a retrospective study and was granted permission to access and use these medical records by the ethics committee of Guangzhou Women and Children’s Medical Center. Trials registration: Registration No. ChiCTR1800018252, NaZhang, Sept 7 2018.

Background: Impaired weight gain is prevalent in Robin Sequence (RS) newborns. Although mandibular distraction osteogenesis (MDO) has been proven to improve oral feeding, its impact on postoperative weight gain remains unclear. The purpose of this study is to explore whether MDO can help RS babies reach a normal weight, as well as the effect of MDO timing on weight velocity. Methods: One hundred infants with severe RS and one hundred with normal controls met the inclusion criteria for the study. Included patients underwent MDO. Weights at different timing points were recorded and analyzed and compared to normal controls. Results: After the distractor removal weights of patients undergoing MDO at <1 month and 1–2 months were close to the normal control (6.81 ± 0.93 kg versus 7.18 ± 0.61 kg, p = 0.012, and 6.82 ± 0.98 kg versus 7.37 ± 0.75 kg, p = 0.033, respectively), the weights of patients undergoing MDO at 2–3 months and 3–4 months still lagged behind (7.56 ± 1.29 kg versus 8.20 ± 0.61 kg, p = 0.000206 and 7.36 ± 1.05 kg versus 8.25 ± 0.77 kg, p = 0.004, respectively). The weights of all RS infants undergoing MDO showed no significant difference compared to the controls when they aged to 1 year (9.34 ± 0.99 kg versus 9.55 ± 0.45 kg, p = 0.254 for MDO at <1 month; 9.12 ± 0.91 kg versus 9.33 ± 0.46 kg, p = 0.100 for MDO at 1 to 2 months; 9.38 ± 0.29 kg versus 9.83 ± 0.53 kg, p = 0.098 for MDO at 2 to 3 months; and 9.38 ± 0.29 kg versus 9.83 ± 0.53 kg, p = 0.098 for MDO at 3 to 4 months). Conclusion: The MDO procedure helped patients with severe RS to reach a normal weight; and MDO intervention was recommended at an early stage for early weight gain.