Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, fast-growing, benign tumor originating from the neural crest. The tumor most often occurs during the first year of life. The predilection site of MNTI is the anterior maxilla, whereas lesions of the mandible are uncommon and account for only 6% of all cases. At present, the most common treatment for MNTI is surgical resection, however, tumor recurrence arises in 10–60% of cases. The optimal extent of surgical resection is a matter of debate; rapid growth and the possibilities of malignant transformation and metastasis indicate aggressive surgical resection. However, extensive resection may interfere with post-operative growth and development. The procedure should therefore preserve as much of the surrounding tissue as possible. The present case study examines a rare case of right-sided mandibular MNTI in a two-month-old female. The association between the tumor and the surrounding sclerotin, affected dental germ and the condition of the inferior alveolar nerve, were observed during the operative and post-operative periods. In addition, previous cases of MNTI were reviewed to evaluate the optimal scope of surgical resection.

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.

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BackgroundAirway 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.MethodThis 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.ResultsThe 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).ConclusionComputed 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 registrationRegistration No. ChiCTR1800018252, NaZhang, Sept 7 2018.

Mandibular distraction osteogenesis (MDO) is an effective treatment for tongue-based airway obstruction in infants with severe Pierre Robin sequence (PRS). Most infants receiving MDO require postoperative mechanical ventilation (MV) to assist breathing. Optimal MV time for each individual patient and factors influencing the time must be identified to guide clinical decision-making. A retrospective analysis was performed on 75 infants with PRS receiving MDO from November 2016 to August 2018. Twenty-six were females and 47 were males. Data extracted from the hospital information system included sex, age, weight, history of preterm labor, preoperative pulmonary infection, laryngomalacia/tracheomalacia, laryngoscope exposure classification, anesthesia duration, operation duration, postoperative treatment site, situation of distraction, postoperative complications and MV duration. Statistical analyses were conducted to investigate the potential associations of these factors with MV time. Seventy-three PRS syndrome patients received anesthesia for MDO device procedures were considered eligible for study. Patient sex, history of preterm labor, preoperative pulmonary infection, laryngomalacia/tracheomalacia, laryngoscopy exposure difficulty, postoperative treatment site (neonatal or pediatric intensive care unit), ventilator-associated pneumonia, age, weight, anesthesia duration, and operation duration had no significant influence on postsurgical MV time (P > .05). Amount of distraction at the time of extubation had statistically significant influence on postoperative MV time (P < .05). In addition, scatter plots revealed linear relationships between postoperative MV time and amount of distraction at extubation. According to this analysis, amount of distraction was associated with MV time following MDO for severe PRS and roughly 6 days post-surgery is a generally safe extubation time.

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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.