Objective: This thesis addresses a neglected aspect of bioinformatics research of hemifacial microsomia (HFM). Existing research stops short of prediction based on big data. This study combines multiple databases to explore underlying pathogenesis using bioinformatic approach. Methods: The research consisted of multiple bioinformatic methods, included pathogenic genes analyses, protein-protein interaction network construction, functional enrichment, and mining target genes related miRNA, for studying pathogenic genes of HFM.
Objective: This study focused on evaluating different parts of the airway in patients with hemifacial microsomia classified by the Pruzansky-Kaban classification system. Methods: Volumetric and morphologic airway parameters of 66 children with hemifacial microsomia were measured on 3D models. Using the Pruzansky-Kaban system, the patient cohort was composed of seven I-type, 14 IIa type, 27 IIb type, and 18 III type patients. Results: The total airway volume was not statistically different among groups. In the 3D oropharynx models, volume and surface area of the oropharynx decreased from the type I group to the type IIb group. However, no statistically significant difference was found for length (P ¼ 0.965) or minimum cross-sectional area (mini-CSA, P ¼ 0.550) of the oropharynx in the type III group compared to the other groups. In the 3D laryngopharynx models, the mean-CSA (P ¼ 0.413) and mini-CSA (P ¼ 0.378) were not statistically different among groups. In contrast, volume (P ¼ 0.014), length (P ¼ 0.005), and surface area (P ¼ 0.032) of the laryngopharynx were reduced from type I to type III. Kruskal-Wallis analysis indicated statistically significant differences of volume (P ¼ 0.004), length (P ¼ 0.017), and surface area (P ¼ 0.010) of the laryngopharynx among groups I, IIa, and IIb. The mean-CSA (P ¼ 0.247) and mini-CSA (P ¼ 0.206) of the laryngopharynx were not correlated with the different clinical types. Conclusion:The mean-CSA varied significantly from type I to IIb at the level of oropharynx. In addition, the volume of the laryngopharynx decreased from type I to type III. However, type III was unique in this study with nonseverely airway measurement results.
Background:Dermatofibrosarcoma protuberans(DFSP)is one of rare and low-grade fibrohistiocytic tumor with malignant potential biological behavior. With the pathological character of the finger-like lesion invading soft tissues, DFSP is considered to be high rate of local recurrence. Method: In this study, we reported a retrospective study of 17 DFSP patients with surgery medical history and long-time follow-up period. All the cases were reviewed by research group with the clinical features, operation outcomes and pathological parameters. Results: 17 Asian patients were included in this study (the mean age is 41.82 years old with 9 females and 8 males). Location of DFSP are the belly (7/17 41.2%), the chest(2/17 11.8%),the shoulder(5/17 29.4%), the face(1/17 5.9%), the perineum(1/17 5.9%) and the right leg(1/17 5.9%). 6 cases were closed by plastic surgical suture(35.29%),3 cases by full thickness skin graft(17.65%)and 8 cases by pedicled flap(47.06%). CD34 and Vimentin positively is 100%, 90% respectively. Desmin and S-100 negatively were 100%. The median value of Ki-67 is 12%. Conclusion: The challenge of DFSP is clinical local control after operation. The first choice of DFSP treatment is surgery with the aim of clear margin and properly reconstruction. For the clinical practice, Enhancing the clinical recognition of DFSP in the initial medical visit is important for proper management and operation opportunity.
The treatment for severe hemifacial microsomia (HFM), especially type III case, is extremely difficult. Mandibular distraction osteogenesis (MDO) was rarely used as the primary choice in the treatment of severe type cases. The authors sought to observe the short-term therapeutic outcomes of patients with severe unilateral HFM who underwent MDO first.A retrospective study of children underwent MDO or later received costochondral graft (CCG) for severe unilateral HFM from 2009 to 2019 was conducted. Cephalometric measurements and clinical variables were analyzed to evaluate the effectiveness of MDO first strategy for severe cases and compare disparity between Pruzansky–Kaban classification type IIb and type III groups.Thirty-six patients (23 males and 13 females) underwent MDO first for severe HFM were included for analysis in the present study. The average age at MDO was 8.33 ± 2.03 years. At the last follow-up, MDO acquired significant improvement in mandibular height, maxillary cant, chin deviation, lip commissural line tilt, and clinical chin deviation (P < 0.05). Distraction results were stable during the short-term follow-up in terms of the mandibular height ratio and maxillary cant (P > 0.05).MDO is a proper primary method for suitable type IIb and type III HFM cases. MDO can immediately and significantly improve the facial skeleton deficiency, extend the associated soft tissue at the same time, and lay foundation for secondary surgery. MDO can achieve the downgrade of HFM deficiency severity. MDO followed by costochondral graft can get satisfactory esthetic and structural consequence for type III patients.
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