Special distraction osteogenesis before bone grafting for alveolar cleft defects to correct maxillary deformities in patients with bilateral cleft lips and palates: Distraction osteogenesis performed separately for each bone segment

IntroductionPatients with bilateral cleft lips and palates have premaxillary protrusion and characteristic jaw deformities involving three-dimensional malposition of the premaxilla and bilateral maxillary bone segments. This study examined patients with bilateral cleft lips and palates who had deviation and hypoplasia of the premaxillas and bilateral maxillary segments. Before bone grafting, the patients were treated with special distraction performed separately for each bone segment using a halo-type external device. This report describes this novel treatment method which produced good results.Material and methodsThe subjects were five patients with severe jaw deformities due to bilateral cleft lip and palate. They were treated with maxillary Le Fort I osteotomy and subsequent distraction performed separately for each bone segment using a halo device. In three of five patients, premaxillary osteotomy was not performed, and osteotomy and distraction were performed only for the right and left lateral segments with severe hypoplasia.ResultsAll patients achieved distraction close to the desired amount. The widths of the alveolar clefts were narrowed, and satisfactory occlusion and maxillary arch form were achieved. After the surgery, three of five patients underwent bone grafting for bilateral alveolar cleft defects and the bone graft survival was satisfactory.ConclusionsThis method had many benefits, including narrowing of alveolar clefts, improvement of maxillary hypoplasia, and achievement of a good maxillary arch form. In addition, subsequent bone grafting for alveolar cleft defects was beneficial, dental prostheses were unnecessary, and frequency of surgery and surgical invasiveness were reduced. This method is a good surgical procedure that should be considered for patients with bilateral cleft lips and palates who have premaxillary protrusion and hypoplasia of the right and left lateral segments.     

面中部牵引成骨结合正颌手术治疗Binder综合征

目的：Binder综合征患者有严重的面部凹陷畸形及咬合功能障碍,治疗相对困难且易复发。本研究探讨面中部牵引结合正颌手术在Binder综合征治疗中的价值。方法：4例Binder综合征患者采用改良Le Fort II型截骨术,术后利用颅骨外置式牵引器进行旋转牵引,并随时调整矢状向及垂直向的量,矫正患者面形。半年后进行正颌手术,矫正咬合关系,并随访1~2 a。结果：4例患者均顺利完成整个治疗过程,无明显并发症发生。牵引过程中无明显疼痛及不适。头影测量显示,患者面中部骨骼显著前移,凹陷畸形得以矫治。经过正颌-正畸联合治疗,获得了良好的咬合关系。结论：上颌骨Le Fort II型截骨牵引可以矫治鼻上颌骨发育不足,通过正颌手术可以矫正咬合关系,两者结合是一种较为理想的治疗Binder综合征的方法。     

Deficient expression of the small proteoglycan decorin in a case of severe/lethal osteogenesis imperfecta

In osteogenesis imperfecta (OI) the effects of mutations in type I collagen genes generally reflect their nature and localization. Unrelated individuals sharing identical mutations present, in general, similar clinical phenotypes. However, in some such cases the clinical phenotype differs. This variable clinical expression could be the result of abnormalities in other connective tissue proteins. Since decorin is a component of connective tissue, binds to type I collagen fibrils and plays a role in matrix assembly, we studied decorin production in skin fibroblasts from OI patients. Cultured fibroblasts from one patient with extremely severe osteogenesis imperfecta (classified as type II/III) who has an α1(I)gly415ser mutation were found to secrete barely detectable amounts of decorin into culture medium. Western blotting using antibodies raised against decorin confirmed the reduction of the decorin core protein and Northern blot analysis showed decorin mRNA levels below the limit of detection. Cells from a patient, with a less severe phenotype, bearing a mutation in the same position of the triple helix (α1(I)gly415) expressed decorin normally. The different clinical phenotypes could be due to the differing genetic backgrounds of the patients so it is tempting to conclude that in our most severely affected patient the absence of decorin aggravates the clinical phenotype. © 1996 Wiley-Liss, Inc.     

骨形成蛋白在胫骨截骨快速延长成骨过程中的表达及意义

目的:探讨快速延长成骨过程中骨形成蛋白(BMP)的表达及意义. 方法: 制作兔胫骨截骨快速延长动物模型,分别于延长第5, 10, 15, 20, 30和40日在延长区取材,以抗骨形成蛋白单克隆抗体(BMP-McAb)行SP法免疫组化染色光镜观察. 结果: 在骨延长的整个过程中均有BMP的表达,不同时相BMP表达的部位不同. 除炎症细胞、类骨样组织及骨小梁外,几乎所有细胞及组织均有BMP存在. 间充质细胞、软骨细胞、成骨细胞、骨细胞、骨髓细胞BMP呈高表达. 结论: BMP在骨延长成骨过程中发挥着重要作用. BMP表达量不足可能是导致快速骨延长骨不连接的重要因素.     

巨噬细胞的骨免疫学效应

背景：巨噬细胞以其显著的骨免疫学效应得到学者们的广泛关注,其功能和应用已成为研究热点。目前研究主要涉及巨噬细胞的起源、极化、骨免疫学效应及其在骨修复中的应用。目的：综述巨噬细胞的骨免疫学效应及其在骨修复中应用的研究进展,证实巨噬细胞在骨组织工程中具有卓越的研究价值和应用前景。方法：利用PubMed、Web of Science和CNKI数据库检索2010-2022年发表的相关文献,检索词为“巨噬细胞极化、骨、成骨、骨免疫学、生物材料、组织工程”“macrophage polarization,bone,osteogenesis,osteoimmunology,biomaterials,tissue engineering”,并纳入少量年份久远的经典文献。通过阅读标题和摘要进行初筛,排除与文章主题不相关的文献,最终纳入120篇文献进行综述分析。结果与结论：(1)巨噬细胞包括单核细胞来源的炎性巨噬细胞和组织驻留巨噬细胞,其中不同组织的驻留巨噬细胞具有不同的发育起源组合,绝大多数组织驻留巨噬细胞起源于胚胎时期的卵黄囊;(2)巨噬细胞具有高度可塑性,在不同刺激下极化为M1/M2表型,分别释放促...     

犬面中份缝牵引成骨过程中骨形成蛋白-2的表达

目的:研究骨形成蛋白(bone morphogenetic protein,BMP)-2在犬面中份缝牵引成骨过程中的表达,探讨缝牵引成骨的机制.方法:18只12周龄杂种犬随机分为牵引成骨组12只和正常对照组6只,在牵引5、10、15 d,固定10、20 d,去固定1个月分别处死实验犬2只和对照犬1只,免疫组化法观察BMP-2在各骨缝的表达情况.结果:牵引期和固定期,BMP-2在实验犬各骨缝边缘大量增殖的未成熟成骨细胞、间充质细胞以及新生骨基质中呈阳性表达,对照犬也有少量表达.随着机械应力牵引, BMP-2在各骨缝表达量逐渐上升,在牵引15 d阳性表达最强,以后逐渐下降,到去固定1个月时表达水平接近正常.结论:机械牵张力刺激可以导致内源性BMP-2产生,BMP-2可能在缝牵引新骨生成过程中扮演重要的角色.     

Airway management in children with cleft palate and/or micrognathia

Cleft lip and/or palate is the commonest congenital craniofacial abnormality affecting approximately 1 in 700 newborns each year. It comprises of a heterogenous group of disorders affecting facial growth and cosmesis, that are associated with increased risk of airway obstruction, sleep disordered breathing (SDB), glue ear and chronic ear disease, feeding difficulties, and failure to thrive (FTT). Cleft palate (CP) can be accompanied by an abnormally undersized jaw, known as micrognathia; although micrognathia can also be found in isolation. The craniofacial abnormalities found in these children can lead to a reduction in airway size due to the tongue falling backwards. The risk of airway obstruction ranges from intermittent airway collapse during sleep (obstructive sleep apnoea, OSA) to potentially life-threatening airway compromise necessitating intubation or a tracheostomy. This paper sets out to describe the pathophysiology of airway compromise in these children, recognising clinical symptoms and appropriate referral strategy, as well as a broad range of management options.