Wirth T
Orthopädische Klinik, Olgahospital, Klinikum Stuttgart, Bismarckstr. 8, 70176 Stuttgart, Deutschland.
Orthopade. 2012 Sep;41(9):773-82; quiz 83-4. doi: 10.1007/s00132-012-1959-y.
The classic Sillence classification of the four types of osteogenesis imperfecta (OI) has been extended by six additional forms in recent years. OI is a heterogeneous disease, which can exhibit a mild, moderate and severe clinical picture. The clinical variability is expressed by a different frequency of fracture incidences and bone deformity risks so that both factors lead to very different degrees of mobility and autonomy of patients. The treatment principles comprise long standing medication of bisphosphonates, rehabilitation measures and orthopedic treatment. The orthopedic treatment uses modern techniques of conservative and operative fracture management for fracture stabilization and modern telescopic rods for deformity correction. These combined treatment modalities have given an improved quality of life to OI patients of all severity grades.
近年来，经典的成骨不全症（OI）的四种类型的西伦分类法又增加了六种类型。成骨不全症是一种异质性疾病，可表现出轻度、中度和重度的临床表现。临床变异性表现为骨折发生率和骨畸形风险的不同频率，因此这两个因素导致患者的活动能力和自主程度差异很大。治疗原则包括长期使用双膦酸盐药物、康复措施和矫形治疗。矫形治疗采用现代保守和手术骨折管理技术来稳定骨折，并使用现代伸缩棒来矫正畸形。这些综合治疗方式提高了所有严重程度等级的成骨不全症患者的生活质量。
