Delniotis Ioannis, Leidinger Benedikt
Department of Paediatric- and Neuro-Orthopaedics/Foot and Ankle Surgery, Orthopaedic Clinic Volmarstein, Wetter (Ruhr), Germany.
Am J Case Rep. 2019 Oct 21;20:1540-1550. doi: 10.12659/AJCR.918560.
BACKGROUND Osteogenesis imperfecta is a rare inherited connective tissue disorder that is mainly characterized by long bone deformities and increased susceptibility to bone fractures. The aim of this study was to present a surgical technique in a child suffering from osteogenesis imperfecta and progressive, severe bowing deformity of both tibias, as an alternative method to advanced, innovative surgical systems. CASE REPORT A 9-year-old child (male) was referred to our orthopedic clinic for inability to walk independently due to extreme anterior bowing of both tibias. After the diagnosis of osteogenesis imperfecta type III was established, bilateral surgical treatment with multiple osteotomies and intramedullary, flexible Titanium Elastic Nail System (TENS) nails was decided. Six months post-operatively callus formation was obvious in x-rays and at the latest follow-up (1 year post-operatively) the patient regained the ability to walk independently. CONCLUSIONS In types of osteogenesis imperfecta which are characterized by extreme deformities (type III) surgical intervention seems to be the only solution for these patients to walk again. We present a relatively simple technique of correcting such deformities, indicating that no matter which technique will be used (simple or more complicated) the final goal should be to restore the walking ability with as little complications as possible.
成骨不全症是一种罕见的遗传性结缔组织疾病,主要特征为长骨畸形和骨折易感性增加。本研究的目的是介绍一种针对一名患有成骨不全症且双侧胫骨进行性严重弓形畸形儿童的手术技术,作为先进创新手术系统的替代方法。
一名9岁男性儿童因双侧胫骨极度前弓而无法独立行走,被转诊至我们的骨科诊所。在确诊为III型成骨不全症后,决定采用双侧多处截骨术及髓内弹性钛钉系统(TENS)进行手术治疗。术后6个月,X线显示骨痂形成明显,在最新一次随访(术后1年)时,患者恢复了独立行走能力。
对于以极度畸形为特征的成骨不全症类型(III型),手术干预似乎是这些患者重新行走的唯一解决方案。我们介绍了一种相对简单的矫正此类畸形的技术,表明无论采用何种技术(简单或复杂),最终目标都应是在尽可能减少并发症的情况下恢复行走能力。