Bhaskar Atul R, Khurana Deepak
Consultant Paediatric Orthopaedic Surgeon, Children Orthopaedic Surgical Services, Mumbai, Maharashtra, India.
Clinical Fellow - Paediatric Orthopaedics, Children Orthopaedic Surgical Services, Mumbai, Maharashtra, India.
Indian J Orthop. 2019 Jul-Aug;53(4):554-559. doi: 10.4103/ortho.IJOrtho_202_18.
Delay in presentation and surgical intervention is quite usual in osteogenesis imperfecta (OI) because of various local and cultural beliefs. The purpose of this study is to review the results of 21 children who had intramedullary rodding and its effect on ambulation and refracture.
We reviewed 21 children with a clinical diagnosis of OI. The mean age of children at presentation was 8.74 years (3-21 years). All children had recurrent fractures of long bones. Twenty eight femurs and 21 tibiae were stabilized with intramedullary rodding. Ambulatory status was assessed by the Hoffers and Bullock's (H and B) grading, and muscle power was recorded using the Medical Research Council, U. K., grade. Ten children had received intravenous bisphosphonates preoperatively. Postoperatively, the children were assessed for ambulatory status, pain, and ability for independent self-care.
The mean followup period was 34 months (24-48 months). Rush rods were used in 20 femurs, the Fassier-Duval (FD) rods in 6 femurs, and in two cases, with narrow intramedullary canals, Kirshner (K) wires were used. For the tibiae, 15 children received rush rods and in 6 cases, an FD rod was used. The mean time to fracture union was 8 weeks (6-12 weeks). Before surgery, 13 children were in H and B Grade 4 (wheel-chair independent or carried by parents usually in a developing country), four were able to ambulate with a walking aid (H and B Grade 3b), and four children were able to walk about in the house without aids (H & B Grade 2). After the rodding procedure, the ambulatoty status improved in 11 (50%) children. Seven children (33%) became household physiologic walkers (H & B Grade 3b), three achieved independent ambulation with orthosis (H & B Grade 1b), and one child with mild OI could walk unaided (H & B Grade 1a). No child had deterioration in ambulatory status. Only two children had refractures at the distal end of the rod due to continual growth of bones.
Intramedullary rodding treatment for recurrent fractures in children with OI improves their mobility potential. It also and prevents repeated cast application, disuse wasting, and osteopenia which can lead to deterioration in the quality of the long bones.
由于各种当地和文化观念，成骨不全症（OI）患者就诊及接受手术干预往往会延迟。本研究的目的是回顾21例接受髓内棒固定术的儿童的治疗结果及其对行走能力和再骨折的影响。
我们回顾了21例临床诊断为OI的儿童。患儿就诊时的平均年龄为8.74岁（3 - 21岁）。所有儿童均有长骨反复骨折。28根股骨和21根胫骨通过髓内棒固定实现稳定。行走状态通过霍弗斯和布洛克（H和B）分级进行评估，肌肉力量采用英国医学研究委员会分级记录。10名儿童术前接受了静脉双膦酸盐治疗。术后，对患儿的行走状态、疼痛情况及独立自理能力进行评估。
平均随访期为34个月（24 - 48个月）。20根股骨使用了拉什棒，6根股骨使用了法西耶 - 杜瓦尔（FD）棒，2例髓腔狭窄的患儿使用了克氏（K）针。对于胫骨，15名儿童使用了拉什棒，另有6例使用了FD棒。骨折愈合的平均时间为8周（6 - 12周）。手术前，13名儿童处于H和B分级4级（在发展中国家通常可独立使用轮椅或由父母携带），4名儿童借助助行器能够行走（H和B分级3b级），4名儿童能够在屋内无需辅助自行走动（H和B分级2级）。髓内棒固定术后，11名（50%）儿童的行走状态得到改善。7名儿童（33%）成为家庭生理性步行者（H和B分级3b级），3名儿童通过矫形器实现独立行走（H和B分级1b级），1名轻度OI患儿能够无需辅助行走（H和B分级1a级）。没有儿童的行走状态恶化。仅2名儿童因骨骼持续生长在棒的远端发生再骨折。
髓内棒固定术治疗OI患儿的反复骨折可改善其行走潜能。它还能避免反复应用石膏、废用性萎缩以及骨质减少，而这些情况可能导致长骨质量下降。
