可延长髓内钉预防成骨不全症儿童股骨再骨折疗效观察

Telescopic intramedullary rod for prevention of recurrent femur fracture in children with osteogenesis imperfecta

目的探讨可延长髓内钉预防成骨不全症儿童股骨再骨折的疗效。方法回顾性分析2009年6月至2013年6月治疗的35例成骨不全症儿童股骨畸形患儿，男19例，女16例；年龄3岁6个月至13岁1个月，平均9岁3个月。患儿术前股骨曾多次骨折并致骨干弯曲畸形，畸形成角度数为10。-90。，平均55。；其中单侧股骨手术10例，双侧股骨手术25例。术前股骨骨折均未行手术治疗，骨折频率2-4次／年，平均2．4次／年。依据Sillence分型：Ⅰ型12例，Ⅲ型9例，Ⅳ型14例。35例患儿均对弯曲的股骨行截骨矫形、可延长髓内钉内固定。结果35例患儿均获得随访，随访时间36-72个月，平均62个月。股骨截骨愈合时间为7-12周，平均8．5周。复查X线片示截骨愈合后患儿开始进行功能锻炼，至末次随访时80％患儿恢复站立和行走功能。患儿骨折频率明显降低，由术前股骨骨折频率2-4次／年（2．4±1．3次／年），降低到股骨骨折频率0-1次，年，平均0．3次／年（0．3±0．1次／年）。35例患儿家长均对手术结果及畸形矫正效果表示满意。末次随访时患儿Barthel指数评分由术前平均71．82分（范围，51-92分）提高到术后平均92．32分（范围，82±100分）；WeeFIM评分由术前平均53．32分（范围，42-72分）提高到术后平均78分（范围，70±86分）。术后患儿生活自理能力明显改善。术后35例患儿中，22例再次或多次发生不同程度的股骨术后再骨折，其中7例行股骨骨折切开复位可延长髓内钉治疗，15例因骨折无明显移位或者成角畸形而仅行保守治疗，骨折均获愈合。结论可延长髓内钉可以矫正成骨不全患儿股骨畸形、增强肢体强度、有效预防成骨不全症儿童股骨再骨折。

Objective To evaluate the efficacy of prevention of recurrent femur fracture in children with osteogenesis imperfeeta. Methods Data of 35 patients （19 boys, 16 girls） who were treated for femur deformity due to osteogenesis imperfecta at our hospital from Jun 2009 to Jun 2013 were retrospectively analyzed. The average age of the patients was 9 years 3 months, ranging from 3 years 6 months to 13 years 1 month. All the patients had suffered from recurrent femur fractures before which led to femoral deformity. The mean angulation angle was 55° （range, 10°-90°）. 10 patients were operated at one side; the other 25 patients were treated bilaterally. None of them received operation before for fractures. The average incidence of femur fracture before operation was 2.4/year （range, 2-4）. According to the modified Sillence classification system, 9 were type III OI, 14 type IV, and 12 type I. The curved deformity of the femurs were corrected by osteotomy and stabilized by telescopic intramedullary rods in 35 patients. Results All the 35 patients were followed up for a mean period of 62 months （range, 36-72）. The average healing time of the osteotomy site of the femur was 8.5 weeks （range, 7-12）. The patient was encouraged to begin weightbearing and walking when the X- ray film showed healing of the osteotomy site. By the latest follow up, 80% of the patients could stand and walk independently. The incidence of femur fracture decreased significantly to the level of 0.3±0.1/year, compared with the level of 2.4± 1.3/year before operation. All the parents of the children were satisfied with the result of deformity correction. At the latest follow up, the Barthel index score increased from 71.82 （range, 51-92） before operation to 92.32 （range, 82-100） postoperatively, while the WeeFIM score increased from 53.32 （range, 42-72） before operation to 78 （range, 70-86） postoperatively. The difference of Barthel index score and WeeFIM score between that before and post operation was significant. The children＇ s selfcare and motion ability improved obviously after operation. During follow up, 22 patients suffered from recurrent fracture of the femur by various degree, 7 of them were treated by open reduction and telescopic rodding surgery, while the other 15 patients were treated conservatively because the fracture displaced or angulated minimally and the fracture healed uneventfully. Conclusion The telescopic intramedullary rod can maintain the correction of the femur deformity and improve the quality of the bone, thus prevent the recurrent fracture of the femur in children with osteogenesis imperfecta effectively.