成骨不全症儿童下肢骨折保守治疗的远期并发症

Long term complications of conservative treatment of lower leg fractures in children with osteogenesis imperfecta

目的分析成骨不全症儿童下肢骨折经保守治疗后的远期结果及其并发症。方法回顾性对比分析患者的系列X线片,2001年9月至2014年7月,我科共收治1100例成骨不全症患者,其中Sillence分型Ⅰ型376例,Ⅲ型320例,Ⅳ型404例。其中就诊时已经出现骨骺闭合者437例。其中既往反复下肢骨折均采用保守治疗（牵引、夹板或者石膏固定）且X线片资料完整者45例,男30例,女15例,就诊时平均年龄17（13~34）岁。Sillence分型：Ⅰ型2例,Ⅲ型20例,Ⅳ型23例。分析患者的骨折次数、每次骨折的肢体固定时间,记录最终随访时的下肢功能、下肢骨骼的形态变化以及最终随访时的X线片表现。结果 45例每次骨折均采用保守治疗,骨折后肢体固定平均时间为50（30~90）天,多数骨折可愈合,但30例假关节形成,27例骨骺早闭。X线片显示反复骨折需要长时间牵引或者石膏固定,患肢制动、不负重超过6个月以上者,X线片显示骨质疏松异常加重。X线片总体表现骨骼纤细、髓腔封闭、关节膨大、皮质变薄、骨骼多平面弯曲畸形。最终随访时5%的患者完全卧床,30%需要轮椅,35%可以拐杖辅助下短距离行走,30%可自行行走,部分伴有步态异常。30%的病例因为矫形和内固定困难已无法进行任何骨科治疗。结论成骨不全症儿童下肢骨折虽然经保守治疗可以愈合,但反复骨折和保守治疗后其远期功能结果和X线片表现差,应及时行截骨矫形髓内固定以尽早恢复肢体的负重功能,从而避免骨骼发育受阻。

Objective To analyze long term results and complications of conservative treatment for lower leg fractures in children with osteogenesis imperfecta. Methods The series X-ray film of 1100 patients admitted in our hospital from September 2001 to July 2014 were analyzed retrospectively. According to Sillence classification, there were 376 cases of type Ⅰ, 320 cases of type Ⅲ, and 404 cases of type Ⅳ. Epiphyseal closure occurred in 437 cases, among which 45 cases（ 30 males and 15 females） had long term series of X-ray film and were treated conservatively for the recurrent fractures of the lower legs（ e.g. traction, splints, spica cast）. The average age was 17 years old（ range： 13-34 years）. Sillence classification： type Ⅰ 2 cases, type Ⅲ 20 cases, type Ⅳ 23 case. The number of fractures and the immobilization time of each fracture were analyzed. Functions of the lower leg, the alignment and the radiological results of the femur and the tibia at the latest follow up were recorded. Results Recurrent fractures were treated by conservative method in 45 cases. The mean period of immobilization was 50 days（ range： 30-90 days）. Most fractures healed, while 30 cases have pseudarthrosis and 27 cases showed premature epiphyseal closure. For those legs immobilized by traction or cast without any weight bearing and the time of immobilization was longer than 6 months due to recurrent fractures, the X-ray film showed progressive process of osteoporosis. Radiologically, the bone became slim, the medullary canal closed, the joint swelled compared with the slim diaphysis. Ⅰf the medullary canal existed, the cotex bone was usually thin and the multiple level deformity was common. At the latest follow up, 5% patients were completely bedridden; 30% needed wheelchair; 35% could walk in short distance with the aid of crutches; 30% could walk independently; although some of them had abnormal gait. Unfortunately, 30% cases could not receive any orthopedic procedures because the bone was too slim to be fixed. Conclusions Although the fracture of the lower leg in children with osteogenesis imperfecta usually can heal by conservative treatment, recurrent fracture and conservative treatment will lead to bad long term functional and radiological results. Timely osteotomy and intramedullary pin fixation are recommended to restore the weight-bearing function to avoid adverse effects on bone growth.