Objective To evaluate the efficacy of prevention of recurrent femur fracture in children with osteogenesis imperfecta.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 Xray 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.

Objective To evaluate the safety and efficacy of telescopic intramedullary rod for treatment of femur fracture or deformity correction in children with osteogenesis imperfecta,and to analysis the result of prevention recurrent fracture as well as the complication.Methods Data of patients who were treated by telescopic intramedullary rod for recurrent femur fracture or curved femoral deformity from March 2015 to December 2015 were prospectively analyzed.There were 39 boys and 26 girls.The average age of the patients was 9 years 2 months,ranging from 3 years 5 months to 13 years 4 month.All the patients had suffered from recurrent femur fractures leading to femoral deformity.The mean angulation angle was 58° (range,30°-95°).Among 69 sides,there were 21 sides of new fracture and 48 sides of deformity.Sixty-one patients were operated at one side and the other 4 patients were treated bilaterally.According to the modified Sillence classification system,there were 5 cases of type Ⅰ,17 type Ⅲ,34 type Ⅴ,3 type Ⅴ,2 type Ⅵ and 4 type ⅩⅤ.Results All the 65 patients were followed up for a mean period of 32 months (range,15-43).The average healing time of the osteotomy site or fracture site of the femur was 8 weeks (range,7-12).The patient was encouraged to begin weight bearing and walking when the Ⅹ-ray film showed healing of the osteotomy or fracture 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 0.5±0.2/year,compared to 2.7±1.8/year before operation.All the parents of the children were satisfied with the result of deformity correction.The children's self care and motion ability improved obviously after operation.During follow up,6 patients suffered from recurrent fracture of the femur by various degree,1 of them was treated by open reduction and telescopic rodding surgery,while the other 5 patients were treated conservatively because the fracture displaced or angulated minimally and 4 patients healed uneventfully while 1 patient need plate fixation to augment the axial stability.In 3 patients (1 type Ⅳ,2 type Ⅲ) the intubator failed to elongate with the growth of the distal femoral epiphysis,and in 2 patients the obturator migrated proximally which needed to be re-fixed.Low toxic infections occurred in 2 patients (type Ⅵ) which were treated successfully by removal of the rod and antibiotics.Conclusion The telescopic intramedullary rod can maintain the correction of the femur deformity and improve the quality the bone,thus prevent the recurrent fracture of the femur in children with osteogenesis imperfecta effectively.

Objective To study the method and therapeutic effect of Peter-Williams intramedullary nail in children with tibia fracture due to osteogenesis imperfecta.Methods A total of 30 children of tibia fracture due to osteogenesis imperfecta,including 17 males and 13 females in Department of the Third Orthopaedics and Osteogenesis Imperfecta Center,Tianjin Wuqing District People's Hospital from June 2015 to January 2017,were operated on the shaft of tibia with Peter-Williams intramedullary nail.Twelve cases were left tibia fracture,13 cases were right tibia fracture,and 5 cases were bilateral tibia fracture.Twenty of 35 tibias were accompanied with no tibial deformity,and 15 of 35 tibias were accompanied with different degrees of anterior arch bowing deformity.The movement and life self-care ability were evaluated according to Functional independence measure (WeeFIM) evaluation form.Results All of the children were followed up for an average of 18 months(ranging from 10 to 26 months).The average operating time was 1.2 hours(0.75-2.50 hours).The average bleeding was 60 mL(30-100 mL).The average bone healing time was 10 weeks (8-16 weeks).Patients began to exercise on bed after removing plaster,and to walk after X-ray showing bone healing.Parents of all children were satisfied with surgical operation,and the WeeFIM score was improved by (60.76 ± 15.57) scores (64-85 scores) preoperatively to (82.00 ± 7.50) scores (75-91 scores) postoperatively.There was a statistically significant difference in scores before and after the treatment(t =2.765,P =0.003).There was no infection,osteomyelitis,nerve damage,or vascular damage.Conclusions Therapy of Peter-Williams intramedullary nail in children with tibia fracture due to osteogenesis imperfect is advantaged.It causes less blood,less damage,with no intruding joint,correcting deformity and improving lower limb functions and life quality.