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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.
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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.
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Objective To evaluate specific clinical and imaging features of osteogenesis imperfecta V and to improve diagnostic accuracy of this disease. Methods Data of 15 patients with osteogenesis imperfecta type V were retrospectively analyzed for their clinical and imaging features. There were 10 males and 5 females, aged from 1 year and 30 years old (median age,12.5 years ). All 15 patients had plain X-ray, and 4 of 15 had CT. All data were analyzed by 3 experienced deputy chief doctors in OI according to OI V standard. Results X-ray:calcification of the interosseous membrane between radius-ulna was detected in all patients and calcification of the interosseous membrane between tibia-fibula was detected in 2 of 15 patients. Dislocation of the radial head was seen in 13 of 15 patients,bilateral in 9 and utilateral in 4.All patients showed restriction in the pronation and supination of the forearm and restricton in the flexion and extention of the elbow joint. Patients with dislocation of raidal head were associated with large coronoid process and olecranon of the ulna. Hyperplastic callus of the extremities were detected in 7 of 15 patients (7 at femur , 3 at humerus, 1 at tibia.In early stage, hyperplastic callus showed thin cortice, and clear boundares with the diaphysis showing and low density, irregular, mesh-like lamellation inside. In the later stage, there were dense calcification inside hyperplastic callus, and no difference in density with the diaphysis. Diaphysis surrounded by hyperplastic callus had clear boundaries with the hyperplastic callus. No cortical destruction was detected. CT:there were sparse needle-dot calcification inside hyperplastic callus, with the patterns of granular, ring-and-arch,irregular streaky mineralization. The cross section of proximal femoral shaft showed irregular shape , flat square shape and tiny medullary cavity, with no calcification on the edge of hyperplastic callus. CT value:-91 HU inside hyperplastic callus; 283 HU in femoral shaft. Conclusions Interosseous membrane between radius-ulna or tibia-fibula, hyperplastic callus ,dislocation of the radial head are specific features in osteogenesis imperfecta V. X-ray can make a definitive diagnosis of osteogenesis imperfecta V. CT scan is helpful in the differential diagnosis of osteogenesis imperfecta V from osteosarcoma.