CAJ | 학술논문

目的探讨对成骨不全症患儿施行多段截骨矫形髓内固定手术的安全性,评价其治疗效果及并发症.方法 2005年8月至2010年8月,共收治123例儿童成骨不全症患儿.共160侧肢体:股骨119侧,胫骨41侧.男85例,女38例.患儿手术时平均年龄为8岁3个月(2岁1个月～15岁7个月).依据修订的Sillence分型:Ⅲ型45例,Ⅳ型74例,Ⅴ型4例.术前根据畸形程度,通过术前X线片确定截骨点,术中均行直视下截骨.选用直径及长度适宜的髓内钉,股骨自大转子,胫骨自足底插入.股骨术后行石膏裤、胫骨术后行长腿石膏托固定；术后6周去石膏,开始在支具保护下逐渐站立及行走.123例患儿均周期性静脉给予帕米膦酸二钠治疗,给药时间距手术至少间隔2个月.结果 123例患儿全部获得平均38个月(13～64个月)随访.截骨平均术后8周愈合.患儿父母对手术结果及畸形矫正均满意,患儿生活自理能力、活动范围较术前明显改善.14例患儿因出现Rush钉偏移接受再次手术,25例患儿术后2年因骨骼生长Rush钉相对变短而需要更换内固定.结论多段截骨矫形髓内固定术是治疗成骨不全症的有效手术方法,可以显著矫正畸形、改善活动能力和避免再次骨折；但需要注意髓内钉移位等并发症,对生长期儿童需要定期更换内固定.
목적 탐토대성골불전증환인시행다단절골교형수내고정수술적안전성,평개기치료효과급병발증.방법 2005년8월지2010년8월,공수치123례인동성골불전증환인.공160측지체:고골119측,경골41측.남85례,녀38례.환인수술시평균년령위8세3개월(2세1개월～15세7개월).의거수정적Sillence분형:Ⅲ형45례,Ⅳ형74례,Ⅴ형4례.술전근거기형정도,통과술전X선편학정절골점,술중균행직시하절골.선용직경급장도괄의적수내정,고골자대전자,경골자족저삽입.고골술후행석고고、경골술후행장퇴석고탁고정；술후6주거석고,개시재지구보호하축점참립급행주.123례환인균주기성정맥급여파미련산이납치료,급약시간거수술지소간격2개월.결과 123례환인전부획득평균38개월(13～64개월)수방.절골평균술후8주유합.환인부모대수술결과급기형교정균만의,환인생활자리능력、활동범위교술전명현개선.14례환인인출현Rush정편이접수재차수술,25례환인술후2년인골격생장Rush정상대변단이수요경환내고정.결론 다단절골교형수내고정술시치료성골불전증적유효수술방법,가이현저교정기형、개선활동능력화피면재차골절；단수요주의수내정이위등병발증,대생장기인동수요정기경환내고정.
Objective To evaluate the safety,therapeutic effect and complications of the operation of multi-section osteotomy and intramedullary fixation for children with osteogenesis impeffecta.Methods One hundred and twenty-three children with osteogenesis imperfecta,including 85 males and 39 females,were selected from August 2005 to August 2008.According to the modified Sillence classification,45 cases was in type Ⅲ,74 in type Ⅳ,and 5 in type Ⅴ.Patients' age was ranging from 2 years and 1 month to 15 years and 7 months (mean,8 years and 3 months).The location of osteotomy was established according to the pre-operative measurement of X-ray image,and all the procedures of osteotomy were completed under direct vision.Then we chose the intramedullary pin with suitable size and insert the pin into femur from greater trochanter,and tibia from pelma.After the surgery,external fixation (spica cast for the femur,long leg plaster cast for the tibia) was made for further stabilization.Plaster supporters were removed 6 weeks later and all children began to stand and walk under the protection of orthoses.In addition,all patients received the treatment of pamidronate disodium periodically.Results All 123 children were followed up for an average of 38months (range,13-64 months).Parents of all children were satisfied with the result of surgical operation,and the children's self-care and motion ability improved obviously.Fourteen children were performed the second operation due to the translocation of Rush pin,and 25 children changed the internal fixation because of the shorter Rush pin 2 years postoperatively.Bone delayed union was not found in all patients.Conclusion Multi-section osteotomy and intramedullary fixation for children with osteogenesis imperfecta could correct skeletal deformity,improve motion ability and avoid second fracture efficiently.However,such complications as translocation of intramedullary pin and changing the internal fixation with the growth of child need to pay more attention.