peter-williams髓内钉治疗成骨不全肱骨畸形

Treatment with peter-williams intramedullary nail on children with humerus deformity due to osteogenesis imperfecta

目的 探讨应用peter-williams髓内钉采用多段截骨矫形手术治疗成骨不全肱骨畸形方法及疗效.方法 回顾性分析2007年6月至2012年12月收治的9例成骨不全肱骨畸形儿童,男7例,女2例.初次手术时年龄6～10岁7个月（平均8岁3个月）.其中7例患儿为单侧肱骨干畸形,2例患儿为双侧肱骨干畸形,伴尺桡骨严重畸形,肱骨骨折次数4 ～13次（平均7.9次）.9例患儿中畸形成角处为2～3处（平均2.09处）,畸形成角度数（畸形两侧骨干轴线的交角）为20°～ 100°,平均57.3°.术前Constant-Murley评分16～24分（平均20.44分）.依据修订后的SiUence分型：Ⅲ型6例,Ⅳ型3例.9例患儿的11处肱骨骨干接受了手术截骨内固定治疗.结果 所有患者均获得随访,随访时间12 ～66个月,平均22个月.肱骨截骨平均9.5周（8～12周）愈合.全部患儿的父母对手术结果及畸形矫正效果满意,末次随访时患儿Constant-Murley评分由术前（20.44±2.79）分（16～24分）提高到术后（35.56±2.60）分（30～ 38分）,评分治疗前后有统计学差异（t=0.20,P＜0.05）.l例患儿,术后3d发现患侧拇指背侧麻木,背伸受限.考虑为桡神经牵拉挫伤造成,经过神经营养药物治疗,3个月后好转.本组无一例患儿出现感染、骨髓炎等并发症,亦无血管损伤病例.所有患儿末次随访检查X线片未发现骨骺板损伤或提前闭合影响生长,未发现骨折不愈合.结论 多段截骨应用peter-williams髓内钉手术治疗成骨不全肱骨畸形,对肢体损伤较小,不进入肩关节,出血少,最大程度地矫正畸形,改善肩关节功能,提高生活质量.

Objective To evaluate the therapeutic effect with peter-williams intramedullary nail on children with humerus deformity due to osteogenesis imperfecta.Methods Data of 9 patients with humerus deformity due to osteogenesis imperfecta were retrospectively analyzed from Jun.2009 to Dec.2012.There were 7 males and 2 females,aged from 6 years to 10 years and 7 months（average 8 years and 3 months）.There were 7 unilateral humerus deformity and 2 bilateral humerus deformity with severe radius and ulna deformity.The humerus fracture frequency was from 4 to 13 times （average,7.9 times）There were 2-3 deformity points in 9 patients （average 2.09 deformity points）.The deformity angle ranged from 20°-100° （average 57.3°）.The Constant-Murley scores were 16-24 scores （average 20.44）.According to revised Sillence classifications,there were 6 cases of Type Ⅲ and 3 cases of Type Ⅳ.Eleven humerus of 9 patients were osteotomied and fixed with peter-williams intramedullary nail.Results All of 9 children were followed up for 12-66 months（average 22 months）.The bone healing time were 8-12 weeks （average 9.5 weeks）.Parents of 9 children were satisfied with surgical operation effect and deformity correction.The Constant-Murley scores ranged from preoperative average of （20.44 ± 2.79） points （16-24 points） to postoperative average of （35.56 ± 2.60） points（30-38 points） at the latest follow-up of patients,there was a statistically significant in score before and after treatment（t =0.20,P 〈0.05）.Number 4 patient,one patient was found suffering from dorsal thumb numbness postoperatively after 3 days back stretching limitation.Considering the radial nerve stretching injury,treatment with neurotrophic drug for 3 months,symptoms disappeared.There was no infection,or osteomyelitis,no vascular damages.Epiphyseal plate injury or premature closure and affecting growth were not found in all of the patients at the latest follow-up examination.Conclusions Treatment with osteotomy and peter-williams intramedullary nail fixation on children with humerus deformity due to osteogenesis imperfecta is advantaged.It gets less damages,no intruding shoulder joint,less bleeding.The greatest degree of correction of the deformity can be achieved,and the shoulder joint function and the quality of life can be improved.