先天性多关节挛缩症双侧髋关节脱位的治疗  

Surgical treatment of bilateral hip dislocation in children with arthrogryposis multiplex congenita

作　　者：胡超[1] 李旭[1] 吴伟平[1] 戴双武[1] 谭为[1] 史强[1] 金大地[1] 

机构地区：[1]南方医科大学第三附属医院(广东省骨科研究院)儿童骨科,广州510630

出　　处：《中华小儿外科杂志》2015年第6期461-465,共5页Chinese Journal of Pediatric Surgery

摘　　要：目的 探讨先天性多关节挛缩症双侧髋关节脱位的治疗方法.方法 回顾性分析2008年7月至2012年7月收治的先天性多关节挛缩症双侧髋关节脱位患儿8例的临床资料.其中,男5例,女3例;年龄5～39个月,平均16个月.采用S-P前侧入路和内侧入路治疗髋关节脱位,同时选择性行骨盆截骨和股骨截骨术.结果 本组随访2～5.2年,平均约3.5年.8例中2例步态基本正常,1例轻微跛行,2例步态不稳,1例需佩戴长腿支具行走,2例不能独立行走.4个髋活动基本正常,8个髋活动轻度受限,4个髋活动明显受限.根据Severin的X线片评定标准和修改的McKay临床评定标准,优4髋,良8髋,一般4髋.髋关节屈伸活动范围左右两侧术后比术前提高约30°.其中,左髋关节术前活动度为70.4°±6.3°,术后活动度为104.1°±9.6°;右髋关节术前活动度为72.0°±10.2°,术后活动度为102.5°±8.9°,两者术前与术后比较,差异均有统计学意义（P＜0.001）.本组有3个髋关节发生股骨头坏死,Kalamchi and MacEwen分级Ⅰ、Ⅲ、Ⅵ各1例.结论 先天性多关节挛缩症双侧髋关节脱位者应早期行切开复位来恢复正常髋关节解剖结构,经S-P前侧入路以及骨盆截骨和股骨截骨术是治疗脱位的有效方法.Objective To explore the surgical strategies of bilateral hip dislocation in children with arthrogryposis multiplex congenita.Methods Retrospective analyses were conducted for the clinical data of 8 children with arthrogryposis multiplex congenita from July 2008 to July 2012.There were 5 males and 3 females with an average age of 16 （5-39） months.They underwent open reduction by S-P incision and medial approach along with pelvic osteotomy,femoral osteotomy.Results The average follow-up period was 3.5 （2-5.2） years.The gaits were relatively normal （n =2）,slightly limping （n =1） and unbalanced （n =2）.One case required wearing a long leg orthosis for walking and 2 cases relied upon a wheelchair.And the ranges of motion （ROM） were relatively normal （4 hips）,slightly limited 8 hips） and significantly limited （4 hips）.According to the radiological Severin evaluation criteria and modified McKay scheme,the outcomes were excellent （4 hips）,good （8 hips） and fair （4 hips） Postoperative ROM of bilateral hip improved 30 versus preoperative one.The preoperative ROM of left hip was 70.4° ± 6.3°,postoperative 104.1° ± 9.6°; the preoperative ROM of right hip 72.0° ± 10.2°,postoperative 102.5° ± 8.9°.And significant differences existed between preoperative and postoperative ROMs （P＜0.001）.However,3 hips had avascular necrosis of femoral head.The grades were Kalamchi ＆ MacEwen Ⅰ （n =1）,Ⅲ （n =1） and Ⅵ（n =1）respectively.Conclusions For children with arthrogryposis multiplex congenital,bilateral hip dislocation should be managed with open reduction for restoring normal anatomic structure of hip joint.S-P anterior approach plus pelvic-femoral osteotomy is effective for these patients.

关 键 词：关节 挛缩 髋脱位 截骨术 

分 类 号：R726.8[医药卫生—儿科]