外固定架尺骨延长治疗遗传性多发性骨软骨瘤所致前臂畸形

Treatment of forearm deformities caused by hereditary multiple osteochondroma with ulnar lengthening by external fixator

目的探讨采用外固定架尺骨延长治疗遗传性多发性骨软骨瘤(HMO)所致前臂畸形的疗效。方法回顾分析2014年4月至2018年4月北京积水潭医院创伤骨科采用外固定架尺骨延长治疗HMO所致前臂畸形患者5例。其中男4例,女1例;平均年龄14.5岁(12~16岁);右侧2例,左侧3例;MasadaⅠ型2例,MasadaⅡB型3例。5例患者均接受尺骨延长手术治疗。对3例MasadaⅡB型患者采用环形外固定架,另2例采用单边外固定架。1例合并桡骨骨折患者同时行桡骨骨折切开复位内固定术。截骨术后8~10 d开始行尺骨牵开延长。结果5例患者均获得随访,平均随访时间16个月(12~30个月)。3例MasadaⅡB型患者桡骨头均自行复位。5例患者尺骨平均延长37.5 mm(30~45 mm),平均应用外固定架时间162 d(122~274 d),平均外固定架指数48.9 d/cm。术前和术后平均梅奥肘关节评分分别为36.4分和92.7分,平均肘关节屈曲活动范围分别为118.0°(110°~130°)和130.0°(120°~150°),平均伸肘活动范围分别为12.7°(10°~20°)和3.5°(0°~10°),平均前臂旋前活动范围分别为18.6°(5°~30°)和44.7°(30°~65°),平均前臂旋后活动范围分别为71.2°(50°~85°)和86.5°(75°~90°)。1例桡骨干骨折患者术后3个月骨折愈合。2例出现针道感染;1例尺骨过早愈合,行第2次截骨手术后延长顺利。未见神经血管并发症。5例患者对治疗结果均满意。结论采用外固定架逐渐延长尺骨治疗HMO患者尺骨短缩畸形和桡骨头脱位安全有效。

Objective To investigate the effects of ulnar lengthening by external fixator and without tumor excision for treatment of forearm deformities caused by hereditary multiple osteochondromas(HMO).Methods Clinical data of 5 patients with forearm deformity caused by HMO were treated by ulnar lengthening between April 2014 and April 2018.According to Masada classification,2 cases without radial head dislocation were typeⅠ,and 3 cases showed ulnar shortening with distal ulnar exostosis and radial head dislocation were typeⅡB.There were 4 males and one female.The right forearm was affected in 2 patients and left in 3 patients.The mean age of the patients was 14.5 years(range:12-16 years).Ulnar lengthening was performed in all 5 cases.Three patients in typeⅡB were treated with Ilizarov ring fixator and 2 patients in typeⅠwere treated with unilateral external fixator.One patient who had a radial shaft fracture was treated with open reduction and internal fixation with a locking plate.Ulnar osteotomy was performed and ulnar lengthening started 8-10 d postoperatively.Results All 5 patients were followed up for average 16 months(12-30 months).Three patients in typeⅡB showed spontaneous reduction of the radial head and correction of the forearm deformity.The average amount of ulnar lengthening was 37.5 mm(30.0-45.0 mm)and the duration of external fixation was 162 d(122-274 d).The average external fixation index was 48.9 d/cm.Average Mayo Elbow Score improved from preoperative 36.4 points to postoperative 92.7 points.The range of motion of elbow:flexion increased from 118.0°(110°-130°)to 130.0°(120°-150°)and average extension decreased from 12.7°(10°-20°)preoperatively to 3.5°(0°-10°).Forearm pronation improved from a preoperative average of 18.6°(5°-30°)to 44.7°(30°-65°),and the supination increased from 71.2°(50°-85°)preoperatively to 86.5°(75°-90°)postoperatively.Premature consolidation occurred in one patient and pin cite infection occurred in 2 patients.No neurovascular complication was found.All 5 patients were satisfied with the results of treatment.Conclusion Gradual lengthening of the ulna by external fixator is an excellent method for correction of forearm deformity in patients with HMO.