基于Ilizarov技术的Achterman I型腓侧半肢畸形肢体功能重建的疗效分析

Outcomes of limb reconstruction in treating achterman type I fibular hemimelia based on Ilizarov tech-nique

目的探讨Ilizarov技术结合组合性手术治疗Achterman I型先天性腓侧半肢畸形的疗效,并总结先天性腓侧半肢畸形肢体功能重建治疗的诊治要点。方法本研究回顾2014年至2021年收治的20例先天性腓侧半肢畸形患者,男12例,女8例,平均年龄(17.5±7.0)岁(4~26岁);AchtermanⅠA型14例,ⅠB型6例。针对肢体短缩采用单纯Ilizarov技术或内外结合进行肢体延长;对于膝外翻、踝外翻畸形,成人患者采用闭口截骨矫形内固定,儿童患者采用骨骺阻滞;松解和延长挛缩的髂胫束、腓骨肌腱和跟腱;肌腱转位纠正叠趾畸形。结果20例患者平均随访(36.1±8.7)月,平均延长(7.2±1.0)cm,EFI(15.9±6.3)d/cm,HI(21.7±2.8)d/cm。所有患者末次随访时患肢短缩、膝外翻[GVA:术前(14.9±3.4)°,末次随访(2.1±1.7)°,t=13.510,P<0.05]、胫骨弯曲畸形[TBA:术前(3.3±3.3)°,末次随访(0.8±0.7)°,t=3.490,P<0.05]及足踝畸形[VAA:术前(9.2±3.4)°,末次随访(1.2±1.3)°,t=9.785,P<0.05]均已得到矫正。LLRS AIM较术前明显改善[术前(10.1±1.2)分,末次随访(2.0±1.4)分,t=21.140,P<0.05]。结论先天性腓侧半肢畸形存在复杂的肢体短缩、力线异常和足踝畸形,Ilizarov技术与髓内延长、组织松解、截骨矫形和骨骺阻滞等手术的结合在治疗先天性腓侧半肢畸形方面疗效满意,能够明显改善患者生活质量。

Objective To explore the clinical outcomes of combination of Ilizarov technique and composite surgeries for treatment of congenital fibula hemimelia,and to summarize the key points of limb re-construction management for such deformity.Method Twenty patients with congenital fibula hemimelia were treated surgically and retrospectively studied in Department of Orthopedics,Shanghai Jiao Tong Univer-sity affiliated Sixth People's Hospital since 2014.Patients included 12 males and 8 females,with an age of(17.5±7.0)y(4 to 26 y).According to Achterman classification,there were 14 cases of type IA and 6 cases of type IB.Single Ilizarov technique or combined intramedullary assistance was applied for limb length defi-ciency;For genu valgus or valgus ankle deformities,close-wedge osteotomy and internal fixation were ap-plied for adult patients,and hemiepiphysiodesis for pediatric patients;Contracted iliotibial band,fibular ten-dons and Achilles'tendon were released and elongated;Overlapping toes were corrected by tendon transfer.Results Twenty patients were followed up for(36.1±8.7)months.The limb was lengthened for(7.2±1.0)cm,with average EFI(15.9±6.3)d/cm and average HI(21.7±2.8)d/cm.All obvious limb length discrepancy,genu valgus[GVA:(14.9±3.4)°and(2.1±1.7)°preoperatively and at last follow-up,t=13.510,P<0.05],tibial angulation[TBA:(3.3±3.3)°and(0.8±0.7)°preoperatively and at last follow-up,t=3.490,P<0.05]and ankle and foot deformities[VAA:(9.2±3.4)°and(1.2±1.3)°preoperatively and at last follow-up,t=9.785,P<0.05]were corrected at the final follow-up.Clinical outcomes indicated significantly improved limb function com-pared with preoperative grading.[LLRS AIM:(10.1±1.2)points and(2.0±1.4)points preoperatively and at last follow-up,t=21.140,P<0.05].Conclusion Congeinital fibular hemimelia is a rare and complicated syn-drome with co-exist limb length deficiency,abnormal alignment of lower-limb and foot and ankle deformi-ties.Combination of Ilizarov technique and composite surgeries can bring satisfying outcomes for treatment of congenital fibula hemimelia and improve the quality of life of the patients.