Ilizarov技术结合组合性手术治疗先天性腓侧半肢畸形

A composite surgical procedure using Ilizarov technique for treatment of congenital fibula hemimelia

[目的]探讨先天性腓侧半肢畸形特点及Ilizarov技术结合组合性手术的疗效。[方法]回顾性研究2001年12月~2014年7月本科收治的30例先天性腓侧半肢畸形患者临床资料,男20例,女10例,年龄1~25岁,平均13岁;Achterman分型:ⅠA型3例3肢,ⅠB型12例13肢,Ⅱ型15例17肢;伴足踝部畸形:马蹄内翻3例3足,马蹄外翻27例30足;腓侧跖列缺失:第5列缺失9例10足,第4、5列缺失13例15足,第3、4、5列缺失2例,6例无跖列缺失。手术方法:腓侧束带切除、腓骨肌腱延长、跟腱延长30例33肢,胫骨下段截骨矫形20例23肢,胫骨近端截骨延长18例次(包括二次手术实施延长者),股骨延长2例。所有患者均采用Ilizarov技术进行矫形和延长。[结果]30例患者均获随访,时间14~130个月,平均39个月;手术次数1次4例,2次12例,3次9例,4次5例;所有患者末次随访时小腿短缩及足踝部畸形均获大部分矫正,恢复跖行足,行走功能及步态较术前均获显著改善。参照作者制定的腓侧半肢畸形肢体功能评价标准,结果优21例,良8例,可1例,优良率96.7%。[结论]先天性腓侧半肢畸形是一种综合征,临床上常见的是部分或全部腓骨缺如,并伴有胫骨、股骨、踝关节及足部畸形等;采用软组织松解结合Ilizarov技术延长矫形手术可取得满意的疗效。

[Objective] To explore the clinical outcomes of a composite Surgical procedure using Ilizarov technique for treatment of congenital fibula hemimelia. [Methods ] Thirty patients with congenital fibula hemimelia were treated surgically in the Rehabilitation Hospital of the National Research Center for Rehabilitation Technical Aids from December 2001 to July 2014. Patients included 20 males and 10 females, with an average age of 13 years （range, 1 to 25 years） . According to Achterman classification, there were 3 cases of type I A （3 limbs）, 12 cases of type I B （13 limbs）, and 15 cases of type II （17 limbs） .Foot and ankle deformities included 3 cases of talipes equinovarus （3 feet） and 27 cases of talipes valgus （30 feet） . Checking of the absence of fibular toe revealed 9 cases （10 feet） without fifth toe, 13 cases （15 feet） without fourth-fifth toe, 2 cases without third-fourth-fifth toe, as well as 6 cases without complete toe. In the operation, 30 cases （33 limbs） received fibular bands excision, peroneal tendon lengthening, and achilles tendon lengthening, 20 cases （23 limbs） received distal tibial osteotomy, 18 cases received proximal tibial osteotomy （including second operation）, while femoral lengthening was performed in 2 cases. Ilizarov technique was carried out for orthopedic lengthening in all cases. IRe- suits] All 30 patients were followed up for 14-130 months （mean, 39 months） . Four cases were operated only once, 12 cases were operated for 2 times, 9 cases were operated for 3 times, and other 5 cases were operated for 4 times. No obvious lower leg shortening or ankle and foot deformities was noted after operation immediately and at the final follow-up. Walking function and gait were significantly improved compared with preoperative state. Clinical outcome was evaluated using the criteria of fibula hemimelia functional score system, the result revealed that the excellent result was achieved in 21 patients, good in 8, and fair in 1. The total excellent and good outcomes rate was 96.7 %. [ Conclusions] Congenital fibula hemimelia is a syndrome that is partial or total aplasia of the fibula, and usually is associated with other anomalies of tibia, femur, ankle joint, and foot, The method of Ilizarov technique combined with soft tissue release has satisfactory therapeutic outcomes for treating congenital hemimelia.