膝关节牵伸技术治疗先天性多发性关节挛缩症屈膝畸形

目的　探讨用膝关节牵伸技术 (Ilizarov)治疗先天性多发性关节挛缩症屈膝畸形的方法及疗效。方法　 1998年 8月至 2 0 0 3年 2月收治先天性多发性关节挛缩症 6例 ,其中双膝 4例 ,共 10个膝关节。男 5例 ,女 1例 ,年龄 3岁 7个月至 13岁 ,平均年龄 8岁 2个月。术前屈膝畸形程度平均5 1° ,合并肢体其它部位畸形 13个。应用改良Ilizarov膝关节牵伸器 ,手术安装牵伸器时 ,应维持膝关节于最大伸展位 ,牵伸器的关节铰链对准膝关节的旋转中心 ,于膝上下股骨和胫骨各穿 2组 2mm克氏针与牵伸器上下的钢环固定。术后 5d旋转延长膝后的螺纹牵伸杆 ,第 1周 2～ 3mm/d ,第 2周后改成 1～ 2mm/d ,直至达到膝关节伸直到 0°位。合并髋、足关节畸形者 ,同期或第二期实施手术矫正。本组术后膝关节牵伸时间 2 3～ 4 8d ,平均 37d ,治疗期间患肢可负重锻炼 ,停止牵伸 2周后拆牵伸器再装支具行走。结果　 10个膝关节屈曲畸形完全矫正 ,无严重并发症 ,其中 9个膝术后随访平均 1年 3个月 ,畸形无复发 ,患者行走功能显著改善。结论　Ilizarov技术治疗先天性多发性关节挛缩症屈膝畸形 ,方法简单、安全 ,效果确实 ,符合生物学原理和微创外科的原则

Ilizarov technique for correcting flexion deformity of the knee of arthrogryposis multiplex congenita

OBJECTIVE: To study the methods and effects of Ilizarov distraction technique in treating the flexion deformity of the knee of arthrogryposis multiplex congenita. METHODS: Between August 1998 and February 2003, 6 patients (10 knees) with the arthrogryposis multiplex congenita were treated, 4 patients in double knees, 5 males, 1 female, mean age 8 years and 2 months, ranged from 3 years and 7 months to 13 years. The preoperative flexion degree was averagely 51 degrees. The patients accompanied 13 other parts malformation of limbs. The modified Ilizarov distraction apparatus of the knee was used. While installing the apparatus in the operation, the knees should be kept in the of location of maximum extension, the center of joint hinges on the apparatus should be placed towards the rotatory center of the knee, two groups of 2 mm K-wires were passed through the femur and tibia around the knee, who were fixed on the proximal and distal rings. Distraction was started after the surgical procedure 5 days via rotating the threaded rods at the posterior of the knee, at an average of 2 to 3 mm per day, at the first week, after 2 weeks with the rate modified to 2 mm per day, up to the knee extended to 0 degrees. The accompanied deformities of the hip and/or the foot might be corrected at the same time or next time. The average duration of the distraction was 37 days (23-48 days). During the correction all limbs might undergo weight. After 2 weeks at the end of distraction the fixator was removed and the patients could walk by a long-leg brace. RESULTS: Ten knees with the flexion contracture were sufficiently corrected without severe complications. Nine knees of all were followed up at an average time of 1 years and 3 mouths, no recurrence of the deformity was seen in all patients, their function of walk was significantly improved. CONCLUSIONS: Ilizarov technique is a simply, safe and effective method for managing the flexion deformity of the knee of the arthrogryposis multiplex congentia. The procedure is conformable to the biological theories and microsurgical principles.