小儿先天性桡偏手的手术治疗

Surgical treatment of congenital radial clubhand in young children

目的探讨先天性桡偏手的诊断和治疗。方法3例男性患儿,右侧2例,双侧1例。手术年龄分别为20个月、6岁8个月、24个月。按Heikel分型,单侧的为Ⅳ型与Ⅲ型,双侧的为左侧Ⅲ型、右侧Ⅰ型。手术由3部分组成:尺骨远端中位化对应腕骨、尺骨干楔形截骨纠正弓状弯曲、尺侧伸腕肌腱移位稳固腕关节。结果手腕桡偏纠正满意,腕关节稳定且有一定活动度,手指屈伸功能明显改善。随访6～43个月,以术前/术后/随访的X线片测量前臂-手之整体角评价术前畸形度、术后纠正量和复发量:例1为88°/-6°/-21°;例2为103°/0°/0°;例3为75°/-7°/-15°。结论先天性桡偏手是一种少见而复杂的手畸形,尺腕中位化手术与尺骨截骨、肌腱转位同期完成可以重建稳定的腕关节,改善手腕与手指的外观和功能。

Objective To review the diagnosis and the treatment of congenital radial clubhand. Methods Four congenital radial clubhands in three boys were reviewed. The two right-side clubhands were type Ⅳ and type Ⅲ respectively. The age of the two patients at the time of surgery was 20 months and 6 years and 8 months respectively. The patients with bilateral clubhand （Left： type Ⅲ, R： type Ⅰ） was 24 months. The operation consisted of three procedures： 1 ） centralization of the carpus on the distal ulna, 2） a wedge osteotomy to straighten the curved ulna,3） and transfer of the extensor carpi ulnaris to balance the wrist. Results The patients had reduced radial deviation of the wrist, good stability of the wrist with motion, and significant improvement of flexion-extension function of fingers. The total angulation （ the combination of the radial deviation of the hand and the ulna bow） was measured to determine the initial deformity,degree of surgical correction, and degree of recurrence. Pre-operative, post-operative, and follow-up x-rays were taken to measure the angles. The angulation of case 1 was 88°/6°/21° respectively（follow-up 6 months ） ; the angulation of case 2 was 103°/ 0°/ 0° respectively（follow-up 14 months ）; the angulation of case 3 was 75°/7°/15° respectively（follow-up 43 months）. Conclusions Congenital radial clubhand is a rare and complex hand anomaly. Centralization of the wrist on the ulna combined with an ulnar osteotomy and tendon transfers procedures are performed simultaneously to reconstruct stabilization of the wrist, to improve function of the wrist and finger,and to enhance appearance of the extremity.