Lamb D W
J Bone Joint Surg Am. 1977 Jan;59(1):1-13.
Based on a review of the embryology, genetics, and anatomy of radial club hand, it is suggested that damage to the apical ectoderm on the anterior aspect of a developing limb bud leads to the deformity. Study of the families of thirty-five children with radial club hand suggested that the condition is not genetically patterned. The anatomical findings and associated congenital abnormalities in the cases known to be related to thalidomide and in those in which thalidomide was not a factor were similar except that the incidence of other skeletal deficiencies was higher in the thalidomide group. Thirty-one of the 117 radial club-hand deformities (in sixty-eight patients) under my personal supervision were treated by centralization of the carpus on the ulna with satisfactory improvement of the deformity. In three cases wrist deformity recurred mainly in a volar direction, apparently the result of muscle imbalance. No significant impairment of ulnar growth occurred and straightening of the wrist did not affect function adversely. Pollicization of the index finger was done on twenty-eight occasions. Although problems developed in the early cases, these can be avoided using the methods described and the operation can improve both function and appearance. A scheme of management is recommended.
基于对桡侧多指(趾)畸形的胚胎学、遗传学和解剖学的回顾,有人提出发育中的肢芽前侧顶端外胚层的损伤会导致这种畸形。对35名患有桡侧多指(趾)畸形儿童的家族研究表明,这种情况并非由遗传模式决定。已知与沙利度胺有关的病例以及沙利度胺并非致病因素的病例中的解剖学发现和相关先天性异常相似,只是沙利度胺组中其他骨骼缺陷的发生率更高。在我个人监督下的117例桡侧多指(趾)畸形(68名患者)中,有31例通过将腕骨集中于尺骨进行治疗,畸形得到了令人满意的改善。3例腕部畸形主要向掌侧复发,显然是肌肉失衡的结果。尺骨生长未出现明显受损,腕部伸直也未对功能产生不利影响。食指转位手术进行了28次。虽然早期病例出现了一些问题,但使用所述方法可以避免这些问题,并且该手术可以改善功能和外观。建议采用一种治疗方案。