James Michelle A, Green Hillary D, McCarroll H Relton, Manske Paul R
Department of Orthopaedic Surgery, Shriners Hospitals for Children, Northern California, 2425 Stockton Boulevard, Sacramento, CA 95817, USA.
J Bone Joint Surg Am. 2004 Oct;86(10):2196-205. doi: 10.2106/00004623-200410000-00010.
Congenital longitudinal deficiencies of the radius and thumb are known to be associated with one another; however, the details of their relationship are unknown. The purpose of this study was to determine whether increased severity of radial deficiencies is associated with increased severity of thumb deficiencies and to review the relationship between radial deficiency and reconstructibility of a hypoplastic thumb.
Radiographs and charts of 227 affected upper extremities of 139 patients with radial longitudinal deficiency were reviewed. The associated thumb deficiency was classified according to a modification of the Blauth and Schneider-Sickert scheme and the radial deficiency was classified according to a modification of the Bayne and Klug criteria for 191 extremities of 119 patients.
The severity of the thumb deficiency was directly proportional to the severity of the radial deficiency (p < 0.0001). Half of the extremities had either a thumb deficiency or thumb and carpal deficiencies without radial deficiency. Two-thirds (sixty-three) of the ninety-five limbs with a normal radius had a thumb that could be surgically reconstructed. Seventy-one (91%) of seventy-eight extremities with a thumb amenable to surgical reconstruction had a radius that did not require surgical reconstruction. All extremities with a radial and/or carpal deficiency had a thumb deficiency. Forty-eight (94%) of fifty-one extremities with complete absence of the radius had a thumb that was not reconstructible.
This study supports the growing body of evidence that the components of radial longitudinal deficiency represent a progressive spectrum of upper extremity abnormalities, and a distal progression of severity, with distal structures likely to be more involved than proximal structures.
已知先天性桡骨和拇指纵向发育不全相互关联；然而，它们之间关系的细节尚不清楚。本研究的目的是确定桡骨发育不全严重程度的增加是否与拇指发育不全严重程度的增加相关，并回顾桡骨发育不全与发育不全拇指可重建性之间的关系。
回顾了139例桡骨纵向发育不全患者227例受累上肢的X线片和病历。根据对布劳斯（Blauth）和施奈德 - 西克特（Schneider - Sickert）方案的修改对相关拇指发育不全进行分类，并根据对贝恩（Bayne）和克鲁格（Klug）标准的修改对119例患者的191个肢体的桡骨发育不全进行分类。
拇指发育不全的严重程度与桡骨发育不全的严重程度成正比（p < 0.0001）。一半的肢体存在拇指发育不全或拇指及腕骨发育不全但无桡骨发育不全。95个桡骨正常的肢体中有三分之二（63个）的拇指可以进行手术重建。78个适合手术重建的拇指的肢体中有71个（91%）的桡骨不需要手术重建。所有存在桡骨和/或腕骨发育不全的肢体都有拇指发育不全。51个完全没有桡骨的肢体中有48个（94%）的拇指不可重建。
本研究支持越来越多的证据表明，桡骨纵向发育不全的各组成部分代表了上肢异常的渐进性谱系，且严重程度呈远端进展，远端结构可能比近端结构受累更严重。
