Jones Neil F, Kaplan Jesse
Center for Hand and Upper Extremity Surgery, University of California Irvine, 101 The City Drive South, Orange, CA 92868 USA ; University of California, Los Angeles, CA USA ; Shriners Hospital for Children, Los Angeles, CA USA.
Hand (N Y). 2012 Dec;7(4):391-9. doi: 10.1007/s11552-012-9450-0.
Congenital absent digits continue to be described by many confusing terms and are currently classified in categories I, V, and VI of the International Federation of Societies for Surgery of the Hand classification and seven subclassification systems. Very few classification systems provide any logical basis for surgical reconstruction. The purpose of this study was to develop a simple alphanumerical documentation system to reproducibly describe the morphological or radiographic appearance of congenital absent digits and facilitate communication of these childrens' hand anomalies from one hand surgeon to another.
Dorsal and palmar photographs and PA radiographs of 235 hands in 204 children born with congenital absent digits over a 15-year period were analyzed to determine which digital rays were missing and their level of absence. Each hand can be described by three letters, R (radial), C (central), and U (ulnar), as well as numbers 1-5. The first letter and number designate which rays are missing and the second and third letters and numbers designate which rays remain present.
There are 15 morphological phenotypes of congenital absent digits. The three most common phenotypes are U4R1 (a thumb but absence of all four fingers), R1U4 (absent thumb), and R5 (aplastic hand).
This new documentation system allows hand surgeons to describe the simple morphological or radiographic appearance of congenital absent digits; incorporates all the previous subclassification systems that have attempted to describe congenital absent digits in radial, central, and ulnar deficiencies, symbrachydactyly, and congenital constriction ring syndrome; and has subsequently allowed the development of an algorithm which predicts whether conventional or microsurgical reconstruction is indicated for each specific phenotype.
先天性缺指仍有许多令人困惑的术语来描述，目前在手外科国际联合会分类的I、V和VI类以及七个亚分类系统中进行分类。很少有分类系统为手术重建提供任何逻辑依据。本研究的目的是开发一种简单的字母数字记录系统，以可重复的方式描述先天性缺指的形态或放射学表现，并促进这些儿童手部异常情况在不同手外科医生之间的交流。
对15年间204例先天性缺指患儿的235只手的背侧和掌侧照片以及正位X线片进行分析，以确定哪些指骨缺失及其缺失程度。每只手可用三个字母，即R（桡侧）、C（中央）和U（尺侧）以及数字1 - 5来描述。第一个字母和数字表示缺失的指骨，第二个和第三个字母及数字表示剩余的指骨。
先天性缺指有15种形态学表型。三种最常见的表型是U4R1（有拇指但四个手指均缺失）、R1U4（拇指缺失）和R5（手部发育不全）。
这种新的记录系统使手外科医生能够描述先天性缺指的简单形态或放射学表现；整合了所有先前试图描述桡侧、中央和尺侧缺指、并指畸形和先天性束带综合征中先天性缺指的亚分类系统；随后还促成了一种算法的开发，该算法可预测每种特定表型适合传统手术还是显微外科重建。
