The Journal of Bone and Joint Surgery (American). 2009;91:3-18.
doi:10.2106/JBJS.I.00008
© 2009 The Journal of Bone and Joint Surgery, Inc.
Classification and Developmental Biology of Congenital Anomalies of the Hand and Upper Extremity
Paul R. Manske, MD1 and
Kerby C. Oberg, MD, PhD2
1 Washington University School of Medicine, Campus Box 8233, 660 South Euclid Avenue, St. Louis, MO 63110. E-mail address: manskep@wudosis.wustl.edu
2 Divisions of Pediatric Pathology and Human Anatomy, Department of Surgery, Loma Linda University, 24785 Stewart Street, Room B09c, Loma Linda, CA 92350
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Introduction
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Congenital anomalies of the hand and upper extremity are classified according to appearance; thus, the myriad of disparate presentations are organized into groups that share common morphologic features. The primary purpose of a classification is to enhance communication about the specific features of a condition by providing a descriptive framework for clinicians. Therefore, classification schemes should reflect the full spectrum of morphologic abnormalities within a given condition, and should be uncomplicated and easy for clinicians to remember and use. While an ideal classification would also guide treatment, provide insight into prognosis, and incorporate the etiologic mechanism of the condition, congenital classification systems often fall short of these goals.
The embryological development of the upper limb proceeds along three axes: proximal-distal, anterior-posterior (referred to postnatally as radial-ulnar by clinicians, because the fetal upper limb rotates during development), and dorsal-ventral1. The apical ectodermal ridge and the underlying mesoderm control proximal-distal development . . . [Full Text of this Article]

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