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INTRODUCTION

… in the first decade the most important priority is function, in the second appearance and in the third and subsequent decades the avoidance of pain.

Staheli

Limb deficiencies (lds) in children are an example of the complexities of embryologic, physical, and emotional development. The two broad categories of LDs are congenital and acquired amputations. Congenital defects are often associated with other nonmusculoskeletal anomalies; these can be dictated by genes or caused by stressors during fetal development. “Acquired amputations” refer to limb loss that occurs after birth. Acquired amputations may be secondary to cancer, infection, or trauma. Unlike the adult population, the residual limbs are not static; growth and development create the potential for complications1 (Fig. 59–1).

Figure 59–1

Taxonomy of pediatric amputations

The care of children with LDs requires a multidisciplinary approach. This chapter focuses on medical, functional, and rehabilitation issues of both congenital and acquired LDs in children.

TERMINOLOGY

Classification and nomenclature attempt to categorize LDs by the use of a descriptive method focusing on anatomic and phenotypic findings despite the etiology. Greek and Latin names were often used to describe congenital LDs. Terms such as hemimelia, meromelia, and phecomelia have been deemed inaccurate and are often confusing. Since the 1960s, several attempts have been made to improve this terminology.27 Consensus was reached, and an international terminology was established by the International Society for Prosthetics and Orthotics (ISPO).6,8 The ISPO classification creates a logical, accurate approach to naming congenital LDs (Fig. 59–2). It focuses on dividing all deformities into transverse or longitudinal. A transverse deficiency has no distal remaining portions, whereas the longitudinal deficiency has distal portions. The transverse level is named after the segment beyond which there is no skeletal portion. Longitudinal deficiencies name the bones that are affected, beginning with the most proximal long bone. Any bone not named is presumed present and of normal form. The affected bone is designated as total or partially absent. The approximate fraction of the limb in a transverse deficiency is estimated in thirds, whereas the longitudinal deficiencies describe a partial or complete bone absence. Involved digits are then identified. Digit numbering proceeds from the radial or tibial side of the limb. “Ray” refers to the metacarpal or metatarsal and corresponding phalanges.

Figure 59–2

ISPO classification for congenital limb deficiencies. (Left) Examples of transverse deficiencies at various levels. (Right) Examples of longitudinal deficiencies. (Day, H.J.B. The ISO/ISPO classification of congenital limb deficiency. Prosthetics and Orthotics International 15(2), pp. 67-69. Copyright © 1991 by The International Society for Prosthetics and Orthotics. Reprinted by permission of SAGE Publications, Ltd.)

The terminology used for acquired ...

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