Weinstein Lovell and Winters Pediatric Orthopaedics 7e

C H A P T E R 30 The Child with a Limb Deficiency

Richard E. Bowen Norman Y. Otsuka

Classification The attempt to classify congenital anomalies has evolved over time to more precisely describe each patient’s particular anom- aly. The first descriptions of limb deficiencies used terms to describe specific phenotypes, such as “phocomelia” (phoke = seal) to describe the loss of the arm and forearm with attach- ment of the wrist and hand to the trunk or “lobster-claw hand” to describe the loss of central digits of the hand. A widely used classification system in the United States for congenital anom- alies was devised by Frantz and O’Rahilly (1) (Fig. 30-1). This system differentiates between complete limb absence (amelia) and partial limb involvement. Partial limb involvement can affect roughly half of the limb (hemimelia), foot (podos), hand (cheir), digits (dactylos), or phalanges (phalanx). Deficiencies can be transverse, where the distal part of the extremity is lost and the proximal part is relatively normal; longitudinal, where one side of the limb (either the preaxial, postaxial, or central portion) is affected; and intercalary, where the proximal and distal limb are relatively unaffected with an intervening affected segment (Table 30.1). An international collaboration by the International Standards Organization (ISO) and the International Society for Prosthetics and Orthotics (ISPO) produced a classification system accepted as the universal language of national orga- nizations that treat these children (2). Many of the concepts of Frantz and O’Rahilly are incorporated into this system, although the Greek word roots have been eliminated. This ­system also uses the concept of transverse and longitudinal defi- ciencies. In the transverse deficiencies, the part of the ­segment at which the limb is missing is named, and the extent within that segment may be stated. Thus, complete limb loss at the midtibial level would be “transverse lower leg mid third.” In a longitudinal deficiency, the bone or bones missing are named from proximal to distal and described as “partial” or “total.” Therefore, a complete tibial deficiency with a hypoplastic great toe would be “longitudinal tibia complete, ray 1 partial.”

SURGICAL PROCEDURE INDEX Syme Amputation. . . . . . . . . . . . . . . . . 1543 Boyd Amputation with Osteotomy of the Tibia for Fibular Deficiency. . . . . . . . . . . . . . . . . . . . . . . . .1548

Definition Generally speaking, limb deficiency is defined as the loss of any part of a limb. This can vary widely in severity, from the unilateral partial loss of a toe as can be seen in constriction band syndrome to the total loss of multiple extremities due to teratogens or genetic syndromes. Limb deficiency can be congenital or acquired. Congenital deficiency may be caused by factors such as genetic syndromes or amniotic bands, while acquired deficiency may be the result of factors such as trauma, severe systemic infection (meningococcemia), or malignant tumor. Child versus Adult Limb Deficiency.  Significant differences exist between the pediatric and adult limb defi- ciency patient. Children more often have congenital defi- ciencies, multiple limb deficiencies, and upper extremity deficiencies. Comorbid conditions such as diabetes that are often present in adult dysvascular amputation patients are usually absent in children. Because children are growing, they undergo length and volume changes in their residual limb, and may need more frequent prosthetic and surgical modifi- cations. Phantom pain, which is common in adults, is not as common in children. Children more readily adapt both physi- cally and psychosocially to their situation, and they often have higher functional demands than their adult counterparts. Just as the dictum “a child is not a small adult” is true in all fields of pediatric specialty care, it is also true in caring for the child with a limb deficiency.

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