C H A P T E R
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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.”
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.
SURGICAL PROCEDURE INDEX
Syme Amputation. . . . . . . . . . . . . . . . . 1543
Boyd Amputation with Osteotomy of the Tibia
for Fibular Deficiency. . . . . . . . . . . . . . . . . . . . . . . . .1548
