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CHAPTER 30
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The Child with a Limb Deficiency
A
C
B
FIGURE 30-27.
The AP pelvis and limbs of a newborn boy
(A)
and at 3 years of age, just before surgery
(B)
, with Aitken
class C PFFD. Note the very short femoral segment and the lack of acetabular development. The same patient is seen in
(C)
at
the age of 12, following a Syme amputation and knee arthrodesis with preservation of the proximal tibial physis. There is still
no appearance of a proximal femoral ossific nucleus.
segment that is <50% of the contralateral side (Fig. 30-30).
Group C patients have only a small tuft of distal femur present
and no acetabular development (Fig. 30-31). He recommends
prosthetic treatment for his group B and C patients.
Paley based his classification on treatment recommenda-
tions as well, with a special emphasis on what is necessary for
limb lengthening and reconstruction (164). He emphasized
the importance of the degree of dysplasia and function of the
knee for a good outcome with lengthening. His type 1 is simi-
lar to Gillespie’s group A but is divided into three subgroups
depending on problems at the hip and knee which will have to
be addressed either before or at the same time as lengthening.
Type 2 has a mobile pseudarthrosis with or without a mobile
femoral head. Stabilization of the pseudarthrosis or of the proxi-
mal femur in relation to the pelvis is an essential prerequisite
of lengthening. When the femoral head is immobile or absent,
stabilization of the external fixator to the pelvis is necessary, fre-
quently combined with a valgus extension proximal femoral oste-
otomy. Type 3 is similar to Gillespie’s group C. If knee motion
is <45 degrees, functional gains with lengthening are doubtful.
An unusual variant of PFFD is that seen with a bifur-
cated distal femur. On radiographs, the femur has the shape
of an inverted “Y” (165). In addition, these patients always
have complete absence of the tibia and often exhibit hand
