Weinstein Lovell and Winters Pediatric Orthopaedics 7e

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CHAPTER 30  |  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