Weinstein Lovell and Winters Pediatric Orthopaedics 7e

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CHAPTER 30  |  The Child with a Limb Deficiency

FIGURE 30-35. A : Preoperative clinical photograph of a patient with Gillespie type 2 PFFD. B : An intraoperative fluoroscopic radiograph demonstrating the extreme shortening of the femoral segment and lack of any acetabular development. C : Post- operative clinical photograph after Van Nes rotationplasty. D : Postoperatie standing clinical photograph at 1 year of follow-up.

arthrodesis and Syme amputation. In the report of Friscia et al. (177), one patient subsequently had a Syme amputa- tion at the parents’ request. Two recent studies evaluating the quality of life in patients who had rotationplasty for sarcoma ­treatment ­demonstrated that although physical function was less than that in healthy peers, psychosocial adaptation and life contentment were about the same (179, 180). This emphasizes the importance of proper presurgical preparation of the ­parents and of the patient, if she or he is old enough. This is best ­accomplished by seeing other patients with a rota- tionplasty, along with the use of videos of patients, teaching dolls, and so on.

It is imperative that the ankle must be sufficiently normal to serve as a knee. This is particularly important to determine, because up to 70% of children with PFFD will also have a fibular deficiency on the same side. Although some valgus alignment of the foot and ankle can be compensated for in the prosthetic alignment, the deformity may progress with age. Severe valgus and equinus deformities, with a deficient foot, are contraindications to the procedure. Additional preoperative preparation includes toe, ankle, and hindfoot strengthening, in particular, because these are the structures that will power the new knee joint. Equinus position should be emphasized, because this will place the