FIGURES AND TABLES
FROM:
The challenges of Proteus syndrome: diagnosis and management
Leslie Biesecker
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Figure 1.
The distinction of overgrowth in a patient with PS (a–c) from that of a patient with hemihyperplasia (d, e). Panel a shows an early CCTN. Note that the appearance of this lesion is distinct from that of wrinkling of normal sole tissue. Panels b and c are from a patient with more advanced PS demonstrates several features of the disorder. Panel b shows distorting overgrowth of the first and fifth digits (arrows) with loss of the normal architecture of the digits. The overgrowth in the patient with PS is associated with calcified tissue around the epiphyses that restricts movement at the affected joints. The overgrowth in the patient with hemihyperplasia (d, e) is described by as 'ballooning' overgrowth as it is not associated with distortion.
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Figure 2.
The progressive nature and severity of overgrowth in PS. Panel a shows a teenager with valgus deformity of the knee, fixation of the knee joint due to bony overgrowth surrounding the joint and external rotation of lower leg of more than 90°. Panel b shows the same patient a few years later with continued external rotation of the leg that now exceeds 135° with onset of bowing of the tibia. The surgical correction of such deformities is challenging.
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