Abstract
Fibrocartilaginous dysplasia (FCD) is a rare variant of fibrous dysplasia (FD) which frequently involves the long bones, and the proximal femur is the most commonly affected site. This benign, lytic, and expansile bone lesion causes progressive deformity in the bones and may lead to pathological fracture. Radiologically, this lesion may mimic cartilaginous benign and malignant bone tumors. Therefore, histopathological differentiation of FCD from other cartilaginous tumors is of the utmost importance. The treatment is often surgical, in the form of curettage and bone grafting or corrective osteotomy, to treat progressive deformity in the long bones. The risk of pathological fracture is high in FCD with bony deformity and often requires surgery.
Highlights
Fibrous dysplasia (FD) is a benign fibro-osseous lesion that occurs due to the developmental anomaly of the bone [1]
The risk of pathological fracture is high in Fibrocartilaginous dysplasia (FCD) with bony deformity and often requires surgery
The fibrous dysplasia (FD) has a predilection for long bones, skull, ribs, and jaw [3]
Summary
Fibrous dysplasia (FD) is a benign fibro-osseous lesion that occurs due to the developmental anomaly of the bone [1]. A fibrocartilaginous dysplasia (FCD) commonly occurs in the lower extremities, especially in the proximal femur, leading to disabling deformity of the limb [6] Awareness about this rare variant of FD is necessary to reach an accurate diagnosis. Anteroposterior (AP) and lateral view radiographs of the hip and proximal thigh were done (Figure 1), which revealed a large, well-defined, expansile lytic lesion in the metaphysis of the proximal femur extending up to the subtrochanteric region of the femur. The neck shaft angle was reduced to 84 degrees, resulting in a severe coxa vara deformity, along with anterolateral bowing of the proximal femur resembling the "shepherd's crook deformity" of FD [8] Considering his current problems, it was decided to do extensive curettage, bone grafting, and corrective osteotomy with internal fixation. At the end of one year, the patient was doing well, and the shortening and limp have improved significantly with the radiological union of the osteotomy; there have been no signs of recurrence of the tumor or deformity (Figure 5)
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