· disturbance of ossification of many epiphyses
· one of more common dysplasias
· heterogenous with spectrum of involvement
· mild form (Ribbing)
· severe form (Fairbank)
· autosomal dominant
· disturbance of enchondral ossification in epiphyseal and physeal region
· deficient proliferative region
· tongue-like projections of cartilage into metaphysis
· varies in severity
· may present with
· problems walking in childhood
· premature osteoarthritis in adulthood
· most commonly affects
· hips
· knees
· ankles
· commonly see
· prominent joints
· angular deformities
· flexion contractures
· digits shortened
· mild shortness of stature
· joint involvement symmetrical
· epiphyseal ossification delayed
· epiphyses appear fragmented
· joint surfaces flattened and irregular
· leads to premature osteoarthritis
· most frequently and severely involved
· small irregular and fragmented capital epiphyses
· progressive flattening and extrusion of femoral head
· premature osteoarthritis
· may be superimposed osteonecrosis of femoral head
· often confused with bilateral Perthes’ disease
· femoral condyles flattened and rectangular
· genu valgum common
· valgus intertrochanteric osteotomy for hip
· supracondylar osteotomy for knee
· arthroplasty of hip or knee