Fig 3. Severe bowing deformity of the distal femur and proximal tibia, bilaterally.
The pencil-thin cortieces and overall osteorosis are consistent with osteogenesis
imperfecta There is an intramedullary rod in right tibia
Fig 4, Severe bowing deformity affecting the entire pelvis and proximal femora.
There is bilate protrusio acetabulae. Gross osteoporosis is identified.
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is assciated with high rate of stillbirths and infant morality. The tarda from has a later onset manifestations, which include fractures , detinogenesis imperfacta, and blue sclera. They may or may not have bowingdeformitiles. Bone fragility commonly decreases after pulberty but dose not completely disappear. However, subdividing patients with osteogenesis imperfecta into congenita tarda frms fails to accommodate the wide variation in expression of this condition. More recently, osteogenesis impfecta has ben clasified into 4 types by Sillence et al. Table 1 outlines these classifications. Although type 1 and do not readily fit into any the categories. The primary finding is one of diffusr osteopenia with pencil-thin cortices. The degree to which the bone density isdecreased is ptoporional to the severity of the condition, andin mild cases may appear completely normal. The bones most commonly appear then and gravile; however a short and thin or cystic apperance may be evident. |
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