Osteogenesis imperfecta

Case contributed by Dr Badis Al Harbawi

Presentation

Known case of osteogenesis imperfecta type III with progressive deformities.

Patient Data

Age: 10 years
Gender: Male
X-ray

Marked bowing deformity in the lower limbs, along with severe osteoporosis, gracile bones, protrusio acetabuli and coxa vara.

Non united left femoral shaft fracture is noted with pseudojoint formation (pseudoarthrosis). 

Dense lines in the tibiae and distal femora compatible with zebra stripe sign due to bisphosphonate therapy.

Metaphyseal widening mainly in the distal femora with lucent and sclerotic foci (popcorn calcification).

Case Discussion

This child is a known case of osteogenesis imperfecta type III with progressive deformities despite cyclic treatment with bisphosphonate therapy.

Several radiographic signs of osteogenesis imperfecta were nicely demonstrated in this case including:

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