Osteogenesis imperfecta with oblique fracture of femur

Case contributed by Kevan English
Diagnosis certain

Presentation

Left thigh pain secondary to an accident during water therapy in a pool. Medical history include osteogenesis imperfecta.

Patient Data

Age: 11 years
Gender: Male
Frontal
Lateral
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Info

There is diffuse osteopenia.

Oblique fracture of the left femoral diaphysis with 5.7 cm overlap between fragments with lateral and anterior apex angulation.

Lower femoral transverse sclerotic lines (zebra stripe lines).

The knee joint appears intact.

Soft tissue swelling at the left thigh.

Case Discussion

This represents a distal femoral fracture. The patient underwent left femur open reduction internal fixation and retrograde intramedullary fixation with flexible rods. Orthopaedic follow-up was subsequently required.

The case shows typical radiological features of osteogenesis imperfecta with severe osteopenia of the examined bones, zebra stripe lines, and pathological fracture.

The zebra stripe sign occurs when children with osteogenesis imperfecta have been treated with cyclical bisphosphonate therapy. When administered cyclically, the drug induces dense bone formation, resulting in radiographically visible dense stripes across the metaphyses.

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