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

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 lines).

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. Orthopedic follow-up was subsequently required.

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

The zebra stripe sign occurs when children with osteogenesis imperfecta have been treated with cyclical bisphosphonate therapy. When the drug is delivered in cycles, dense bone is formed while treatment is being given. This results in dense stripes across the metaphyses of bones which can be visualized radiographically. 

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.