MIR Teaching file case bs109

Case Author(s): David A. Hillier, M.D., Ph.D. and Henry Royal, M.D. , . Rating: #D4, #Q4

Diagnosis: Engelmann's disease (progressive diaphyseal dysplasia)

Thirty-eight-year-old woman with a known history of a skeletal dysplasia.

Bone scintigraphy

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View second image(xr). Plain film of tibia

Full history/Diagnosis is available below

Thirty-eight-year-old woman with a known history of a skeletal dysplasia.

Bone scintigraphy, 21.1 mCi methylene diphosphonate

1. Bone scintigraphy:

- Diffusely increased uptake throughout the skeleton. Hands and feet are relatively spared.

- Focal intense foci in skull, proximal humeri, femora and tibiae.

2. Skull, femur plain films:

- Sclerotic undertubulated bones.

- Sclerotic changes throughout the skull, especially the skull base.

- Lytic areas corresponding to foci of intense uptake on bone scan.

Progressive diaphyseal dysplasia (Engelmann’s disease) is hereditary. The long bones become undertubulized and the medullary cavity becomes narrowed.

This appearance, with diffusely increased uptake throughout the skeleton with undertubulation of the bones is characteristic of Engelmann’s disease.

References and General Discussion of Bone Scintigraphy (Anatomic field:Skeletal System, Category:Misc)

Case number: bs109