Melorheostosis

Case contributed by Craig Hacking
Diagnosis certain

Presentation

Fall on outstretched hand

Patient Data

Age: 35 years
Gender: Male
x-ray

A well defined 17.5 mm oval region of sclerosis lies within the proximal end of the left second metacarpal. It extends to the cortical surface and there is associated smooth, benign-type periosteal reaction extending along the radial aspect of the second metacarpal over a distance of several centimeters. No associated bony erosion, fracture, soft tissue mass or soft tissue calcification.

Although indeterminate, the appearances favors a benign etiology. Given the clinical history however an old fracture cannot be completely excluded. In the first instance, correlation with any prior imaging is recommended.

Elsewhere, no evidence of recent fracture or dislocation.

ct

No scaphoid fracture identified. Scapholunate interval is normal.

Incidental eccentric densely sclerotic lesion contiguous with the cortex of the base and proximal shaft of the second metacarpal with well-defined margins. There are similarly indolent appearances on the radiograph. Features are in keeping with melorheostosis.

Case Discussion

Melorheostosis is an uncommon mesenchymal dysplasia. It causes sclerotic lesions with a characteristic dripping wax appearance or flowing candle wax appearance.

It can be either monostotic or polyostotic  and tends to be monomelic, usually in the long bones of the limbs. Involvement of the axial skeleton is rare.

The condition has a tendency to affect a sclerotomal distribution.

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