Presentation
Long-standing (>20 years) left side lower limb progressive swelling and limping.
Patient Data
Diffuse periosteal and endosteal sclerotic cortical thickening with the characteristic dripping wax appearance, involving the left bony pelvis and entire visualized head, neck and proximal femur with associated “myositis ossificans-like” gross muscle calcifications and extensive muscle atrophy of the left iliopsoas, pelvic and thigh muscles. The constellation of findings is characteristic of polyostotic melorheostosis.
Case Discussion
Melorheostosis is a rare mesenchymal dysplasia manifested by regions of benign sclerosing bone dysplasia. It commonly affects the long bones, though it can affect any bone in the skeletal system. The classic imaging manifestation includes sclerotic periosteal and endosteal thickening reminiscent of flowing candle wax appearance and myositis ossificans-like calcifications, as seen in this case. It also has a known association with muscle atrophy, as seen in this case.
Different radiologic presentations of hyperostosis in melorheostosis include 1
classic dripping candle wax pattern
"osteoma-like” hyperostosis involves only the endosteal surface and shows orientation in the long axis of the bone
osteopathia striata-like hyperostosis, which shows unilateral, long, and dense hyperostotic striations near the inner surface of the cortex in two or more bones
myositis ossificans-like pattern in two or more unilateral regions, with or without intraosseous hyperostosis (as in this case)
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