Fact-sheet: Melorheostosis
Updated on 10/06/2016 at 9:12 AM
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Definition
Osteodysplasia with predominant or exclusive involvement of membranous ossification: exuberant periosteal bone formation predominantly affecting the diaphysis.
Clinical features
Asymptomatic or may present with pain, swelling, limitation of joint mobility, and, in severe forms, limb-length discrepancy and/or bone deformity.
Overlying soft tissue abnormalities, often preceding the bony changes, may be associated (fibrosis and subcutaneous atrophy, pigmentation abnormalities, periarticular masses).
Melorheostosis may affect a single bone (monostotic form), a single limb (monomelic form), or multiple bones (polyostotic form).
It preferentially involves the appendicular skeleton and rarely the axial skeleton.
Radiography
Wavy bone proliferation resembling "flowing candle wax," running parallel to the long axis of the bone, along one or more bones.
Affects only one cortical surface of the bone.
Later on, endosteal hyperostosis may be associated, narrowing or obliterating the medullary canal.
Affects long bones.
MRI
Hypointense flow-like lesion on all sequences.
Inconsistent presence of contrast enhancement immediately adjacent to the lesion or of focal T2 hyperintensity of the bone marrow.
MRI is useful when surgery is being considered.
Nuclear medicine
Scintigraphy: intense uptake along the bony lesion
Management
Primarily symptomatic treatment