Melorheostosis
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Posted by braker19@comcast.net, 8/28/03 at 9:36:39 AM.
"...a rare
bone disorder (of unknown etiology) whose initial manifestations
include swelling of joints, pain, and limitation of motion. Eventually
profound muscle contractures, tendon and ligament shortening, and soft
tissue involvement with severe growth disturbances may ensue.
Scoliosis, joint contracture and foot deformities may be seen." Encyclopaedia of Medical Imaging Volume III:1
Melorheostosis usually presents in a sclerotomal distribution.
Early
clinical findings include joint swelling, limitation of motion and
pain. Contractures, ligamentous and tendinous shortening and even soft
tissue involvement may ensue.(1)
Radiographic
findings are often limited to a single limb where more than one bone is
often involved. The lower extremity is more often affected than
the upper extremity. The axial skeleton is often spared.
The classic finding is that of "peripherally
located (cortical) hyperostosis...in one bone or a series of
bones. The appearance of the osseous excrescences extending along
the length of the bone simulates that of candle wax flowing down the
side of the lit candle" Resnick: Bone and Joint Imaging, Second Edition. W.B Saunders Company 1996.
The appearance of the lesion(s) gave the anomaly its name, which is "taken
from the Greek words for member (melos) and flow (rhein). There is
usually a distinct demarcation between the affected and normal bone.
Dense, sclerotic linear areas are seen mainly in the cortex but also
extending into the cancellous bone. It may co-exist with
osteopoikilosis and osteopathia striata as well as with tumours or
malformations of blood vessels or lymphatics. Soft-tissue ossifications
at the site of the joint are common." (2)
Other Modalities...
Scintigraphy
is positive and shows moderately increased
uptake. Characterization can be accomplished with advanced
cross-sectional imaging with Computed Tomography and MRI, but these
rarely add value.
The forme
fruste of melorheostosis can mimic other pathology such as
osteoma, myositis ossificans and parosteal osteosarcoma.
Treatment includes surgical resection of soft tissue associated lesions and in extreme cases amputation may be necessary.
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