Paediatric ImagingEnchondromatosis, multiple
(also called Ollier's disease), multiple enchondromas, i.e. disorganized cartilaginous foci within the bones which causes bony swellings of the affected bones. In the long bones, streaky linear depositions of
cartilage within the metaphysis may also occur. The disease often dominantly affects one side of the body. On other occasions it may affect the hands or feet, or be generalized. It is not an inherited disease. Clinically, there are bony swellings, limb shortening and mechanical difficulties, associated with joint disruption and short stature, which may be asymmetrical. The condition usually presents between 2 and 10 years of age. There may be
focal restriction of growth at the site of a
lesion which may result in limb shortening, Madelung's deformity or kyphosis.
The characteristic radiological appearances are columnar radiolucencies extending from the metaphyses into the shafts of tubular bones; there may be associated stippled calcification (Fig.1). Similar bands of calcification may radiate into the iliac crest. Enchondromas may affect the spinal appendages and cause kyphoscoliosis. In the ribs, enchondromas may cause bony swellings, though this is rare. In the pelvis, the iliac blade is affected rather than the pubic symphysis. In the long bones, streaky or bulbous lesions may occur. These typically occur in the metaphyses and can lead to secondary deformity of the growth plates. A secondary Madelung deformity may occur at the wrists. There is a small risk of malignant transformation to chondrosarcoma, particularly in flat bones, in adult life. See Maffuccis syndrome, enchondroma solitary, Madelungs deformity and metachondromatosis
HC
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Radiograph of the lower limbs in an affected six-year-old child. There are multiple streaky radiolucencies involving the tibial and fibular shafts proximally and distally with irregular punctate calcification in some of the lesions. There is growth deformity and leg length discrepancy.
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Enchondromatosis, multiple, Fig.1 | |