半肢骨骺发育不良一例
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Child with pain. Tibial epiphysis. Additional images are shown on upcoming pages.
Trevor’s disease. AKA dysplasia epiphysealis hemimelica (DEH).
Findings: Two rounded/ovoid, corticated calcific densities in the medial ankle. The smaller and anterior
calcification measures 13 x 5 mm and is located in the anterior and medial aspect of the tibiotalar joint. The
calcification is widening/expanding the joint. The larger irregular calcification measures 12 x 14 mm and is
positioned posterior to the medial right tibiotalar joint. The is also an abnormal calcification adjacent to the
anterior process of the talus. CT confirms multiple irregular calcifications within the tibiotalar joint of the
ankle. Larger calcifications are located anterior and posterior to the medial aspect of the distal tibial
epiphysis. There are areas of fusion of the calcifications with the epiphysis.
Differential diagnosis:
•Dysplasia epiphysealis hemimelica (DEH)
•Post-traumatic osseous fragment
•Osteochondromatosis
Diagnosis: Trevor's disease (dysplasia epiphysealis hemimelica) of distal tibial epiphysis.
Key points:
•Trevor's disease or dysplasia epiphysealis hemimelica (DEH) is an uncommon skeletal developmental disorder representing an osteochondroma arising from the epiphysis. Etiology is unclear.
•Reported incidence is 1/1,000,000
•DEH becomes evident in children and young adults. Men:Women 3:1.
•There is no evidence that this is hereditary or cases of malignant transformation.
•Clinical manifestations include swelling and less commonly pain and deformity, loss of normal mechanical function.
•Common sites of involvement include is the distal tibia > distal femur, and is usually restricted to either the medial or lateral side. (medial is more common)
•There may be single or multiple lesions. Two-thirds of patients have multiple lesions.
•In infants and young children, small, multifocal, irregular ossifications are seen adjacent to one side of an ossifying epiphysis. The ossification center becomes confluent with adjacent bone, eventually appearing as a lobulated mass
protruding from the epiphysis.。
