| 607278 | ||||||||||||
| OSTEOFIBROUS DYSPLASIA | ||||||||||||
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| Osteofibrous dysplasia is a tumor-like bone lesion that usually presents as a painless swelling or anterior bowing of the tibia (Park et al., 1993), although pain may occur in up to 25% of cases and presentation may follow pathologic fracture. Most reports of osteofibrous dysplasia describe isolated tibial lesions, although a significant subgroup describe isolated and ipsilateral fibular involvement. Cases with ulnar and radial involvement have been reported. Hunter and Jarvis (2002) could find only 2 cases in which tibial involvement was reported as bilateral. They described 2 brothers, 1 with bilateral and 1 with probable bilateral tibial involvement, and a girl from an unrelated family with bilateral tibial involvement. In 1 of the brothers, onset was at the age of 6 years, when swelling over the right leg was noted; in the other brother, a 'small bump' over the right anterior tibia was noted at the age of 4 years. The girl had presentation at the age of 8 years with bilateral shin pain and a bumpy prominence over the anterior aspect of the midshaft of both tibias. Hunter and Jarvis (2002) noted that there may be a relationship between osteofibrous dysplasia and adamantinoma of long bones (102660), although the latter condition usually presents at a later age. | ||||||||||||
| History | ||||||||||||
| Frangenheim (1921) is credited with the first description of this disorder, which has gone by a variety of names, with osteofibrous dysplasia (OFD), suggested by Campanacci (1976), the most frequently used term. The abbreviation OFD has the disadvantage of confusion with the several orofaciodigital syndromes, e.g., OFD1 (311200). | ||||||||||||
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