The Journal of Bone and Joint Surgery (American). 2005;87:2297-2307.
doi:10.2106/JBJS.D.02575
© 2005 The Journal of Bone and Joint Surgery, Inc.
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Familial Osteofibrous Dysplasia

A Case Series

Lori A. Karol, MD1, David S. Brown, MD2, Carol A. Wise, PhD1 and Michael Waldron, MD1

1 Departments of Orthopaedic Surgery (L.A.K.) and Pathology (M.W.) and the Sarah M. and Charles E. Seay Center for Musculoskeletal Research (C.A.W.), Texas Scottish Rite Hospital for Children, 2222 Welborn, Dallas, TX 75219. E-mail address for L.A. Karol: lori.karol@tsrh.org
2 Department of Orthopaedics, Cook Children's Hospital, 801 Seventh Avenue, Fort Worth, TX 76104

Investigation performed at the Texas Scottish Rite Hospital for Children, Dallas, Texas

The first 150 words of the full text of this article appear below.


   Introduction
 
Osteofibrous dysplasia is a benign bone dysplasia in which lytic lesions occur in the cortex of the tibia and/or fibula in skeletally immature patients. Campanacci proposed the term "osteofibrous dysplasia" and described the clinical and pathologic findings1,2. The dysplasia may be clinically silent, so the true incidence of the disease is unknown. Symptomatic children present with anterolateral bowing of the affected bones or with pathologic fractures. As a result of the site of involvement and the propensity for pathologic fractures, this condition may be confused with congenital pseudarthrosis of the tibia.

Pathologic specimens from sites affected by osteofibrous dysplasia show bland fibroblastic tissue associated with immature woven bone rimmed by osteoblasts3-5. While there has been a questionable association with evolution into adamantinoma, the pathologic appearance of osteofibrous dysplasia does not include neoplastic features.

Osteofibrous dysplasia was not thought to be an inherited condition until a recent report, . . . [Full Text of this Article]


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