Abstract
The surgical treatment of fibrous dysplasia remains a challenge for the pediatric orthopedist because of its high recurrence rate. Although a few successful treatments have been reported by using en bloc resection and bone graft in adults, this has not been reproduced in children. In this report, the authors present two children (2.5 and 6 years old) with monostotic expansile fibrous dysplasia involving the ulna and fibula, respectively, who underwent en bloc resection and autograft to replace the involved bones. Good bone union and functional recovery were obtained postoperatively. However, during a follow-up period of 8 and 5 years, respectively, the lesions recurred completely, and the deformities remained progressing over time. En bloc resection and bone graft cannot prevent recurrence in skeletally immature patients with monostotic expansile fibrous dysplasia, and cannot alter for the natural history of the disease. A combination of other management should be considered in children with fibrous dysplasia.
Highlights
Fibrous dysplasia is an uncommon benign disorder of the bone, characterized by the progressive replacement of normal bone by fibrous tissue and dysplastic woven bone
71.9% to 86% of fibrous dysplasia is associated with activating mutations of the GNAS gene [1,2], which codes for the alpha subunit of the signaling G protein (Gsα)
We present two children with monostotic expansile fibrous dysplasia involving the ulna and fibula, respectively, where en bloc resection and autograft were performed to replace the involved bone
Summary
Fibrous dysplasia is an uncommon benign disorder of the bone, characterized by the progressive replacement of normal bone by fibrous tissue and dysplastic woven bone. 71.9% to 86% of fibrous dysplasia is associated with activating mutations of the GNAS gene [1,2], which codes for the alpha subunit of the signaling G protein (Gsα). Fibrous dysplasia is not inherited as the mutation occurs in the postzygotic somatocyte [3]. The range of involvement may vary from one bone (monostotic) to multiple bones (polyostotic), and it may present in association with café-au-lait skin pigmentation and endocrine disorders (McCune-Albright syndrome) [4,5] or intramuscular myxoma (Mazabraud’s syndrome) [6]. Fibrous dysplasia represents approximately 5 to 7% of benign bone tumors [7].
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