Introduction: Osteofibrous Dysplasia is a rare benign self-limiting fibro-osseous lesion most commonly seen in the diaphysis of the tibia. Its incidence is reported to be 0.2% of all primary bone tumors. It occurs in the first two decades of life with a slight male preponderance. Surgical options include extra periosteal resection, autologous graft, limb lengthening procedures etc. There are no case reports mentioning the use of synthetic bone graft to fill the defect following extraperiosteal excision.
Case Report: A 13 year old girl presented with pain and swelling of the (R) leg since 2 months following a trivial injury at school. Examination revealed a 5×3cm tender swelling on the anteromedial aspect of the middle third tibia. Radiographs and MRI, revealed an eccentric expansile lytic lesion, which was multilocular and was present at the junction of the metaphysis and diaphysis on the antero -medial aspect of tibia. The cortex had ballooned out and there was a possibility of an impending fracture. Biopsy was done which revealed osteofibrous dysplasia. We did an extraperiosteal excision of the lesion. To fill the cavity we harvested 10 cm of the contralateral fibula and since there was still space in the cavity, we packed bone graft substitute (hydroxyapatite crystals) into the defect. The surgical management of osteofibrous dysplasia is controversial. Various methods of treatment of such cases have been described in literature. The use of synthetic graft is an option in these patients as it reduces morbidity; and in our case we had good graft incorporation with this method.
Conclusion: Extraperiosteal Excision of Osteofibrous dysplasia combined with autologous free fibular graft and bone graft substitute is a good surgical option to prevent recurrence and mange bone defects in this rare lesion.
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http://dx.doi.org/10.13107/jocr.2250-0685.252 | DOI Listing |
J Orthop Case Rep
October 2024
Department of Orthopaedic Surgery, Duke University Hospital, Durham, North Carolina, United States.
FASEB J
July 2024
Department of Medical Genetics, University of Alberta, Edmonton, Alberta, Canada.
Osteofibrous dysplasia (OFD) is a rare, benign, fibro-osseous lesion that occurs most commonly in the tibia of children. Tibial involvement leads to bowing and predisposes to the development of a fracture which exhibit significantly delayed healing processes, leading to prolonged morbidity. We previously identified gain-of-function mutations in the MET gene as a cause for OFD.
View Article and Find Full Text PDFJ Pediatr Orthop B
January 2025
Department of Orthopedics, Children's Hospital of Chongqing Medical University.
Osteofibrous dysplasia (OFD) is a rare disease that may lead to tibial lesions. Currently, no gold standard method exists for the treatment of OFD. Recurrence is the most severe complication in OFD.
View Article and Find Full Text PDFJ Hand Microsurg
March 2024
Department of Orthopaedics & Spine Surgery, Ganga Hospital, Coimbatore, Tamil Nadu, India.
Clin Pathol
April 2024
Department of Pathology, Faculty of Medicine, University of Rijeka, Rijeka, Croatia.
Adamantinoma (AD) is a rare, slow-growing primary malignant bone tumor characterized by a biphasic morphology of clusters of epithelial cells and spindle cell osteofibrous components. A strong relationship between AD and osteofibrous dysplasia (OFD) has been proposed, while fibrous dysplasia (FD) has been rarely associated with AD. We present an AD case that was followed and histologically evaluated 3 times over 6 years with different morphological patterns.
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