Osteofibrous Dysplasia Treatment & Management

Updated: Aug 17, 2021
  • Author: Darin Davidson, MD; Chief Editor: Omohodion (Odion) Binitie, MD  more...
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Treatment

Medical Therapy

Nonoperative treatment usually is recommended until skeletal maturity is reached. [56] Recurrent pathologic fractures may be an ongoing problem in some active children. Using a tibial brace similar to those used for congenital pseudarthrosis of the tibia may minimize recurrent pathologic fractures. A lace-up leather support from just below the knee to the ankle may be used. Fractures usually are nondisplaced and can be treated in a walking patellar tendon-bearing cast. Cast immobilization is sufficient for fracture healing, though healing is slower than normal.

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Surgical Therapy

There are no absolute contraindications for surgical intervention in children, with the exception of any underlying medical or anesthetic issues. Operative management is not recommended in patients who are skeletally immature, because of the high recurrence rate after resection and curettage [57] and because of the predisposition to fracturing after the bone has been weakened by biopsy.

Once skeletal maturity has been reached, marginal resection and bone grafting may be performed without increased risk of recurrence. [58, 59] Pathologic fracture does not necessarily require surgical management, because cast immobilization frequently results in good healing.

For patients of any age, surgical correction of associated deformities may be required. Campanacci and Laus recommended wide resection with extensive bone grafting in children who are skeletally immature if the lesion is aggressive, with marked expansion and bone destruction or multiple pathologic fractures. [4, 60] Intramedullary prophylactic rodding of the tibia may also be an option in children who frequently present with fractures; this approach is similar to that used in osteogenesis imperfecta. Resection of large portions of the lesion usually is not necessary and only increases susceptibility to recurrent fractures.

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Complications

The recurrence rate after resection and curettage has been reported to be 64-100%. [61] Goergen et al reported multiple recurrences in a 3-year-old boy and a 6-month-old boy after attempts at resection. [62] Wang et al also reported multiple recurrences after surgical intervention. [9] Campanacci and Laus indicated that recurrence does not develop in patients older than 10 years. [4]

Malignant transformation of the lesion is very rare. Ben Arush et al described the course of a boy diagnosed at age 4 years with osteofibrous dysplasia of the tibia who subsequently presented at age 14 years with synovial sarcoma of the peroneal muscles of the same leg. [63] At the time of the latter diagnosis, computed tomography (CT) confirmed multiple pulmonary metastases.

Malignant transformation to soft-tissue sarcoma has been reported in fibrous dysplasia, most commonly in the polyostotic variation. [64, 65, 66] However, the case reported by Ben Arush et al is the only report of sarcomatous degeneration of osteofibrous dysplasia.

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