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Osteofibrous Dysplasia Treatment & Management

  • Author: Darin Davidson, MD; Chief Editor: Harris Gellman, MD  more...
 
Updated: Mar 11, 2016
 

Medical Therapy

Nonoperative treatment usually is recommended until skeletal maturity is reached. 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 to 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[56] 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.[57, 58] 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, 59] 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%. Goergen et al reported multiple recurrences in a 3-year-old boy and a 6-month-old boy after attempts at resection.[60] 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.[61] 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.[62, 63, 64] However, the case reported by Ben Arush et al is the only report of sarcomatous degeneration of osteofibrous dysplasia.

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Contributor Information and Disclosures
Author

Darin Davidson, MD Resident Physician, Department of Orthopedics, University of British Columbia Faculty of Medicine, Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Sean P Scully, MD 

Sean P Scully, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, International Society on Thrombosis and Haemostasis, Society of Surgical Oncology

Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami, Leonard M Miller School of Medicine, Clinical Professor, Surgery, Nova Southeastern School of Medicine

Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, Arkansas Medical Society

Disclosure: Nothing to disclose.

Acknowledgements

Robert Mervyn Letts, MD, FRCS(C), FACS Former Chief, Department of Surgery, Division of Pediatric Orthopedics, Children's Hospital of Eastern Ontario, University of Ottawa; Consultant Pediatric Orthopedic Surgeon, Sheikh Khalifa Medical City, UAE

Disclosure: Nothing to disclose.

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Radiograph of osteofibrous dysplasia of tibia in 5-year-old girl.
Characteristic radiographic findings of osteofibrous dysplasia. Note eccentric intracortical lesion with sclerosis of internal surface, bubbled appearance of lesion, and anterior tibial bowing.
Typical histologic appearance of osteofibrous dysplasia lesion (×100). Note zonal architecture with periphery of active osteoblasts surrounding bone trabeculae.
Histologic section (×100) demonstrating vascular channels within osteofibrous dysplasia lesion, which has been proposed as etiologic factor in development.
Histologic appearance of fibrous dysplasia, revealing appearance similar to osteofibrous dysplasia but lacking periphery of active osteoblasts.
 
 
 
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