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Osteofibrous Dysplasia

Benign fibro-osseous lesion of the tibia and fibula in children, often confused with adamantinoma.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

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What is Osteofibrous Dysplasia?

Osteofibrous dysplasia (OFD), or Campanacci disease, is a benign self-limiting fibro-osseous lesion that almost exclusively involves the diaphysis of the tibia and occasionally the fibula in children younger than 15 years.

Histology shows woven bone trabeculae rimmed by osteoblasts within a fibrous stroma; cytokeratin-positive cells suggest a relationship with adamantinoma, the principal differential.

Clinical presentation includes painless tibial bowing or pathologic fracture; treatment is generally observation in skeletally immature patients with curettage, bracing or osteotomy reserved for progressive deformity, while biopsy and definitive resection are pursued when adamantinoma cannot be excluded.

Symptoms

Painless anterior bowing of tibia
Localized cortical thickening on radiographs
Ground-glass intracortical lesions
Pathologic fracture in some cases
Limb-length discrepancy
Slow progression in childhood
Often diagnosed incidentally on imaging

Risk Factors

Pediatric age below 15 years
Tibial diaphyseal location
Family history rare
Genetic association with adamantinoma in differential
No known modifiable risk factors
Incidentally discovered on imaging
Misdiagnosis as fibrous dysplasia or adamantinoma

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Anterior tibial bowing in a child
  • Pathologic fracture without trauma
  • Persistent leg pain in young patient
  • Radiographic ground-glass cortical lesion
  • Suspicion of adamantinoma
  • Need for biopsy and oncology consultation
  • Progressive deformity requiring surgical correction

Treatment Methods

01
Observation with serial radiographs in skeletally immature patients
02
Bracing for severe deformity
03
Curettage and bone grafting for symptomatic lesions
04
Tibial osteotomy for progressive bowing
05
Biopsy when adamantinoma cannot be excluded
06
En bloc resection if adamantinoma confirmed
07
Long-term follow-up to monitor recurrence

Which Department to Visit?

You can visit our Ortopedi ve Travmatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.