Management and Treatment of Osteofibrous Dysplasia
Primary Treatment Approach
For symptomatic osteofibrous dysplasia with pain, pathological fracture, or significant deformity, surgical intervention with curettage, allograft, and elastic stable intramedullary nailing (ESIN) provides the best balance of low recurrence rates and acceptable complication profiles. 1
Initial Diagnostic Confirmation
Open biopsy is mandatory in all cases with imaging suggestive of osteofibrous dysplasia to definitively exclude adamantinoma and other bone tumors or infection before determining management strategy. 2, 1 The osteofibrous dysplasia-like subtype of adamantinoma is low grade but will recur if not completely resected, while other adamantinoma subtypes have substantial metastatic potential 2.
Treatment Algorithm Based on Clinical Presentation
Asymptomatic Patients
- Observation alone is appropriate after open biopsy confirms the diagnosis 1
- Regular clinical and radiographic follow-up to monitor for symptom development or lesion progression 1
Symptomatic Patients with Limited Lesions
- Curettage with allograft and ESIN fixation is the preferred approach 1
- This method achieves:
Large Circumferential Lesions or Recurrent Disease
- Extra-periosteal resection with bone transport is indicated 1
- This approach provides:
- Important caveat: Consolidation time is significantly longer (6.8 months) and complication rates are higher compared to ESIN fixation 1
- Alternative option: Extra-periosteal excision with autologous free fibular graft combined with bone graft substitute (hydroxyapatite crystals) reduces donor site morbidity while achieving good graft incorporation 3
Surgical Timing and Indications
Surgery should be performed proactively in symptomatic patients rather than waiting for progression 1. Specific indications include:
- Persistent pain despite conservative measures 1
- Pathological fracture 1
- Significant tibial deformity 1
- Lesions at risk for impending fracture 3
What NOT to Do
Avoid curettage and allograft alone without ESIN fixation, as this approach results in:
- High recurrence rate of 66.7% 1
- Increased deformity risk of 50% 1
- Higher pathological fracture incidence of 16.7% 1
Complete resection with wide margins is necessary if adamantinoma cannot be definitively excluded, as incomplete resection of adamantinoma leads to recurrence and potential metastasis 2.
Follow-Up Protocol
- Regular clinical examination of the affected site 1
- Serial radiographic imaging to assess bone consolidation 1
- Monitor for complications including recurrence, deformity, and pathological fracture 1
Key Clinical Pearls
The management strategy must be tailored to lesion extent and location, with ESIN providing optimal outcomes for most symptomatic cases while reserving more aggressive resection techniques for extensive or recurrent disease 1. The higher complication rate with bone transport procedures must be weighed against their superior recurrence prevention 1.