Campanacci disease/Osteofibrous dysplasia

Osteofibrous dysplasia/ossifying fibroma of long bones/Campanacci disease:

– a rare lesion usually affecting the tibia and fibula.
-most of the pt. in the first 2 decades of life.
– the most frequently affected site: the middle 3rd of the tibia
– the lesion usually diaphyseal, but may encroach on the metaphysis.

Clinical Features:
-Usu. asymptomatic
-Painless swelling
– enlarged tibia with anterolateral bowing
-if pathological fracture →Pain
-Pseudoarthrosis in some pt.

Radiological Features:
-intracortical osteolysis
-expansion of the cortex
D/DAdamantinoma, Monostotic fibrous dysplasia.

Histopathology: (similar to ‘Fibrous dysplasia’ except Osteoblastic rimming present)
-zonal architecture with loose fibrous tissue in the center
-a band of bony trabeculae rimmed by active osteoblasts at the periphery (Osteoblastic rimming absent in “Fibrous dysplasia”)

Natural course of the lesion– unpredictable.
-Some regress spontaneously during childhood;
-most progress during childhood, but not after puberty.
1. Conservative
OBSERVATION: -as many cases regress spontaneously
-to follow any development of deformity
USE of BRACING: – to correct deformity

  1. Surgery* -prevention or correction of deformity with osteotomy (rarely if deformity significant)
    -usually after skeletal maturity

*Recurrence rates- high after curettage or marginal resection in children.
*Recurrence rates- low after surgery in skeletally mature patients.
*Pathological fractures can be treated nonoperatively.


Also read- Osteofibrous dysplasia of the tibia, IS THERE A NEED FOR A RADICAL SURGICAL APPROACH?
R. S. Lee, S. Weitzel, D. M. Eastwood, F. Monsell, J. Pringle, S. R. Cannon, T. W. R. Briggs
DOI: 10.1302/0301-620X.88B5.17358 Published 27 April 2006)