FIBROUS DYSPLASIA

 

Definition:

Fibrous dysplasia is a developmental disorder where areas of normal 

bone and marrow are replaced by cellular fibrous tissue and flecks of 

osteiod and woven bone.

 

 Type:—a. Monostotic, b. Polyostotoc.     (Monomalic)

 

  Clinical Feature: 

Small , Single lesion –asymptomatic

Large lesion-Pain, Limp, 

Bony enlargement, 

Deformity ,     

Pathological fracture 

Common Age- Childhood and adolescence.

Common Site- Proximal femur, tibia, humerus, ribs, Cranio-facial bone. 

 

Albright Syndrome:

      Fibrous dysplasia when associated with Café-au lait skin patch and (in girl)

       precocious puberty.

 

    Mazabraud Syndrome:

    When fibrous dysplasia associated with skin pigmentation and myxoma.

 

X-ray:

            Radiolucent Cystic area at metaphysis or diaphysis of long bone.

 

                Slightly hazy or ground glass appearance.

 

                Deformity in weight bearing bone (Shepherd’s Crock Deformity in femur).

 

Bone scan: Increase activity.

 

Pathology:  

                Tissue has course and gritty feel.

                Loose cellular fibrous tissue with wide spread.

                Patch of woven bone and scattered giant cells.  

 

Treatment:  

           Small lesion – Require no treatment.

           Large lesion, painful, impending fracture- Curettage and bone  graft.(Mixed Graft)

           Very large lesion-  Curettage and cemented graft. (Bone graft substitute)

Deformity- Corrective Osteotomy.

 

  Malignant Changes-  

         Monostotic- 0.5%

        Polyostotic -5%.