1. FIBROUS DYSPLASIA (FD) By : Dwi Damar Andriyani Consultant : dr. Edy Moeljono, Sp.Rad (K)RA TEXT BOOK READING DAVID SUTTON VOL.2 PAGE 1130-1140

2. Sutton page 1130-1140  Congenital skeletal anomalies  Skeletal dysplasia 1.Cleidocranial dysplasia 2.Picnodisostosis 3.Acroosteolisis 4.Osteogenesis imperfecta 5.Fibrogenesis imperfecta 6.Osteopetrosis 7......... 8.Fibrous dysplasia 9........ 40. Achondrogenesis  Chromosomal disorder

3. Introduction  Aetiology : unknown  Female>male  Bone may be affected –Pelvis –Femur –Skull –Spine/vertebral collapse unusual  Usually found incidentally/ follow pathological fracture  Onset : 10-30 yr old or first decade  Prognosis : worse when lesion occurs early in life

4. Pathology Medullary bone replaced by: Fibrous tissue Cystic containing blood/serous fluid Fibrous tissue undergoes varying degree of abnormal ossification Increase density (dependent on the extent ossification) Patchy Cotton wool appearance Homogenous  ground glass appearance

5. Characteristic Fibrous Dysplasia  Location –Diametaphyseal –Bone end may be involved after fusion –In child may Involve epiphysis  Found in 2 form: 1.Poliostotic  50% skeleton may be involved  Asimetri & distributed unilateral 2.Monostotic  More likely to enlarge in adult life

6. Radiologycal Features  Margin –Smooth dense –Well define –Expanded the bone  Cortex scalloped & thinned but intact –Tend to be multilocular –Expand down the medulla rather than cause cortical expansion  Various degree of central ossification

7. FD in the Proximal Femur. Fig. 35.47 The lesions are well defined, often with a very thick 'rind' around them, and show various degrees of central mineralisation. The site is typical for fibrous dysplasia.

8. Fibrous dysplasia Of the Tibia  Fig. 35.48. A multilocular, partly cystic, expansile lesion  of the midshaft tibia is surrounded by a thick rim of reactive sclerosis.

9. Polycystic case of FD Fig. 35.51.(A) marked enlargement and deformity of the pelvis and proximal femur. There is marked bony expansion with extensive cyst formation. The shepherd's crook deformity has been stabilised. (B) The CT scan shows the mixed pattern of tissues seen in fibrous dysplasia, ranging from cystic through ground-glass to heavily mineralised tissue.

10. Fibrous dysplasia. (A) There is thickening with sclerosis of the frontal bone, the floor of the anterior cranial fossa and the base of the skull extending back to the sphenoid sinus, which is replaced by dense amorphous bone. (B) The CT scan shows expanded and abnormally mineralised bone occupying mainly the left side of the skull base. Considerable facial deformity is present.

11. Resume Fibrous dysplasia 1.Aetiology & Patology 2.Form –Polyostotic –Monostotic 3.Radialogical feature –Location : diametafisial, medulla –Margin  Well define, expansion to the cortex (cortex thin, scalloped, but intact) –Center  Varying degree central mineral ossification

12. QUIZ 1.Please describe this pictures! 2.What is the most likely diagnosis for this pictures? Fig. 35.47, PAGE 1131

13. TERIMAKASIH