FIBROUS DYSPLASIA (FD)

FIBROUS DYSPLASIA (FD)

By : Dwi Damar AndriyaniConsultant : dr. Edy Moeljono, Sp.Rad (K)RA

TEXT BOOK READING DAVID SUTTON VOL.2PAGE 1130-1140

Sutton page 1130-1140

Congenital skeletal anomalies Skeletal dysplasia

1. Cleidocranial dysplasia2. Picnodisostosis3. Acroosteolisis4. Osteogenesis imperfecta5. Fibrogenesis imperfecta6. Osteopetrosis7. ........8. Fibrous dysplasia9. .......40. Achondrogenesis

Chromosomal disorder

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

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•Homogenousground glass appearance

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

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

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.

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.

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.

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.

Resume

Fibrous dysplasia1. Aetiology & Patology2. 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

QUIZ

1. Please describe this pictures!

2. What is the most likely diagnosis for this pictures?

Fig. 35.47, PAGE 1131

TERIMAKASIH