this slide show gives you an overview of pathogenesis and treatment option in patients with FD

1. Fibrous Dysplasia
Pathogenesis and Treatment
Dr. Sunil Mann
PG3 Ortho

2. Introduction
• Benign intramedullary fibro-osseous lesion
• 5-7% of all tumors
• Can involve one bone- Monostotic or multiple
bones- polyostotic
• Common sites of occurrence: Long bones like
proximal femur, pelvis, ribs
• No gender predilection

3. Patho-physiology
1. Failure in remodeling of
primitive bone mature bone
2. Failure of bone to re-align trabeculae in
response to mechanical stress
Due to this failure in maturation there will be

4. Immature isolated trabeculae enmeshed in
dysplastic fibrous tissue
Which turns over constantly but never complete
the remodeling process

5. Combination of lack of stress alignment and
insufficient mineralization
leads to
Loss of mechanical strength, development of
pain, deformity and pathologic fracture

6. Types of presentations
• Monostotic: MC, lesion enlarges in proportion
to skeletal growth
• Polyostotic: LC, lesion cont to enlarge after
skeletal maturity, progressive deformity
• McCune Albright Syndrome: Bone lesions,
Endocrine D/O, Skin lesion(café-au-lait spots)
• Mazabraud syndrome: Skeletal lesion+
Intramuscular Myxomas

7. Management- Ix
• X-ray
• CT (Best)
• MRI

9. Treatment
1-- Observation and Patient education
• Asymptomatic and incidental finding on X-
RAY/CT
• Clinical observation and follow with x-ray for 6
months to see for progression of lesion
• Bone scan to rule out Polyostotic FD
• If present refer to endocrinologist

10. 2. Pharmacotherapy
Bisphosphonates
• Indication: Symptomatic polyostotic FD
• MOA:
Anti resorptive effects (osteoclast apoptosis)
Potent inhibitor of bone resorption
Lasting impact on bone turnover

11. • Drugs: Alendronate oral 10mg daily or 70 mg/wk
Pamidronate IV 180mg every 6 mo.
Etidronate oral/IV wt based daily (not>6months)
• Major Effects
Clinically- bone pains,
Radiologically- thickening of cortices, ossification of
radiolucent areas,
Bichemically- Sr ALP and other bone remodelling
parameters

12. • Side effects
Transient mineralization defect
Osteonecrosis of Jaw

13. Other pharma agents
• Denosumab: RANKL antibody (inhibit
osteoclast )
• Anti IL6 (Tocilizumab) and Anti FGF 23 are
under trial

14. 3. Surgery
AIM
• Open Biopsy (in non classic presentation)
• For correction of deformity
• Prevention and treatment of pathologic
fracture
• Eradication of symptomatic lesion

15. Procedures
• Curettage and Bone grafting
• Internal fixation
• Excision
• Amutation