Fibrous Dysplasia & other fibrous lesions
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FIBROUS LESIONS OF BONE SPECIALLY FIBROUS DYSPLASIA AND NON-OSSIFYING FIBROMA Spheread crook deformity and Cafe au lait spots
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Fibrous Dysplasia & other fibrous lesions
- 1. BONE TUMORSFIBROUS DYSPLASIA PRESENTOR- UMESH YADAV PGIMS ,ROHTAK UMY
- 2. UMY
- 3. Benign tumors Bone-forming • Osteoid Osteoma • Bone island Cartilage Lesions • Chondroma • Osteochondroma Fibrous lesions • Non ossifying fibroma • Cortical desmoid • Benign fibrous histocytoma • Fibrous displasia • Osteofibrous dysplasia • Desmoplastic fibroma UMY Cystic Lesions Unicameral bone cyst Aneurismal bone cyst Intraosseous ganglion cyst Epideromoid cyst Fatty tumors Lipoma Vascular tumors Hemangioma
- 4. FIBROUS LESIONS Nonossifying fibroma Cortical desmoid Benign fibrous histiocytoma Fibrous dysplasia Osteofibrous dysplasia UMY
- 5. FIBROUS DYSPLASIA • DYS- “bad” + PLASIS – “formation ( the presence of cells of an abnormal type within a tissue) • replacement of the normal lamellar cancellous bone by abnormal fibrous tissue.
- 6. PATHO-PHYSIOLOGY • Defect in Osteoblast differntiation & maturation. • Replacement of normal bone & marrow by fibrous tissue & small woven spicules of bone weak & prone to expansion • Females > Males • Age group- 3-15 yrs (Initial Manifestation) • GNAS1 Mutation
- 7. TYPES
- 8. Natural history • Polystotic FD is more aggressive than monostotic FD. • The lesions usually progress in number till the end of skeletal maturation, by then they become quiescent • In only about 5% of cases it continue to enlarge after that.
- 9. HOW IT LOOKS ON XRAY • Geographical lytic lesions in medullary canal (diaphysis or metaphysis) • May have cortical thinning with expansile lesion. • Highly lytic lesions or a ground glass appearance
- 10. Well defined margin of sclerotic bone is common
- 11. UMY
- 13. Shepherd's crook deformity • Coxa varus angulation of the proximal femur, classically seen in femoral involvement by fibrous dysplasia, although may be seen in other disorders such as Paget disese of bone and osteogenesis imperfecta. • The shape of the proximal femur resembles that of the staff carried by herders (shepherds), which is known as a crook. UMY
- 15. Associated conditions Mc Cune Albright syndrome • Almost exclusively affect females
- 18. Cherubism Familial fibrous dysplasia of the jaws.
- 19. TREATMENT - Monostotic lesions - Asymptomatic Lesion------- OBSERVATION • - Bisphosphonate therapy Indications – Symptomatic polyostotic fibrous dysplasia Effective in decreasing pain and reducing bone turnover UMY
- 20. OPERATIVE Indications : Severe deformity Persistent pain Pathological # • INTERNAL FIXATION & BONE GRAFTING ( NEVER AUTOGRAFT ?????????) COXA VARA - OSTEOTOMY
- 21. NON OSSIFYING FIBROMA Non-ossifying Fibroma (NOF) is a benign fibrogenic lesion that is the most common benign bone tumor in childhood Also called Fibrous cortical defect Metaphyseal cortical defect • Symptoms • Asymptomatic and found incidentally • Painless • May present with pathologic fracture UMY
- 22. Radiographs • Diagnostic • Metaphyseal eccentric "bubbly" lytic lesion surrounded by sclerotic rim • Cortex may be expanded and thin • Treatment : Observation
- 23. UMY