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Pagetsdiseaseofthebone
- 1. PAGETâS DISEASE OF THE BONE DR.HARI PRASATH P 1ST YEAR POST GRADUATE
- 2. INTRODUCTION The condition was initially described by Dr. James Paget in 1877 ï” Also called as Osteitis Deformans ï” Partial or complete involvement of a single or multiple bones by exaggerated rates of resorptive and osteogenic activity leading to bony thickening and deformity. ï” Schmorl believed that approximately 3% of everyone above 40 years had osteitis deformans
- 3. INTRODUCTION (Cont) ï” It has a predilection for the axial skeleton ï” Pelvis>tibia > Femur > Skull>spine >clavicle ï” But any bone may be affected ï” Paget disease is common in Europe, North America ï” It is rare in Asia and Africa
- 4. ETIOLOGY ï” UNKNOWN ï” Occasionally hereditary influence is noted on chromosome 18q ï” On electron microscopy of bone biopsies has demonstrated nuclear inclusions, ï” similar to those found in viral diseases (Paramyxoviridae family) are found in the highly nucleated osteoclasts ï” Endocrine and metabolic disturbances are unlikely because despite extensive involvement , many bones are free of disease
- 5. PATHOPHYSIOLOGY ï” 3 Phases: i) Lytic ii) mixed Lytic and Blastic iii) Sclerotic ï” Different skeletal lesions may progress at different rates. ï” At a given time, multiple stages of disease may be demonstrated in different skeletal regions of same patient
- 6. LYTIC PHASE ï” Disease begins with lytic phase ï” The bone is resorbed by osteoclasts that are more numerous, larger, and have more nuclei (upto 100) ï” Bone turnover rate increased as much as 20times normal
- 7. Mixed Lytic and Blastic phase ï” Rapid increase in bone formation from numerous osteoblasts ï” Morphologically osteoblasts are normal ï” The newly formed bone is abnormal with collagen fibers deposited in haphazard fashion rather than linear ï” As osteoclastic and osteoblastic activity repeats, high degree of bone turn over occurs
- 8. Sclerotic Phase ï” The bone formation dominates and has a disorganized woven pattern and is weaker than normal bone ï” Woven pattern allows the bone marrow to be infiltrated by excessive fibrous connective tissue and blood vessels leading to hyper vascular bone state ï” Eventually osteoblastic activity also declines and enters a sclerotic or burned-out phase ï” Continued bone resorption is minimal or absent
- 9. Histology
- 10. Osteoclast & osteoblasts Jigsaw puzzle / Mosaic pattern 1 2
- 11. Complications ï” Fractures and bony deformity ï” Secondary osteoarthritis ( when pagets disease around a joint) ï” Neurological complications â nerve root compression and cauda equina syndrome ï” Skull involvement- deafness vertigo tinnitus dental malocclusion basilar invagination cranial nerve disorders Sarcomatous degeneration - Osteosarcoma ï” Increased bone vascularity â high output cardiac failure
- 12. SURGICAL COMPLICATIONS: ï” Highly vascular marrow â Profuse bleeding ï” Structurally weak bone ï” Spinal / Epidural Anesthesia may be difficult
- 13. Investigations ï” Serum Alkaline phosphatase , BSAP ï” Serum Acid phosphatase ï” Urinary Markers â hydroxyproline, deoxypyridinoline, C-telopeptide, N- telopeptide ï” Serum calcium and phosphate levels will be normal
- 14. X-RAYS ï” Long bones: bowing thickening of cortex, narrowing of medulla honey combed or spongy, large dense bone looserâs zone of transformation
- 15. Brim Sign: thickening of the right pelvic brim (ileopectineal line) as compared to the left
- 16. Skull osteitis circumscripta or cotton wool exudates
- 17. tam o' shanter sign widening of the diploic space and an overall enlargement of the cranium
- 18. Pagets Spine Ivory Vertebra Picture frame sign
- 19. Blade of Grass or Candle flame sign: begins as a subchondral area of lucency with advancing tip of V-shaped osteolysis, extending towards the diaphysis
- 20. Looserâs Zone:
- 21. Scintigraphy Technetium 99 scan shows increased uptake in right hemipelvis and spine
- 22. DIFFERENTIAL DIAGNOSIS: ï” Osteomalacia ï” Fibrous Dysplasia ï” Multiple Myeloma ï” osteopetrosis
- 23. TREATMENT ï” Inactive lesions doesnât require any intervention ï” Goals of treatment: Suppression of Active disease Relief of Pain Prevention of Deformity and fractures High output cardiac dysfunction Reducing the Sarcomatous transformation
- 24. Suppressive Agents ï” BISPHOSPHONATES ï” 2nd generation bisphosphonates like Tiludronate, Alendronate, risendronate produces longer remission at lower doses. ï” Pamidronate â 30mg IV/day over 3hours for 3days ï” Zolidronic Acid- 5mg IV over 5 mins First choice where rapid mineralization is required in neurological symptoms, severe bone pain, risk of fracture prior to elective surgery Vitamin D and calcium supplements ï” It normalizes the ALP in 6 months ï” Bisphosphonates should not be used in patients with renal impairment
- 25. Calcitonin ï” Dosage â 100 IU / day SC/IM for 6-18 months reduced to 50 IU / day x 3/week ï” Calcitonin therapy can temporarily arrest active disease ï” ALP, urine Hydroxyproline is reduced ï” Positive Calcium balance ï” High output heart failure is improved ï” Bone pain relieved Surgical treatment is reserved for fractures, correction of bone deformity, THR, Spinal surgery Preoperatively and postoperatively calcitonin therapy gives good results and reduces bleeding
- 26. CASE Alkaline phosphatase â 1510 IU/L Sr. calcium â 8.7mg/dl Sr. Phosphorus â 3.1 mg/dl
- 28. THANK YOU