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- 2. Paget’s Disease of Bone •SUBMITTED BY : • R MUTHUMADHUMITHA BPT 3rd year
- 3. Definition • Paget’s diseaseof bone (PDB) is a chronic disorder of bone remodeling characterized by excessive and disorganized bone turnover, leading to structurally weak and deformed bones. • Common sites: Pelvis, skull, spine, femur, tibia.
- 5. Pathophysiology • • Excessiveosteoclastic activity Increased bone → resorption • • Compensatory osteoblastic activity Disorganized bone → formation • • Formation of woven bone (poorly mineralized, structurally weak) • • Increased vascularity Risk of high-output cardiac failure → • Key Pathological Hallmark: Mosaic pattern of lamellar bone on histology
- 6. Clinical Features • •🦴 Bone pain – Most common symptom • • 📏 Bone deformities – Bowing of long bones, skull enlargement (frontal bossing) • • ⚡ Increased fracture risk – Pathologic fractures, chalk-stick fractures • • 🎧 Hearing loss – Due to skull involvement and compression of CNVIII • • 🔺 Increased warmth over affected bones – Due to hypervascularity • • 💓 High-output cardiac failure (rare) – Due to increased AV shunting
- 8. Saber Shin Deformityin Paget’s Disease •Definition: Anterior bowing of the tibia due to excessive bone remodeling. •Cause: Weakened bone structure leading to progressive deformity. •Associated Conditions: Severe Paget’s disease, congenital syphilis. •Clinical Presentation: Visible tibial curvature, possible gait abnormalities. •Radiographic Findings: Thickened cortices with bowing, mixed lytic & sclerotic changes. •Management: •Bisphosphonates – To reduce excessive bone turnover. •Orthopedic intervention – If deformity causes pain or functional impairment.
- 9. Diagnosis - LabFindings • • 🧪 Labs: • - Alkaline phosphatase (ALP) (marker of bone ↑ turnover) • - Normal calcium, phosphate, and PTH • - Urinary hydroxyproline (bone resorption marker) ↑ • • 🎨 Imaging: • - X-ray:Thickened cortices, mixed lytic & sclerotic lesions • - Bone scan: Increased uptake in affected bones
- 10. Lab Findings –Detailed Explanation • 1. Serum Alkaline Phosphatase (ALP) – 🔑 Key Marker • - Elevated in active disease (due to increased osteoblastic activity) ↑ • - ALP correlates with disease burden & activity • - Can be normal in early/monostotic disease • Clinical Correlation: • - High ALP + normal calcium & phosphate = Paget’s disease • - High ALP + high calcium = Consider hyperparathyroidism, malignancy • 2. Calcium and Phosphate – Normal Levels • - Serum calcium = Normal (unlike primary hyperparathyroidism or malignancy) • - Serum phosphate = Normal (not a phosphate-related disorder) • - Calcium only if immobilization or secondary hyperparathyroidism ↑
- 11. • 3. UrinaryMarkers of Bone Resorption • - Urinary Hydroxyproline (Released during collagen breakdown) ↑ 🟢 • - Urinary N-telopeptide (NTX) & C-telopeptide (CTX) (Bone matrix 🟢 ↑ degradation) • 4. BoneTurnover Markers • - Serum C-terminal telopeptide (CTX) – Marker of osteoclast activity 🟢 ↑ • - Procollagen type 1 N-terminal propeptide (P1NP) – Marker of 🟢 ↑ osteoblast activity • - Both elevated in active disease and decrease with effective treatment • 5. Parathyroid Hormone (PTH) –Typically Normal • - PDB does not directly affect PTH • - PTH only in secondary hyperparathyroidism (e.g., due to vitamin D ↑ deficiency)
- 12. Management • • 💊First-line: Bisphosphonates (Zoledronic acid,Alendronate) – Inhibit osteoclast activity • • 💉 Calcitonin: Used if bisphosphonates contraindicated • • 🩼 Pain management: NSAIDs, physical therapy • • ️ 🛠️Surgical intervention: For fractures, deformities, or severe arthritis
- 13. Orthopedic Surgery/Intervention inPaget’s Disease (Including Saber Shin Deformity) • Orthopedic intervention is considered when Paget’s disease causes severe deformities, fractures, or functional impairment.The goals are to relieve pain, correct deformities, and restore function.
- 14. 1. Indications forSurgery • ✅ Severe bone deformities (e.g., saber shin deformity) causing pain or mobility issues ✅ Pathologic fractures (e.g., chalk-stick fractures) that fail to heal conservatively ✅ Severe arthritis (secondary osteoarthritis from bone misalignment) ✅ Spinal involvement causing nerve compression (spinal stenosis) ✅ Osteosarcoma (rare complication) requiring tumor resection
- 15. 2. Surgical Procedures 🔹Osteotomy (Bone Cutting & Realignment): • Used for severe saber shin deformity to straighten the tibia • Bone is cut, repositioned, and fixed using plates, screws, or rods • Requires post-op immobilization and rehabilitation 🔹 Fracture Fixation (Internal Fixation): • Plates, screws, or intramedullary rods used to stabilize pathologic fractures • Needed due to poor bone quality and delayed healing in Paget’s
- 16. Joint Replacement (Arthroplasty): •Total knee or hip replacement if severe arthritis develops • Common in long-standing disease affecting weight- bearing joints 🔹 Spinal Decompression Surgery: • Performed if Paget’s disease causes spinal stenosis with nerve compression • Laminectomy or spinal fusion may be done
- 17. 3. Pre- &Post-Surgical Considerations ✅ Pre-Surgery: • Optimize bone health with bisphosphonates (reduces bleeding risk from hypervascular bone) • Evaluate bone quality with imaging (X-ray, CT, MRI) ✅ Post-Surgery: • Rehabilitation & physiotherapy for mobility recovery • Pain management (NSAIDs, physical therapy) • Monitor for complications (delayed healing, implant failure, infection)
- 18. Key Takeaways • ✅Paget’s disease = Isolated elevated ALP with normal calcium & phosphate • ✅ Most common symptom = Bone pain • ✅ Classic X-ray findings = Mixed lytic & sclerotic bone lesions • ✅ Best initial treatment = Bisphosphonates • ✅ Complications = Fractures, osteosarcoma (rare), hearing loss, cardiac failure • 🔑 ExamTip: Paget’s disease is often an incidental finding on labs (isolated ALP in an older patient).Always correlate with ↑ symptoms & imaging!
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