Osteitis fibrosa cystica

Osteitis fibrosa cystica by Dr. Bipul Borthakur

1. OSTEITIS FIBROSA CYSTICA Dr. Bipul Borthakur ( Professor ) Dept of Orthopaedics, SMCH

2. OVERVIEW Definition Epidemiology Pathogenesis Causes Clinical Features Diagnosis Management Prognosis

3. DEFINITION Osteitis fibrosa cystica skeletal disorder loss of bone mass calcified supporting structures are replaced with fibrous tissue ( peritrabecular fibrosis) formation of brown tumors (cyst ) in and around the bone.

4. also known as osteitis fibrosa , osteodystrophia fibrosa , von Recklinghausen's disease of bone caused by hyperparathyroidism - surplus of parathyroid hormone from over-active parathyroid glands

5. EPIDEMIOLOGY Rare disease Occurs in 2% of individuals with primary Hyper PTH – accounting for 90 % of it 10 % - associated with primary hyperplasia < 1 % - parathyroid carcinoma - in individuals around 50 yrs of age

6. Male = Female Primarily affects younger individuals with age < 40 yrs 70 % cases occurs < 20 yrs of age 85 % cases occurs < 35 yrs of age Common in Asiatic countries

7. PATHOPHYSIOLOGY CHRONIC RENAL DISEASE ( decrease functional mass ) CHRONIC RENAL DISEASE ( decrease functional mass ) Decrease excretion of phosphorus Decrease activation of vitamin D3 Increase serum phosphorus Lower serum calcium level Increase in PTH secretion

8. Rapid osteoclastic turnover of bone Calcium mobilized from bone Normal or increase serum calcium level In regions with rapid bone loss , Haemorrhage , reparative granulation tissue & vascular proliferating fibrous tissue - Replace the normal marrow content OSTEITIS FIBROSA CYSTICA

9. CAUSES PARATHYROID ADENOMA Benign , metabolically active Comprises 80 -85 % of documented case of Hyper PTH HEREDITARY FACTORS Familial hyperparathyroidism MEN Type 1 - Autosomal dominant disorder Hyperparathyroidism - jaw tumour syndrome

10. mutations leading to hyperparathyroidism - parathyroid harmone receptor - G – proteins - Adenyl cyclase - Gene HRPT2 – protein parafibromin 3 ) PARATHYROID CARCINOMA

11. 4 ) RENAL COMPLICATIONS Renal osteodystrophy - skeletal complication of ESRD decrease calcitriol production from kidney increase PTH level mobilize calcium from bone 5 ) FLUORIDE INTOXICATION

12. CLINICAL FEATURES Bone pain or tenderness Bone fractures Skeletal deformities - Bowing of the bones

13. Hyper PTH - Kidney stones , nausea , constipation Parathyroid carcinoma - weight loss appetite loss polyuria polydipsia palpable neck mass in approx. 50 % of sufferers

14. DIAGNOSIS Lab test reveals ↑ Calcium level ( N – 8.5 - 10.2 mg/ dl ) ↑ PTH level ( > 250 pg /dl ) ( N – 65 pg / ml ) ↑ ALP level ( N – 20 – 140 IU /L )

15. X – Ray extreme thin bones - bowed or fractured M/C affected - fingers (bone resorption ) Skull x-ray - Ground glass/salt & pepper appearance SESTAMIBI NUCLEAR SCAN diagnose Brown tumour - cyst lined by osteoclast & sometimes blood pigments

16. FINE NEEDLE ASPIRATION ( FNA ) biopsy of the bone lesion - fibrosis & intertrabecular tunnels Brown tumours - display osteoclast Benign , dense , granular cytoplasm nucleus - ovular in shape fine chromatin nucleoli smaller than average

17. MANAGEMENT MEDICAL Vitamin D ( alfacalcidiol or calcitriol ) - given IV Treat not only Hyper PTH , but also regress the brown tumour or other symtom of OFC

18. SURGERY Severe case of OFC - PARATHYROIDECTOMY Result in removal of bone resorption & complete regression of brown tumours Bone transplants - succesful in filling lesion caused by OFC

19. PROGNOSIS After parathyroidectomy * increase bone density & repair of skeleton within weeks * regression of brown tumours within six months Following parathyoidectomy - hypocalcemia is common

20. THANK YOU “ krodhādbhavati sammohaḥ sammohātsmṛtivibhramaḥ smṛtibhraṃśād buddhināśo buddhināśātpraṇaśyati ” “From anger there comes delusion; from delusion, the loss of memory; from the loss of memory, the destruction of discrimination; and with the destruction of discrimination, he is lost .”