Approach to generalised increased bone density


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Approach to generalised increased bone density

  1. 1. By Dr Sachin
  2. 2.  Intramembranous Ossification: Mesenchymal cells within embryonic connective tissue Proliferates Early Mesenchymal condensations Differentiate directly Osteoblasts
  3. 3. Mesenchymal Condensation Cartilage Anlage (Model) Bone Collar Mesenchymal Cells Differentiate IntoPrechondroblasts And Chondroblasts & Later In To ChondrocytesCalcification Of Bone Collar – Osteoclasts & Blood Vessels, Enter Primary Ossification Center
  4. 4. Osteoclast Invasion And Wave Of Resorbing Activity Removal Of Calcified Cartilage Woven Bone- The Primary SpongiosaFurther Remodeling -Woven Bone And The Cartilaginous Remnants Replaced With Lamellar Bone The Mature Trabecular Bone- Secondary Spongiosum
  5. 5. Growth in Bone Shape and Diameter (Modeling) During Longitudinal Growth Of A Long Bone, Continuous Resorption By Osteoclasts Beneath PeriosteumProgressively Destroys Lower Part Of Metaphysis Transforming It Into Diaphysis
  6. 6.  Dysplasias Osteopetrosis Pyknodysostosis Craniotubular dysplasias Craniotubular hyperostoses Metabolic Renal osteodystrophy Poisoning Chronic hypervitaminosis A Idiopathic Caffeys diesase (infantile cortical hyperostosis) Idiopathic hypercalcemia of infancy Lead Fluorosis Hypervitaminosis D
  7. 7. Dysplasias Of Endochondral Ossification (Primary Spongiosa) Failure In Resorption & Remodeling Of Primary Immature Spongiosa By OsteoclastsAccumulation Of Calcified Cartilage Matrix Packing The Medullary Cavity - Osteopetrosis - Pyknodysostosis Target sites: tubular & flat bones, vertebrae, skull base, ethmoids, ends of clavicle
  8. 8.  Defective Carbonic anhydrase function Lack of alkaline environment for osteoclast function Defective osteoclast functionTypes Infantile autosomal recessive osteopetrosis Benign adult autosomal dominant osteopetrosis
  9. 9.  Failure to thrive Cranial nerve entrapment (Optic nerve) Snuffling (nasal sinus architecture abnormalities) Hypercalcaemia Pancytopaenia (anaemia, leukopaenia and/or thrombocytopaenia) Hepatosplenomegaly (extramedullary haemopoesis) Intracerebral haemorrhage (thrombocytopaenia) Lymphadenopathy Premature senile facies
  10. 10.  Mandible : triangular opacity representing calcification within the secondary condylar cartilage ossification center Paranasal sinuses : poorly pneumatized (ethmoid sinuses least severely affected) Hypertelorism Calvarium : high-attenuation inner table, a broad, low- attenuation diploic space, and a less high-attenuation outer table “Hair-on-end" appearance : increased haematopoietic activity
  11. 11. Osteosclerosis in superiorand inferior portions of thevertebral bodies -sandwichappearance
  12. 12.  A lysosomal disorder due to genetic deficiency in Cathepsin K Short stature particularly limbs Delayed closure of cranial sutures Frontal and occipital bossing Nasal beaking Obtuse mandibular gonial angle with relative prognathism Segmentation defects
  13. 13.  Errors in resorption & remodeling of secondary spongiosa, focal densities / striations ◦ Focal densities / striations ◦ Enostosis ◦ Osteopoikilosis ◦ Osteopathia striata
  14. 14.  Dysplasias Of Intramembranous Ossification ◦ Target sites: flat bones, calvaria, bones of upper face, tympanic parts of temporal bone, vomer, medial pterygoid ◦ Progressive diaphyseal dysplasia ◦ Hereditary multiple diaphyseal sclerosis (Ribbing disease) ◦ Hyperostosis corticalis generalisata  Van Buchem disease  Sclerosteosis (Truswell-Hansen disease)  Worth disease  Nakamura disease ◦ Diaphyseal dysplasia with anemia ◦ Oculodento-osseous dysplasia ◦ Trichodento-osseous dysplasia ◦ Kenny-Caffey syndrome
  15. 15.  Hyperirritability Soft tissue swelling Cortical thickening Bones commonly affected mandible, ribs, clavicle, ulna, any long bone but not spine
  16. 16. ◦ Predominantly endochondral disturbance  Dysosteosclerosis  Metaphyseal dysplasia (Pyle disease)  Craniometaphyseal dysplasia  Frontometaphyseal dysplasia◦ Predominantly intramembranous defects  Melorheostosis  Craniodiaphyseal dysplasia  Lenz-Majewski hyperostotic dwarfism  Progressive diaphyseal dysplasia
  17. 17.  Diagnosed as an incidental finding Monostotic, Monomelic, Or Polyostotic Sclerotomal distribution Dripping wax appearance or flowing candle wax appearance
  18. 18.  Renal osteodystrophy Chronic hypervitaminosis A Lead Fluorosis Hypervitaminosis D
  19. 19.  Myelosclerosis/ Myeloproliferative disorders Metabolic ◦ Renal osteodystrophy Poisoning ◦ Fluorosis Neoplastic - Osteoblastic metastases : prostate and breasts - Lymphoma. Mastocytosis : Pagets disease
  20. 20.  Group of diseases Too many of certain types of cells made in the bone marrow Fibrosis of the bone marrow with extramedullary hematopoiesis
  21. 21.  Chronic myeloproliferative diseases e.g. essential thrombocytopenia and polycythemia vera and chronic neutrophilic, eosinophilic, and myeloid leukemia Acute myeloid leukemia & lymphocytic leukemia Hodgkins disease & non-hodgkins lymphoma Hairy cell leukemia Multiple myeloma
  22. 22.  Fatigue, Weight Loss Easy Bruising And Bleeding Fever, Night Sweats Splenomegaly Gout And Renal Colic Due To Hyperuricemia
  23. 23. Replacement of the normalmarrow cavity with fibrous tissue with no trabecular or cortical disorganization
  24. 24. A superscanappearance -intense symmetricactivity in thebones withdiminished renaland soft tissueactivity on a Tc99mdiphosphonatebone scan
  25. 25.  Musculoskeletal abnormalities secondary to chronic renal failure, due to concurrent and superimposed Osteomalacia (adults) / rickets (children) Secondary hyperparathyroidism (abnormal calcium and phosphate metabolism) ◦ Bone resorption ◦ Osteosclerosis ◦ Soft tissue & vascular calcifications ◦ Brown tumours Aluminum intoxication
  26. 26.  Osteopaenia : Early, thinning of cortices and trabeculae Salt and pepper skull Subperiosteal resorption : on radial aspects of middle phalanges of index and long fingers Rugger-jersey spine Demineralization Soft tissue calcification
  27. 27. Renal failure - phosphate retentionSubsequent hyperplasia of parathyroid gland chief cells Decrease in serum calcium and an increase in serum parathyroid hormone Acts on kidneys
  28. 28. Increased osteoclastactivity - release of calcium from boneOsteoblasts form an increased amount of osteoid in response to bone resorption Excess osteoid does not contain hydroxyapatite but appearsopaque on radiographs - Rugger jersey Spine
  29. 29. D/d•Paget disease(“picture frame”vertebral body)•Osteoporosis•Metastatic lesions
  30. 30.  Fluorosis- Endemic areas (asia - india and china; rajasthan and gujarat, andhra, punjab, haryana, M.P. And maharashtra,t.N.,W.B.,U.P.,Bihar and assam)- Increased osteoclastic response- Cortical thickening encroachment upon medullary cavity, ossification of ligamentous attachments
  31. 31.  Metastatic disease ◦ Prostatic carcinoma ◦ Breast cancer ◦ Transitional cell carcinoma (TCC) ◦ Multiple myeloma ◦ Lymphoma ◦ Carcinoid ◦ Medulloblastoma ◦ Neuroblastoma ◦ Mucinous adenocarcinoma of the gastrointestinal tract: , e.G. Colon carcinoma ◦ Lymphoma
  32. 32.  Hypercalcaemia, and increase in periosteal new bone with cortical thickening Pseudotumour cerebri D/D Infantile cortical hyperostosis : > 1yrs of age
  33. 33. Thank you