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By Dr Sachin
   Intramembranous Ossification:
     Mesenchymal cells within embryonic connective
                    tissue
    Proliferates

           Early Mesenchymal condensations

    Differentiate directly

                       Osteoblasts
Mesenchymal Condensation

            Cartilage Anlage (Model)

                   Bone Collar

      Mesenchymal Cells Differentiate Into
Prechondroblasts And Chondroblasts & Later In To
                  Chondrocytes

Calcification Of Bone Collar – Osteoclasts & Blood
    Vessels, Enter Primary Ossification Center
Osteoclast Invasion And Wave Of Resorbing
                   Activity

     Removal Of Calcified Cartilage

   Woven Bone- The Primary Spongiosa


Further Remodeling -Woven Bone And The
         Cartilaginous Remnants
      Replaced With Lamellar Bone

 The Mature Trabecular Bone- Secondary
              Spongiosum
Growth in Bone Shape and Diameter
               (Modeling)

 During Longitudinal Growth Of A Long Bone,
 Continuous Resorption By Osteoclasts Beneath
                  Periosteum




Progressively Destroys Lower Part Of Metaphysis
          Transforming It Into Diaphysis
   Dysplasias
    Osteopetrosis
    Pyknodysostosis
    Craniotubular dysplasias
    Craniotubular hyperostoses
   Metabolic
    Renal osteodystrophy
   Poisoning
   Chronic hypervitaminosis A
   Idiopathic
    Caffey's diesase (infantile cortical hyperostosis)
    Idiopathic hypercalcemia of infancy
    Lead
    Fluorosis
    Hypervitaminosis D
Dysplasias Of Endochondral Ossification
               (Primary Spongiosa)

  Failure In Resorption & Remodeling Of Primary
          Immature Spongiosa By Osteoclasts


Accumulation Of Calcified Cartilage Matrix Packing The
                   Medullary Cavity
                   - Osteopetrosis
                 - Pyknodysostosis


   Target sites: tubular & flat bones, vertebrae, skull
              base, ethmoids, ends of clavicle
   Defective Carbonic anhydrase function
   Lack of alkaline environment for osteoclast
    function
   Defective osteoclast function



Types
 Infantile autosomal recessive osteopetrosis
 Benign adult autosomal dominant osteopetrosis
   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
   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
Osteosclerosis in superior
and inferior portions of the
vertebral bodies -'sandwich'
appearance
   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
   Errors in resorption & remodeling of secondary
    spongiosa, focal densities / striations
    ◦ Focal densities / striations
    ◦ Enostosis
    ◦ Osteopoikilosis
    ◦ Osteopathia striata
   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
   Hyperirritability

   Soft tissue swelling

   Cortical thickening

   Bones commonly
    affected mandible,
    ribs, clavicle, ulna,
    any long bone but not
    spine
◦ 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
   Diagnosed as an incidental finding

   Monostotic, Monomelic, Or Polyostotic

   Sclerotomal distribution

   Dripping wax appearance or flowing candle wax
    appearance
 Renal osteodystrophy
 Chronic hypervitaminosis A
 Lead
 Fluorosis
 Hypervitaminosis D
   Myelosclerosis/ Myeloproliferative disorders
   Metabolic
    ◦ Renal osteodystrophy
   Poisoning
    ◦ Fluorosis
   Neoplastic
    - Osteoblastic metastases : prostate and breasts
    - Lymphoma.
   Mastocytosis :
   Paget's disease
   Group of diseases

   Too many of certain types of cells made
    in the bone marrow

   Fibrosis of the bone marrow with
    extramedullary hematopoiesis
   Chronic myeloproliferative diseases e.g. essential
    thrombocytopenia and polycythemia vera and
    chronic neutrophilic, eosinophilic, and myeloid
    leukemia

   Acute myeloid leukemia & lymphocytic leukemia

   Hodgkin's disease & non-hodgkin's lymphoma

   Hairy cell leukemia

   Multiple myeloma
   Fatigue, Weight Loss

   Easy Bruising And Bleeding

   Fever, Night Sweats

   Splenomegaly

   Gout And Renal Colic Due
    To Hyperuricemia
Replacement of the normal
marrow cavity with fibrous tissue
 with no trabecular or cortical
        disorganization
A superscan
appearance -
intense symmetric
activity in the
bones with
diminished renal
and soft tissue
activity on a Tc99m
diphosphonate
bone scan
   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
 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
Renal failure - phosphate retention


Subsequent hyperplasia of parathyroid gland chief
                     cells


  Decrease in serum calcium and an increase in
          serum parathyroid hormone


                Acts on kidneys
Increased osteoclast
activity - release of calcium from bone

Osteoblasts form an increased amount
        of osteoid in response
         to bone resorption

   Excess osteoid does not contain
     hydroxyapatite but appears
opaque on radiographs - Rugger jersey
                Spine
D/d
•Paget disease
(“picture frame”
vertebral body)
•Osteoporosis

•Metastatic lesions
   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
   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
   Hypercalcaemia, and
    increase in periosteal new
    bone with cortical
    thickening

   Pseudotumour cerebri


   D/D
    Infantile cortical
    hyperostosis : > 1yrs of age
Thank you

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Approach to generalised increased bone density

  • 2. Intramembranous Ossification: Mesenchymal cells within embryonic connective tissue Proliferates Early Mesenchymal condensations Differentiate directly Osteoblasts
  • 3. Mesenchymal Condensation Cartilage Anlage (Model) Bone Collar Mesenchymal Cells Differentiate Into Prechondroblasts And Chondroblasts & Later In To Chondrocytes Calcification Of Bone Collar – Osteoclasts & Blood Vessels, Enter Primary Ossification Center
  • 4. Osteoclast Invasion And Wave Of Resorbing Activity Removal Of Calcified Cartilage Woven Bone- The Primary Spongiosa Further Remodeling -Woven Bone And The Cartilaginous Remnants Replaced With Lamellar Bone The Mature Trabecular Bone- Secondary Spongiosum
  • 5. Growth in Bone Shape and Diameter (Modeling) During Longitudinal Growth Of A Long Bone, Continuous Resorption By Osteoclasts Beneath Periosteum Progressively Destroys Lower Part Of Metaphysis Transforming It Into Diaphysis
  • 6. Dysplasias Osteopetrosis Pyknodysostosis Craniotubular dysplasias Craniotubular hyperostoses  Metabolic Renal osteodystrophy  Poisoning  Chronic hypervitaminosis A  Idiopathic Caffey's diesase (infantile cortical hyperostosis) Idiopathic hypercalcemia of infancy Lead Fluorosis Hypervitaminosis D
  • 7. Dysplasias Of Endochondral Ossification (Primary Spongiosa) Failure In Resorption & Remodeling Of Primary Immature Spongiosa By Osteoclasts Accumulation Of Calcified Cartilage Matrix Packing The Medullary Cavity - Osteopetrosis - Pyknodysostosis Target sites: tubular & flat bones, vertebrae, skull base, ethmoids, ends of clavicle
  • 8. Defective Carbonic anhydrase function  Lack of alkaline environment for osteoclast function  Defective osteoclast function Types  Infantile autosomal recessive osteopetrosis  Benign adult autosomal dominant osteopetrosis
  • 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.
  • 11. 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
  • 12. Osteosclerosis in superior and inferior portions of the vertebral bodies -'sandwich' appearance
  • 13.
  • 14. 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
  • 15.
  • 16.
  • 17. Errors in resorption & remodeling of secondary spongiosa, focal densities / striations ◦ Focal densities / striations ◦ Enostosis ◦ Osteopoikilosis ◦ Osteopathia striata
  • 18. 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
  • 19. Hyperirritability  Soft tissue swelling  Cortical thickening  Bones commonly affected mandible, ribs, clavicle, ulna, any long bone but not spine
  • 20. ◦ 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
  • 21. Diagnosed as an incidental finding  Monostotic, Monomelic, Or Polyostotic  Sclerotomal distribution  Dripping wax appearance or flowing candle wax appearance
  • 22.
  • 23.
  • 24.  Renal osteodystrophy  Chronic hypervitaminosis A  Lead  Fluorosis  Hypervitaminosis D
  • 25. Myelosclerosis/ Myeloproliferative disorders  Metabolic ◦ Renal osteodystrophy  Poisoning ◦ Fluorosis  Neoplastic - Osteoblastic metastases : prostate and breasts - Lymphoma.  Mastocytosis :  Paget's disease
  • 26. Group of diseases  Too many of certain types of cells made in the bone marrow  Fibrosis of the bone marrow with extramedullary hematopoiesis
  • 27. Chronic myeloproliferative diseases e.g. essential thrombocytopenia and polycythemia vera and chronic neutrophilic, eosinophilic, and myeloid leukemia  Acute myeloid leukemia & lymphocytic leukemia  Hodgkin's disease & non-hodgkin's lymphoma   Hairy cell leukemia  Multiple myeloma
  • 28. Fatigue, Weight Loss  Easy Bruising And Bleeding  Fever, Night Sweats  Splenomegaly  Gout And Renal Colic Due To Hyperuricemia
  • 29. Replacement of the normal marrow cavity with fibrous tissue with no trabecular or cortical disorganization
  • 30.
  • 31.
  • 32.
  • 33.
  • 34. A superscan appearance - intense symmetric activity in the bones with diminished renal and soft tissue activity on a Tc99m diphosphonate bone scan
  • 35.
  • 36. 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
  • 37.  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
  • 38. Renal failure - phosphate retention Subsequent hyperplasia of parathyroid gland chief cells Decrease in serum calcium and an increase in serum parathyroid hormone Acts on kidneys
  • 39. Increased osteoclast activity - release of calcium from bone Osteoblasts form an increased amount of osteoid in response to bone resorption Excess osteoid does not contain hydroxyapatite but appears opaque on radiographs - Rugger jersey Spine
  • 40. D/d •Paget disease (“picture frame” vertebral body) •Osteoporosis •Metastatic lesions
  • 41.
  • 42. 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
  • 43.
  • 44. 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
  • 45.
  • 46. Hypercalcaemia, and increase in periosteal new bone with cortical thickening  Pseudotumour cerebri  D/D Infantile cortical hyperostosis : > 1yrs of age