approach to generalised increased bone density
TRANSCRIPT
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Imaging Approach To Generalized Increased Bone Density
By Dr Sachin
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Intramembranous Ossification:Mesenchymal cells within embryonic connective
tissue Proliferates
Early Mesenchymal condensations
Differentiate directly
Osteoblasts
Skeletal Development
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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
Endochondral Ossification
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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
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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
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DysplasiasOsteopetrosisPyknodysostosisCraniotubular dysplasias Craniotubular hyperostoses
MetabolicRenal osteodystrophy
Poisoning Chronic hypervitaminosis A Idiopathic
Caffey's diesase (infantile cortical hyperostosis)Idiopathic hypercalcemia of infancy Lead FluorosisHypervitaminosis D
Generalized Increased Bone Density In Children
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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
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Defective Carbonic anhydrase function Lack of alkaline environment for osteoclast
function Defective osteoclast function
Types Infantile autosomal recessive osteopetrosis Benign adult autosomal dominant osteopetrosis
Osteopetrosis
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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
Clinical Manifestations
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Radiological Manifestations
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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
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Osteosclerosis in superior and inferior portions of the vertebral bodies -'sandwich' appearance
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Autosomal dominant osteopetrosis
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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
Pyknodysostosis
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Errors in resorption & remodeling of secondary spongiosa, focal densities / striations◦Focal densities / striations◦Enostosis◦Osteopoikilosis◦Osteopathia striata
Dysplasias Of Endochondral Ossification (Secondary Spongiosa)
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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
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Hyperirritability
Soft tissue swelling
Cortical thickening
Bones commonly affected mandible, ribs, clavicle, ulna, any long bone but not spine
Kenny-caffey Syndrome/ Infantile Cortical Hyperostosis
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◦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
MIXED SCLEROSING DYSPLASIAS
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Diagnosed as an incidental finding
Monostotic, Monomelic, Or Polyostotic
Sclerotomal distribution
Dripping wax appearance or flowing candle wax appearance
Melorheostosis
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Renal osteodystrophy Chronic hypervitaminosis A Lead Fluorosis Hypervitaminosis D
Secondary Causes Of Generalized Increased Bone Density In Children
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Myelosclerosis/ Myeloproliferative disorders Metabolic◦Renal osteodystrophy
Poisoning◦Fluorosis
Neoplastic- Osteoblastic metastases : prostate and breasts- Lymphoma.
Mastocytosis : Paget's disease
Secondary Causes Of Generalized Increased Bone Density In Adults
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Group of diseases
Too many of certain types of cells made in the bone marrow
Fibrosis of the bone marrow with extramedullary hematopoiesis
Myelofibrosis→Osteosclerosis
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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
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Fatigue, Weight Loss
Easy Bruising And Bleeding
Fever, Night Sweats
Splenomegaly
Gout And Renal Colic Due To Hyperuricemia
Clinical presentations
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Replacement of the normal marrow cavity with fibrous tissue
with no trabecular or cortical disorganization
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A superscan appearance - intense symmetric activity in the bones with diminished renal and soft tissue activity on a Tc99m diphosphonate bone scan
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Mastocytosis
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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
Renal osteodystrophy
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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
Radiological abnormalities
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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
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Increased osteoclastactivity - release of calcium from bone
Osteoblasts form an increased amount of osteoid in responseto bone resorption
Excess osteoid does not contain hydroxyapatite but appears
opaque on radiographs - Rugger jerseySpine
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Rugger-jersey spine
D/d •Paget disease (“picture frame” vertebral body)•Osteoporosis
•Metastatic lesions
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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
Toxins and Minerals
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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
Osteoblastic Metastasis
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Hypercalcaemia, and increase in periosteal new bone with cortical thickening
Pseudotumour cerebri
D/D Infantile cortical hyperostosis : > 1yrs of age
Hypervitaminosis A
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Thank you