approach to generalised increased bone density
TRANSCRIPT
Imaging Approach To Generalized Increased Bone Density
By Dr Sachin
Intramembranous Ossification:Mesenchymal cells within embryonic connective
tissue Proliferates
Early Mesenchymal condensations
Differentiate directly
Osteoblasts
Skeletal Development
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
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
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
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
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
Clinical Manifestations
Radiological Manifestations
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
Autosomal dominant osteopetrosis
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
Errors in resorption & remodeling of secondary spongiosa, focal densities / striations◦Focal densities / striations◦Enostosis◦Osteopoikilosis◦Osteopathia striata
Dysplasias Of Endochondral Ossification (Secondary Spongiosa)
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
Kenny-caffey Syndrome/ Infantile Cortical Hyperostosis
◦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
Diagnosed as an incidental finding
Monostotic, Monomelic, Or Polyostotic
Sclerotomal distribution
Dripping wax appearance or flowing candle wax appearance
Melorheostosis
Renal osteodystrophy Chronic hypervitaminosis A Lead Fluorosis Hypervitaminosis D
Secondary Causes Of Generalized Increased Bone Density In Children
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
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
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
Clinical presentations
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
Mastocytosis
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
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
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 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
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
Toxins and Minerals
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
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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