Systemic Diseases Manifested in the Jaws

12/5/2008 S Systemic i Di Diseases M Manifested if d iin the h Jaws Reading Material: Chapter 24 24, White & Pharoah Mohammad Islam BDS, PhD islam00...
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12/5/2008

S Systemic i Di Diseases M Manifested if d iin the h Jaws Reading Material: Chapter 24 24, White & Pharoah

Mohammad Islam BDS, PhD [email protected]

Systemic Diseases Manifested in Radiographs • Endocrine disorders – Parathyroid gland: hyper & hypoparathyroidism – Pituitry gland: hyper & hypopituitarism – Thyroid gland: hyper & hypo thyroidism – Adrenal gland: Cushing syndrome • Bone metabolism disorders – Vitamin D related: Rickets, Osteomalacia. Renal osteodystrophy, Hypophosphatemia – Osteoporosis, hypophosphatasia : Bone loss – Osteopetrosis: Lack of resorption> increased bone • Blood disorders & others – Sickle cell anaemia – Thalassemia – Progressive systemic sclerosis

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Bony Changes in Systemic Disease

• • • •

Size and shape of the bone Number, size and orientation of trabeculae Thickness and density of cortical layer Increase/decrease in overall bone density

Bony Changes in Systemic Disease

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Dental Changes in Systemic Disease • • • •

Eruption rate Hypoplasia Hypocalcification Loss of lamina dura

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Dentomaxillofacial Radiology (2002) 31, 84 - 92

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Hyperparathyroidism • Excess of PTH & serum calcium due to – Neoplasm in one of the four parathyroid glands (0.1% prevalence) – Secondary to hypocalcemia, increase secretion of PTH

• Clinical features: – Women^ than men, adult – Renal calculi, peptic ulcers, bone & joint pain: due to hypercalcemia

Radiological Features • Detectable in one out of five patients • Increase resorption activity leads to erosion of cortical layers, radiolucent appearance of jaws, browns tumor, occasionally loss of lamina dura around teeth

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Hypoparathyroidism • Reduced level of serum PTH due to d damage/removal / l off parathyroid th id gland l d • Reduced PTH>hypocalcemia>variety of neurologic and muscular symptoms

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Hypoparathyroidism • Calcification of basal ganglia • Enamel hypoplasia, yp p , root resorption, p , dilaceration, delayed eruption

Hyperpituitarism • Increase in growth hormone release usually due g tumor of anterior lobe of p pituitary y to a benign gland • Increase growth hormone causes overgrowth of tissues that are capable of growth at the time of onset • Childhood onset: Giantism (prominently skeletal growth, causing large stature) • Adult onset: Acromegaly (less skeletal growth, characteristic growth of mandible, condyle, lips, tongue, nose)

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Radiological Features • • • •

Large sella turcica Enlargement of paranasal sinuses Thickening of the outer table of the skull Mandibular prognathism due to condylar growth, large angle, flaring of anterior maxillary teeth teeth, anterior open bite bite, large tongue

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Acromegaly

Hypopituitarism • Less GH secretion • Individuals are of short stature with proportionate body parts • Delayed exfoliation of primary and late eruption of permanent dentition • Smaller jaws, jaws crowding of teeth

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Hyperthyroidism • Increase production and level of serum th thyroxin i d due either ith tto diff diffuse ttoxic i goitre it (Grave’s disease) or toxic nodular goitre (benign tumor) • Advanced dental development, early loss of primary dentition

Hyperthyroidism Diffuse toxic goitre

Toxic nodular goitre

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Hypothyroidism • Insufficient production of thyroxin while TSH level remains normal • In children (cretinism), slower development, delayed eruption, slow exfoliation of primary teeth, delayed ossification and development of paranasal sinuses • In adult (myxedema) no dental abnormalities are seen

Cushing’s Syndrome • ^ glucocorticoids by adrenal glands • Loss L off b bone mass d due tto reduced d d osteoblast t bl t function & increased osteoclast activity • Clinical features: obesity sparing extremities, kyphosis, weakness, hypertension etc. • Radiographically signs of bone loss in the jaw ( (granular l pattern), tt ) lamina l i d dura, skull k ll ((mottled ttl d appearance)

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Cushing’s Syndrome

Rickets & Osteomalacia • Inadequate calcium and phosphate level in th serum and the d extracellular t ll l space • In Rickets hypocalcemia leads to tetany, convulsion and other symptoms • In Osteomalacia, bone pain, muscle weakness greenstick fracture weakness, fracture, tetany and a waddling gait is seen

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Rickets & Osteomalacia Rickets: Delayed dental eruption, generalized radiolucency of the jaws jaws, enamel hypoplasia hypoplasia, bowed leg, widening and fraying of epiphyses of long bones, greenstick fractures in weight bearing bones. Osteomalacia: cortical thinning, large radiolucency (pseudo fractures), (p ), no radiographic g p manifestation in the jaws

Rickets Loss of skull sutures

Bowing of long bones

Hypoplastic enamel

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Rickets

Hypophosphatemia • Inherited disorders that cause renal t b l malfunction tubular lf ti and d excessive i lloss off phosphorus • Clinical and radiological features are similar to that of rickets • Teeth may have large pulp chambers and root canals, periapical abscess

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Hypophosphatemia

Renal Osteodystrophy • In chronic renal failure, 1,25(OH)2D can not f form in i kidney. kid Thi This affects ff t calcium l i absorption in intestine. Kidney damage also prevents phosphorus excretion causing development of hyperphosphatemia. Prolonged hypocalcemia causes increase secretion of PTH and secondary hyperparathyroidism

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Renal Osteodystrophy • Radiological features are mixed: radiolucency di l and d radiodensity di d it may present • Features of hyperparathyroidism are present • Changes in may present, present hypoplasia & hypocalcification may affect dentition

Renal Osteodystrophy

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Quiz 3 •

In general, displacement of adjacent structures t t suggests t a ________ lesion, l i while resorption indicates a __________ lesion. A. B. C. D.

Malignant; benign g ; malignant g Benign; Rapidly growing, slowly growing Slowly growing; rapidly growing

Systemic Diseases Manifested in Radiographs • Endocrine disorders – Parathyroid gland: hyper & hypoparathyroidism – Pituitry gland: hyper & hypopituitarism – Thyroid gland: hyper & hypo thyroidism – Adrenal gland: Cushing syndrome • Bone metabolism disorders – Vitamin D related: Rickets, Osteomalacia. Renal osteodystrophy, Hypophosphatemia – Osteoporosis, hypophosphatasia : Bone loss – Osteopetrosis: Lack of resorption> increased bone • Blood disorders & others – Sickle cell anaemia – Thalassemia – Progressive systemic sclerosis

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Diseases of Bone in the Jaws • Fibro-osseous conditions – Fibrous dysplasia – Periapical cemental dysplasia – Florid osseous dysplasia

• • • • • •

Cemento-ossifying fibroma (COF) Central giant cell granuloma (CGCG) Aneurysmal y bone cyst y ((ABC)) Cherubism Paget’s disease Langerhans’ cell histiocytosis

Osteoporosis • Imbalance between bone formation and bone resorption • Less formation relative to resorption leads to net bone loss • Histlologically bone appears normal

Dentomaxillofacial Radiology (2002) 31, 84 - 92

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Sites of Bony Changes

• Number, size and orientation of trabeculae: Intraoral peri-apical • Thickness and density of cortical layer: P Panoramic i radiograph di h

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• Decrease in overall bone density

Taguichi & Tanimoto et al. AJR:183, December 2004

Osteopetrosis • Lack of bone resorption due to defect in osteoclast t l t biology bi l att th the llevell off differentiation or function • Bone remodelling (a simultaneous process of formation and resorption) is altered, only bone formation takes place, without any or enough resorption

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Osteopetrosis

Osteitis Deformans (Paget’s Disease of Bone) • Abnormal resorption and apposition of osseous structures in many bones in the body. • 90% of patients are over 40 years of age. • 2:1 males. • When jaws are involved involved, maxilla more frequent.

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Paget’s Disease • Radiographically: – Discrete radiolucent areas known as “osteoporosis circumscripta.” – “Ground glass” appearance of bone. – “Cotton-wool” appearance of bone. – Generalized hypercementosis. yp

Paget’s Disease

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Thalassemia • Cooley’s anemia; Mediterranean anemia • Diminished synthesis of  - or  - globin chain • RBC: hemoglobin content , life span  • Autosomal dominant trait • Individuals of Mediterranean origin

Thalassemia • Radiographic signs: – In the skull skull, widening of diploe space causes the outer table to thin out, resulting in trabeculae projecting from surface. This is described as “hair-onend” or “crew-cut” appearance. – Generalized granular appearance of skull. – Thinning of cortical borders of jaw bones. – Periapaical radiographs show large marrow spaces.

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Thalassemia

Thalassemia

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Sickle-cell Anemia • Hemolytic anemia • Abnormal hemoglobin- under low oxygen tension results in sickling of RBC • Autosomal recessive trait • Occurs almost exclusively in people of African descent

Sickle-cell Anemia (R/F) • Identical to those seen in Thalassemia – “Hair-on-end” effect – Thinning of cortical plates – Large marrow spaces, etc

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Progressive Systemic Sclerosis • Scleroderma, dermatosclerosis • Generalized connective tissue disease • Hardening (sclerosis) of skin and wide variety of visceral organs, e.g. GI tract, heart, lungs, kidneys. • Women affected 3:1 ratio • Greatest incidence 30 – 50 years.

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Progressive Systemic Sclerosis • Xerostomia • Caries and periodontal disease  • Skin – Thick – Leathery, waxy appearance

• In 25% of patients, mandibular erosions at regions of muscle attachment such as angles, coronoid process and condyle

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30-75% of patients show extreme widening of PDL space (2-4 times the normal) that is not associated with tooth mobilityy or g gingival g attachment

Langerhans’ Cell Disease

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LCH

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