L15 Bone disease 2 Flashcards

1
Q

What are gigantism and acromegaly?

A
  • Overproduction of growth hormone (typically due to an adenoma of the pituitary gland)
  • Gigantism = overproduction before growth is complete
  • Acromegaly = overproduction after growth is complete (only affects the small bones of hands and feet, mandibular condyles, facial soft tissues- lips, nose, tongue)
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2
Q

What are the effects of acromegaly on the skull?

A
  • Enlarged thickening of bones
  • Enlargement of paranasal sinuses including maxillary sinuses
  • Enlargement of pituitary fossa (if caused by tumour of pituitary gland)
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3
Q

What are the effects of acromegaly on the jaws?

A
  • Enlarged mandible
  • Enlargement of ID canal
  • Spacing of anterior teeth due to jaw and tongue enlargement
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4
Q

What are the 3 types of hyperparathyroidism?

A
  • Primary: caused by pathology of the parathyroid gland e.g. adenoma
  • Secondary: glands are fine, but there is underlying disease e.g. chronic renal disease, causing low calcium levels causing more PTH to be excreted
  • Tertiary: when long-standing secondary hyperparathyroidism starts to behave like primary hyperparathyroidism
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5
Q

What is the function of parathyroid hormone?

A

Regulates serum calcium and phosphate.
Affects the bones, kidneys and small intestine.

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6
Q

Describe the events that occur due to hyperparathyroidism.

A
  • Increased PTH mobilises calcium from the skeleton
  • Increase in serum calcium, decrease in serum phosphate
  • Serum alkaline phophatase increases
  • Stones, bones, abdominal groans and psychic moans
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7
Q

What are the effects of hyperparathyroidism on the skull?

A

Osteopenia aka pepper-pot skull.
Speckled appearance of skull vault.

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8
Q

What are the effects of hyperparathyroidism on the jaws?

A
  • Osteopenia = ground glass/orange peel appearance
  • Loss of lamina dura and other corticated structures e.g. floor of maxillary antrum
  • No bony expansion
  • May be localised Brown tumours
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9
Q

Describe Brown tumours.

A
  • Typically displace the teeth
  • May be lightly corticated
  • Often multilocular in appearance
  • When enucleated they have a brown appearance due to the haemosiderin within them
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10
Q

What are the 2 types of fibrous dysplasia?

A
  • Monostotic fibrous dysplasia: affects 1 bone only, most common type, typically starts in childhood and then ceases
  • Polyostotic fibrous dysplasia: affects multiple bones, more common in women, cafe au lait spots, 2 types
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11
Q

What are the 2 types of polyostotic fibrous dyplasia?

A
  • Jaffe-Lichenstein type: skin lesions without endocrine disturbance
  • McCune-Albright syndrome: endocrine disturbance and skin lesions. Comprises polyostotic fibrous dysplasia, patchy melanotic skin pigmentation (café au lait spots), precocious puberty (especially in females)
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12
Q

What are the oral manifestations of fibrous dysplasia?

A
  • Jaws often involved (Mx>Md)
  • Present in childhood or adolescence
  • Gradually increasing painless swelling of the jaw, producing facial asymmetry
  • Causes tooth displacement
  • Usually smooth surfaced
  • Maxillary lesion may involve the sinus, zygomatic process, floor of orbit
  • More pronounced buccally than palatally
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13
Q

Describe the radiographic features of polyostotic fibrous dysplasia.

A
  • Localised lesion of ground glass bone
  • Margins of ground glass merge with normal bone
  • Loss of lamina dura around tooth
  • Enlargement of bone
  • Teeth displaced
  • Antrum may be displaced
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14
Q

Describe the histology of fibrous dysplasia.

A
  • Replacement of normal bone by fibrous tissue containing islands and trabeculae of metaplastic bone
  • Newly formed bone trabeculae form delicate, irregular shapes known as Chinese characters
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15
Q

How is fibrous dyplasia managed?

A
  • Conservative surgical removal to reduce deformity
  • Best to perform surgery once bone growth has ceased
  • Bisphosphonate therapy
  • Cannot be treated with radiotherapy
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16
Q

What is Paget’s disease of bone?

A
  • Defects of bone turnover
  • Possibly due to issues in osteoclast activity
  • Increased alkaline phosphatase
17
Q

What are the 3 phases of Paget’s disease?

A

Phases overlap
1) Initial predominantly osteolytic phase
2) Active stage of mixed osteolysis and osteogenesis
3) Predominantly osteoblastic stage, bone production

18
Q

What are the clinical features of Paget’s?

A
  • Predominantly affects people over 40
  • More common in men
  • More common in Caucasians
  • Incidence of 3% of over 40s in the UK
  • Most common in the weight bearing bones of the axial skeleton: sacrum, vertebrae then femur and skull
  • Infrequently found in the jaws
  • Produces bone deformity, distortion and bone pain
  • Diagnosis: blood sample, serum calcium and phosphate normal, alkaline phosphatase elevated due to bone turnover
19
Q

What are the oral mainfestations/complications of Paget’s?

A
  • If jaws are affected (rare), the maxilla is more commonly affected, osteopenia of bone = ground glass, loss of lamina dura
  • Progressive enlargement of alveolar ridge
  • Derangement of occlusion, spaced teeth
  • Difficulty wearing dentures
  • If jaw is affected, skull usually is too
  • Sensory and motor disturbance due to compression of nerves: blindness, deafness, facial paralysis
  • Early stage disease: post extraction haemorrhage as the bone is being replaced by fibro-vascular tissue
  • Late stage: bone being laid down, increased ankylosis and hypercementosis making extractions difficult- can lead to post-extraction infection and osteomyelitis
20
Q

What is osteoporosis circumscripta?

A
  • Feature of Paget’s disease
  • Bone being replaced by fibrous tissue, clear margin between normal bone and abnormal bone
21
Q

Describe the histopathology of Paget’s disease.

A
  • Disorganised bone remodelling producing combinations of osteoclastic and osteoblastic activity
  • Early stages: osteoclastic activity
  • Late stages: osteoblastic activity
  • Reversal lines produce mosaic appearance in bone
22
Q

What are the complications of Paget’s disease?

A

0.1-0.2% risk of sarcomatous change

23
Q

What conditions feature giant cell lesions?

A
  • Giant cell granuloma
  • Cherubism
24
Q

What is central giant cell granuloma?

A
  • Can affect anyone but predominantly under 20s
  • Giant cell lesion present
  • Mandible more commonly affected than maxilla
  • More common in men than women
  • Most anterior part of the jaws affected (sites of primary dentition)
  • Presents as a bony swelling
  • Growth can be rapid (if it is rapid, the typical radiological features may not be seen)
  • Treatment: curettage
  • Recurrence unusual even when lesion is not completely removed
25
Q

What is cherubism?

A
  • Autosomal dominant condition
  • More common in men
  • Disease of childhood
  • Bilateral expansion of jaws
  • Affects posterior mandible or posterior maxilla
  • If maxilla affected it gives a cherubic appearance (check enlargement, orbital floors moved upwards making eyes more upwards)
  • Treatment: slow progression so intervention isn’t always necessary
  • Surgical intervention if patient is severely affected
26
Q

What does this image show?

A

The histology of giant cell lesions:
- Large numbers of multinucleated giant cells

27
Q

Describe the radiological features of giant cell lesions.

A
  • Often multilocular
  • Well defined corticated outline
  • Uniformly radiolucent with radiopaque septa
  • Displaces teeth
  • Expansion of buccal/lingual bone
  • No tooth resorption
28
Q

What does this image show?

A

Cherubism, giant cell lesions.

29
Q

What is multiple myeloma?

A

Malignancy of plasma cells.

30
Q

Describe the main features of multiple myeloma.

A
  • Middle aged pts
  • Multiple sites including skull vault and mandible
  • Variable sizes
  • Round shape
  • Punched out appreance, well defined and non-corticated
  • Uniformly radiolucent
  • If large, may lead to pathological fracture
31
Q

Describe Langerhan’s cell histiocytosis.

A
  • Previously called histiocytosis X
  • Tumour of the Langerhan’s cells
  • May be single or multifocal lesions
  • Treatment includes curettage, resection and chemo
  • Similar in appearance to multiple myeloma (punched out appearance- well defined but non-corticated)
  • Floating tooth appearance
32
Q

Explain the main features of langerhans’ cells histiocytosis.

A
33
Q

What does this image show?

A

Langerhans’ cell histiocytosis.
Floating teeth, uniformly radiolucent, round.