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developmental bone disorders > DBD > Flashcards

DBD Flashcards

1
Q

______ ______ is one of the most common heritable bone diseases

A

Osteogenesis Imperfecta

“brittle bone disease”

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2
Q

Osteogenesis imperfecta is characterized by a mutation in the ______ gene:

  • Abnormal _____
  • _______
A

collagen gene

  • Abnormal mineralization
  • Osteopenia (low bone density)
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3
Q

What is the heritable variation of osteogenesis imperfecta (AD vs AR)

A

Autosomal dominant: 90%

Autosomal recessive: 10%

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4
Q

Clinical (non-dental) findings of osteogenesis imperfecta:

A
  • Blue sclera
  • Hearing loss
  • Craniofacial alterations (CL III occl.; triangular face)
  • Bowing deformity of bones
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5
Q

Clincal dental findings of osteogenesis imperfecta:

A

Identical to dentogenesis imperfecta:

  • blue/yellow/brown translucensy (“opalescent”)
  • severe attrition (loss of VDO)
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6
Q

Radiographic findings of osteogenesis imperfecta:

A
  • “shell teeth”
  • pulpal obliteration
  • roots narrow/corn cob shaped
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7
Q

Tx of osteogenesis imperfecta:

A
  • Classically: physiotherapy, rehab, and orthopedic surgery
  • Minimize factors that cause fractures
  • IV bisphosphonates to kids w/ mod-severe disease
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8
Q

Prognosis of osteogenesis imperfecta:

A

Variable depending on type/gene expression

Normal - death at birth

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9
Q

_____ is characterized by a lack of osteoclastic activity (continued bone formation and ossification)

A

Osteopetrosis

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10
Q

Compare AD osteopetrosis vs AR osteopetrosis

A

AD: less severe

AR: severe

  • blindness
  • deafness
  • fractures
  • osteomyelitis (infection/inflammation of marrow)
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11
Q

In osteopetrosis, marrow spaces are filled by ___ ___ resulting in loss of _____ ____ which results in _____

A

In osteopetrosis, marrow spaces are filled by dense bone resulting in loss of hematopoietic precursors which results in pancytopenia (deficiency of all blood cell types)

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12
Q

Radiographic presentation of osteopetrosis:

A
  • Diffuse density of skeleton
  • tooth roots hard to visualize (dense bone surrounds them)
  • Failure of eruption
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13
Q

Tx of osteopetrosis:

A
  • Supportive: transfusion and AB when necessary
  • Marrow transplant = limited success
  • Alternative: interferon w/ calcitriol and restrition of Ca2+ intake, corticosteroids, and EPO
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14
Q

Prognosis of osteopetrosis:

A

AD: long term survival possible

AS: poor (life expectancy <20)

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15
Q

_____ _____ is an AD disorder that mainly affects the skull, jaws, and clavicles

A

Cleidocranial dysplasia

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16
Q

Clinical presentation of cleidocranial dysplasia

A
  • Prominent forehead and hypoplastic face

- Primary dentition retained (permanent present but un-erupted)

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17
Q

Tx and Prognosis of cleidocranial dysplasia

A

Tx:

  • Surgery: correct skeletal relations and remove supernumerary teeth
  • Ortho: correct tooth relations

Prognosis: Good (essentially normal lifespan)

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18
Q

Osteoporotic bone marrow defect presentation and epidemiology:

A
  • Asymptomatic: incidental finding
  • Body of mandible at an old extraction site
  • Middle aged females
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19
Q

Osteoporotic bone marrow defect histopathology:

A
  • Fatty and hematopoietic marrow seen

- May resemble metastatic disease (BIOPSY!)

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20
Q

Osteoporotic bone marrow defect radiographic findings:

A

PANO: radiolucent and circumscribed

PA: ill-defined borders and fine central trabeculations

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21
Q

Idiopathic osteosclerosis is an ______ (incidental finding), _____ lesion with no expasion.

It is also known as ____ ____ ___ and _____

A

Idiopathic osteosclerosis is an asymptomatic (incidental finding), radiopaque lesion with no expasion.

It is also known as “dense bone island” and “enostosis”

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22
Q

Idiopathic osteosclerosis is most commonly found in the _____ region and consists of _____ ____ ____

A

premolar region

dense viable bone

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23
Q

What three lesions is idiopathic osteosclerosis often confused with?

A

Condensing osteitis

Hypercementosis

Cementoblastoma

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24
Q

_____ is a painless, bilateral expansion of the jaw; it may also involve the ____ ___ and cause displacement of teeth

A

Cherubism

orbital wall

25
Q

Cherubism is _____ ____ or a ___ ___ mutation; it is detected in ______

A

Autosmal dominant or de novo mutation

childhood

26
Q

Radiographic presention of cherubism:

A

Bilateral, multilocular radiolucencies of posterior mandible

27
Q

Histologic presentation of cherubism:

A
  • Edematous, cellular fibrous CT w/ hemorrhagic background
  • Sparse, benign-appearing multinucleated giant cells
  • Perivascular hyalinization (sometimes)
28
Q

Tx of cherubism:

A

NO optimal Tx

  • Surgery known to accelerate growth of some lesions
  • Some cases involute during puberty
29
Q

_____ ____ ___ is an idiopathic (possibly trauma induced) lesion that presents in the 1st and 2nd decade with a ____ predilection

A

Simple bone cyst

male

aka “traumatic bone cyst”

30
Q

Simple bone cyst clinical and radiographic presentation:

A
  • Posterior mandible (reported in all bones)
  • Well-circumscribed radiolucency with scalloping between roots
  • Empty cavity w/in bone with no epithelial lining (not a true cyst)
31
Q

Tx of simple bone cyst:

A

Enter lesion and induce bleeding

-hemorrhage organizes and heals lesion

32
Q

_____ ____ is the abnormal resorption-deposition of bone resulting in thickened and weakened bone

A

Osteitis deformans

aka “Paget disease of bone”

33
Q

Osteitis deformans presents in:

___ in white ____ (2:1)

Jaws involved in ___ of cases (__ > __)

A

40+ white males (2:1)

Jaws 17% of cases (Mx > Mn)

34
Q

Clinical presentation of osteitis deformans:

A
  • Elevated serum alkaline phosphatase
  • Bone pain
  • Simian stance (monkey like) with femur involvement
35
Q

Histologic presentation of osteitis deformans:

A
  • Irregular trabeculae w/ resting and reversal lines (“mosaic pattern”)
  • Rimmed by osteoblasts and osteoclasts
  • Marrow replaced by vascular fibrous CT
36
Q

Tx of osteitis deformans:

A

Asymptomatic: no tx
-monitor for giant cell tumor of bone and osteosarcoma

Symptomatic: bisphosphonates

37
Q

Dental complications of osteitis deformans:

A
  • Difficult ext. (hypercementosis/ankylosis)
  • Extensive hemorrhage if surgery during vascular lytic phase
  • Poor wound healing = susceptible to osteomyelitis during avascular sclerotic phase
  • Require new dentures periodically due to progressive alveolar enlargement
  • Unfavorable osseointigration of implants (esp. if taking bisphosphonates)
38
Q

Radiographic presentation of osteitis deformans:

A
  • “cotton-wool” appearance

- Extensive hypercementosis of teeth

39
Q

______ _____ is a developmental tumor-like lesion caused by a mutation of tumor suppressor gene GNAS1

A

Fibrous dysplasia

40
Q

Fibrous dysplasia presents in the ___ or ___ decade as a _____, _____ swelling that is slow growing

A

1st or 2nd decade

painless, unilateral swelling

41
Q

How often is fibrous dysplasia monostotic? What jaw does it affect more commonly?

A

75-80% monostotic

Mx > Mn = facial deformity

42
Q

Radiographic presentation of fibrous dysplasia:

A
  • “ground glass” pattern
  • poorly define, blended margins
  • sinus obliteration if Mx involved
43
Q

Histologic presentation of fibrous dysplasia:

A
  • “Chinese characters:” irregular trabeculae of immature (woven) bone
  • no capsule: abnormal bone fuses to adjacent normal bone
  • cellular intertrabecular CT
44
Q

Tx of fibrous dysplasia

A
  • small lesions: no tx or en bloc resection
  • cosmetic/functional deformity may require surgical reduction
  • disease may stabilize w/ maturation
  • mesenchymal malignancy transformation rare; radiation to lesion is a risk factor
45
Q

What is the recurrence of treated fibrous dysplasia lesions?

A

25-50% (esp. in younger pts)

46
Q

What is the presentation of Jaffe type polyostotic fibrous dysplasia?

A
  • 2+ bones affected

- cafe-au-lait spots with jagged borders

47
Q

What is the presentation of Mccune-Albright type polyostotic fibrous dysplasia?

A
  • 2+ bones affected
  • cafe-au-lait pigmentation
  • endocrine disturbances (causes early puberty)
48
Q

From what cells does cemento-osseous dysplasia originate?

A

Periodontal ligament fibroblasts (benign, possibly reactive process)

49
Q

What population does cemento-osseous dysplasia generally affect?

Do the teeth test vital with this lesion?

A

Females (AA > East Asian > Whate)

Yes, the teeth test vital

50
Q

What is the least severe cemento-osseous dysplasia?

How is it dx, tx, prognosis?

A

Periapical cemento-osseous dysplasia (mild)

Dx: clinically and radiographs

Tx: none

Prognosis: excellent

51
Q

What is the clinical presentation of focal cemento-osseous dysplasia (moderate)?

A

Swelling/discomfort in the body of the mandible

52
Q

What is the radiographic presentation of focal cemento-osseous dysplasia (moderate)?

A
  • Unilocular radiolucency (may have central radiopacity)

- Poorly defined; multiple small, gritty fragments

53
Q

What is the histologic presentation of focal cemento-osseous dysplasia (moderate)?

A
  • CT w/ embedded mineralized tissue

- Ginger-root shape

54
Q

What is the tx/prognosis of focal cemento osseous dysplasia (moderate)?

A

Tx: biopsy –> tx most likely unnec.

Prognosis: good

55
Q

Florid cemento-osseous dysplasia is a _____ lesion that is _____ until its ulcerates resulting in ______

A

hypovascular

asymptomatic

sequestration

56
Q

What is the radiographic presentation of florid cemento-osseous dysplasia (severe)?

A

“cotton-wool” radiopacities in 2+ quadrants

57
Q

What are the complications of florid cemento-osseous dysplasia (severe)?

A

Prone to necrosis and secondary infection

Biopsy unnec.; surgical procedures should be avoided

58
Q

What is the tx/prognosis of florid cemento-osseous dysplasia (serious)?

A

Tx:

  • Asympt.: Recall/Prophy/OHI
  • Sympt: Debriedment and AB

Prognosis: good
-2nd infection may result

59
Q

What are the differential diagnoses of cemento-osseous dysplasia?

A
  • Hypercementosis: radiodensity within PDL
  • Idiopathic osteosclerosis: not nec. at apex; no radiolucent periphery
  • Benign cementoblastoma: root resorption and fusion with radiopaque mass

developmental bone disorders (1 decks)

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