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Week 77 > Bone pathology > Flashcards

Bone pathology Flashcards

1
Q

Osteogenesis Imperfecta

A
  • Mutation
    • COL1A1 (Chromosome 12)
    • COL1A2 (Chromosome 7)
  • Types:
    • Mild (type 1)
      • increased fracture of limbs
      • hearing loss or premature osteoporosis
    • Moderate (Type 3-9)
      • more bone fractures and deformities
      • Cardiovascular defects
      • short stature
      • hyperflexible
    • Lethal (type 2)
      • prenatal lesions
        • pulmonary failure
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2
Q

Osteogenesis Imperfecta: Bone Manifestations

A
  • Brittle bones
  • Short Stature
  • Basilar Skull deformities
    • pressure on cranial nerves
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3
Q

Osteogenesis imperfecta: Clinical Manifestations

A
  • Blue sclera
  • Wormian bones
  • increased Class III Malocclusion
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4
Q

Osteogenesis Imperfecta: Oral Manifestations

A
  • Opalescent teeth
    • wear quickly
    • Yellow/gray color
  • Radiograph:
    • CEJ constriction→Tulip shaped teeth
    • short and blunted roots
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5
Q

Osteopetrosis

A
  • aka: Marble Bone Disease
    • Erlenmeyer Flask Appearance
  • Types:
    • Malignant Type
    • Intermediate Type
    • Benign Type
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6
Q

Osteopetrosis: Malignant Type

A
  • aka autosomal recessive infantile type
  • Severe form
  • at birth/early age
  • Anemia
  • Hypertelorism (Broad Face)
  • widespread skeletal density (X-ray)
    • increased density in all bones
  • Poor prognosis (<1 year after birth)
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7
Q

Osteopetrosis: Intermediate Type

A
  • aka Autosomal Recessive Intermediate Type
  • Asymptomatic at birth
    • fractures appear at end of 1st decade
  • Less severe form of infantile
  • RANKL
  • RANK
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8
Q

Osteopetrosis: Benign Type

A
  • Aka Autosomal Dominant Adult Type
  • Most common type
  • Adolescents (10-19)
  • less severe manifestations
    • 40% asymptomatic
  • LRP5
    • bone formation by osteoblasts
  • CLCN7
    • chloride channel
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9
Q

Osteopetrosis: Management

A
  • No Tx=poor prognosis
    • die during 1st decade
  • Bone marrow transplant
  • Corticosteroids
  • limit calcium intake
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10
Q

Massive Osteolysis

A
  • Aka
    • Vanishing Bone Disease
    • Phantom Bone Disease
  • Mostly children and young adults
  • Spontaneous progressive destruction of 1+ bones
    • replaced w/vascular proliferation
    • fills w/fibrous tissue (does not regenerate/repair)
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11
Q

Massive Osteolysis: Clinical Features

A
  • mobile teeth
  • pain
  • malocclusion
  • deviation of mandible
  • obvious deformities
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12
Q

Massive Osteolysis: Radiographic Findings

A
  • Large portions of bone disappear
  • New area→before obvious radiolucency
    • loss of lamina dura
    • thinning of cortical plates
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13
Q

Massive Osteolysis: Management

A
  • Radiation Therapy
    • most successful
    • risk-post radiation sarcoma
  • Bisphosphonate Therapy
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14
Q

Cleidocranial Dysplasia

A
  • Autosomal Dominant mutation
    • RUNX2 gene on chromosome 6q21
  • Bone defects of:
    • clavicles
    • skull
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15
Q

Cleidocranial Dysplasia: Clinical Features

A
  • Short Stature
  • Long “Swan” Neck
  • Hypertelorism
  • Depressed Nasal Bridge
  • Delayed Closing of Skull sutures
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16
Q

Cleidocranial Dysplasia: Oral Manifestations

A
  • Maxilla:
    • thin zygomatic arch
    • Small/absent maxillary sinus
  • Numerous impacted teeth
    • permanent teeth delayed or failure to erupt
  • retention of deciduous teeth
  • Palate-Narrow, High arch
    • increased prevalence of cleft palate
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17
Q

Cleidocranial Dysplasia: Management

A
  • Full mouth extraction and give denture
  • Before adult
    • prevent lower face height and mandibular prognathism
18
Q

Focal Osteoporotic Bone Marrow Defect

A
  • area of hematopoietic marrow→ produce radiolucency
  • Asymptomatic
    • identified by accident
  • Women
  • Posterior Mandible
  • Incisional Biopsy
    • find bone marrow
    • necessary to establish dx
19
Q

Idiopathic Osteosclerosis

A
  • focally/focused increased bone density
    • unknown cause
  • must rule out
    • inflammation
    • dysplastic
    • neoplastic
    • systemic disorders
  • If multiple lesions-rule out Gardner Syndrome
20
Q

Paget’s Disease of Bone

A
  • aka osteitis deformans
  • 2nd most common metabolic bone disorder
    • osteoporosis=1st
  • Anarchic resorption and deposition of bone
    • result=distortion and weakened
  • Angio-Saxon ancestor
    • rare before 40 y.o.
  • most are asymptomatic
    • some pain due to turnover or secondary complications
21
Q

Paget’s disease of Bone: Clinical Manifestation

A
  • Monkey Leg Stance
  • Bone gets thicker and thinner over time
22
Q

Paget’s disease of Bone: Head and Neck Manifestations

A
  • Enlarged middle ⅓ of Face
  • generalized hypercementosis
  • Osteoporosis circumscripta
    • large radiolucency in skull
    • early stage
  • cotton wool appearance
  • Lincoln’s Sign (Black Beard)
    • Mandible
23
Q

Paget’s disease of Bone: Histology

A
  • Jigsaw Puzzle w/Basophilic reverse lines
    • “mosaic appearance”
      • looks like enamel formation
24
Q

Paget’s Disease of Bone: Complications

A
  • Hypercementosis
  • Osteomyelitis (late disease)
  • Osteosarcoma
  • Giant Cell tumors
25
Q

Central Giant Cell Granuloma

A
  • Not a true granuloma
  • Unknown Etiology
  • Type:
    • Non-aggressive: Mainly
    • Aggressive
  • Multifocal presentaiton
    • Cherubism
    • Hyperparathyroidism
26
Q

Central Giant Cell Granuloma: Non-aggressive granuloma

A
  • most common
  • small, asymptomatic
  • slow, painless jaw expansion
27
Q

Central Giant Cell Granuloma: Aggressive Type

A
  • rapid growth
  • painful
  • Cortical Proliferation
  • root resorption
  • tooth displacement
28
Q

Cherubism

A
  • Manifestation of Central Giant Cell Granuloma
  • Eyes turned up to heaven
  • 2 balls at chin
  • displaced teeth
29
Q

Hyperparathyroidism

A
  • manifestation of Central Giant Cell Granuloma
  • Brown tumor
    • involves:
      • mandible
      • clavicle
    • Secondary Hyperparathyroidism of chronic renal disease
  • Renal Osteodystrophy
    • striking enlargement of jaw
    • Secondary HPT
30
Q

Simple Bone Cyst

A
  • aka traumatic bone cyst
  • empty or fluid filled cavity in bone
    • no epithelium
  • Unknown Etiology:
    • Trauma-hemorrhage Theory
      • internal hematoma due to trauma
  • Mostly In long bone
    • Humorous
    • proximal femur
  • Painless swelling
    • posterior mandible
    • apex of teeth don’t have support
31
Q

Aneurysmal Bone Cyst

A
  • Intraosseous rdiolucency
    • blood filled
    • surrounded by fibrotic tissue and reactive bone
  • Classified as::
    • Primary-arising de novo
    • Secondary: associated with another bone disease
  • Oral Manifestations:
    • Rapidly enlarging swelling
      • Mandible>maxilla
      • cortical expansion and thinning
    • Blood-soaked sponge
  • Histology:
    • Contains Multinucleated Giant cells
32
Q

Fibrous Dysplasia

A
  • Benign
  • bone replace with cellular fibrous tissue and irregular trabeculations
  • Types:
    • Monostotic Fibrous Dysplasia
    • Polystotic Fibrous Dysplasia
33
Q

Monostotic Fibrous Dysplasia

A
  • Type of Benign Fibrous Dysplasia
    • mostly limited to 1 bone
    • “Ground Glass Appearance
    • Superior displacement of Inferior alveolar nerve is possible
    • Craniofacial Fibrous Dysplasia
34
Q

Polyostotic Fibrous Dysplasia:

A
  • Type of Benign Fibrous Dysplasia
    • 2+ bones
    • Dx before 10 y.o.
    • Painful
  • associated with:
    • Jaffe-Lichtenstein Syndrome
    • McCune-Albright Syndrome
    • Mazabraud syndrome
      • “Coast of maine”
35
Q

Cemento-Osserous Dysplasia

A
  • Most common Benign Fibrooseous lesion (BFOL) in clinic
    • tooth bearing areas
    • derived from:
      • PDL or…
      • bone=reactive lesion
    • Types:
      • Focal COD
      • Periapical COD
      • Florid COD
36
Q

Focal Cemento-Osseous Dysplasia

A
  • Type of COD
  • single site
    • mainly Posterior Mandible
  • Caucasian Females
  • mixed radiolucent and radiopaque pattern
    • well defined w/slightly irregular borders
  • Vitality test
37
Q

Periapical Memento-Osseous Dysplasia

A
  • Type of COD
  • Mainly Periapical region of anterior mandible
  • African American Females
  • Early Stage:
    • circumscribed radiolucency at apex
  • End Stage
    • circumscribed dense calcification surrounded by narrow radiolucent rim
38
Q

Florid Cements-Osseous Dysplasia

A
  • multifocal involvement (more than one focus)
  • African American female
  • middle aged
  • Dx:
    • multiple lesions w/vital anterior teeth
      • no need for biopsy?
39
Q

Familial Gigantiform Cementoma

A
  • Autosomal Dominant Trait
  • Significant facial deformity
    • limited to jaws-Maxilla and mandible
  • Radiograph:
    • look similar to COD
      • multiple radiolucencies in periodical regions
  • Tx:
    • reseection
    • if treated to early, may grow back
40
Q

Ossifying Fibroma

A
  • True neoplasm w/growth potential
    • mostly women
    • 3rd-4th decade
  • Mandible
    • premolar/molar area
  • commonly identified in pt w/Hyperthyroidism-Jaw Tumor
    *

Week 77 (11 decks)

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