Chapter 14

Question 1

What are some signs that bone lesions are benign?

Answer 1

Asymptomatic, grows slowly (corticated rim) and by expansion (displaces teeth and expands cortex), symmetrical, and does not metastasize

Question 2

What are some signs that bone lesions are malignant?

Answer 2

Symptomatic, grows rapidly, invades and destroys adjacent structures, asymmetrical, ragged/poorly defined margins (destroys cortex), capable of metastases

Question 3

What term describes a group of heritable disorders that has defective type I collagen maturation?

Answer 3

Osteogenesis Imperfecta

Question 4

What are the characteristics of bone in osteogenesis imperfecta?

Answer 4

Thin cortex, fine trabeculation, diffuse osteoporosis, and fractures heal inappropriately

Question 5

When is osteogenesis imperfecta usually noticed?

Answer 5

At birth, as infants are born with multiple bone fractures

Question 6

How many people are affected with osteogenesis imperfecta?

Answer 6

About 1 in 8000

Question 7

What are some signs and symptoms of osteogenesis imperfecta?

Answer 7

Bone fragility, blue sclera, altered teeth, hearing loss, long bone and spine deformation, joint hyperextensibility

Question 8

What are some radiographic features of osteogenesis imperfecta?

Answer 8

Osteopenia (low bone density), bowing/angulation/deformity or long bones, multiple fractures, wormian bones in the skull

Question 9

What are some oral manifestations of osteogenesis impefecta?

Answer 9

Teeth with a blue to brown translucency (opalescent), premature pulpal obliteration, class III malocclusion

Question 10

What is the goal of therapy for osteogenesis imperfecta?

Answer 10

Manage symptoms, manage fractures (so patients may be on bisphosphonates)

Question 11

What term describes a skeletal disorder with a marked increase in bone density?

Answer 11

Osteopetrosis (marble bone disease)

Question 12

What is the cause of osteopetrosis?

Answer 12

A defect in remodeling caused by a failure of normal osteoclast function

Question 13

What are the 2 major clinical patterns of osteopetrosis?

Answer 13

Infantile and Adult osteopetrosis

Question 14

What is the difference between infantile and adult osteopetrosis?

Answer 14

Infantile is discovered early on and has severe manifestations with a poor prognosis. Adult osteopetrosis is discovered later in life is has less severe manifestations, with a long term survival

Question 15

What are some of the manifestations of infantile osteopetrosis?

Answer 15

Widespread increase in skeletal density, marrow failure, frequent fractures, cranial nerve compression, facial deformities, delayed tooth eruption, osteomyleitis in post tooth infraction

Question 16

What is another name for adult osteopetrosis?

Answer 16

Benign osteopetrosis

Question 17

What are some significant oral complications that are a result from osteopetrosis?

Answer 17

Fracture and osteomyelitis after tooth extraction

Question 18

What term describes a syndrome characterized by dental and clavicle abnormalities?

Answer 18

Cleidocranial dysplasia

Question 19

How many people are affected by cleidocranial dysplasia?

Answer 19

1 in 1,000,000

Question 20

Are the clavicles usually present in cleidocranial dysplasia?

Answer 20

Yes, but they show varying degrees of hypoplasia (they are absent in 10% of cases)

Question 21

What are some characteristics of people with cleidocranial dysplasia?

Answer 21

Short stature, big head with pronounce frontal bossing, ocular hypertelorism, broad base of nose, unusual mobility of shoulders

Question 22

What are some oral manifestations associated with cleidocranial dysplasia?

Answer 22

High arched palate, increased prevalence of cleft palate, retention of deciduous teeth, delayed eruption of permanent teeth, and supernumerary teeth

Question 23

What is the treatment for cleidocranial dysplasia?

Answer 23

No treatment exists for bone anomalies, and treatment of dental problems is difficult

Question 24

What term describes an area of hematopoietic marrow that produces a radiolucency?

Answer 24

Focal osteoporotic marrow defect

Question 25

What is the clinical presentation of a focal osteoporotic marrow defect?

Answer 25

A lesion of variable size with ill defined borders and no jaw expansion, typically asymptomatic

Question 26

Where and in what population do focal osteoporotic marrow defects usually occur?

Answer 26

75% in woman in the posterior mandible

Question 27

How is a focal osteoporotic marrow defect diagnosed?

Answer 27

Incisional biopsy, but once dx is confirmed no tx is necessary

Question 28

What term describes a focal area of increased radiodensity that is of unknown causes and cannot be attributed to anything else?

Answer 28

Idiopathic osteoscleorosis

Question 29

What are some other conditions that may be confused with idiopathic osteosclerosis, and why are they different from idiopathic osteosclerosis?

Answer 29

Condensing osteitis - infection; focal cemento-osseous dysplasia, has radiolucent rim; cementoblastoma - is fused with the tooth

Question 30

How many Americans have idiopathic osteosclerosis?

Answer 30

About 5% of Americans

Question 31

When does idiopathic osteosclerosis usually occur?

Answer 31

Cases arise in teenage years, then remain static

Question 32

What are the clinical characteristics of idiopathic osteosclerosis?

Answer 32

Asymptomatic, no cortical expansion, well defined

Question 33

Where does idiopathic osteosclerosis usually occur?

Answer 33

In the mandible, and about 80% are associated with the root apex

Question 34

When should idiopathic osteoscerosis be biopsied?

Answer 34

If there are symptoms, continued growth or cortical expansion

Question 35

What term describes a disease characterized by abnormal resorption and deposition of bone, and is of unknown cause?

Answer 35

Paget's disease of bone

Question 36

What does Paget's disease cause?

Answer 36

Distortion and weakening of affected bones = bowing, simian stance (monkey like stance)

Question 37

What population is most commonly affected by Paget's disease of bone?

Answer 37

Older adults, males, caucasians

Question 38

What is a common complaint of Paget's disease of bone?

Answer 38

Bone pain, osteoarthritis, joint pain and limited mobility

Question 39

What bones are most commonly affected by Paget's disease of bone?

Answer 39

Vertebrae, pelvis, skull and femur

Question 40

In how many cases does jaw involvement occur in Paget's disease of bone?

Answer 40

About 20%

Question 41

What does jaw involvement in Paget's disease of bone result in?

Answer 41

Maxillary disease is more common than mandibular, resulting in an enlargement of the middle 1/3 of the face = leontiasis ossea (lionlike face); alveolar ridges are symmetrical and grossly enlarged

Question 42

What are some radiographic characteristics of Paget's disease of bone?

Answer 42

Patchy or sclerotic areas (cotton wool appearance), general hypercementosis (premolars usually affected)

Question 43

What diagnostic test can be done to confirm Paget's disease of bone?

Answer 43

A blood test = high elevations of alkaline phosphatase levels with normal calcium and phosphorous levels

Question 44

How is Paget's disease of bone treated?

Answer 44

NSAIDs for pain, antiresorption agents for those with phosphatase levels above normal (bisphosphonates)

Question 45

What is a recognized complication of Paget's disease of bone?

Answer 45

Development of a malignant bone tumor (osteosarcoma) in up to 10% of cases = poor prognosis

Question 46

What term describes a non-neoplastic lesion of bone, that causes painless bone expansion from medullary bone?

Answer 46

Central giant cell granuloma

Question 47

At what age and in what population are central giant cell granulomas more common?

Answer 47

Before 30, and in females

Question 48

Where are central giant cell granulomas most common?

Answer 48

In the mandible, anterior and they frequently cross the midline!

Question 49

What are some radiographic features of a central giant cell granuloma?

Answer 49

Radiolucent lesion which may be unilocular or multilocular, typically well defined with noncorticated margins

Question 50

What must patients be evaluated for if they have a central giant cell granuloma?

Answer 50

Hyperarathyroidism -- histopathology identical to brown tumor of hyperparathyroidism and lesions of cherubism

Question 51

How is a central giant cell granuloma treated?

Answer 51

Currettage

Question 52

How likely is a central giant cell granuloma to recur?

Answer 52

About 20% recur

Question 53

What is cherubism?

Answer 53

A developmental, autosomal dominant jaw condition that involves the bilateral posterior mandible, and the infraorbital rim/orbital floor = chubby cheeks, wide rim of exposed sclera

Question 54

When does cherubism occur?

Answer 54

Between the ages of 2-5

Question 55

What is unique about the progression of cherubism?

Answer 55

The disease will progress until puberty then stabilize and slowly regress

Question 56

What are some oral manifestations of cherubism?

Answer 56

Widening and distortion of the alveolar ridges, and failure of tooth eruption

Question 57

What are some radiographic characteristics of cherubism?

Answer 57

Multilocular, expansile and radiolucent lesions

Question 58

What term describes a benign, empty or fluid filled cavity within bone?

Answer 58

Traumatic bone cyst, aka simple bone cyst

Question 59

Why is the name "traumatic bone cyst" a misnomer?

Answer 59

Because the lesion does not have an epithelial lining

Question 60

What is the cause of a traumatic bone cyst?

Answer 60

Trauma-hemorrhage theory is the most widely accepted etiology -- clot forms and dissoves but never triggers bone formation

Question 61

What is the most common location of tramautic bone cysts?

Answer 61

Long bones, but some are found in the mandible of patients aged between 10 and 20

Question 62

What are some clinical and radiograhic characteristics of a traumatic bone cyst?

Answer 62

Asymptomatic, vital teeth, with a well defined, radiolucent defect with scalloping around teeth

Question 63

How is a traumatic bone cyst treated?

Answer 63

Surgical exploration to confirm diagnosis and is usually curative

Question 64

What term describes a blood filled cavity within bone?

Answer 64

Aneurysmal bone cyst

Question 65

Why is the term "aneurysmal bone cyst" a misnomer?

Answer 65

Because it is not a true cyst because it has no epithelial lining

Question 66

Where do aneurysmal bone cysts usually occur?

Answer 66

In long bones, but rarely in the posterior mandible of patients around 20

Question 67

What are some clinical and radiographic findings of an aneurysmal bone cyst?

Answer 67

Painful, rapid swelling with a well defined, radiolucent lesion with cortical expansion, usually unilocular = "blow out" or ballooning distention of the affected bone (BLOOD SOAKED SPONGE)

Question 68

How is an aneurysmal bone cyst treated?

Answer 68

Curretage or enucleation

Question 69

How likely is an aneurysmal bone cyst to recur?

Answer 69

About 10-60%, usually due to inadequate removal of the first lesion