ch 14 Bone Pathology Flashcards
Most common type of inherited bone disorder
Osteogenesis imperfect
Osteogenesis imperfecta is an impairment of what tissue maturation
type I collagen
What parts of the body are affected by OI
bone Dentin
Sclera Ligaments Skin
What is the bone character in O.I.
thin cortex, fine trabeculations, diffuse osteoporosis
How will bone heal in O.I.
heal, but with abundant callus
What are the radiographic hallmarks of O.I.
osteopenia Bowing
Angulation/deformed long bones Multiple fractures
Wormian skull bones
What are wormian bones
10 or more sutural bones that are 6X4mm in diameter or larger and mosaic patterned, but not specific to O.I.
O.I. can appear dentally identical to what other disorder
Dentinogenesis imperfect
How will the dentition look in both Osteogenesis Imperfecta and Dentinogenesis Imperfecta
blue to brown opalescent teeth
What should you call teeth that have the characteristic brown or blue opalescence? Should it be D.I. or O.I outright
No, just opalescent teeth until determined if only D.I.
What is the characteristic malocclusion in O.I. and why
1.) Class III due to maxillary hypoplasia
What is the most common, mildest form of O.I.
Type I
What is the most severe form of O.I.
Type II
Other physical characteristics of O.I.
Blue sclera
Hypoacusis(hearing loss)
Long bone & spine deformaties Joint hyperextensibility
Most severe form of O.I. noted in patients beyond
the perinatal period and has moderately severe to severe bone fragility
Type III
Type III O.I. patients die from cardiopulmonary complications from what
kyphoscoliosis = abnormal spin curvature in coronal and sagittal plane
O.I. type associated with mild to moderate bone fragility
Type IV
What happens to bone throughout life in O.I.
Cortical bone is attenuated
Reduced bone matrix production
Bone immature throughout life
Woven bone never transforms to lamellar
What is the primary goal of O.I. treatment
No cure, goal to improve symptoms (bisphosphonates later in life)
rare hereditary skeletal disorder characterized by marked increase in bone density
Osteopetrosis
Osteopetrosis cause
defect in osteoclast function
What causes the thickening of cortical bone and sclerosis of cancellous bone
defective osteoclast function + continued osteoblast function + endochondral bone formation
2 types of osteopetrosis
Infantile/Malignant Adult/Benign
Character of Infantile/Malignant Osteopetrosis
diffusely sclerotic skeleton Marrow failure
Frequent fracture (accusations of child abuse)
Cranial nerve compression (blind/deaf/facial paralysis)
Facial character of Infantile/Malignant Osteopetrosis
broad face Hypertelorism
Snub nose
Frontal bossing
Tooth eruption in Infantile/Malignant Osteopetrosis
Delayed
Common complication of tooth extraction in Infantile/Malignant Osteopetrosis
osteomyelitis
What are common in Infantile/Malignant Osteopetrosis even though bone is dense
pathologic fractures
How will bone look radiographically in Osteopetrosis
increased radiographic density with loss of distinction between cortical and cancellous bone
O.I with opalescent teeth normally show what
severe attrition
Which part of skeleton has significant sclerosis in Adult/Benign Osteopetrosis
axial skeleton, long bones not as sclerotic
When is Adult/Benign Osteopetrosis diagnosed and how
dental radiographs by increased radiopacity of medullary portions of bone
How do patients that are symptomatic with Adult/Benign Osteopetrosis present
with pain
Most common treatment with best prognosis for Infantile Osteopetrosis
bone marrow transplant
Secondary treatment for Osteopetrosis that decreases bone mass, decrease infection prevalence, and lower frequency of cranial nerve compression
Interferon gamma 1b with calcitriol
Cleidocranial dysplasia affects what type of bone formation
endochondral ossification
Most prominent abnormalities of Cleidocranial dysplasia affect what bones
clavicles and skull
Absent/hypoplastic clavicles with underdeveloped neck muscles allowing shoulders to be approximated in front of the chest is characteristic of
cleidocranial dyplasia
Physiologic appearance of pt with Cleidocranial dysplasia
short stature
Large head
Prominent frontal and parietal bossing Hypertelorism
Broad nasal base and depressed nose bridge
Skull bone character in Cleidocranial dysplasia
delayed or never closed fontanels with sutures featuring wormian bones
Palate character in Cleidocranial dysplasia patient
narrow, high arched palate
Increased prevalence cleft palate
Teeth character of Cleidocranial dysplasia
prolonged deciduous retention, permanent eruption failure, numerous unerupted supernumand permanent teeth with dilacerated roots or abnormal crowns
What do Cleidocranial dysplasia teeth lack that may be associated with eruption failure
Lack of secondary cementum
Cleidocranial dysplasia mandible character on radiograph
coarse trabeculation
Narrow ascending rami
Narrow pointed coronoid process
Cleidocranial dysplasia maxilla character on radiograph
Thin zygomatic arch
Hypoplastic maxillary sinus
Profile of Cleidocranial dysplasia patient as they age
short lower face height
Acute gonial angle
Anterior incline of mandible
Prognathic
Treatment of choice for Cleidocranial dysplasia dentally
removal of primary and supernum teeth followed by exposure of permanent teeth to be extruded orthodontically
An area of hematopoietic marrow that is sufficient in size to produce an area of radiolucency that may be confused with an intraosseous neoplasm (i.e. it is not a pathologic process)
Focal Osteoporotic Marrow Defect
Demographics of Focal Osteoporotic Marrow Defect
Post menopausal females, posterior mandible, endentulous areas
Is there expansion in the mandible from Focal Osteoporotic Marrow Defect
No
How treat Focal Osteoporotic Marrow Defect
incisional biopsy for diagnosis, if it is FOMD, then no further treatment required
Focal area of increased radiodensity that is of unknown cause and cannot be attributed to any inflammatory, dysplastic, neoplastic, or systemic disorder
Focal Idiopathic Osteosclerosis
What must do to the tooth if see a radiopaque lesion at the root apex
Test Tooth vitality
If teeth are nonvital with testing then what should be the diagnosis of the radiopacity at the root apex
condensing osteitis or focal chronic sclerosis osteomyelitis
When does Focal Idiopathic Osteosclerosis begin and end
begins 1st to 2nd decade and ceases with bone maturity ~ 4th decade
Where does Focal Idiopathic Osteosclerosis normally appear
90% mandible, most common near 1st molar, but also common in 2nd PM and 2nd Molar
What should be included in the differential diagnosis if more than one area of osteosclerosis is noted
multiple osteomas associated with Gardner’s syndrome
Radiographic character of Focal Idiopathic Osteosclerosis (size, shape, rim)
radiopaque 3mm-2cm, well-defined round to irregular with no rim
Is root resorption common in Focal Idiopathic Osteosclerosis
No
Radiographic character of Focal Idiopathic Osteosclerosis
dense lamellar bone with scant fibrofatty marrow
Treatment for Focal Idiopathic Osteosclerosis
serial radiographs until growth stops, then no treatment required
What would be an indication for biopsy with focal idiopathic osteosclerosis
if cortical expansion occurs (indicates a neoplasm)
Abnormal and anarchic resportion and deposition of bone resulting in distortion and weakening of the affected bones
Paget’s Disease (Osteitis Deformans)
Paget’s disease demographics
more common in Britain, after 4th decade, Males > women, Whites > blacks
Most commonly affected bones in Paget’s
lumbar vertebrae
Pelvis
Skull
Femur
Can Paget’s jump a joint space to a previously unaffected bone
No
Can just the mandible be affected in Paget’s
No, maxilla must be involvd if mandible involved
Paget affected bone character
thickened, enlarged, weakened
Characteristic stance of Paget’s disease and why
weight bearing bones bow, resulting in Simian stance
Paget’s disease head circumference
Large head circumference
Term for extreme cases of maxillary expansion in Paget’s disease
leotiasis ossea (gross expansion of midface)
Phase I of Paget’s Disease is osteolytic or osteoblastic
Osteolytic
What is osteoporosis circumscripta
waves or fronts of progressive demineralization occur in skull in Paget’s disease
What is the cause of the increased cranial circumference in Paget’s disease
expansion of diploic table of skull
Phase II of Paget’s Disease is osteolytic or osteoblastic
osteoblastic
Descriptive term of the radiographic appearance of Phase II Paget’s Disease with patchy areas of sclerotic bone
cotton wool appearance
Character of Phase III Paget’s Disease
mixed osteolytic/osteoblastic, but no coordination between the two
What is pathognomonic for Paget’s Disease
Generalized hypercementosis
What type of root resorption is seen in Paget’s Disease
external root resorption
Change in teeth due to Osteoblastic (Phase II) of Paget’s disease
diastema
What other pathology is included in the differential when diagnosing Paget’s Disease
cement-osseous dysplasia
Consideration of Pagetoid bone
hypervascular, therefore poor hemostasis, sensitive to inflammation, high output cardiac failure (?)
Malignant bone tumor associated with Paget’s disease (because it is rare to find in someone older than 40)
Osteosarcoma
What is required for diagnosis of Paget’s disease
clinical, radiographs, lab tests (high alkaline phosphatase among other things)
What should patient be sent for once diagnosed with Paget’s disease
Bone scintography to detect skeletal involvement
What is histologically characteristic of Paget’s disease
numerous basophilic reversal lines forming mosaic or jigsaw pattern
What replaces marrow in Pagetoid bone
highly vascular fibrous connective tissue
Common treatment for the bone pain common in Paget’s disease
NSAID
What can result due to compression of cranial nerves during Phase II (osteoblastic) Paget’s
deafness, blindness
What can be prescribed to reduce bone resorption
parathyroid hormone antagonist (calcitonin or bisphosphonates)
What pagetoid bone is an osteosarcoma likely to occur in
pelvis or lower extremities
What tumors normally develop in Pagetoid bone in the craniofacial skeleton
benign and malignant giant cell tumors
Character of anterior Mandibular Central Giant Cell Granuloma
anterior can cross midline
Central Giant Cell Granuloma commonly found before what age and in what gender
before age 30, females
majority presentation of Central Giant Cell Granuloma
asymptomatic, but have swelling
Are central giant cell granulomas aggressive, fast growing, perforate cortical plate, and do they cause root resorption
No, nonaggressive slow growing with no cortical expansion and no root resoprtion (Nonaggressive Central Giant Cell Granuloma)
Can Central giant cell granulomas be aggressive and grow quickly causing perforation of the cortical plant and root resorption
Yes (Aggressive Central Giant Cell Granuloma)
Central Giant Cell Granulomas can be what flavor (unilocular or multilocular)
Either unilocular or multilocular
Central Giant Cell Granulomas are radiopaque or radiolucent
radiolucent with well-defined rim, but rim is not corticated (does not have a definite white line)
What is a differential diagnosis for a multilocular radiolucent lesion of the jaw
Central Giant Cell Granuloma
Ameloblastoma
Unilocular radiolucent lesion of the jaw differential diagnosis
Central Giant Cell Granuloma
Periapical Granuloma
Cyst
Giant Cell Granulomas are histopathologically identical to what tumor associated with hyperparathyroidism
brown tumors
Common histology in all Central Giant Cell Granulomas
multinucleated giant cells in background of ovoid to spindle mesenchymal cells & round Macrophages
Before preceeding with treatment of Central Giant Cell Granuloma, what disease must be first ruled out
hyperparathyroidism (because brown tumor of hyperparathyroidism is histologically identical to Central Giant Cell Granuloma)
What disease should be investigated in a child that has multiple foci of apparent central giant cell granuloma and why
Cherubism. Because Central Giant Cell Granuloma normally occurs as 1 lesion while Cherubism is bilateral lesions presenting commonly at age 2-5
Treatment of nonaggressive Central Giant Cell Granuloma
Curettage (has 15%-20% recurrence)
3 alternatives to surgery for Aggressive Central Giant Cell Granuloma
Corticosteroids
Calcitonin
Interferon alpha-2a
Where do giant cell tumors normally occur in the body
epiphysis of long tubular bones
Rare developmental condition of painless bilateral expansion of the posterior mandible involving the angles and ascending rami
Cherubism
Cherubism: genetic or random mutation
can be autosomal dominant genetic transfer or spontaneouls mutation
Cherubism usually manifests at what age range
2-5 years
Besides the chubby cheeks, what is the other physiologic feature that leads to the naming of Cherubism
“eyes upturned to heaven) appearance due to infraorbital rim and orbital floor involvement tilting eye up while pulling lower lid down exposing more sclera below iris
When will the lesion regress
stabilize and slowly regress at puberty
What is the diagnostic (pathognomonic) radiography for Cherubism
multilocular expansile radiolucency with a bilateral symmetrical presentation
Side effects of the bilateral symmetrical jaw expansion of cherubism
displaced teeth, delayed eruption, impaired mastication and speech
What is contraindicated for Cherubism treatment and why
No radiation therapy, rick of post irradiation sarcoma
Benign empty or fluid-filled cavity within bone that is devoid of epithelial lining
simple bone cyst
Where do Simple Bone Cysts commonly occur in the body
long bone