bone pathologies Flashcards
Osteogenesis imperfecta (OI)
most common
mutation in genes that form collagen
bone fragility and repeated bone fractures
brittle bone disease
type I collagen (impaired collagen synthesis)
dentinogenesis imperfecta, blue sclera
OI type I
mild from insufficient quantity, normal quality blue sclera open bite wormian bones
OI type II
lethal form
insufficient quality and quantity
dead within first month of life
OI III
defective quality
muscle weakness, growth retardation, early death
OI IV
defective quality
do not fit other categories
short stature, period of fractures
dentiogenesis imperfecta (DI)
hereditary
tooth discoloration (opalescent)
enamel fracture
bulbous crowns
DI type I
associated with OI
primary teeth more than permanent
DI type II
not associated with OI
both primary and permanent equally affects
DI type III
thin dentin, enormous pulp chamber
shell teeth
extremely rare
osteopetrosis
hereditary
marked increase in bone density
failure of normal osteoclast function
skeletal fragility, haematopoietic insufficiency, nerve entrapment syndrome, growth impairment
infantile osteopetrosis
poor prognosis failure to thrive and grow cranial nerve entrapment delayed dentition pancytopenia (defective osseous tissue tends to replace bone marrow which may lead to bone marrow failure)
adult osteopetrosis
good prognosis
diagnosed based on radiographs
bone pain, fractures, cranial entrapment
bone marrow functions normally
cleidocranial dysplasia (CCD)
genetic
affect CBFA1 (guiding intramembranous bone formation and dentinogenesis)
shoulder hyper mobility
multiple unerupted teeth
CCD jaw changes
high palatal arch
coarse trabeculation
mandibular rami is nearly parallel -sided anterior and posterior borders
coronoid processes may be slender and pointed with a distal curve
benign fibro-osseous lesions
focal or diffuse replacement of normal bone by fibrovascular tissue
bone is weakened and prone to expansion
FD, PCOD, FCOD
fibrous dysplasia (FD)
Monostotic (affecting a single bone)
Polyostotic (affecting several bones, cafe au lait spots=Jaffe type, hyperfunctioning endocrine, stunted growth, precocious puberty = McCune-Albright syndrome)
craniofacial type (maxilla, sinuses, occipital bone)
ground glass radio graphic appearance
Periapical Cemento-Ossifying Dysplasia (PCOD)
focal type florid type PCOD predilection (females, african americans, lower anterior mand region)
PCOD characteristics and stages
assymptomatic, incidentally on radiographs
Early lesions (radiolucency at apices of teeth)
lesions tend to mature (mixed lesion on radiographs)
seldom exceed 1.0cm in diameter
self-limiting
florid cemento-osseous dysplasia (FCOD)
multi-quadrant radio-opaque cementum like masses
assymptomatic
multiple masses within a peripheral radiolucent rim
Cherubim
genetic, rare
increased activity of osteoclasts and osteoblasts
bilateral expansion of the mand (bone is replaced with fibrovascular connective tissue)
early primary tooth loss, uneruption of permanent teeth
varying degrees of remission
Paget’s disease of bone (PD)
chronic condition
abnormal, woven bone (cotton-wool appearance)
sporadic and hereditary types
enlargement of skull bone
neuro-compression, elevated serum alkaline phosphate
males
50 yrs or older
chronic, slow progression (NSAIDs, calcitonin and bis-phosphonates)