3 - bone disorders Flashcards
4 inherited bone disorders
osteogenesis imperfecta
osteopetrosis (denser)
cleidocranial dysplasia
cherubism
5 acquired bone disorders
osteitis deformans (pagets) fibro-osseous lesions of the jaw (fibrous dysplasia, cemento-osseous dysplasia COD) osteroporotic bone marrow defect idiopathic psteosclerosis simple bone cyst
90% autosomal dominant inherited “brittle bone disease”
several RARE disorders of bone
defective collagen > abnormal bone mineralization > low bone density(osteopenia)
osteogenesis imperfecta
big spectrum, defective collagen gene
some sporadic cases
most common binherited bone disease
most common bone disorder and severity is on a spectrum
osteogenesis imperfecta
90% AD
blue sclerae
hearing loss
bones fragile and fracture easily - varies widely with the type of mutation
osteogenesis imperfecta
craniofacial alterations Class 3 occlusion, triangular face
bowing deformity long bones
osteogenesis imperfecta
osteogenesis imperfecta dental alterations identical to _
3 things
dentinogenesis imperfecta
blue, yellow, brown TRANSLUCENCE (more noticeable in primary teeth)
opalescent teeth
severe attrition leading to loss of VDO and potential tooth loss
osteogenesis imperfecta and dentinogenesis imperfecta can have similar tooth alterations and blue sclerae, but they are
the same or different mutations
they are distinct mutations, different diseases
dentinogenesis imperfecta is reserved for alterations isolated to the teeth
dental defects associated with OI should be designated as _ teeth
opalescent teeth
radiographic features
“shell teeth” or premature pulpal obliteration
narrow or “corn cob” shaped roots
Osteogenesis Imperfecta or dentinogenesis imperfecta
shell teeth - normal enamel thickness, thin dentin
tx for osteogenesis imperfecta
physiotherapy/rehab
orthopedic surgery
minimize factors leading to fracture
IV bisphosonates for children with mod/severe pain, reduce fracture rates
crown/bridge
full/partial dentures
implants(possibly IV bisphos prob not)
osteogenesis impefecta Px
depends on the mutation and expression of the gene
fractures bones heal - usually with _ (weaker)
abnormal callus formation
ranges from minimal bone deformity to essesntially normal growth to severe forms leading to death from passage thru the birth canal
Several forms - autosomal dominant and recessive (recessive more severe)
failure of osteoclasts to fxn normally - bone is not resorbed
continued bone formation and ossification - increased bone density
osteopetrosis
loss of hematopoietic precursor cells (pancytopenia)
increased susceptibility to infections and osteomyelitis
RARE
blindness and deafness due to cranial nerve compression
fractures
osteomyelitis (frequent complication of tooth extraction)
osteopetrosis - fucked up fxn of osteoclasts - densebone
tooth eruption is often delayed
radiograhic findings
diffuse density of skeleton
marrow spaces filled in by dense bone
tooth roots diffiuclt to visualize due to surrounding dense bone
failure of tooth eruption
osteopetrosis
uniformly sclerotic bone, failure of tooth eruption
TX: supportive measures (transfusions antibiotics)
bone marrow transplant - limit success
alternative therapies - interferon with calcitriol, restriction of Ca++ intake, steroids
osteopetrosis
AD can have longterm survival
poor Px for autosomal recessive form - usually die before 20years old
AD
spontaneous mutations up to 40%
also autorecessive as well
uncommon, affects skull, jaws and clavicles primarily
prominent forehead, hypo plastic face
cleidocranial dysplasia
long neck
shoulders narrow and droppings (hypoplastic or missing clavicles)
hypermobility of shoulders
primary dentition retained because permanent teeth do not erupt
numerous impacted and supernumerary teeth
plenty of teeth; not erupting in the correcting space or not erupting at all
cleidocranial dysplasia
prominent forehead, hypoplastic midface, long neck, dropping shoulders, missing clavicles
combined surgical and orthodontic care
correct skeletal relations, remover supernumerary teeth, correct alignment of permanent teeth
Px - good, life span is essentially normal
cleidocranial dysplasia
AD
spontaneous mutations occur
variable expressivity - lesions may cause tooth displacement, tooth mobility, failure of tooth eruption, impaired mastication, speech difficulties, upper airway obstruction, and vision or hearing loss
Cherubism
detected in childhood (2-7)
painless bilateral expansion of jaws, especially mandible
chubby cheeks
cherubism
chubby cheeks - cherubs depicted in Renaissance etchings
maxilla involved less frequently
involvement of inferior and/or lateral orbital wals may tilt the eyeballs upward and retract lower eyelid - “eyes upturned to heaven”
radiographic features
bilateral multiocular radiolucencies posterior mandible, maxilla
occasionally unilocular
often significant displacement of teeth
Cherubism
histopathologic features
cellular fibrous CT
sparse benign-appearing multinucleated giant cells
may see perivascular hyalinization 9 eosinophilic cuffing)
cherubism
Cherubism tx
optimal tx has not been determined
surgical intervention sometimes accelerates growth of lesions
many cases involute during puberty
px - difficult to predict for any given case
persistent facial deformity or continued disease progression possible
unknown etiology (genetic? envirconmental? virus?)
abnormal resorption and deposition, resulting distorted, weaker bone
affected bones become thickened
Osteitis deformans (paget disease)
acquired bone disorders
often discovered incidentally on routine blood test or dental radiographs, elevated serum alkaline phosphatase
primarily Anglo-Saxon ancestry
2:1 male or female predilection
Osteitis deformans (paget)
2;1 male predilection, older patients
rare <40
“Simian” monkey like stance if femurs involved - bowing of legs
most cases polyostotic
bone pain in up to 40% of cases
Osteitis deformans - paget
skull involvement - progressive enlargement, “hat won’t fit”
jaws involved 17% of cases - thickened, enlarged alveolar bone “dentures don’t fit”
acquired Osteitis deformans - pagets
radiographic features
“cotton wool” appearance of bone - “moth-eaten”
may have extensive hypercementosis
Osteitis deformans - pagets
histopathologic features:
“Mosaic” pattern of irregular trabeculae with resting and reversal lines (from abnormal resorption and deposition)
osteoblastic rimming
marrow replaced by vascular fibrous CT
osteitis deformans (paget)
osteitis deformans
tx for osteitis deformans - pagets
if asymptomatic - no tx
bisphosphonates
Osteitis deformans - pagets
Px - chronic and progressive, but usually no life-threatening
patients should be monitored for development of a giant cell tumor or malignant tumors, especially _
especially osteosarcoma
difficulty extracting teeth with hypercementosis and/or ankylosis
Hemorrhage during vascular/lytic phase
poor wounding healing, increased susceptibility to osteomyelitis during avascular/sclerotic phase
osteitis deformans - pagets
new dentures may be required periodically
although successful implant placement reported in a few cases, Px generally unfavorable and more so with use of bisphosphonates
diverse group varying etiologies
developmental(harmartomatous)
reactive
dysplastic
neoplastic
fibro-osseous lesions
fibrous dysplasia
cemento-osseous dysplasia COD (periapical, focal, florid)
Fibro-osseous lesions
post-zygotic mutation: pluripotent stem cell, skeletal progenitor cells, post-natal life
time of occurrence of mutation determines extent
Fibrous dysplasia
Fibro-Osseous lesions
presents ~1st or 2nd decade (younger than Paget disease)
no gender predilection
polyostotic or monostotic (many or one bone)
75-80% monostotic
Fibrous Dysplasia
Fibrous-osseous lesions
painless, unilateral swelling
slow growth
jaws among most commonly affected bones
MAXILLA more than mandible
fibrous dysplasia
craniofacial lesions may involve adjacent facial bones
results in marked facial deformity
radiographic features
early stages radiolucent or mottled opacify as they grow obliteration of maxillary sinus class "ground glass" pattern poorly defined, blending margins
fibrous dysplasia of fibro-osseous lesions
2 types of polyostotic fibrous dysplasia type
2 or more bones affected, and cafe-au-lait spots with jagged borders (coast of Maine)
Jaffe type Fibrous Dysplasia
Fibrous dysplasia type
2 or more bones affected by fibrous dysplasia, cafe-au-lait spots and endocrine disturbances - precocious puberty
McCune-Albright type
Histopathologic features
irregular-shaped trabeculae of immature (woven) bone in cellular fibrous stroma “Chinese characters”
no capsule - abnormal bone fuses to adjacent normal bone
fibrous dysplasia
TX Fibrous Dysplasia
none if lesion is small
delay surgery until disease _
radiation CONTRAINDICATED
delay surgery until disease quiescent
surgical reduction if cosmetic or fxnal problem
En-bloc resection
Px
sometimes disease stabilizes with skeletal maturation
spontaneous regression has been reported
25%-50% surgically treated lesions show regrowth, especially younger pts
malignant transformation rare, usually in lesions which have received radiation
fibrous dysplasia
Proteus syndrome is _ man disease
elephant man disease
Fibro-osseous lesions
benign, possibly reactive process
may originate from fibroblasts of PDL vs defect in bone remodeling
most common fibro-osseous lesion encountered in clinical practice of dentistry
Cemento-osseous dysplasia COD
acquired bone disorder
periapical COD
Focal COD (most common white female)
Florid COD
term may be changing to “osseous dysplasia” instead of COD
Fibrous-osseous dysplasia
Biopsy multiple small gritty fragments obtained
most common in females but can affect both genders and any ethnic groups
cemento osseous dyspalsia - more common in female
black> east asian>white
focal type reported to be more common in white females
usually found incidentally on x-ray tooth-bearing areas of jaws asymptomatic swelling, discomfort unusual TEETH TEST VITAL
COD - cemento-osseous dysplasa
radiographic features
ranges from completely radiolucent to densely radiopaque with a thin radiolucent rim (PDL remains intact)
may be associated with simple bone cyst
Cemento-osseous dysplasia C)D
_ COD shows multiple cotton wool type radiopacities in at least 2 quads of the jaws
may
Florid COD
Focal - white women
Cemento-osseous dysplasia - COD spectrum of severity
Periapical - mild
focal - moderate (white women)
Florid - severe - cotton wool in 2 quads
_ COD
mandibular anterior region usually, maxillary anterior as well
middle-aged black females especially
initially unilocular radiolucencies at apices, central opacity develops gradually
asymptomatic
Periapical COD - mild
periapical COD can be confused with hypercementosis(radiodensity is all within the PDL),
idiopathic osteosclerosis (no radiolucent rim, borders blend with surrounding trabeculae)
, benign cementoblastoma (radiolucent rim is contigous with PDL and PDL is not intact at he involved portion of the root)
_ COD
more often affects white females; may be biased by studies
body of mandible
most common 20-40 younger demographic
unilocular radioluceny or radiopacity with thin radiolucent rim
asymptomatic
focal COD
can be confused with an ossifying fibroma, a true neoplasm, but focal COD is seen more often (similar demographic - younger adult women)
_ COD
middle-age or older black females
multiple quads of jaws affected
generally asymptomatic, unless overlying mucosa ulcerates, resulting in bony sequestration (eg from ill-fitting denture)
Florid COD - most severe
yellowish, avascular cementum-like material is beginning to exfoliate thru the oral mucosa
DENTAL IMPLANT PLACEMENT NOT RECOMMENDED
lesions tend to be hypovascular (prone to necrosis, infection, osteomuelitis with minimal provocation)
recuded ability to heal
Histopathologic features:
cellular fibrous CT with embedded mineralize tissue resembling either immature(woven) bone or cellular cementum
fragmented specimen
mineralized product resembles GINGER ROOT
cemento-osseous dysplasia
mature lesions have more mineralized product than cellular stroma
FLORID COD can show densely mineralized tissue with necrotic debris and inflammation
Cemnto-osseous dysplasia dx based on clinical and radiographic feactures
can be confirmed by Bx if indicated
ginger root
vital teeth
spicules of bone and cementum like hard tissue within moderately cellular fibrous CT
TX for COD
none indicated for _ COD
regular vists for dental prophy and OHI to prevent periodontal disease and need for extractions
good fit of dentures
none indicated for periapical COD
Bx for focal to ruleout other disease, Bx not necessary for Florid COD
encourage patients to retian their teeth
ideally avoid surgical procedures - onset of symptoms asssociated with exposure to sclrotic bone to oral cavity
tx for COD symptomatic patient with secondary osteomyelitis
is difficult
debridement
antibiotics - often not effective
CHX rinse
fibro-osseous lesions COD Px
excellent for _ and _, although initial appearance of _ COD may be first sign of _ COD
generally good fo _ COD
COD malginant transformation common or rare?
excellent Px for periapical and focal
focal might be first sign of florid
generally good for florid
guared if secondarily infected requireing debridement and ATB
reported, but RARE
area of hematopoietic bone marrow of sufficient size to cause a radiographic radiolucency
pathogenesis unknown
may resemble metastatic disease
acquired
osteoporotic bone marrow defect
usually posterior body of mandible often at old extraction site usually middle age FEMALE incidentally on radiograph asymptomatic radiolucency
osteoporotic bone marrow defect
radiolucency can appear circumscribed, but on closer inspection may show ill-defined borders and a fine trabecular pattern
radiolucency can appear circumscribed, but on closer inspection may show ill-defined borders and a fine trabecular pattern, old extraction site, females,
osteoporotic bone marrow defect
histopathologic features
fatty and hematopoietic marrow
no abnormal osteoblastic or osteoclastic activity
maybe megakaryocytes
osteoporotic bone marrow defect
Osteoporotic bone marrow defect Tx and Px
Bx often indicated to establish Dx
Px - excellent once Dx’d, no further tx needed
dense bone island, Enostosis, bone whorl, focal periapical osteopretrosis, bone scar
Idiopathic osteosclerosis
focally increased area of dense bone
unknown cause
usually found incidentally on radiograph
asympotmatic
remain static or slowly enlarge - usually stabilize at skeletal maturity (occasionally regress)
acquired
Idiopathic osteosclerosis
no gender predilection
no expansion
3rd decade!!
radiographic features
radiopaque!
mandibular premolar/molar area most common site
borders blend with surround trabeculae, BUT occasionally may be sharp
Idiopathic osteosclerosis
borders will be blended for the most part with surround trabecular bone
does Idiopathic sclerosis cause tooth displacement
rarely does
usually does not
lamina dura and PDL fine - vital teeth
idiopathic osteosclerosis and fibrous dysplasia are well or poorly defined on CT
IO - well defined on CT usually, blended on xray
fibrous dysplasia is poorly defined on CT
in the past this acquired bone disorder was not distinguished from inflammatory or other lesions; so it may be confused with: condensing osteitis, hypercementosis, cementoblastoma
idiopathic osteosclerosis
condensing osteitis - big lesion appearing pulp and widen PDL change inflammation bone at apex
hypercementosis - within PDL
cementoblastoma - PDL around root of tooth and surround lesion - classic circle dome of endo
Histopatholgoic features
dense vital bone
may see fibrofatty marrow
Idiopathic osteosclerosis
tx for idiopathic osteosclerosis
none indicated unless _ or _
Px?
none indicated unless symptoms or cortical expansion
if noted in childhood, periodic radiographs until lesion stabilizes, Bx if confirmation needed to establish Dx
Px = excellent, not likely to progress or change in adulthood
empty or fluid filled bone cavity
lacks an epithelial lining, therefore a pseudocyst
usually an incidental findings
simple bone cyst
other terms traumatic bone cyst, hemorrhagic bone cyst
etiology of simple bone cyst
unknown - many theories
trauma-hemorrhage theory - trauma causing hematoma but not fracture - without subsequent organization and repair of hematoma - instead liquefies
trauma not a consistent finding
seen in 1st or 2nd decade
no gender predilection in jaws but in other bones, male
posterior mandible and symphysis
typically asymptomatic and no expansion
simple bone cyst
radiographic features
well delineated
usually unilocular, can be multilocular
often scallops betwen roots( sugggestive but not dx)
simple boen cyst
histopatholgical features
difficult to obtain specimen; usually just fragments of bone
microscopically these bone fragments are lined by inflamed granulation tissue, NO EPITHELIAL lining
loose CT and Bone
simple bone cyst
“teenage male with radiolucenyc right mandible”
simple bone cyst tx and Px
surgical exploration and curetage to induce bleeding (empty cavity within bone is found at time of surgery)
causes hemorrhage which organizes and lesions heals
Px = normal radiographs ~12-17 months postop
periodic radiographs warranted until complete resolution
recurrence rate low - up to 27% though
patients with _ disease have an increased risk of malignant tumors, especially osteosarcoma
Paget disease
