Bone Disorders Flashcards
4 Inherited Bone Disorder
- Osteogenesis Imperfecta
- Osteopetrosis
- Cleidocranial Dysplasia
- Cherubism
5 Acquired Bone Disorders
- Osteitis Deformans (Paget Disease)
- Fibro-Osseous Lesions of the Jaw (Fibrous dysplasia and Cemento-osseous dysplasia)
- Osteoporotic bone marrow defect
- Idiopathic osteosclerosis
- Simple bone cyst
“Brittle bone disease”
Osteogenesis Imperfecta
Osteogenesis Imperfecta has to do with defective ____
Collagen
Defective collagen –> Abnormal bone ____ –> Low bone ____
Mineralization
Density (osteopenia)
Most cases of Osteogenesis Imperfecta are autosomal ____
Dominant
One of the most heritable bone disorders
Osteogenesis Imperfecta
Clinical features of Osteogenesis Imperfecta
- Bones fragile
- Blue sclera
- Hearing loss
- Bowing deformity
- Craniofacial alterations
Dental alterations in Osteogenesis Imperfecta are identical to ____ _____
Dentinogenesis Imperfecta
3 dental alterations in Osteogenesis Imperfecta
- Blue/Yellow/Brown translucence
- Opalescent teeth
- Severe attrition leading to loss of VDO and potential tooth loss
Dental defects associated with Osteogenesis Imperfecta should be designated as ___ ___
Opalescent teeth
Term reserved for alterations isolated to the teeth
Dentinogenesis imperfecta
Radiographic features of Osteogenesis Imperfecta
“Shell teeth” or premature pulpal obliteration
Narrow or “corn-cob” shaped roots
Treatment for dentition of Osteogenesis Imperfecta patients
Crown/bridge, partial/complete dentures/ implants/ orthognathic surgery, orthodontics
Prognosis of Osteogenesis Imperfecta
Variable
Minimal bone deformity –> death from passing through birth canal
Is the autosomal dominant form or the autosomal recessive form of Osteopetrosis more severe?
Recessive
Osteopetrosis is the failure of ___ to function normally
Osteoclasts (bone is not resorbed)
Continued bone formation and ossification in Osteopetrosis leads to
Increased bone density
Osteopetrosis involves the loss of ____ ___ cells
Hematopoietic precursor cells (pancytopenia)
Because of the loss of hematopoietic precursor cells in Osteopetrosis, there is is an increased susceptibility to ___ and ____
Infections and osteomyelitis
If there is CN compression, then patients with Osteopetrosis may experience ___ and ____
Blindness and deafness
____ is a frequent complication of tooth extraction in patients with Osteopetrosis
Osteomyelitis
____ ____ is often delayed with Osteopetrosis
Tooth eruption
Radiographic features of Osteopetrosis
Diffuse density of skeleton
Marrow spaces filled by dense bone
Tooth roots difficult to visualize
Failure of tooth eruption
Prognosis for autosomal dominant vs. autosomal recessive Osteopetrosis
Poor for recessive
Uncommon bone disorder that affects the skull, jaws, and clavicles primarily
Cleidocranial dysplasia
Patients with cleidocranial dysplasia often have a prominent ____, and _____ midface
Forehead
Hypoplastic
Describe the neck and shoulders of a patient with Cleidocranial dysplasia
Long neck
Shoulders are narrow, drooping, and have hypermobility
Dental findings in a patient with Cleidocranial dysplasia
Primary dentition retained
Numerous impacted and supernumerary teeth
Dental treatment for patient with Cleidocranial dysplasia
Correct skeletal relations, remove supernumerary teeth, correct alignment of permanent teeth
Prognosis for patient with Cleidocranial dysplasia
Good
Bone disorder detected in childhood where patient has painless bilateral expansion of the jaws (esp. mandible)
Cherubism
Why might the “eyes be turned to heaven” in a patient with Cherubism
Involvement of the inferior and/or lateral orbital walls may tilt the eyeballs upward and retract lower eyelid
Radiographic features of Cherubism
Bilateral multilocular radiolucencies in the posterior
Occasionally unilocular
Often significant tooth displacement
You may see ___ ___ in the histology of Cherubism
Perivascular hyalinization
Surgical intervention in Cherubism sometimes:
Accelerates growth of lesion
Osteitis deformans is also called:
Paget disease
Abnormal resorption and deposition, resulting in distorted/weaker bone
Paget disease
The affected bones in Paget disease become ____
Thickened
When is Paget disease typically discovered
On routine blood test or dental radiographs
Paget disease has a ____ predilection
Male
Paget disease typically affects ____ patients
Older
Many Paget disease patients have a ____ stance if their ____ is involved
“Simian” (monkey-like)
Femurs (bowing of legs)
There is elevated serum ___ ___ in patients with Paget disease
Alkaline phosphatase
What might a patient with Paget disease complain about if there is skull involvement?
Their hat won’t fit - the skull has progressive involvement
What might a patient with Paget disease complain about if their jaw is involved?
Denture won’t fit - thickened, enlarged alveolar bone
Radiographic feature of Paget disease
“Cotton wool” appearance of bone
Extensive hypercementosis
What is the marrow replaced with in Paget disease
Vascular fibrous connective tissue
In Paget disease, you will see a ___ pattern of irregular ____ with resting and ___ lines
Mosaic
Trabeculae
Reversal
Treatment for Paget disease
Bisphosphonates (or no treatment if asymptomatic)
Patients with Paget disease should be monitored for development of a ___ ___ ___ or ___ ___, especially ___
Giant cell tumor
Malignant tumors
Osteosarcoma
You may have difficulty extracting teeth in patients with Paget disease due to:
Hypercementosis and/or ankylosis
2 additional dental complications with Paget disease
Hemorrhage during vascular/lytic phase
Poor wound healing, increased susceptibility to osteomyelitis during avascular/sclerotic phase
Prognosis for implants in Paget disease
Unfavorable (more so with use of bisphosphonates)
Etiologies of fibro-osseous lesions
Developmental (hamartomatous), Reactive, Dysplastic, Neoplastic
2 Fibro-osseous lesions
- Fibrous dysplasia
2. Cemento-Osseous Dysplasia
3 types of Cemento-Osseous Dysplasia
- Periapical
- Focal
- Florid
3 times of occurrence for fibrous dysplasia post-zygotic mutation
- Pluripotent stem cell
- Skeletal progenitor cell
- Post-natal life
Are patients with Fibrous dysplasia younger or older than patients with Paget disease
Younger
Do most cases of fibrous dysplasia involve many or one bone?
One bone
____ are among the most commonly affected bones for fibrous dysplasia
Jaws
Is the maxilla or the mandible more involved in fibrous dysplasia
Maxilla
Why might a patient with craniofacial fibrous dysplasia have facial deformity?
Maxillary lesions may involve adjacent facial bones
Early radiographic stages of fibrous dysplasia are ___ or ____. They ____ as they grow
Radiolucent or mottled
Opacify
Obliteration of the ____ ____ can occur in fibrous dysplasia
Maxillary sinus
Fibrous dysplasia has a classic ___ ____ pattern
Ground glass
2 types of polyostotic fibrous dysplasia
- Jaffe type
2. McCune-Albright type
Jaffe type
2 or more bones affected and cafe-au-lait spots with jagged borders (coast of Maine)
McCune-Albright type
2 or more bones affected, cafe-au-lait spots, and endocrine disturbances (precocious puberty)
Type of bone associated with fibrous dysplasia
Woven
Because there is no capsule, abnormal bone in this disorder will fuse to the adjacent normal bone
fibrous dysplasia
____ is contraindicated for treatment of fibrous dysplasia
Radiation
Prognosis for fibrous dysplasia
Sometimes disease stabilizes or regresses. Rare malignant transformation
Most common fibro-osseous lesion encountered in the clinical practice of dentistry
Cemento-Osseous dysplasia
Cemento-Osseous dysplasia is possibly a ____ process
Reactive
Cemento-Osseous dysplasia is most commonly seen in ___ ____
Black females
Focal type Cemento-Osseous dysplasia is more common in ___ ___
White females
Teeth usually test _____ (vital/non-vital) in Cemento-Osseous dysplasia
Vital
Florid Cemento-Osseous dysplasia shows multiple ___ ____ type radio____ (pacities/lucities) in at least ____ quadrants of the jaws
Cotton wool
Radiopacities
2 quadrants
Cemento-Osseous dysplasia may be associated with a ___ ___ cyst
Simple bone
Types of Cemento-Osseous dysplasia that are mild, moderate, and severe
Periapical (mild)
Focal (moderate)
Florid (Severe)
Periapical Cemento-Osseous dysplasia is usually in the ____ (anterior/posterior) ____ (maxilla/mandible)
Anterior mandible
What can periapical Cemento-Osseous dysplasia be confused with?
Hypercementosis, Idiopathic osteosclerodid, benign cementoblastoma
Focal Cemento-Osseous dysplasia is more common in ____ (older/younger) patients
Younger
Focal Cemento-Osseous dysplasia can be confused with _____ _____. Which is more common?
Ossifying fibroma
Focal Cemento-Osseous dysplasia is more common
Overlying mucosa can ulcerate and result in ___ ____ with florid Cemento-Osseous dysplasia
Bony sequestration
Lesions in florid Cemento-Osseous dysplasia tend to be hypovascular, resulting in:
Reduced healing ability
Prone to necrosis, infection, osteomyelitis
What does the mineralized product in Cemento-Osseous dysplasia resemble?
Ginger root
How do you diagnose Cemento-Osseous dysplasia
Radiographs and confirm with biopsy if indicated
Treatment for periapical Cemento-Osseous dysplasia
None
Why might a biopsy be indicated for focal Cemento-Osseous dysplasia
To rule out another disease
Biopsy is generally not necesssary for ____ Cemento-Osseous dysplasia
Florid
Appearance of focal Cemento-Osseous dysplasia may be the first sign of ___ ___ ___ ___
Florid Cemento-Osseous dysplasia
Area of hematopoietic bone marrow of sufficient size to cause a radiographic radiolucency
Osteoporotic bone marrow defect
Osteoporotic bone marrow defect is usually seen in the ____ (anterior/posterior) _____ (maxilla/mandible)
Posterior mandible
Usually seen at an old extraction site
Osteoporotic bone marrow defect
Osteoporotic bone marrow defect has a ____ predilection
Female
In a radiograph, Osteoporotic bone marrow defect is a ____ (radiopaque/radiolucent) lesion with ___ (well/ill) defined borders and a fine ___ pattern
Radiolucent
Ill
Trabecular
Osteoporotic bone marrow defect shows fatty and ____ marrow
Hematopoietic
True/False: The osteoblast and osteoclast activity is abnormal in Osteoporotic bone marrow defect
False - no abnormal activity
Treatment for Osteoporotic bone marrow defect
Biopsy often indicated to establish diagnosis
Prognosis for Osteoporotic bone marrow defect
Excellent
Other terms for Idiopathic Osteosclerosis
Dense bone island, Enostosis, Bone whorl, Focal periapical osteopetrosis, bone scar
Focally increased area of dense bone
Idiopathic Osteosclerosis
Is there expansion with Idiopathic Osteosclerosis
No
Peak prevalence of Idiopathic Osteosclerosis is in the ____ decade
3rd
Most common site for Idiopathic Osteosclerosis
Mandibular pre-molar/molar area
For Idiopathic Osteosclerosis, are the borders sharp or do they blend?
They blend, but occasionally may be sharp
Does Idiopathic Osteosclerosis cause tooth displacement?
Rarely
Idiopathic Osteosclerosis may be confused with
- Condensing osteitis
- Hypercementosis
- Cementoblastoma
In Idiopathic Osteosclerosis, you see dense ___ bone
Vital
Other terms for simple bone cyst
Traumatic bone cyst, Hemorrhagic bone cyst
Empty or fluid-filled bone cavity can be seen in a ___ ___ ___
Simple bone cyst
Why is a simple bone cyst not a true cyst?
It lacks an epithelial lining
Trauma-hemorrhage theory
Trauma causing hematoma but not fracture - without subsequent organization and repair of hematoma - instead liquifies (possible theory for simple bone cyst)
Does a simple bone cyst have a gender predilection?
None in the jaws, but male in other bones
Simple bone cyst is mostly seen in the ____ (anterior/posterior) _____ (mandible/maxilla) and _____
Posterior mandible and symphysis
Simple bone cyst often _____ between roots. Is this diagnostic?
Scallops - not diagnostic, but is suggestive
Bone fragments in a simple bone cyst are lined by ___ ___ tissue
Inflamed granulation
Why is the treatment for a simple bone cyst surgical exploration and curettage?
Induce bleeding –> causes hemorrhage –> organizes and heals lesion