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