Fibrous Dysplasia and Paget's disease Flashcards
What are known as the “Great Imitators of Bone Disease?”
Fibrous Dysplasia and Paget’s disease
What is the most common Fibrous Dysplasia?
Monostotic fibrous dysplasia
At what age does Monostotic fibrous dysplasia affect?
14 year olds
What is the 2nd most common FD that affects 11 year olds?
Polystotic FD
Is FD symptomatic or asymptomatic?
asymptomatic
What part of the body does FD most commonly affect?
Proximal Femur
What are the 2 characteristic features of FD?
Shepherd’s Crook deformity
Leg length discrepancy
What is known as an enlargement and deformity of cranial and facial bones that looks like a lion?
Leontiasis Ossea
What epidermal sign of FD has dark brown pigmented skin macules that resemble the coast of Maine appearance (irregular margins)?
Café au Lait Spots
What is a radiographic sign of FD?
ground glass appearance with poorly formed bony trabeculae of woven bone (endosteal scalloping)
What type of FD is most commonly associated with benign rib lesions?
Monostotic FD
What type of FD has a higher incidence of pathologic Fx and is seen with a Shepherd’s Crook deformity?
Polystotic FD
What syndrome is associated with polyostotic FD with skin pigmentation and precocious sexual development such as premature irregular menstrual bleeding at age 5-6 and early development of secondary sex characteristics in females?
McCune-Albright Syndrome
What is seen with swelling of lower face affects the jaw, and has an “Eyes raised to heaven attitude” (cheek swelling stretches skin and retracts lower eyelids causing eye to turn upward)?
Cherubism
What age does Cherubism typically affect?
18 months - 2 years
What parts of the body is monostotic FD most commonly seen in?
ribs and proximal femur
What are 3 key radiographic findings for monostotic FD?
Radiolucent
ground glass appearance w/ endosteal scalloping
thick ring of sclerosis
What part of the bone does monostotic FD usually affect?
diametaphyseal
What is the most obvious and common symptom of Polystotic FD?
bowing deformities (Shephard Crook deformity) and pathologic fx
T/F: Paget’s Disease THICKENS outer table of skull, whereas FD THINS outer table
True
T/F: The spine is NOT involved with fibrous dysplasia?
True
What is another name for osteitis deformans and is involved with Osteolysis followed by extensive attempts at repair?
Paget’s disease
What age group is most affected by Paget’s disease?
> 50 yo
Is Paget’s disease symptomatic or asymptomatic?
asymptomatic
What is the hallmark sign of Paget’s disease?
Bony enlargement (increase in hat and foot size)
What deformity is involved with bowing of the anterior shin and is associated with Paget’s disease?
Sabre shin deformity
What deformity is a condition in which a vertebra at the top of the spine moves up and back, toward the base of the skull which may lead to brainstem compression and deafness and is also associated with Paget’s disease?
Basilar invagination
What 3 deformities may be associated with Paget’s disease?
Shephard crook’s deformity
Sabre Shin deformity
Basilar invagination
What is a radiographic feature of the skull with Paget’s disease?
“cotton ball” appearance
What is the most common complication of Paget’s disease?
Bilateral and symmetrical Pathological Fx
What parts of the body are most commonly affected with Paget’s disease?
Hip and knee
What is a common radiographic feature of the hip ith Paget’s disease?
Protrusio acetabuli
What is a complication of Paget’s disease that can cause compressive neuropathy, cauda equina syndrome, and weakness in legs, urinary incontinence, sensory disturbances?
Spinal Stenosis
What is a complication of Paget’s disease that is due to hypercalcemia and hypercalciuria?
Urinary Calculus formation
What is a complication of Paget’s disease due to Pagetic bone has 20X normal vascularity needing more blood supply to that area?
High-Output Cardiac Failure
What is the most common malignant formation of Paget’s disease that is seen in the femur and commonly associated with cannon-ball mets of the lung?
osteosarcoma
T/F: Metastatic disease typically does NOT affect Pagetic bone
True
What is the key difference between Paget’s disease and Fibrous Dysplasia?
Paget’s begins in subarticular region of long bone
FD is NOT subarticularly located (metaphysis)
What bone is least likely affected by Paget’s disease?
Fibula
What lab result is elevated 20x in Paget’s disease?
Alkaline phosphatase
What stage of Paget’s disease is the osteolytic stage?
ex: osteoporosis circumscripta of skull
Stage 1
What stage of Paget’s disease is the most common stage seen involving destruction and production of bone, cortical thinning with bony enlargement? commonly seen in pelvis
(ex: Cotton Wool Appearance of skull)
Stage 2
What radiographic sign of stage 2 resembles a flame shaped abnormal lucency?
Blade of grass shape
What stage of Paget’s disease has an Ivory appearance of bone usually located in pelvis and spine?
Stage 3
What stage of Paget’s disease has Malignant Transformation (Most common is osteosarcoma)?
Stage 4
What is a complete Transverse insufficiency fracture also known as?
Banana Fx
T/F: If hot bone scan, then do plain film to confirm Paget’s disease
True
T/F: Paget’s always begins at one end of a bone in subarticular region, and progresses towards other end
True
If Paget’s disease affects the spine, what radiographic finding will likely be present?
picture frame appearance (Enlarged vertebra with rim of thickened cortex)
When Paget’s disease affects the pelvis, what is a radiographic sign that is characteristic of Cortical thickening of pelvic brim, and obliteration of Kohler’s teardrop?
Rim/Brim sign
What are the most common radiographic signs of the Tibia with Paget’s disease?
Sabre shin deformity and blade of grass appearance
How do you treat Paget’s disease?
bracing and Bisphosphonates
