fibrous dysplasia

Question 1

what is it?

Answer 1

benign, developmental disorder of bone, causes normal skeletal tissue to be replaced by fibrous tissue

Question 2

where does it commonly affect?

Answer 2

bones of head and neck

Question 3

what causes it and in who?

Answer 3

genetic mutation
paediatric patients

Question 4

what is the pathophysiology?

Answer 4

genetic mutation results in lesions of fibrous tissue and immature bone
can affect one bone or more
defective mineralisation may result in angular deformities and affected bone is wider with thinned cortices

Question 5

how does it clinically present?

Answer 5

bone pain and deformities
pathological fractures

Question 6

what investigations are used?

Answer 6

bone scan, x-ray

Question 7

what is seen on bone scan?

Answer 7

intense increase in uptake during development but the lesion usually becomes inactive

Question 8

what is the buzzword on x-ray?

Answer 8

sheperds crook deformity

Question 9

what causes sheperds crook deformity?

Answer 9

involvement of proximal femur