Week 3. Fibrous Dysplasia. Flashcards
(5 cards)
1
Q
Give a brief definition of fibrous dysplasia
A
- A focal, slow expanding bone lesion in which bone is replaced by a mass of: fibroblasts, randomly distibuted collagen fibres and islands of woven bone trabeculae
2
Q
Distinguish between monostotic and polyostotic fibrous dysplasia
A
MONOSTOTIC FD Incidence & Epidemiology - ~70% of cases - Affects males and females equally - Usually occurs in childhood, but may affect age Clinical Manifestations - Only affects one bone - Usually asymptomatic - May observe: Bone enlargement, pain due to pathological fractures or pressure on nerves
POLYSTOTIC FD Incidence & Epidemiology - 25% of cases - Affects males and females equally - Usually appears at earlier age than monostotic disease Clinical Manifestations - Affects multiple bones - May be uni or bilateral - Shoulder and pelvic girdles often severedal affected - Multiple skeletal deformities observed - Pain due to pathological fractures
3
Q
Describe the typical bony lesion in fibrous dysplasia
A
- Tends to be unilateral
- Shoulder and pelvic girdles usually affected
- Histologically: Increased osteoclast and fibroblast numbers, scattered trabeculae of irregular woven bone
4
Q
Discuss the clinical manifestations of Albright’s disease
A
- Bone lesions – Usually unilateral, multiple
- Endocrinopathy: Precocious sexual development (Most common - 50% of affected females, males less common), hyperthyroidism, Cushing’s disease, acromegaly
- Skin lesions – Large, pigmented areas of café-au-lait patches with irregular serpiginous borders (“Coast of Maine”). Usually found on the neck, shoulder, chest, back and pelvic region
5
Q
Discuss the radiological diagnosis of fibrous dysplasia
A
- Well circumscribed, minimally expansive (“geographic”) lytic lesions – severity varies depending on ratio of fibrous/osseous tissue)
- Ground-glass/frosted appearance (uniformly opaque) – due to absence of trabeculae
- Diaphyseal or diametaphyseal location
