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Questions and Answers
Fibrous dysplasia is a condition primarily seen in adults over the age of 30.
Fibrous dysplasia is a condition primarily seen in adults over the age of 30.
False
Monostotic cases of fibrous dysplasia are more common than polyostotic cases.
Monostotic cases of fibrous dysplasia are more common than polyostotic cases.
True
The classic appearance of fibrous dysplasia on an x-ray is described as 'glass-like' in matrix.
The classic appearance of fibrous dysplasia on an x-ray is described as 'glass-like' in matrix.
False
Patients with fibrous dysplasia have a normal risk of fractures due to the integrity of the bone.
Patients with fibrous dysplasia have a normal risk of fractures due to the integrity of the bone.
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Cherubism is inherited in an autosomal recessive fashion.
Cherubism is inherited in an autosomal recessive fashion.
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Cranial nerve palsies in fibrous dysplasia are caused by canal stenosis due to bony expansion.
Cranial nerve palsies in fibrous dysplasia are caused by canal stenosis due to bony expansion.
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Protrusio acetabuli caused by fibrous dysplasia results from the remodeling of the femoral head.
Protrusio acetabuli caused by fibrous dysplasia results from the remodeling of the femoral head.
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Fibrous dysplasia is unlikely to complicate with secondary degeneration into osteosarcoma.
Fibrous dysplasia is unlikely to complicate with secondary degeneration into osteosarcoma.
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Study Notes
Fibrous Dysplasia
- Benign condition affecting bones, often in childhood/adolescence, rarely after 30.
- Caused by abnormal osteoblasts, replacing healthy bone with fibrous tissue.
- Most cases are monostotic (affecting one bone), but about 20% are polyostotic (affecting multiple bones).
- Long bones are more commonly affected in polyostotic cases, but virtually any bone can be involved.
- Diagnosis is often by X-ray or CT, showing "ground glass" appearance in the medullary (center) of the bone, commonly causing bony expansion.
- Increased risk of fractures, but without acute injury, there's usually no periosteal reaction.
- Bone scan shows increased uptake, not helpful in diagnosis.
- Secondary degeneration can lead to osteosarcoma, particularly in those exposed to radiation, signaled by pain and soft tissue mass growth.
Eponymous/Descriptive Terms Related to Fibrous Dysplasia
- Shepherd's Crook Deformity: Coxa varus deformity of the proximal femur, caused by chronic bowing and remodeling secondary to microfractures in the bone. Similar appearances are possible in Paget disease and osteogenesis imperfecta.
- Protrusio Acetabuli: Fibrous dysplasia can cause femoral head protrusion into the pelvis due to acetabulum remodeling.
- Leontiasis Ossea: Facial involvement, resembling a lion, due to canal stenosis (narrowing) by bony expansion. Can cause cranial nerve palsies.
- Cherubism: Maxilla and mandible facial involvement resulting in a cherubic appearance. Also causes canal stenosis and potential cranial nerve palsies. Inherited in an autosomal dominant pattern.
McCune-Albright Syndrome
- Primarily affects females.
- Triad of symptoms:
- Cafe-au-lait spots (often on the back and respecting the midline).
- Precocious puberty (or other endocrine issues).
- Polyostotic fibrous dysplasia.
Mazabraud Syndrome
- Very rare.
- Polyostotic fibrous dysplasia, along with soft tissue tumors.
- Increased risk of malignant degeneration to osteosarcoma.
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Description
This quiz covers the essential aspects of fibrous dysplasia, including its etiology, common manifestations, diagnosis, and associated risks. Participants will learn about the differences between monostotic and polyostotic forms, as well as related complications. Perfect for students and medical professionals seeking to improve their understanding of this condition.
