What is polyostotic fibrous dysplasia?
Polyostotic fibrous dysplasia—more than one bone is involved. It can affect more than one bone within the same limb or multiple bones throughout the body. This form of the condition is usually more severe. For this reason, it is typically discovered earlier in life.
What causes polyostotic fibrous dysplasia?
Fibrous dysplasia is linked to a gene mutation present in certain cells that produce bone. The mutation results in the production of immature and irregular bone tissue. Most often the irregular bone tissue (lesion) is present at a single site on one bone.
What causes McCune-Albright syndrome?
McCune-Albright syndrome is caused by a mutation in a gene called GNAS1. This gene mutation occurs after fertilization of the embryo (somatic mutation) and is therefore not inherited, nor will affected individuals pass the mutation on to their children.
What are the types of fibrous dysplasia?
The main types of fibrous dysplasia include:
- monostotic fibrous dysplasia – only one bone is affected.
- polyostotic fibrous dysplasia – two or more bones are affected.
- McCune-Albright syndrome – fibrous dysplasia can be associated with hormone disturbances and skin pigment changes.
What does polyostotic mean?
Polyostotic means the abnormal areas (lesions) may occur in many bones; often they are confined to one side of the body. Replacement of bone with fibrous tissue may lead to fractures, uneven growth, and deformity.
How is fibrodysplasia treated?
Your doctor may recommend surgery in order to:
- Correct a deformity.
- Correct a difference in limb lengths.
- Repair a fracture that does not heal with casting.
- Prevent fractures.
- Relieve pressure on a nerve, particularly if the lesion is in your skull or face.
Is there a cure for McCune-Albright syndrome?
The bony disease associated with MAS (PFD) is very difficult to treat. Currently, no clinically proven medical therapies are available.
How many cases of fibrous dysplasia are there?
Fibrous dysplasia (FD) is one of those conditions. With an estimated prevalence of 1 in 15000-30000 individuals , it is unsurprising that I had never come across it.
Is fibrous dysplasia a tumor?
Background: Fibrous dysplasia is a benign fibroosseous bone tumor that accounts for 5% to 10% of benign bone tumors. It can present as monostotic fibrous dysplasia (70% to 80%), polyostotic fibrous dysplasia (20% to 30%), McCune-Albright syndrome (2% to 3%), or Mazabraud’s syndrome in rare cases.
What is a Polyostotic process?
Osteology. Polyostotic fibrous dysplasia is a form of fibrous dysplasia affecting more than one bone. Fibrous dysplasia is a disorder where bone is replaced by fibrous tissue, leading to weak bones, uneven growth, and deformity. McCune-Albright syndrome includes polyostotic fibrous dysplasia as part of its presentation …
What does polyostotic fibrous dysplasia mean in medical terms?
Polyostotic fibrous dysplasia. Jump to navigation Jump to search. Polyostotic fibrous dysplasia is a form of fibrous dysplasia affecting more than one bone. Fibrous dysplasia is a disorder where bone is replaced by fibrous tissue, leading to weak bones, uneven growth, and deformity.
When does fibrous dysplasia of the bone lead to death?
Untreated, progressive scoliosis is one of the few features of fibrous dysplasia that can lead to early fatality. Bone pain is a common complication of fibrous dysplasia. It may present at any age, but most commonly develops during adolescence and progresses into adulthood.
What kind of surgery is used for fibrous dysplasia?
Surgical techniques that are effective in other disorders, such as bone grafting, curettage, and plates and screws, are frequently ineffective in fibrous dysplasia and should be avoided. Intramedullary rods are generally preferred for management of fractures and deformity in the lower extremities.
Is there a genetic inheritance of fibrous dysplasia?
Fibrous dysplasia is not hereditary, and there has never been a case of genetic inheritance from parent to child.