Fibrous Dysplasia?

Fibrous dysplasia is a rare bone disorder that involves the growth of scar-like (fibrous) tissue rather than normal bone. This irregular tissue can weaken the affected bone and cause it to become deformed or fractured.

In most cases, fibrous dysplasia appears in only one place on a bone, but may appear in several places on different bones. Single-bone disease usually occurs in adolescents and young adults. Normally, people who have more than one affected bone have symptoms by age 10.

Although fibrous dysplasia is a genetic disorder, it is caused by a genetic mutation that is not passed down through families. There is no cure for this disorder. Treatment, which may consist of surgery, focuses on relieving pain and repairing or stabilizing bones.

Fibrous dysplasia may cause few or no signs and symptoms, especially if the disease is mild. Severe fibrous dysplasia can result:

• Bone pain, usually a mild to moderate dull ache
• Swelling
• Bone deformity
• Bone fractures, especially in the arms or legs
• Curvature in the bones of the legs

Fibrous dysplasia can affect any bone in the body, but the bones that are most affected are: Thigh (femur), Tibia, Upper arm bone (humerus), Skull, Ribs and Pelvis.

Rarely, fibrous dysplasia may be associated with the syndrome that affects the hormone-producing glands of the endocrine system. Some of these abnormalities may include

• Very precocious puberty
• Overactive hormone production
• Light brown skin patches

Increased bone pain may also be associated with normal hormonal changes in the menstrual cycle or pregnancy.

Fibrous dysplasia is linked to a genetic mutation present in certain cells that make bones. As a result of this mutation, immature or irregular bone tissue forms. Most of the time, irregular bone tissue (lesion) occurs in a single site or bone. Less often, several bones are affected and there may be more than one injury to them.

Lesions usually stop growing at some point during puberty. However, lesions may grow back during pregnancy.

The genetic mutation associated with fibrous dysplasia occurs after conception, in the early stages of fetal development. Therefore, the mutation is not inherited from the parents and cannot be passed on to your children.

Severe fibrous dysplasia can cause bone deformities or fractures. The weakened part of an affected bone can cause the bone to bend. These weakened bones are more likely to fracture.