What is fibrous dysplasia?
Fibrous dysplasia is a chronic disorder in which bone expands due to abnormal development of fibrous tissue, often resulting in one or more of the following symptoms:
* Uneven growth of bones.
* Pain.
* Brittle bones.
* Deformity of the bones.
It can affect any bone. It is possible that several bones are affected at once, and when that happens, it’s not unusual that everyone is on the same side of the body. However, fibrous dysplasia does not spread from one bone to another. The bones most commonly affected include:
* The femur (thigh bone).
* The tibia (shin bone).
* The ribs.
* The skull.
* The facial bones.
* The humerus (the bone of the upper arm).
* The pelvis.
* The vertebrae of the spine (less frequently).
Some people develop hormonal problems and a condition called McCune-Albright syndrome. This syndrome, another form of fibrous dysplasia, includes different symptoms, such as early onset of puberty and skin spots called cafe au lait spots.
Fibrous dysplasia usually occurs in children ages 3 to 15, but sometimes not diagnosed until adulthood. It is found equally among men and women.
What causes fibrous dysplasia?
It is unknown the exact cause of fibrous dysplasia, but is suspected to be due to a chemical irregularity in a specific bone protein. This bone protein abnormality may be caused by a genetic mutation at birth, but has not been shown to be an inherited disorder.
What are the symptoms of fibrous dysplasia?
Below are the most common symptoms of fibrous dysplasia. However, each individual may experience symptoms differently. Symptoms may include:
* Waddling walk.
* Bone pain because of the expanding fibrous tissue in the bone.
* Bone deformity.
* Bone fractures.
* Scoliosis – lateral curvature and rotation (to the side) of the back bones (vertebrae) that make a person look tilted to one side.
The symptoms of fibrous dysplasia may resemble other conditions or medical problems of the bones. Always consult your physician for diagnosis.
How is fibrous dysplasia diagnosed?
In addition to examination and complete medical history, diagnostic procedures for fibrous dysplasia may include the following:
* X-rays – a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs onto film.
* Biopsy – a procedure in which tissue samples are removed (with a needle or during surgery) for examination under a microscope to determine if cancer or other abnormal cells are present to remove tissue from the affected bone.
* CT scan (Also called CT or CAT scan.) – A diagnostic imaging procedure that uses a combination of X-rays and computer technology to produce cross-sectional images (often called slices) of the body, both horizontally and vertically. A CT scan shows detailed images of any part of the body, including bones, muscles, fat and organs. CT scans are more detailed than x-rays.
* Blood tests.
Treatment of fibrous dysplasia
Specific treatment for fibrous dysplasia will be determined by your physician based on:
* Your age, overall health and medical history.
* Extent of the disease.
* Your tolerance for specific medications or therapies.
* Expectations for the course of the disease.
* Your opinion or preference.
Treatment may include:
* Surgery, including:
or removal of affected bone followed by bone grafting (surgical procedure that is transplanted into the affected area healthy bone from another part of the body of the patient).
or removal of a wedge of bone.
or placement of a rod inside the shaft of the bone.
Medications.
* Pain Control.
* Physiotherapy.
credit to: Dra. Muriel Parisi
Tags: disorder, fibrous dysplasia, fibrous tissue, McCune-Albright syndrome
