Fibrous Dysplasia: Symptoms, Diagnosis and Treatment
Fibrous dysplasia occurs when bone tissue is replaced by fibrous tissue in side bones. It can involve one or many bones of the body. In more than two thirds of patients one bone is involved. Both sexes are equally affected.
Rarely it is seen as a condition called McCune Albright syndrome. In this condition along with fibrous dysplasia there is increased pigmentation of the skin at places along with other endocrine abnormalities such as early sexual development in children.
Cause of fibrous dysplasia is unknown. It is believed to occur during development of the baby within the womb. During the formation of the fetus a defect occurs in the DNA of the bone forming cells and this causes the formation of fibrous tissue in place of bone tissue.
As fibrous tissue is weaker than bone tissue, hence its presence in bones causes them to become weak. These weak bones are unable to withstand the forces acting across them. Therefore these bones become deformed and can even fracture during normal day to day activities.
So the symptoms of can be
- asymptomatic or no symptoms (discovered incidentally during x ray examination for some other condition)
- or there may be a deformity of the bone causing the limb to appear abnormal in shape or cause a defect in gait
- or may present as a fracture of the bone
- or there may be just bone pain
If associated with McCune Albright syndrome the initial presentation may be early development of secondary sexual features or abnormal vaginal bleeding in girls.These symptoms usually develop by the age of 8 to 10 years.
Most commonly involved bones are the
- femur or thigh bone
- tibia or shin bone
- bones of the pelvis
- humerus or arm bone
Diagnosis is by x rays. Sometimes the diagnosis may not be clear on x rays. In such conditions a biopsy may be required.
Treatment is by surgically removing the fibrous tissue and filling the space by bone chips taken from the pelvic bone of the patient. At times if the defect is large then a commercially available bone substitute is mixed along with the bone chips. With time the bone chips and bone substitute get incorporated into the defective bone and strengthen it.
This is a x ray of a patient showing a fracture through the dysplasia. The fibrous tissue was removed, bone grafting was done and the fracture was stabilized with a plate and screws. The next x ray was taken after surgery.
Complications of fibrous dysplasia are
- bone deformity causing a defective gait, reduced height and arthritis due to wrong alignment of the joints
- abnormality in vision and hearing
- rarely cancer formation can occur
Fractures in this condition heal easily. The fractures can be fixed surgically with a plate and screws or other such devices and simultaneously the fibrous tissue can be removed and the defect filled with bone chips.
Correcting the deformity surgically can correct the gait, improve height and prevent arthritis by properly aligning the joints.
Frequently Asked Questions
Can I pass on this condition to my children?
You can not pass this condition to your children as it is not inherited but has occurred due to the defect in DNA during the development of one bone. This defect defect in not present in the remaining cells of your body.
I have fibrous dysplasia but no symptoms. Do I require any treatment?
You require no treatment.
I hope the information provided was helpful. If you have any query you can ask me at the contact me page.
This page was created on 22nd October 2010
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