Fibrous dysplasia is a rare, benign bone disease. It occurs when abnormal fibrous, scar-like tissue replaces healthy bone.
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Fibrous dysplasia is a rare bone disorder. Abnormal, weak bone forms in place of normal bone tissue, thereby weakening the bone.
The disease starts early in life. Though it doesn't spread beyond the original scope of affected bones, it's irreversible.
The most common bones affected by this disease are:
Types of fibrosis dysplasia include:
In most people, fibrous dysplasia affects only one bone (monostotic).
Polyostotic makes up about 20 to 30 percent of cases.
The polyostotic form often becomes apparent before age 10, whereas detecting the monostotic form depends on the affected bone.
Symptoms of fibrous dysplasia may include:
Other symptoms may include light brown birth marks café-au-lait spots) and endocrine dysfunctions such as:
These are signals of a condition called McCune-Albright syndrome, of which polyostotic fibrous dysplasia is one manifestation.
To help diagnose fibrous dysplasia your doctor will:
Images provided by x-rays, MRI, and CT scans can reveal the presence and extent of abnormal tissue in the affected bones.
Your doctor may also order a bone scan.
In cases of polyostotic fibrous dysplasia, you may also need blood tests to assess endocrine function.
If your doctor suspects cancer — which is rare — they may order a biopsy. In severe cases of fibrous dysplasia, the bone lesions can become cancerous.
Surgery may be necessary for more problematic cases of fibrous dysplasia involving chronic pain, fractures, or deformity.
Surgical treatment for fibrous dysplasia involves partial removal of the affected bone. Surgeons may also insert stabilizing agents such as metal plates, rods, and screws.
At UPMC, the preferred surgical treatment for fibrous dysplasia of the skull base is the Endoscopic Endonasal Approach (EEA).
Although fibrous dysplasia is irreversible, this state-of-the-art, minimally invasive treatment allows surgeons to:
Benefits of EEA include:
To preserve sight, surgeons will perform optic nerve decompression in cases where fibrous dysplasia causes compression of the optic canal.
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