Fibreuze dysplasia

• Article
• 2021-01-29

Fibrous dysplasia occurs when abnormally fibrous (scar-like) tissue replaces healthy bone. The fibrous tissue weakens the bone over time, which can lead to fractures (fractures) and deformed bones.

Some people with fibrous dysplasia have no or only a few symptoms, usually in one bone (monostotic). Other people may have multiple bones affected (polyostotic) and experience more symptoms. The disease can occur on its own or as part of a condition known as McCune-Albright syndrome, which affects bone as well as skin and endocrine (hormone-producing) tissues.

Unfortunately, there is no cure for fibrous dysplasia; however, treatments can help to relieve pain, and supportive measures such as physical therapy can help strengthen muscles and improve range of motion.

What happens in fibrous dysplasia?

Fibrous dysplasia occurs when a gene mutates (changes) while the baby is developing in the womb. The changes in the gene keep bone-forming cells from maturing. Instead, they produce abnormal connective tissue in certain bones. Because the gene change takes place while the baby is developing, only specific bones will get the disease. This means that fibrous dysplasia does not spread from one bone to another.

The disease can affect any bone in the body; however, in some people it occurs in the bones on one side of the body. The most common bones affected by fibrous dysplasia are:

• Skull and facial bones.
• Thigh (upper leg) and tibia and fibula (lower leg).
• Humerus (upper arm bone).
• Basin.
• Ribs.

Symptoms

People with a milder form of the disease may not have symptoms and only learn that they have fibrous dysplasia if they have an X-ray taken for some other reason. Other people may have a more severe form of the disease and develop symptoms in early childhood. The most common symptoms of fibrous dysplasia are:

• Bone pain, which can occur as a result of fractures or changes in connective tissue in the bones.
• Deformed bones or bending of bones. This is most common in the femur (thigh bone) and is called a coxa vara (shepherd's crook).
• Fractures due to weak bone structure.

Other symptoms can occur depending on which bones are affected. Changes in:

• Leg bones can cause the bones to shorten, bend, or change in length, leading to limping or changes in mobility.
• Facial bones and sinuses can cause long-lasting sinus congestion.
• Spine can lead to scoliosis .
• Skull and facial bones around the eyes and ears can rarely cause vision and hearing loss.

In very rare cases, some people can develop malignant bone cancer.

Therapy

There is no cure for fibrous dysplasia. The goals for treatment can include:

• Treating and preventing fractures.
• Correct deformed bones when the bend is severe.
• Dealing with pain.

If you or your child have no symptoms and are not at risk for a fracture, your doctor may recommend monitoring the condition. If there are symptoms, treatments can include:

• Physical therapy to strengthen muscles and improve range of motion.
• Cast, splint, or brace to immobilize fractures or improve mobility.
• Surgery to prevent and repair fractures, treat scoliosis, and repair malformed bone.

While no drugs have been approved by the U.S. Food and Drug Administration to treat fibrous dysplasia, your doctor may recommend therapy approved for a related condition. Your doctor may prescribe the following:

• Painkillers to treat pain caused by fractures and chronic bone pain.
• Medicines for the hormoneTreat problems that some patients with fibrous dysplasia may have.

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