Fibrous Dysplasia

Fibrous Dysplasia –  Know It All!

Editorial

Fibrous Dysplasia –  Know It All!

All you need to know about Fibrous Dysplasia.

Fibrous Dysplasia
Fibrous Dysplasia

Know your ailment well, so you can manage it better!!

Here we come with Fibrous Dysplasia today!

Overview:

  • Fibrous dysplasia occurs when an irregular (scarlike) fibrous tissue replaces healthy bone. Over time, the fibrous tissue weakens the bone, which may result in fractures (breaks) and bones that are misshapen.
  • Some people with fibrous dysplasia typically have no symptoms, or just a few symptoms, in one (monostotic) bone. Many people may have many (polyostotic) affected bones, and may have more symptoms. The disease may occur on its own or as part of a disorder known as McCune-Albright syndrome that affects both the bone and the tissues of the skin and endocrine (hormone-producing).
  • Unfortunately, there is no remedy for fibrous dysplasia; however, medications may help alleviate discomfort, and supporting interventions such as physical therapy may help reinforce muscle and enhance motion ability.

What happens in Fibrous Dysplasia:

Fibrous dysplasia occurs when a gene mutates (changes), as the baby grows in the womb. The gene changes cause cells that form bones to fail to mature. Instead, they produce certain bones with irregular fibrous tissue. Since the gene shift occurs when the baby is developing, the disorder can only occur to different bones. This means that fibrous dysplasia doesn’t spread from bone to bone.

The disease can affect any bone in the body, but it happens in the bones on one side of the body for certain people. Bones most commonly affected by fibrous dysplasia include:

  • Skull and facial bones.
  • The femur (upper leg) and tibia and fibula (lower leg).
  • Humerus (upper arm bone).
  • Pelvis.
  • Ribs.

Symptoms of Fibrous Dysplasia:

People with a milder form of the disease can have no symptoms and may not learn to have fibrous dysplasia until, for some cause, they have an x-ray. Many people may have a more serious form of the disorder, and may experience early childhood symptoms. Among the most common symptoms of fibrous dysplasia are:

  • Bone pain, which may happen because of fractures or fibrous tissue changes in the bones.
  • Misshapen bones or bowing 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 may develop depending on which bones are affected. Changes in the:

  • Leg bones may cause the bones to shorten, bow, or change in length, leading to a limp or changes in mobility.
  • Facial bones and sinuses can cause long-term sinus congestion.
  • The spine can lead to scoliosis.
  • Skull and facial bones around the eyes and ears may rarely lead to vision and hearing loss.
  • In very rare cases, some people may develop a malignant form of bone cancer.
Fibrous Dysplasia
Fibrous Dysplasia

Causes:

Fibrous dysplasia occurs after birth, early in pregnancy, when a gene mutates (changes). Mother can do little to keep this from happening. Researchers sadly do not know what causes the gene to mutate; however, they have identified the gene and are continuing to research why fibrous dysplasia occurs. Since the disease arises from a defective gene, their parents will not inherit the gene and they do not pass the disease on to their offspring.

Complications:

Severe fibrous dysplasia can cause:

  • Bone deformity or fracture. The damaged area of an injured bone will bend the bone. Even these debilitated bones are more likely to crack.
  • Vision and hearing loss. The affected bone that surround the nerves to your eyes and ears. Serious facial bone deformity can cause vision and hearing loss, but it is a rare complication.
  • Arthritis. If the leg and pelvic bones are deformed, the joints of those bones that develop arthritis.
  • Cancer. Rarely will a bone region affected get cancerous. Typically this unusual condition only affects individuals who have undergone previous radiation therapy.

Who Develops this disease?

  • Fibrous dysplasia is not widespread but the condition can be acquired by anyone. It is usually diagnosed in children and young adults, though it was possibly present at birth but not diagnosed. Race, age, exposures to the environment, and geographic location have little impact on how the disease evolves.

How to diagnose:

Depending on the location and severity of symptoms, your doctor may order one of the following tests:

  • X-rays. This is the most common test that doctors use to diagnose fibrous dysplasia. An x-ray can evaluate the bone structure for the disease and diagnose fractures and misshapen bones.
  • Magnetic resonance imaging (MRI) or computed tomography (CT). These tests provide detailed images that are analyzed by a computer and are helpful in evaluating the skull and facial bones for the disease.
  • Bone scan. This test evaluates the entire skeleton, helping doctors understand the amount of bone in the body affected by the disease.
  • Bone biopsy. During this test, a doctor takes a small amount of bone tissue from an area affected by the disease to examine under a microscope.
  • Some children may need additional testing to determine if fibrous dysplasia is part of another syndrome or disorder. Genetic testing is usually performed on a case-by-case basis.

Treatment Options:

If you have mild, incidentally discovered fibrous dysplasia, and you do not have any signs or symptoms, the risk of developing deformity or breaking the bone is small. With regular X-rays your doctor will possibly monitor your condition.

Medications:

Osteoporosis medications called bisphosphonates help prevent bone loss by decreasing the activity of cells that normally dissolve bone. Some studies suggest that bisphosphonates may strengthen bones affected by fibrous dysplasia and may relieve bone pain.

Surgery:

Your Physician may recommend surgery in order to:

  • Correct a deformity
  • Correct a difference in limb lengths
  • Repair a fracture that does not heal with casting
  • Prevent fractures
  • Relieve pressure on a nerve, particularly if the lesion is in your skull or face

Surgery may include extracting and replacing the bone lesion with bone graft: bone from another part of your body, donor bone tissue or synthetic material. In some cases, a lesion from fibrous dysplasia can recur. Your surgeon can also insert sheets, rods or screws of metal to prevent fractures or to strengthen a bone or bone graft.

Coping with Fibromyalgia:

Fibrous Dysplasia
Fibrous Dysplasia

For every person living with fibrous dysplasia is different. Some people have little to no symptoms, while others have several symptoms that impair their everyday activity capacity. The following tips may be of use:

  • See your health care providers on a regular basis to help keep bones as healthy as possible.
  • Talk to your doctor or physical therapist about which types of exercises are best.
  • Ask your doctor about taking calcium, vitamin D, and phosphorus supplements to support general bone health.
  • Ask family and friends for help when you need it.
  • Reach out to online and community support groups.

 

References:

https://www.mayoclinic.org/diseases-conditions/fibrous-dysplasia/diagnosis-treatment/drc-20353201

https://www.ncbi.nlm.nih.gov/books/NBK274564/

https://www.niams.nih.gov/health-topics/fibrous-dysplasia

 

By,

Gopala Krishna Varshith,

Content Developer & Editor,

Clipo.

 

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