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Fibrous Dysplasia

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Fibrous dysplasia is a skeletal disorder of where normal bones are replaced by fibrous tissue. This causes a number of problems like fractures, bone deformity, pain, etc. Let’s learn more on fibrous dysplasia in the following paragraphs.

What is Fibrous Dysplasia?

Fibrous dysplasia is a rare bone disorder where fibrous tissue develops in place of bone tissue. This causes weakening of the bone. The condition can develop over the years without causing any symptoms or, in some cases a few symptoms of the condition. For some, it could lead to physical as well as facial deformity.

In some people, just one bone is affected and in a few the condition is widespread. Thus, leading to three types of the condition:

Monomelic

The simplest form of the condition where only one bone is affected. The affected bone is usually the thigh, shin, rib cage or any one of the facial bones (upper or lower jaw).

Polyostotic

In this condition, more than one bone is affected. It can occur in young adults and more than half the skeletal system is affected. Polyostotic fibrous dysplasia leads to fractures, skin lesions, legs of different length as well as bone deformity.

McCune-Albright Syndrome

This is the most severe form of polyostotic fibrous dysplasia. It is associated with different abnormalities of the endocrine system. These abnormalities can include some or all of the following:

  • Skin pigmentation
  • Acromegaly
  • Rickets
  • Premature puberty
  • Hyperthyroidism
  • Cushing syndrome
  • Dwarfism due to premature closing of growth plate

Symptoms of Fibrous Dysplasia

The symptoms of fibrous dysplasia may differ in individuals affected. Some of the common symptoms include:

  • Bone pain due to the continuous expansion of the fibrous tissue in the bone
  • Problems walking
  • Bone deformity
  • Fractures

People with monostotic fibrous dysplasia may never develop any symptoms. In some rare instances, the condition may lead to abnormalities like:

  • Early puberty, especially in girls wit McCune-Albright syndrome
  • Skin discoloration
  • Enlarged thyroid gland

Causes of Fibrous Dysplasia

There is no the exact cause known for fibrous dysplasia. A gene mutation has been identified that leads to premature production of bone cells. However, it is not very clear if the gene can be inherited or it mutates during fetal development in the womb. Even the causes of mutation are unknown.

Diagnosis of Fibrous Dysplasia

The doctor will take a complete medical history of the patient and conduct a complete physical examination. Some of the diagnostic tests for fibrous dysplasia include:

  • X-ray to check the bones, organs and internal tissues for abnormality
  • Bone or tissue biopsy to check for abnormalities
  • CT scan to get more details regarding the bone, muscles, fats, etc.

Treatment for Fibrous Dysplasia

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People with mild dysplasia, with no symptoms rarely require treatment. The doctor will check the risk of developing bone fractures and if it is low, no treatment will be suggested. However, if the patient does show signs and symptoms of fibrous dysplasia, then the treatment includes:

  • Medications such as bisphophonates that help in the prevention of bone breakdown. These medications also help in maintaining the bone mass and density of the spine and hip bone. Thus, decreasing the chances of fractures.
  • Surgery will be required if the patient develops a bone deformity or to correct the length of the legs. In some, it may be required to release any pressure on a nerve in the face or skull. In some, it is carried out to treat a fracture.

The prognosis for fibrous dysplasia varies greatly in individual cases. When it develops in young patients, it leads to many complications such as bone deformity, etc. In some people, the disease is very mild and the symptoms are as good as none. People with fibrous dysplasia should avoid weight gain and make sure they live an active life. This will help in maintaining joint mobility. Exercise will help in maintaining bone health and avoid fractures.

Written by: Saptakee sengupta
Date last updated: April 01, 2015