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Fibrous dysplasia

Inflammatory fibrous hyperplasia; Idiopathic fibrous hyperplasia; McCune-Albright syndrome

Fibrous dysplasia is a bone disease that destroys and replaces normal bone with fibrous bone tissue. One or more bones can be affected.

Causes

Fibrous dysplasia usually occurs in childhood. Most people have symptoms by the time they are 30 years old. The disease occurs more often in females.

Fibrous dysplasia is linked to a problem with genes (gene mutation) that control bone-producing cells. The mutation occurs when a baby is developing in the womb. The condition is not passed from parent to child.

Symptoms

Symptoms may include any of the following:

  • Bone pain
  • Bone sores (lesions)
  • Endocrine (hormone) gland problems
  • Fractures or bone deformities
  • Unusual skin color (pigmentation), which occurs with McCune-Albright syndrome

The bone lesions may stop when the child reaches puberty.

Exams and Tests

The health care provider will perform a physical examination. X-rays of bones are taken. An MRI may be recommended.

Treatment

There is no cure for fibrous dysplasia. Bone fractures or deformities are treated as needed. Hormone problems will need to be treated.

Outlook (Prognosis)

The outlook depends on the severity of the condition and the symptoms that occur.

Possible Complications

Depending on the bones that are affected, health problems that may result include:

  • If skull bone is affected, there can be vision or hearing loss
  • If a leg bone is affected, there can be difficulty walking and joint problems such as arthritis

When to Contact a Medical Professional

Call your provider if your child has symptoms of this condition, such as repeated bone fractures and unexplained bone deformity.

Specialists in orthopedics, endocrinology, and genetics may be involved in your child's diagnosis and care.

Prevention

There is no known way to prevent fibrous dysplasia. Treatment aims to prevent complications, such as recurrent bone fractures, to help make the condition less severe.

References

Czerniak B. Fibrous dysplasia and related lesions. In: Czerniak B, ed. Dorfman and Czerniak's Bone Tumors. 2nd ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 8.

Heck RK, Toy PC. Benign bone tumors and nonneoplastic conditions simulating bone tumors. In: Azar FM, Beaty JH, Canale ST, eds. Campbell's Operative Orthopaedics. 13th ed. Philadelphia, PA: Elsevier; 2017:chap 25.

Merchant SN, Nadol JB. Otologic manifestations of systemic disease. In: Flint PW, Haughey BH, Lund V, et al, eds. Cummings Otolaryngology: Head and Neck Surgery. 6th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 149.

Shiflett JM, Perez AJ, Parent AD. Skull lesions in children: dermoids, langerhans cell histiocytosis, fibrous dysplasia, and lipomas. In: Winn HR, ed. Youmans and Winn Neurological Surgery. 7th ed. Philadelphia, PA: Elsevier; 2017:chap 219.

Text only

  • Anterior skeletal anatomy

    Anterior skeletal anatomy - illustration

    The skeleton is made up of 206 bones in the adult and contributes to the form and shape of the body. The skeleton has several important functions for the body. The bones of the skeleton provide support for the soft tissues. For example, the rib cage supports the thoracic wall. Most muscles of the body are attached to bones which act as levers to allow movement of body parts. The bones of the skeleton also serve as a reservoir for minerals, such as calcium and phosphate. Finally, most of the blood cell formation takes places within the marrow of certain bones.

    Anterior skeletal anatomy

    illustration

    • Anterior skeletal anatomy

      Anterior skeletal anatomy - illustration

      The skeleton is made up of 206 bones in the adult and contributes to the form and shape of the body. The skeleton has several important functions for the body. The bones of the skeleton provide support for the soft tissues. For example, the rib cage supports the thoracic wall. Most muscles of the body are attached to bones which act as levers to allow movement of body parts. The bones of the skeleton also serve as a reservoir for minerals, such as calcium and phosphate. Finally, most of the blood cell formation takes places within the marrow of certain bones.

      Anterior skeletal anatomy

      illustration

     

    Review Date: 10/18/2017

    Reviewed By: Neil K. Kaneshiro, MD, MHA, Clinical Professor of Pediatrics, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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