Fibrous dysplasia

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Definition

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

Alternative Names

Inflammatory fibrous hyperplasia; Idiopathic fibrous hyperplasia

Causes

Fibrous dysplasia occurs in childhood, usually between ages 3 and 15. The condition 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)
  • Difficulty walking
  • Endocrine (hormone) gland problems
  • Fractures or bone deformities (rare)
  • Unusual skin color (pigmentation), which occurs with McCune-Albright syndrome

The bone lesions may stop when the child reaches puberty.

Exams and Tests

The doctor will perform a physical examination. X-rays of bones are taken.

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 health care 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

Blanco P, Wientroub S. Fibrous dysplasia. In: Glorieus FH, Perrifor JM, Juppner H, eds. Pediatric Bone. 2nd ed. Philadelphia, PA: Elsevier; 2012:chap 22.

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

Version Info

  • Last reviewed on 11/19/2015
  • Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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