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Anterior skeletal anatomy
Anterior skeletal anatomy


Fibrous dysplasia

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, incidence, and risk factors:

Fibrous dysplasia occurs in childhood, usually between ages 3 - 15. The condition does not run in families (not hereditary), and the cause is unknown.



Symptoms:
  • Bone pain
  • Bone sores (lesions)
  • Difficulty walking
  • Endocrine gland problems
  • Fractures or bone deformities (rare)
  • Unusual skin color (pigmentation)

The bone lesions may stop when the child reaches puberty.



Signs 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 appropriate. The patient is screened for endocrine disorders as needed.



Support Groups:



Expectations (prognosis):

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



Complications:
  • Cushing's disease
  • Gigantism or acromegaly
  • Heart rhythm disturbance
  • Hyperthyroidism
  • Rickets


Calling your health care provider:

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:

Heck RS Jr. Benign bone tumors and nonneoplastic conditions simulating bone tumors. In: Canale ST, Beaty JH, eds. Campbell's Operative Orthopaedics. 11th ed. Philadelphia, Pa: Mosby Elsevier; 2007:chap 20.

Arndt CAS. Benign tumors and tumor-like processes of bone. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 495.2.




Review Date: 11/13/2011
Reviewed By: Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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