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Fibrous dysplasia
Inflammatory fibrous hyperplasia; Idiopathic fibrous hyperplasia
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 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)
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Unusual skin color (pigmentation), which occurs with
McCune-Albright syndrome
McCune-Albright syndrome
McCune-Albright syndrome is a genetic disease that affects the bones and color (pigmentation) of the skin.
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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
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If a leg bone is affected, there can be difficulty walking and joint problems such as
arthritis
Arthritis
Arthritis is inflammation of one or more joints. A joint is the area where 2 bones meet. There are more than 100 different types of arthritis....
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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.
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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
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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: 11/19/2015
Reviewed By: 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.
