Fairbank disease/ Trevor disease/ Dysplasia Epiphysealis/ Hemimelica

Fairbank disease is a rare inherited disorder that affects the secondary growth centers of bone mainly in ankles, knees and hips. Fairbank disease is always confined to single limb and results in mid dwarfism.

 

What is Fairbank disease?

Fairbank disease is a congenital bone development disorder. Fairbank disease is a rare inherited disorder that affects the secondary growth centers of bone mainly in ankles, knees and hips. Fairbank disease is always confined to a single limb and results in mid dwarfism. It is believed that Fairbank disease affects the limb bud in early fetal life.

Trevor’s disease was first described by Albert Mouchet and J. Belot. The name dysplasia epiphyseal hemimelia was given by researcher Fairbank. Fairbank disease is a benign disorder; cases of malignant transformation have not been reported.

Who is more affected by this disease?

Fairbank disease is three times more common in males and in females. The disease manifests in childhood and early adulthood.

Individuals of any race can be affected.

Causes of this disease-

The cause of Fairbank disease is unknown. There is no evidence weather hereditary factors have a role in the development of the disorder.

• Localized: one epiphysis is involved.
• Classic: more than one area in a single limb is affected.
• Generalized: whole limb from pelvis to foot is affected.

What are the symptoms of this disease?

• Painless mass in middle of the affected joint. Overgrowth of cartilage of long bone is the reason of swelling.
• Occasionally pain and discomfort in affected area.
• Range of motion of affected limb is decreased.
• Joint deformity.
• Muscle wasting in affected area.
• Rarely, joint lock.
• Limping gait due to damage to affected joint of lower limb.

How can be fairbank disease diagnosed?

The disease can be diagnosed by X-ray.

• Short term splinting of joint.
• Surgical therapy: excision of mass and correction of angular deformity.

What are the complications of fair bank disease?

Considerable disability due to direct involvement of articular surface of joint.

• Recurrence of angular deformity after surgery.
• Degenerative osteoarthritis.
• Fixed deformity.
• Leg discrepancy.

• Post traumatic osseous fragments.
• Osteochondroma.
• Synovial chondromatosis.
• Anterior spur of ankle.

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