What is Behçet’s disease?
Behçet’s disease is an autoimmune disease that results from damage to blood vessels throughout the body, particularly veins. In an autoimmune disease, the immune system attacks and harms the body’s own tissues. This disease is also known as adamantiades.

What causes Behçet’s disease?
The exact cause is unknown. It is believed that an autoimmune reaction may cause blood vessels to become inflamed, but it is not clear what triggers this reaction.

What are the symptoms of Behçet’s disease?
Behçet’s disease affects each person differently. The four most common symptoms are mouth sores, genital sores, inflammation inside of the eye, and skin problems. Inflammation inside of the eye (uveitis, retinitis, and iritis) occurs in more that half of those with Behçet’s disease and can cause blurred vision, pain, and redness.

Other symptoms may include arthritis, blood clots, and inflammation in the central nervous system and digestive organs.

How is Behçet’s disease treated?
There is no cure for Behçet’s disease. Treatment typically focuses on reducing discomfort and preventing serious complications. Corticosteroids and other medications that suppress the immune system may be prescribed to treat inflammation.
What is the prognosis for someone with Behçet’s disease?
Behçet’s is a chronic disease that recurs. However, patients may have periods of time when symptoms go away temporarily (remission). How severe the disease is varies from patient to patient. Some patients may live normal lives, but others may become blind or severely disabled.

Source: The National Eye Institute (NEI) is part of the National Institutes of Health (NIH) and is the Federal government’s lead agency for vision research that leads to sight-saving treatments and plays a key role in reducing visual impairment and blindness.

Last Reviewed September 2009

Anophthalmia and microphthalmia are generally used interchangeably. Microphthalmia is an eye disorder in which one or both eyes are abnormally small, while anophthalmia is the absence of one or both eyes. These very rare disorders develop during pregnancy and may be associated with other birth defects.

What causes anophthalmia and microphthalmia?

Causes of these conditions may include genetic mutations and abnormal chromosomes. Researchers also believe that environmental factors, such as exposure to X-rays, chemicals, drugs, pesticides, toxins, radiation, or viruses, increase the risk of anophthalmia and microphthalmia, but research is not conclusive. Sometimes the cause in an individual patient cannot be determined.

Can anophthalmia and microphthalmia be treated?

There is no treatment for severe anophthalmia or microphthalmia that will create a new eye or restore vision. However, some less severe forms of microphthalmia may benefit from medical or surgical treatments. In almost all cases improvements to a child’s appearance are possible.

Children can be fitted for a prosthetic (artificial) eye for cosmetic purposes and to promote socket growth. A newborn with anophthalmia or microphthalmia will need to visit several eye care professionals, including those who specialize in pediatrics, vitreoretinal disease, orbital and oculoplastic surgery, ophthalmic genetics, and prosthetic devices for the eye.

Each specialist can provide information and possible treatments resulting in the best care for the child and family. The specialist in prosthetic diseases for the eye will make conformers, plastic structures that help support the face and encourage the eye socket to grow.

As the face develops, new conformers will need to be made. A child with anophthalmia may also need to use expanders in addition to conformers to further enlarge the eye socket. Once the face is fully developed, prosthetic eyes can be made and placed. Prosthetic eyes will not restore vision.

How do conformers and prosthetic eyes look?

A painted prosthesis that looks like a normal eye is usually fitted between ages one and two. Until then, clear conformers are used. When the conformers are in place the eye socket will look black. These conformers are not painted to look like a normal eye because they are changed too frequently.

Every few weeks a child will progress to a larger size conformer until about two years of age. If a child needs to wear conformers after age two, the conformers will be painted like a regular prosthesis, giving the appearance of a normal but smaller eye. The average child will need three to four new painted prostheses before the age of 10.

How is microphthalmia managed if there is residual vision in the eye?

Children with microphthalmia may have some residual vision (limited sight). In these cases, the good eye can be patched to strengthen vision in the microphthalmic eye. A prosthesis can be made to cap the microphthalmic eye to help with cosmetic appearance, while preserving the remaining sight.

Source: The National Eye Institute (NEI) is part of the National Institutes of Health (NIH) and is the Federal government’s lead agency for vision research that leads to sight-saving treatments and plays a key role in reducing visual impairment and blindness.

Source page was last modified in October 2009