Eye disorder: Wagner syndrome

Wagner’s syndrome is a hereditary eye disorder. This is a rare condition affecting the retina within the eye and the vitreous humor that fills the eyeball. There is a decrease in choroid tissue. If you have this condition, healing is not possible. It can cause cataracts at an early age or a retinal detachment. What is Wagner’s syndrome and how does this disease manifest itself?

What Wagner’s syndrome entails

In 1938, the Swiss ophthalmologist described the symptoms of this condition affecting the retina and vitreous humor of the eye after which the condition is named. In 2005, the gene responsible for this hereditary condition was discovered in Japan. Eye examination shows that this condition causes abnormalities in the structure of the vitreous body. Because blood vessels at the edge of the retina are missing or weaker, the retina is weaker than it should be. This can cause night blindness. This condition can cause cataracts to develop at a young age, from the age of thirty. As a result of Wagner’s syndrome, there is a decrease in choroid tissue. Most people with this condition are moderately (from 2) to very nearsighted (from 6).

Heredity

Wagner’s syndrome is a hereditary condition. Children have a 50% chance of developing this condition. It doesn’t matter whether the child is a boy or a girl. The syndrome only occurs in white, Japanese and Chinese families. Not everyone who has this syndrome will have complaints. A quarter of these people experience little or almost no symptoms. Half suffer moderately from the condition and a quarter of these patients have serious problems, such as detachment of the retina. As the patient ages, the disease will have a progressive course due to an increase in degenerative symptoms.

Vitreous and retinal abnormalities

As mentioned, the vitreous and retina are affected by Wagner’s syndrome. These two deviations are discussed below:

Vitreous humour

The most striking phenomenon is that the normal structure of the vitreous is missing. Normally the eye is filled with a gel-like substance. In people who have Wagner’s syndrome, the doctor’s eye is optically empty in which veils are visible. Characteristic is a circular vitreous condensation that appears as a white glossy line in front of the retina. This is sometimes clearly visible as a white band or sometimes less clearly as a subtle shine line. From the age of 2 years it can be determined whether there is abnormal vitreous humor.

Retina

Due to weak or even missing blood vessels at the edge of the retina, it is weaker than usual. This can cause night blindness, visual field limitations, atrophy (shrinking) of the choroid and cataracts at a younger age. As one ages, the retina’s light sensitivity decreases. In more severe cases, the retina may detach. This is usually caused by shrinking of the membranes, the veils in the vitreous or by fluid getting under the retina.

Expression of the disease

The symptoms of the disease almost always manifest themselves. This may be barely noticeable for one person and the other may have serious complaints. If you have this defective gene, you will also have the symptoms. The following symptoms may be manifested:

Cataract

Cataracts are more common in people with Wagner syndrome and especially at a younger age. A cloudiness may develop in the lens nucleus, along the axes of the lens in a star-shaped pattern or in the posterior or anterior lens cortex. The treatment is the same as regular cataract surgery, but must be carried out by an experienced doctor due to the abnormal vitreous and risks of retinal detachments.

Myopia

Nearsightedness (myopia) occurs because the lens does not have enough refractive power to allow the image to reach the retina. The eye is slightly too long in shape or the refractive system consisting of cornea, aqueous humor, lens and vitreous is insufficient.

Therapy

Retina

If tears appear in the retina, this can be treated with laser or freezing. This is only possible if there is no fluid under the retina. The area around the retinal tear is secured to a base layer, so that further tears and moisture under the retina are prevented. If there is a retinal detachment, the surgeon will have to intervene. A band may then be placed around the eye or glass surgery may be performed. During a glass operation (vitrectomy), the vitreous will be removed and temporarily replaced by gas or oil. Later, the eye will fill again with aqueous humor that is produced in the eye.

Light sensitivity

Because people with Wagner’s syndrome have reduced light sensitivity in most cases, there is a greater need for contrast. PL daylight lamps can contribute to this. These are available at the better lighting stores. Wearing contact lenses also creates more contrast. You can contact an contact lens specialist for this.

Dry eyes

If many eye drops are used, dry eyes may develop. An ophthalmologist can prescribe artificial tears for this. An alternative that will also help in most cases is linseed. It is recommended to add 10 grams of broken linseed per day, for example in yogurt or homemade bread.

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