Since the 1980s our eye surgeons have had the opportunity to educate and treat patients regarding many rare eye conditions. Here are three of particular interest.
Birdshot Retinochoroidopathy (BR)
Birdshot retinochoroidopathy is a rare, usually painless inflammation of the blood vessels under the retina. It can flare up and then spontaneously subside. We do not know the cause of BR but it occurs most frequently in white women over the age of 40. It can affect both eyes. The patient can recover good vision although it can become a cause of vision loss. Other symptoms could be night blindness and color vision disturbances.
When looking into the eye, the retina appears as if it has been sprayed by birdshot pellets from a shotgun.
Keratoconus is an infrequent disease where the corneal surfaces lose their optical properties as they protrude like a cone.
As a consequence the corneal layer loses its focusing shape, transforms into a thin conical bulge generating distorted visual images. The condition is usually bilateral but it may not progress at the same rate for each eye.
Keratoconus typically starts in teenagers and could slowly progress for the next 10 to 20 years sometimes suddenly stopping altogether. Symptoms and findings are progressive blurring, astigmatism with nearsightedness, frequent optical prescription changes, and inability to use contact lenses.
While the reasons people develop keratoconus are poorly understood genetics may play a role. It is calculated 10% of keratoconus patients have a family member with the condition.
Keratoconus management includes spectacle and contact lens correction. Surgical care can include crosslinking with laser vison but on occasions a corneal transplant may become the only alternative to restore vision.
Another inherited disorder is Stargardt’s disease. Stargardt’s disease typically begins around age eight and fourteen, affecting boys and girls equally. It presents with slight vision blurriness that gets worse over time. By the late 20s, the acuity prevents driving. To perform reading tasks patients need magnification devices.
The retinal cells develop fatty deposits that make it appear as if it is “beaten bronze”.
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