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What Is Proliferative Sickle Cell Retinopathy?

Proliferative sickle cell retinopathy is caused by sickle cell disease and leads to the growth of abnormal blood vessels in the eye. Proliferative sickle cell retinopathy (PSR) is a complication in the eyes that can happen in some people who have sickle cell disease. Sickle cell disease is an inherited disorder that affects how red blood cells work. PSR causes new, abnormal blood

  • Posted on 04th May, 2022 12:35 PM
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What Is Proliferative Sickle Cell Retinopathy? Image

Proliferative sickle cell retinopathy (PSR) is a complication in the eyes that can happen in some people who have sickle cell disease. Sickle cell disease is an inherited disorder that affects how red blood cells work. PSR causes new, abnormal blood vessels to grow and leak behind the light-sensitive tissue in the back of the eye called the retina. The leakage from these blood vessels can lead to vision loss.

About 10% of people with sickle cell disease will experience some sort of vision problem due to their condition.

This article will address symptoms, causes, diagnosis, and treatment for proliferative sickle cell retinopathy.

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Proliferative Sickle Cell Retinopathy Symptoms

For many people, PSR does not have any symptoms. This is one reason why, starting at age 10, people with sickle cell disease are encouraged to set annual appointments with a retina specialist. This type of healthcare provider can check for signs of PSR.

When PSR does have symptoms, they include:

  • Blind spots
  • Blurred vision
  • Light flashes
  • Loss of peripheral (side) vision
  • Sudden onset of floaters, which are spots, dots, or circles that you see in your field of vision

Causes of PSR

Proliferative sickle cell retinopathy is caused by sickle cell disease. Sickle cell disease is an inherited blood disorder that causes red blood cells to become hard and sticky. These red blood cells die sooner, causing a constant shortage of red blood cells in the body. Surviving red blood cells can cause blockages in the blood vessels.

These complications can cause your eyes to have less blood flow and, therefore, less oxygen is getting to them. The blockages can lead to the growth of irregular blood cells. These irregular blood cells leak blood behind the retina.

Diagnosis

An eye doctor, such as a retina specialist, can use several tools to help diagnose PSR, including:

  • Fluorescein angiography: This is a type of imaging that can obtain a better view of blood vessels in the retina.
  • Optical coherence tomography angiography: This type of imaging can detect blocked blood vessels due to sickle cell disease, as well as changes in the vessels of the retina and choroid. The choroid is a thin area of tissue in the middle layer of the eye wall.
  • Spectral domain optical coherence tomography: This is a type of imaging that shows cross-sectional images of the retina, which can help detect areas with retina thinning.

During an exam, your eye care specialist will check for other eye conditions with signs and symptoms that resemble proliferative sickle cell retinopathy. These include:

  • Diabetic retinopathy: A condition characterized as damage to the blood vessels in the retina due to type 1 or type 2 diabetes
  • Retinal vascular occlusion: When one or more arteries carrying blood to the retina are blocked
  • Sarcoidosis: A systemic inflammatory disease that occurs when cells in the immune system form clumps called granulomas—in this case, in the eyes (ocular sarcoidosis).
  • Other conditions and diseases that affect the eye

The Goldberg Stages

To help identify PSR, eye doctors often use what's called the Goldberg stages that describe what is happening to the eye. These range from stages 1 to 5.

Stage 5 proliferative sickle cell retinopathy, the most advanced form includes retinal detachment. This is what happens when the retina pulls away from the back of the eye.

A retinal detachment is a medical emergency.

Treating Proliferative Sickle Cell Retinopathy

The treatments available for proliferative sickle cell retinopathy are:

  • The use of laser treatment for blood vessel blockages. Laser treatment can help ward off abnormal blood vessel growth.
  • Drugs such as anti-vascular endothelial growth factors (anti-VEGF) can stop new blood vessels from forming in the eye. This type of drug is given through an injection in the eye.
  • Surgery for a retinal detachment or a vitreous hemorrhage. Vitreous hemorrhage is bleeding that occurs in the vitreous, a jelly-like area of the eye.

If there is only a small area of abnormal blood vessels, your eye doctor may choose to observe the area via regular eye exams and not offer any specific treatment. This is because these small areas of growth often go away on their own.

If you have sickle cell disease, it's important to maintain your treatments to help prevent further problems.

PSR Prognosis

The prognosis (likely outcomes) for PSR will depend on the stage. It can cause vision loss, including total blindness, at its most advanced stages. This is another reason why annual eye exams are crucial when you have sickle cell disease.

Summary

Proliferative sickle cell retinopathy is an eye condition that is caused by sickle cell disease. It may not have any symptoms, but it can cause vision loss at its advanced stages. Eye doctors can diagnose PSR with several types of imaging. Treatment may include lasers, surgery, and the use of drugs injected into the eye.

PSR is just one way that sickle cell disease can affect the body. Maintain recommended checkups with your healthcare team to better manage your sickle cell disease. Consistent visits can help identify any emerging problems earlier, before they become advanced.

A Word From Verywell

Having an eye condition that can cause vision loss is scary. Know that total vision loss is rare with PSR—about 10% of people with sickle cell disease experience vision problems, and a fraction of those with vision problems experience total vision loss. Detection is key, which is why it's important to get an annual eye exam and discuss prevention and treatment options with your eye doctor.

What Is Proliferative Sickle Cell Retinopathy? View Story