Table of ContentsView AllTable of ContentsSymptomsCausesDiagnosisTreatmentPrognosisSummaryA Word From Verywell

Table of ContentsView All

View All

Table of Contents

Symptoms

Causes

Diagnosis

Treatment

Prognosis

Summary

A Word From Verywell

Proliferative sickle cell retinopathy(PSR) is a complication in the eyes that can happen in some people who havesickle 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 theretina. 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.

What Is Sickle Cell Retinopathy?

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Illustration of damage to the retina from retinopathy

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 aretina specialist. This type of healthcare provider can check for signs of PSR.

When PSR does have symptoms, they include:

Causes of PSR

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.

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

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:

The Goldberg StagesTo 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 includesretinal detachment.This is what happens when the retina pulls away from the back of the eye.A retinal detachment is a medical emergency.

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 includesretinal detachment.This is what happens when the retina pulls away from the back of the eye.A retinal detachment is a medical emergency.

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 includesretinal 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:

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.

What to Expect When You Go For a Routine Eye Exam

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.

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.

How Sickle Cell Disease Can Affect Your Eyes

5 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.St. Jude Children’s Research Hospital.Retinopathy and sickle cell disease.Akhter M, Latting MW, Scott AW, et al.Management of proliferative sickle cell retinopathy. American Academy of Ophthalmology.American Society of Retina Specialists.Sickle cell retinopathy.Sambhara D.Sickle cell retinopathy. American Academy of Ophthalmology.Columbia University Department of Ophthalmology.Sickle cell proliferative retinopathy.

5 Sources

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.St. Jude Children’s Research Hospital.Retinopathy and sickle cell disease.Akhter M, Latting MW, Scott AW, et al.Management of proliferative sickle cell retinopathy. American Academy of Ophthalmology.American Society of Retina Specialists.Sickle cell retinopathy.Sambhara D.Sickle cell retinopathy. American Academy of Ophthalmology.Columbia University Department of Ophthalmology.Sickle cell proliferative retinopathy.

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.

St. Jude Children’s Research Hospital.Retinopathy and sickle cell disease.Akhter M, Latting MW, Scott AW, et al.Management of proliferative sickle cell retinopathy. American Academy of Ophthalmology.American Society of Retina Specialists.Sickle cell retinopathy.Sambhara D.Sickle cell retinopathy. American Academy of Ophthalmology.Columbia University Department of Ophthalmology.Sickle cell proliferative retinopathy.

St. Jude Children’s Research Hospital.Retinopathy and sickle cell disease.

Akhter M, Latting MW, Scott AW, et al.Management of proliferative sickle cell retinopathy. American Academy of Ophthalmology.

American Society of Retina Specialists.Sickle cell retinopathy.

Sambhara D.Sickle cell retinopathy. American Academy of Ophthalmology.

Columbia University Department of Ophthalmology.Sickle cell proliferative retinopathy.

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