Polypoidal Choroidal Vasculopathy: Diagnosis and Treatment
Understanding Polypoidal Choroidal Vasculopathy
PCV is a disorder of the choroid, the layer of blood vessels that nourishes the retina. In this condition, abnormal blood vessels grow beneath the retinal pigment epithelium (RPE), the supportive layer under the retina. These abnormal vessels form a branching network with balloon-like bulges at their tips, called polyps or polypoidal lesions.
The polypoidal lesions are fragile. They can leak fluid or burst and bleed beneath the retina. When this happens in or near the macula, the central part of the retina responsible for sharp vision, it can cause significant vision loss.
PCV shares features with wet age-related macular degeneration (AMD) because both involve abnormal blood vessel growth beneath the retina. However, PCV has distinct characteristics. The polyp-like bulges at the ends of blood vessels are a hallmark of PCV and are not typically seen in standard wet AMD.
PCV also tends to affect a somewhat younger age group and has a stronger association with certain ethnic backgrounds. These differences matter because they can influence which treatments work best and how the condition behaves over time.
In PCV, the walls of small blood vessels in the choroid begin to degenerate. This leads to a type of abnormal vessel growth called type 1 neovascularization, where new vessels push through Bruch membrane, a thin barrier between the choroid and the retina. These new vessels form a branching network beneath the RPE. At certain points along this network, the vessel walls weaken and bulge outward, creating the characteristic polypoidal lesions.
Vascular endothelial growth factor (VEGF), a protein that promotes blood vessel growth, has been found in the RPE of patients with PCV. However, its exact role in driving the disease is still being studied.
Who Is Affected and Risk Factors
PCV is most commonly diagnosed in patients between age 50 and 65, typically presenting in the sixth to seventh decade of life (AAO EyeWiki). The condition affects both men and women, though the ratio between sexes can vary depending on the population studied.
Ethnicity is one of the strongest risk factors for PCV. Hospital-based studies show PCV prevalence as high as 22 to 62 percent among presumed wet AMD cases in Asian populations and about 10 to 20 percent in Caucasian populations (PMC, 2023). Among European patients undergoing detailed retinal examination for suspected wet AMD, the prevalence of PCV is approximately 8.3 percent (PMC, 2022). PCV is also more common in individuals of African descent.
Research has identified several genetic variants linked to PCV risk. These include variants in genes known as CETP and LOC387715/HTRA1. Having these variants does not mean a person will develop PCV, but they may increase susceptibility.
Other recognized risk factors include:
- Higher body mass index (BMI)
- Higher education level, which may reflect differences in screening and diagnosis rates
- Family history of macular degeneration
Signs and Symptoms
The most common symptom of PCV is a change in central vision. Many patients notice that straight lines appear wavy or distorted, a symptom called metamorphopsia. Others may experience a gradual blurring of central vision that makes it harder to read, recognize faces, or see fine details.
PCV can cause sudden and severe vision loss. This happens when a polypoidal lesion ruptures and bleeds beneath or into the retina. A large bleed, called a submacular hemorrhage, can damage the delicate photoreceptor cells that capture light and send signals to the brain. In rare cases, bleeding can extend into the vitreous, the gel that fills the eye, causing a sudden onset of floaters or a dark curtain across the visual field.
If you experience a sudden decrease in vision, a sudden increase in floaters, or a shadow or curtain over your vision, see a retina specialist or go to the emergency room immediately. These symptoms require urgent evaluation.
Some patients with PCV experience repeated episodes of bleeding in the macula. Each episode can cause additional damage to the retina. Over time, recurrent bleeding and fluid leakage may lead to irreversible central vision loss in one or both eyes. This is why ongoing monitoring by a retina specialist is important even after initial treatment.
Diagnosis and Testing
Diagnosis of PCV begins with a thorough dilated eye exam. During this exam, a retina specialist can see characteristic findings such as orange-red nodular lesions beneath the retina. Fluid-filled or blood-filled elevations of the RPE, known as serosanguineous pigment epithelial detachments, may also be visible.
Indocyanine green angiography (ICGA) has traditionally been considered the gold standard for confirming a PCV diagnosis. In this test, a special dye is injected into a vein in the arm. The dye travels to the choroidal blood vessels, where a camera captures detailed images. ICGA reveals the branching vascular network and the polypoidal lesions that define PCV.
Optical coherence tomography (OCT) is a noninvasive imaging test that creates high-resolution cross-sectional images of the retina. OCT can show fluid beneath the retina, elevations of the RPE, and sometimes the polypoidal lesions themselves. Recent advances in OCT technology have made it possible to identify PCV features in many cases, and OCT-guided treatment can now successfully target therapy sites, covering 90 percent of branching neovascular networks and 100 percent of polypoidal lesions in some studies (ASRS).
OCT angiography (OCTA) is a newer imaging technique that maps blood flow in the retina and choroid without the need for dye injection. OCTA can detect the abnormal branching vascular network associated with PCV. While it may not replace ICGA in all situations, it provides valuable information and can reduce the need for invasive testing.
Treatment Options
Anti-VEGF medications are a primary treatment for PCV. These drugs are injected directly into the eye, a procedure called an intravitreal injection. They work by blocking VEGF, a protein that drives abnormal blood vessel growth and leakage. Treatment typically begins with a series of three monthly loading injections, followed by additional injections as needed based on how the eye responds.
Common anti-VEGF medications used for PCV include:
- Eylea (aflibercept), typically given every 4 to 8 weeks after loading doses
- Lucentis (ranibizumab), typically given every 4 weeks
- Avastin (bevacizumab), which is FDA-approved for cancer treatment but used off-label for eye conditions, typically given every 4 to 6 weeks
- Vabysmo (faricimab), a bispecific antibody that targets both VEGF and a protein called angiopoietin-2, with intervals that may extend to every 4 to 16 weeks
Photodynamic therapy (PDT) is a light-activated drug treatment used for PCV. A light-sensitive medication called verteporfin is injected into a vein. The medication collects in the abnormal blood vessels beneath the retina. A retina specialist then shines a low-energy laser on the affected area. The laser activates the drug, which helps close the abnormal polypoidal lesions.
PDT is particularly effective at causing polyp regression. It can be used alone or, more commonly, in combination with anti-VEGF injections.
Combining anti-VEGF injections with PDT has shown superior polyp regression rates compared to anti-VEGF injections alone. The landmark EVEREST II trial demonstrated that combination therapy led to better polyp closure and visual outcomes for many patients. In this approach, PDT targets the polypoidal lesions directly while anti-VEGF medication addresses the broader vascular abnormalities and fluid leakage.
A retina specialist will determine whether combination therapy is appropriate based on the location and severity of the polyps, the amount of fluid and bleeding, and how the eye has responded to prior treatments.
In rare cases where PCV causes a large hemorrhage beneath the retina or into the vitreous cavity, a retina specialist may recommend vitrectomy (a surgery to remove the gel inside the eye). During vitrectomy, the surgeon can remove or displace the blood to reduce damage to the photoreceptor cells. This is typically reserved for severe bleeding that threatens significant and permanent vision loss.
What to Expect
Intravitreal injections are performed in a retina specialist's office. The eye is numbed with anesthetic drops or a local anesthetic. An antiseptic solution is applied to reduce infection risk. The injection itself takes only a few seconds. Most patients feel pressure or mild discomfort rather than sharp pain. Temporary floaters or mild soreness after the injection are common.
PCV treatment usually requires ongoing care. After the initial loading phase of three monthly injections, a retina specialist will monitor your eye with regular OCT imaging and clinical exams. Additional injections may be needed if fluid or bleeding returns. Some patients achieve stable vision with fewer injections over time, while others need consistent treatment to maintain their results.
The PLANET study showed that Eylea (aflibercept) monotherapy achieved meaningful visual gains without the need for PDT in most cases (ASRS). However, each patient's response is different, and your retina specialist will tailor the treatment plan to your specific needs.
Many patients with PCV can maintain or improve their vision with timely and consistent treatment. However, outcomes vary depending on the extent of damage at the time of diagnosis, the location of the polyps, and how well the condition responds to therapy. Some patients may experience vision that stabilizes but does not fully recover, especially if significant bleeding or scarring has already occurred.
Living With PCV
Between appointments, it is important to monitor your vision regularly. An Amsler grid is a simple tool that can help you detect changes in your central vision. To use it, cover one eye at a time and look at the center dot of the grid. If any of the lines appear wavy, broken, or missing, contact your retina specialist promptly.
Pay attention to any new distortion, blurriness, or dark spots in your central vision. Early detection of changes allows for faster treatment, which can improve outcomes.
While there is no proven way to prevent PCV, general eye health practices may support retinal health. These include:
- Eating a diet rich in leafy green vegetables, fish, and other sources of omega-3 fatty acids
- Maintaining a healthy body weight
- Protecting your eyes from excessive ultraviolet light with sunglasses
- Not smoking, as smoking is harmful to blood vessel health throughout the body
Living with a chronic eye condition can be stressful. Vision changes may affect your ability to drive, read, or perform daily tasks. Low vision rehabilitation services can help you make the most of your remaining vision with specialized tools and strategies. Support groups for people with macular conditions can also provide valuable encouragement and practical advice.
When to See a Retina Specialist
Certain symptoms require immediate attention. See a retina specialist or go to the emergency room right away if you experience:
- A sudden decrease in vision in one or both eyes
- A sudden increase in floaters
- Flashes of light
- A dark curtain or shadow across your field of vision
- New or worsening distortion of straight lines
If you have been diagnosed with PCV, regular follow-up appointments are essential. Even if your vision feels stable, your retina specialist may detect fluid or early signs of recurrence on imaging before you notice any symptoms. Consistent monitoring allows for timely retreatment and helps protect long-term vision.
If you are over 50, especially if you are of Asian or African descent, and you notice any changes in your central vision, ask your eye care provider about a referral to a retina specialist for a thorough evaluation.
Questions and Answers
PCV is now considered a subtype of wet age-related macular degeneration, but it has important differences. The hallmark polypoidal lesions, the tendency to cause larger hemorrhages, and the strong response to photodynamic therapy set PCV apart from typical wet AMD. These differences can influence which treatment approach a retina specialist recommends.
The duration of treatment varies from person to person. Some patients achieve long-term stability after an initial course of treatment and can be monitored with less frequent visits. Others may need periodic injections over many years to control fluid leakage and prevent recurrence. Your retina specialist will evaluate your response at each visit and adjust the treatment schedule accordingly.
Yes, PCV can affect both eyes, though it often begins in one eye first. If you have been diagnosed with PCV in one eye, your retina specialist will monitor your other eye closely for any early signs of the condition. Prompt treatment of the second eye, if needed, can help preserve vision.
Research into PCV treatment continues to advance. Vabysmo (faricimab), which targets both VEGF and angiopoietin-2, has shown promise for increased treatment durability, potentially allowing longer intervals between injections. Advances in OCT-guided photodynamic therapy are also improving how retina specialists target treatment. Clinical trials continue to explore new therapies and combination approaches to improve outcomes for patients with PCV.
PCV primarily affects central vision. Even in severe cases, most patients retain some degree of peripheral, or side, vision. However, untreated or poorly controlled PCV can cause significant and irreversible central vision loss, making it difficult to read, drive, or recognize faces. This is why early diagnosis and consistent treatment are so important for preserving functional vision.