Choroidal Hemangioma: Understanding This Benign Eye Tumor
Understanding Choroidal Hemangioma
A choroidal hemangioma is a benign vascular tumor that grows within the choroid, the blood vessel layer beneath the retina. The choroid supplies oxygen and nutrients to the outer layers of the retina. When an abnormal collection of blood vessels forms in this layer, it creates a raised, orange-red mass that can disrupt normal retinal function.
These tumors are not cancers. They do not spread to other parts of the body. However, they can cause vision problems if they leak fluid beneath or into the retina, especially when they are located near the macula, the central area responsible for sharp, detailed vision.
There are two main types of choroidal hemangioma. The circumscribed type is a single, well-defined tumor usually found in the back of the eye near the macula. The diffuse type spreads more broadly across the choroid and is frequently associated with a condition called Sturge-Weber syndrome.
Under a microscope, these tumors are classified by the type of blood vessels they contain. The cavernous type has large vessels separated by limited connective tissue. The capillary type has smaller vessels separated by loose connective tissue. A mixed type contains features of both. Regardless of type, these tumors do not show evidence of active cell growth. Any enlargement is most likely the result of venous congestion rather than new cell production.
Circumscribed choroidal hemangioma (CCH) is the more common form seen in general practice. It appears as a solitary, orange-red mass in the posterior pole of the eye. CCH has no known association with other systemic conditions. In contrast, diffuse choroidal hemangioma (DCH) occurs frequently alongside Sturge-Weber syndrome, a rare condition characterized by a facial birthmark called a port-wine stain, along with brain and eye abnormalities. Approximately 20 to 71 percent of patients with Sturge-Weber syndrome develop diffuse choroidal hemangioma, which may also increase the risk of glaucoma (Radiopaedia).
Who Is Affected and Risk Factors
Choroidal hemangiomas are rare. To put this in perspective, there is roughly one circumscribed choroidal hemangioma diagnosed for every 15 choroidal melanomas (ScienceDirect). Over a 26-year period at one major eye center, approximately 200 cases of circumscribed choroidal hemangioma were diagnosed (Radiopaedia).
These tumors are most often discovered in middle-aged adults. In a review of 200 consecutive patients with CCH, the mean age at presentation was 47 years, with a range from 4 to 81 years. A more recent study of 238 patients diagnosed between 2002 and 2018 found a mean age of 53.8 years, with 60 percent of patients being male. Nearly all reported cases of circumscribed choroidal hemangioma have been in Caucasian individuals.
Sturge-Weber syndrome (SWS) is a rare, sporadic condition with an estimated incidence of 1 in 20,000 to 1 in 50,000 infants (Radiopaedia). It has no known sex or racial predilection. The hallmark features include a port-wine stain on the face in the area supplied by the trigeminal nerve, a brain leptomeningeal hemangioma, and diffuse choroidal hemangioma. If you or a family member has been diagnosed with Sturge-Weber syndrome, regular eye examinations are important because of the increased risk of both choroidal hemangioma and glaucoma.
Choroidal hemangiomas are believed to be congenital vascular hamartomas, meaning they are present from birth as developmental abnormalities of blood vessels. They occur sporadically and are not inherited in a predictable pattern. There is no known way to prevent them from forming.
Signs and Symptoms
Many choroidal hemangiomas are asymptomatic, meaning they cause no noticeable problems. These are often discovered incidentally during a routine eye examination. When symptoms do occur, they typically develop between the third and sixth decades of life.
Common visual symptoms include:
- Blurred or reduced vision
- Metamorphopsia (distorted vision where straight lines appear wavy or bent)
- Hyperopic shift (a change toward farsightedness caused by the tumor pushing the retina forward)
- Photopsia (flashing lights)
- Floaters
- Visual field defects (blind spots or missing areas in your field of vision)
The tumor itself does not directly damage the retina in most cases. Instead, vision problems arise from the secondary effects of the tumor. Fluid can leak from the abnormal blood vessels and collect beneath the retina, causing a condition called exudative retinal detachment. The tumor can also cause cystoid macular edema, a condition where fluid-filled cysts develop in the macula.
In long-standing cases, additional complications may develop. These include changes to the retinal pigment epithelium (the supportive layer beneath the retina), subretinal fibrosis (scar tissue beneath the retina), retinoschisis (splitting of retinal layers), and photoreceptor degeneration. Early detection and treatment can help prevent these advanced changes.
If you experience a sudden change in vision, new floaters, flashes of light, or a curtain-like shadow across your field of vision, see a retina specialist or go to the emergency room immediately. While these symptoms may be related to a known choroidal hemangioma, they can also indicate other serious conditions such as retinal detachment that require prompt evaluation.
Diagnosis and Testing
A retina specialist can often identify a choroidal hemangioma during a dilated eye examination. Through the ophthalmoscope, circumscribed choroidal hemangiomas appear as orange or reddish-orange masses with indistinct margins that blend into the surrounding choroidal tissue. Some tumors may have areas of increased pigmentation that can resemble a choroidal melanoma, making careful evaluation critical.
One of the most important aspects of diagnosis is distinguishing a choroidal hemangioma from more serious conditions. An amelanotic (non-pigmented) choroidal melanoma and choroidal metastasis (cancer that has spread to the eye from another part of the body) can look similar. A retina specialist will use a combination of imaging tools to make an accurate diagnosis.
Several imaging techniques help confirm the diagnosis and guide treatment decisions. Ultrasound of the eye (B-scan ultrasonography) shows characteristic features of choroidal hemangiomas, including high internal reflectivity that helps differentiate them from melanomas. Fluorescein angiography uses a special dye injected into a vein in the arm to highlight the blood vessel pattern of the tumor. Indocyanine green angiography provides additional detail about the choroidal blood vessels.
Optical coherence tomography (OCT) creates detailed cross-sectional images of the retina and can detect subretinal fluid, cystoid macular edema, and retinal changes caused by the tumor. OCT angiography (OCTA) is a newer, noninvasive tool that reveals a dense, irregular vascular network in the choroidal layers. The vessels within the tumor appear larger than the surrounding normal choroidal vessels. OCTA can also be used to monitor treatment response over time.
Because the treatment for a benign choroidal hemangioma is vastly different from the treatment for a choroidal melanoma or metastatic tumor, accurate diagnosis is essential. Retina specialists and ocular oncologists use the combination of clinical appearance, ultrasound features, angiographic patterns, and OCT findings to reach a confident diagnosis. In rare cases where the diagnosis remains uncertain, additional testing or observation over time may be recommended.
Treatment Options
Not every choroidal hemangioma requires treatment. If the tumor is not causing visual symptoms and there is no subretinal fluid or macular edema, a retina specialist may recommend careful monitoring. Regular examinations and imaging help track the tumor for any changes over time. Treatment is typically reserved for cases where the tumor is causing symptoms or threatening vision.
Photodynamic therapy (PDT) with verteporfin (Visudyne) is considered the treatment of choice for symptomatic circumscribed choroidal hemangiomas. PDT is a non-thermal laser treatment that works differently from traditional laser. A light-sensitive dye called verteporfin is injected into a vein in the arm. The dye concentrates in the abnormal blood vessels of the tumor. A specially tuned laser is then directed at the tumor, activating the dye. The activated dye causes the abnormal blood vessels to close, shrink, and stop leaking fluid.
In one of the largest studies of PDT for choroidal hemangioma, 31 eyes were followed for at least 12 months. The results showed resolution of subretinal and intraretinal fluid in 93 percent of cases, with visual improvement in 69 percent and visual stability in 27 percent (ASRS). PDT has effectively transformed the management of these once difficult-to-treat tumors.
Before PDT became widely available, other treatments were used and may still play a role in certain cases. Laser photocoagulation uses thermal laser energy to seal leaking blood vessels but carries a higher risk of damaging surrounding retinal tissue. Transpupillary thermotherapy (TTT) delivers heat through the pupil to the tumor surface. Plaque brachytherapy involves placing a small radioactive disc on the outside of the eye directly over the tumor to deliver targeted radiation. External beam radiotherapy delivers radiation from outside the body.
Each of these approaches has specific advantages and limitations. A retina specialist or ocular oncologist will recommend the most appropriate treatment based on the size and location of the tumor, the degree of fluid leakage, and the overall health of the eye.
In some cases, PDT may be combined with an intravitreal injection of an anti-VEGF (anti-vascular endothelial growth factor) medication. Anti-VEGF drugs block the signals that cause abnormal blood vessel growth and leakage. This combination approach may be considered when there is a large amount of subretinal fluid, when the tumor is located directly beneath the fovea (the center of the macula), or both. The goal is to reduce fluid more quickly and improve the chances of visual recovery.
What to Expect
PDT is performed as an outpatient procedure. The verteporfin dye is infused into a vein over approximately 10 minutes. After the infusion, the retina specialist applies the PDT laser to the tumor through a special contact lens placed on the eye. The laser treatment itself takes about 83 seconds. The procedure is generally well tolerated. After treatment, you will need to avoid direct sunlight and bright indoor light for about 48 hours because the dye makes your skin and eyes temporarily sensitive to light.
Following PDT, your retina specialist will schedule follow-up appointments to monitor the response. OCT imaging is used to check whether subretinal fluid and macular edema have resolved. OCT angiography may also be used to assess changes in the tumor's blood vessel network. In some cases, more than one PDT session may be needed to achieve a complete response.
Visual recovery depends on several factors, including how long the fluid was present before treatment, the location of the tumor relative to the macula, and whether any permanent retinal changes had already occurred. Patients treated earlier in the course of the disease tend to have better visual outcomes.
Choroidal hemangiomas are benign and do not spread to other parts of the body. With modern treatment, most patients experience stabilization or improvement of vision. However, long-standing cases with chronic retinal detachment, subretinal fibrosis, or photoreceptor degeneration may have limited visual recovery even after successful treatment. Ongoing monitoring is important because fluid can sometimes recur, and additional treatment may be necessary.
Living With Choroidal Hemangioma
If you have been diagnosed with a choroidal hemangioma, your retina specialist may recommend using an Amsler grid at home. This simple tool is a grid of straight lines with a central dot. By looking at the grid with one eye at a time, you can detect changes in your central vision such as waviness, blurriness, or missing areas. Report any new changes to your retina specialist promptly.
Even after successful treatment, regular follow-up with a retina specialist is important. These visits allow early detection of any fluid recurrence or changes in the tumor. If you have Sturge-Weber syndrome, your eye care should also include monitoring for glaucoma, which is a common associated condition. Coordinating care between your retina specialist and other physicians involved in your Sturge-Weber syndrome management helps ensure comprehensive treatment.
Being told you have a tumor in your eye can be frightening, even when the tumor is benign. It is completely normal to feel anxious about your diagnosis and the potential impact on your vision. Understanding that choroidal hemangiomas are not cancers and that effective treatments exist can provide reassurance. If you have concerns or questions between appointments, do not hesitate to contact your retina specialist.
When to See a Retina Specialist
You should see a retina specialist if you notice blurred vision, distorted vision, a change in your glasses prescription toward farsightedness, flashing lights, or new floaters. If a general eye doctor identifies an unusual mass in the back of your eye during a routine examination, a referral to a retina specialist or ocular oncologist is an important next step for proper evaluation.
Sudden vision loss, a rapid increase in floaters, persistent flashes of light, or a shadow or curtain moving across your vision require immediate attention. See a retina specialist or go to the emergency room right away. These symptoms can indicate an exudative retinal detachment or other serious complication that needs prompt treatment.
Questions and Answers
No. Choroidal hemangiomas are benign vascular growths. They do not transform into cancer and they do not metastasize (spread to other parts of the body). However, because other types of eye tumors such as choroidal melanoma can look similar, an accurate diagnosis by a retina specialist or ocular oncologist is essential to rule out more serious conditions.
Some patients respond fully to a single session of photodynamic therapy, while others may require additional treatments. Your retina specialist will use OCT imaging and clinical examination to determine whether the subretinal fluid has resolved completely. If fluid persists or recurs, a repeat PDT session or combination therapy may be recommended. The number of treatments needed varies from person to person.
Circumscribed choroidal hemangioma is almost exclusively a unilateral condition, meaning it affects only one eye. Diffuse choroidal hemangioma associated with Sturge-Weber syndrome also typically involves one eye, usually on the same side as the facial port-wine stain. Bilateral involvement is extremely rare.
Treatment with photodynamic therapy is highly effective at resolving fluid and stabilizing or improving vision. However, the degree of visual recovery depends on how long the retina was affected before treatment. If chronic subretinal fluid caused permanent changes to the retinal pigment epithelium or photoreceptors, some degree of vision loss may persist even after the fluid is completely resolved. This is one reason why early evaluation and treatment of symptomatic cases are important.
Circumscribed choroidal hemangiomas occur sporadically and are not inherited. Routine screening of children is not necessary unless there are other concerning findings. However, if a child has features of Sturge-Weber syndrome, such as a port-wine stain on the face, comprehensive eye examinations should begin in infancy to check for diffuse choroidal hemangioma and glaucoma.