Choroidal Hemangioma: Symptoms, Diagnosis & Treatment

A choroidal hemangioma is a benign vascular tumor that grows within the choroid, the blood-vessel-rich layer lining the back of the eye. It never spreads to other parts of the body and is not cancerous, but it can still threaten vision by leaking fluid underneath the retina and causing detachment or swelling. These tumors come in two forms: a circumscribed type that appears as a solitary, well-defined mass, and a diffuse type that spreads more broadly across the choroid and is almost always linked to a condition called Sturge-Weber syndrome.

What a Choroidal Hemangioma Looks Like Inside the Eye

During a dilated eye exam, an ophthalmologist typically sees an orange-red, dome-shaped elevated mass sitting behind the equator of the eye. Circumscribed choroidal hemangiomas are usually solitary and affect only one eye.¹ Diffuse hemangiomas, by contrast, can blanket a large portion of the choroid, sometimes giving the back of the affected eye a deeper reddish hue compared with the other eye. Because the tumor is made up of tangled, dilated blood vessels rather than rapidly dividing cells, it behaves more like a structural abnormality than a typical tumor. Still, those leaky vessels are the root of most problems.

Symptoms

Many circumscribed choroidal hemangiomas produce no symptoms at all and are discovered incidentally during a routine eye exam. When symptoms do appear, they tend to show up in middle-aged adults and include gradually worsening vision, distorted or wavy vision (metamorphopsia), floaters, and blind spots or visual field defects.² The severity depends largely on location. A hemangioma sitting directly beneath the macula, the part of the retina responsible for sharp central vision, is far more likely to cause noticeable visual loss than one tucked away toward the periphery.

The visual symptoms are not caused by the tumor mass itself pressing on anything. Instead, they result from fluid that seeps out of the abnormal blood vessels and collects under or within the retina. This subretinal fluid lifts the retina away from the tissue that nourishes it, producing a serous retinal detachment. Swelling within the macula, called cystoid macular edema, can also develop. Both conditions distort the image the retina sends to the brain.³ If the fluid is left unaddressed for a long time, permanent photoreceptor damage can set in, and vision loss may become irreversible even after the fluid is eventually cleared.

Why Diagnosis Can Be Tricky

One of the challenges with choroidal hemangiomas is that their appearance can overlap with far more dangerous conditions. On a standard exam, a circumscribed choroidal hemangioma can look a lot like a choroidal melanoma (the most common primary cancer inside the eye) or a metastatic tumor that has spread to the eye from somewhere else in the body.⁴ Getting the diagnosis right matters enormously, because the treatment paths diverge completely. A melanoma may require enucleation (removal of the eye) or intensive radiation, while a hemangioma might need nothing more than observation.

To sort this out, clinicians lean on multiple imaging tools. Because no single test is definitive on its own, the standard approach is multimodal imaging, combining several techniques to build a clear picture.⁵

Imaging Tools Used for Diagnosis

Ultrasound is often the first imaging study performed. On A-scan ultrasonography, choroidal hemangiomas show characteristically high internal reflectivity without obvious signal drop-off, a pattern that helps distinguish them from melanomas, which tend to show low internal reflectivity. On B-scan, the tumor appears as a smooth, dome-shaped mass with uniformly strong internal echoes.⁶

Optical coherence tomography (OCT), especially the enhanced depth imaging variant, gives a detailed cross-sectional view of the retina and choroid. In one study, the most consistent OCT finding was a normal-looking honeycomb pattern in the layer of tiny blood vessels just beneath the retina, present in every lesion examined. Other common features included damage to the photoreceptor layers and subretinal fluid containing small reflective speckles.⁷

Indocyanine green angiography (ICG) is particularly useful for hemangiomas because the dye highlights choroidal blood flow. Choroidal hemangiomas fill with dye early, reaching peak brightness at an average of about four minutes, and then show a characteristic “washout” in the later frames as the dye drains away. In one series, roughly three-quarters of eyes demonstrated this washout pattern.⁸ That early-fill-then-washout sequence is a strong pointer toward hemangioma and away from melanoma or metastasis.

Fluorescein angiography and MRI can provide additional information. MRI is sometimes used when the clinical picture remains ambiguous, though even MRI findings can occasionally mislead. In rare cases, a choroidal hemangioma has appeared dark on T2-weighted MRI sequences, a pattern more commonly associated with melanoma, reinforcing the need to interpret imaging findings in combination rather than in isolation.⁵

When Treatment Is and Is Not Needed

If a choroidal hemangioma is not leaking fluid and your vision is unaffected, the standard approach is observation with periodic follow-up. There is no urgency to treat a stable, asymptomatic lesion, and unnecessary intervention carries its own risks.¹ Treatment becomes appropriate when the tumor causes a serous retinal detachment, cystoid macular edema, or progressive visual loss.

Photodynamic Therapy

For symptomatic circumscribed choroidal hemangiomas, photodynamic therapy (PDT) with the drug verteporfin has become the go-to treatment. The procedure involves infusing the light-sensitive drug intravenously and then activating it with a low-energy laser aimed at the tumor. The activated drug damages the lining of the abnormal blood vessels, causing them to close off. In a study of 10 patients, every patient showed tumor flattening, resolution of subretinal fluid, and reduced abnormal choroidal blood flow on follow-up angiography.⁹ Another series found complete resolution of subretinal fluid within two weeks, improved visual acuity in every treated eye, and no complications over an average follow-up of about five months.¹⁰

The arrival of PDT represented a meaningful leap forward for patients. A large comparative study of 458 cases found that visual outcomes improved dramatically in the PDT era compared with the years before it was available. The average final visual acuity was roughly equivalent to 20/63 in the PDT era versus about 20/400 in the pre-PDT era. Among patients who started with moderate vision loss, nearly half achieved 20/40 or better after PDT, compared with about a quarter in the earlier era.¹¹

Radiation-Based Treatments

When PDT does not work or is not feasible, radiation offers an alternative. Proton beam therapy delivers a precisely shaped beam of protons to the tumor while sparing surrounding structures. In cases treated with proton beam radiation at a dose of 20 cobalt gray equivalents, one study reported full resolution of subretinal fluid in every patient, tumor shrinkage in about 90%, and improved vision in over half.¹² A longer-term study of 71 patients treated with low-dose proton beam therapy found that vision improved by two lines or more in about half of all patients, and in three-quarters of those treated within six months of their first symptoms.¹³ That last detail is worth underscoring: early treatment appears to matter a lot for visual recovery.

Plaque brachytherapy is another radiation approach. A small radioactive plaque is surgically stitched onto the outside of the eye directly over the tumor, left in place for a calculated period, and then removed in a second procedure. This method works well for circumscribed tumors and can be effective at doses ranging from 25 to 50 Gy, but it is less practical for large diffuse hemangiomas because the plaque may not cover the full extent of the tumor.¹²

Other Treatment Approaches

Transpupillary thermotherapy (TTT) uses a diode laser to deliver heat through the pupil directly to the tumor surface. In a pooled analysis of 38 eyes, foveal detachment resolved in every case. Vision improved in about three-quarters of eyes that started with at least some functional vision, and roughly 40% showed complete tumor regression. However, complications included macular swelling, scar tissue formation on the retinal surface, focal iris damage, and retinal blood vessel blockage in a small number of cases.¹⁴ Older laser photocoagulation techniques can initially absorb subretinal fluid, but recurrence after these treatments is common.¹⁵

Anti-VEGF injections, most commonly bevacizumab, have been used as an add-on to other treatments. In one case treated with a combination of PDT and intravitreal bevacizumab, the tumor remained shrunken and the subretinal fluid and macular edema were fully resolved at twelve months.¹⁶ In a separate series, bevacizumab helped resolve macular detachment in five of nine patients, including some who had already undergone TTT.¹⁷ Anti-VEGF drugs are not considered a standalone cure, but they can be useful for managing the fluid component, especially when it persists after primary treatment.

Long-Term Outlook and Complications of Treatment

Choroidal hemangiomas do not transform into cancer, and the tumor itself is not life-threatening. The real concern is preserving vision, and the long-term picture depends on both the tumor’s behavior and side effects from whatever treatment is used. In a 15-year follow-up of a patient treated with proton beam therapy, the tumor gradually shrank over three years and the retinal detachment improved, but a cataract developed in the treated eye 11 years after radiation, and eye coordination issues appeared at 15 years.¹⁸

A larger long-term study of 50 patients after proton beam irradiation found that about 46% developed some degree of radiation retinopathy, though the vast majority of those cases were mild and did not affect function. More serious complications included cataract formation in about 20% and radiation-related damage to the optic nerve in a small number of patients.¹⁹ These numbers reinforce why observation remains the right choice for patients without symptoms: treatment can create problems of its own, and you only want to accept those risks when the tumor is actively harming vision.

For proton beam patients treated early, the visual prognosis is encouraging. As noted in the low-dose proton study, three-quarters of patients treated within six months of symptom onset gained meaningful vision improvement.¹³ The main complication at longer follow-up in that cohort was cataract in about 28% and radiation maculopathy in about 8%. Cataracts can be surgically removed with good results, so their development is generally considered manageable.

The Sturge-Weber Connection

Diffuse choroidal hemangiomas occur almost exclusively in people with Sturge-Weber syndrome, a rare condition involving abnormal blood vessel growth that typically produces a port-wine birthmark on the face, brain abnormalities, and eye problems. Roughly 40 to 50% of Sturge-Weber patients develop a choroidal hemangioma.²⁰ Unlike the circumscribed type, these diffuse tumors spread across a broad area of the choroid and tend to cause more extensive retinal detachments.

Glaucoma is the most common eye-related complication in Sturge-Weber patients, and in one case series of patients with confirmed choroidal hemangioma, over 70% had a history of glaucoma.²¹ Managing both glaucoma and a diffuse hemangioma in the same eye is a clinical balancing act. Glaucoma surgery in Sturge-Weber patients carries a higher risk of complications, including bleeding beneath the choroid during or after the procedure.²⁰

Treatment decisions for diffuse hemangiomas depend on several factors: how much vision remains, whether subretinal fluid is present, how long the fluid has been there, and whether glaucoma surgery is also being planned.²⁰ PDT can be used but may need larger or repeated treatment spots to cover the broader tumor area. In children with Sturge-Weber who develop total retinal detachments, modified proton therapy techniques have been described, though treating young children poses additional challenges because they cannot cooperate with the precise positioning that standard radiation protocols require.²²

Misidentification and Second Opinions

Because choroidal hemangiomas can so closely mimic melanoma or metastatic cancer, misdiagnosis is a genuine risk. A patient told they have a suspicious choroidal mass may face the prospect of aggressive cancer treatment, including possible removal of the eye, before the true nature of the lesion is established. This makes getting a second opinion from an ocular oncologist or retinal specialist a reasonable step whenever the diagnosis is uncertain.

The imaging hallmarks described above, particularly the high reflectivity on ultrasound, the ICG washout pattern, and the OCT findings, can collectively build a strong case for hemangioma. But each of these features occasionally overlaps with other diagnoses in individual patients. The MRI case where a hemangioma appeared dark on T2-weighted images, mimicking melanoma, is a good example of how a single test can lead clinicians astray.⁵ The combined use of multiple imaging methods is what ultimately protects patients from an incorrect diagnosis and inappropriate treatment.

For circumscribed tumors that remain stable and asymptomatic, many patients live their entire lives without needing any intervention at all. For those who do need treatment, the PDT era has shifted the baseline dramatically, turning what was once a condition with a significant risk of severe vision loss into one where the majority of patients retain functional sight.