Choroidal Hemangioma: Circumscribed vs. Diffuse

Choroidal hemangioma is a benign vascular tumor of the choroid.

It comes in two clinically distinct forms — circumscribed and diffuse — with different associations, presentations, and management priorities.

Both are congenital vascular hamartomas rather than true neoplasms.

However, their clinical behavior diverges enough that they are best thought of as separate entities sharing a name.

Circumscribed Choroidal Hemangioma

The circumscribed form is an isolated, sporadic lesion, most often located near the posterior pole.

It is usually discovered in adulthood when it causes visual symptoms from associated subretinal fluid.

Less often, it is found incidentally on routine fundus exam.

It is not associated with any systemic syndrome and occurs as an isolated ocular finding in an otherwise healthy patient.

On fundus exam it appears as an orange-red, dome-shaped, well-circumscribed choroidal mass.

Its color is a distinctive feature that helps separate it from other choroidal tumors that can mimic it.

One such mimic is amelanotic choroidal melanoma.

Diffuse Choroidal Hemangioma and Sturge-Weber Syndrome

The diffuse form is strongly associated with Sturge-Weber syndrome.

It accompanies the facial port-wine stain and leptomeningeal vascular malformation that define that syndrome.

Rather than a discrete mass, the diffuse form thickens the choroid over a much wider area.

This is sometimes described as a “tomato ketchup” fundus, from the diffusely thickened, deep red choroid visible through the retina.

Because the diffuse form is part of a recognized syndrome, it should prompt confirmation of the syndrome’s other features.

This warrants coordination with the broader care already in place for that diagnosis, including glaucoma surveillance.

This is because ipsilateral glaucoma is common in Sturge-Weber syndrome independent of the choroidal finding itself.

Clinical Presentation

Circumscribed lesions are frequently asymptomatic when small and peripheral.

They cause metamorphopsia, blurred vision, or a visual field defect when they involve or approach the macula.

This is largely through the exudative subretinal fluid they generate over time, rather than through the mass itself.

Diffuse lesions in Sturge-Weber syndrome often present earlier, in childhood.

They sometimes cause a shallow, chronic exudative retinal detachment, subtle and easy to miss without a careful, dilated exam.

Differential Diagnosis

• Amelanotic choroidal melanoma — the most important alternative diagnosis to exclude, given the difference in management and prognosis
• Choroidal metastasis — typically less well-circumscribed, with a relevant primary malignancy history
• Choroidal osteoma — calcified, seen on ultrasound or CT, more common in young women
• Posterior scleritis — inflammatory, painful, associated with other signs of scleral inflammation

Distinguishing circumscribed choroidal hemangioma from amelanotic melanoma is the single most consequential differential decision.

It relies heavily on multimodal imaging rather than fundus appearance alone.

Diagnostic Evaluation

B-scan ultrasonography shows a characteristically high internal reflectivity for a choroidal hemangioma.

This contrasts with the low-to-medium reflectivity typical of choroidal melanoma, making ultrasound a discriminating test.

Indocyanine green angiography classically shows early, rapid hyperfluorescence of the tumor’s intrinsic vasculature.

This is followed by a “washout” phenomenon in later phases.

This pattern, when present, is distinctive enough to strongly support the diagnosis.

OCT is used to characterize and monitor any associated subretinal fluid.

This is often the more clinically relevant driver of vision loss and treatment decisions.

It matters more than the size of the tumor itself.

Management

Asymptomatic, small circumscribed lesions without subretinal fluid can simply be observed with periodic monitoring.

Symptomatic lesions with vision-threatening subretinal fluid are treated to induce tumor regression and resolve the fluid.

Photodynamic therapy has become a preferred option for many circumscribed lesions, given its selective effect on the abnormal vasculature.

Argon laser photocoagulation, external beam, or plaque radiotherapy are used for larger or diffuse lesions less amenable to it.

Diffuse hemangiomas in Sturge-Weber syndrome are managed similarly when symptomatic.

This adds the consideration of coordinating care around the broader syndrome, including glaucoma control.

This can itself affect visual outcomes independent of the choroidal lesion.

References

• Shields CL, Honavar SG, Shields JA, et al. Circumscribed choroidal hemangioma: clinical manifestations and factors predictive of visual outcome. Ophthalmology.
• Witschel H, Font RL. Hemangioma of the choroid: a clinicopathologic study of 71 cases. Survey of Ophthalmology.
• Sullivan TJ, Clarke MP, Morin JD. The ocular manifestations of the Sturge-Weber syndrome. Journal of Pediatric Ophthalmology and Strabismus.
• American Academy of Ophthalmology. Basic and Clinical Science Course, Section 4: Ophthalmic Pathology and Intraocular Tumors.