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Patient referred for evaluation of retinal elevation

An oval-shaped reddish choroidal lesion was observed on examination.

Issue: July 10, 2012

ByBryan K. Monson, MD

ByJay S. Duker, MD

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A 60-year-old man was referred to the retina service for evaluation of retinal elevation noted on routine dilated fundus examination. The patient reported mild decreased vision in the right eye but denied any new distortion, ocular pain, flashes or floaters. Medical history was unremarkable, and review of systems was negative. Outpatient medications included daily aspirin.

Examination

On examination, the patient’s best corrected visual acuity was 20/40 in the right eye and 20/25 in the left eye without improvement on pinhole. IOP was 14 mm Hg in the right eye and 15 mm Hg in the left eye. Pupillary exam was normal with no afferent pupillary defect. Slit lamp biomicroscopy demonstrated mild bilateral, symmetric nuclear sclerosis. Dilated funduscopic examination of the right eye demonstrated an oval-shaped reddish elevation inferotemporal to the fovea that measured approximately 8 mm 3 4 mm with 2 mm of elevation. There was no subretinal fluid or retinal pigment hyperplasia associated with the lesion. The optic nerve and retinal vasculature appeared normal (Figure 1). Dilated funduscopic examination of the left eye was unremarkable.

Intravenous fluorescein angiography (IVFA) of the retinal lesion showed early faint, mottled hyperfluorescence (Figure 2) without late leakage. Optical coherence tomography demonstrated subfoveal choroid elevation without subretinal or intraretinal fluid (Figure 3). Indocyanine green (ICG) angiography showed early hyperfluorescence of the lesion with washout of dye in the late frames, late hyperfluorescence of the rim surrounding the lesion, and late focal dye accumulation without leakage on the lesion surface (Figure 4).

 

Figure 1. Fundus photograph of the right eye demonstrated an oval-shaped orange-red lesion inferotemporal to the fovea.

 

Figure 2. IVFA (22.2 sec) demonstrated early faint, mottled hyperfluorescence.

 

Figure 3. OCT demonstrated elevation of the subfoveal choroid. Subretinal fluid was absent.

 

Figure 4. ICG angiography (48.9 sec) demonstrated a well-defined area of early intense, uniform hyperfluorescence.

Images: Monson BK, Duker JS

What is your diagnosis?

Elevated choroidal lesion

The differential diagnosis of a patient with an elevated choroidal lesion includes choroidal hemangioma, choroidal metastasis, amelanotic choroidal melanoma and choroidal osteoma.

Circumscribed choroidal hemangioma can closely resemble other benign and malignant conditions of the choroid. Typically, choroidal hemangiomas have a distinctive orange-red color similar to the surrounding choroid, are located in the posterior pole, and are almost always solitary and unilateral, as in our patient. By contrast, amelanotic melanomas are typically yellow-tan in color with overlying drusen. Choroidal metastases generally appear as a creamy yellow mass and are frequently bilateral or multifocal. Renal cell carcinoma, thyroid carcinoma and carcinoid tumor may metastasize to the choroid and can appear as an orange lesion, but typically there is a previous history of neoplasm. Choroidal osteoma may have a similar color to choroidal hemangioma, but the lesion is rarely elevated. Given the clinical appearance and associated ancillary testing, this lesion was felt to be most consistent with a choroidal hemangioma.

Discussion

Choroidal hemangioma is the most common vascular tumor of the choroid, and the circumscribed type must be differentiated from other types of amelanotic choroidal tumors. Most often, this differentiation can be made clinically with indirect ophthalmoscopy by experienced observers. In difficult or subtle cases, ancillary studies can help confirm the diagnosis. IVFA, ICG, ocular ultrasonography, contrast-enhanced MRI and radioactive phosphorus uptake testing may be helpful adjunctive measures in determining the diagnosis.

IVFA features of choroidal hemangioma characteristically demonstrate irregular linear hyperfluorescence of large choroidal vessels within the lesion during the choroidal phase, followed by progressive staining of the extravascular tissue within the tumor and pinpoint foci of hyperfluorescence over the tumor in the arterial or venous phases. Progressive intraretinal hyperfluorescence secondary to leakage of fluorescein into the cystoid spaces within the retina follows. IVFA patterns of choroidal tumors may be incomplete in smaller lesions or appear similar to other amelanotic choroidal tumors, which may limit the diagnostic usefulness of IVFA.

ICG angiography, with well-demarcated, early, uniform hyperfluorescence that transitions to an ill-defined, late hypofluorescence, or “dye washout” of the tumor, is considered pathognomonic for choroidal hemangioma. Filling on ICG is typically slower and less intense for choroidal melanoma or metastasis. Additionally, there may be late hyperfluorescence of the rim surrounding the tumor and hot spots on the surface of the tumor.

Because choroidal hemangiomas are benign, treatment is reserved for vision loss secondary to subretinal fluid or cystoid macular edema. Photodynamic therapy with Visudyne (verteporfin, QLT) is the treatment of choice for symptomatic hemangioma, and it is effective and safe in resolving exudative subretinal fluid and cystoid macular edema, therefore improving visual outcome. Argon laser photocoagulation, transpupillary thermotherapy and xenon arc have all been utilized as treatment as well, but recurrences can occur, and these treatments may induce scarring of the fovea in central tumors. For lesions resistant to laser or PDT, low-dose external beam radiation may be helpful. Subfoveal lesions typically result in poor visual acuity in the long term even in the absence of subretinal fluid, due to cystic degeneration of the overlying retina.

Follow-up

The decreased vision in this patient may be due to the Stiles-Crawford effect, which is caused when the choroidal lesion results in tilting of the overlying retina, requiring hyperopic over-refraction to improve visual acuity. Unfortunately, refraction was not performed at the initial visit to prove this theory. Given the absence of significant subretinal or intraretinal fluid and lack of impending threat to vision, the patient was observed at 3-month intervals and continues to be followed up.