Case of the Month

Edited by Robert N. Johnson, MD

Presented by Ananda Kalevar, MD

April, 2017

A 61-year-old man presents with blurred vision in his right eye.

Fig 1: Wide-field photograph of the right eye. A mass (amelanotic on ophthalmoscopic evaluation) is present superiorly to the macula. Fluid leakage  extends inferiorly from the mass on the temporal side of the macula to the inferior periphery.

Case History

A 61-year-old man presents with a blurred central vision in his right eye for several weeks.  His past medical history was relevant for hypertension.  His past ocular history, family history, social history and medications were non-contributory.

On examination, best-corrected visual acuity was 20/32 and 20/25 in his right and left eye, respectively.  Intraocular pressure was normal in both eyes.  The anterior segment examination was unremarkable.  The posterior segment exam of the right eye was remarkable for an elevated amelanotic choroidal mass superior to the superotemporal arcade with significant subretinal fluid tracking inferiorly through the macula (Figure 1).  The posterior segment examination of the left eye was unremarkable.  Wide-field fluorescein angiography of the right eye in the early phase reveals hyperfluorescence over the choroidal mass (Figure 2A) and in the late phase reveals hyperfluorescence consistent with staining of the mass along with stippled hyperfluorescence tracking inferiorly into the macula (Figure 2B).  Widefield fundus autofluorescence of the right eye reveals hypoautofluorescence in the area of the choroidal mass with hyperautoflourescence surrounding the lesion and tracking down the retina consistent with a “guttering” configuration (Figure 3).

Enhanced depth spectral domain OCT (EDI SD-OCT) of the right eye vertically through the mass reveals elevation of the retina/choroidal complex with a large choroidal mass with cystic thickening of the overlying retina and subretinal fluid inferior to the mass (Figure 4).  SD-OCT of the right macula vertically reveals elevation of the retina/choroidal complex with some cystoid thickening superiorly and subretinal fluid tracking under the fovea (Figure 5).  B-scan ultrasonography of the right eye superotemporally reveals a dome-shaped solid lesion measuring 3.2mm in thickness with high and homogeneous reflective signal (Figure 6).

Fig 2A: Early phase wide-field fluorescein angiogram of the right eye. Note the area of early hyperfluorescence superior to the macula. Mottled fluorescence extends inferiorly.

Fig 2B: Late phase wide-field fluorescein angiogram of the right eye. Leakage is present over the surface of the lesion superior to the macula.

Fig 3: Wide-field fundus autofluorescence of the right eye. Hypoautofluorescence over the surface of the mass is noted, with significant hyperautofluorescence surrounding the mass and trailing inferiorly.

Fig 4: Enhanced depth imaging SD-OCT of the mass shows a smooth contour, with hyperreflective pillars within the lesion, cystic edema of the retina and a surrounding serous retinal detachment

Fig 5: SD-OCT, vertical scan of the right macula shows a serous detachment extending into the macula from lesion superiorly.

Fig 6: B-scan ultrasound of the mass shows a solid mass measuring 3.2mm in thickness. A-scan (not shown) demonstrated homogeneous hyperreflectivity within the lesion.

What is your Diagnosis?

Differential diagnosis

Choroidal melanoma, choroidal metastasis, posterior nodular scleritis, central serous chorioretinopathy, choroidal osteoma, choroidal granuloma, choroidal detachment, extramacular choroidal neovascularization

 

Additional case history

As the patient was symptomatic from the subretinal fluid tracking from the choroidal hemangioma into the fovea, treatment options were discussed.  Due to the location of the tumor, laser photocoagulation was performed to the mass.  Two months after laser photocoagulation, best-corrected visual acuity was 20/25 in the right eye.  EDI SD-OCT of the right eye through the mass demonstrates resolved subretinal fluid and reduced cystoid retinal edema (figure 7).  In addition, SD-OCT of the right macula reveals an attached fovea with some outer retinal and pigment changes seen temporally (figure 8).

Fig 8: SD-OCT of the right macula shows resolution of the serous detachment, but outer retinal atrophy and EZ disruption temporally.

Fig 7: Enhanced depth imaging SD-OCT of the mass shows resolved serous fluid, and substantially reduced cystoid retinal edema over the surface of the lesion.

Discussion

Choroidal hemangioma is typically a benign vascular hamartoma classified into two subtypes, either circumscribed or diffuse.  Generally, it is considered a congenital condition.  Diffuse choroidal hemangiomas are associated with systemic conditions such as Sturge-Weber syndrome whereas circumscribed hemangiomas are typically not.1,2  Diffuse choroidal hemangiomas occupy a significant part of the choroid and circumscribed hemangiomas present as a unilateral solitary orange-red mass in the choroid.  Circumscribed hemangiomas are predominantly found in the posterior pole.  In the majority of cases, subretinal fluid can develop and produce symptoms.  Occasionally, macular edema can develop overlying the mass.1,2

Choroidal hemangiomas are often mistaken and masquerade as malignancy such as choroidal melanoma or metastasis which possess significantly more morbidity and mortality.1,2  Multimodal imaging can be helpful to accurately diagnose these masses, reassure patients and properly treat them.  Fluorescein angiography reveals early hyperfluorescence with increasing late hyperfluorescence.  Indocyanine green angiography reveals early hyperfluorescence and late hypofluorescence with a hyperfluorescent rim around the tumor.  Ultrasonography typically depicts a dome-shaped lesion that is acoustically solid, identical in character to the surrounding choroid.  On A-scan, there is high internal reflectivity present which is useful to distinguish it from choroidal melanomas which are acoustically hollow with low to medium internal reflectivity.1,2  EDI SD-OCT can be helpful to determine if there is any subretinal fluid, macular edema and assess the retina/choroid complex.  Widefield fundus autofluorescence is particularly helpful to determine the extent of subretinal fluid tracking in the retina.3

For symptomatic circumscribed hemangioma patients, treatment options that exist are laser photocoagulation, photodynamic therapy (PDT), transpupillary thermotherapy, radiation or anti-VEGF agents.4  PDT is frequently the procedure of choice, and in many cases, location of the tumor either in the macula or close to the fovea require consideration of PDT. However, thermal laser photocoagulation is a cost effective option in some cases as demonstrated in our patient.

Take Home Points

  • Choroidal hemangioma is typically a benign vascular hamartoma, classified as either diffuse or circumscribed.
  • Circumscribed choroidal hemangioma can be mistaken as a malignancy.
  • Symptomatic patients should undergo one of various treatment options that exist.

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References

1. Ryan S.  Retina, 5th ed.  2013:  2340-2350.

2. Shields CL, Honavar SG, Shields JA, Cater J, Demirci H. Circumscribed choroidal hemangioma: clinical manifestations and factors predictive of visual outcome in 200 consecutive cases. Ophthalmology. 2001 Dec 31;108(12):2237-48.

3. Ramasubramanian A, Shields CL, Harmon SA, Shields JA. Autofluorescence of choroidal hemangioma in 34 consecutive eyes. Retina. 2010 Jan 1;30(1):16-22.

4. Tsipursky MS, Golchet PR, Jampol LM. Photodynamic therapy of choroidal hemangioma in Sturge-Weber syndrome, with a review of treatments for diffuse and circumscribed choroidal hemangiomas. Survey of ophthalmology. 2011 Feb 28;56(1):68-85.

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