This article is more than 5 years old. Information may no longer be current.
Woman presents with unilateral central vision loss
An elevated reddish-orange mass was found in the patient’s left eye.
Click Here to Manage Email Alerts
 Isabel M. Balderas |  Tom Hsu | ||
A 52-year-old woman was referred to the retina service at the New England Eye Center for evaluation and management of a 2-week history of decreased vision in her left eye. She described a cloudiness within her central vision with a generalized blur in the left eye, first noticed while reading on an airplane flight. She denied any vision complaints in her right eye. Her medical history was unremarkable, and ocular history was negative.
Examination
On examination, distance visual acuity without correction was 20/20 in the right eye and 20/60-2 in the left eye with no pinhole improvement. Near visual acuity was 20/25 in the right eye and 20/30 in the left eye. IOP by applanation was 12 mm Hg in the right eye and 10 mm Hg in the left eye. Pupils were equally reactive bilaterally without an afferent pupillary defect. Slit lamp examination of the anterior segment was unremarkable with no anterior chamber cell.
Dilated fundus examination of the left eye revealed an elevated reddish-orange mass nasal to the optic disc with retinal pigment epithelium hyperplasia and fine retinal telangiectasias on the surface (Figure 1). Examination of the vitreous showed one-half to 1+ cells. Mild cystoid macular edema was present. The right fundus had two flat choroidal nevi within the macula but was otherwise normal.
Fluorescein angiography in the left eye showed early filling of the mass during the choroidal phase. The fluorescence appeared mottled initially (Figure 2a) and later became more confluent (Figure 2b). Hyperfluorescent retinal telangiectasias were visible centrally overlying the lesion beginning during the arterial phase. Mild late leakage from the mass was present. Within the macula, petaloid hyperfluorescence was observed by fluorescein angiography, and cystoid macular edema was confirmed by optical coherence tomography. Indocyanine green angiography revealed early diffuse hyperfluorescence of the lesion (Figure 3). B-scan ultrasonography demonstrated high internal reflectivity.
 Elevated reddish-orange mass nasal to optic disc in the left eye with retinal pigment epithelium hyperplasia and fine retinal telangiectasias on the surface. Images: Patron ME, Duker JS |  Fluorescein angiography showing early filling of mass during choroidal phase with mottled hyperfluorescence. |
 Later frames with more confluent hyperfluorescence and late leakage. |  Early, diffuse hyperfluorescence of lesion by indocyanine green angiography. |
What is your diagnosis?
Reddish-orange mass
The clinical appearance of a reddish-orange mass near the optic disc with characteristic fluorescein angiography, indocyanine green angiography and B-scan findings led to a diagnosis of circumscribed choroidal hemangioma. Associated cystoid macular edema accounted for the patient’s presentation with decreased vision in the involved eye.
Differential diagnosis
The differential diagnosis for this reddish-orange subretinal mass includes choroidal tumors such as choroidal hemangioma, amelanotic choroidal melanoma, choroidal osteoma and metastases to the choroid, specifically of thyroid and carcinoid origin. An amelanotic choroidal melanoma is less likely to be reddish in color and has low internal reflectivity on A-scan ultrasound, whereas hemangiomas have high internal reflectivity.
A choroidal osteoma has a similar color to a hemangioma, but it is typically flat and has a characteristic ultrasound pattern of a high initial spike that persists when the gain is at lowered sensitivity. Metastases are more often multiple and bilateral. Inflammatory processes such as posterior scleritis can also have a similar clinical appearance but do not show choroidal hyperfluorescence on fluorescein angiography.
Discussion
Choroidal hemangioma is a benign vascular tumor of congenital origin. These lesions are classified into two forms: the circumscribed type that typically has no systemic associations and the diffuse form associated with Sturge-Weber syndrome, although circumscribed tumors have been reported in Sturge-Weber patients. Choroidal hemangioma is a rare tumor, and its exact incidence is unknown. Almost all cases have been reported in Caucasians with no sex predilection. The age of diagnosis is usually in the late 20s to 50s.
On ophthalmoscopy, circumscribed choroidal hemangiomas are elevated round or oval masses that are nonpigmented and characteristically orange-red in color. The tumors have rather indistinct margins and may have flecks of pigment, splotchy yellow material or orange pigment on the surface. Overlying drusen are rare. Circumscribed choroidal hemangiomas are typically located posterior to the equator, near and usually temporal to the optic disc. Almost all are solitary, with multiple tumors being extremely rare.
From a retrospective review of 200 consecutive cases of choroidal hemangioma at the Wills Eye Hospital ocular oncology service, symptoms at presentation include vision loss, visual field defects, metamorphopsia, floaters, progressive hyperopia, photopsias and pain. The most common chief complaint is decreased visual acuity in 81% of patients, with visual acuity 20/200 or worse in 53% of patients. Six percent of patients are asymptomatic at presentation. Associated clinical findings include subretinal fluid within the fovea in 81% of patients and macular edema in 17% of patients.
Treatment
The goal of treatment is preservation or improvement of visual acuity through resolution of subretinal fluid or macular edema. Asymptomatic tumors can be observed. Treatment options include laser photocoagulation; transpupillary thermotherapy; plaque, external beam and proton beam radiotherapy; and photo dynamic therapy. PDT has become the most widely used method to treat choroidal hemangiomas.
The patient underwent two rounds of PDT and one round of laser photocoagulation to the surface of the tumor. Her vision improved to 20/50+2 with pinhole improvement to 20/30-2 in the left eye. However, persistent CME remains with mild improvement from initial presentation.
For more information:
- Mark E. Patron, MD, and Jay S. Duker, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.
- Edited by Isabel M. Balderas, MD, and Tom Hsu, MD. Drs. Balderas and Hsu can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com. Drs. Balderas and Hsu have no direct financial interest in the products mentioned in this article, nor are they paid consultants for any companies mentioned.
References:
- Barbazetto I, Schmidt-Erfurth U. Photodynamic therapy of choroidal hemangioma: Two case reports. Graefes Arch Clin Exp Ophthalmol. 2000;238(3):214-221.
- Fuchs AV, Mueller AJ, Grueterich M, Ulbig MW. Transpupillary thermotherapy (TTT) in circumscribed choroidal hemangioma. Graefes Arch Clin Exp Ophthalmol. 2002;240(1):7-11.
- Gündüz K. Transpupillary thermotherapy in the management of circumscribed choroidal hemangioma. Surv Ophthalmol. 2004;49(3):316-327.
- Kivelä T, Tenhunen M, et al. Stereotactic radiotherapy of symptomatic circumscribed choroidal hemangiomas. Ophthalmology. 2003;110(10):1977-1982.
- Madreperla SA, Hungerford JL, et al. Choroidal hemangiomas: visual and anatomic results of treatment by photocoagulation or radiation therapy. Ophthalmology. 1997;104(11):1773-1778; discussion 1779.
- Madreperla SA. Choroidal hemangioma treated with photodynamic therapy using verteporfin. Arch Ophthalmol. 2001;119(11):1606-1610.
- Mashayekhi A, Shields CL. Circumscribed choroidal hemangioma. Curr Opin Ophthalmol. 2003;14(3):142-149.
- Porrini G, Giovannini A, et al. Photodynamic therapy of circumscribed choroidal hemangioma. Ophthalmology. 2003;110(4):674-680.
- Sanborn, GE. Circumscribed choroidal hemangioma. In: Ryan SJ, ed. Retina. Vol. 1. 4th ed. St. Louis: Mosby; 2005:829-842.
- Schilling H, Sauerwein W, et al. Long-term results after low-dose ocular irradiation for choroidal haemangiomas. Br J Ophthalmol. 1997;81(4):267-273.
- Shields CL, Honavar SG, et al. Circumscribed choroidal hemangioma: Clinical manifestations and factors predictive of visual outcome in 200 consecutive cases. Ophthalmology. 2001;108(12):2237-2248.
- Shields CL, Materin MA, et al. Resolution of advanced cystoid macular edema following photodynamic therapy for choroidal hemangioma. Ophthalmic Surg Lasers Imaging. 2005;36(3):237-239.
- Shields JA. Photodynamic therapy for choroidal hemangioma. Graefes Arch Clin Exp Ophthalmol. 2006;244(9):1071-1072.
- Singh AD, Kaiser PK, Sears JE. Choroidal hemangioma. Ophthalmol Clin North Am. 2005;18(1):151-161, ix.
- Zografos L, Egger E, et al. Proton beam irradiation of choroidal hemangiomas. Am J Ophthalmol. 1998;126(2):261-268.