Vascular tumors of the retina and choroid: diagnosis and treatment

Abstract

The vascular tumors of the retina and choroid comprise a diverse group of congenital and acquired lesions. The major vascular tumors of the retina include retinal capillary hemangioma, cavernous hemangioma of the retina, retinal vasoproliferative tumor, and racemose hemangiomatosis of the retina or Wyburn-Mason syndrome. Choroidal vascular tumors include circumscribed choroidal hemangioma and diffuse choroidal hemangioma. While classified as benign, visual symptoms secondary to exudative retinal detachment and a variety of other mechanisms are common and are a major source of long-term visual disability. While many therapeutic modalities exist, treatment of symptomatic cases can be challenging. Of particular importance, many of the vascular tumors of the retina and choroid have significant associations with systemic disease. As ocular symptoms are often the most common presenting disease manifestation, the ophthalmologist plays an important role in accurate and early diagnosis. The ability to initiate prompt screening and treatment in appropriate cases is critical. In the following article, the key clinical and diagnostic features of the major retinal and choroidal vascular tumors, their systemic associations, and the literature pertaining to the most currently available treatment strategies are reviewed.

Keywords: Cavernous Hemangioma; Choroidal Hemangioma; Retinal Capillary Hemangioma; Retinal Vasoproliferative Tumor; Wyburn–Mason Syndrome.

Figure 1

(A) Fundus photograph of a retinal capillary hemangioma. (B) Note prominent feeder vessels that show hyperfluorescence on fluorescein angiography. (C) B-scan ultrasonography showing a circumscribed retinal mass with a localized retinal detachment

Figure 2

(A) Fundus photograph of a peripapillary cavernous hemangioma of the retina. (B) Note the absence of retinal exudation. OCT confirms multilobulated consistency

Figure 3

(A) Fundus appearance of a vasoproliferative retinal tumor. (B) OCT reveals irregularly thickened retina on the surface of the tumor. Due to limited penetration, deeper aspects of the tumor are not visualized

Figure 4

Fundus appearance of a typical retinal arteriovenous malformation. Reproduced with permission from: Singh AD, Rundle PA, Rennie IG. Retinal vascular tumors. In: Singh AD, Damato BE, Pe'er J, Murphree AL, Perry JD, editors. Clinical Ophthalmic Oncology. Philadelphia: Saunders-Elsevier, 2007:341-7

Figure 5

(A) Fundus photograph of the right eye showing a circumscribed choroidal haemangioma. (B) On B-scan ultrasonography, there is a smooth-contoured, dome-shaped choroidal mass (C) that demonstrates high internal reflectivity on A-scan

Figure 6

(A) Fundus photograph showing diffuse choroidal thickening with shallow subretinal fluid in the right eye. (B) Note diffuse orange color of the fundus as compared to the normal left eye. (C) On B-scan ultrasonography, there is a smooth-contoured, diffuse thickening of the choroid