A classical case of cavernous hemangioma of the retina

Case Report

A 12-year-old female presented with gradually progressive, painless diminution of vision in her left eye for 1 year. On examination best corrected visual acuity in her right eye was 6/6 and left eye was 6/36. Her anterior segment examination was unremarkable. Left eye fundus examination - normal disc and vessels. At 3 DD temporal to disc, saccular grape like clusters of thin-walled angiomatous lesions was seen in the parafoveal region extending upto the ora serrata. (Figure 1) Periphery of the lesion showed chorioretinal atrophy that spilled on to the macula. (Figure 2). There was fibrous tissue at the periphery of the lesion near ora serrata (Figure 3). A clinical diagnosis of left eye cavernous hemangioma was made. A-Scan showed a high initial spike with high internal reflectivity.

B-Scan showed an irregular elevated hyperechoic lesion with no choroidal excavation. OCT Macula revealed thickening, multiple saccules with bridges in between them (Figure 4). Systemic examination by a pediatrician was normal and MRA Brain was normal study.

Figure 1

Fundus image of left eye showingsaccular grape like clusters of thin-walled angiomatous lesions was seen in the parafoveal region exrending upto the ora serrate

Figure 2

Bunch of grape appearance with numerous dilated vessel and dot shaped intra-retinal hemorrhages

Figure 3

Fibrotic tissue near the macula

Figure 4

OCT Macula showingsaccules with bridges between them

Discussion

A hamartoma is a benign lesion consisting of normal tissues in an abnormal configuration. Cavernous hemangioma of the retina is a vascular hamartoma. It usually arises in the second decade of life and is more common in females.1 Most cases of cavernous hemangiomas are sporadic, but they have been classified as part of Phakomatosis, which are multisystem disorders characterized by development of hamartomatous lesions involving CNS, skin and visceral organs in the body.

Cavernous hemangiomas are saccular aneurysms which are filled with venous blood. They mainly present between the 3 to 5^th decade of life2 (2) and account for 5 to 13% of vascular malformations.3 Typically the tumour is around 1-2 disc diameters in size and resemble “a bunch of grapes”. It may be an isolated lesion or it can fill the entire fundus. It may even follow the course of a major blood vessel. The lesion becomes symptomatic when it causes vitreous hemorrhage or when it is located on or next to the macula which has a reported incidence of 10% in literature.2, 4 Similarly, lesions have been noted to occur on the optic nerve head. These patients had normal visual acuity with enlargement of the blind spot.5

Investigations like fluorescein angiography typically show slow filling of the aneurysm and plasma-erythrocyte separation which can be seen as pooling of the dye in the superior portion of the aneurysm. OCT usually demonstrates additional features like epiretinal membrane which can be present in the lesion. Other investigations like A-Scan shows high initial spike with high internal reflectivity and B-Scan demonstrates an irregular surface, large acoustic internal reflectivity and the absence of choroidal excavation.6

Periodic observation is advised in most cases as cavernous hemangiomas rarely increase in size or cause vitreous hemorrhage. Photocoagulation or cryotherapy is advised when vitreous hemorrhage occurs. Klein et al reported a case where the hemangioma increased in size following photocoagulation.7 In cases of non-clearing vitreous hemorrhage vitrectomy has proven to be beneficial.

Source of funding

None.

Conflict of interest

None.