Optical coherence tomography angiography features of choroidal hemangioma.

This is an observational case series of four cases of choroidal hemangioma. All cases underwent OCT angiography with swept source OCTA (Topcon DRI OCT Triton plus) 6 mm * 6 mm protocol. OCTA at the level of large choroidal vessels demonstrated peculiar vascular pattern in all four eyes. Patterns observed were bag of worms, spaghetti like and vessels with terminal bulbs. In eye with diffuse choroidal hemangioma comparison of choroidal vascular pattern with the contralateral eyes showed significant difference in the vascular architecture. OCTA is an excellent non invasive tool in assessing the choroidal vascular pattern in eyes with choroidal hemangioma.

Choroidal hemangioma (CH) is a benign vascular tumor of the choroid.[1] It has been classified as circumscribed CH and diffuse CH (DCH) based on the extent of involvement.[2] The diagnosis of CH is usually clinical but ancillary tests such as ocular ultrasonography, fluorescein angiography (FA), indocyanine green angiography (ICGA), enhanced depth imaging optical coherence tomography (EDI-OCT), and magnetic resonance imaging aid in confirming the diagnosis and ascertaining the extent of the lesion.[3456] We describe various features of four cases of CH using swept source OCT angiography (OCTA) (Topcon DRI OCT Triton plus) 6 mm × 6 mm protocol.

Case Reports

Case 1

A 52-year-old female presented with blurred vision in the left eye for the past 1 month. On fundus examination, an elevated orange-colored mass lesion was noted superior to the disc [Fig. 1a], with shallow subretinal fluid (SRF) at macula [Fig. 1b]. FA showed a well-demarcated area of mottled hyperfluorescence in early phase with minimal staining in late phase [Fig. 1c]. OCTA at the level of superficial choriocapillary layer showed multiple whitish irregular vessels resembling bag of worms, and deeper choroidal vessels demonstrated club-like appearance. At the margin of the tumor, there was a sudden transition from irregularly arranged choroidal vessels to regularly arranged normal vessels [Fig. 1d and e].

Figure 1

(a) Color photograph of the left eye showing circumscribed choroidal hemangioma superior to the disc with subretinal fluid at macula (b) optical coherence tomography showing subretinal fluid at the fovea (c) fluorescein angiography showing mottled hyperfluorescence in early phase. (d) Optical coherence tomography angiography at the level of superficial choriocapillary layer showed multiple whitish irregular vessels resembling bag of worms. Yellow dotted line indicates the line of transition from irregularly arranged tumor vessels to regularly arranged normal vessels. (e) Optical coherence tomography angiography at deeper choroidal vessels demonstrated club-like appearance (yellow arrows)

Case 2

A 40-year-old male presented to us with complaints of distorted images in the right eye. Fundus examination in the right eye showed orange elevated lesion superior to disc [Fig. 2a] with shallow SRF at the fovea. OCTA at the level of superficial choriocapillary layer showed irregularly arranged tumor vessels, and deeper choroidal slabs showed irregular choroidal vessels with “club-like” appearance [Fig. 2b and c].

Figure 2

(a) color photograph of the right eye showing an orange lesion superior to disc with shallow subretinal fluid at the fovea. (b) Optical coherence tomography angiography at the level of superficial choriocapillary layer showed irregularly arranged vessels with corresponding level of segmentation. (c) Optical coherence tomography angiography at deeper choroidal slabs showed irregular choroidal vessels with “club-like” appearance (yellow arrows)

Case 3

A 46-year-old male presented with complaints of metamorphopsia in the left eye. On examination, an orange elevated lesion was noted temporal to the fovea [Fig. 3a]. On FA mottled hyper fluorescent areas were noted in early phase which showed minimal increase in intensity in late phase [Fig. 3b]. OCTA at the level of superficial choriocapillary layer showed irregularly arranged vessels, and deeper choroidal slabs showed irregular choroidal vessels with few “club-like” appearance. Signal void areas could be noted in the deeper choroidal slabs [Fig. 3c and d].

Figure 3

(a) Color photograph showing reddish orange elevated choroidal mass temporal part of the fovea. (b) Early phase of fluorescein angiography of the left eye showing mottled hyperfluorescence. (c) Optical coherence tomography angiography at the level of superficial choriocapillary layer showed irregularly arranged vessels. (d) Optical coherence tomography angiography at the level of deeper choroidal slabs showed irregular choroidal vessels with few “club-like” appearance. Yellow dotted line indicates the signal void areas

Case 4

A 24-year-old male diagnosed with uniocular glaucoma was referred to our outpatient department. The right side of his face had port wine stain. On right eye fundus examination, significant cupping was noted with pigment clumps and retinal pigment epithelium atrophy inferonasal to the disc. A diffuse ill-defined circumferential reddish lesion was noted below the fovea [Fig. 4a and b]. OCT showed abrupt increase in choroidal thickness below the fovea suggestive of DCH [Fig. 4c]. OCTA at the level of choriocapillary and deeper choroidal vessels over the DCH showed choroidal vessels arranged like fine threads entangled with each other resembling spaghetti-like appearance with intervening signal void areas.[Fig. 4d]. The OCTA of the left eye showed normal choroidal vasculature [Fig. 4e].

Figure 4

(a) Right eye photo showing significant cupping, reddish flat lesion in inferior half of fundus below the fovea. (b) color photograph of the right eye showing inferonasal margin of diffuse choroidal hemangioma (red arrows). (c) Optical coherence tomography of the right eye shows that the choroidal thickness abruptly increased inferior to fovea (white arrow). White dotted lines indicate the choroid sclera interface. (d) Optical coherence tomography angiography in the right eye showed choroidal vessels arranged like fine threads. Red arrows indicate the margin of tumor. (e) Optical coherence tomography angiography of the left eye showing normal choroidal vasculature

Discussion

Histopathologically, based on the type of vasculature, CH has been classified into cavernous type, capillary, and mixed type.[2] In vivo choroidal vascular pattern of CH has been studied using FA and ICGA.[34] On FA, CH in choroidal phase shows hyperfluorescence with a fine, lacy network of vessels, and the late phase shows variable increase in hyperfluorescence with leakage.[3] Early washout of the indocyanine dye from the choroidal vessels is a characteristic feature of CH on ICGA.[4] The limitation of FA and ICGA is that the procedure is invasive and repeating test more often is not feasible.

Clinical diagnosis of DCH is challenging because of pigmentation of the fundus in the Asian population, making it difficult to identify the lesion. In such scenarios, we propose comparing the choroidal vascular pattern of the two eyes using OCTA, which aids in the identification of abnormal choroidal vasculature.

In our case series, we found that OCTA of all lesions showed whitish choroidal vessels with few intervening signal void areas. Dark signals in OCTA can be either due to masking by retinal pigment epithelium or limited laser light penetration.[7] We hypothesize that this change in the appearance of choroidal vessels could be due to change in dynamics of blood flow in the tumor choroidal vessels. Dark signals in CH on OCTA were reported by Takkar et al.[8]

Characteristic OCTA features of CH which we observed were the presence of irregularly arranged choroidal vessels in both superficial and deeper choroidal slabs, the presence of club-like appearance in deeper choroidal slab which was more prominently seen in active tumors, and signal void areas in deeper choroidal slabs which was more prominent in dormant tumors.

Advantages of using OCTA in CH are that the procedure is noninvasive. OCTA can be used as a screening tool in patients with port wine stain to identify abnormal choroidal vessels of CH.

Limitation of this study is that it is an observational cross-sectional study and only four eyes were studied. With our present knowledge on OCTA in CH, it can be just an adjunctive investigation to FA, ICGA, and not a replacement.

Conclusion

OCTA is an excellent noninvasive tool in assessing the choroidal vascular pattern in eyes with CH. Further studies with larger sample size are required to study the vascular pattern in CH and to formulate an OCTA-based diagnostic criteria for diagnosing CH.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.