Comparison of optical coherence tomography angiography and fundus fluorescein angiography features of retinal capillary hemangioblastoma

Abstract

The aim of this study is to compare the optical coherence tomography angiography (OCTA) and fundus fluorescein angiography (FFA) features of retinal capillary hemangioblastoma (RCH). This is an observational case series of three patients with von Hippel-Lindau (VHL) disease and one patient with juxtapapillary RCH. All patients underwent FFA with a mydriatic fundus camera and OCTA with swept-source angio OCT. The FFA and OCTA characteristics of tumors were compared. In our series, FFA could identify tumors as small as the width of a third-order retinal artery, which was missed on clinical examination. OCTA identified these tiny tumors, but only those closer to the posterior pole. Both FFA and OCTA could identify the intrinsic vasculature and feeder vessel in juxtapapillary RCH. On OCTA, the tumors were better defined than in FFA. The depth of the lesion can be identified on OCTA. Feeder and the draining vessels could be identified precisely in OCTA than FFA, particularly in small tumors. OCTA can identify tumors in VHL missed on clinical examination. It can identify the feeder vessel and intrinsic vasculature of sessile juxtapapillary RCH and aids in its diagnosis. Tumors are better defined in OCTA than FFA due to the absence of leakage. However, FFA can identify nearly all the early tumors, but OCTA fails to image the peripheral tumors due to its smaller field and prolonged acquisition time. Technological advances and the development of wide-field OCTA in the future can be helpful in identifying all the unsuspected tumors in VHL disease.

Keywords: Fundus fluorescein angiography; optical coherence tomography angiography; retinal capillary hemangioblastoma; von Hippel–Lindau disease.

Figure 1

Case 1 – (a) Color photograph showing a retinal capillary hemangioblastoma located at disc, (b) fundus fluorescein angiography of disc retinal capillary hemangioblastoma showing its communication with inferotemporal major vessels and absence of leakage, (c) tiny retinal capillary hemangioblastoma identified on fundus fluorescein angiography, (d) optical coherence tomography angiography of disc retinal capillary hemangioblastoma showing communication with inferotemporal major vessels

Figure 2

Case 2 – (a) Fundus fluorescein angiography of a large retinal capillary hemangioblastoma and small retinal capillary hemangioblastoma. The large tumor is ill defined due to leakage. Multiple hyperfluorescent satellite lesions around the large retinal capillary hemangioblastoma can be seen which would give a false impression of multiple tiny tumors, (b) optical coherence tomography angiography of the lesion in Figure 2a. Margins of the tumor are well defined compared to fundus fluorescein angiography. The small retinal capillary hemangioblastoma is identifiable even in optical coherence tomography angiography. Due to motion artifact duplication of tumor is seen, (c) fundus fluorescein angiography of a paired retinal capillary hemangioblastoma. The feeder and the draining vessels and communication between the tumors are seen, (d) optical coherence tomography angiography of the lesion in Figure 2c. The feeder and the draining vessels and the communication are more distinct than fundus fluorescein angiography

Figure 3

Case 2 – (a and c) Fundus fluorescein angiography of cluster of tumors, (b and d) optical coherence tomography angiography of the lesions in Figure 3a and c. Individual tumor can be identified distinctly in optical coherence tomography angiography than fundus fluorescein angiography

Figure 4

Case 3 – (a) Color photograph of large retinal capillary hemangioblastoma with precisely identifiable feeder and draining vessel, (b) optical coherence tomography through the large tumor showing intraretinal cyst, (c) early phase fundus fluorescein angiography of large tumor with well-defined margins and feeder and draining vessel, (d) late phase fundus fluorescein angiography of tumor with blurring of margin due to leakage, (e) optical coherence tomography angiography of large tumor with well-defined margins and feeder and draining vessel, (f) Deeper retinal sections of optical coherence tomography angiography showing signal void areas in the deep capillary plexus around the tumor

Figure 5

Case 3 – (a) Fundus fluorescein angiography of a small retinal capillary hemangioblastoma with blurring of margin due to leakage. Feeder and draining vessels are not seen, (b) optical coherence tomography angiography of lesion on Figure 5a with well defined margins. Feeder and draining vessel are precisely identifiable

Figure 6

Case 4 – Colour photograph showing greyish, horizontally oval lesion obscuring the inferior one third of optic disc

Figure 7

Case 4 – (a) Early phase fundus fluorescein angiography showing retinal feeder vessel (arrowhead) and stippled hyper fluorescence within the lesion, (b) late phase fundus fluorescein angiography showing intense staining of lesion, (c) superficial slabs of optical coherence tomography angiography showing feeder vessel (arrow) with its branches. The branches are not evident in fundus fluorescein angiography, (d) deeper slabs of optical coherence tomography angiography showing bright spots (dashed arrow) within the lesion suggestive of intrinsic vascularity (e and f) Corresponding b scans of c and d