Retinal capillary hemangioma (RCH), or hemangioblastoma, is a rare vascular hamartoma, occurring either as an isolated tumor or as a component of von Hippel-Lindau (VHL) disease.1,2 The mean age of diagnosis of RCH is 25 years among patients with VHL disease and 31 years among patients in whom hemangioma occurs sporadically.3,4 These vascular hamartomas grow on the optic nerve head, within the juxtapapillary area, or on the peripheral retina, occurring most commonly at the temporal side of the optic disc.5
Ophthalmoscopically, a RCH is a round, orange-red lesion with feeder vessels. The diagnosis is based on fundus examination and is confirmed by fluorescein angiography (FA).
In this report, we describe an unusual retinal capillary hemangioma studied by multimodal imaging, including optical coherence tomography angiography (OCTA).
A 25-year-old Caucasian woman with no previous ocular history presented with metamorphopsias in the right eye 3 months ago. She underwent best-corrected visual acuity (BCVA), fundus biomicroscopy, FA with a confocal scanning laser ophthalmoscopy (Spectralis HRA+OCT; Heidelberg Engineering, Heidelberg, Germany), swept-source optical coherence tomography (OCT), and OCT angiography (OCTA) (DRI OCT Triton; Topcon, Tokyo, Japan). The study adhered to the tenets of the Declaration of Helsinki, and the participant provided written informed consent before examination.
BCVA was 20/20 both eyes. Anterior segment findings were unremarkable in both eyes. Fundus examination of both eyes revealed macula and optic nerve head within normal limits (Figure 1A).
(A) Color fundus photograph of right eye showing macula and optic nerve head within normal limits. (B) Swept-source optical coherence tomography (OCT) B-scan revealing dome-shaped elevation of neurosensory retina with disorganized architecture in correspondence of the nasal optic disc and with optical shadowing of the choroid. (C–E) OCT angiography of the retinal capillary hemangioma. En face reconstructions show a hyperintense signal localized in the nasal sector of the optic nerve head, suggestive of abnormal capillaries developed within radial peripapillary capillary plexus. (F, G) Fundus fluorescein angiography showing early hyperfluorescence and late leakage and staining of the lesion.
No feeder vessels were found, and no optic disc edema was detected. Peripheral vasculature had no obvious abnormalities.
The structural OCT of the right optic disc revealed a dome-shaped localized lesion between retinal pigment epithelium (RPE) and neurosensory retina, with a disorganized retinal architecture and optical shadowing of the choroid (Figure 1B).
OCTA of the disc presented a nasal circumscribed area at the edge of optic nerve head, characterized by abnormal capillaries detected at the level of radial peripapillary capillaries (RPC) plexus segmentation (Figures 1C–1E).
FA of the right eye showed an early filling of the vascular network overlying the optic nerve head and a late leakage, thus confirming the diagnosis of papillary RCH (Figures 1F and 1G). Indocyanine green angiography of right eye did not shown significant alterations. OCT and OCTA resulted within normal limits in the left eye.
Juxtapapillary hemangioma might be misdiagnosed as papilledema, papillitis, choroidal neovascularization, or choroiditis due to its location on or near the optic disc.6
Even if a fundus examination could be sufficient to make diagnosis of this lesion, FA can be helpful to confirm hamartomas with subtle clinical features. It reveals early hyperfluorescence of the tumor vessels and progressive leakage in the late phase of the angiogram. On the other hand, it is an invasive technique and does not define which of the retinal strata are affected.7
OCT can be advantageous in defining dimensions of the tumor, localizing the presence of retinal fluid, and monitoring the response to treatment.4 OCTA is a recent and noninvasive imaging technique that, analyzing the blood flow, visualizes the vasculature of the retina and the optic disc.8
The imaging features of OCTA in a juxtapapillary RCH have been recently reported.9
As reported by Thirumalesh MB et al.,9 the hemangioma was clearly detected by fundus examination and OCTA confirmed its vascularization. On the contrary, in our case, we reported an unusual presentation of the RCH without any apparent sign of vascularization. The small size and deep localization indeed made the hemangioma not detectable by simple fundus examination; only the use of OCT methodologies allowed the identification of this lesion. In addition, OCTA showed the vascular nature of the tumor, revealing vessels' interdigitation in a localized area between the optic nerve and the RPC. This area is the same as the one that showed dilated retinal arterioles and late leakage in FA examination.
Then, OCT and OCTA increased the sensitivity for the diagnosis of retinal vascular abnormalities, outperforming the slit-lamp-based fundus examination, thus allowing the final diagnosis.
In conclusion, a really small vascular hamartoma on optic nerve can be difficult to detect by means of fundus biomicroscopy. Even though FA has been the most useful diagnostic tool for the diagnosis of hemangioma until now, this case emphasizes the important role of OCT and OCTA for the identification and follow up of RCH. Even if RCH is classified as a benign lesion, it can be dangerous for the VA. Vision impairment can appear from exudation from the retinal vasculature or by glial proliferation, causing epiretinal membrane development or tractional retinal detachment.10
Infrequently, these vascular hamartomas regress in a spontaneous way.11
In our case, the small and asymptomatic hemangioma was localized in the nasal area of the optic disc and did not involve the macula. Consequently, no therapy was started, but the patient was assigned to a follow-up program.
- Knutsson KA, De Benedetto U, Querques G, Del Turco C, Bandello F, Lattanzio R. Primitive retinal vascular abnormalities: Tumors and telangiectasias. Ophthalmologica. 2012;228(2):67–77. doi:10.1159/000338230 [CrossRef]
- Pierro L, Marchese A, Gagliardi M, Bandello F. Optical coherence tomography angiography of retinal cavernous hemangioma. Ophthalmic Surg Lasers Imaging Retina. 2017;48(8):684–685. doi:10.3928/23258160-20170802-14 [CrossRef]
- Bowling B. Kanski's Clinical Ophthalmology. 8th ed. Philadelphia, PA: Saunders Ltd.; 2016.
- Turell ME, Singh AD. Vascular tumors of the retina and choroid: Diagnosis and treatment. Middle East Afr J Ophthalmol. 2010;17(3):191–200. doi:10.4103/0974-9233.65486 [CrossRef]
- Mitropoulos PG, Chatziralli IP, Peponis VG, Tsiotra VA, Parikakis EA. Photodynamic therapy for juxtapapillary retinal capillary hemangioma. Case Rep Ophthalmol Med. 2014;2014:756840.
- Gass D, Braunstein R. Sessile and exophytic capillary angiomas of the juxtapapillary retina and optic nerve head. Arch Ophtalmol. 1980;98 (10):1790–1797. doi:10.1001/archopht.1980.01020040642011 [CrossRef]
- McCabe CM, Flynn Jr. HW, Shields CL, et al. Juxtapapillary capillary hemangiomas. Ophthalmology. 2018;107(12):2240–2248. doi:10.1016/S0161-6420(00)00422-X [CrossRef]
- Kalevar A, Patel K, McDonald R. Optical coherence tomography angiography of retinal cavernous hemangioma. Retina. 2017;37(5):e50–e51. doi:10.1097/IAE.0000000000001482 [CrossRef]
- Thirumalesh MB, Jain A, Agrawal S, Bhujang Shetty K. In vivo microvascular pattern of solitary juxtapapillary capillary hemangioma on OCT angiography. Ophthalmic Surg Lasers Imaging Retin. 2017;48(7):592–595. doi:10.3928/23258160-20170630-12 [CrossRef]
- Papastefanou VP, Pilli S, Stinghe A, Lotery AJ, Cohen VML. Photodynamic therapy for retinal capillary hemangioma. Eye (Lond). 2013;27(3):438–442. doi:10.1038/eye.2012.259 [CrossRef]
- Saitta A, Nicolai M, Giovannini A, Mariotti C. Juxtapapillary retinal capillary hemangioma: New therapeutic strategies. Med Hypothesis. Discov Innov Ophthalmol. 2014;3(3):71–75.