Sir,

Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) is a rare retinal condition occurring more commonly in females, typically in the third decade, with no associated systemic conditions. Capillary nonperfusion is a prominent feature.

We present a young girl with characteristic features of IRVAN without capillary nonperfusion and present our findings of a literature review of cases of IRVAN, which suggests preserved capillary perfusion may be more commonly seen in paediatric cases.

Case report

A 15-year-old girl, of Indian origin, presented with discomfort in both eyes and previous transient difficulty with reading. Visual acuity was 6/4 bilaterally. She had a mild anterior uveitis. Fundus examination showed bilateral peripheral vascular sheathing suggestive of previous retinal vasculitis. There were retinal macroaneurysms at both discs and peripapillary retinal exudates (Figure 1). OCT scanning did not reveal any macular oedema (Figure 2).

Figure 1
figure 1

Colour fundus photographs of both eyes showing peripapillary retinal exudates.

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Figure 2
figure 2

Ocular coherence tomography scans through the fovea showing the absence of macular oedema in both eyes.

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Fluorescein angiography showed bilateral aneurysmal changes clustered over the optic discs and frequently at the bifurcation of retinal arterioles associated with leakage, typical of IRVAN (Figure 3). There was no evidence of active vasculitis or peripheral ischaemia in either eye (Figures 4a and b). Late-phase ICG clearly highlights the aneurysms alone, which remain hypercyanescent (Figure 5). Extensive investigations including cerebral imaging did not identify any systemic vasculitis or other abnormality. A diagnosis of IRVAN was made. Follow-up over 7 months did not show any progression of the condition and visual acuity was maintained.

Figure 3
figure 3

Fundus fluorescein angiography (FFA) of both eyes showing leaking retinal artery. aneurysms predominantly at the discs and at the bifurcation of vessels.

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Figure 4
figure 4

FFA showing the absence of peripheral nonperfusion in the right eye (a) and left eye (b).

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Figure 5
figure 5

Indocyanine green angiography of both eyes showing focal hypercyanescence, which clearly highlights the aneurysms.

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Comment

Capillary nonperfusion is almost universally present in adult IRVAN cases.1 Of the seven reported cases of IRVAN in patients less than 18 years old, three out of seven had no evidence of peripheral capillary nonperfusion.1, 2 Including our case, this makes four out of eight cases. Of the remaining four cases, three had capillary nonperfusion and one had evidence of neovascularisation.3, 4, 5 and 6

This case also shows that the presentation of IRVAN may be delayed in view of the resolving retinal exudates and no associated oedema. Furthermore, angiography did not reveal active vasculitis, despite clinical evidence of sheathed vessels, or capillary nonperfusion.

It appears that preserved capillary perfusion may be more commonly seen in paediatric cases of IRVAN but vigilance for capillary nonperfusion at presentation and close monitoring thereafter is still essential to reduce the risk of vision loss.