Poster 3 Joaquin Castro Navarro

Advantages:

To report a case of an incomplete IRVAN (Idiopathic Retinal Vasculitis, Aneurysms and Neuroretinitis) syndrome.

Methods:

A 21-year-old women presented with blurred vision for 10 days in her left eye (LE). A complete ocular exploration, including Visual Acuity (VA), anterior and posterior segment examination, fluorescein angiography (FA) was carried out. Complementary exams were also performed.

Effectiveness / Safety:

VA was 0.9 in right eye (RE) and hand movement in LE. Anterior segment examination revealed the presence of tyndall ++, with no further abnormalities in both eyes. Fundoscopy examination showed small areas of vasculitis in periphery of RE, and mild vitritis, perivenous sheathing, disc oedema, intraretinal hard exudates, macular scar, and retinal diffuse oedema in LE. FA showed focal venous leakage along with areas of capillary nonperfusion in periphery of both eyes, and diffuses retinal and optic nerve leakage in LE. The work-up included: complete blood analysis with autoimmune tests (rheumatoid factor, anti-native DNA, ANAs, ENAs, Anticardiolipin antibodies -ACA- antibodies), evoked potentials, VDRL, FTA-ABS, urinalysis, tuberculin skin testing, infectious serology (CMV, Toxoplasma, HSV, HZO, Brucella, Borrelia, syphilis), lumbar puncture, cranial imaging (MRI), and chest radiograph. No abnormalities were found in all these complementary exams. Slow tapering of systemic and topical steroids was started. One year later, VA decreased to light perception. Anterior segment showed no signs of inflammation. Fundoscopy revealed absence of activity in both eyes, and the presence of an atrophic optic nerve in LE.

Conclusion:

This case might represent an incomplete form of the IRVAN syndrome, without aneurysms. There are no cases described in peer-reviewed literature about incomplete IRVAN syndrome. However, the optic disc oedema and the extensive intraretinal lipid deposition in our patient resembled it. However, the absence of aneurysms make it fairly impossible, as it is one of the major criteria considered for IRVAN syndrome until now. Another option would be the existence of an atypical form of idiopathic vasculitis with papillitis and great hard exudation. Anyway, this kind of clinical presentation deserves careful examination, as oral steroids can be ineffective and visual function can be severely deteriorated.