Author: Stephen Ong
DB, 34 year old labourer asymptomatic
male: age 34
Past Ocular History:
1. Bilateral keratoconus.
2. Left corneal graft (2013)
DB’s vision is 6/7.5 right and 6/24 left, pinhole 6/12. His eyes were quiet. He has quiet left corneal graft with no signs of rejection.
His retina shows evidence of neuro-retinitis, with hard exudates originating from both optic discs associated with disc swelling. There is also vasculitis, retinitis and retinal arterial aneurysm formation.
Fluorescein angiogram revealed evidence of inflammation along the arterial walls as well as a marked disc leak. There is arterial aneruysm formation close to or at the arterial bifurcations. Later, scattered retinal non-perfusion led to neovascularisation in the retina in both eyes.
Additional investigations included:
1. Imaging to exclude a central mass occupying lesion. MRI, A V normal
2. A full systemic work up for an infective cause: FBC, EUC inflammatory markers negative, neuroretinitis screen Lyme etc Negative syphillis serology and Quantiferonn Gold Assay
3. An autoimmune screen. negative
4. Baseline blood testing. negative
DB has an unusual spectrum of retinal findings including retinitis, vasculitis, anurysmal formation and neuro-retinitis which are consistent with IRVAN Syndrome (idiopathic retinitis, vasculitis, aneurysm and neuro-retinitis)
Treatment and Clinical Course
DM underwent panretinal laser to both eyes to treat the retinal non-perfusion and successfully regressed the new vessels.
The neuro-retinitis and vasculitis has clinically improved which is not the usual clinical course in these cases, which usually carries a poor prognosis.
Take home points
- Idiopathic Retinitis, Vasculitis, Aneurysms, and Neuroretinitis (IRVAN) New Observations and a Proposed Staging System
Ophthalmology 2007;114:1526 –1529: 2007 the American Academy of Ophthalmology