Retina Associates’ Case of the Month: January 2016

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Author: Stephen Ong

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DB, 34 year old labourer asymptomatic

Case History

DB
male: age 34
labourer

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.

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Fig. 1

The following colour photos show his fundus findings:

Clinical Findings

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.

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Fig. 2

Additional Investigations

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

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Fig. 3



BURGESS-OCT1

Fig. 4 OCT of the right eye showing intra-retinal exudates

Diagnosis

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
  • IRVAN is a rare, ocular disease with poor prognosis
  • early treatment of retinal neovascularisation can prevent vision loss in these patients
  • it is an idiopathic disease of unknown aeitiology
References:
  1. 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