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A Mouse Model of Retinal Ischemia-Reperfusion Injury Through Elevation of Intraocular Pressure
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Published on: July 14, 2016

Elevated intracranial pressure associated with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome

Matthew D Hammond1, Thomas P Ward, Barrett Katz

  • 1Ophthalmology Service, Walter Reed Army Medical Center, 5900 Georgia Avenue NW, Washington, DC 20307, USA.

Journal of Neuro-Ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society
|September 7, 2004
PubMed

Insights

Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN) syndrome can present with optic disc swelling and elevated intracranial pressure. This case highlights a potential initial presentation of IRVAN syndrome in young patients.

Area of Science:

  • Ophthalmology
  • Neurology
  • Vascular Biology

Background:

  • Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN) syndrome is a rare condition typically affecting young individuals.
  • It is characterized by retinal macroaneurysms, neuroretinitis, and peripheral capillary nonperfusion.

Observation:

  • A 12-year-old girl presented with bilateral optic disc swelling and peripapillary hemorrhage.
  • Brain MRI was normal, but lumbar puncture revealed elevated intracranial pressure (360 mm H2O).
  • MR venography showed poor filling of transverse venous sinuses.

Findings:

  • Fluorescein angiography confirmed saccular aneurysms in the retinal arteriolar vasculature.
  • IRVAN syndrome was diagnosed based on clinical presentation and angiographic findings.
  • The patient's optic nerve swelling and elevated intracranial pressure preceded the characteristic vascular abnormalities.

Implications:

  • This case suggests that optic nerve swelling and elevated intracranial pressure may be initial manifestations of IRVAN syndrome.
  • Early recognition of these signs could lead to earlier diagnosis and management of IRVAN syndrome.
  • Understanding the varied presentations of IRVAN syndrome is crucial for effective patient care.

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