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

Matthew D. Hammond, Thomas P. Ward, Barrett Katz, Prem S. Subramanian

Research output: Contribution to journalArticle

14 Citations (Scopus)

Abstract

The idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome typically occurs in young patients and may produce multiple retinal macroaneurysms, neuroretinitis, and peripheral capillary nonperfusion. Optic disc edema has been described, but elevated intracranial pressure has not been previously documented. We report a case of a 12-year-old girl who presented with bilateral disc swelling and peripapillary hemorrhage. Brain magnetic resonance imaging (MRI) was normal, but lumbar puncture yielded an opening pressure of 360 mm H2O with normal constituents. Fluorescein angiography delineated saccular aneurysms of the retinal arteriolar vasculature, and IRVAN syndrome was diagnosed. MR venography disclosed poor filling of both transverse venous sinuses. Acetazolamide treatment of 14 months did not alter the fundus findings. IRVAN syndrome may present initially with optic nerve swelling and elevated intracranial pressure with subsequent development of the characteristic retinal vascular abnormalities.

Original languageEnglish (US)
Pages (from-to)221-224
Number of pages4
JournalJournal of Neuro-Ophthalmology
Volume24
Issue number3
DOIs
StatePublished - Sep 2004
Externally publishedYes

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Retinal Vasculitis
Retinitis
Intracranial Hypertension
Aneurysm
Transverse Sinuses
Retinal Vessels
Papilledema
Acetazolamide
Spinal Puncture
Phlebography
Fluorescein Angiography
Optic Nerve
Magnetic Resonance Imaging
Hemorrhage
Pressure
Brain

ASJC Scopus subject areas

  • Clinical Neurology
  • Ophthalmology

Cite this

Elevated intracranial pressure associated with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome. / Hammond, Matthew D.; Ward, Thomas P.; Katz, Barrett; Subramanian, Prem S.

In: Journal of Neuro-Ophthalmology, Vol. 24, No. 3, 09.2004, p. 221-224.

Research output: Contribution to journalArticle

Hammond, Matthew D. ; Ward, Thomas P. ; Katz, Barrett ; Subramanian, Prem S. / Elevated intracranial pressure associated with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome. In: Journal of Neuro-Ophthalmology. 2004 ; Vol. 24, No. 3. pp. 221-224.
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