Elevated intracranial pressure associated with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome.
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.
Hammond
Matthew D
MD
Ophthalmology Service, Walter Reed Army Medical Center, 5900 Georgia Avenue NW, Washington, DC 20307, USA.
Ward
Thomas P
TP
Katz
Barrett
B
Subramanian
Prem S
PS
D000783:Aneurysm / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D002648:Child; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D019586:Intracranial Hypertension / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D010211:Papilledema / Q000175:diagnosis / Q000209:etiology; D012161:Retinal Artery / Q000473:pathology*; D012166:Retinal Hemorrhage / Q000175:diagnosis / Q000209:etiology; D031300:Retinal Vasculitis / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D012173:Retinitis / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D013577:Syndrome; D014794:Visual Fields