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Show ORIGINAL CONTRIBUTION Elevated Intracranial Pressure Associated With Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis Syndrome Matthew D. Hammond, MD, Thomas P. Ward, MD, Barrett Katz, MD, and Prem S. Subramanian, MD, PhD Abstract: The idiopathic retinal vasculitis, aneurysms, and neuroretinitis ( IRVAN) syndrome typically occurs in young patients and may produce multiple retinal macroan-eurysms, neuroretinitis, and peripheral capillary nonperfu-sion. 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 H20 with normal constituents. Fluorescein angiography delineated saccular aneurysms ofthe retinal arteriolar vasculature, and IRVAN syndrome was diagnosed. MR venography disclosed poor filling of both transverse venous sinuses. Acet-azolamide 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. ( JNeuro- Ophthalmol 2004; 24: 221- 224) Arare syndrome consisting of multiple retinal macroan-eurysms, neuro- retinitis, and peripheral retinal capillary nonperfusion is known as the idiopathic retinal vasculitis, aneurysms, and neuroretinitis ( IRVAN) syndrome ( 1- 3). The vascular lesions are pathognomonic but of uncertain cause. We report a case of IRVAN syndrome that had prominent disc edema, increased intracranial pressure as measured by lumbar puncture opening pressure, and du-ral venous sinus abnormalities. This is the first case to document elevated intracranial pressure. Ophthalmology Service ( MDH, TPW, PSS), Walter Reed Army Medical Center, Washington, DC; Uniformed Services University ofthe Health Sciences ( TPW, PSS), Bethesda, MD; Department of Ophthalmology ( BK), The George Washington University School of Medicine, Washington, DC. Address correspondence to Prem S. Subramanian, MD, PhD, Ophthalmology Service, Walter Reed Army Medical Center, 5900 Georgia Avenue NW, Washington, DC 20307; E- mail: prem. Subramanian@ na. amedd. army. mil Ellen Foer was the Ophthalmic Photographer for this article. CASE REPORT A 12- year- old girl experienced intermittent blurred vision OU. Ophthalmologic examination disclosed a visual acuity of 20/ 20 OU, normal pupillary reactions, and indistinct optic disc margins OU. Three months later, optic nerve edema was apparent, associated with dilated parapapillary vessels and retinal hemorrhages in the posterior pole OU. Brain magnetic resonance imaging ( MRI) and computed tomography ( CT) scans were normal. Lumbar puncture yielded an opening pressure of 360 mm H20 with normal fluid constituents. The patient had no predisposing characteristics for idiopathic intracranial hypertension such as obesity, chronic obstructive pulmonary disease, or pregnancy, nor did she use contraceptive pills, tetracycline, or corticosteroids. Therapy was started with oral acetazol-amide, 250 mg twice daily. Examination one month later showed no change in visual function. Fluorescein angiography delineated saccular aneurysms ofthe retinal arteriolar vasculature. IRVAN syndrome was diagnosed and the patient was referred to our neuro- ophthalmology service for further evaluation of the persisting disc edema. Our initial examination disclosed a normal blood pressure, visual acuities of 20/ 20 OU, normal color vision by Ishihara plate testing, and normal pupillary reactions. Ocular motility was full, and the patient was orthophoric at distance and near. The anterior segment examination was unremarkable. Ophthalmoscopic examination revealed mild anterior vitreous cells ( small, pigmented, and immobile) without haze OU. Both optic nerves were moderately elevated with peripapillary edema ( Figs. 1A and B). There were saccular aneurysmal dilatations present in the peripapillary retinal vasculature OU. The maculae were flat with some mottling. There were aneurysmal dilatations at arteriolar bifurcations and prominent venous beading. Multiple intraretinal hemorrhages and a few nerve fiber layer hemorrhages were present in the temporal retinas OU ( Figs. 1C and D). Choroidal neovascularization and retinal exudates and hemorrhages were present in the inferior periphery OU. The intraretinal hemorrhages appeared slightly increased compared with photographs taken one month earlier. J Neuro- Ophthalmol, Vol. 24, No. 3, 2004 221 JNeuro- Ophthalmol, Vol. 24, No. 3, 2004 Hammond et al FIG. 1. Ophthalmoscopic findings of IRVAN syndrome. A: Optic disc edema, lipid deposition, and aneurysms OD. B: Optic disc edema, lipid, and hemorrhage OS. C: Retinal neovascularization OD ( corresponds to Fig. 2A). D: Intraretinal hemorrhage, vascular sheathing, and vascular attenuation OD. Additional evaluation with fluorescein angiography revealed vascular leakage around the optic nerves, vascular enhancement, saccular dilations, and peripheral retinal ischemia OU ( Fig. 2). These dilatations were more obvious on the fluorescein angiographic study than by indirect ophthalmoscopy. Automated perimetry once again showed an enlarged blind spot OU, unchanged from studies one month earlier. Lumbar puncture showed an opening pressure of 270 mm H20 with normal fluid constituents. Complete blood count, Lyme antibody titer, anti- ds deoxyribonucleic acid, antinuclear antibodies, Sjogren- specific antibody A, Sjogren- specific antibody B, and anti- Smith antibodies, C3, C4, thyroid- stimulating hormone, RPR, and Bartonella titer were all negative. The patient continued to deny headache or new visual changes. Her acetazolamide dose was increased to 1500 mg per day. Eight weeks later, disc edema appeared to be worsening. Magnetic resonance renography revealed that both transverse sinuses had incomplete filling ( Fig. 3). However, the presence of an intramural thrombus could not be determined conclusively. Serial examinations over the next several months showed no reproducible changes in visual function. Visual acuity remained 20/ 20 OU, and visual field testing by automated perimetry showed enlarged blind spots and a variable mean deviation over time, as evidenced by visual fields obtained six months and 14 months after presentation ( Fig. 4). Disc edema was stable, and fluorescein angiography showed continued peripheral retinal ischemia with posterior pole aneurysmal vascular dilatations. DISCUSSION Our patient had the ocular manifestations of a syndrome first described by Kincaid and Schatz ( 1), now named the IRVAN syndrome ( 3). To date, only 20 cases of this syndrome have been reported ( 1- 10), with 10 in a mul-ticenter collaborative report ( 3). The most common ocular findings are the characteristic aneurysmal dilatations of the retinal and optic nerve head arterioles. Although these aneurysmal dilatations generally leak on fluorescein angiography, they do not have a predisposition to form subretinal, retinal, or vitreous hemorrhages ( 6,7). Peripapillary subreti- 222 © 2004 Lippincott Williams & Wilkins Elevated Intracranial Pressure JNeuro- Ophthalmol, Vol. 24, No. 3, 2004 FIG. 2. Fluorescein angiography. A: Retinal neovascularization and dye leakage nasal to optic nerve OD. B: Aneurysms and intraretinal microvascular abnormalities in the posterior pole OD. C, D: Peripheral retinal ischemia with capillary obliteration OD. nal fluid and lipid deposition, along with peripheral capillary nonperfusion, are common findings in IRVAN syndrome. These can lead to retinal neovascularization ( 3). Vitritis has been reported in several cases and was present in our case. No specific pathogenic mechanism has been identified, although an inflammatory process, possibly autoimmune- mediated, has been postulated ( 4,8). It has been suggested that an inflammatory component may contribute to arteriolar wall weakening, which could lead to aneurysmal dilatation ( 8). Treatment of IRVAN syndrome is problematic. Local and systemic corticosteroids have been shown to have little to no effect on disease progression ( 3,5,7,10), but analysis of their effect is confounded by other interventions. Panretinal photocoagulation of the ischemic retina has been associated with subsequent aneurysmal resolution ( 3,5,10). Capillary nonperfusion may be the most important factor in predicting vision loss ( 3), but data remain limited ( 6). Visual loss is usually secondary to complications of retinal neovascularization and may be severe despite aggressive therapeutic retinal photocoagulation or pars plana vitrectomy. FIG. 3. Magnetic resonance venogram demonstrates incomplete filling of the transverse sinuses ( arrows), the right greater than the left. 223 JNeuro- Ophthalmol, Vol. 24, No. 3, 2004 Hammond et al • : - . B w.:' - J^. FIG. 4. Visual fields ( Humphrey 24- 2 SUA standard) performed 6 ( A) and 14 ( B) months after initial presentation show little change over time. The case that we present illustrates a new aspect of the IRVAN syndrome: increased intracranial pressure. Elevated intracranial pressure has not been previously assessed in association with IRVAN syndrome, even though disc edema and prominent disc leakage on fluorescein angiography have been common findings in such patients. Two patients have been reported to have had normal cerebrospinal fluid contents but opening pressure was not described ( 3). Our patient's disc edema failed to respond to acetazolamide therapy up to 2000 mg per day ( with patient compliance noted through symptoms of tingling in the extremities and dysgeusia for carbonated beverages). In our case, intracranial venous obstruction appeared to be present, but we could not determine if this finding was primary or secondary to the elevated intracranial pressure ( 11- 13). Although a recent study suggests a significantly higher incidence of MRV transverse sinus abnormalities in idiopathic intracranial hypertension patients ( 14), MRV may falsely indicate absent transverse sinus filling ( 15). It is possible that the association between IRVAN syndrome and elevated intracranial pressure has gone unrecognized in previous reports, because opening pressures may not have been recorded. 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