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Year : 1999  |  Volume : 47  |  Issue : 1  |  Page : 38-39

Bilateral retinal arteritis with multiple aneurysmal dilatations

Medical Research Foundation, 18 College Road, Chennai - 600 006, India

Correspondence Address:
Mahesh P Shanmugam
Medical Research Foundation, 18 College Road, Chennai - 600 006
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Source of Support: None, Conflict of Interest: None

PMID: 16130286

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How to cite this article:
Shanmugam MP, Sharma T, Karna SD. Bilateral retinal arteritis with multiple aneurysmal dilatations. Indian J Ophthalmol 1999;47:38-9

How to cite this URL:
Shanmugam MP, Sharma T, Karna SD. Bilateral retinal arteritis with multiple aneurysmal dilatations. Indian J Ophthalmol [serial online] 1999 [cited 2023 Jan 29];47:38-9. Available from: https://www.ijo.in/text.asp?1999/47/1/38/22808

Bilateral retinal arteritis with aneurysmal dilatations is rare and to date only 15 cases have been reported.[1] It is an inflammatory periarteritis of as yet unknown etiology, associated with significant visual loss and peculiar aneurysmal dilatation of retinal arteries.

  Case Report Top

A 19-year-old female patient presented with decreased vision associated with recurrent attacks of pain, redness, and watering in both eyes, of 4 years duration. There was no history of any systemic or ocular disease. She had been treated with topical and systemic steroids without any visual or clinical improvement.

Her best corrected visual acuity was 6/36 N10 in the right eye and 6/60 N12 in the left eye respectively. Vitreous cells were found on slitlamp examination. Intraocular pressure (IOP) was within normal limits in both eyes.

Fundus examination of both eyes revealed minimal haziness of the vitreous, and macroaneurysmal dilatations with saccular outpouchings along the major arteries in the posterior pole [Figure - 1]. These aneurysmal dilatations were found on the retinal arteries overlying the disc and predominantly along the superior and inferior temporal arcades. There was evidence of disc edema in both eyes. Discrete hard exudates were noted in the mid-peripheral retina. Extensive hard exudate clumps were seen in the macular region of both eyes. Sclerosis of peripheral vessels was seen. There were suspicious areas of neovascularisation in the inferior quadrants of both eyes.

Fundus fluorescein angiography showed saccular aneurysmal dilatation of the retinal arterioles, especially along the temporal arcades [Figure - 2]. Clear-cut zones of capillary nonperfusion were seen in the periphery of both eyes. The left eye showed an area of neovascularisation inferiorly. Minimal late artero-venous (AV) phase leakage was seen from the aneurysmal dilatations.

Other than feeble femoral and dorsalis pedis pulsations, the patient was not found to have any systemic problems. Except for a mild rise in ESR (35 mm/1 hour) routine laboratory tests were normal. Investigations for auto-immune and other systemic diseases were negative.

The patient was advised calf-muscle biopsy to rule out poly arteritis nodosa but she refused. Focal laser photocoagulation to the neovascular frond in the left eye with scatter treatment to the peripheral areas of nonperfusion was done.

  Discussion Top

Ten patients with retinal arteritis, aneurysmal dilatations, and neuro-retinitis were studied by Chang et al,[1] who have proposed the acronym IRVAN (Idiopathic retinal vasculitis, aneurysms, and neuro-retinitis) for this entity. Kincaid et al[2] were the first to report 2 patients with bilateral retinal arteritis with multiple aneurysmal dilatations, but their description did not include neuro-retinitis which apparently was present in their patients. The presence of arterial aneurysmal dilatations, along the first and second order arterioles associated with peripheral capillary nonperfusion and exudative retinopathy is the classical clinical picture associated with this syndrome.[1][2][3][4]

One patient with aneurysms involving the venules has been reported.[1] This syndrome typically affects young healthy individuals (7-49 years) of white and dark pigmented races, and affects females more often than males.[1][2][3][4] Neovascularisation at the disc or elsewhere can occur as a consequence of peripheral capillary nonperfusion leading to vitreous hemorrhage.[1][2][3][4] Vitritis as seen in our patient is an inconsistent finding. Presence of anterior uveitis has also been noted.[1][2][3][4]

No satisfactory treatment exists as yet for this syndrome. Despite the obvious inflammatory nature of the condition, systemic and topical steroids do not seem to have any effect on the arteritis or the capillary nonperfusion.[1][2][3][4] In the natural course of the disease, aneurysms have been found to increase in size, and also have been documented to become smaller, and vanish.[3],[4] Segmental or pan-retinal laser photocoagulation is advocated in the presence of neovascularisation. Laser photocoagulation to the aneurysms can result in branch retinal artery occlusion and is not recommended.[1] Vitrectomy may be necessary if recurrent or massive nonclearing vitreous haemorrhage occurs. Owens et al have noted that on follow up the disease becomes quiescent.[4] Profound loss of vision can occur in these patients due to macular exudation, vitreous haemorrhage, and rubeotic glaucoma. Despite extensive investigations, no systemic disease or laboratory abnormality have been found to be associated with this syndrome.

The retinal arteries contain 5-7 layers of smooth muscle cells up to the equator, beyond which it thins down to 1-2 layers. The veins have 3-4 smooth muscle layers at the disc, which are replaced by fibroblasts within a short distance from the disc.[5] Kincaid et al have postulated that the site of inflammation is primarily the smooth muscle layer.[2] This explains the paucity of venous involvement and the predominant involvement of the posterior pole. The smooth muscle destruction leads to thinning of the vascular wall, that leads to aneurysmal dilatation.

Macular exudation associated with aneurysmal dilatations and periarteritis should alert the clinician to the presence of IRVAN.

  References Top

Chang TS, Aylward W, Davis JL, Mieler WF, Oliver GL, Maberley AL. Idiopathic retinal vasculitis, aneurysms, and neuro-retinitis. Ophthalmology 1995;102:1089-97.  Back to cited text no. 1
Kincaid J, Schatz H. Bilateral retinal arteritis with multiple aneurysmal dilatations. Retina 1983;3:171-78.  Back to cited text no. 2
Malan P, Ciurana AJ, Boudet C. Bilateral retinal arteritis with multiple aneurysmal dilatations. J Fr Ophthalmol 1986;9:23-28.  Back to cited text no. 3
Owens SL, Gregor ZJ. Vanishing retinal arterial aneurysms: a case report. Br J Ophthalmol 1992;76:637-38.  Back to cited text no. 4
Hogan MJ, Alvarado JA, Weddell JE. Histology of the Human Eye. Philadelphia: W.B. Saunders; 1971.  Back to cited text no. 5


  [Figure - 1], [Figure - 2]


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