Year : 1992 | Volume
: 40 | Issue : 3 | Page : 96--98
Bilateral acute retinal necrosis-A case report
Palimar Prasad, NS Upadhyaya
O.E.U. Institute of Ophthalmology, Kasturba Medical College, Manipal, India
O.E.U. Institute of Ophthalmology, Kasturba Medical College, Manipal-576119
A 42 year old man presented with acute bilateral uveitis and necrotizing retinitis. Systemic investigations including test for AIDS and CMV retinitis were negative. Despite oral Acyclovir, both eyes progressed rapidly to retinal detachment with loss of vision. Early recognition is necessary to diagnose the bilateral acute retinal necrosis syndrome and initiate treatment. Bilateral acute retinal necrosis (BARN) is a term first coined by Young and Bird in 1978 although the syndrome had been originally described by Urayama et al as an unilateral condition. This syndrome is characterized by the triad of acute confluent peripheral necrotizing retinitis, moderate to severe vasculitis and vitritis in an otherwise healthy individual. Rhegmatogenous retinal detachment occurs within two to three months of the onset of the disease and the second eye is involved in 36% of patients, usually within 6 weeks. We herein report a patient who presented with simultaneous BARN leading to retinal detachment in a matter of days. Also, to our knowledge this is the first report of this condition in India.
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Prasad P, Upadhyaya N S. Bilateral acute retinal necrosis-A case report.Indian J Ophthalmol 1992;40:96-98
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Prasad P, Upadhyaya N S. Bilateral acute retinal necrosis-A case report. Indian J Ophthalmol [serial online] 1992 [cited 2022 Oct 5 ];40:96-98
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Mr. K., a 42 year old man was brought to our out patient department with a history of rapid loss of vision in the right eye 10 days and in the left eye 3 days prior to this visit. It was associated with low grade but constant fever. He had been treated elsewhere with oral Prednisolone 10 mg t.i.d., oral Chloramphenicol 500mg bid and gutt. Atropine bd. to both eyes. He was moderately built and nourished and was febrile (99.6 -F). Both eyes were congested. The right eye showed signs of moderate uveitis with multiple fine keratic precipitates, 3+ cells and 2+ flare. The pupil was atropinized. Ophthalmoscopic examination revealed hazy media, disc edema and diffuse retinal edema including the posterior pole. There were dot and blot hemorrhages more in the mid periphery and large yellowish white infiltrates in the peripheral fundus. The left eye showed milder uveitis. Diffuse retinal edema and ischemia were seen. The perivascular zones had a darker hue with vessel dilatation and blotchy hemorrhages along the vessels, particularly superiorly. [Figure 1][Figure 2]. He had no perception of light in the right eye; the left eye had bare perception with faulty projection of light. Intraocular pressures were 14 and 16mm Hg respectively.
An urgent medical consultation was sought. No neurological deficit nor other systemic anomalies were detected. A provisional diagnosis of panuveitis with neuro-retinitis was made and treatment begun with oral Ciprofloxacin 500 mg bd and Prednisolone 20 mg tid. Over the next 48 hours, his fever subsided but he lost all vision in the left eye. The right eye showed a large giant necrotic retinal tear with retinal detachment extending from the 4 to 8 O'clock meridian.
The following investigation were carried out: X-ray chest and sinuses normal, hemoglobin 10.5gm/dI, ESR 30mm first hr, WBC 9,800 cells/cu mm, Widal test - negative, VDRL non-reactive, urine analysis normal, blood culture for bacteria and fungi - sterile, ELISA for Human Immunodeficiency Virus (HIV) and Cytomegalovirus (CMV) - negative. Fluorescein angiography demonstrated obstructive vasculitis with staining of vessel walls and extensive leak from all the vessels. There was no perfusion beyond the third order arteriole. Peripheral to this area retinal edema masked background fluorescence initially to be replaced in the later phases with subretinal leak and staining [Figure 3][Figure 4][Figure 5][Figure 6]. With a revised diagnosis of acute retinal necrosis syndrome, oral Acyclovir 200 mg five times a day was started and oral corticosteroids tapered. Two days later, the inferotemporal retina in the left eye peeled away into the vitreous in the form of a necrotic tear, [Figure 7]. B-scan showed retinal detachments in both eyes.
The clinical picture remained essentially the same for the next week and he was sent home on low dose prednisolone. At last follow-up the retinal tear in the left eye had extended to almost 180° with corresponding detachment.
A presenting picture of bilateral panuveitis and neuroretinitis with features of severe ischemia should arouse the suspicion of CMV retinitis. However, yellowish retinal infiltrates in CMV retinitis progress relatively slowly in a centripetal manner and necrotic tears leading to retinal detachment is not a features Our patient was otherwise immuno-competent, an unusual setting for CMV infection, and, the ELISA test was negative.
The BARN syndrome has been reported in patients with the acquired. immuno-deficiency syndrome (AlDS) ; the ELISA test was negative in our case.
The usual mode of presentation in BARN is with periorbital pain, episcleritis and anterior uveitis followed by dimness of vision. A picture of neuro-retinitis changes to one of necrotic tear with retinal detachment within 2-3 months. This patient is quite unusual in that the condition deteriorated rapidly to a frank detachment within two weeks of the onset of symptoms. Similarly, simultaneous bilateral involvement has been only infrequently reported. Does this rapid progression and bilaterality represent some form of decreased racial immunity to unknown organisms? Immune complexes of herpes group of viruses and more specifically varicella zoster, per se , have been demonstrated in patients with BARN.
Various modalities of treatment have been tried. Systemic Acyclovir was tried in 13 eyes of 12 patients by Blumenkranz et al . Significantly, fresh retinal infiltrates did not appear once treatment was begun, although the incidence of retinal detachment remained high. However, the overall prognosis in their group appears to be better than in a series prior to the use of Acyclovir . Unfortunately, we had no access to the intravenous preparation and hence used the oral form. Systemic corticosteroids have been used before the relationship between BARN and varicella was discovered, with limited success. In view of no perception of light in both eyes, no surgical procedure was carried out. Heroic measures, again with variable success, include 360 0 encirclage, and vitrectomy with intravitreal Acyclovir .
This dreaded condition needs aggressive management to be instituted very early to salvage even little vision. Hence, awareness that such a syndrome does occur in this country is essential for early referral.
|1||Young NJA, Bird A C. Bilateral acute retinal necrosis, Br J Ophthalmol 1978; 62:581-90.|
|2||Urayama A., Yamada N., Susaki T., et al, Unilateral acute uveitis with retinal periarteritis and detachment Jpn J Clin Ophthalmol 1971; 25:607-19|
|3||Clarkson J.G., Blumenkranz M.S., Culbertson W.W., et al Retinal detachment following the acute retinal necrosis syndrome Ophthalmol 1984; 91:1665-68|
|4||Ryan S.,J., Scharchat A., P., Murphy R., P., Patz Retina Vol II, The C.V Mosby Company, St. Louis 1989, 617|
|5||Ryan S.,J., Glaser B.,M., Michels R.G., Retina Vol III, The C V Mosby Company, St. Louis, 1989, 591|
|6||Holland G.,N., Gottlieb M.,S., Yee R.,D., Schanker H.,M., Pettit T.,H., Ocular disorders associated with a new acquired cellular immunodeficiency syndrome Am J Ophthalmol 1982:93: 393-402|
|7||Kestelyn P., Van de perre P., Rouvroy D., et al A prospective study of the ophthalmologic findings in the acquired immune deficiency syndrome in Africa Am J Ophthalmol 1985:100:23038|
|8||Price F.,W., Schlaegel T.,F., Bilateral acute retinal necrosis Am J Ophthalmol 1980:89:419-24|
|9||Culbertson WW, Blumenkrancz M S, Pepose J S, Stewart J A, Curtin W T. Varicella zoster virus is a cause of the acute retinal necrosis syndrome. Ophthalmol 1986; 93:559-69|
|10||Blumenkrancz M S, Culbertson W W, Clarkson J G, Dix R. Treatment of acute retinal necrosis syndrome with intravenous Acyclovir Ophthalmol 1986;93:296-300|
|11||Peyman GA, Goldberg MF, Uninsky E et al. Vitrectomy and intravitreal drug therapy in acute retinal necrosis syndrome. Arch Ophthalmol 1984;91:1618-21|