BILATERAL ACUTE RETINAL NECROSIS: A Case Series.

PURPOSE: To investigate the clinical characteristics and visual outcome of bilateral acute retinal necrosis. METHODS: The study included 30 patients (60 eyes) who were diagnosed with bilateral acute retinal necrosis. The medical records were reviewed. RESULTS: Twenty-five patients developed the disease in the contralateral eye within 5 months and 5 patients at >2 years after the initial onset. At presentation, 14 of 21 eyes suffered from retinal necrosis of more than 180° in the initially affected eye, whereas 3 of 22 eyes suffered it in the later-affected eye. Retinal detachment occurred in 23 of the 27 initially affected eyes and in 5 of the 27 later-affected eyes. The mean logarithm of the minimum angle of resolution best-corrected visual acuity decreased from 2.0 ± 1.1 (Snellen equivalent counting fingers) to 2.2 ± 1.0 (Snellen equivalent counting fingers) in the initially affected eyes after a follow-up of 34.1 ± 48.2 months (P = 0.529), and improved from 0.5 ± 0.4 (Snellen equivalent 20/66) to 0.3 ± 0.4 (Snellen equivalent 20/40) in the later-affected eyes after a follow-up of 21.2 ± 23.3 months (P = 0.005). CONCLUSION: Bilateral acute retinal necrosis usually occurs in the contralateral eye within a few months, but sometimes after several years. Inflammation and retinal necrosis are less severe in the later-affected eye, with less retinal detachment and a better visual outcome.

Varicella-zoster virus as a causative agent of acute retinal necrosis in younger patients.

BACKGROUND: Herpes virus is considered to be the pathogen of acute retinal necrosis (ARN) infection. Previous studies have found that patients with ARN caused by the varicella-zoster virus (VZV) are often older, and patients with herpes simplex virus (HSV) induced ARN are considerably younger. However, in our clinical work, we find that VZV is also a pathogen in younger ARN patients. We, therefore, aimed to analyze the common etiology of younger ARN patients. METHODS: A retrospective analysis was made of 20 eyes (18 patients) diagnosed as having ARN in the Department of Ophthalmology of Peking Union Medical College Hospital from 2014 to 2016. All patients were reviewed for demographic data, clinical course, clinical manifestations, time from onset to initial physician visit, duration of follow-up, visual acuity at both presentation and final visit, and treatment strategies. A paired t test was used to compare visual acuity between the presenting vision and those of final follow-up. Vitreous or aqueous specimens from 18 eyes of 18 patients were analyzed with multiplex polymerase chain reaction (mPCR)/quantitative PCR (qPCR) and xTAG-liquid chip technology (xTAG-LCT) to determine the causative virus of ARN. RESULTS: Final best visual acuity (BCVA) improved significantly from 1.36 ±â€Š0.95 (median 20/400) to 0.95 ±â€Š0.82 (median 20/100) (t = 2.714, P = 0.015) after systemic and intravitreal antiviral treatment combined with or without pars plana vitrectomy. PCR and xTAG-LCT results showed four of the five samples in the younger group (32.2 ±â€Š5.2 years) and 12 of the 13 samples in the senior group (53.6 ±â€Š4.9 years) were positive for VZV, and two of the five samples in the younger group were positive for HSV-1. CONCLUSIONS: This study demonstrates that VZV is also a common causative virus for ARN in younger patients. Considering this finding, a systemic antiviral treatment protocol should be immediately changed to intravenous ganciclovir when the patient does not respond to acyclovir before determining the causative virus, especially in younger patients.

Clinical feature and visual prognosis of acute retinal necrosis according to the initially involved zone and extent: 10-year experience.

PURPOSE:: We aimed to analyze the visual prognosis and clinical characteristics of acute retinal necrosis according to the initially involved zone and extent. STUDY DESIGN:: Retrospective, consecutive, cross-sectional study. METHODS:: We retrospectively reviewed acute retinal necrosis patients who were treated with systemic antiviral treatment, and assessed the visual prognosis of acute retinal necrosis following antiviral treatment based on the initially involved zone and the extent of acute retinal necrosis at the time of diagnosis. Moreover, we evaluated whether prophylactic laser photocoagulation had preventive effects on retinal detachment. RESULTS:: A total of 43 eyes from 35 patients with acute retinal necrosis were included. Eyes with zone I involvement/extensive retinal lesions showed poor visual prognosis (logMAR: 1.17 ± 0.97 vs 0.49 ± 0.81; p = 0.014) and a higher incidence of retinal detachment (9/30 (30.0%) vs 0/13 (0%); p = 0.039) after treatment. Retinal detachment was observed in 20.9% of cases, and its incidence was significantly lower in eyes that had undergone prophylactic laser photocoagulation. CONCLUSION:: The overall visual prognosis of acute retinal necrosis is poor and can be determined at the time of diagnosis. Prophylactic laser photocoagulation exhibits preventive effects with regard to the development of retinal detachment. Therefore, laser photocoagulation should be actively considered in eyes with extensive retinal lesions to prevent retinal detachment and thus eventually improve the overall visual prognosis of acute retinal necrosis.

Progressive, subretinal fibrosis mimicking retinal necrosis with poor visual prognosis in sympathetic ophthalmia: A rare finding.

We report a rare finding of progressive subretinal fibrosis mimicking retinal necrosis in 2 cases of sympathetic ophthalmia. Histopathology of the inciting eye and vitreous biopsy of the sympathizing eye ruled out infections and masquerades. Progression of inflammation and rapid deterioration of vision inspite of maximum immunosuppression are key findings in this variant of sympathetic ophthalmia.

Varicella-zoster virus necrotising retinitis, retinal vasculitis and panuveitis following uncomplicated chickenpox in an immunocompetent child.

A 4-year-old girl presented with acute left visual loss 4 weeks after uneventful chickenpox. She was found to have left necrotising retinitis and profound retinal vasculitis and vitritis. Aqueous humour was PCR positive for varicella-zoster virus. Combined intravenous and intravitreal antiviral treatment led to rapid improvement with settled retinitis, no vascular occlusion and good recovery of vision. Her recent coinfection with Epstein-Barr virus may have acted to provoke the retinitis.

Acute retinal necrosis results in low vision in a young patient with a history of herpes simplex virus encephalitis.

Acute retinal necrosis (ARN), secondary to herpes simplex encephalitis, is a rare syndrome that can present in healthy individuals, as well as immuno-compromised patients. Most cases are caused by a secondary infection from the herpes virus family, with varicella zoster virus being the leading cause of this syndrome. Potential symptoms include blurry vision, floaters, ocular pain and photophobia. Ocular findings may consist of severe uveitis, retinal vasculitis, retinal necrosis, papillitis and retinal detachment. Clinical manifestations of this disease may include increased intraocular pressure, optic disc oedema, optic neuropathy and sheathed retinal arterioles. A complete work up is essential to rule out cytomegalovirus retinitis, herpes simplex encephalitis, herpes virus, syphilis, posterior uveitis and other conditions. Depending on the severity of the disease, the treatment options consist of anticoagulation therapy, cycloplegia, intravenous acyclovir, systemic steroids, prophylactic laser photocoagulation and pars plana vitrectomy with silicon oil for retinal detachment. An extensive history and clinical examination is crucial in making the correct diagnosis. Also, it is very important to be aware of low vision needs and refer the patients, if expressing any sort of functional issues with completing daily living skills, especially reading. In this article, we report one case of unilateral ARN 20 years after herpetic encephalitis.

[The clinical features of 10 cases of acute retinal necrosis complicated by viral encephalitis].

OBJECTIVE: To investigate the clinical features of acute retinal necrosis (ARN) complicated by viral encephalitis. METHODS: Ten cases of ARN complicated by viral encephalitis were treated in the Department of Ophthalmology, Beijing Tongren Hospital from November 2013 to August 2014. Clinical manifestation, especially the fundus characteristics, was summarized. RESULTS: In the10 patients (19 eyes; 6 men and 4 women) with an age of (40.1 +/- 13.44) years, 1 patients had unilateral ARN, and 9 patients had bilateral ARN. The visual acuity was no light perception in 9 eyes, light perception to hand motion in 7 eyes, 0.05 in 1 eye, 0.2 in 1 eye, and 0.3 in 1 eye. Seven cases suffered ARN during the onset of viral encephalitis, and other cases suffered ARN at 2 to 3 months after the recovery of viral encephalitis. Seventeen eyes had mild to moderate vitreous opacity, and 2 eye shad severe vitreous opacity. Sixteen eyes had focal (1 or 2 quadrants) retinal necrosis, and 2 eyes had massive ( > 2 quadrants) retinal necrosis. Occlusive vasculitis obviously occurred in 18 eyes. Sixteen eyeshad retinal detachment. All affected eyes had early optic nerve atrophy. CONCLUSION: ARN can occur during the onset of viral encephalitis or after the recovery of viral encephalitis. The clinical features of ARN complicated by viral encephalitis may be generally mild to moderate vitreous opacity,small range retinal necrosis foci, early and severe optic atrophy, and occlusive retinal vasculitis.

Use of intravitreal triamcinolone for the treatment of optic nerve edema in a patient with acute retinal necrosis: case report.

PURPOSE: To report the use of combined intravitreal triamcinolone and foscarnet therapy in a patient with active acute retinal necrosis. METHODS: Retrospective case report. A 40-year-old white woman with a history of ulcerative proctitis, intolerant to oral steroids, developed an aggressive case of acute retinal necrosis complicated by severe optic nerve edema resulting in 20/200 vision. RESULTS: Intravitreal foscarnet provided an initial improvement of the vitritis and retinitis; however, optic nerve edema and 20/200 vision persisted. After 2 weeks, intravitreal triamcinolone was combined with the foscarnet dose. Four days later, the optic nerve edema had resolved, the vision returned to 20/40, and the herpetic infection remained controlled. Eight months later, the patient had not experienced a retinal detachment, and acuity was 20/20. Oral steroids are an accepted adjunct to antiviral therapy in acute retinal necrosis; however, combined intravitreal steroid and antiviral therapy has not been previously reported. After combined intravitreal therapy was attempted, the patient experienced a rapid resolution of symptoms without exacerbating her retinitis. CONCLUSION: In select patients, combined intravitreal triamcinolone and foscarnet may be effective in treating acute retinal necrosis and its inflammatory sequelae.

Intravitreal triamcinolone acetonide as an adjuvant in the management of acute retinal necrosis.

OBJECTIVE: To determine the role of intravitreal triamcinolone acetonide (IVTA) as an adjuvant in the management of acute retinal necrosis (ARN). DESIGN: Interventional case series. PARTICIPANTS: Four eyes of 4 patients diagnosed with ARN were studied. METHODS: This pilot case series included 4 patients who presented with clinical features suggestive of ARN. IVTA was injected 1 to 2 weeks after initiation of antiviral therapy and concurrent usage of oral corticosteroids. The disease course and clinical outcome were observed. The patients received additional treatment depending on the sequelae of ARN. RESULTS: Signs of reduction in vitritis and resolution of retinitis were observed as early as 1 week after IVTA was administered. In all 4 patients, inflammation promptly resolved with no untoward effects like raised intraocular pressure or worsening of the retinitis. A final visual acuity of 20/40 or better was achieved in 3 of 4 patients after appropriate treatment of ARN-related complications. CONCLUSIONS: Intravitreal triamcinolone under antiviral cover could be a useful adjunct to systemic steroids in the treatment of ARN.

Acute retinal necrosis: factors associated with anatomic and visual outcomes.

PURPOSE: To examine the factors associated with anatomic and visual outcomes in Japanese patients with acute retinal necrosis (ARN). METHODS: One hundred four patients with ARN who were followed for more than 1 year at nine referral centers were reviewed. Retinal involvement at initial presentation was classified into four groups: zone 1 (posterior pole, n = 22), zone 2 (midperiphery, n = 54), zone 3 (periphery, n = 25), and unknown (n = 3). Forty-eight eyes underwent prophylactic vitrectomy before development of retinal detachment (vitrectomy group); 56 eyes were treated conventionally without prophylactic vitrectomy (observation group). RESULTS: The retina was attached in 28 of 48 eyes (58.3 %) in the vitrectomy group and 42 of 56 eyes (75.0 %) in the observation group at the final visit (P = 0.071). At 1 year, 56 eyes (53.8 %) had a best-corrected visual acuity (BCVA) of 20/200 or worse. Multivariate logistic regression analyses identified zone 1 disease (odds ratio = 4.983) and optic nerve involvement (odds ratio = 5.084) as significantly associated with BCVA of 20/200 or worse. Among the zone 3 eyes, significantly (P = 0.012) more eyes in the observation group than in the vitrectomy group had an attached retina. CONCLUSIONS: Prophylactic vitrectomy did not improve the final BCVA in any eyes. Zone 3 eyes had better outcomes without prophylactic vitrectomy.

Progressive outer retinal necrosis: outcomes in the intravitreal era.

OBJECTIVE: To describe the functional and anatomic outcomes of progressive outer retinal necrosis treated with intravitreal ganciclovir sodium injections. METHODS: A retrospective, interventional case series of all patients fitting established clinical diagnostic criteria for progressive outer retinal necrosis was conducted at a single institution in South Africa. Eyes with salvageable vision were treated with repeated intravitreal ganciclovir injections until regression was achieved or the eye lost light perception. Pars plana vitrectomy was performed when retinal detachments failed to resolve spontaneously. The main outcome measures were visual acuity (VA) and response to intravitreal ganciclovir. RESULTS: Thirty-nine patients (67 eyes), all of whom were HIV-positive (median CD4+ T-lymphocyte count, 30/µL), were included; 12 patients (31%) died during the study period. Twenty-eight of 36 patients (78%) had a recent history of cutaneous varicella zoster virus infection. At the initial evaluation, the mean VA was 6/120, with 12 eyes (18%) having already lost perception of light. Intravitreal ganciclovir injections were started immediately in all salvageable eyes (n = 50). Improvements in visual outcomes trended toward significance in eyes responding early (≤21 days), achieving a median final VA of 6/36 (P = .046). Retinal detachment occurred in 34 eyes (51%), predicating a significantly worse visual outcome (P < .001). Excluding eyes with no light perception at the start of the study period, median final VA was hand movements (range, 6/4 to no light perception); 9 eyes lost perception of light despite treatment. CONCLUSIONS: Progressive outer retinal necrosis remains a devastating condition, often with acute and profound loss of vision. Intravitreal ganciclovir may offer a more targeted approach and, compared with earlier reports using systemic therapy alone, may result in better visual outcomes.

Four cases of bilateral acute retinal necrosis with a long interval after the initial onset.

AIMS: To report the clinical features and causative virus of bilateral acute retinal necrosis (BARN) with a long interval after the initial onset. METHODS: The causative virus and clinical features were retrospectively investigated in four patients with delayed-onset BARN with an interval of more than 3 years after the onset of the disease in the initially affected eye. RESULTS: The intervals between the initially affected eye and the latter affected eye of the four cases were 8 years 7 months, 19 years 3 months, 9 years 7 months and 3 years 6 months. The fourth patient developed a second recurrence in the latter affected eye 17 years 6 months after the initial inflammation in the fellow eye. In all four cases, the same virus species, either varicella-zoster virus or herpes simplex virus, was detected in both eyes by PCR or antibody detection. In all cases, the final best-corrected visual acuity of the latter affected eye (20/20, 18/20, 20/20 and 12/20, respectively) was better than that of the initially affected eye (20 cm hand motion, light perception-negative, light perception-negative and light perception-positive, respectively). CONCLUSION: The present findings indicate that delayed-onset BARN in the fellow eye was caused by the same herpes virus species that induced the disease in the first affected eye.

A patient with acute visual loss and transient neurologic symptoms. Diagnosis: an acute central retinal artery occlusion (CRAO) in the left eye with severe retinal ischemia.

A 73-year-old man presented with acute visual loss in his left eye associated with transient left upper extremity numbness. Diagnosis was made in the emergency room, where treatment strategies were discussed.

Long-term follow-up of acute retinal necrosis.

PURPOSE: The purpose of this study was to report long-term visual outcome of acute retinal necrosis. METHODS: Medical records of patients with acute retinal necrosis were reviewed. RESULTS: Thirty-two patients were diagnosed with acute retinal necrosis from 1998 to 2007. Twenty patients (25 eyes) had at least 1 follow-up and available medical records. Intravitreal injections of ganciclovir and/or foscarnet were administered in 11 of 25 eyes. Intravenous and oral antiviral medications were used in 14 of 20 and 19 of 20 patients, respectively. Eleven of 25 eyes had <25% of retina affected, 8 of 25 had 25% to 50% of retina affected, and 6 of 25 had >50% of retina affected. Mean visual acuity at all time points was best when retinitis involved <25% and decreased as area increased. All but 1 eye with >50% involvement experienced decreased vision regardless of treatment. Three of 4 eyes with 25% to 50% involvement that received intravitreal antivirals had an improvement in visual acuity of > or =2 Snellen lines. Five of 25 eyes developed retinal detachment. None of the six eyes treated with prophylactic laser detached. CONCLUSION: Greater extent of retinitis portends a worse visual prognosis. Although intravitreal treatment did not prevent visual acuity loss in patients with severe disease, patients with moderate disease (25-50% retina involved) did well with intravitreal therapy with most having stable or improved visual acuity. Prophylactic laser decreased the rate of detachment.

Isolated unilateral acute retinal necrosis syndrome as the initial manifestation of HIV infection.

We report a rare case of isolated unilateral acute retinal necrosis syndrome without any history of associated herpes zoster or other conditions attributed to HIV infection as the initial manifestation of HIV infection. A 30-year-old man presented with a history of sudden diminished vision in the right eye with no apparent predisposing factors. Examination revealed right eye acute retinal necrosis. All laboratory test results were normal except the patient tested positive for HIV. This case of an atypical ophthalmic manifestation as initial presentation of HIV infection highlights the need for increased index of suspicion for HIV infection in young patients with acute retinal necrosis syndrome.

Acute retinal necrosis caused by herpes simplex virus type 2 in a 3-year-old Japanese boy.

Acute retinal necrosis (ARN), which is characterized by rapidly progressing peripheral retinal necrosis, is caused mainly by herpes simplex virus type 1, herpes simplex virus type 2 (HSV-2), or varicella-zoster virus. A previously healthy 3-year-old Japanese boy developed ARN in his left eye after being bruised by a milk container. HSV-2 DNA was detected in the aqueous humor of the affected eye. Serological testing suggested that the route of infection was from mother to child, although there was no past history of apparent HSV-2 infection. Childhood ARN has not been previously reported in Japan, possibly because of the low seroprevalence of HSV-2 in Japanese women. Pediatricians must be aware of this rare disease, which can affect individuals without a previous history of HSV even in a country with a low seroprevalence of HSV-2.

[Clinical features of acute retinal necrosis at Hokkaido University Hospital].

PURPOSE: To study clinical features of acute retinal necrosis (ARN) at Hokkaido University Hospital. METHODS: Twenty-one eyes of 19 patients with ARN (10 male and 9 female patients) who were treated at Hokkaido University Hospital between 1992 and 2006 were retrospectively examined from clinical records. RESULTS: The average age of the patients was 53.4 years (range, 13 to 91 years). 17 cases were unilateral and 2 were bilateral. The pathogenic virus was herpes simplex virus-1 (HSV-1) in 2 patients, and varicella-zoster virus (VZV) in 17 patients. Clinical severity was assessed from spreading speed and area of the retinal exudation, and 5 eyes were judged as fulminant cases (4 VZV eyes, 1 HSV eye), 6 eyes as severe cases (6 VZV eyes), and 10 eyes as mild cases (9 VZV eyes, 1 HSV eye). The range of retinal exudation was 1 to 2 quadrants in 7 eyes, 3 to 4 quadrants in 3 eyes, and increased to all quadrants in 11 eyes. Retinal detachment (RD) was observed in 8 eyes (38%), and the final visual acuity was less than 0.1 in 9 eyes (43%). CONCLUSIONS: The leading cause of ARN at Hokkaido University Hospital was VZV, and no HSV-2 ARN was seen. Compared with other areas in Japan, ARN at Hokkaido University Hospital seems to show less frequent RD, but the same prognosis for final visual acuity.

Long-term preservation of vision in progressive outer retinal necrosis treated with combination antiviral drugs and highly active antiretroviral therapy.

PURPOSE: To report the successful long-term treatment of varicella zoster virus-associated progressive outer retinal necrosis (VZV-PORN) with aggressive antiviral combination drugs along with highly active antiretroviral therapy (HAART). DESIGN: Interventional case report. METHODS: Combined treatment of progressive outer retinal necrosis in a university-based tertiary eye hospital with ganciclovir implant, intravenous acyclovir (10 mg/kg every 8 h), intravitreal foscarnet (2.4 mg), and HAART. RESULTS: Successful treatment of progressive outer retinal necrosis with disease remission and preservation of 20/20 visual acuity out to 1 year. CONCLUSIONS: Combination antiviral therapy and HAART may improve long-term visual outcomes for VZV-PORN.

Angiogenesis: molecular mechanisms of activation, promotion and maintenance.

The formation of new blood vessels is a fundamental process that occurs during embryonic and post-natal development but also in a number of pathologies ranging from cancer to chronic inflammatory disease. The molecular regulations of this process are complex since they require signalling that modifies both the quantity and the quality of the vasculature. Many players have been characterized and there are many more still to come. In this article I will review basic concepts and some of the major regulatory pathways and there implication in physiological and pathological angiogenesis.