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Acute retinal necrosis syndrome.

Acute retinal necrosis syndrome (ARN) is a rare and potentially visually devastating syndrome that occurs in otherwise healthy patients. We present ten cases seen at the Veterans General Hospital-Taipei from 1985 to 1990. All patients were male. The onset occurred at the age of 19 to 71 years (43.7 in average). All except one case were unilaterally involved. Among the 11 diseased eyes, 4 eyes of 3 cases recovered a fair vision and the rest 7 eyes nearly lost vision. There was nearly total blindness in the earlier cases. Retinal detachment occurred in 7 cases (70%). Intraocular antibody production by determining the Goldmann-Witmer coefficient was calculated in 9 cases. It was found significant elevation of varicella zoster virus in 8 cases and of herpes simplex virus in 1. Although all patients appeared healthy with nothing particular in the past history, a decreased reaction to delayed type hypersensitivity as performed with skin anergy panel test was found in 4 of 5 performed patients.

Adolescent↗

Chickenpox-associated acute retinal necrosis syndrome.

Acute retinal necrosis (ARN) syndrome usually occurs as the result of secondary reactivation of latent, previously acquired, varicella-zoster or herpes simplex virus. The authors report four patients who developed a mild form of ARN within 1 month (5 to 28 days) after the onset of chickenpox. In contrast to typical cases of ARN, these cases were less severe, with retinitis limited to two quadrants or less (three patients), no retinal detachment (four patients), minimal vitreitis (four patients), and no loss of visual acuity (four patients). Thus, ARN may occur during the course of primary varicella-zoster infection.

Acyclovir↗

Acute retinal necrosis syndrome.

Acute retinal necrosis (ARN) is a rare syndrome with characteristic fundal appearances which can have devastating effects on vision. We present six cases (nine eyes) seen in the Medical Eye Unit of St Thomas's Hospital over the past six years and discuss the clinical features, aetiology, and management. Our findings support the present consensus that the condition is caused by varicella zoster virus (VZV) or herpes simplex virus (HSV). One of our patients, who was atypical in having common variable hypogammaglobulinaemia, had suffered a widespread zosteriform rash immediately prior to the onset of ARN, while another had suffered a herpes simplex uveomeningoencephalitis. All cases had characteristic confluent peripheral retinal necrosis, and three of the nine eyes developed retinal detachment. Retinal arteritis was a prominent and helpful diagnostic feature in one case. From combining all reports to date of this rare condition it is possible to conclude that ARN is unilateral in 65% of cases.

Adult↗

Viral causes of the acute retinal necrosis syndrome.

Acute retinal necrosis has been described as a clinical entity for nearly 30 years. Acute retinal necrosis is a potentially visually devastating necrotizing vaso-occlusive retinitis affecting both healthy and immunocompromised patients. Acute retinal necrosis is caused by the herpes group of viruses, mainly varicella zoster, herpes simplex types 1 and 2, and, rarely, cytomegalovirus. Recently, polymerase chain reaction techniques have enabled detection of very small amounts of viral DNA from intra-ocular fluid samples. This can help in both the diagnosis of atypical cases of retinitis and uveitis and directing treatment in cases of acute retinal necrosis.

Antiviral Agents↗

Treatment of acute retinal necrosis syndrome.

Acute retinal necrosis is a distinct ocular viral syndrome with a potentially devastating visual outcome, traditionally treated with intravenous acyclovir followed by oral acyclovir. The goals of therapy include accelerating the resolution of the infection in the affected eye and prevention of contralateral involvement. Recently, several small case series and case reports have described the use of newer oral antivirals such as valacyclovir, famciclovir or valganciclovir, alone or in combination with adjunctive intravitreal foscarnet or ganciclovir injections for the treatment of acute retinal necrosis (1-4). The purpose of this article is to describe various therapies available to practitioners who may treat patients with acute retinal necrosis.

Animals↗

Laser photocoagulation in the acute retinal necrosis syndrome.

Acute retinal necrosis (ARN) is a syndrome of fulminant necrotizing vaso-occlusive retinitis associated with a high incidence of retinal detachment due to retinal breaks and vitreous traction. We performed argon or krypton laser photo-coagulation to demarcate areas of active retinitis as prophylaxis against retinal detachment in five patients with ARN. Patients were concomitantly treated with antiviral agents, systemic steroids, and antiplatelet therapy. One patient required multiple additional treatments as retinal necrosis progressed. The retinas remained attached in all five patients over a mean follow-up period of 15 months. By creating a chorioretinal adhesion in areas of potential retinal break formation, laser photocoagulation may be an effective prophylaxis against retinal detachment in ARN.

Acute Disease↗

Association of herpetic encephalitis with acute retinal necrosis syndrome.

Bilateral acute retinal necrosis syndrome developed shortly after a severe acute encephalitis in two previously healthy adults. The clinical and laboratory data suggested that herpes simplex virus was the cause of the encephalitis and acute retinal necrosis in these patients. To the authors' knowledge, this is the first report of an association of herpetic encephalitis with acute retinal necrosis in the literature.

Adult↗

Diagnosis and management of the acute retinal necrosis syndrome.

The acute retinal necrosis (ARN) syndrome is an increasingly occurring entity characterized by the triad of acute confluent peripheral retinitis with papillitis and anterior-chamber uveitis. We present case reports on four patients (age, 12-65 years) with an ARN syndrome caused by herpes simplex or varicella zoster virus and discuss diagnostic and therapeutic modalities. Immediate antiviral therapy in three patients exhibiting the typical clinical features reduced the intraocular inflammation. However, due to proliferative vitreoretinopathy with peripheral retinal necrosis, vitrectomy with encircling band and silicone oil instillation was necessary in all patients. The suspected diagnosis of an ARN syndrome induced by herpes simplex virus (HSV) was confirmed in one case during the early stage of the disease by the detection of increased levels of HSV-IgA in the vitreous and in another case by the measurement of increased titers of HSV-IgG in the vitreous. For the first time, we found intraocular HSV DNA sequences using the polymerase chain reaction (PCR) in one of these patients. In a fourth patient intraocular varicella zoster virus (VZV) infection was confirmed by the detection of elevated VZV-IgA levels and by positive PCR in the intraocular fluids. Two patients who were diagnosed and treated early retained a visual acuity of 0.4 and 0.5, respectively, whereas in the other two patients, whose diagnosis and therapy were delayed (> 6 weeks), visual acuity was reduced to light perception. We conclude that use of the PCR in the intraocular fluids together with detection of autochthonous antibodies in the vitreous seem to be the most important diagnostic laboratory tools.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

Acute retinal necrosis syndrome.

1. Acute retinal necrosis (ARN) represents a specific pattern of clinical presentations for certain herpes virus infections in the posterior segment of the eye. These include varicella zoster virus and herpes simplex virus. 2. Patients with ARN usually complain of mild to moderate ocular or periorbital pain, foreign body sensation, and a red eye. Visual symptoms usually include hazy vision, floaters, and, rarely, decreased peripheral vision. 3. Medical treatment of choice is intravenous acyclovir. Surgical treatment of choice is pars plana vitrectomy. If retinal detachment is present, vitrectomy with endolaser, internal drainage of subretinal fluid, air-fluid exchange, and long-acting gas tamponade are recommended.

Acyclovir↗

[Expression of the varicella zoster virus thymidine kinase and cytokines in patients with acute retinal necrosis syndrome].

PURPOSE: Acute retinal necrosis (ARN) is caused by varicella zoster virus (VZV) infection. In this study, we investigated the activity of this virus and expressions of some cytokines. PATIENTS AND METHODS: The expression of VZV thymidine kinase and some cytokines were investigated by reverse transcriptase-polymerase chain reaction (RT-PCR) in 9 eyes of 8 patients with ARN. RESULTS: Thymidine kinase expression was observed in all samples except one. Several cytokines, such as interferon (IFN) gamma, tumor necrosis factor (TNF) alpha, interleukin (IL)-1 beta, IL-6, and transforming growth factor (TGF) beta 1 were observed in the samples. Among these cytokines, a statistically significant expression of IFN gamma was observed in the samples of ARN, when compared to those of proliferative vitreoretinopathy (PVR) or other uveitis. The expression of IFN gamma also decreased during successive follow-ups. CONCLUSION: These cytokines may play an important role in the immune response in ARN.

Adolescent↗

So-called 'acute retinal necrosis syndrome'--an acute ocular panvasculitis syndrome.

An acute ocular disorder, characterized by panuveitis, retinal vasculitis, retinal necrosis (with frequent secondary retinal detachment) and frequent optic atrophy, has been reported since 1971 under different eponyms in 74 patients (103 eyes). I report 4 more cases. This paper presents, based on a critical review of 107 eyes in 78 patients, a detailed clinical description, pathogenesis and terminology of this distinct ocular disease entity. The available clinical and histopathological evidence very strongly indicates that the basic pathology is acute vasculitis of the iris, choroid, retina and optic nerve head, producing vascular occlusion in the choroid, retina and optic nerve head; the clinical findings thus represent acute ischemic lesions of those tissues, in addition to panuveitis. From the available evidence, it seems more appropriate to call this condition 'acute ocular panvasculitis syndrome' rather than 'acute retinal necrosis syndrome' since retinal necrosis is only one of a number of lesions seen in this disease. The etiology of the panvasculitis still remains a mystery.

Acute Disease↗

Expression of the Varicella Zoster Virus Thymidine Kinase and Cytokines in Patients with Acute Retinal Necrosis Syndrome.

Purpose: Acute retinal necrosis (ARN) is caused by varicella zoster virus (VZV) infection. In this study, we investigated the activity of this virus and expressions of some cytokines.Patients and Methods: The expressionof VZV thymidine kinase and some cytokines were investigated by reverse transcriptase-polymerase chain reaction (RT-PCR) in 9 eyes of 8 patients with ARN.Results: Thymidine kinase expression was observed in all samples except one. Several cytokines, such as interferon (IFN) gamma, tumor necrosis factor (TNF)alpha, interleukin (IL)-1 beta, IL-6, and transforming growth factor (TGF)beta 1 were observed in the samples. Among these cytokines, a statistically significant expression of IFNgamma was observed in the samples of ARN, when compared to those of proliferative vitreoretinopathy (PVR) or other uveitis. The expression of IFNgamma also decreased during successive follow-ups.Conclusion: These cytokines may play an important role in the immune response in ARN.

Journal Article↗

Varicella-zoster virus retinitis in a patient with AIDS-related complex: case report and brief review of the acute retinal necrosis syndrome.

Retinitis reminiscent of the acute retinal necrosis syndrome was recognized in a patient with AIDS-related complex after he had experienced several episodes of a sacral, dermatomic zosteriform eruption. Varicella-zoster virus (VZV) was subsequently recovered from cell culture of retinal tissue. The literature on VZV retinitis, including that on acute retinal necrosis, is reviewed. Dissemination of VZV infection in AIDS is also reviewed. Features that differentiate the findings and course of VZV retinitis in patients with AIDS from those in otherwise healthy adults are noted and related to potentially different pathogenic mechanisms. This unusual and recently recognized complication of herpesvirus infection may be promoted by AIDS-related immunosuppression. Acute retinal necrosis and other more-recently described forms of VZV retinitis, which have primarily been subjects of the ophthalmologic literature, merit the attention of clinicians and investigators of infectious diseases.

AIDS-Related Complex↗

Herpes simplex encephalitis and bilateral acute retinal necrosis syndrome after craniotomy.

PURPOSE: Acute retinal necrosis (ARN) syndrome is associated with members of the herpes virus family, but the mechanisms of infection remain unclear. The purpose of this study is to report a unique case of acute retinal necrosis syndrome associated with herpetic encephalitis in order to elucidate possible factors involved in herpetic central nervous system disease. METHOD: Case report. RESULTS: A 64-year-old woman who developed acute herpes simplex virus encephalitis associated with bilateral acute retinal necrosis syndrome after craniotomy for resection of a suprasellar craniopharyngioma is presented. The results of lumbar puncture, magnetic resonance imaging, and ophthalmologic examination are consistent with herpetic infection. The origin of acute retinal necrosis syndrome and the association of acute retinal necrosis syndrome with encephalitis are reviewed. CONCLUSIONS: After craniotomy, we hypothesize reactivation of previously latent herpes simplex virus in the area of the inferior frontal lobe and optic chiasm. Reactivated virus may have migrated to the retina by axonal transport, through the optic nerves, to produce the acute retinal necrosis syndrome.

Acyclovir↗

Optic nerve involvement in the acute retinal necrosis syndrome.

Two patients with acute retinal necrosis syndrome demonstrated enlarged computed tomographic images of the optic nerves, a previously undescribed manifestation of this disorder to the best of our knowledge. One patient, with the bilateral variety of this syndrome, demonstrated bilateral optic nerve enlargement and, in one eye, appeared to achieve visual improvement following an optic nerve sheath decompression. The second patient, with the unilateral variety of the syndrome, demonstrated only unilateral optic nerve enlargement. These observations suggest that the intraorbital optic nerve may be primarily or secondarily involved in the acute retinal necrosis syndrome.

Acute Disease↗

Immunological studies of uveitis. 1. Immune complex containing herpes virus antigens in four patients with acute retinal necrosis syndrome.

Four patients with acute retinal necrosis syndrome were examined. Three of them showed elevated antibody titers to varicella-zoster virus during the convalescent stage, while one of them showed an elevated titer to herpes simplex virus type 1, especially in the intraocular fluid. Also, these patients had an elevated circulating immune complex in the acute phase. Western blotting demonstrated that the immune complexes contained varicella-zoster or herpes simplex type 1 antigen, corresponding to the elevated antibody titer to either virus.

Antigen-Antibody Complex↗

Vitrectomy and silicone oil tamponade as an initial surgery for retinal detachment after acute retinal necrosis syndrome.

PURPOSE: To show the feasibility of vitrectomy and silicone oil tamponade as an initial surgical procedure for retinal detachments with multiple tears that develop after the fulminant type of acute retinal necrosis syndrome. METHODS: In a prospective noncomparative interventional case series, vitrectomy, silicone oil tamponade, and encircling scleral buckling with a solid silicone, combined with phacoemulsification and intraocular lens implantation (except for one pseudophakic patient [Case 2]), were performed in three consecutive patients during the four-year period from 1999 to 2002. These patients had developed retinal detachment with multiple retinal tears after the resolution of acute retinal necrosis syndrome. As an initial standard treatment for acute retinal necrosis syndrome, all patients received maximum-dose intravenous acyclovir (1500 mg daily) for two weeks combined with intravenous prednisolone tapered from 200 mg daily. RESULTS: Case 1 was a 65-year-old woman who developed retinal detachment 44 days after the onset of acute retinal necrosis syndrome; Case 2, a 79-year-old man, developed retinal detachment 51 days after the onset; and Case 3, a 59-year-old man, developed retinal detachment 70 days after the onset. Before surgery, all patients showed multiple tears scattered on the detached atrophic retina extending over the entire midperipheral fundus with vitreoretinal adhesions and vitreous opacity. The retina was reattached during the initial surgery. The patients showed no recurrence of retinal detachment and maintained a visual acuity of 20/200 or 20/100 during the one- to three-year follow-up period. CONCLUSIONS: Vitrectomy and silicone oil tamponade could be a treatment option as the initial surgical procedure for retinal detachments that develop after the fulminant type of acute retinal necrosis syndrome.

Aged↗

A comparative study of the polymerase chain reaction and local antibody production in acute retinal necrosis syndrome and cytomegalovirus retinitis.

BACKGROUND: It has been thought that herpes viruses may play an important role in acute retinal necrosis syndrome (herpes simplex and varicella-zoster viruses) as well as in cytomegalovirus retinitis and it would be useful to know the specificity of the methods for detecting the viruses. METHODS: Amplification of the herpetic viral genome DNA by polymerase chain reaction (PCR) in aqueous and vitreous humor was compared with Goldmann-Witmer coefficients against herpetic antigens in five patients with acute retinal necrosis syndrome and in two patients with cytomegalovirus retinitis, using vitreous samples to determine the specificity of these diagnostic methods. RESULTS: The varicella-zoster virus genome DNA was amplified by PCR in four of the five patients with acute retinal necrosis syndrome, and cytomegalovirus genome DNA was enhanced in both patients with cytomegalovirus retinitis. Four patients who exhibited the varicella-zoster viral genome showed marked increase of the Goldmann-Witmer coefficient against varicella-zoster virus. Conversely, the two patients with cytomegalovirus retinitis showed no remarkable changes among the antigens. CONCLUSION: Our results showed that the amplification of the viral genome DNA in the samples by PCR is specific in both diseases, and that the increased level of local antibody production also is specific in varicella-zoster retinitis. In cytomegalovirus retinitis, however, antibody production against cytomegalovirus does not show increase of the Goldmann-Witmer coefficient.

Aged↗