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Progressive outer retinal necrosis syndrome is a variant of necrotizing herpetic retinopathy, a group of retinal infections caused by the herpes viruses. It has been described only in immunosuppressed patients. We present a healthy immunocompetent 16-year-old male who suffered a bilateral progressive outer retinal necrosis. Varicella-zoster virus infection was confirmed on the basis of serologic study. Treatment with intravenous acyclovir and oral prednisone was successful.

Purpose: To determine the cause of bilateral retrobulbar optic neuritis followed by progressive outer retinal necrosis in a patient with human immunodeficiency virus (HIV)., Methods: Extensive ophthalmologic, neurologic, infectious disease, rheumatologic, and radiologic examinations were performed., Results: Cerebrospinal fluid samples taken after the onset of bilateral retrobulbar optic neuritis and before the development of clinical progressive outer retinal necrosis disclosed varicella-zoster virus from polymerase chain reaction and viral culture., Conclusion: Ophthalmologists and neurologists should consider varicella-zoster virus optic neuritis as a potential precursor of progressive outer retinal necrosis and as a cause of retrobulbar optic neuritis in patients infected with HIV.

Background: Acute retinal necrosis is a rare complication of blunt eye injury. We report about a patient with this type of retinal break., Patient: A 20-year-old male was referred to our hospital after a blunt injury of his left eye due to a ball. Visual acuity was 1.0 and the anterior segment was completely normal. A large retinal necrosis surrounded by a Berlin edema was present in the upper temporal part of the globe. A partial retinal detachment was present. The retina reattached within two weeks without further treatment and a characteristic granular hyperpigmentation developed. The edge of the retinal break was treated by photocaogulation., Conclusion: The affected patient was young and had a solid vitreous, providing internal tamponade of the retina in spite of a large retinal break. In these patients, the clinical course of an acute retinal necrosis after blunt injury should be waited for. However, a retinal detachment that persists after retinal edema disappeared is caused by pathologic vitreous changes and requires surgical treatment.

Purpose: Progressive outer retinal necrosis syndrome is a devastating retinopathy seen primarily in patients with acquired immune deficiency syndrome. To provide additional details of the pathogenesis of this disease, the authors describe the evolution of clinical and fluorescein angiographic changes during the course of progressive outer retinal necrosis syndrome., Methods: The authors performed serial clinical examinations, fundus photography, and fluorescein angiography in a patient with acquired immune deficiency syndrome with progressive outer retinal necrosis syndrome. Clinical and fluorescein angiographic findings were correlated to provide detailed sequential analysis of the pathologic changes occurring during the course of this disorder., Results: The angiographic changes seen during the various stages of the disease consisted of zonal microvascular alterations, retinal pigment epithelium (RPE) destruction, and choroidal leakage. Retinal damage was correlated closely with regions of choroidal leakage and was clinically evident as outer retinal whitening. Disease reactivation occurred as a prominent brush-fire border of intense leakage involving the retina, RPE, and choroid. Extensive damage to the retinal vasculature and RPE was noted in the wake of clinical infection., Conclusions: The angiographic findings in our patient demonstrate that the progressive outer retinal necrosis syndrome is a retinochoroiditis that involves the full thickness of retina as well as the RPE and choroid. The inflammatory changes seen throughout the course of this disease correlate with the histopathologic patterns reported to date.

AIDS causes severe depression of the immune system through selective infection of CD4+ T lymphocytes. As such, certain infections and neoplasms occur in these patients often with ocular involvement. Among these, cytomegalovirus retinitis is by far the most common and remains a leading cause of visual loss. Intravenous ganciclovir and foscarnet, the two Food and Drug Administration-approved treatments for cytomegalovirus retinitis, are effective in most cases, but recurrences are the rule. In addition, these virustatic drugs require lifelong administration and dose-limiting toxicity affects many of these patients. Ongoing investigation into current and alternative therapies has produced some promising developments which are discussed here. Also reviewed are recent findings associated with other opportunistic pathogens and neoplasms that affect the eye in AIDS, including varicella-zoster virus, Toxoplasma gondii, and lymphoma.

Purpose: The authors present a retrospective study of fourteen eyes of eleven patients suffering from acute retinal necrosis syndrome (ARN) and compare the results with previously published data., Material and Methods: All patients were referred to us between 1985 and 1993. The results of initial examination, the epidemiological and aetiologic data, as well as the clinical course and treatment are summarized in two tables., Results: First clinical examination found a prominent ocular inflammatory reaction, several sites of peripheral retinal necrosis and one retinal vasculopathy. Bilateralisation occurred in 28% of the patients. An optic neuropathy developed in 57% of the cases, and a retinal detachment in 78%. Initial examination and data on the clinical course are discussed and compared with those described in the literature., Conclusion: The results of the current study are representative of the various clinical and biological profiles of acute retinal necrosis and are similar to those described in previous publications. Earlier care in specialized departments of ophthalmology should allow a better anatomical and functional prognosis in the future.

A case of bilateral progressive outer retinal necrosis occurred after herpes zoster ophthalmicus in a patient with acquired immunodeficiency syndrome. This case does not correspond to the classical picture of progressive outer retinal necrosis. The disease led to blindness despite intravenous therapy with acyclovir and foscarnet. PCR could not identify any virus in the aqueous humour, but VZV is evidenced in cerebrospinal fluid. Acute retinal necrosis is now clearly defined by the American Uveitis Society, which should allow to determine its incidence and risk factors. Herpes zoster usually precedes the acute outer retinal necrosis. The infectious theory (VZV, HSV, CMV) widely prevails over the immune theory. We prefer the virus genome identification in the aqueous humor or in the vitreous by PCR to confirm diagnosis rather than the specific antibody titration. Therapy consists in acyclovir, foscarnet and ganciclovir. But whatever the treatment, the visual prognosis is poor.

A man aged 43 in good health complaints of sudden blurred vision in his right eye, 12 days after a generalized chickenpox eruption. Examination shows an intraocular inflammation with retinal necrosis in temporal periphery. The serum antibodies against varicella-zoster are positive for the IgM and IgG, confirming a recent infection by varicella zoster. Bacterial serology is negative, as well as the serology for the HSV, HIV and CMV. An intraocular production of anti varicella-zoster antibodies is also found by an anterior chamber puncture (Goldmann-Witmer ratio = 1338). A general treatment by acyclovir and corticoids is started, completed by local treatment and cryocoagulation of the retinal periphery. The evolution is favorable, with recovery of the visual acuity and cicatrization of the lesions. The severity of acute retinal necrosis as a complication of a chickenpox infection usually is moderate, with a good visual prognosis as by our patient.

Acute retinal necrosis is a severe form of necrotizing retinitis. Acute retinal necrosis has been demonstrated to be caused by varicella-zoster virus and herpes simplex virus type 1. We treated three patients with acute retinal necrosis apparently caused by recrudescence of latent herpes simplex virus type 2. Primary viral infection was probably congenital, with documented perinatal herpes simplex virus type 2 infection in two patients. Bilateral chorioretinal scars were present in two patients, neither of whom had a history of ocular herpetic infection, suggesting that earlier subclinical chorioretinitis had occurred. In each case, periocular trauma preceded the development of retinitis by two to three weeks. These cases are evidently caused by trauma-induced reactivation of latent virus rather than the onset of a primary infection.

The progressive outer retinal necrosis (PORN) syndrome is a recently described clinical variant of necrotizing herpetic retinopathy in patients with the acquired immunodeficiency syndrome (AIDS). It is caused by varicellazoster virus infection of the retina. Its course and clinical features distinguish it from the acute retinal necrosis syndrome and CMV retinopathy. Early disease is characterized by multifocal deep retinal opacification. Lesions rapidly coalesce and progress to total retinal necrosis over a short period of time. Despite aggressive therapy with intravenous antivirial drugs, prognosis is poor; disease progression and/or recurrence is common, and the majority of patients develop no light perception vision. Total retinal detachments are common. Prophylaxis against retinal detachment using laser retinopexy has not been useful in most cases. PORN syndrome is an uncommon, but devastating complication of AIDS.

Background: Although acute retinal necrosis (ARN) syndrome is caused by a herpes group virus, association of herpetic keratitis with ARN syndrome is uncommon. A case of unilateral ARN syndrome with herpes simplex keratitis is discussed., Methods: A 40-year-old man developed unilateral keratitis, necrotic retinitis, retinal vasculitis, vitritis, and iritis consistent with ARN syndrome 1 month after treatment for ipsilateral facial nerve palsy and auricular herpetic vesicles (Tolosa-Hunt syndrome). Impression cytologic examination of the corneal epithelial ulcer that developed concurrent with the intraocular findings and of the aqueous humor (obtained by paracentesis) was performed., Results: Cells that reacted with anti-herpes simplex virus type 1 (HSV-1) antibody were detected on impression cytology, and DNA fragments corresponding to the HSV-1 DNA sequence were detected in the aqueous humor., Conclusion: This case shows that ARN syndrome may, on rare occasions, be associated with herpes keratitis secondary to HSV-1.

A 53-year-old man with Wegener's granulomatosis and a co-existing pituitary adenoma developed bilateral acute retinal necrosis (BARN), probably secondary to varicella-zoster virus (VZV) infection as IgM antibodies were detected in the serum. Intravenous acyclovir and ganciclovir limited the spread of necrosis, but to prevent recurrence he was maintained on oral acyclovir. A left cataract developed 17 months later which was extracted and replaced with a heparin surface modified intraocular lens. Intraocular specimens removed at the time of surgery were analysed by the polymerase chain reaction (PCR) using primers specific for a number of the herpes group of viruses, but no herpesviral DNA could be detected.

The case is described of a male patient who had a history of acute retinal necrosis (ARN) OS at age 13 years. The eye subsequently had an inoperable retinal detachment with residual light perception visual acuity. After a 34-year disease-free interval, ARN developed OD that responded to medical treatment. This case represents the longest reported interval of ARN quiescence with eventual bilateral involvement and illustrates the importance of long-term patient follow-up.

We conducted a review to investigate the prevalence of human immunodeficiency virus (HIV), or acquired immunodeficiency syndrome (AIDS), in patients with herpes zoster ophthalmicus, as well as the incidence of acute retinal necrosis after herpes zoster ophthalmicus. All charts of patients seen at our institution between 1987 and 1992 with a primary diagnosis of herpes zoster ophthalmicus were reviewed. Of 112 patients with herpes zoster ophthalmicus, 29 (26%) had HIV or AIDS. All these patients were younger than 50 years at the time of diagnosis. Five of 29 (17%) immunocompromised patients had acute retinal necrosis after herpes zoster ophthalmicus. No acute retinal necrosis was identified in the nonimmunocompromised patients after herpes zoster ophthalmicus. We recommend that all patients younger than 50 years who have herpes zoster ophthalmicus at initial examination be tested for HIV. Additionally, HIV-infected patients should be monitored closely after herpes zoster ophthalmicus for development of acute retinal necrosis. Long-term oral prophylactic as well as initial high-dose intravenous acyclovir may be appropriate in HIV-infected individuals with herpes zoster.

Retinal opacity was experimentally induced by blunt trauma to pigmented rabbit eyes, and the lesions of severe traumatic retinal opacity (experimental concussion necrosis) where leakage of florescence was observed in conventional fluorescein (FLUO) fundus angiography were further examined by indocyanine green (ICG) angiography to study circulatory disturbances. Immediately after the trauma, as in our previous report on the lesion of retinal opacity where no FLUO leakage was observed, there was a delay in choroidal inflow with its peak around the point where choroidal arteries branched off from the ciliary arteries, and ICG leakage due to breakdown of the large choroidal vessels. In addition, regions showing ICG leakage were nearly coincident with those showing FLUO leakage. Observation by light microscope revealed irregular caliber of choriocapillaries (CCs) and marked dilation of the choroidal small vessels connected to the CCs. These findings were consistent with previous reports that ICG leakage occurs where there is choroidal vascular damage. Thus, the lesions are indicative of impaired barrier function of the retinal pigment epithelium and increased CCs permeability. We concluded that concussion necrosis is a combined result of direct physical damage to the outer retina and severe impairment of choroidal circulation.

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.

A healthy man had acute retinal necrosis (ARN) two weeks after diffuse varicella eruption. Treatment with acyclovir and corticosteroids was associated with a favorable clinical outcome. To our knowledge, there are no reported occurrences of permanent visual loss or retinal detachment in healthy patients with postvaricella ARN.

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.

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.

The acute retinal necrosis (ARN) syndrome represents a specific pattern of clinical presentation for certain herpes virus infections in the posterior segment of the eye. The classically described triad of the ARN syndrome consists of (1) an arteritis and phlebitis of the retinal and choroidal vasculature, (2) a confluent, necrotizing retinitis that preferentially affects the peripheral retina, and (3) a moderate to severe vitritis. Anterior segment inflammation, optic neuritis, and late retinal detachment are also common features of this disorder. Definitive evidence now implicates at least two members of the herpes virus family; varicella zoster virus and herpes simplex virus as causative agents. This paper summarizes the clinical presentation, as well as the currently recommended treatment regimen for the ARN syndrome, highlighting recent advances that have resulted in a significant improvement in the visual prognosis for affected patients.

A 76-year-old woman with unilateral acute retinal necrosis had an elevated titer of cytomegalovirus (CMV) antibodies with both in the aqueous humor and serum. The serological pattern indicated recent CMV infection, and the Goldmann-Witmer coefficient comparing aqueous and serum titers was above 4 indicating intraocular production of specific antibodies. The clinical characteristics and results as well as a new possible etiopathogenic hypothesis are presented.

The authors report a case of unusual toxoplasma chorioretinitis presenting a branch retinal occlusion of an artery passing through the acute necrotic focus. This complications has been very rarely described previously and may have been caused by the acute retinal necrosis. Arterial inflammatory phenomena constitute another etiologic hypothesis. As this branch vascular obstruction may change the appearance of the fundus and obscure the initial inflammatory focus, ocular toxoplasmosis should be included in the differential diagnosis of branch retinal vascular occlusion associated with posterior uveitis, particularly in young patients.

A 35-year-old patient with systemic lupus erythematodes is presented. Two weeks after initiation of an immunosuppressive therapy with steroids and cyclophosphamide she developed a bilateral acute necrotising retinitis. Although the infiltration of the vitreous body was only slight the right eye developed a traction retinal detachment within a few days. A vitrectomy was performed but the situation was found to be inoperable due to central necrotic areas. Two days later serological specimen revealed an acute toxoplasmosis as the cause of the bilateral retinitis in this patient. A therapy with sulfadiazine and pyrimethamine was started immediately. Nevertheless two weeks later traction retinal detachment occurred also in the left eye. A reattachment of the retina could be achieved by vitreoretinal surgery.