Progressive Outer Retinal Necrosis Syndrome Research Articles

Progressive outer retinal necrosis syndrome in the course of systemic lupus erythematosus

Progressive outer retinal necrosis syndrome (PORN) is a severe clinical variant of necrotizing herpetic chorioretinitis, which occurs almost exclusively in patients with advanced acquired immunodeficiency syndrome (AIDS). To date, only a few cases of PORN have been reported in patients, mostly among those who were immunocompromised. To our knowledge, only one case of PORN in a patient with systemic lupus erythematosus (SLE) has been described. We report the case of a 44-year old HIV-negative patient with lupus nephritis, whom was being treated by mycophenolate mophetil (MMF), arechin and prednisone. After 14 months of MMF therapy, the patient revealed PORN symptoms; and several months later, the patient developed Type B primary central nervous system lymphoma (PCNSL). PORN is usually compared to acute retinal necrosis (ARN) syndrome, because of having the same causative agent: varicella zoster virus (VZV). There are also some similarities in clinical findings. Our observation supports the hypothesis that PORN symptoms in HIV-negative patients can be an intermediate form between ARN and PORN, and can vary according to the patient's immune status.

PCR in posterior segment viral diseases

AbstractSince its introduction in the early eighties, PCR has dramatically changed our approach to the diagnosis and understanding of intraocular viral diseases. Even though serology remains the effective diagnostic tool for diseases such as West‐Nile virus or dengue‐virus retinitis, PCR is the gold‐standard for herpetic retinopathies. Two major subtypes have been described including necrotizing and nonnecrotizing herpetic retinopathies. In Europe, diagnosis is performed based on the analysis of the aqueous humor, whereas vitrectomy is more frequently performed in the USA. Less than 100 microlitters of liquid is sufficient for the procedure. Real‐time PCR is now available for most of the herpesviruses allowing a quantitative determination of the viral load. The identification of the viral agent is paramount in the management of the disease by adapting the antiviral strategy. VZV, HSV‐1, HSV‐2 and CMV are the main causes of viral retinitis. Acute retinal necrosis syndrome is mainly observed in immunocompetent patients whereas, progressive outer retinal necrosis syndrome and CMV retinitis occur classically in immunocompromised patients. Intravitreal injection of corticosteroids must be considered as a risk factor. The decrease of the viral load is a major prognostic factor and must be taken into consideration for an optimal management.

Progressive outer retinal necrosis syndrome in a lymphoma patient with good visual outcome

PURPOSE: To report an HIV-negative lymphoma patient who developed progressive outer retinal necrosis syndrome and who had a good visual outcome after treatment with two-drug antiviral therapy and intravenous immunoglobulin. METHODS: Case report. RESULTS: A 43-year-old man with small lymphocytic lymphoma was diagnosed with progressive outer retinal necrosis in his left eye. Treatment was initiated with intravenous foscarnet and ganciclovir as well as intravenous gammaglobulin at a dose of 0.5 gm/kg per day for 5 days. On the second hospital day he was started on decadron 4 mg orally four times daily. No further posterior retinitis progression was observed despite severe immunosuppression. Visual acuity remained stable at 20/30 with 10 months’ follow-up. CONCLUSIONS: The benefit of using gammaglobulin in progressive outer retinal necrosis is unknown. Given the rapid improvement seen in this patient’s retinitis, it may be reasonable to consider the use of gammaglobulin in other cases of infectious retinitis in immunocompromised patients.

Progressive Outer Retinal Necrosis in a Patient with Nephrotic Syndrome

Progressive outer retinal necrosis syndrome (PORN) is a variant of necrotizing herpetic retinopathy and the majority of the described cases were related to acquired immunodeficiency syndrome. We present a patient who is HIV negative with nephrotic syndrome and prednisolone use for 4 months who showed clinical features of PORN. Low CD4 counts and lymphocytopenia suggested immunosuppression. In the left eye, tractional retinal detachment at the posterior pole followed by incomplete posterior vitreous detachment developed. In addition to intravenous administration of acyclovir, vitreous surgeries including stripping of the posterior hyaloid and silicone-oil tamponade were successfully performed to repair the retinal detachment in the left eye and to prevent it in the right eye.

Progressive outer retinal necrosis syndrome: a comprehensive review of its clinical presentation, relationship to immune system status, and management

Progressive outer retinal necrosis (PORN) syndrome is a form of the Varicella zoster virus (VZV) chorioretinitis found almost exclusively in people with the acquired immunodeficiency syndrome (AIDS). This destructive infection has an extremely rapid course that may lead to no light perception in affected eyes within days or weeks. Attempts at its treatment have had limited success. Rhegmatogenous retinal detachments often occur after the development of atrophic retinal holes, and silicone oil temponade has been found to be the most successful reattachment procedure. Unfortunately, cataract formation is common after such surgery. PORN needs to be differentiated from acute retinal necrosis (ARN) syndrome, a necrotizing retinitis that can also be caused by VZV. PORN and ARN are found at opposite ends of the spectrum of necrotizing herpetic retinopathies (NHR), where its clinical presentation depends upon immune system status. After a brief case presentation, the distinguishing clinical characteristics of PORN, its differentiation from ARN, attempts at its treatment, the role of the immune system status on its clinical appearance and treatment, and management of complications such as retinal detachment and subsequent cataracts are discussed.

The Progressive Outer Retinal Necrosis Syndrome: Successful Treatment With Combination Antiviral Therapy

To assess a two-drug combination of antiviral therapy for the progressive outer retinal necrosis syndrome (PORN), given the current poor outcome with acyclovir alone. A retrospective review was performed on six consecutive patients who were diagnosed with PORN and were treated with various combinations of intravenous or oral plus intravenous antiviral therapy. The relative efficacies of these modalities were compared. Six eyes of six patients showed active retinitis at the time of presentation. Three patients had unilateral retinitis, and the remaining patients had necrotic, end-stage disease in their fellow eye. All the patients were treated with combination therapy, consisting of either ganciclovir and acyclovir (three patients), foscarnet and ganciclovir (two patients), or foscarnet and acyclovir (one patient). Standard induction doses were employed. During the combination therapy, all six eyes showed resolution of the retinitis, manifested by complete fading of the original retinal lesions and an absence of new lesion formation. At the final follow-up, the areas of prior active retinitis had resolved and remained quiescent. A mild recurrence developed in one eye when ganciclovir and foscarnet were both tapered to a single daily dose. This recurrence promptly resolved with reinduction (twice daily) dosing. Two patients maintained a visual acuity of 20/50 or better in their involved eye for the duration of follow-up (38 and 27 weeks, respectively). One patient maintained a visual acuity of 20/40 for 14 weeks. The remaining three patients had macula-off retinal detachments despite resolution of active retinitis. In addition, for the duration of follow-up, one of the three patients with unilateral disease had retinitis in the uninvolved eye; all three uninvolved fellow eyes maintained a visual acuity of 20/20. One patient had progressive optic atrophy. Prolonged combination antiviral therapy for PORN may successfully arrest the progression of retinitis, maintain remission, and prevent involvement of the fellow eye. Furthermore, if aggressive therapy is begun early, good vision may be preserved.

Herpes zoster ophthalmicus in patients with human immunodeficiency virus infection

To investigate the ocular complications of herpes zoster ophthalmicus in patients with human immunodeficiency virus (HIV) infection. This was a retrospective cohort study of 48 HIV-infected patients (48 eyes) treated at San Francisco General Hospital for herpes zoster ophthalmicus from December 1985 through March 1994. All patients were initially treated with either intravenous or oral acyclovir. The median CD4 lymphocyte count at diagnosis was 48 per mm3 (range, 2 to 490 per mm3). Fifteen patients (31%) had mild or no ocular involvement. Seventeen patients (35%) had stromal keratitis, mostly mild, and two (4)% developed chronic infectious pseudodendritic keratitis. Twenty-four study patients (50%) had iritis, but only three (6%) had elevations in intraocular pressure. Two patients (4%) developed postherpetic neuralgia, and two others (4%) had zoster-associated central nervous system disease. Only two patients (4%) developed necrotizing retinitis, both in the form of the progressive outer retinal necrosis syndrome. Excluding the patients with retinitis and central nervous system disease, the rate of sight-threatening complications in our series was lower than expected. Almost one third of study patients had no ocular complications or only mild surface epithelial disease. Although the relatively low incidence of sight-threatening disease in our study population may have been a consequence of aggressive management with acyclovir, chronic infectious pseudodendritic keratitis, retinitis, and central nervous system disease, complications of ophthalmic zoster whose pathogenesis is largely a consequence of active viral replication, were particularly devastating and difficult to manage.

A Polymerase Chain Reaction-based Assay for Diagnosing Varicella-zoster Virus Retinitis in Patients With Acquired Immunodeficiency Syndrome

To develop a rapid, sensitive, and specific laboratory assay based on the polymerase chain reaction for the diagnosis of varicella-zoster virus retinitis in patients with acquired immunodeficiency syndrome (AIDS). We developed and tested a polymerase chain reaction-based assay for the detection of varicella-zoster virus DNA in vitreous samples. We attempted to detect varicella-zoster virus DNA in 14 vitreous samples from patients with AIDS and a clinical diagnosis of progressive outer retinal necrosis syndrome. For controls, we also attempted to detect varicella-zoster virus DNA in vitreous samples from 75 immunocompetent patients with vitreoretinal disease and 88 patients with AIDS and vitreoretinal inflammatory disease not related to progressive outer retinal necrosis syndrome. Varicella-zoster virus DNA was detected in 11 of 14 vitreous samples from AIDS patients with progressive outer retinal necrosis syndrome. All three samples that scored negative for varicella-zoster virus DNA came from eyes that had been treated aggressively with antiviral drugs and had clinically inactive disease at the time of vitreous biopsy. Varicella-zoster virus DNA was detected in only two of 75 control vitreous samples from immunocompetent patients with vitreoretinal disease and two of 88 control vitreous samples from patients with AIDS and vitreoretinal inflammatory disease not related to progressive outer retinal necrosis syndrome. We have developed a rapid, sensitive, and specific polymerase chain reaction-based diagnostic assay for varicella-zoster virus DNA that will assist in the diagnosis of varicella-zoster virus retinitis in patients with AIDS.

Opportunistic infections of the eye in immunocompromised patients.

All opportunistic infections of the eye have their origin in the suppression of the immune system of the host. The immunosuppression can be acquired through the human immune deficiency virus or as a result of immunosuppressive therapy in solid-organ transplant recipients with a maintenance therapy of cyclosporin and prednisone or during chemotherapy in patients with myeloproliferative disorders. Generally, in less than 2% could severe opportunistic infections of the eye, like cytomegalovirus (CMV) or herpes virus infection, be found in transplant and cancer patients with chemotherapy. About 35-70% of patients with AIDS exhibit ocular manifestations of disease, so the most notable opportunistic infection with an incidence of 35% would be CMV retinitis. Less frequent are progressive outer retinal necrosis syndrome, Kaposi's sarcoma and optic neuropathies. This paper reviews recent advances in the diagnosis and special local therapies of the worst opportunistic infections in immunocompromised hosts.

Progressive Outer Retinal Necrosis After Bone Marrow Transplantation

To describe a case of progressive outer retinal necrosis in a human immunodeficiency virus-negative immunosuppressed patient undergoing treatment for graft-versus-host disease after bone marrow transplantation. Onset, course, and outcome of the patient's eye disease were analyzed. Findings and course were typical for the recently described progressive outer retinal necrosis syndrome. Progressive outer retinal necrosis is not limited to the acquired immunodeficiency syndrome but may occur in patients immunocompromised because of other conditions.

Spontaneous Regression and Successful Laser Prophylaxis in Progressive Outer Retinal Necrosis Syndrome

To describe a case of progressive outer retinal necrosis syndrome that regressed spontaneously after discontinuation of all systemic antiviral medications with macular sparing after prophylactic laser photocoagulation of the posterior pole. Prophylactic laser photocoagulation surrounding the posterior pole was performed in an attempt to spare the macula from detachment in a patient with progressive outer retinal necrosis. Progressive outer retinal necrosis regressed spontaneously without systemic antiviral medications. The posterior pole within the area of photocoagulation remained attached. Prophylactic laser treatment surrounding the posterior pole rather than peripheral retina may allow more time for formation of firm chorioretinal adhesions and protect the macula from detachment.

Ocular manifestations of HIV infection

To evaluate the frequency of ocular complications and the clinical outcomes of these complications in patients with various stages of HIV infection.Retrospective review of all HIV-infected patients seen in an AIDS ophthalmology clinic from November 1983 through December 31, 1992.Eleven-hundred sixty-three patients were seen for ophthalmologic evaluation. Of these, 781 had the acquired immune deficiency syndrome (AIDS), 226 had symptomatic HIV infection (AIDs-related complex [ARC]), and 156 had asymptomatic HIV infection. Non-infectious HIV retinopathy was the most common ocular complication, affecting 50% of the patients with AIDS, 34% of the patients with ARC, and 3% of the patients with asymptomatic HIV infection. Cytomegalovirus (CMV) retinitis was the most common opportunistic ocular infection, affecting 37% of the patients with AIDS. Other opportunistic ocular infections, including ocular toxoplasmosis, varicella zoster virus retinitis, and Pneumocystis choroidopathy were all much less common, each occurring in < or = 1% of the patients with AIDS. Treatment of CMV retinitis with either foscarnet or ganciclovir was successful in initially controlling the retinitis. However, relapse represented a significant problem and required frequent re-inductions. As a consequence of the retinal damage associated with relapse, loss of visual acuity occurred. The median time to a visual acuity of 20/200 or worse for all eyes with CMV retinitis was 13.4 months, and the median time to a visual acuity of 20/200 or worse in the better eye was 21.1 months. At last follow-up, 75% of the patients had a final visual acuity of 20/40 or better in at least one eye. Retinal detachments were a frequent ophthalmologic complication of CMV retinitis with a cumulative probability of a retinal detachment in at least one eye of 57% at 12 months after the diagnosis of CMV retinitis. Herpes zoster ophthalmicus developed in 3% of the overall series and was seen in all stages of HIV infection. Fifty-six percent of the cases of ocular toxoplasmosis had simultaneous toxoplasmic cerebritis. Ocular toxoplasmosis responded to standard anti-microbial therapy. Varicella zoster virus retinitis, when manifested by the acute retinal necrosis (ARN) syndrome, responded to intravenous acyclovir therapy. Conversely, in a limited number of patients with the progressive outer retinal necrosis syndrome, the disease responded poorly to intravenous acyclovir therapy, but appeared to respond to combination foscarnet and acyclovir therapy. Neuro-ophthalmic lesions were present in 6% of the patients with AIDS. The most common cause of a neuro-ophthalmic lesion was cryptococcal meningitis, and 25% of the patients with cryptococcal meningitis developed a neuro-ophthalmic complication.Ocular manifestations are common in patients with AIDS. CMV retinitis represented a major vision-threatening problem in these patients. While available therapy was successful in initially controlling the retinitis, the phenomenon of relapse resulted in some degree of long-term visual loss. Preservation of the patient's visual acuity in at least one eye was generally successful. Other opportunistic ocular infections were substantially less common than CMV retinitis but require aggressive therapy.

The Progressive Outer Retinal Necrosis Syndrome: A Variant of Necrotizing Herpetic Retinopathy in Patients with AIDS

The progressive outer retinal necrosis syndrome is a recently recognized variant of necrotizing herpetic retinopathy. This report characterizes more fully its clinical features and course. Using standardized clinical criteria, patients with progressive outer retinal necrosis syndrome from four institutions were identified. Patient records were reviewed retrospectively for the following data: medical and demographic characteristics, presenting symptoms, physical findings, course, responses to treatment, and outcomes. Thirty-eight patients (65 involved eyes) were studied. All had acquired immune deficiency syndrome. A known history of cutaneous zoster was documented in 22 (67%) of 33 patients. Median CD4 lymphocyte count was 21/mm3 (range, 0-130/mm3). Median follow-up was 12 weeks. The most common presenting symptom was unilateral decreased vision (35 of 65 eyes, 54%); median visual acuity at presentation was 20/30 (range, 20/20 to no light perception [NLP]). Anterior chamber and vitreous inflammatory reactions were absent or minimal in all patients. Typical retinal lesions were multifocal, deep opacities scattered throughout the periphery, although macular lesions also were present in 21 eyes (32%) at diagnosis. Lesions progressed rapidly to confluence. Initial intravenous antiviral therapy appeared to reduce disease activity in 17 (53%) of 32 eyes, but treatment did not alter final visual outcome. Visual acuity was NLP in 42 (67%) of 63 eyes within 4 weeks after diagnosis. Retinal detachment occurred in 43 (70%) of 61 eyes, including 13 (93%) of 14 eyes that received prophylactic laser retinopexy. The progressive outer retinal necrosis syndrome is characterized by features that distinguish it from cytomegalovirus retinopathy, acute retinal necrosis syndrome, and other necrotizing herpetic retinopathies. Visual prognosis is poor with current therapies.

The progressive outer retinal necrosis syndrome.

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.