Atypical Peripheral Retinal Necrosis Without Ocular Inflammation and Retinal Whitening in a Patient With Acute Myelogenous Leukemia: A Case Report.

Retinal necrosis is a severe condition that threatens visual function. It is caused by viruses that are known to cause acute retinal necrosis (ARN) and progressive outer retinal necrosis (PORN), which are called necrotizing herpetic retinopathies (NHR). ARN causes severe intraocular inflammation, including anterior chamber intravitreal cells, keratic precipitate, vitreous opacity, and retinal vasculitis, whereas intraocular inflammation in PORN is considered mild or virtually absent. In addition, PORN is a disease that manifests in immunosuppressive patients, such as those with acquired immunodeficiency syndrome. Here, we present a case of unilateral retinal necrosis after chemotherapy, allogeneic peripheral blood stem cell transplantation, and cord blood transplantation for acute myelogenous leukemia (AML) in a 31-year-old male patient. AML treatment resulted in metabolic remission, and oral steroids and tacrolimus were continued. After two days, the patient visited an ophthalmologist because he noticed a sudden onset of floaters and visual field disturbance in the left eye. The peripheral retina was already necrotic in all layers, causing total retinal detachment. Intraocular inflammation, retinal opacity, or hemorrhagic spots in the fundus were not observed. His previous CD4 count was 43 cells/µL. A polymerase chain reaction test of the anterior chamber fluid revealed varicella-zoster virus (VZV), and vitrectomy was performed four days after disease onset. The excised vitreous demonstrated minimal opacity. The peripheral necrotic retina was excised, photocoagulation was performed on the residual retinal limbus, and silicone oil was injected to maintain retinal attachment. The retinal restoration was maintained under silicone oil tamponade, and corrected visual acuity improved to 20/32 without strong inflammation after vitrectomy. However, two months postoperatively, he contracted coronavirus disease 2019 (COVID-19), his general condition rapidly deteriorated, and he died. This case of retinal necrosis without inflammatory results in an immunocompromised patient and VZV detection in an intraocular sample led us to suspect PORN. However, the patchy or spread retinal whitening characteristic of PORN was completely absent, whereas the well-defined, peripheral, full-layer retinal necrosis characteristic of ARN was present. Thus, this is a rare case of VZV-induced NHR with partial features of PORN and ARN that progressed very silently.

A Case of Birdshot Chorioretinopathy following Varicella Zoster Virus-Induced Progressive Retinal Necrosis in the Contralateral Eye.

Birdshot chorioretinopathy is an immune-mediated ocular inflammatory disease of the retina. We report a case of birdshot chorioretinopathy in one eye following progressive retinal necrosis caused by varicella zoster virus (VZV) in the contralateral eye. A 79-year-old female patient complained of decreased vision due to anterior chamber inflammation and vitreous opacity in the left eye starting 10 days earlier. Acute retinal necrosis was suspected and polymerase chain reaction analysis was performed using the anterior chamber aqueous. VZV DNA was detected, and antiviral drugs and steroids were systemically administered together with local therapy. As retinal necrotic changes and retinal detachment gradually developed, vitrectomy and silicone oil tamponade were performed. Despite these procedures, retinal necrosis gradually progressed. Eventually, extensive retinochoroidal atrophy resembling progressive outer retinal necrosis was observed. Two years and 4 months after onset, anterior chamber inflammation and white spots of the retina started to appear in the right eye. The number of white spots gradually increased all around the retina, regardless of systemic treatment with acyclovir and prednisolone. The fundus findings seemed consistent with birdshot chorioretinopathy. Diseases exhibiting white dots in the retina, such as sarcoidosis, were excluded. Ss-OCT examination revealed destruction of the layer structure mainly in the outer layers of the retina and a section of a white dot revealed that the retina appeared to be pulled into the RPE. To the best of our knowledge, this is the first report of birdshot chorioretinopathy in one eye after the onset of VZV-induced retinal necrosis in the contralateral eye.

Case series: Two cases of herpetic retinitis presenting as progressive outer retinal necrosis in immunocompetent individuals.

PURPOSE: To raise awareness of ophthalmologists that Varicella Zoster Virus (VZV) retinitis should be considered in the differential diagnosis of retinitis that presents with features of progressive outer retinal necrosis (PORN) in healthy immunocompetent patients. OBSERVATIONS: Case 1 is a 39-year-old healthy Caucasian male who presented after one week of decreased vision in the left eye. Patient was found to have optic disc edema and multifocal retinitis primarily localized to the posterior pole with only a few lesions in the periphery and minimal vitritis. Viral PCR of a vitreous tap was positive for 1 million copies of VZV. Patient ultimately progressed to no light perception vision despite multiple intravitreal injections of foscarnet and several days of intravenous acyclovir therapy. Case 2 is another 39-year-old healthy Caucasian male that presented after 2 weeks of decreased vision in the left eye. On initial exam by his primary ophthalmologist, patient was found to have optic disc edema and multifocal retinitis primarily localized to the posterior pole with no peripheral lesions and no vitritis. The patient then presented to our clinic with extensive retinitis throughout the posterior pole and periphery, and he underwent a vitreous tap with viral PCR positive for 3160 copies of VZV. He was treated with intravitreal injections of foscarnet and intravenous acyclovir therapy with subtle progression to the right eye and only minimal improvement of left eye vision to 20/200. CONCLUSIONS AND IMPORTANCE: Progressive outer retinal necrosis (PORN) is a herpetic retinopathy characterized by rapidly progressive necrosis of the outer retina in severely immunocompromised subjects. As demonstrated in this case series, VZV retinitis should be considered as a differential diagnosis in patients with hallmark features of PORN, even in the absence of obvious systemic immunosuppression.

Varicella zoster virus (VZV) oculomeningoencephalomyeloradiculitis: a previously undescribed constellation.

A 53-year-old immunocompromised woman developed acute left eye blindness and paraparesis suspected to be due to neuromyelitis optica (NMO). During treatment for NMO, right eye blindness and progressive multiple cranial neuropathies developed. Cerebrospinal fluid polymerase chain reaction (PCR) revealed Varicella zoster virus (VZV). This case emphasizes the importance of considering VZV in individuals, particularly the immunocompromised, presenting with a constellation of neurological signs and symptoms, even in the absence of rash.

Surgical management of complications in a case of progressive outer retinal necrosis.

We report a case of bilateral progressive outer retinal necrosis (PORN) in a patient with acquired immune deficiency syndrome with CD4 count 50 cells/µL. He was treated with standard intravenous and intravitreal antivirals but ultimately developed complications such as retinal detachment and epiretinal membrane. His vision was preserved with early pars plana vitrectomy. This case demonstrates that prompt clinical diagnosis of PORN with its successful medical and surgical management can help prevent progression of this frightening disease.

Atypical posterior pole retinitis.

PURPOSE: Necrotizing retinopathy is an uncommon, devastating, potentially blinding condition, which can be seen in both immunocompetent and immunocompromised patients. The purpose of this case is to report a new subset of necrotizing viral retinopathy. OBSERVATION: A 34-year old lady presented to our outpatient department with sudden diminution of vision in both eyes following a brief history of viral fever with vesicular rashes. Fundus examination showed areas of necrotizing retinitis in posterior pole involving macula with scattered superficial retinal hemorrhages, cotton-wool spots in both the eyes. She was treated with intravenous acyclovir and oral valacyclovir. CONCLUSION AND IMPORTANCE: Our case may represent a new subset of necrotizing viral retinopathy, which may intermediate the clinical pictures of acute retinal necrosis and progressive outer retinal necrosis.

Viral retinitis: diagnosis and management in the era of biologic immunosuppression: A review.

Necrotising retinitis is a rare ocular infection that historically led to high rates of visual morbidity. While acute retinal necrosis occurs in immunocompetent patients, the majority of cases are associated with immunocompromise such as in cytomegalovirus retinitis and progressive outer retinal necrosis. This review summarises the clinical and diagnostic features, management, and outcomes of herpetic retinitis. Iatrogenic immunosuppression is increasingly being utilised for a wide range of indications, and biologic agents especially so due to their targeted nature. While the intended actions are well-studied, the flow-on effects and complex interaction with host immunity are not well understood. Furthermore, biologics are frequently used concomitantly with other immunosuppressive agents, potentiating the immunodepression. This article reviews the literature on biologic immunosuppression and viral retinitis, and presents an approach to the vulnerable or affected patient. Early identification, prompt and aggressive treatment, and a multidisciplinary approach to managing immunodeficiency are the cornerstones of management.

Progressive outer retinal necrosis after rituximab and cyclophosphamide therapy.

We report a case of progressive outer retinal necrosis (PORN) in a patient of microscopic polyangitis (MPA), being treated with immunosuppressive drugs such as cyclophosphamide and rituximab. Her aqueous tap was positive for Varicella Zoster virus and she was treated with oral and intravitreal antivirals, along with discontinuation of one of the immunosuppressive agents, i.e. rituximab, which might have led to reactivation of the virus causing necrotizing retinitis lesions. Rituximab and cyclophosphamide are extremely potent drugs, which are necessary to manage immunological disorders such as MPA. However, they may predispose the patient to serious complications like viral infections, including PORN.

Atypical VZV Retinitis in a Patient with Good Syndrome.

PURPOSE: To describe atypical varicella zoster virus (VZV) retinitis in a patient with Good syndrome. METHODS: A 63-year-old patient with Good syndrome presented with bilateral necrotizing retinitis starting from the posterior pole. He had a history of thymoma status post thymectomy 4 years previously, left-sided sinusitis, and recent pulmonary aspergillosis. Qualitative PCR was performed on aqueous fluid. RESULTS: Immunological investigations revealed reduced levels of CD4+ T cells and immunoglobulins. Qualitative PCR was positive for VZV and negative for cytomegalovirus, herpes simplex virus (HSV)-1, and HSV-2. The patient was treated with oral valacyclovir and three courses of immunoglobulin supplementation. The atypical retinitis showed improvement after therapy. CONCLUSION: Good syndrome should be considered in a patient with opportunistic infections and history of thymoma in the absence of human immunodeficiency virus. Atypical retinitis can occur in patients with Good syndrome and quantitative PCR is important for accurate diagnosis.

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.

Varicella Zoster Virus Necrotizing Retinitis in Two Patients with Idiopathic CD4 Lymphocytopenia.

PURPOSE: Progressive outer retinal necrosis (PORN) associated with varicella zoster virus (VZV) is usually diagnosed in HIV positive or immunosuppressed patients. We report two cases of immunocompetent patients with necrotizing viral retinitis found to have idiopathic CD4 lymphocytopenia. METHODS: Clinical presentation, examination, imaging, and laboratory testing of two patients with VZV retinitis are presented. RESULTS: An HIV negative patient with history of herpes zoster presented with rapid loss of vision and examination consistent with PORN. PCR testing confirmed VZV. Lymphocytopenia was noted with a CD4 count of 25/mm(3). A second HIV negative patient presented with blurred vision and lid swelling and was found to have peripheral VZV retinitis confirmed by PCR. Laboratory workup revealed lymphocytopenia with a CD4 count of 133/mm(3). CONCLUSIONS: VZV necrotizing retinitis classic for PORN can occur in HIV negative patients. Idiopathic CD4 lymphocytopenia should be considered healthy patients who develop ocular infections seen in the immunocompromised.

Progressive outer retinal necrosis associated with occlusive vasculitis in acquired immunodeficiency syndrome.

A 45-year-old man, a case of acquired immunodeficiency syndrome, received a highly active antiretroviral therapy at the outpatient service for 4 years without regular follow-up. He experienced progressively blurred vision for 6 months and a cutaneous zoster on his back 3 months ago. He was diagnosed with progressive outer retinal necrosis by polymerase chain reaction-restriction fragment length polymorphism using an aqueous humor sample, which revealed an existence of varicella zoster virus. He was given a combination of systemic, intravitreal antiviral and a highly active antiretroviral therapy. Occlusive vasculitis, an unusual finding for progressive outer retinal necrosis, developed in both eyes 1 week after the secondary intravitreal injection. Unfortunately, his vision deteriorated to no light perception in both eyes within 2 weeks. Progressive outer retinal necrosis is characterized clinically as showing minimal or no inflammation in the aqueous and vitreous humors, absence of retinal vasculitis, and patches of yellowish spots located deep in the retina. Physicians should pay attention to this rare case of progressive outer retinal necrosis associated occlusive vasculitis with very poor prognosis in spite of aggressive treatment.

Progressive outer retinal necrosis in a renal transplant recipient: a rare treatment success.

Renal transplant recipients (RTRs) are subject to a variety of opportunistic infections. We present a rare case of varicella zoster virus-derived progressive outer retinal necrosis in an RTR, who presented with painless visual blurring. This clinical entity heralds an extremely poor visual prognosis and is an important condition to consider in any immunocompromised host. Early diagnosis by aqueous fluid sampling and immediate institution of combined systemic and intravitreal antiviral therapy was successful in this individual.

Progressive outer retinal necrosis in immunocompromised kidney allograft recipient.

Ocular complications in patients who underwent renal transplantation are attributed to side effects of the immunosuppressive regimen. Progressive outer retinal necrosis (PORN) syndrome is a clinical variant of necrotizing herpetic retinopathy and it occurs almost exclusively in patients with acquired immunodeficiency syndrome. We present a case of a human immunodeficiency virus-negative patient who underwent renal transplant and, after a few years, developed bilateral PORN associated with viral infections. Varicella zoster virus (VZV) and BK virus were identified by polymerase chain reaction from the vitreous fluid. It is unclear which of the viruses identified had the dominant role in the pathogenesis of PORN and other organ damage, or whether their actions were synergistic. Adequate antiviral immune surveillance, as well as pre-transplant vaccination against VZV, may reduce the incidence of VZV infection and its complications.

Necrosis retinal aguda vs. necrosis retinal externa progresiva: reporte de caso y revisión de la literatura / Acute retinal necrosis vs. progressive outer retinal necrosis: case report and literature review

Se describen las características principales de la necrosis retinal aguda, la relación fisiopatológica con su principal diagnóstico diferencial, la necrosis retinal externa progresiva. Exponemos las diferencias clínicas y paraclínicas de ambas enfermedades, con base en las cuales construimos un sistema de clasificación que las contempla como un espectro continuo de enfermedad dependiente del estado inmunológico.

The main characteristics of acute retinal necrosis, the pathophysiological connection with the main differential diagnosis and progressive outer retinal necrosis are described. We analize the clinical and paraclinical differences of both diseases, based on which we built a classifi cation system that take them into a continuous spectrum dependent of the immune state.

A systematic approach to emergencies in uveitis.

Uveitis is a common cause of preventable blindness although it is consider a sight-threatening condition particularly in cases with posterior segment inflammation. To deal with emergency conditions in uveitis, we must aware of the essential signs and symptoms that reflect a true uveitic emergency. Failure to recognize these essential signs and symptoms of a true uveitic emergency may result in a devastating visual outcome. This review provides general ophthalmologists and residents, clinical guidelines for the main uveitic entities that require immediate recognition and urgent intervention in the emergency room to prevent severe permanent visual loss.

Progressive outer retinal necrosis and immunosuppressive therapy in myasthenia gravis.

INTRODUCTION: Progressive outer retinal necrosis (PORN) is a rare but devastating infectious retinitis associated with varicella zoster virus (VZV) and responsible for severe visual loss. CASE REPORT: A 59-year-old man treated for generalized myasthenia with oral azathioprine and prednisone presented with severe unilateral necrotizing retinitis. Polymerase chain reaction of the aqueous and vitreous humors was diagnostic for VZV PORN. CONCLUSION: VZV PORN is a severe potential ocular complication of immunosuppression, prompting urgent diagnosis and appropriate treatment.