Search

Introduction: Other than well-known herpetic retinopathies like acute retinal necrosis, progressive outer retinal necrosis and cytomegalovirus retinitis, there are few reports on atypical forms of viral retinitis caused by herpes virus from around the world. Cases: Presenting symptom was sudden onset of diminution of vision in all 6 cases. Mean duration of symptoms at presentation was 7.6 days. The mean age was 27.3 years (range 22-40 years). All were immunocompetent (4 females and 2 males), with unremarkable review of system. All had unilateral involvement. Presenting visual acuity ranged from CFCF to 6/60. Granulomatous anterior uveitis was present in 4 cases. All had focal retinitis with irregular margins, either juxtapapillary, macular or over one of the major vascular arcades. The diagnosis of focal viral retinitis was made solely based upon the clinical findings. All recovered after a course of oral acyclovir (one was given additional intravitreal acyclovir as well) and oral prednisolone, with final visual acuity of 6/6P to 6/9 in all except one. Conclusion: In case of focal retinitis with irregular margin, viral etiology should be borne in mind. Clinical features are typical enough to help in diagnosing without PCR test.

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.

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.

A 10-year-old boy underwent stem cell transplant for Hodgkin’s lymphoma and developed vomiting and seizure in the postoperative period. An ophthalmic referral was made from intensive care unit, to rule out papilledema. On examination, there was no papilledema in both eyes, instead there were areas of retinal necrosis with no haemorrhages or vitritis in right eye. Cerebrospinal fluid serology was negative for herpes but MRI showed hyperintensity in temporal lobe. A clinical diagnosis of progressive outer retinal necrosis (PORN) was made and fundus picture was documented with help of a smartphone and 20D lens. High-dose intravenous injection acyclovir was started and PORN lesion improved on treatment.

Herpes zoster ophthalmicus (HZO) is a reactivation of HZ virus that is latent in ophthalmic division of trigeminal ganglion. Patients over 50 years old, premature infant, pregnancy woman, receiving immunosuppressive agents, and malignancies are at risk of having HZO. Ocular manifestations of HZ are ectropion, entropion, ectopic eyelash, keratitis, conjunctivitis, symblepharon, hypoesthesia, episcleritis, scleritis, scleral atrophy and thinning, uveitis, iris atrophy, posterior synechiae, acute retinal necrosis, progressive outer retinal necrosis, retinal detachment, retina atrophy, optic neuritis, optic atrophy, and strabismus. Polymerase chain reaction, antigen detection, and antibody detection can help to confirm diagnosis. Pharmacology treatments for HZ ophthalmicus are antiviral drugs, corticosteroids, analgesics, tricyclic antidepressants, and antiepileptic drug. Non-pharmacology therapies are scleral contact lens, phototherapeutic keratectomy, photorefractive keratectomy, and penetrating keratoplasty. There are two kinds of vaccination which can be given to patients: Live-attenuated varicella zoster vaccine and recombinant zoster vaccine. It is recommended by Centers for Disease Control and Prevention and Food and Drugs Administration to use recombinant zoster vaccine by 50 years old.

Necrotizing retinopathies, including acute retinal necrosis (ARN) and progressive outer retinal necrosis (PORN), are a group of uncommon posterior segment complications associated with infections caused by the herpes virus. ARN was first described in 1971 by Urayama et al., Japanese ophthalmologists who termed the condition as Kirisawa uveitis and described it as acute unilateral panuveitis associated with optic neuritis and retinal panarteritis in healthy adults, with subsequent procession to diffuse necrotizing retinitis and/or subsequent rhegmatogenous retinal detachment (RRD) (Urayama et al. 1971). Standard diagnostic criteria for ARN were published by the American Uveitis Committee (Table 27.1) (Holland 1994).

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. Keywords: Necrotizing retinopathy, Viral retinitis, Acute retinal necrosis, Progressive outer retinal necrosis

Ocular infections in transplant patients can be rapidly progressive and result in significant vision loss and morbidity. Approximately 2% of transplant patients in several large series developed serious eye infections, and the most common were viral retinitis and fungal endophthalmitis. Viral retinitis is due to cytomegalovirus, herpes simplex, or herpes zoster. A particularly fulminant form of viral retinitis, progressive outer retinal necrosis, may occur and result in blindness within 1 to a few days. Hematogenous spread of bacterial and fungal infections to the eye may result in endophthalmitis. Ocular infections that occur in non-immunocompromised patients also occur in the transplant population, and these include keratitis (infection of the cornea), herpes zoster ophthalmicus, and ocular toxoplasmosis. Invasive fungal infections involving the orbit, such as rhinocerebral mucormycosis, are seen in transplant patients as they are in other immunocompromised patients. In transplant patients, sometimes the first signs of a life-threatening systemic infection occur in the eye. Patients with fungemia, for example, may present with vision loss from fungal endophthalmitis. Eye infections in the transplant population are important to diagnose promptly both to prevent irreversible vision loss, which may occur very quickly in the immunocompromised host, and to recognize an underlying systemic infection that may be life-threatening.

Many conditions, both infectious and noninfectious, may lead to inflammation of the retina and choroid. The entities discussed in this chapter are presented to provide a broad survey of conditions encountered by the uveitis and retinal specialist, but this list is by no means comprehensive. A nuanced approach that considers medical and ocular history, constitutional symptoms, ocular manifestations, and targeted ancillary testing is required to provide patients with appropriate therapies and follow-up. Close collaboration with infectious disease specialists, rheumatologists, and other medical professionals is usually a critical aspect of care in these often complicated cases.

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 (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.Clinical presentation, examination, imaging, and laboratory testing of two patients with VZV retinitis are presented.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).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, also known as PORN, has been described as a variant of necrotizing herpetic retinopathy, occurring particularly in patients with acquired immune deficiency syndrome (AIDS). Although the etiologic organism has been reported to be Varicella-zoster virus, cytomegalovirus (CMV) can be an etiologic agent. Our case illustrates the occurrence of two opportunistic infections: PORN associated with CMV and Mycobacterium avium intracellulare duodenitis in a patient with uncontrolled HIV infection.

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.

The herpes virus family is ubiquitous in the population and causes a wide spectrum of ocular disease. The name herpes originates from the Greek word herpein, meaning to creep reflecting both creeping/spreading nature of skin lesions associated with many members of the herpes virus family and the ability of the virus to remain latent prior to causing recurring infections in their host. The use of this term dates back to ancient Greece, confirming the longevity and hardiness of this viral family. The herpesviridae family each share the common structure of double-stranded DNA (dsDNA) enclosed by a viral envelope. All are nuclear replicating, with viral DNA being transcribed to mRNA within an infected cell. Once a person is infected, generally by close physical contact, the virus remains viable for life and can reactivate at any time following the primary infection. There are eight known herpes virus types that affect humans: herpes simplex virus 1 (HSV-1), herpes simplex virus 1 (HSV-2), varicella zoster virus (VZV), cytomegalovirus (CMV), Epstein--barr virus (EBV), human herpes virus 6, and human herpes virus 8. The first four are most likely to cause ocular disease. Ocular disease caused by the herpesviridae family can occur either in the anterior or posterior segment and includes some of the most devastating forms of retinitis. The pattern and presentation of disease is related to the immune status of the patient with the severely immunocompromised at greatest risk for developing a severe necrotizing retinitis. Treatment is based on the location of disease with anterior segment disease managed with a combination of systemic antivirals and topical agents. Posterior segment disease always requires systemic antiviral therapy and local therapy with intravitreal injections of antiviral medications in selected patients. Atypical cases of herpes virus associated disease often lead to delay in diagnosis. However, the increasing availability of PCR analysis of intraocular fluids has increased our diagnostic efficiency. To benefit from this improved technology and advances in treatment, it is critical to maintain a high level of clinical suspicion for this virus in all cases of ocular inflammation.

Background: Progressive outer retinal necrosis (PORN) is a necrotizing chorioretinopathy caused by herpes simplex virus, varicella-zoster virus, or cytomegalovirus. Objective: To describe the clinical morphology of PORN presenting with asymmetrical isolated bilateral retrobulbar optic neuropathy. Method: The medical records of a 45-year-old human immunodeficiency virus infected patient presenting with bilateral visual loss at King Chulalongkorn Memorial Hospital were reviewed with respect for the privacy of patient. Result: Gradual visual loss progressed to no light perception in the right eye and 20/200 in the left eye within 1 month. The patient was initially diagnosed with retrobulbar optic neuritis in both eyes. The investigations included MRI, vitreous tapping, lumbar puncture, and serological testing. MRI elicited enhancement around both optic nerves, which suggested optic neuritis. Thereafter, multifocal areas of retinal necrosis developed with rapid progression and additional cutaneous zoster lesions were established. These findings led to a diagnosis of PORN. The patient was promptly treated with intravenous ganciclovir, anti-retroviral therapy, and vitrectomy with endophotocoagulation and silicone injection. Unfortunately, his visual prognosis in the left eye was still poor Conclusion: PORN was uncommonly present with bilateral isolated optic neuropathy before retinal lesions developed. Retrobulbar optic neuropathy from herpetic infection in immunocompromised patients should be considered despite a normal fundus at initial presentation. Early diagnosis and prompt intervention may preserve vision. This case demonstrates the importance of careful physical examination and follow up of retinal lesions.

Human herpes viruses cause significant morbidity in patients with the acquired immunodeficiency syndrome. Even after the introduction of highly active anti-retroviral therapy (HAART), herpes viruses remain the leading causes of blindness in AIDS patients. Cytomegalovirus (CMV) retinitis and the closely-related immune reconstitution uveitis syndrome are the most common causes of blindness, but progressive outer retinal necrosis and acute retinal necrosis due to varicella zoster and herpes simplex are also important causes of vision loss. Successful treatment of these conditions requires an aggressive approach with multi-drug intravenous therapy or repeated intravitreal antiviral injections. Since the rate of retinal detachment is alarmingly high despite successful antiviral therapy, internists and ophthalmologists must work closely together to recognize and treat complications as they arise. Fortunately, Epstein-Barr virus is a rare cause of retinal infection and human herpes virus (HHV)-6, HHV-7, and HHV-8 do not appear to be primary pathogens. However, increasing evidence suggests that HHV-6 and HHV-7 play important roles in modulating the immune system and potentiating infection by CMV.

Purpose To report our experience with intra-silicone oil (SO) injection of antiviral agents for treatment of viral retinitis and to review the relevant literature. Design Two case reports and a literature review. Participants Two patients with viral retinitis and SO tamponade. Methods Two patients with viral retinitis were treated with intravitreal injections of low-dose ganciclovir (2 mg/0.05 ml), foscarnet (1.2 mg/0.05 ml), or both after retinal detachment repair with SO tamponade, in addition to systemic antiviral therapy from 2014 through 2015. The literature on the use of intraocular antiviral agents in the setting of SO vitreous substitute was reviewed. Main Outcome Measures Clinical outcomes after administration of intra-SO antiviral therapy. Results A patient with progressive outer retinal necrosis received 5 intra-SO injections of low-dose ganciclovir and foscarnet after surgery over 6 weeks. Another patient with acute retinal necrosis received weekly low-dose foscarnet injections into his SO-filled eye for 8 weeks after surgery. Significant retinitis regression with long-term retinitis control was achieved in both patients throughout follow-up. No articles reporting the administration of soluble antiviral agents into an SO-filled human eye were identified. Conclusions Our preliminary findings indicate that administration of low-dose ganciclovir and foscarnet into an SO-filled eye may be used as adjunctive treatment for viral retinitis. Further studies are needed to confirm these results.

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...

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.

Editors' Note: Commenting on the “Progressive outer retinal necrosis in a multiple sclerosis patient on natalizumab” Neuro Image , Bourre et al. share their experience with a patient who developed a monocular diffuse necrotizing varicella-zoster virus retinitis while on natalizumab. Patel et al., authors of the Neuro Image , respond, and further discuss the theory of ocular immune privilege. Chaturvedi et al. and Munster et al. agree that modern trials are needed to compare intensive medical therapy (IMT) alone vs IMT plus carotid endarterectomy in patients with asymptomatic carotid stenosis. Furthermore, Chaturvedi et al. alert neurologists and others involved in the care of patients with carotid disease in North America that …

Progressive outer retinal necrosis (PORN) is rapidly progressive necrotizing retinitis characterized by early patchy multifocal retinal lesions that appear deep on fundoscopy.