Progressive Outer Retinal Necrosis Research Articles

Herpes zoster in neuro-ophthalmology: a practical approach.

Herpes Zoster (HZ) or shingles is the reactivation of the Varicella Zoster Virus (VZV), usually along a single sensory nerve, but can affect both sensory and motor cranial nerves. Major risk factors for HZ include immunosuppressed status and age older than 60 years. In the United States, the lifetime risk of HZ is approximately 30%. Worldwide, the median incidence of HZ is 4-4.5 per 1000 person-years across the Americas, Eurasia, and Australia. HZ ophthalmicus, occurring in 10-20% of patients, is an ophthalmic emergency characterized by VZV reactivation along the V1 branch of the trigeminal nerve. Approximately half of this patient subgroup will go on to develop ocular manifestations, requiring prompt diagnosis and management. While anterior segment complications are more common, neuro-ophthalmic manifestations are rarer and can also occur outside the context of overt HZ ophthalmicus. Neuro-ophthalmic manifestations include optic neuropathy, acute retinal necrosis or progressive outer retinal necrosis, cranial neuropathy (isolated or multiple), orbitopathy, and CNS manifestations. Although typically a clinical diagnosis, diagnosis may be aided by neuroimaging and laboratory (e.g., PCR and serology) studies. Early antiviral therapy is indicated as soon as a presumptive diagnosis of VZV is made and the role of corticosteroids remains debated. Generally, there is wide variation of prognosis with neuro-ophthalmic involvement. Vaccine-mediated prevention is recommended. In this review, we summarize neuro-ophthalmic manifestations of VZV.

Ocular complications and factors affecting visual outcome in herpes zoster ophthalmicus: A prospective study in a tertiary care teaching hospital, Telangana, India

Background: Herpes zoster ophthalmicus (HZO) is caused by varicella zoster virus and affects the ocular branch of the trigeminal nerve. Aims and Objectives: To investigate the ocular complications and factors affecting visual prognosis in patients with HZO, a Varicella zoster virus infection affecting the ocular branch of the trigeminal nerve. Materials and Methods: 40 patients meeting the criteria were enrolled in the study, which was conducted at the Department of Ophthalmology, SVS Medical College, Mahbubnagar, Telangana, India. Results: The study found that more than 50% of HZO patients experienced ocular involvement, and acute corneal lesions, such as punctate and dendriform keratitis, uveitis, and severe post-herpetic discomfort, were associated with lower visual acuity. However, none of the patients in the study experienced posterior segment HZO problems such as acute retinal necrosis or progressive outer retinal necrosis. Older age groups were found to be more likely to experience post-herpetic neuralgia (PHN), which was also associated with nasociliary nerve involvement and vision loss. Conclusion: The study suggests early treatment with tricyclic antidepressants to aid in the early resolution of PHN. In addition, the study aimed to evaluate the effect of HIV/AIDS on ocular complications in HZO patients but did not report any findings in this regard.

First-line management of necrotizing herpetic retinitis by prioritizing the investigation of immune status and prognostic factors for poor visual outcomes

PurposeTo review management, treatment, and outcomes of patients with necrotizing herpetic retinitis (NHR) to propose an algorithm for first-line management of NHR.MethodsRetrospective evaluation of a series of patients with NHR at our tertiary center between 2012 and 2021 using demographic, clinical, ophthalmologic, virological, therapeutic, and prognostic characteristics was performed. Patients were classified by NHR type: acute retinal necrosis (ARN), progressive outer retinal necrosis (PORN), cytomegalovirus (CMV) retinitis.ResultsForty-one patients with NHR were included: 59% with ARN, 7% with PORN, and 34% with CMV retinitis. All patients with CMV retinitis and PORN were immunocompromised versus 21% of patients with ARN. CMV infection was found in 14 (34%) patients, varicella zoster virus infection in 14 (34%) patients, herpes simplex virus type 2 infection in 8 (20%) and type 1 infection in 5 (12%) patients. Intravenous antiviral therapy was received by 98% of patients and intravitreal antiviral injections by 90% of patients. The overall complication rate during follow-up was 83% of eyes. Most frequent complications were retinal detachment (33% eyes) and retinal break (29% eyes). Prognostic factors for poor visual outcomes were pre-existing monocular vision loss in contralateral eye among 17% of patients, bilateral NHR in 17% of patients, posterior pole involvement in 46% of eyes, and involvement > 2 retinal quadrants in 46% of eyes.ConclusionsThe visual prognosis of patients with NHR remains poor. Prompt investigation of immune status and presence of factors justifying intravitreal antiviral injections must be prioritized to initiate and adapt management while awaiting causative virus confirmation.

Clinical and Virological Characteristics and Prognostic Factors in Viral Necrotizing Retinitis.

Purpose: Describe the clinical and virological characteristics of viral necrotizing retinitis (VNR) and assess its prognostic factors. Methods: Retrospective study (Pitié Salpêtrière Hospital, Paris) of consecutive VNR patients diagnosed and monitored by qPCR on aqueous humor between 2015 and 2019. All patients received induction therapy with intravenous +/− intravitreal injections (IVI) of antivirals. Results: Forty-one eyes of 37 patients with a mean age of 56 years were included. Involved viruses were VZV (44%), CMV (37%) and HSV2 (19%). Acute retinal necrosis represented 51%, progressive outer retinal necrosis 12% and CMV retinitis 37% of eyes. Forty-six percent of patients were immunocompromised. Median BCVA was 0.7 LogMAR at baseline and 0.8 LogMAR after an average of 14.1 months. VNR bilateralized in 27% of cases after 32 months. Retinal detachment (RD) occurred in 27% of cases after a mean duration of 98 days. Factors associated with a “poor BCVA” at 1 month were: advanced age, low baseline BCVA, high vitritis grade and viral load (VL) at baseline and the “slow responder” status (i.e., VL decrease <50% after 2 weeks of treatment). Factors associated with RD were: advanced age, immunocompetence, low baseline BCVA, high vitritis grade at baseline and use of ≤5 IVIs. Conclusions: Clinical factors including advanced age, immunocompetence, low BCVA and high vitritis grade at baseline were associated with a poor prognosis. New virological factors were predictive of a poor outcome: high baseline VL and the “slow responder” status. Sequential intraocular fluid sampling might help prognosticate the outcomes of VNR.

Triple trouble: syphilitic neuroretinitis preceding progressive outer retinal necrosis in a HIV-infected man.

Triple trouble: syphilitic neuroretinitis preceding progressive outer retinal necrosis in a HIV-infected man.

Clinical characteristics in the misdiagnosis of cytomegalovirus retinitis: A retrospective analysis of eight patients.

To highlight characteristics in the misdiagnosis of cytomegalovirus retinitis (CMVR). Misdiagnosed cases related to CMVR were analyzed retrospectively at the Department of Ophthalmology, Beijing Youan Hospital, from July 2017 to October 2019. The medical records were reviewed by two independent senior ophthalmologists and the patients' clinical characteristics were analyzed. Eight patients (16 eyes) were identified with misdiagnoses related to CMVR. Six of the patients with CMVR were previously unaware of their human immunodeficiency virus (HIV) infection; one patient with CMVR concealed their history of HIV infection. The cases were initially misdiagnosed as diabetic retinopathy (1/7, 14.3%), branch retinal vein occlusion (1/7, 14.3%), ischemic optic neuropathy (1/7, 14.3%), Behçet's disease (1/7, 14.3%), iridocyclitis (2/7, 28.6%), and progressive outer retinal necrosis (1/7, 14.3%). One patient with binocular renal retinopathy and chronic renal insufficiency was misdiagnosed with CMVR. Four eyes (4/16, 25%) presented with pan-retinal involvement. Fourteen eyes (14/16, 87.5%) had optic disc or macular area involvement. At the final diagnosis, one patient was blind, and two patients had low vision. Seven AIDS patients showed an extremely low level of CD4+ T lymphocytes (median of 5 cells/μl; range 1-9 cells/μl). CMVR may be misdiagnosed in the absence of known immune suppression. CMVR and HIV screening cannot be overlooked if a young male patient presents with yellowish-white retinal lesions. These misdiagnosed patients had severe retinitis associated with poor vision.

Human Vaccines and Herpes Zoster Ophthalmicus: Clinical Manifestation, Treatment, and Prevention

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.

Progressive outer retinal necrosis: Dual virus involvement.

Progressive outer retinal necrosis: Dual virus involvement.

Infectious uveitis in immunodeficient HIV-negative patients: A retrospective study

Cases of infectious uveitis in immunodeficient patients may present with atypical clinical features because the clinical course of disease is usually affected by the compromised immune system. Therefore, it is sometimes difficult to determine the correct diagnosis. The aim of this study was to evaluate a prevalence of immunodeficient HIV-negative individuals among patients with infectious uveitis and to assess diagnostic and therapeutic approaches. A retrospective study. In years 2003-2017, we diagnosed 594 patients with infectious uveitis. In 35 of them, infectious uveitis occurred on the basis of immunodeficiency (malignancy, immunosuppressive therapy etc.). The most common infectious uveitis was cytomegalovirus retinitis, followed by acute retinal necrosis, herpetic anterior uveitis, endogenous fungal endophthalmitis, toxoplasmic retinochoroiditis, progressive outer retinal necrosis and syphilis. In indicated cases, intraocular fluid examination was a valuable diagnostic tool. After initiation of treatment, improvement of BCVA was observed in 13 eyes, stabilization in 13 eyes and deterioration in 11 eyes. In some patients who experienced adverse effects of medication, the recommended doses were decreased. Our experience shows that patients with diagnoses of acute retinal necrosis or progressive outer retinal necrosis have a poor visual prognosis. The occurrence of cytomegalovirus retinitis signifies a very unfavorable survival prognosis in patients who underwent hematopoietic stem cell transplantation; the patients in our study died within 1 year from cytomegalovirus retinitis diagnosis.

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.

Multimodal Imaging of Progressive Outer Retinal Necrosis

Multimodal Imaging of Progressive Outer Retinal Necrosis

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.

Intravitreal Administration of Antiviral Agents in Silicone Oil–Filled Human Eyes

To report our experience with intra-silicone oil (SO) injection of antiviral agents for treatment of viral retinitis and to review the relevant literature. Two case reports and a literature review. Two patients with viral retinitis and SO tamponade. Two patients with viral retinitis were treated with intravitreal injections of low-dose ganciclovir (2mg/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. Clinical outcomes after administration of intra-SO antiviral therapy. 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. 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.

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.

Progressive outer retinal necrosis in a multiple sclerosis patient on natalizumab

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 …

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/mm3. 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/mm3.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.

VARICELLA ZOSTER VIRAL RETINITIS IN A PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSIS

AbstractA young female a known case of systemic lupus erythematosus who underwent renal transplantation for end stage lupus nephritis was on long term immunosuppression for her systemic condition. She had an episode of varicella three months before she presented to us with ocular complaints in the right eye. She was diagnosed to have progressive outer retinal necrosis with a differential of cytomegaloviral retinitis in the right eye and treatment was initiated. She had begun to show improvement in visual acuity but within two weeks her systemic condition worsened rapidly and she succumbed to septic shock.The ocular infection in an immunocompromised SLE patient can be a precursor of mortality as seen in our patient.Due consideration is to be given for prophylactic treatment post varicella infection along with a multidisciplinary approach in managing the immunocompromised, to prevent devastating complications or mortality.

Vintage Progressive Outer Retinal Necrosis—Reply

Vintage Progressive Outer Retinal Necrosis—Reply

The role of sex in uveitis and ocular inflammation.

Uveitides can be due to non-infectious and infectious etiologies. It has been observed that there is a gender difference with a greater preponderance of non-infectious uveitis in women than in men. This review will describe both non-infectious and infectious uveitides and describes some of the current autoimmune mechanisms thought to be underlying the gender difference. It will specifically look at non-infectious uveitides with systemic involvement including juvenile idiopathic arthritis, spondyloarthopathies, sarcoidosis, Behcet’s disease, and Vogt-Koyanagi-Harada disease and at uveitides without systemic involvement including sympathetic ophthalmia, birdshot chorioretinitis, and the white dot syndromes. Infectious uveitides like acute retinal necrosis, progressive outer retinal necrosis, and cytomegalovirus mediated uveitis will be mentioned. Different uveitides with female- or male- predominance are presented and discussed.

Could Progressive Outer Retinal Necrosis Begin With Retinal Deep Capillary Ischemia?—Reply

Could Progressive Outer Retinal Necrosis Begin With Retinal Deep Capillary Ischemia?—Reply