Clinical Features of Acute Retinal Necrosis with Optic Neuropathy and Central Retinal Artery Occlusion as Initial Manifestations.

Acute retinal necrosis normally occurs at the periphery retina and gradually merges and progresses to the posterior pole. Optic neuropathy and central retinal artery occlusion as initial manifestation is very rare. We report the case of a patient with optic neuropathy and central retinal vessels as the first manifestations of acute retinal necrosis. Antiviral drugs, corticosteroids, and drugs that improve blood circulation were given. The necrotic retina and swollen optic disc disappeared gradually. However, the final vision of this eye declined to no light perception. From the first case report in 2001 to now, a total of 8 sporadic cases have been reported. The average onset age is 60.85±14.05 years. Most of them had no history of virus infection. Cardiovascular disease history maybe a risk factor. Acute retinal necrosis should be considered in patients with retinal vascular occlusion accompanied by granulomatous anterior uveitis. Further research is needed to determine whether treatments in addition to antiviral and corticosteroid therapy are needed.

Acute Retinal Necrosis as a presenting ophthalmic manifestation in COVID 19 recovered patients.

Purpose: Infection by the SARS-CoV2 virus results in an immune dysregulated state which can predispose to reactivation of Herpes viruses. This report describes the development of Acute Retinal Necrosis (ARN) in two patients who had recovered from SARS-CoV2 infection.Methods: Observational report of two consecutive patients who presented with ARN after having recovered from SARS-CoV-2 infection.Results: Case 1 was a 5-year-old child with extensive peripheral necrotizing retinitis. Case 2 was a 61-year-old gentlemen with bilateral retinal detachment with sieve-like breaks and optic atrophy. Both patients had recovered from SARS- Cov-2 infection 1 month ago. PCR from vitreous sample of both patients was positive for Herpes simplex virus. Case 1 was treated with oral valacyclovir. Case 2 underwent surgery in both eyes for retinal detachmentConclusion: Immune dysregulation after COVID-19 infection can result in reactivation of herpesvirus and may lead to development of ARN.

Visual outcome and poor prognostic factors in acute retinal necrosis syndrome.

OBJECTIVE: To evaluate the impact of selected clinical parameters on the mid-/long-term visual outcome of patients with acute retinal necrosis (ARN) DESIGN: A retrospective cohort study SETTING: Two University Hospitals (Parma, Italy; Lausanne, Switzerland). PARTICIPANTS: Thirty-nine non-HIV patients (39 eyes) with ARN, as confirmed by polymerase chain reaction on intraocular samples. The following potential predictors were tested using linear regression models: age, sex, etiology, best-corrected visual acuity (BCVA) on admission, delay between ARN symptom onset and treatment initiation, and surgery (performed or not). MAIN OUTCOME: BCVA at the final follow up RESULTS: Thirty-nine of 39 non-HIV patients (22 men and 17 women; mean age, 50 years) diagnosed with ARN were enrolled in the study. Etiologies were: varicella-zoster virus in 25 eyes (64%), herpes simplex viruses in the remaining 14 eyes. The average follow-up duration was 19 ± 13 months. All patients had undergone systemic antivirals; surgery was performed in 16 eyes. The mean delay between onset of visual symptoms and antiviral treatment initiation was 15 ± 31 days (range, 1-180 days). The mean BCVA at baseline was 0.83 ± 0.75 logMAR, while the mean final BCVA was 0.75 ± 0.81 logMAR. Both initial BCVA and treatment delay (TD) were significantly correlated with the final BCVA (p < 0.05). CONCLUSIONS: Initial BCVA and TD seem to be significant predictors of mid-/long-term visual outcome in non-HIV patients affected by ARN.

Retinitis necrotizante aguda. A propósito de un caso / Acute necrotizing retinitis. Case report

RESUMEN La retinitis necrotizante aguda, es una necrosis retinal de todas las capas de la retina. Se caracteriza por necrosis fibrinoides de la pared de los vasos y oclusión vascular. Se presentó un caso de una paciente de 42 años de edad, con pérdida brusca de la visión del ojo derecho, con una semana de evolución. Al examen oftalmológico se observó haze vítreo 2+, edema sucio del disco óptico, con borramiento de todos sus bordes. Presencia de exudación blanca-amarillenta retiniana extensa, en toda la periferia y área ecuatorial asociada a hemorragias intraretinianas. Se realizó además del examen fundoscòpico toma de muestra de humor acuoso para reacción en cadena de la polimerasa y angiografía fluoresceínica. Los signos fundoscópicos de la paciente, así como las anomalías detectadas en la angiografía fluoresceínica sugirieron el diagnóstico clínico de síndrome de necrosis retinal aguda. Se confirmó el diagnóstico etiológico viral, semanas después por el resultado de polimerasa. Se practicó la terapéutica médica y fotocoagulación laser focal retiniano correspondiente, asociado a cirugía del desprendimiento de retina mixto mediante vitrectomía pars plana y se logró la re aplicación de la retina.

ABSTRACT The acute necrotizing retinitis is a retinal necrosis of all the retinal layers. It is characterized by fibrinoid necrosis of the vases' wall and vascular occlusion. The case presented is the case of a female patient aged 42 years, who suffers sudden loss of the left eye vision, with a week of evolution. At the ophthalmologic examination it was observed a 2+ vitreous haze, dirty edema of the optic disk, with effacement of all of its margins. There it was an extended white-yellowish retinal exudation in the entire periphery and the equatorial area, associated to intra-retinal hemorrhages. Besides the funduscopic examination, it was taken a sample of the aqueous humor for a polymerase chain reaction (PCR) and fluorescein angiography. The patient's funduscopic signs, and also the anomalies detected in the fluorescein angiography suggested the clinical diagnosis of acute retinal necrosis syndrome. After several weeks, the viral etiologic diagnosis was confirmed by polymerase chain reaction. It was practiced the correspondent medical therapeutics and focal retinal laser coagulation associated to mixt retinal detachment through pars plana vitrectomy; the retina reapplication was achieved.

Bilateral Acute Retinal Necrosis: Clinical Features and Outcomes in a Multicenter Study.

Purpose: To describe clinical features and outcome in bilateral acute retinal necrosis (BARN). Methods: Observational retrospective longitudinal review of ocular findings. Results: Thirty eyes of 15 patients (age 44.1 ± 15.8). Delay of involvement between eyes was 57.2 ± 105.2 months (median 3, range 0.5-360). Herpes simplex virus (HSV)-1 was the most frequent (20 eyes, 66.6%), followed by HSV-2 (five eyes, 16.7%) and varicella zoster virus (VZV, four eyes, 13.3%). Visual acuity worsened in 7 (23%) eyes, improved in 4 (13%), and remained stable in 19 (63%). Major complications included retinal detachment (11 eyes, 36%), optic atrophy (11 eyes, 33%), proliferative vitreoretinopathy (four eyes, 13.3%), neovascular glaucoma (four eyes, 13.3%), phthisis bulbi (three eyes, 10%). Symptoms-to-referral average time was 2.7 ± 1.0 weeks (range 1-4). Conclusions: In our study BARN was associated with severe visual outcome and high rate of ocular complications. Although BARN is a rare disease, the course is aggressive, regardless prompt referral in tertiary-care uveitis centers.

Bilateral acute retinal necrosis after bilateral cataract surgery in an immunocompromised patient: a case report.

PURPOSE: To report a case of bilateral varicella zoster virus (VZV)-associated acute retinal necrosis (ARN) occurring after both eyes sequential cataract surgery in an elderly immunocompromised patient. METHODS: Medical records and investigation results of the patient were reviewed. RESULTS: Patient experienced floaters and blurring of vision in both eyes 4 weeks after her second uncomplicated cataract surgery. Clinical signs of granulomatous keratic precipitates, prominent vitritis, retinitis and vascular thrombosis were noted in both eyes. Aqueous samples from both eyes were positive for VZV. Disease was treated with intravitreal foscarnet bilaterally and 10 days of systemic intravenous acyclovir (10 mg/kg) followed by oral valaciclovir 1 g three times daily. Final visual acuity at 4 months after initial presentation was 20/60 in both eyes with no retinal detachment noted. CONCLUSIONS: Cataract surgery may have been the trigger for bilateral VZV-associated ARN. Immunocompromised patients can develop ARN and require close observation after cataract surgery. This is, to our knowledge, the first report of bilateral ARN following routine cataract surgery.

Retinal necrosis as the initial presentation of primary intraocular lymphoma.

A 72 years old female presented with bilateral painless progressive loss of vision over one year. She was diagnosed as non-resolving bilateral panuveitis. Her visual acuity in right eye was hand movement close to face and left eye was perception of light with inaccurate projection of rays. Bilateral anterior chamber had 1+ cells and flares. Vitreous cells had 1+ cells and haze in right eye but the left eye had 3+ vitreous cells and haze. Right eye fundus had multiple, discrete sub retinal yellowish deposits with subretinal haemorrhage and macular edema with perivascular infiltrates. In left eye, disc was just visible. The patient underwent diagnostic vitrectomy in left eye and undiluted vitreous sample on cytology showed reactive large lymphoid cells with necrotic background pattern suggestive of intraocular lymphoma. Patient underwent external beam radiotherapy and chemotherapy.

Retained intraocular iron foreign body presenting with acute retinal necrosis.

We report an unusual case of retained metallic intraocular foreign body (IOFB) presenting with acute retinal necrosis (ARN). A healthy young man presented with signs of ARN including hypopyon, dense vitritis, and peripheral retinal necrosis following alleged history of trauma with a high-velocity projectile. After initial management of ARN with systemic antivirals, a retained metallic IOFB was identified and subsequently removed surgically. The patient was followed up for 12 months postoperatively and retained excellent vision without recurrence of the ARN. The diagnosis of an IOFB in a case with associated inflammation can be challenging. A strong clinical suspicion with proper investigations can achieve optimum results.

Acyclovir resistant acute herpes simplex encephalitis associated with acute retinal necrosis: A case report and review of the literature.

A 55-year-old man was admitted to our hospital for investigation of high fever, decreased consciousness and bilateral visual impairment. His cerebrospinal fluid analysis revealed pleocytosis of mononuclear cells and an increased protein concentration. FLAIR images revealed multiple high-intensity lesions in the frontal lobe, part of which was enhanced with gadolinium. Despite initiating treatment with acyclovir and corticosteroids, his consciousness and visual acuity deteriorated. Immunopathological examination of brain biopsies showed numerous herpes simplex virus type 2-positive neurons and macrophages, leading to a diagnosis of herpes simplex encephalitis (HSE). Fundoscopic examination revealed multiple foci of retinitis with vasculopathies, and inflammation in the anterior chamber and vitreous, indicating acute retinal necrosis (ARN). Foscarnet treatment was initiated in place of acyclovir and his consciousness improved, with a slight improvement in visual acuity. ARN is typically caused by a herpes virus infection limited to the eyeball, and rarely in combination with HSE. In such cases, there is a latency of approximately 2-4 weeks between ARN and the onset of encephalitis. Our case is unique in that HSE and ARN developed simultaneously, and it highlights that there may not always be a latency between the onsets of the two disorders. Finally, foscarnet should be considered in cases of HSE and ARN with acyclovir resistance.

Necrotizing retinitis of multifactorial etiology.

Introduction. We present the case of a 73-year-old woman with osteoporosis, who presented to the emergency room with a sudden vision loss and ocular pain in the right eye, which appeared two days before. The patient mentioned loss of appetite, weight loss for three months and low fever for two weeks. Materials and methods. Among the ophthalmological findings, the most important were panuveitis, and large confluent necrotic areas in the peripheral retina. The patient was diagnosed with RE Panuveitis and acute necrotizing retinitis. Results. Blood exams showed leukocytosis and monocytosis, thrombocytosis and anemia. Further investigations showed high levels of Cytomegalovirus (CMV) anti IgG and Herpes Simplex (HS) type 1 virus anti IgM, urinary infection, and secondary hepatic cytolysis. The CT and MRI of the thorax and abdomen showed no sign of neoplastic disease, and no explanation for the CMV infection was found. The patient received general corticotherapy and antiviral therapy, and, after one month, RE BCVA was 20/ 30. Particularity of the case. Acute necrotizing retinitis in an old patient with CMV and HSV type 1, associated with secondary hepatic cytolysis, without any other immunosuppressive disease and very good outcome.

Fulminant bilateral acute retinal necrosis after chickenpox - a case report.

We present the case of a 34-year-old male, admitted for progressive bilateral loss of vision after a recent episode of chickenpox. Ophthalmological exam revealed bilateral acute retinal necrosis. As the patient was following a drug detoxification program, he was tested for HIV, HVB, HVC, and results highly positive. Immediate intravenous therapy with high doses of acyclovir and methylprednisolone was initiated, but the evolution was extremely severe resulting in necrotic retinal detachment. Surgery was performed in right eye, but no improvement of visual acuity was observed. CONCLUSIONS: The fulminant evolution of bilateral acute retinal necrosis and the lack of response to maximal intravenous therapy were clinical elements indicating coexistent immunosuppressive disease. Very severe acute retinal necrosis may occur in immunosuppressed patients, leading to blindness.

Acute retinal necrosis following intravitreal dexamethasone (Ozurdex®) implant.

A 52-year-old woman undergoing azathioprine treatment for rheumatoid arthritis developed acute retinal necrosis a month after intravitreal dexamethasone (Ozurdex ®) implantation for posterior uveitis in the left eye. Varicella zoster virus (VZV) DNA was detected in the anterior chamber and vitreous samples on polymerase chain reaction (PCR) analysis. Retinal detachment occurred despite systemic and intravitreal antiviral therapy. Favorable structural and functional outcomes were achieved after retinal surgery with silicone oil. To the authors' knowledge, this is the first reported case of acute retinal necrosis following placement of an Ozurdex® implant. Physicians practicing Ozurdex® implantations should be aware of this unusual but devastating complication. Extra caution and frequent follow-up are required in all immunocompromised patients receiving Ozurdex® implantation.

Bilateral acute retinal necrosis due to varicella zóster virus in an elderly patient.

CASE REPORT: The case is reported of acute retinal necrosis with bilateral involvement due to Varicella Zoster virus in a 77 year-old man. Polymerase chain reaction (PCR) of aqueous humor was positive for Varicella Zoster virus (VZV). He developed a Kyrieleis' vasculitis a month after the starting treatment, when the PCR analysis was negative. DISCUSSION: PCR is a quick and safe technique, with a high sensitivity and specificity of 97%, useful to diagnose and monitor the viral activity. The intervention must be urgent, due to the dramatically rapid evolution. Oral famciclovir oral is good alternative owing to its bioavailability.

[Atypical case of acute retinal necrosis secondary to the primary herpes simplex infection]. / Nietypowy przypadek ostrej martwicy siatkówki w przebiegu pierwotnego zakazenia wirusem herpes simplex.

Acute retinal necrosis is a rare manifestation of viral chorioretinitis, accompanied by occlusive vasculitis, which is associated with poor visual prognosis. The main causal factors include varicella-zoster virus in older patients and herpes simplex in younger ones. The disease typically manifests as a reactivation of latent infections. We present a case of a 57-year-old female with atypical clinical manifestation of acute retinal necrosis secondary to the primary viral infection with herpes simplex. The serology panel of vitreous tap and blood sample confirmed viral aetiology (H. simplex). The initial clinical signs included optic disc edema with retinitis presenting as self-limiting, slowly progressing, peripheral lesions, later followed by uveitis. The antiviral therapy resolved the symptoms of uveitis and enabled healing of retinal lesions, however the natural course of disease was later complicated with retinal detachment. It was successfully treated with vitreoretinal surgery. Despite aggressive treatment, the final visual outcome was unfavourable, due to optic nerve atrophy.

Combination systemic and intravitreal antiviral therapy in the management of acute retinal necrosis syndrome.

BACKGROUND AND OBJECTIVE: Acute retinal necrosis (ARN) may lead to severe visual loss because of its rapid progression and high likelihood of retinal detachment (RD). This study investigates whether combination systemic and intravitreal antiviral therapy is superior to systemic antiviral therapy alone. PATIENTS AND METHODS: Single-center, interventional, comparative case series of patients with ARN treated with combination systemic antiviral and intravitreal foscarnet injection therapy or systemic antiviral therapy alone. Survival analysis and incidence rates of visual acuity (VA) gain of two lines or greater, severe visual loss of 20/200 or worse, and RD were assessed. RESULTS: Twelve patients received combination therapy and 12 received systemic therapy alone. Patients receiving combination therapy were more likely to gain two or more lines of VA and showed decreased incidences of severe visual loss and RD. CONCLUSION: Combination oral and intravitreal antiviral therapy may improve the likelihood for VA gain and decrease the risk of RD in patients with ARN. Clinicians should consider administering combination systemic and intravitreal antiviral therapy for patients with the ARN syndrome.

Necrose retiniana aguda por presumível etiologia viral por herpes simples resultando em endoftalmite: relato de caso / Acute retinal necrosis due to herpes simplex resulting in endofthalmitis: case report

A necrose retiniana aguda é uma rara e devastadora retinite necrotizante, que pode ser causada pelo vírus herpes simples tipo 1 ou 2. Afeta tipicamente pacientes saudáveis de todas as idades. Este estudo é um relato de caso de um paciente de 18 anos, previamente hígido, que apresentou necrose aguda de retina por presumível etiologia viral por herpes simples, e descreve sua apresentação clínica e ultrassonográfica. Por não ter sido diagnosticado e tratado precocemente, a necrose retiniana aguda cumpriu sua história natural e evoluiu para um quadro compatível com endoftalmite.

The acute retinal necrosis is a rare and devastating necrotizing retinitis, which can be caused by the herpes simplex virus type 1 or 2. It typically affects healthy patients of all ages. This study report a case of 18 year-old healthy male patient, with the the diagnosis of the acute retinal necrosis due to herpes simplex, and presents its clinical and ultrassonographic features. The acute retinal necrosis results in endofthalmitis because of lack of early diagnosis and therapy.

Viral retinitis after intravitreal triamcinolone injection in patients with predisposing medical comorbidities.

PURPOSE: To review the cases of viral retinitis after intravitreal steroid administration at a single center, to estimate the incidence, and to propose risk factors for its occurrence. DESIGN: Retrospective, observational case series. METHODS: Seven hundred thirty-six intravitreal triamcinolone (IVTA) injections were administered in the clinic and operating room by 3 retina specialists at a single academic medical center between September 2002 and November 2008. Inclusion criteria were simply a history of 1 or more IVTA injections during the period. The overall incidence of viral retinitis after IVTA injection was calculated. Subsequently, a chart audit was performed to estimate the number of patients with immune-altering conditions who had received IVTA during the period, and the incidence within this subgroup was calculated. RESULTS: Viral retinitis developed after IVTA injection in 3 patients, yielding an overall incidence of 3 in 736 or 0.41%. An estimated 334 injections were administered to patients with an immune-altering condition, including diabetes. All 3 of the patients in whom viral retinitis developed after IVTA injection possessed abnormal immune systems, yielding an incidence rate of 3 in 334 or 0.90% within this subgroup. CONCLUSIONS: Our high reported incidence for this potentially devastating complication can be attributed to multiple factors, including coexisting medical immunocompromising comorbidities, a higher dose with a longer duration of local immunosuppression in the vitreous, multiple injections, as well as previous viral retinitis. Caution with a high index of clinical suspicion and frequent follow-up is advised in patients receiving IVTA injection with potentially immune-altering conditions, even after apparent immune recovery.

[Acute retinal necrosis: Clinical features and therapy options]. / Akute retinale Nekrose : Klinik und Therapieoptionen.

Varicella zoster virus is the most frequent cause of acute retinal necrosis (ARN) followed by herpes simplex virus. Retinal ischemia and optic nerve atrophy are the main causes of the frequently poor final visual outcome in severe cases of ARN. The clinical diagnosis of ARN should be made as early as possible. Acyclovir should be administered intravenously due to its unreliable oral bioavailability. Systemic corticosteroids should be applied to suppress tissue damage caused by the host's inflammatory response. Severe cases of ARN should be treated by early vitrectomy with diagnostic vitreous biopsy, intravitreal aciclovir lavage, intraoperative laser retinopexy and silicone oil tamponade. The role of prophylactic laser retinopexy for prevention of secondary retinal detachment remains to be determined. The cause of different degrees of severity of ARN is unknown. The degree of severity of ARN is probably an independent predictor of the functional outcome.

Retinal complications in patients with solid organ or bone marrow transplantations.

BACKGROUND: The administration of systemic immunosuppressive agents to recipients of solid organ or bone marrow transplants results in an immunocompromised status. As the number of organ recipients and their life span increase with recent progress in organ transplantation, ocular complications tend to become more diverse and serious. METHODS: From 1995 to 2005, 3656 cases of organ transplantations were performed at Asan Medical Center. The medical records of 1198 of these patients who had been examined at the Department of Ophthalmology were reviewed. RESULTS: Retinal complications were diagnosed in 33 of the transplant recipients; five with bone marrow transplantation, 16 with kidney transplantation, seven with liver transplantation (LT), and five with heart transplantation. Diagnoses included 11 cases of CMV (cytomegalovirus) retinitis, three of acute retinal necrosis, one of progressive outer retinal necrosis, five of fungal chorioretinitis, one of toxoplasmic retinochoroiditis, three of central retinal vein occlusion, and nine of central serous chorioretinopathy. While CMV, fungal, or toxoplasmic chorioretinitis developed frequently in association with extraocular infection or organ rejection, herpetic infection manifested only in the eye without any rejection. Most infectious cases responded well to the standard therapeutic regimen. Interestingly, central retinal vein occlusion developed exclusively following LT, possibly in relation to coagulation cascade abnormality. CONCLUSIONS: To our best knowledge, this comprehensive review presents the largest series of ocular complication in organ transplant recipients. Familiarity with the potential ocular complications as well as a high index of suspicion is warranted to the practicing ophthalmologists.