Necrosis retinal aguda. Dos casos clínicos / Acute retinal necrosis. A report of two clinical cases

La necrosis retinal aguda es una afección grave que amenaza la visión. Es frecuente en adultos, tanto inmunocompetentes como inmunocomprometidos. Se presentan dos pacientes, uno de 38 años, con antecedentes de salud anterior que acude a consulta con síntomas y signos de necrosis retinal aguda en el ojo izquierdo, la que fue diagnosticada luego; y otro de 48 años de edad con antecedentes de infección por herpes zóster, tres meses antes de los síntomas oculares, que concluyó con igual diagnóstico. No existió evolución satisfactoria, a pesar del tratamiento adecuado, lo que demostró que independientemente de datos estadísticos y estudios realizados que demuestran lo infrecuente de esta enfermedad, se diagnosticaron dos casos en el periodo de un año, dato que nos exhorta al estudio y práctica de alternativas diagnósticas y terapéuticas para minimizar las consecuencias devastadoras de esta afección.

Acute retinal necrosis is a serious vision-threatening condition. It is common in both immunocompetent and immunocompromised adults. We present two male patients; one aged 38 years, with a previous health history who comes to consultation with symptoms and signs of acute retinal necrosis in his left eye, which was later diagnosed; and another one aged 48 years with a history of herpes zoster infection three months before the ocular symptoms, which concluded with the same diagnosis. Regardless of the statistical data and research carried out on this rare disease, there was no satisfactory evolution despite adequate treatment. Two cases were diagnosed in a period of one year, data that urges us to study and practice diagnostic and therapeutic alternatives to minimize the devastating consequences of this condition.

Response to "Comment On: Acute Retinal Necrosis: Is the Current Valacyclovir Regimen Adequate?"

Acute Retinal Necrosis (ARN) is a potentially devastating form of Uveitis. Antivirals are the mainstay treatment for this syndrome. In this letter, we question the current oral Valacyclovir dosage, based on the experience we had with a recent unresponsive ARN case.

[Acute retinal necrosis by varicella zoster virus in an immunocompetent patient with therapeutic follow up by PCR]. / Necrosis retiniana aguda por virus varicela zoster en un paciente inmunocompetente con seguimiento de la respuesta terapéutica mediante reacción de polimerasa en cadena.

We present the case of a 63-year-old immunocompetent man with unilateral acute retinal necrosis (ARN). He consulted for blurred vision, eye pain, photophobia, and headache. Papillitis and peripheal chorioretinitis associated with vasculitis and peripheral retinal ischemia was confirmed. PCR from aqueous humor sample detected varicella zoster virus. The patient was treated with a combined therapy of oral corticosteroids, oral / intravenous acyclovir along with weekly intravitreous ganciclovir doses followed by oral valaciclovir for three months. A progressive decrease in viral load in aqueous humor was demonstrated during treatment. Follow-up showed improvement in the inflammatory condition and a slight recovery of visual acuity, however, finally he presented a retinal detachment with total loss of one-sided vision. ARN is an uncommon complication caused by some herpesviruses with a poor visual prognosis, an outcome that can be improved with early diagnosis and treatment using appropriate antivirals. Prolonged treatment reduces relapse frequency and fellow eye compromise.

Necrosis retiniana aguda por virus varicela zoster en un paciente inmunocompetente con seguimiento de la respuesta terapéutica mediante reacción de polimerasa en cadena / Acute retinal necrosis by varicella zoster virus in an immunocompetent patient with therapeutic follow up by PCR

Resumen Presentamos el caso de un varón de 63 años, inmunocompetente, con una necrosis retinal aguda (NRA) unilateral. Consultó por visión borrosa, dolor ocular, fotofobia y cefalea. Se confirmó una papilitis y coriorretinitis periférica asociada a vasculitis e isquemia retinal periférica. El estudio molecular por RPC de humor acuoso detectó la presencia de virus varicela zoster. El paciente fue tratado con terapia combinada con corticoesteroides orales, aciclovir oral/intravenoso, ganciclovir intravítreo semanal y luego valaciclovir oral por tres meses. Se demostró una disminución progresiva de la carga viral en el humor acuoso durante el tratamiento. El seguimiento mostró una mejoría del cuadro inflamatorio y una leve recuperación de la agudeza visual, sin embargo, finalmente presentó un desprendimiento de retina con pérdida casi total de la visión unilateral. La NRA es una complicación infrecuente provocada por algunos virus herpes con mal pronóstico visual, desenlace que puede ser mejorado con un diagnóstico y tratamiento precoz con antivirales. El tratamiento prolongado permite evitar la recaída y el compromiso contralateral.

Abstract We present the case of a 63-year-old immunocompetent man with unilateral acute retinal necrosis (ARN). He consulted for blurred vision, eye pain, photophobia, and headache. Papillitis and peripheal chorioretinitis associated with vasculitis and peripheral retinal ischemia was confirmed. PCR from aqueous humor sample detected varicella zoster virus. The patient was treated with a combined therapy of oral corticosteroids, oral / intravenous acyclovir along with weekly intravitreous ganciclovir doses followed by oral valaciclovir for three months. A progressive decrease in viral load in aqueous humor was demonstrated during treatment. Follow-up showed improvement in the inflammatory condition and a slight recovery of visual acuity, however, finally he presented a retinal detachment with total loss of one-sided vision. ARN is an uncommon complication caused by some herpesviruses with a poor visual prognosis, an outcome that can be improved with early diagnosis and treatment using appropriate antivirals. Prolonged treatment reduces relapse frequency and fellow eye compromise.

Association of retinal detachment with age 50 years or younger at onset in patients with acute retinal necrosis.

BACKGROUND: Due to the guarded prognosis of acute retinal necrosis (ARN), it is relevant to develop a strategy to early categorize those patients in a higher risk of worse outcomes. The purpose of this study is to describe clinical features and predictive factors for retinal detachment (RD) in patients with ARN. METHODS: Retrospective observational case series of 34 adult patients (38 eyes) with ARN examined between January 2005 and July 2015 in the National Eye Institute (Bethesda, USA), the Department of Ophthalmology, University of Chile (Santiago, Chile), and APEC (CDMX, Mexico). RESULTS: A total of 16 males and 18 females with a mean age at presentation of 44.5 ± 16.8 years were included. Twenty-seven patients (79.4%) received intravenous acyclovir as first-line treatment, and 7 patients received either oral antiviral (4 patients) or oral plus intravitreal antiviral (3 patients). All subjects were treated with prednisone, with a mean initial dose of 57.7 ± 16.3 mg per day. Seventeen patients (50.0%) developed retinal detachment. An association of retinal detachment with age at onset was observed (p = 0.04), with patients younger than 50 years presenting a higher risk (OR = 14.86, p = 0.0009). Additionally, patients in this higher risk group had more inflammation in both anterior chamber and vitreous (p = 0.04 and 0.03, respectively). No other predictive factor for retinal detachment was found in the present study. CONCLUSIONS: RD represents an important complication in patients with ARN. Younger patients may be at higher risk of this complication, possibly secondary to the presence of a higher level of inflammation.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FINDINGS IN ACUTE RETINAL NECROSIS.

PURPOSE: To report clinical features of acute retinal necrosis (ARN) using optical coherence tomography angiography. METHODS: A 59-year-old female patient presented with blurred vision in the left eye for 1 day. The patient presented posterior uveitis with multiple peripheral areas of retinal pallor with presumed acute retinal necrosis. Herpes simplex virus Type 1 infection was confirmed after serologic tests, and the polymerase chain reaction analysis of the aqueous humor tested positive. RESULTS: The left eye examination revealed anterior chamber reaction, mild vitritis, optic disk swelling, and yellowish white retinal lesions with discrete borders along the superotemporal arcade and temporal periphery. Baseline optical coherence tomography angiography revealed decreased vascular density of superficial and deep plexuses of superotemporal macular region. One month after oral valacyclovir 2,000 mg twice daily, visual acuity and retinal lesions improved, and optical coherence tomography angiography images showed improvement of vascular density. CONCLUSION: Occlusive arterial vasculopathy is one of the main clinical characteristics of acute retinal necrosis. We herein describe for the first time the features of retinal vasculature in acute retinal necrosis revealed by optical coherence tomography angiography, showing decreased vascular density of superficial and deep plexuses.

Toxoplasmose sistêmica associada à forma ocular bilateral atípica mimetizando necrose retiniana aguda / Systemic toxoplasmosis associated to the atypical bilateral ocular involvement mimicking acute retinal necrosis

RESUMO A toxoplasmose ocular pode se manifestar de forma atípica, rara, bilateral e associada à necrose retiniana aguda. É apresentada em pacientes imunossuprimidos, resultando em grave perda visual, se não for solucionada rapidamente. Relata-se um caso atípico de toxoplasmose ocular em paciente diabético, que, em sua internação prévia, já evidenciava aspecto sistêmico, o qual foi elucidado pelo exame clínico oftalmológico e pela anamnese. Além disso, a rotina do setor de uveítes, ao solicitar as sorologias de forma direcionada e criteriosa, foi imprescindível para o diagnóstico da toxoplasmose sistêmica associado à lesão ocular atípica bilateral, mimetizando necrose retiniana aguda com desfecho favorável.

Abstract Ocular toxoplasmosis can present with an atypical, rare, bilateral involvement, and associated with acute retinal necrosis. It occurs in immunosuppressed patients, resulting in severe visual loss, if not quickly solved. We report an atypical case of ocular toxoplasmosis in a diabetic patient, who already showed a systemic aspect in a previous hospitalization, which was elucidated by the ophthalmologic examination and history. In addition, the routine of the uveitis sector requesting serology in a directed and careful way was essential for the diagnosis of systemic toxoplasmosis associated with atypical bilateral ocular lesion, mimicking acute retinal necrosis with good outcome.

Acute Retinal Necrosis: Is The Current Valacyclovir Regimen Adequate?

Acute Retinal Necrosis (ARN) is a potentially devastating form of Uveitis. Antivirals are the mainstay treatment for this syndrome. In this letter, we question the current oral Valacyclovir dosage, based on the experience we had with a recent unresponsive ARN case.

Neonatal bilateral acute retinal necrosis in a neonate with a history of severe intrauterine growth restriction.

We present the case of a baby girl born at term with severe intrauterine growth restriction (IUGR) to a gravida 1 mother who was previously healthy and HIV negative. The newborn was evaluated by an ophthalmologist because of her history of IUGR and was diagnosed with intraretinal hemorrhages associated with areas of peripheral retinal necrosis at the posterior pole of both eyes. A diagnosis of acute retinal necrosis of presumed viral origin due to cytomegalovirus virus was considered, and the infant was started on and responded well to valganciclovir.

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.

Acute retinal necrosis and immune reconstitution inflammatory syndrome in a natalizumab-treated patient with multiple sclerosis.

PURPOSE: To describe a case of acute retinal necrosis and subsequent immune reconstitution inflammatory syndrome in a patient with multiple sclerosis treated with natalizumab. METHODS: Case report. RESULTS: A 51-year-old man with multiple sclerosis developed acute retinal necrosis during treatment with natalizumab. The patient was successfully managed with natalizumab discontinuation, oral valacyclovir, and prophylactic laser retinal photocoagulation. A few months after natalizumab interruption and 1 month after valacyclovir completion, the patient presented with an episode of presumed noninfectious anterior and intermediate uveitis, which responded well to oral steroid mini-pulse therapy. CONCLUSION: Natalizumab is a potent immune suppressor used in the treatment of multiple sclerosis and Crohn disease. It has already been linked to herpetic infections and immune reconstitution inflammatory syndrome in the central nervous system. These complications may also affect ocular tissues.

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.

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.

Analysis of acyclovir in vitreous humor by a validated HPLC method.

An HPLC-UV method was developed and validated for the determination of acyclovir in vitreous humor. The method was carried out in isocratic mode using 0.02 mol/L acetic acid/methanol (95:5) as mobile phase, a C18 column at 25 degrees C and UV detection at 254 nm. The method was linear (r2> 0.99) over the range of 35-700 microg/mL, precise (RSD <5%), accurate (recovery ranged from 98.18 to 99.64%), robust, selective regarding of the vitreous humor, and robust remaining unaffected by deliberate variations in relevant parameters. The validated HPLC-UV method can be successfully applied to determine acyclovir directly injected into the vitreous cavity of rabbits' eye.

Review for disease of the year: differential diagnosis of ocular toxoplasmosis.

The diagnosis of ocular toxoplasmosis is mainly clinical, based in the presence of focal necrotizing retinochoroiditis often associated with a preexistent chorioretinal scar, and variable involvement of the vitreous, retinal blood vessels, optic nerve, and anterior segment of the eye. Recognition of this clinical spectrum of toxoplasmic retinochoroiditis is crucial, but other infectious, noninfectious, and neoplastic entities should also be considered in the differential diagnosis. Investigations such as serological tests, polymerase chain reaction of ocular fluids, and assessment of intraocular antibody synthesis are helpful in uncertain cases. This article provides an overview of the differential diagnosis of ocular toxoplasmosis, focusing on the most important entities to be considered and emphasizing distinctive features of each one of them in the clinical setting. Ocular toxoplasmosis has multiple clinical manifestations, which partially overlap with those of other entities and these should be carefully considered when making the differential diagnosis, particularly in less typical cases.

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.

Syphilitic retinitis and panuveitis simulating acute retinal necrosis: case report.

Retinitis and panuveitis in immunocompetent patients is a rare and sight-threatening disease, of difficult diagnosis. A case of a 31-year-old male who presented with unilateral placoid retinitis and panuveitis, unsuccessfully treated as acute retinal necrosis, that in fact was syphilis, with neurosyphilis and excellent response to treatment is reported.

Syphilitic retinitis and panuveitis simulating acute retinal necrosis: case report / Retinite e panuveíte por sífilis simulando necrose aguda de retina: relato de caso

Retinitis and panuveitis in immunocompetent patients is a rare and sight-threatening disease, of difficult diagnosis. A case of a 31-year-old male who presented with unilateral placoid retinitis and panuveitis, unsuccessfully treated as acute retinal necrosis, that in fact was syphilis, with neurosyphilis and excellent response to treatment is reported.

Retinite com panuveíte em pacientes imunocompetentes é um condição rara e ameaçadora para a visão, e de difícil diagnóstico. É relatado um caso de um paciente do sexo masculino com 31 anos de idade, que se apresentou com retinite em placas e panuveíte, inicialmente tratado sem sucesso como necrose aguda de retina, que na verdade era um caso de sífilis ocular, com afecção do sistema nervoso central e ótima resposta ao tratamento sistêmico para sífilis.

[Acute retinal necrosis in an acute leukemia pediatric patient]. / Necrosis retinal aguda en un paciente pediátrico con leucemia aguda.

Acute retinal necrosis (ARN) is a serious condition that can impair vision. It mostly occurs in adult patients, especially those severely immunocompromised, in association with a reactivation of a herpes virus infection. We present a 4 years old patient with high risk acute leukemia, whom during a course of intense chemotherapy acquired chickenpox with visceral involvement that affected the retina, causing unilateral blindness. Varicella-zoster virus was detected by PCR in the vitreous humor, in spite of previous acyclovir treatment. The contralateral vision remained undamaged.

[Acute retinal necrosis: an adult disease visits pediatrics]. / Necrosis retinal aguda: Una entidad de adultos en pediatría.

Acute retinal necrosis (ARN) is a serious condition that can impair vision. It mostly occurs in adult patients, especially those severely immunocompromised, in association with a reactivation of a herpes virus infection. Clinical and ophtalmological features of ARN and recommended diagnostic and management strategies are reviewed.