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.

Changes of viral DNA load in the early stages of antiviral treatment in varicella zoster virus-induced acute retinal necrosis / 中华眼底病杂志

Objective:To observe the changes of varicella zoster virus (VZV)-DNA load in aqueous humour samples in VZV-induced acute retinal necrosis (ARN) in the early stages of antiviral treatment.Methods:A retrospective observational clinical study. From April 2016 to April 2018, 24 patients with 24 eyes of VZV-induced ARN who were diagnosed by Department of Ophthalmology, Eye and ENT Hospital of Fudan University and received complete aqueous humor sampling were included in the study. Among them, there were 13 males with 13 eyes, 11 females with 11 eyes; 12 left eyes and 12 right eyes; the age was 52.0±9.5 years old (39-71 years old). The time from the onset of ocular symptoms to the diagnosis of ARN was 16.6±6.1 days (7-30 days). Best-corrected visual acuity (BCVA) and ultra-wide-field fundus imaging were performed in all affected eyes. The BCVA examination was carried out using the Snellen visual acuity chart, which was converted into the logarithm of the minimum angle of resolution (logMAR) visual acuity. All patients were given intravitreal injection of 40 mg/ml ganciclovir 0.1 ml (including 4 mg of ganciclovir), 2 times a week, until the active necrotizing retinal lesions subsided, at most after the diagnosis 4 weeks, with a maximum of 9 injections. The follow-up period was 12.8±5.6 months. The aqueous humor samples were collected at presentation and 4, 7, 14, 21, 28 days after the initiation of antiviral therapy, and the VZV-DNA load was detected by real-time quantitative polymerase chain reaction. A plateau phase and a logarithmic reduction phase of the DNA load changes were observed after antiviral treatment began. Wilcoxon rank sum test was used to compare and analyze the differences in BCVA between the eyes at baseline and last follow-up.Results:The mean viral load at presentation was 8.6×10 7±1.3×10 8 copies/ml. The initial plateau phase last for an average of 7.4±2.4 days. In the following logarithmic reduction phase, the mean slope of the decline in viral load was -0.13±0.04 log/day, and the expected time for half reduction of the initial viral load was 2.5±0.7 days. After 28 days antiviral treatment, the viral load decreased to 1.7×10 5±1.8×10 5 copies/ml. In the course of the disease, rhegmatogenous retinal detachment occurred in 16 eyes. Before treatment and at the last follow-up, the logMAR BCVA of the affected eye was 1.1±0.6 and 0.8±0.7, respectively. The results of correlation analysis showed that the logMAR BCVA at the last follow-up was correlated with the initial VZV-DNA load ( r=0.467, P=0.033). Conclusion:The VZV-DNA load in the aqueous humor of eyes with VZV-induced ARN is significantly decreased after antiviral treatment, which is closely related to the clinical process of ARN.

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.

Bilateral Acute Retinal Necrosis and Encephalomalacia Due to Herpes Simplex Virus Infection in a Premature Infant

Herpes simplex virus (HSV) is a common pathogen, that causes a broad spectrum of diseases, ranging from minor skin infections to severe encephalitis and widespread infections. Acute retinal necrosis (ARN), one of the most serious manifestations of HSV infection, is defined as a rapidly progressing necrotizing retinopathy that presents discrete areas of circumferential retinal necrosis, along with signs of uveitis, vitreitis, and retinal vasculitis. We encountered a case of a female infant, born at 33 weeks of gestation with a body weight at birth of 2,080 g, who had ARN and encephalomalacia due to HSV infection. ARN associated with HSV infection should be suspected when nonspecific retinal exudates are observed in neonates, especially preterm infants.

Acute retinal necrosis following intravitreal dexamethasone (Ozurdex®) implant / Necrose aguda de retina após implante de dexametasona intravítrea (Ozurdex®)

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.

Uma mulher de idade de 52 anos em tratamento azatioprina para a artrite reumatóide desenvolveu necrose aguda de retina um mês após implantação Ozurdex® para uveíte posterior do olho esquerdo. DNA de varicela zoster (VZV) foi detectado em amostras de câmara anterior e vítreo por análise de PCR. Apesar da terapia antiviral sistêmica e intravítrea, o paciente apresentou descolamento de retina. Desfecho favorável estrutural e funcional foi obtida após a cirurgia retiniana com óleo de silicone. Pelo conhecimento dos autores, este é o primeiro caso relatado de necrose aguda de retina após a colocação de um implante Ozurdex®. Os médicos que implantam Ozurdex® devem estar cientes desta complicação incomum, mas devastadora. É necessário cuidado extra e acompanhamento frequente dos pacientes que recebam o implante Ozurdex® e apresentem qualquer condição imunocomprometedora.

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.

Therapeutic effects of ganciclovir with different injection methods on experimental retinal necrosis / 中华眼底病杂志

Objective To observe the therapeutic effects of ganciclovir(GCV)with differentInjection methods on experimental acute retinal necrosis(ARN).Methods The right eyes of 41pigmented rabbits were infected by herpes simplex virus(HSV-1)(COS strain)to establish ARN animalmodel.After 24 and 72 hours,GCV was given by intravitreal injection(10 eyes),intravenous injection(11 eyes)and the intravitreal+intravenous injection(10 eyes);intravitreal injection of GCV anddexamethasone(6 eyes)was also included.Four eyes were not treated as the control.The dosage of GCVin intravitreal and intravenous injection was 800 μg and 5 mg/kg weight,respectively.Retina necrosis wasobserved and the grade was recorded 1-21 days after injection according to the grade standard ofretinopathy.The maximum grades of retinal necrosis in different groups were compared.Results Thegrade of retinal necosis was 3.8 in the control group,and 0.2,0.4,0.8,and 2.2 in intravitreal iniection,intravitrcal+intravenous injection,intravitreal injection with GCV and dexamethasone,and intravenousrejection,respectively,24 hours after the model was set up.The effects of the first 3 groups wereobviously better than the Iast group(P=0.003,0.011,0.045);while the difference among the first 3groups were not significant(P=0.881,0.054,0.107).Seventy-two hours after the mode J was set up,thegrades of retinal necrosis were above 1.4 in 4 groups,and the differences among the 4 groups were notapparent(P=0.214).Conclusions In the animal model of ARN,intravitreal injection with GCV caneffectively decrease the grade of retinal necrosis.The difference among intravitrealinjection,intravitreal+intravenous injection,intravitreal injection with GCV and dexamethasone,and intravenous injection is notsignificant.

Characteristics of fundus angiography in acute retinal necrosis syndrome / 中华眼底病杂志

Objective To observe the characteristics of images of fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) in patients with acute retinal necrosis syndrome (ARNS), and investigate the applied value of FFA and ICGA in clinical diagnosis. Method The data of the ocular fundus, FFA and ICGA of 20 patients (28 eyes) with ARNS were retrospectively analyzed. Results The images of FFA indicated hyperfluorescence of optic disc in 24 eyes (85.71%) at the late phase; widespread retinal vascular occlusion at the peripheral focus in 23 eyes (82.14%) with some occlusive vascular shadow and the fluorescein in some white-line-like blood vessels; fluorescent leakage at the junction of normal and abnormal retina in 22 eyes (78.57%); retinal detachment in 20 eyes (71 42%), including 9 with retinal hole (45% of the patients retinal detachment); and macular cystoid edema in 8 eyes (28 57%). The images of ICGA showed hyperfluorescence of optic disc in 8 eyes (28 57%) including 5 with dotted staining at the optic disc at the late phase; unclear choroidal vasculature in the peripheral focus in 20 eyes (71.42%); and choriodal scattered hypofluorescent patch at the focus area in 19 eyes (67.85%). At the late phase of ICGA, some intravascular emboli and segments of retinal vascular occlusion were clearly demonstrated. Conclusion The main manifestations of patients with ARNS in the images of FFA were hyperfluorescence of optic disc and retinal vascular occlusion; and unclear choroidal vessels and scattered hypofluorescent patch at the focus area.Combination of FFA and ICGA is helpful to understand the extent of the lesions and the relations between choroids and retina, which has great significance in diagnosing ARNS.