A case of atypical progressive outer retinal necrosis after highly active antiretroviral therapy

This is a report of an atypical case of progressive outer retinal necrosis (PORN) and the effect of highly active antiretroviral therapy (HAART) on the clinical course of viral retinitis in an acquired immunodeficiency syndrome (AIDS) patient. A 22-year-old male patient infected with human immunodeficiency virus (HIV) presented with unilaterally reduced visual acuity and a dense cataract. After cataract extraction, retinal lesions involving the peripheral and macular areas were found with perivascular sparing and the mud-cracked, characteristic appearance of PORN. He was diagnosed as having PORN based on clinical features and was given combined antiviral treatment. With concurrent HAART, the retinal lesions regressed, with the regression being accelerated by further treatment with intravenous acyclovir and ganciclovir. This case suggests that HAART may change the clinical course of PORN in AIDS patients by improving host immunity. PORN should be included in the differential diagnosis of acute unilateral cataract in AIDS patients.     

Progressive outer retinal necrosis combined with vitreous hemorrhage in a patient with acquired immunodeficiency syndrome

PURPOSE: To describe an unusual case of rapidly progressive outer retinal necrosis (PORN) with vitreous hemorrhage in a 41-year-old woman with acquired immunodeficiency syndrome (AIDS), who had retinitis developed from what was probably varicellar-zoster virus combined with cytomegalovirus (CMV) and herpes simplex type 1,2, as proven by the polymerase chain reaction restriction fragment length polymorphism method (PCR-RFLP). METHODS: This study is a case report detailing clinical follow-up and an aqueous humor test by PCR-RFLP. RESULTS: The deep, white retinal lesions coalesced and progressively expanded in a circumferential manner, with sparing of the perivascular retina. However, retinal and vitreous hemorrhages, unusual findings for PORN, could be noted around the optic nerve. Varicellar-zoster virus (VZV), cytomegalovirus (CMV), and herpes simplex types 1,2 (HSV-1,2) were detected in the aqueous humor by PCR. CONCLUSIONS: PORN has been described as a variant of necrotizing herpetic retinopathy, occurring particularly in patients with AIDS. Although the etiologic agent has been reported to be VZV, concurrent or combined etiologic agents can include HSV-1, HSV-2, and CMV in AIDS patients. Therefore, combined antiviral therapy with acyclovir and ganciclovir could be more reasonable as an initial therapy.     

A case of AIDS complicated by progressive outer retinal necrosis

BACKGROUND: The retina may be involved in patients with acquired immunodeficiency syndrome (AIDS). Progressive outer retinal necrosis (PORN) is a liability. CASE: A 46-year-old female had repeated exacerbations of pulmonary tuberculosis since two years before. Herpes zoster developed in her right trigeminal nerve area two weeks before, leading to a diagnosis of AIDS. She was referred to us for ophthalmological evaluation. FINDINGS: Both eyes showed numerous yellowish white patches in the deeper retinal layers. The anterior chamber and the vitreous were almost intact. Herpes zoster virus was identified in the acqueous by the polymerase chain reaction (PCR) method. Systemic acyclovir or ganciclovir failed to prevent rapid extension of fundus lesions, resulting in whole-layer necrosis of the retina. Retinal detachment with multiple breaks developed in both eyes whthin eleven days after the patient was first seen by us. The clinical course was different from acute retinal necrosis and was characteristic of PORN. CONCLUSION: This case illustrates that PORN may develop in patients affected by AIDS.     

Progressive outer retinal necrosis in a patient with nephrotic syndrome

Progressive outer retinal necrosis syndrome (PORN) is a variant of necrotizing herpetic retinopathy and the majority of the described cases were related to acquired immunodeficiency syndrome. We present a patient who is HIV negative with nephrotic syndrome and prednisolone use for 4 months who showed clinical features of PORN. Low CD4 counts and lymphocytopenia suggested immunosuppression. In the left eye, tractional retinal detachment at the posterior pole followed by incomplete posterior vitreous detachment developed. In addition to intravenous administration of acyclovir, vitreous surgeries including stripping of the posterior hyaloid and silicone-oil tamponade were successfully performed to repair the retinal detachment in the left eye and to prevent it in the right eye.     

Progressive outer retinal necrosis caused by herpes simplex virus type 1 in a patient with acquired immunodeficiency syndrome

OBJECTIVE/BACKGROUND: To identify the etiologic agent of rapidly progressive outer retinal necrosis (PORN) in a 32-year-old man with acquired immunodeficiency syndrome (AIDS), who had retinitis developed from cytomegalovirus (CMV). Multiple yellowish spots appeared in the deep retina without evidence of intraocular inflammation or retinal vasculitis, diagnosed clinically as PORN. Death occurred after failure of multiple organs. DESIGN: Case report. METHODS: Both globes were taken at autopsy, fixed in formalin, and examined histopathologically and immunohistochemically to identify causative agents in the retinal lesions. MAIN OUTCOME MEASURE: Immunohistochemistry. RESULTS: All layers of the retina were severely damaged and contained focal calcification. Cytomegalic inclusion bodies were found in cells in the damaged retina of the right eye. Immunohistochemical studies for herpesviruses revealed the presence of CMV antigens in the right retina at the posterior pole and herpes simplex virus type 1 (HSV-1)-specific antigen in the periphery of both retinas. No varicella-zoster virus (VZV) antigen was detected in either retina. CONCLUSIONS: PORN has been described as a variant of necrotizing herpetic retinopathy, occurring particularly in patients with AIDS. Although the etiologic agent has been reported to be VZV, HSV-1 can be an etiologic agent.     

获得性免疫缺陷综合征并发进行性外层视网膜坏死的临床分析

目的 探讨获得性免疫缺陷综合征（AIDS）并发进行性外层视网膜坏死（PORN）的全身情况、眼底特点、治疗方法及预后。设计 回顾性病例系列。研究对象 2015年4月-2019年11月北京佑安医院AIDS合并PORN患者6例（11眼）。方法 分析其最佳矫正视力（BCVA）、裂隙灯、眼底照相、相干光断层扫描（OCT）及房水中水痘带状疱疹病毒脱氧核糖核酸（VZV-DNA）含量，外周血CD4+T淋巴细胞计数和人免疫缺陷病毒（HIV）病毒载量。3 眼行更昔洛韦或膦甲酸钠玻璃体注射联合全身治疗。随诊3~26个月。主要指标 AIDS合并PORN的眼底变化、房水中VZV-DNA值、玻璃体注药前后眼底变化、外周血CD4+T淋巴细胞计数和HIV病毒载量。结果 患者均为男性，平均年龄（27.6±3.2）岁。11眼均表现为无痛性视力下降，9眼（81.8%）初诊BCVA均≤0.2。眼底表现为进行性周边环形视网膜坏死，多灶性边界清楚的黄白色坏死灶向心性进展，无或轻微玻璃体反应。6眼合并渗出性视网膜脱离。早期病变OCT仅累及视网膜外层，后期出现外层视网膜变薄。11眼房水VZV-DNA含量均阳性，外周血CD4+T淋巴细胞计数平均（10.8±2.5）个/μl， HIV病毒载量平均（246289±462.7）拷贝/ml。3眼更昔洛韦和（或）膦甲酸钠玻璃体注射联合全身抗病毒治疗周期72~123天，病灶控制，视力好转（1眼由光感到手动，1眼由指数到0.1，1眼保持0.5）。结论 PORN是AIDS患者严重的眼部机会性感染，病变累及视网膜外层，前房及玻璃体反应无或轻微，病情进展速度快，预后差。眼内液病毒核酸检测有助于诊断。（眼科， 2020， 29： 375-379）     

并发视网膜中央动静脉阻塞的特殊PORN1例

目的:报道1例艾滋病患者特殊的进行性外层视网膜坏死(PORN),同时合并视网膜中央动脉及静脉阻塞。方法:病例报告。结果:患者表现为进行性外层视网膜坏死,视神经亦受累,合并视网膜中央动静脉阻塞,与带状疱疹性视网膜病变的最初表现一样。积极的治疗包括玻璃体腔和特异性系统抗带状疱疹病毒治疗,以及强化的抗逆转录病毒治疗(HAART)。视网膜坏死静止,对侧眼未受累,而患眼的视力极差。结论:首次报道了1例并发视网膜中央动静脉阻塞的特殊PORN,积极的局部联合系统治疗使得局部病情控制,并预防了对侧眼发病。     

Failure of sorivudine therapy in progressive outer retinal necrosis caused by varicella zoster virus

Background: We report on the case of a 23-year-old female who presented with ocular signs of progressive outer retinal necrosis (PORN) syndrome and who failed to respond to acyclovir, ganciclovir, foscamet and oral sorivudine. Methods: The patient was treated with the antiviral drugs acyclovir, ganciclovir, foscarnet and oral sorivudine. Results: The patient failed to respond to a combination of antiviral drugs. Unfortunately, progression of the retinitis occurred, which led to blindness. Conclusion: Despite new drugs, the prognosis of PORN is poor and recurrence is common.     

Association of progressive outer retinal necrosis and varicella zoster encephalitis in a patient with AIDS.

BACKGROUND: A patient with AIDS who developed the clinical picture of bilateral progressive outer retinal necrosis (PORN) in combination with varicella zoster encephalitis is described. The picture developed more than 2 years after an episode of ophthalmic zoster infection, and following intermittent exposure to oral acyclovir because of recurrent episodes of cutaneous herpes simplex infection. METHODS: Aqueous humour, obtained by paracentesis of the anterior chamber, was analysed using immunofluorescence and polymerase chain reaction (PCR). Postmortem analysis of eye and brain tissue was performed by using conventional techniques and in situ hybridisation. RESULTS: While conventional techniques all failed to detect a causative agent, analysis of the aqueous humour using PCR, and histological examination of necropsy specimens from eyes and brain using in situ hybridisation were conclusive for the diagnosis varicella zoster virus (VZV) infection. CONCLUSION: This case documents the presumed association of PORN and VZV encephalitis in a severely immunocompromised AIDS patient.     

Cytomegalovirus retinitis in AIDS.

Cytomegalovirus (CMV) retinitis is the most common retinal opportunistic infection in people with the acquired immune deficiency syndrome (AIDS). Ordinarily, CMV causes little morbidity in an immunocompetent person. However, in the immunosuppressed patient this virus is responsible for progressive retinal destruction with necrosis leading to a devastating loss of vision. This paper will provide the practitioner with a clinical approach to the presentation, diagnosis and management of patients with CMV retinitis secondary to AIDS.     

Retinal findings in Malawian patients with AIDS.

The purpose of this study was to determine the prevalence of retinal disease in a group of patients with AIDS in Malawi. Indirect ophthalmoscopy was performed on 99 patients with AIDS who were admitted consecutively to a medical ward at a central hospital. Necrotising retinitis was present in one eye of one patient examined. Non-infectious retinopathy was present in 13%. Retinitis is less common in AIDS patients from Africa compared with those from developed countries; it is believed that most patients die before acquiring it. Non-infectious retinopathy may also be less common.     

Good visual outcome in an immunocompromised patient with bilateral acute retinal necrosis syndrome: A case report

Acute retinal necrosis (ARN) is an uncommon necrotizing, fulminant retinopathy caused by the herpes simplex virus types 1 or 2 or by the varicella zoster vírus with visually devastating consequences. Generally it occurs in patients who are systemically healthy, but occasionally occurs in immunocompromised host. We report a case of bilateral ARN in a patient with AIDS with a good final visual outcome.     

Rhegmatogenous retinal detachments in patients with AIDS and necrotizing retinal infections.

Rhegmatogenous retinal detachments can occur in patients with acquired immune deficiency syndrome (AIDS) and necrotizing retinal infections. Of 68 patients with AIDS and necrotizing retinal infections seen between 1983 and 1987, rhegmatogenous retinal detachments developed in 16 patients (27 eyes). In this group, cytomegalovirus retinopathy was present in 75% (12 of 16) of patients, 18.8% (3 of 16) had probable herpes simplex virus retinopathy, and 6.2% (1 of 16) had toxoplasmic retinochoroiditis. Retinal detachment was bilateral in 68.8% (11 of 16) of patients. The retina was reattached successfully in 91% (10 of 11) of operated eyes. Proliferative vitreoretinopathy was present preoperatively in 95% of these eyes. Seven of 11 operated eyes had initial improvement in visual acuity. However, 5 of 11 continued to lose vision despite successful reattachment. Techniques included pneumatic reattachment, scleral buckle, vitrectomy, and silicone oil injection. Complicated retinal detachments in AIDS patients with rhegmatogenous retinal detachments and necrotizing retinal infections are common and can be repaired, but the prognosis is guarded in many patients.     

Retinopathy before the diagnosis of AIDS

We reviewed the records of ocular examinations of patients referred or examined for inflammatory retinal disease over a two-year period and found 18 cases in which retinopathy was documented before the diagnosis of acquired immunodeficiency syndrome (AIDS). Seventeen patients were either homosexual men or intravenous drug abusers. Although 13 patients had AIDS-related complex, no patients met the Centers for Disease Control criteria for AIDS before their ophthalmologic examination. In all 18 cases, the diagnosis of severe immunodeficiency suggestive of infection by the human immunodeficiency virus (HIV) was made by the ophthalmologist. These ophthalmologic findings included five patients with asymptomatic retinal cotton-wool patches, two patients with endogenous Staphylococcus epidermidis bacterial endophthalmitis, and 11 cases of isolated cytomegalovirus retinitis. All patients were ambulatory outpatients at the time of ophthalmologic examination. Noninfectious retinopathy and intraocular opportunistic infections suggest the diagnosis of HIV infection and AIDS, and the ophthalmologist may play an important role in early diagnosis of this disease.     

Survival of patients with the acquired immune deficiency syndrome after development of cytomegalovirus retinopathy. UCLA CMV Retinopathy Study Group

To study the survival of patients with acquired immune deficiency syndrome (AIDS) who develop cytomegalovirus (CMV) retinopathy, the medical records of 100 consecutive patients with AIDS and CMV retinopathy were reviewed Data of AIDS diagnosis, CMV retinopathy diagnosis, and death were determined for each patient. The median interval from CMV retinopathy diagnosis to death for patients whose infection was diagnosed between May 1984 and September 1987 was 5 months. The interval had increased significantly since 1981. The interval from AIDS diagnosis to CMV retinopathy diagnosis (median, 9 months) did not increase. Based on extent and location of retinal lesions at the time patients were first examined, increased survival could not be attributed to earlier diagnosis of CMV retinopathy. Patients treated with ganciclovir lived longer after diagnosis of CMV retinopathy (median, 7 months) than untreated patients (median, 2 months; P less than 0.001). Although this finding suggests that ganciclovir may prolong survival, the effect of treatment cannot be established conclusively because patients were not randomly assigned to treatment or no treatment groups. The location of retinal lesions had no apparent prognostic significance for survival. Survival after diagnosis of AIDS was significantly shorter if CMV retinopathy was the initial manifestation of the syndrome.     

人类免疫缺陷病毒感染及获得性免疫缺陷综合征患者眼部病变的诊断与治疗

目的 探讨人类免疫缺陷病毒(HIV)感染及获得性免疫缺陷综合征(AIDS)患者的眼部病变特点、临床症状及治疗原则.方法 回顾性系列病例研究.回顾性分析110例(220只眼)HIV感染和AIDS患者的临床资料,包括患者视力、眼前节、眼底检查和荧光素眼底血管造影及外周血CD_4~+T淋巴细胞检测结果,其中2例(4只眼)AIDS合并巨细胞病毒(CMV)性视网膜炎患者施行了更昔洛韦玻璃体腔注药治疗.患者年龄、HIV感染时间与HIV视网膜病变及CMV性视网膜炎的相关性采用Pearson相关分析法,性别与HIV视网膜病变及CMV性视网膜炎的相关性采用Pearson ChiSquare分析法,正常眼底组、HIV视网膜病变组、CMV性视网膜炎组间CD_4~+T淋巴细胞计数比较采用多个独立样本的秩和检验.结果 患者初诊视力为无光感者5只眼,光感至0.04者10只眼,0.05～0.2者14只眼,0.3～0.7者62只眼,0.8及以上者129只眼.110例(220只眼)HIV感染和AIDS患者中,有25只眼角膜后有灰白色细小或色素性沉着物.22只眼房水闪光(+)或(++).4只眼虹膜后粘连.28只眼晶状体混浊.34只眼确诊为HIV视网膜病变,眼底表现为棉絮斑、视网膜出血及微血管瘤.32只眼确诊为AIDS合并CMV性视网膜炎,26只眼的眼底表现为沿血管分布的浓厚黄白色病损区,其上片状出血,边缘有不规则黄白色颗粒.3只眼为眼底病变晚期,表现为视网膜萎缩、视网膜血管硬化和狭窄、视神经萎缩.3只眼合并视网膜脱离.正常眼底的HIV感染者及AIDS患者CD_4~+T淋巴细胞计数中位数为100.0个/mm~3,HIV视网膜病变患者CD_4~+T淋巴细胞计数中位数为41.0个/mm~3,CMV性视网膜炎患者CD_4~+T淋巴细胞计数中位数为18.0个/mm~3.CD_4~+T淋巴细胞计数比较,正常眼底组与HIV视网膜病变组相比,差异有统计学意义(x~2=4.848,P=0.028);正常眼底组与CMV性视网膜炎组相比,差异有统计学意义(x~2=15.696,P=0.000);HIV视网膜病变组与CMV性视网膜炎组相比,差异有统计学意义(x~2=4.860,P=0.027).2例(4只眼)CMV性视网膜炎患者行更昔洛韦(400 μg)玻璃体腔注药后,视力提高,眼底病变明显消退.结论 视网膜微血管病变是HIV感染及AIDS常见的眼部并发症,CMV性视网膜炎是AIDS晚期最严重的眼部并发症.高效抗逆转录病毒治疗可重建患者的免疫功能,更昔洛韦玻璃体腔注药可有效治疗CMV性视网膜炎并挽救患者视力.     

急性视网膜坏死综合征诊治新进展

急性视网膜坏死综合征(acute retinal necrosis syndrome,ARNS)是以急性葡萄膜炎、闭塞性视网膜动脉血管炎、融合性坏死性视网膜炎及后期的视网膜脱离为主要临床表现的一组眼部综合征,部分患者早期可合并有眼压升高.目前病因及发病机制尚不完全明确,多认为可能和病毒感染有关,主要以单纯疱疹病毒(herpes simplex virus,HSV)、水痘-带状疱疹病毒(varicella-zoster virus,VZV)、EB病毒及巨细胞病毒(cytomegalovirus,CMV)等病毒感染为主,诊断主要依靠临床表现、专科检查及病原学检查等.急性视网膜坏死综合征发病急,进展速度快,早期临床表现缺乏特异性,误诊率高,治疗棘手,预后较差,是眼部致盲的重要眼病,一旦确诊,及时给予局部及全身抗病毒、预防性视网膜激光光凝及必要时需行玻璃体切除术联合硅油填充术治疗.研究表明,早期有效的治疗措施,可阻止病情进展,提高患者视力.因此,早期确诊及治疗急性视网膜坏死综合征非常重要,本文将结合文献对急性视网膜坏死综合征的诊断及治疗进行综述.     

Arterial obstruction and ocular neovascularization

A series of 12 patients (13 eyes) with neovascular glaucoma in association with arterial obstructive disease is presented. Appropriately characterized as having an ocular ischemic syndrome, the anterior segment findings in each included aqueous flare and rubeosis iridis. Posterior segment manifestations included midperipheral intraretinal hemorrhages (venous stasis retinopathy), narrowed retinal arteries, often a cherry red spot, and neovascularization of the disc and/or retina. Most eyes with the ocular ischemic syndrome have either ipsilateral common carotid artery obstruction or severe bilateral obstruction of the internal carotid arteries.     

Progression of varicella-zoster virus necrotizing retinopathy in an HIV-negative patient with transient immune deviation

Purpose To report a case of unilateral varicella-zoster virus (VZV) necrotizing retinopathy that progressed from outer retinitis with features of progressive outer retinal necrosis (PORN) to typical acute retinal necrosis (ARN) in an HIV-negative patient with a transient decrease in CD4 lymphocyte counts and CD4/CD8 ratio.Method Case report.Results A 41-year-old Chinese man presenting with blurred vision in the right eye was diagnosed with herpetic necrotizing retinitis without vitritis. Fundus examination revealed retinal arteritis and extensive deep whitish retinal lesions in the mid-periphery with minimal vitritis. Aqueous humor and vitreous PCR were positive for VZV. His CD4 count on presentation was depressed (239 cells/ul) and the CD4/CD8 ratio was low (0.8). The referring ophthalmologist had treated him with prednisolone 60 mg/day. At our institution, when intravenous acyclovir was started and the steroid therapy discontinued, he developed severe vitritis and the deep retinal lesions progressed to full-thickness retinitis typical of ARN. Repeat CD4 count was 512 cells/ul at day 14. In total, he was treated with 14 days of IV acyclovir (12 mg/kg 8-hourly) followed by oral valaciclovir 500 mg three times a day for 3 months. Prednisolone 30 mg once daily was restarted and tapered over 3 months. Despite prophylactic argon retinal photocoagulation to the edge of the retinitis, the patient developed a total retinal detachment at 3 months.Conclusions VZV retinal infection in an HIV-negative patient with transient immune deviation can manifest initially as outer retinitis with features similar to PORN and progress to typical ARN when CD4 counts return to normal.No financial support was received for this paper. The authors have no proprietary interests in the drugs and equipment used in this paper.     

Ocular toxoplasmosis in patients with the acquired immunodeficiency syndrome

In seven of eight cases of presumed ocular toxoplasmosis in patients with AIDS, the diagnosis was supported by a reduction or resolution of intraocular inflammation and healing of necrotic retinal lesions after initiation of antiparasitic drug therapy including one or more of the following medications: pyrimethamine, sulfadiazine, clindamycin, tetracycline, or spiramycin. In two cases the diagnosis was confirmed histologically. The cases differed clinically and histopathologically from those in immunocompetent patients. There was no evidence that disease originated in preexisting retinochoroidal scars. Lesions frequently were bilateral and multifocal. Vitreous inflammatory reaction was a common clinical finding, but histopathologic examination demonstrated scant retinal inflammation in areas of necrosis. Ocular toxoplasmosis in these patients with AIDS probably resulted from newly acquired infection or dissemination of organisms from nonocular sites of disease. Infections became clinically inactive with drug therapy in all treated patients, but reactivation and progression of disease occurred when therapy was stopped in two of three patients. Severe retinal necrosis led to retinal tears or detachment in three cases. Ocular lesions were the first manifestation of Toxoplasma gondii infection in four of five patients with evidence of multisystem infection.