Acute retinal necrosis (ARN) syndrome is a devastating intraocular inflammatory disease. It is characterized by anterior uveitis, vitritis, and necrotizing retinitis that rapidly extend posteriorly from the peripheral retina.1 Antiviral treatment, as the most common treatment, has been proven therapeutically effective, but the effectiveness of other adjunctive treatment options, such as prophylactic laser and early vitrectomy, remains unclear.2 Secondary retinal detachment (RD) is a frequent late complication of ARN syndrome. Here, we describe a case of RD following ARN, which was treated with pars plana vitrectomy (PPV), silicone oil tamponade, silicone oil replacement, cataract extraction, and foldable capsular vitreous body (FCVB; Guangzhou Vesber Biotechnology Co. Ltd., Guangzhou, China) implantation successively.
A 35-year-old male presented with a 9-day history of vision loss, floaters, and pain in his left eye. Exam at admission revealed best-corrected visual acuity (BCVA) of 20/20 in right eye and 20/40 in the left with normal intraocular pressure (IOP). Slit-lamp examination showed 1+ aqueous cell and mutton-fat keratic precipitates on the endothelium. Funduscopy showed swollen optic disk and retinal arteriolar sheathings, with extensive white-yellowish retinal lesions and hemorrhages in peripheral retina (Figure 1). No abnormality of anterior segment or fundus was found in the right eye.
Swollen optic disk and retinal arteriolar sheathings in the left eye, with extensive white-yellowish retinal lesions and hemorrhages in the peripheral retina.
Fluorescein fundus angiography of the left eye indicated occlusive retinal vasculitis (Figure 2). An extensive array of laboratory workup revealed that the patient was immunoglobulin M (IgM)- and IgG-positive for herpes simplex virus (HSV) and IgM-negative and IgG-positive for cytomegalovirus (CMV).
Fluorescein fundus angiography showed widespread areas of nonperfusion in peripheral retina, which indicated occlusive retinal vasculitis.
A diagnosis of unilateral ARN was made and was treated with intravenous acyclovir (10 mg/kg at every 8 hours), corticosteroids (oral prednisone 50 mg per day and prednisolone acetate 1% eye drops), intravitreal injection (ganciclovir [Zirgan; Bausch + Lomb, Rochester, NY] 0.8 mg per week), and laser.
In the next month, retinal necrosis in the left eye significantly resolved, but similar lesions were found in the right. The right eye was also diagnosed with ARN and was given intravitreal injection and photocoagulation. After treatment, funduscopic lesions of the right eye gradually improved and remained stable in future follow-up.
At Day 44 of admission, RD was found in the left eye, which was successfully treated with PPV and silicone oil tamponade. In about a year, cataract of the left eye evolved. Due to the widespread necrosis lesions and defects of retina, we performed silicone oil replacement along with cataract extraction to avoid recurrent RD and possible silicone oil emulsification.
About 2 years after the initial presentation, the patient returned with a complaint about eye pain. His BCVA was 20/20 in the right eye and hand movement (HM) in the left. IOPs were 17 mm Hg and 48 mm Hg in the right and left eyes, respectively. Slit-lamp examination of the left eye showed severe flat peripheral anterior chamber, total adhesion of the iris, and lens capsule. Fundal condition was not clear because of the lens posterior capsular opacity. With IOP not able to be satisfactorily controlled, we considered it to be glaucoma secondary to silicone oil emulsification and planned its surgical removal. Proliferative vitreoretinopathy (PVR) was found intraoperatively. Given the severity of PVR, we implanted FCVB with silicone oil injection after epiretinal membrane peeling.
One week after the implantation, BCVA of the left eye was HM and IOP was 9 mm Hg, with normal-depth anterior chamber, dilated pupil, FCVB in place, and attached retina. And his condition remained stable afterwards (Figure 3).
Photographs of the left eye 1 month after the implantation. (A) Normal-depth anterior chamber, dilated pupil, and foldable capsular vitreous body in place. (B) Attached retina, widespread necrosis lesions, and defects in periphery.
ARN syndrome was first reported by Japanese researchers in 1971.3 In 1994, the American Uveitis Society4 defined the diagnostic criteria of ARN based on its clinical characteristics: (1) one or more retinal necrosis lesions with discrete borders in periphery; (2) rapid progression without antiviral treatment; (3) circumferential spread; (4) occlusive vasculopathy with arterial involvement; (5) inflammatory reaction in the vitreous and anterior chamber. However, evidence of etiology was not included in the diagnostic criteria until 2015, using polymerase chain reaction (PCR) to detect intraocular fluid.5 With the help of PCR, antibody testing, viral culture, biopsy and immunocytochemistry, evidence suggests that virus such as Varicella-zoster virus, HSV, CMV, and toxoplasmosis may be the infectious agent causing the disease.6
Bilateral disease may appear in up to one-third of patients, and the contralateral involvement often occurs within weeks.7 In the presented case, the disease was unilateral at first, and the patient's right eye was asymptomatic when necrosis foci were found. Therefore, we proposed the importance of dilated funduscopic exam of both eyes, and we believed that the good visual result of the right eye may be explained by the prompt diagnosis and proper and early treatment.
Necrosis lesions may spread even with adequate diagnosis and antiviral treatment, so the incidence of RD in ARN patients is high and it can occur even after the resolution of ARN.8,9 Vitrectomy with silicone oil tamponade is mandatory to achieve retinal reattachment for rhegmatogenous and traction RD with multiple retinal tears after the fulminant type of ARN, but only 23% can successfully reattach.10 PPV can effectively remove virus, necrosis, inflammatory factors, relieve vitreous traction, and create a chance for complete laser photocoagulation. However, researchers found that PVR developed in nearly every patient which resulted in recurrent RD.11 As a result, there are a considerable number of patients that cannot remove silicone oil without risking recurrent RD.
Although intravitreal silicone oil works as an effective endotamponade, complications such as emulsification, cataract, and secondary glaucoma may occur. In the presented case, the patient returned with IOP elevation, which might be caused by emulsified silicone oil migrating into the anterior chamber and obstructing the trabecular meshwork. Silicone oil emulsification is a multifactorial process in which the biochemical properties of silicone oil, endotamponade duration, and ocular inflammation all play roles.12
FCVB was brought to us as a new vitreous substitute to avoid complications caused by silicone oil. The FCVB consists of a thin vitreous-shaped capsule with a tube-valve system (Figure 4). While silicone oil is injected into the capsule to support the retina, the tube-valve system allows control of the IOP.13 Several studies have shown that the FCVB was flexible, effective, and safe as a vitreous substitute after long-term observation. In addition, it was proven to have little effect on the function of the ciliary body, and can effectively prevent eyeball atrophy after implantation.14–17
The foldable capsular vitreous body consists of a thin, vitreous-shaped capsule with a tube-valve system.
In this paper, we demonstrate the evolution of an ARN case and how RD was developed even after treatment as the disease processed itself. Additionally, FCVB, a novel vitreous substitute, should be considered as an application for ARN with severe RD or PVR.
- Hillenkamp J, Nölle B, Bruns C, Rautenberg P, Fickenscher H, Roider J. Acute retinal necrosis: clinical features, early vitrectomy, and outcomes. Ophthalmology. 2009;116(10):1971–5.e2. doi:10.1016/j.ophtha.2009.03.029 [CrossRef] PMID:19592111
- Schoenberger SD, Kim SJ, Thorne JE, et al. Diagnosis and Treatment of Acute Retinal Necrosis: A Report by the American Academy of Ophthalmology. Ophthalmology. 2017;124(3):382–392. doi:10.1016/j.ophtha.2016.11.007 [CrossRef] PMID: 28094044
- Urayama A, Yamada N, Sasaki T, et al. Unilateral acute uveitis with retinal periarteritis and detachment. Jpn J Clin Ophthalmol. 1971;25:607–619.
- Holland GNExecutive Committee of the American Uveitis Society. Standard diagnostic criteria for the acute retinal necrosis syndrome. Am J Ophthalmol. 1994;117(5):663–667. doi:10.1016/S0002-9394(14)70075-3 [CrossRef] PMID:8172275
- Takase H, Okada AA, Goto H, et al. Development and validation of new diagnostic criteria for acute retinal necrosis. Jpn J Ophthalmol. 2015;59(1):14–20. doi:10.1007/s10384-014-0362-0 [CrossRef] PMID:25492579
- Chen S, Malik KJ, Shen D, Chan CC. Acute Retinal Necrosis with Multiple Viral Infections: A Case Report. Int J Ophthalmic Pathol. 2014;3(5):1000151 doi:10.4172/2324-8599.1000151 [CrossRef] PMID:25584358
- Donovan CP, Levison AL, Lowder CY, Martin DF, Srivastava SK. Delayed recurrence of acute retinal necrosis (ARN): A case series. J Clin Virol. 2016;80:68–71. doi:10.1016/j.jcv.2016.04.021 [CrossRef] PMID:27179886
- Usui Y, Goto H. Overview and diagnosis of acute retinal necrosis syndrome. Semin Ophthalmol. 2008;23(4):275–283. doi:10.1080/08820530802111325 [CrossRef] PMID:18584564
- Matsuo T. Vitrectomy and silicone oil tamponade as an initial surgery for retinal detachment after acute retinal necrosis syndrome. Ocul Immunol Inflamm. 2005;13(1):91–94. doi:10.1080/09273940490518838 [CrossRef] PMID:15804777
- Marrocos de Aragão RE, Barreira IM, Arrais BL, Pereira LA, Ramos CS. Good visual outcome in an immunocompromised patient with bilateral acute retinal necrosis syndrome: A case report. Saudi J Ophthalmol. 2014;28(3):243–246. doi:10.1016/j.sjopt.2013.11.005 [CrossRef] PMID:25278806
- Liu S, Wang D, Zhang X. The necessity and optimal time for performing pars plana vitrectomy in acute retinal necrosis patients. BMC Ophthalmol. 2018;18(1):15 doi:10.1186/s12886-018-0674-9 [CrossRef] PMID:29357831
- Qian Z, Xu K, Kong X, Xu H. Ahmed Glaucoma Valves versus EXPRESS Devices in Glaucoma Secondary to Silicone Oil Emulsification. J Ophthalmol. 2018;2018:8539689 doi:10.1155/2018/8539689 [CrossRef] PMID:30026986
- Gao Q, Mou S, Ge J, et al. A new strategy to replace the natural vitreous by a novel capsular artificial vitreous body with pressure-control valve. Eye (Lond). 2008;22(3):461–468. doi:10.1038/sj.eye.6702875 [CrossRef] PMID:17525767
- Lin X, Ge J, Gao Q, et al. Evaluation of the flexibility, efficacy, and safety of a foldable capsular vitreous body in the treatment of severe retinal detachment. Invest Ophthalmol Vis Sci. 2011;52(1):374–381. doi:10.1167/iovs.10-5869 [CrossRef] PMID:20811065
- Lin X, Sun X, Wang Z, et al. Three-Year Efficacy and Safety of a Silicone Oil-Filled Foldable-Capsular-Vitreous-Body in Three Cases of Severe Retinal Detachment. Transl Vis Sci Technol. 2016;5(1):2 doi:10.1167/tvst.5.1.2 [CrossRef] PMID:26855843
- Zhang R, Wang T, Xie C, et al. Evaluation of supporting role of a foldable capsular vitreous body with magnetic resonance imaging in the treatment of severe retinal detachment in human eyes. Eye (Lond). 2011;25(6):794–802. doi:10.1038/eye.2011.61 [CrossRef] PMID:21423138
- Wang P, Gao Q, Jiang Z, et al. Biocompatibility and retinal support of a foldable capsular vitreous body injected with saline or silicone oil implanted in rabbit eyes. Clin Exp Ophthalmol. 2012;40(1):e67–e75. doi:10.1111/j.1442-9071.2011.02664.x [CrossRef] PMID:21883770