History

Typically, acute retinal necrosis is a disease of immunocompetent individuals. Initially, patients may complain of the following:

Red eye
Periorbital pain
Hazy vision or decreased vision
Appearance of floaters
Decreased color vision
Other areas of previous infections - Primary varicella infections, herpes zoster

Infection with herpes simplex virus (HSV-1 or HSV-2) and VZV can cause widely variable clinical manifestations, ranging from the typical severe acute retinal necrosis to more atypical, slowly progressing necrotizing and nonnecrotizing inflammation.^[5]

In the era prior to the advent of antiviral therapy, bilateral involvement occurred in one third to three quarters of cases. The commencement of second eye infection ranged from 24 hours to 6 months months after the first eye’s involvement.^[6]

Physical

Examination findings may include the following:

Anterior uveitis (iritis)
Panuveitis
Episcleritis or scleritis
Keratic precipitates - Fine or granulomatous
Occlusive retinal vasculitis involving arteries and veins
One or more focus of retinitis, resulting in necrosis with discrete borders located in the retinal periphery with circumferential spread
Vitritis (shown in the image below)
Optic neuropathy

Severe vitritis with occlusive arteriolitis.
View Media Gallery

Causes

Most cases of acute retinal necrosis have been reported to be caused by the following^[7]:

Varicella-zoster virus
Herpes simplex type 1
Herpes simplex type 2^[8]

Physical Examination

Careful and complete ophthalmological examination should be undertaken in all patients with anterior uveitis. Fundoscopy following pupillary dilation should include examination of the peripheral retina to search for areas of retinal whitening or vasculitis.

Complications

Retinal detachment is the principal complication of ARN. Prompt diagnosis and early treatment decreases the risk of retinal detachment and improves visual acuity outcomes.^[9]

Differential Diagnoses

Muthiah MN, Michaelides M, Child CS, Mitchell SM. Acute retinal necrosis: a national population-based study to assess the incidence, methods of diagnosis, treatment strategies and outcomes in the UK. Br J Ophthalmol. 2007 Nov. 91(11):1452-5. [QxMD MEDLINE Link]. [Full Text].
Lau CH, Missotten T, Salzmann J, Lightman SL. Acute retinal necrosis features, management, and outcomes. Ophthalmology. 2007 Apr. 114(4):756-62. [QxMD MEDLINE Link].
Usui Y, Takeuchi M, Goto H, Mori H, Kezuka T, Sakai J. Acute retinal necrosis in Japan. Ophthalmology. 2008 Sep. 115(9):1632-3. [QxMD MEDLINE Link].
Tibbetts MD, Shah CP, Young LH, Duker JS, Maguire JI, Morley MG. Treatment of acute retinal necrosis. Ophthalmology. 2010 Apr. 117(4):818-24. [QxMD MEDLINE Link].
Wensing B, de Groot-Mijnes JD, Rothova A. Necrotizing and nonnecrotizing variants of herpetic uveitis with posterior segment involvement. Arch Ophthalmol. 2011 Apr. 129(4):403-8. [QxMD MEDLINE Link].
Ryan SJ, Schachat AP, Wilkinson CP, Hinton DR, Sadda SR, Wiedemann P. Retina. 5th ed. 2013.
Walters G, James TE. Viral causes of the acute retinal necrosis syndrome. Curr Opin Ophthalmol. 2001 Jun. 12(3):191-5. [QxMD MEDLINE Link].
Moesen I, Khemka S, Ayliffe W. Acute retinal necrosis secondary to herpes simplex virus type 2 with preexisting chorioretinal scarring. J Pediatr Ophthalmol Strabismus. 2008 Jan-Feb. 45(1):59-61. [QxMD MEDLINE Link].
Crapotta JA, Freeman WR, Feldman RM, Lowder CY, Ambler JS, Parker CE, et al. Visual outcome in acute retinal necrosis. Retina. 1993. 13 (3):208-13. [QxMD MEDLINE Link].
Duker JS, Blumenkranz MS. Diagnosis and management of the acute retinal necrosis (ARN) syndrome. Surv Ophthalmol. 1991 Mar-Apr. 35(5):327-43. [QxMD MEDLINE Link].
Holland GN. Standard diagnostic criteria for the acute retinal necrosis syndrome. Executive Committee of the American Uveitis Society. Am J Ophthalmol. 1994 May 15. 117(5):663-7. [QxMD MEDLINE Link].
Sergott RC, Belmont JB, Savino PJ, Fischer DH, Bosley TM, Schatz NJ. Optic nerve involvement in the acute retinal necrosis syndrome. Arch Ophthalmol. 1985 Aug. 103(8):1160-2. [QxMD MEDLINE Link].
Blumenkranz MS, Culbertson WW, Clarkson JG, Dix R. Treatment of the acute retinal necrosis syndrome with intravenous acyclovir. Ophthalmology. 1986 Mar. 93(3):296-300. [QxMD MEDLINE Link].
Aizman A. Treatment of acute retinal necrosis syndrome. Drugs Today (Barc). 2006 Aug. 42(8):545-51. [QxMD MEDLINE Link].
Schoenberger SD, Kim SJ, Thorne JE, Mruthyunjaya P, Yeh S, Bakri SJ, et al. Diagnosis and Treatment of Acute Retinal Necrosis: A Report by the American Academy of Ophthalmology. Ophthalmology. 2017 Mar. 124 (3):382-392. [QxMD MEDLINE Link].
Shantha JG, Weissman HM, Debiec MR, Albini TA, Yeh S. Advances in the management of acute retinal necrosis. Int Ophthalmol Clin. 2015 Summer. 55 (3):1-13. [QxMD MEDLINE Link].
Baltinas J, Lightman S, Tomkins-Netzer O. Comparing Treatment of Acute Retinal Necrosis With Either Oral Valacyclovir or Intravenous Acyclovir. Am J Ophthalmol. 2018 Apr. 188:173-180. [QxMD MEDLINE Link].
Aizman A, Johnson MW, Elner SG. Treatment of acute retinal necrosis syndrome with oral antiviral medications. Ophthalmology. 2007 Feb. 114(2):307-12. [QxMD MEDLINE Link].
Emerson GG, Smith JR, Wilson DJ, Rosenbaum JT, Flaxel CJ. Primary treatment of acute retinal necrosis with oral antiviral therapy. Ophthalmology. 2006 Dec. 113(12):2259-61. [QxMD MEDLINE Link].
Khurana RN, Charonis A, Samuel MA, Gupta A, Tawansy KA. Intravenous foscarnet in the management of acyclovir-resistant herpes simplex virus type 2 in acute retinal necrosis in children. Med Sci Monit. 2005 Dec. 11(12):CS75-8. [QxMD MEDLINE Link].
Palay DA, Sternberg P Jr, Davis J, Lewis H, Holland GN, Mieler WF. Decrease in the risk of bilateral acute retinal necrosis by acyclovir therapy. Am J Ophthalmol. 1991 Sep 15. 112(3):250-5. [QxMD MEDLINE Link].
Hillenkamp J, Nolle B, Bruns C, Rautenberg P, Fickenscher H, Roider J. Acute retinal necrosis: clinical features, early vitrectomy, and outcomes. Ophthalmology. 2009 Oct. 116(10):1971-5.e2. [QxMD MEDLINE Link].
Cochrane TF, Silvestri G, McDowell C, Foot B, McAvoy CE. Acute retinal necrosis in the United Kingdom: results of a prospective surveillance study. Eye (Lond). 2012 Mar. 26 (3):370-7; quiz 378. [QxMD MEDLINE Link].
Kim SJ, Lo WR. Acute retinal necrosis. Ophthalmology. 2008 Jun. 115(6):1104-5; author reply 1105-6. [QxMD MEDLINE Link].
Nussenblatt RB, Palestine AG. Acute retinal necrosis. Uveitis: Fundamentals and Clinical Practice. 1989. 407-14.
Park SS, Holz HA, Ravage ZB, Merrill PT, Nguyen QD. Diagnostic and therapeutic challenges. Acute retinal necrosis syndrom. Retina. 2008 Apr. 28(4):660-4. [QxMD MEDLINE Link].

Media Gallery

The white area is necrotic retina.
Severe vitritis with occlusive arteriolitis.

of 2

Author

Andrew A Dahl, MD, FACS Assistant Professor of Surgery (Ophthalmology), New York College of Medicine (NYCOM); Director of Residency Ophthalmology Training, The Institute for Family Health and Mid-Hudson Family Practice Residency Program; Staff Ophthalmologist, Telluride Medical Center

Andrew A Dahl, MD, FACS is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, American Intraocular Lens Society, American Medical Association, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, Medical Society of the State of New York, New York State Ophthalmological Society, Outpatient Ophthalmic Surgery Society

Disclosure: Nothing to disclose.

Coauthor(s)

David T Wong, MD, FRCSC Associate Professor of Ophthalmology and Vision Sciences, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Ophthalmologist-in-Chief, St Michael's Hospital, Canada

David T Wong, MD, FRCSC is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Society of Retina Specialists, Association for Research in Vision and Ophthalmology, Canadian Medical Association, Canadian Ophthalmological Society, College of Physicians and Surgeons of Ontario, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Novartis, Alcon, Bayer Received research grant from: Novartis, Alcon, Bayer, Genetech Received consulting fee from Alcon for consulting; Received consulting fee from Novartis for consulting; Received consulting fee from Bayer for consulting; Received consulting fee from Allergan for consulting; Received consulting fee from B & L for consulting.

Saad Waheeb, MB, BCh, FRCSC Consulting Staff, Department of Ophthalmology, King Abdulaziz University Hospital

Saad Waheeb, MB, BCh, FRCSC is a member of the following medical societies: American Academy of Ophthalmology, Canadian Ophthalmological Society, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Steve Charles, MD Founder and CEO of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine

Disclosure: Received royalty and consulting fees for: Alcon Laboratories.

Chief Editor

C Stephen Foster, MD, FACS, FACR, FAAO, FARVO Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution

C Stephen Foster, MD, FACS, FACR, FAAO, FARVO is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association of Immunologists, American College of Rheumatology, American College of Surgeons, American Federation for Clinical Research, American Medical Association, American Society for Microbiology, American Uveitis Society, Association for Research in Vision and Ophthalmology, Massachusetts Medical Society, Royal Society of Medicine, Sigma Xi, The Scientific Research Honor Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Aldeyra Therapeutics (Lexington, MA); Bausch & Lomb Surgical, Inc (Rancho Cucamonga, CA); Eyegate Pharma (Waltham, MA); Novartis (Cambridge, MA); pSivida (Watertown, MA); Xoma (Berkeley, CA); Allakos (Redwood City, CA) Serve(d) as a speaker or a member of a speakers bureau for: Alcon (Geneva, Switzerland); Allergan (Dublin, Ireland); Mallinckrodt (Staines-upon-Thames, United Kingdom) Received research grant from: Alcon; Aldeyra Therapeutics; Allakos Pharmaceuticals; Allergan; Bausch & Lomb; Clearside Biomedical; Dompé pharmaceutical; Eyegate Pharma; Mallinckrodt pharmaceuticals; Novartis; pSivida; Santen; Aciont Stock for: Eyegate Pharma.