eMedicine Specialties > Ophthalmology > Retina

Acute Retinal Necrosis

Author: Andrew A Dahl, MD, Director of Ophthalmology Teaching, Mid-Hudson Family Practice Institute, The Institute for Family Health; Assistant Professor of Surgery (Ophthalmology), New York College of Medicine
Coauthor(s): David T Wong, MD, FRCS(C), Associate Professor of Ophthalmology, Director of Fellowship Programs, Department of Ophthalmology, St Michael's Hospital, Faculty of Medicine, University of Toronto, Canada; Saad Waheeb, MB, BCh, FRCS(C), Consulting Staff, Department of Ophthalmology, King Abdulaziz University Hospital
Contributor Information and Disclosures

Updated: Jan 21, 2009

Introduction

Background

Acute retinal necrosis (ARN) can lead to uveitis, retinal detachment, and blindness. Acute retinal necrosis was first described in the Japanese literature in 1971 and termed Kirisawa uveitis. During the past 3 decades, acute retinal necrosis syndrome has been a source of fear, frustration, and fascination for many ophthalmologists. Unfortunately, it usually is a visually devastating condition for the patient.


The white area is necrotic retina.

The white area is necrotic retina.

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The white area is necrotic retina.

The white area is necrotic retina.


Pathophysiology

Acute retinal necrosis may be a result of dormant herpes simplex virus 1 (HSV-1), herpes simplex virus 2 (HSV-2), or varicella-herpes zoster virus (VZV) viral reactivation in the retina. The exact etiology of this reactivation is still elusive; however, an immunogenetic predisposition to the disease is likely.

Frequency

United States

Acute retinal necrosis accounts for 5.5% of uveitis cases over a 10-year period.1

International

In Switzerland, acute retinal necrosis accounts for 1.7% of uveitic cases.

Mortality/Morbidity

Significant visual loss may occur. Retinal detachment complicates most cases (~75%) and is a major cause of legal blindness in acute retinal necrosis.

Race

No clear racial predilection exists.

Sex

This condition appears to have a predilection for males; however, the extent is not clear.

Age

Acute retinal necrosis is a disease of young healthy individuals aged 20-50 years.

  • A bimodal age distribution possibly exists, peaking at approximately ages 20 and 50 years. This distribution may be related to differences in etiologic agents.
    • When varicella-zoster virus or herpes simplex virus type 1 is involved, the median age is 57 and 47 years, respectively.
    • When herpes simplex virus type 2 is involved, the median age is 20 years.

Clinical

History

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

  • Red eye
  • Periorbital pain
  • Hazy decreased vision
  • Other areas of previous infections
    • Primary varicella infections
    • Herpes zoster

Physical

  • Episcleritis or scleritis
  • Keratic precipitates
    • Fine
    • 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
  • Optic neuropathy
Severe vitritis with occlusive arteriolitis.

Severe vitritis with occlusive arteriolitis.

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Severe vitritis with occlusive arteriolitis.

Severe vitritis with occlusive arteriolitis.


Causes

Most cases of acute retinal necrosis have been reported to be caused by the following:2

  • Varicella-zoster virus
  • Herpes simplex type 1
  • Herpes simplex type 23

More on Acute Retinal Necrosis

Overview: Acute Retinal Necrosis
Differential Diagnoses & Workup: Acute Retinal Necrosis
Treatment & Medication: Acute Retinal Necrosis
Follow-up: Acute Retinal Necrosis
Multimedia: Acute Retinal Necrosis
References
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References

  1. 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. Nov 2007;91(11):1452-5. [Medline].

  2. Walters G, James TE. Viral causes of the acute retinal necrosis syndrome. Curr Opin Ophthalmol. Jun 2001;12(3):191-5. [Medline].

  3. Moesen I, Khemka S, Ayliffe W. Acute retinal necrosis secondary to herpes simplex virus type 2 with preexisting chorioretinal scarring. J Pediatr Ophthalmol Strabismus. Jan-Feb 2008;45(1):59-61. [Medline].

  4. Duker JS, Blumenkranz MS. Diagnosis and management of the acute retinal necrosis (ARN) syndrome. Surv Ophthalmol. Mar-Apr 1991;35(5):327-43. [Medline].

  5. Holland GN. Standard diagnostic criteria for the acute retinal necrosis syndrome. Executive Committee of the American Uveitis Society. Am J Ophthalmol. May 15 1994;117(5):663-7. [Medline].

  6. Sergott RC, Belmont JB, Savino PJ, Fischer DH, Bosley TM, Schatz NJ. Optic nerve involvement in the acute retinal necrosis syndrome. Arch Ophthalmol. Aug 1985;103(8):1160-2. [Medline].

  7. Aizman A. Treatment of acute retinal necrosis syndrome. Drugs Today (Barc). Aug 2006;42(8):545-51. [Medline].

  8. Aizman A, Johnson MW, Elner SG. Treatment of acute retinal necrosis syndrome with oral antiviral medications. Ophthalmology. Feb 2007;114(2):307-12. [Medline].

  9. Blumenkranz MS, Culbertson WW, Clarkson JG, Dix R. Treatment of the acute retinal necrosis syndrome with intravenous acyclovir. Ophthalmology. Mar 1986;93(3):296-300. [Medline].

  10. Emerson GG, Smith JR, Wilson DJ, Rosenbaum JT, Flaxel CJ. Primary treatment of acute retinal necrosis with oral antiviral therapy. Ophthalmology. Dec 2006;113(12):2259-61. [Medline].

  11. 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. Dec 2005;11(12):CS75-8. [Medline].

  12. Lau CH, Missotten T, Salzmann J, Lightman SL. Acute retinal necrosis features, management, and outcomes. Ophthalmology. Apr 2007;114(4):756-62. [Medline].

  13. Carney MD, Peyman GA, Goldberg MF, et al. Acute retinal necrosis. Retina. Spring-Summer 1986;6(2):85-94. [Medline].

  14. Fisher JP, Lewis ML, Blumenkranz M, et al. The acute retinal necrosis syndrome. Part 1: Clinical manifestations. Ophthalmology. Dec 1982;89(12):1309-16. [Medline].

  15. Gariano RF, Berreen JP, Cooney EL. Progressive outer retinal necrosis and acute retinal necrosis in fellow eyes of a patient with acquired immunodeficiency syndrome. Am J Ophthalmol. Sep 2001;132(3):421-3. [Medline].

  16. Nussenblatt RB, Palestine AG. Acute retinal necrosis. In: Uveitis: Fundamentals and Clinical Practice. 1989:407-14.

  17. Park SS, Holz HA, Ravage ZB, Merrill PT, Nguyen QD. Diagnostic and therapeutic challenges. Acute retinal necrosis syndrome. Retina. Apr 2008;28(4):660-4. [Medline].

  18. Rodriguez A, Calonge M, Pedroza-Seres M, et al. Referral patterns of uveitis in a tertiary eye care center. Arch Ophthalmol. May 1996;114(5):593-9. [Medline].

  19. Severin M, Neubauer H. Bilateral acute vascular retinal necrosis. Ophthalmologica. 1981;182(4):199-203. [Medline].

  20. Tan JCH, Byles D, Stanford MR, Frith PA, Graham EM. Acute retinal necrosis in children caused by herpes simplex virus. Retina. 2001;21(4):344-7. [Medline].

  21. Urayama A, Yamada N, Sasaki T. Unilateral acute uveitis with retinal periarteritis and detachment. Jpn J Clin Ophthalmol. 1971;25:607.

  22. Young NJ, Bird AC. Bilateral acute retinal necrosis. Br J Ophthalmol. Sep 1978;62(9):581-90. [Medline].

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Further Reading

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Keywords

ARN, bilateral acute retinal necrosis, BARN, bilateral ARN, Kirisawa's uveitis, Kirisawa uveitis, blindness, retinal detachment, uveitis, red eye, eye pain, periorbital pain, decreased vision, vision problems, herpes simplex virus 1, HSV-1, herpes simplex virus 2, HSV-2, varicella-herpes zoster virus, VZV

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Contributor Information and Disclosures

Author

Andrew A Dahl, MD, Director of Ophthalmology Teaching, Mid-Hudson Family Practice Institute, The Institute for Family Health; Assistant Professor of Surgery (Ophthalmology), New York College of Medicine
Andrew A Dahl, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Coauthor(s)

David T Wong, MD, FRCS(C), Associate Professor of Ophthalmology, Director of Fellowship Programs, Department of Ophthalmology, St Michael's Hospital, Faculty of Medicine, University of Toronto, Canada
David T Wong, MD, FRCS(C) 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, and Royal College of Physicians and Surgeons of Canada
Disclosure: Alcon Consulting fee Consulting; Novartis Consulting fee Consulting; Alcon Grant/research funds Other; Labtician Consulting fee Consulting

Saad Waheeb, MB, BCh, FRCS(C), Consulting Staff, Department of Ophthalmology, King Abdulaziz University Hospital
Saad Waheeb, MB, BCh, FRCS(C) is a member of the following medical societies: American Academy of Ophthalmology, Canadian Ophthalmological Society, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Medical Editor

Brian A Phillpotts, MD, Former Vitreo-Retinal Service Director, Former Program Director, Clinical Assistant Professor, Department of Ophthalmology, Howard University College of Medicine
Brian A Phillpotts, MD is a member of the following medical societies: American Academy of Ophthalmology, American Diabetes Association, American Medical Association, and National Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine
Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society
Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Consulting

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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