Acute Retinal Necrosis 

  • Author: Andrew A Dahl, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Apr 29, 2011
 

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.

A necrotic retina is shown in the image below.

The white area is necrotic retina. The white area is necrotic retina.
Next

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.

Previous
Next

Epidemiology

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.

Previous
 
 
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 and Vision Sciences, Director of Fellowship Programs, Department of Ophthalmology and Vision Sciences, St Michael's Hospital, University of Toronto Faculty of Medicine, 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; Labtician Consulting fee Consulting; Diagnos Royalty 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.

Specialty Editor Board

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.

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

Disclosure: Medscape Salary Employment

Steve Charles, MD  Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians and Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong

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 Other; Topcon Medical Lasers Consulting fee Consulting

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.

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. Wensing B, de Groot-Mijnes JD, Rothova A. Necrotizing and nonnecrotizing variants of herpetic uveitis with posterior segment involvement. Arch Ophthalmol. Apr 2011;129(4):403-8. [Medline].

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

  4. 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].

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

  6. 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].

  7. 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].

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

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

  10. 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].

  11. 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].

  12. 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].

  13. Hillenkamp J, Nolle B, Bruns C, Rautenberg P, Fickenscher H, Roider J. Acute retinal necrosis: clinical features, early vitrectomy, and outcomes. Ophthalmology. Oct 2009;116(10):1971-5.e2. [Medline].

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

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

  16. 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].

  17. 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].

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

  19. 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].

  20. 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].

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

  22. 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].

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

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

Previous
Next
 
The white area is necrotic retina.
Severe vitritis with occlusive arteriolitis.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.