Acute retinal necrosis: Difference between revisions

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__NOTOC__
__NOTOC__
{{SI}}
{{Acute retinal necrosis}}
{{CMG}}; {{AE}} {{Faizan}}; {{LRO}}


==Overview==
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
'''Acute retinal necrosis''' is a type of [[retinitis]] which can be associated with viral infections.


It was first characterized in 1971.<ref name="titleeMedicine - Acute Retinal Necrosis : Article by Andrew A Dahl, MD">{{cite web |url=http://www.emedicine.com/oph/topic377.htm |title=eMedicine - Acute Retinal Necrosis : Article by Andrew A Dahl, MD |accessdate=2008-02-05 |work=| archiveurl= http://web.archive.org/web/20080216011141/http://www.emedicine.com/oph/topic377.htm| archivedate= 16 February 2008 <!--DASHBot-->| deadurl= no}}</ref><ref name ="Urayama">Urayama A, Yamada N, Sasaki T: Unilateral acute uveitis with retinal periarteritis and detachment. Jpn J Clin Ophthalmol 1971; 25: 607.</ref>
{{CMG}}


One study indicated an incidence of 1 per 1.6 to 2.0 million.<ref name="pmid17504853">{{cite journal |author=Muthiah MN, Michaelides M, Child CS, Mitchell SM |title=Acute retinal necrosis: a national population‐based study to assess the incidence, methods of diagnosis, treatment strategies and outcomes in the UK |journal=Br J Ophthalmol |volume=91 |issue=11 |pages=1452–5 |year=2007 |pmid=17504853 |doi=10.1136/bjo.2007.114884 |url=http://bjo.bmj.com/cgi/pmidlookup?view=long&pmid=17504853 |pmc=2095441}}</ref>
{{SK}}  


==Historical Perspective==
==[[Acute retinal necrosis overview|Overview]]==
*Acute [[retinal]] [[necrosis]] was first officially classified as bilateral acute [[retinal]] [[necrosis]] in 1978 by N.J. Young and A.C. Bird.<ref name="pmid708676">{{cite journal |vauthors=Young NJ, Bird AC |title=Bilateral acute retinal necrosis |journal=Br J Ophthalmol |volume=62 |issue=9 |pages=581–90 |year=1978 |pmid=708676 |pmc=1043304 |doi= |url=}}</ref>
**The classification was applied to 4 cases of bilateral necrotizing [[retinitis]], of which the patients developed bilateral confluent [[retinitis]] progressing to [[retinal detachment]] and [[phthisis]] despite [[corticosteroid]] and [[antibiotic]] therapy.<ref name="pmid24385671">{{cite journal |vauthors=Flaxel CJ, Yeh S, Lauer AK |title=Combination systemic and intravitreal antiviral therapy in the management of acute retinal necrosis syndrome (an American Ophthalmological Society thesis) |journal=Trans Am Ophthalmol Soc |volume=111 |issue= |pages=133–44 |year=2013 |pmid=24385671 |pmc=3868412 |doi= |url=}}</ref>
*The first extension of the classification of acute [[retinal]] [[necrosis]] to unilateral cases was given in 1983 by Hayasaka S. et al.<ref name="pmid6860612">{{cite journal |vauthors=Hayasaka S, Asano T, Yabata K, Ide A |title=Acute retinal necrosis |journal=Br J Ophthalmol |volume=67 |issue=7 |pages=455–60 |year=1983 |pmid=6860612 |pmc=1040094 |doi= |url=}}</ref>
**They identified that cases of bilateral acute [[retinal]] [[necrosis]] and cases of Kirisawa-type [[uveitis]] presented nearly identical characteristics:<ref name ="Urayama">Urayama A, Yamada N, Sasaki T: Unilateral acute uveitis with retinal periarteritis and detachment. Jpn J Clin Ophthalmol 1971; 25: 607.</ref><ref name="pmid708676">{{cite journal |vauthors=Young NJ, Bird AC |title=Bilateral acute retinal necrosis |journal=Br J Ophthalmol |volume=62 |issue=9 |pages=581–90 |year=1978 |pmid=708676 |pmc=1043304 |doi= |url=}}</ref>
***Periarteritis
***Opaque, dense [[vitreous]]
***Peripheral [[retinal]] [[exudates]]
***[[Retinal detachment]]
***[[Vision loss]]
***Resistance to [[antibiotic]] therapy
***Negative test results for [[bacterial]] infection
*In the 1980s, emergence of [[pathological]] and [[electron]] findings from analysis of [[vitrectomy]] and [[enucleation]] specimens led to the discovery of acute [[retinal]] [[necrosis]]' cause as members of the herpes virus family.
*The official diagnostic criteria for acute [[retinal]] [[necrosis]] was proposed by the American [[Uveitis]] Society in 1994.


==Classification==
==[[Acute retinal necrosis historical perspective|Historical Perspective]]==
*Acute retinal necrosis (ARN) may be classified by staging and severity into the following:<ref name="pmid1645179">{{cite journal |vauthors=Gartry DS, Spalton DJ, Tilzey A, Hykin PG |title=Acute retinal necrosis syndrome |journal=Br J Ophthalmol |volume=75 |issue=5 |pages=292–7 |year=1991 |pmid=1645179 |pmc=1042358 |doi= |url=}}</ref>
**'''Acute stage''': Occurs at onset of disease and usually progresses past acute classification after a few weeks.
***Presents with coalescence of white, necrotic tissue in the peripheral retina.
***Vaso-[[occlusion|occlusive]] retinal [[vasculitis]] is usually present.
***The [[Optic nerve|optic nerve head]] of the affected eye will appear swollen, but the [[posterior pole]] will usually not be affected during the acute stage.
**'''Late stage''': Is the natural progression of the disease and will present differentiating characteristics after a few weeks up to a few months.
***Characterized by a regression of the coalesced [[necrosis]] in the peripheral [[retina]], presenting starkly contrasted [[necrotic]]/non-[[necrotic]] tissue and mild [[pigmentation]] [[scarring]] and increased [[vitreous]] debris
***[[Retinal detachment]], severe [[vision loss]], and potential [[blindness]] in the affected eye is indicative of late stage ARN.
***If the infection is bilateral, the second eye will usually present signs of ARN in the weeks and months following the initial symptom manifestation in the first eye.
*Acute retinal necrosis can also be classified by severity into the following:<ref name="pmid25356955">{{cite journal |vauthors=Brydak-Godowska J, Borkowski P, Szczepanik S, Moneta-Wielgoś J, Kęcik D |title=Clinical manifestation of self-limiting acute retinal necrosis |journal=Med. Sci. Monit. |volume=20 |issue= |pages=2088–96 |year=2014 |pmid=25356955 |pmc=4226315 |doi=10.12659/MSM.890469 |url=}}</ref>
**'''Mild''': Is used to characterize ARN that is stable and non-progressive.
***There is usually no sign of [[retinal detachment]].
**'''Fulminant''': ARN that is progressive and will usually lead to [[retinal detachment]] and further complications if untreated.


==Pathophysiology==
==[[Acute retinal necrosis classification|Classification]]==
===Pathogenesis===
*The pathogenesis of Acute retinal necrosis is characterized by [[retinal]] [[inflammation]] due to ocular [[viral]] infection:<ref name="pmid10682968">{{cite journal |vauthors=Ganatra JB, Chandler D, Santos C, Kuppermann B, Margolis TP |title=Viral causes of the acute retinal necrosis syndrome |journal=Am. J. Ophthalmol. |volume=129 |issue=2 |pages=166–72 |year=2000 |pmid=10682968 |doi= |url=}}</ref>
**Particles from [[Herpes simplex virus]] 1 (HSV-1), [[Herpes simplex virus]] 2 (HSV-2), and [[Varicella zoster]] virus (VZV) infiltrate the [[retina]] via various modes of transmission:<ref name="pmid22889540">{{cite journal |vauthors=Grose C |title=Acute retinal necrosis caused by herpes simplex virus type 2 in children: reactivation of an undiagnosed latent neonatal herpes infection |journal=Semin Pediatr Neurol |volume=19 |issue=3 |pages=115–8 |year=2012 |pmid=22889540 |pmc=3419358 |doi=10.1016/j.spen.2012.02.005 |url=}}</ref>
***[[Epithelial]] penetration of the skin: transmitted through the [[Ophthalmic nerve|ophthalmic]] branch of the [[Trigeminal nerve]].
***[[Epithelial]] penetration of the [[conjunctiva]]: transmitted directly through the [[optic nerve]].
***[[Epithelial]] penetration of the [[cornea]]: transmitted through the [[Maxillary|maxillary]] branch of the [[Trigeminal nerve]].
***[[Epithelial]] penetration of the [[nasal cavity]]: transmitted through the [[Olfactory nerve]] in the [[Subarachnoid space]].
**Acute retinal necrosis develops from HSV-1, HSV-2, and VZV due to the viruses' unique ability to transmit and replicate in the [[Central Nervous System]] (CNS), as well as their ability to transport [[anterograde]] through the [[optic nerve]], establish [[virus latency|latency]], reactivate, and cause [[retinal]] [[inflammation]].<ref name ="HumanHerpes">{{cite book |last1=Whitley |first1=Richard |last2=Kimberlin |first2=David W. |last3=Prober |first3=Charles G. |date=2007 |title=Human Herpesviruses: Biology, Therapy, and Immunoprophylaxis |url=http://www.ncbi.nlm.nih.gov/books/NBK47449/ |location=Cambridge, UK |publisher=Cambridge University Press |isbn=978-0511545313}}</ref>
***[[Retinal]] [[inflammation]] is caused by the up-regulated production of [[cytokines]].


===Genetics===
==[[Acute retinal necrosis pathophysiology|Pathophysiology]]==
*There is evidence of genetic predisposition to Acute retinal necrosis:
**For Caucasian populations: possessing the HLA-DQw7, HLA-Bw62, and HLA-DR4 [[antigens]] are correlated to genetic predisposition to ARN.<ref name="pmid2801857">{{cite journal |vauthors=Holland GN, Cornell PJ, Park MS, Barbetti A, Yuge J, Kreiger AE, Kaplan HJ, Pepose JS, Heckenlively JR, Culbertson WW |title=An association between acute retinal necrosis syndrome and HLA-DQw7 and phenotype Bw62, DR4 |journal=Am. J. Ophthalmol. |volume=108 |issue=4 |pages=370–4 |year=1989 |pmid=2801857 |doi= |url=}}</ref>
**For Japanese populations: possessing the HLA-Aw33, HLA-B44, and HLA-DRw6 [[antigens]] are correlated to genetic predisposition to ARN.<ref name="pmid25356955">{{cite journal |vauthors=Brydak-Godowska J, Borkowski P, Szczepanik S, Moneta-Wielgoś J, Kęcik D |title=Clinical manifestation of self-limiting acute retinal necrosis |journal=Med. Sci. Monit. |volume=20 |issue= |pages=2088–96 |year=2014 |pmid=25356955 |pmc=4226315 |doi=10.12659/MSM.890469 |url=}}</ref>
*Possession of the above [[antigens]] in their respective demographics are correlated to impaired immunity and increased predisposition to infection.


===Associated Conditions===
==[[Acute retinal necrosis causes|Causes]]==
*Acute retinal necrosis is associated with the following ocular conditions:
**[[Progressive outer retinal necrosis]]<ref name="pmid24926266">{{cite journal |vauthors=Coisy S, Ebran JM, Milea D |title=Progressive outer retinal necrosis and immunosuppressive therapy in myasthenia gravis |journal=Case Rep Ophthalmol |volume=5 |issue=1 |pages=132–7 |year=2014 |pmid=24926266 |pmc=4036147 |doi=10.1159/000362662 |url=}}</ref>
**[[Uveitis]]<ref name="urlFacts About Uveitis | National Eye Institute">{{cite web |url=https://nei.nih.gov/health/uveitis/uveitis |title=Facts About Uveitis &#124; National Eye Institute |format= |work= |accessdate=}}</ref>
**[[Cytomegalovirus retinitis]]<ref name="urlCMV retinitis: MedlinePlus Medical Encyclopedia">{{cite web |url=https://medlineplus.gov/ency/article/000665.htm |title=CMV retinitis: MedlinePlus Medical Encyclopedia |format= |work= |accessdate=}}</ref>
**[[Toxoplasmic chorioretinitis]]<ref name="pmid22116459">{{cite journal |vauthors=Davis JL |title=Diagnostic dilemmas in retinitis and endophthalmitis |journal=Eye (Lond) |volume=26 |issue=2 |pages=194–201 |year=2012 |pmid=22116459 |pmc=3272204 |doi=10.1038/eye.2011.299 |url=}}</ref>
**[[Endophthalmitis]]


==Causes==
==[[Differentiating Acute retinal necrosis from other diseases|Differentiating Acute retinal necrosis from other Diseases]]==
*Acute retinal necrosis (ARN) is usually caused by the reactivation of the following pathogenic [[viruses]] in the ''[[Herpesviridae]]'' family:<ref name="pmid24932179">{{cite journal |vauthors=Pikkel YY, Pikkel J |title=Acute retinal necrosis in childhood |journal=Case Rep Ophthalmol |volume=5 |issue=2 |pages=138–43 |year=2014 |pmid=24932179 |pmc=4049010 |doi=10.1159/000363130 |url=}}</ref>
**''[[Herpes simplex virus]]'' 1 (HSV-1)
**''[[Herpes simplex virus]]'' 2 (HSV-2)
**[[Varicella-zoster virus]] (VZV)
**Less commonly, ARN can be caused by [[Epstein-Barr virus]] and [[cytomegalovirus]].<ref name="pmid10682968">{{cite journal |vauthors=Ganatra JB, Chandler D, Santos C, Kuppermann B, Margolis TP |title=Viral causes of the acute retinal necrosis syndrome |journal=Am. J. Ophthalmol. |volume=129 |issue=2 |pages=166–72 |year=2000 |pmid=10682968 |doi= |url=}}</ref>
*[[Varicella-zoster virus|VZV]] and [[Herpes simplex virus|HSV-1]] are usually the causes of ARN in individuals older than 25 years.
**The majority of the ARN cases for individuals older than 50 years are caused by VZV and HSV-1.<ref name="pmid25356955">{{cite journal |vauthors=Brydak-Godowska J, Borkowski P, Szczepanik S, Moneta-Wielgoś J, Kęcik D |title=Clinical manifestation of self-limiting acute retinal necrosis |journal=Med. Sci. Monit. |volume=20 |issue= |pages=2088–96 |year=2014 |pmid=25356955 |pmc=4226315 |doi=10.12659/MSM.890469 |url=}}</ref>
*[[Herpes simplex virus|HSV-2]] is usually the cause of ARN in individuals younger than 25 years.


==Differentiating {{PAGENAME}} from Other Diseases==
==[[Acute retinal necrosis epidemiology and demographics|Epidemiology and Demographics]]==
*Acute retinal necrosis must be differentiated from other diseases that cause [[eye pain]], [[conjunctival infection]], [[photophobia]], and [[vision loss]]. Accurate and prompt diagnosis is critical to prevent [[blindness]] and complications.<ref name="pmid22116459">{{cite journal |vauthors=Davis JL |title=Diagnostic dilemmas in retinitis and endophthalmitis |journal=Eye (Lond) |volume=26 |issue=2 |pages=194–201 |year=2012 |pmid=22116459 |pmc=3272204 |doi=10.1038/eye.2011.299 |url=}}</ref><ref name="pmid3099921">{{cite journal| author=Dart JK| title=Eye disease at a community health centre. | journal=Br Med J (Clin Res Ed) | year= 1986 | volume= 293 | issue= 6560 | pages= 1477-80 | pmid=3099921 | doi= | pmc=1342247 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3099921  }} </ref><ref name="pmid10922425">{{cite journal| author=Leibowitz HM| title=The red eye. | journal=N Engl J Med | year= 2000 | volume= 343 | issue= 5 | pages= 345-51 | pmid=10922425 | doi=10.1056/NEJM200008033430507 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10922425  }} </ref><ref name=umichredeye>University of Michigan Eyes Have it (2009)http://kellogg.umich.edu/theeyeshaveit/red-eye/</ref>
**[[Progressive outer retinal necrosis]]<ref name="pmid24926266">{{cite journal |vauthors=Coisy S, Ebran JM, Milea D |title=Progressive outer retinal necrosis and immunosuppressive therapy in myasthenia gravis |journal=Case Rep Ophthalmol |volume=5 |issue=1 |pages=132–7 |year=2014 |pmid=24926266 |pmc=4036147 |doi=10.1159/000362662 |url=}}</ref>
**[[Uveitis]]<ref name="urlFacts About Uveitis | National Eye Institute">{{cite web |url=https://nei.nih.gov/health/uveitis/uveitis |title=Facts About Uveitis &#124; National Eye Institute |format= |work= |accessdate=}}</ref>
**[[Uveitis]]<ref name="urlFacts About Uveitis | National Eye Institute">{{cite web |url=https://nei.nih.gov/health/uveitis/uveitis |title=Facts About Uveitis &#124; National Eye Institute |format= |work= |accessdate=}}</ref>
**[[Endophthalmitis]]
**[[Toxoplasma chorioretinitis]]<ref name="pmid22116459">{{cite journal |vauthors=Davis JL |title=Diagnostic dilemmas in retinitis and endophthalmitis |journal=Eye (Lond) |volume=26 |issue=2 |pages=194–201 |year=2012 |pmid=22116459 |pmc=3272204 |doi=10.1038/eye.2011.299 |url=}}</ref>
**[[Cytomegalovirus retinitis]]<ref name="urlCMV retinitis: MedlinePlus Medical Encyclopedia">{{cite web |url=https://medlineplus.gov/ency/article/000665.htm |title=CMV retinitis: MedlinePlus Medical Encyclopedia |format= |work= |accessdate=}}</ref>
**[[Conjunctivitis]]
**[[Scleritis]]
**[[Corneal abrasion]]
**[[Glaucoma]]
**[[Corneal ulcer]]
**[[Retinal]] [[vasculitis]]<ref name="pmid20404987">{{cite journal |vauthors=Abu El-Asrar AM, Herbort CP, Tabbara KF |title=Differential diagnosis of retinal vasculitis |journal=Middle East Afr J Ophthalmol |volume=16 |issue=4 |pages=202–18 |year=2009 |pmid=20404987 |pmc=2855661 |doi=10.4103/0974-9233.58423 |url=}}</ref>
*Differentiating Acute retinal necrosis from other diseases is crucial due to varying etiologies of ocular diseases, particularly to ensure the best prognosis by applying the proper therapy.
**Acute [[papillitis]]<ref name="pmid20645925">{{cite journal |vauthors=Witmer MT, Pavan PR, Fouraker BD, Levy-Clarke GA |title=Acute retinal necrosis associated optic neuropathy |journal=Acta Ophthalmol |volume=89 |issue=7 |pages=599–607 |year=2011 |pmid=20645925 |doi=10.1111/j.1755-3768.2010.01911.x |url=}}</ref>


==Epidemiology and Demographics==
==[[Acute retinal necrosis risk factors|Risk Factors]]==
===Incidence===
*Research in the United Kingdom resulted in an estimated incidence of approximately 6.3 per 100,000 individuals.<ref name="pmid22281865">{{cite journal |vauthors=Cochrane TF, Silvestri G, McDowell C, Foot B, McAvoy CE |title=Acute retinal necrosis in the United Kingdom: results of a prospective surveillance study |journal=Eye (Lond) |volume=26 |issue=3 |pages=370–7; quiz 378 |year=2012 |pmid=22281865 |pmc=3298997 |doi=10.1038/eye.2011.338 |url=}}</ref>
**There is evidence that this incidence is underestimated due to biases in case adjudication and under-reporting of data.<ref name="pmid17504853">{{cite journal |vauthors=Muthiah MN, Michaelides M, Child CS, Mitchell SM |title=Acute retinal necrosis: a national population-based study to assess the incidence, methods of diagnosis, treatment strategies and outcomes in the UK |journal=Br J Ophthalmol |volume=91 |issue=11 |pages=1452–5 |year=2007 |pmid=17504853 |pmc=2095441 |doi=10.1136/bjo.2007.114884 |url=}}</ref>
*Worldwide, the increase of [[immunocompromised]] and aged populations in most countries evidences an increase in Acute retinal necrosis.


===Age===
==[[Acute retinal necrosis screening|Screening]]==  
*Acute retinal necrosis (ARN) developed from [[Herpes simplex virus]] 1 and [[Varicella-zoster virus]] is most common among patients older than 50 years.<ref name="pmid25356955">{{cite journal |vauthors=Brydak-Godowska J, Borkowski P, Szczepanik S, Moneta-Wielgoś J, Kęcik D |title=Clinical manifestation of self-limiting acute retinal necrosis |journal=Med. Sci. Monit. |volume=20 |issue= |pages=2088–96 |year=2014 |pmid=25356955 |pmc=4226315 |doi=10.12659/MSM.890469 |url=}}</ref>
*[[Herpes simplex virus]] (HSV) 2 infection is more common among children and adolescents; the incidence of HSV-2 caused ARN is highest in children and young adults between age 9 and 22 years.


===Gender===
==[[Acute retinal necrosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
*There is no gender predisposition to Acute retinal necrosis.
 
===Race===
*There is no racial predisposition to Acute retinal necrosis.
 
==Risk Factors==
*Risk factors for the development of Acute retinal necrosis (ARN) include the following:
**For caucasian populations: possessing the HLA-DQw7, HLA-Bw62, and HLA-DR4 antigens are correlated to genetic predisposition to ARN.<ref name="pmid2801857">{{cite journal |vauthors=Holland GN, Cornell PJ, Park MS, Barbetti A, Yuge J, Kreiger AE, Kaplan HJ, Pepose JS, Heckenlively JR, Culbertson WW |title=An association between acute retinal necrosis syndrome and HLA-DQw7 and phenotype Bw62, DR4 |journal=Am. J. Ophthalmol. |volume=108 |issue=4 |pages=370–4 |year=1989 |pmid=2801857 |doi= |url=}}</ref>
**For Japanese populations: possessing the HLA-Aw33, HLA-B44, and HLA-DRw6 antigens are correlated to genetic predisposition to ARN.<ref name="pmid25356955">{{cite journal |vauthors=Brydak-Godowska J, Borkowski P, Szczepanik S, Moneta-Wielgoś J, Kęcik D |title=Clinical manifestation of self-limiting acute retinal necrosis |journal=Med. Sci. Monit. |volume=20 |issue= |pages=2088–96 |year=2014 |pmid=25356955 |pmc=4226315 |doi=10.12659/MSM.890469 |url=}}</ref>
**Experiencing [[encephalitis]] from ''[[herpes simplex virus]]''<ref name="pmid18852442">{{cite journal |vauthors=Vandercam T, Hintzen RQ, de Boer JH, Van der Lelij A |title=Herpetic encephalitis is a risk factor for retinal necrosis |journal=Neurology |volume=71 |issue=16 |pages=1268–74 |year=2008 |pmid=18852442 |doi=10.1212/01.wnl.0000327615.99124.99 |url=}}</ref>
**[[Immunocompromise]] from prior or concurrent disease.<ref name="pmid1397473">{{cite journal |vauthors=Moutschen MP, Scheen AJ, Lefebvre PJ |title=Impaired immune responses in diabetes mellitus: analysis of the factors and mechanisms involved. Relevance to the increased susceptibility of diabetic patients to specific infections |journal=Diabete Metab |volume=18 |issue=3 |pages=187–201 |year=1992 |pmid=1397473 |doi= |url=}}</ref>
**Immunosuppresion from extended [[corticosteroid]] therapy.<ref name="pmid12714420">{{cite journal |vauthors=Yamamoto JH, Boletti DI, Nakashima Y, Hirata CE, Olivalves E, Shinzato MM, Okay TS, Santo RM, Duarte MI, Kalil J |title=Severe bilateral necrotising retinitis caused by Toxoplasma gondii in a patient with systemic lupus erythematosus and diabetes mellitus |journal=Br J Ophthalmol |volume=87 |issue=5 |pages=651–2 |year=2003 |pmid=12714420 |pmc=1771672 |doi= |url=}}</ref>
 
==Screening==
*There is no established, diagnostic screening process for Acute retinal necrosis.
 
==Natural History, Complications, and Prognosis==
===Natural History===
*Symptoms of Acute retinal necrosis (ARN) develop rapidly upon onset of pathogenic infection.<ref name="pmid25356955">{{cite journal |vauthors=Brydak-Godowska J, Borkowski P, Szczepanik S, Moneta-Wielgoś J, Kęcik D |title=Clinical manifestation of self-limiting acute retinal necrosis |journal=Med. Sci. Monit. |volume=20 |issue= |pages=2088–96 |year=2014 |pmid=25356955 |pmc=4226315 |doi=10.12659/MSM.890469 |url=}}</ref>
**Initial signs and symptoms include [[conjunctivitis]], [[vision loss]] and [[photophobia]], and [[eye pain]] and pressure.
*The natural progression of ARN depends on whether the case is mild or fulminant.
**Mild cases of ARN presents with white-yellow [[necrotic]] [[lesions]] that do not coalesce or lead to [[retinal detachment]]; the disease is [[self-limited]].<ref name="pmid2837090">{{cite journal |vauthors=Matsuo T, Nakayama T, Koyama T, Koyama M, Matsuo N |title=A proposed mild type of acute retinal necrosis syndrome |journal=Am. J. Ophthalmol. |volume=105 |issue=6 |pages=579–83 |year=1988 |pmid=2837090 |doi= |url=}}</ref>
**Fulminant cases of ARN will lead to progressive [[necrosis]] of [[retinal]] tissue, leading to pigmentation [[scarring]], [[vitreous]] debris, and [[retinal detachment]]
***There is a much greater chance of [[blindness]] in the affected eye.
*Without treatment, ARN will usually progress to Bilateral acute retinal necrosis (BARN) within weeks to a few months.<ref name="pmid1645179">{{cite journal |vauthors=Gartry DS, Spalton DJ, Tilzey A, Hykin PG |title=Acute retinal necrosis syndrome |journal=Br J Ophthalmol |volume=75 |issue=5 |pages=292–7 |year=1991 |pmid=1645179 |pmc=1042358 |doi= |url=}}</ref>
**There are exceptions in which the disease spread from the affected to the previously unaffected eye occurred up to 17 years later, due to reactivation of latent [[viral]] infection.<ref name="pmid21242577">{{cite journal |vauthors=Okunuki Y, Usui Y, Kezuka T, Takeuchi M, Goto H |title=Four cases of bilateral acute retinal necrosis with a long interval after the initial onset |journal=Br J Ophthalmol |volume=95 |issue=9 |pages=1251–4 |year=2011 |pmid=21242577 |doi=10.1136/bjo.2010.191288 |url=}}</ref>
 
===Complications===
*Complications resulting from Acute retinal necrosis occur due to [[retinal]] tissue damage and subsequent infection from the causative pathogen, including the following:<ref name="pmid25356955">{{cite journal |vauthors=Brydak-Godowska J, Borkowski P, Szczepanik S, Moneta-Wielgoś J, Kęcik D |title=Clinical manifestation of self-limiting acute retinal necrosis |journal=Med. Sci. Monit. |volume=20 |issue= |pages=2088–96 |year=2014 |pmid=25356955 |pmc=4226315 |doi=10.12659/MSM.890469 |url=}}</ref><ref name="pmid24385671">{{cite journal |vauthors=Flaxel CJ, Yeh S, Lauer AK |title=Combination systemic and intravitreal antiviral therapy in the management of acute retinal necrosis syndrome (an American Ophthalmological Society thesis) |journal=Trans Am Ophthalmol Soc |volume=111 |issue= |pages=133–44 |year=2013 |pmid=24385671 |pmc=3868412 |doi= |url=}}</ref>
**[[Retinal detachment]]
**[[Neurological]] conditions, such as [[encephalitis]]<ref name="pmid26622338">{{cite journal |vauthors=Liang ZG, Liu ZL, Sun XW, Tao ML, Yu GP |title=Viral encephalitis complicated by acute retinal necrosis syndrome: A case report |journal=Exp Ther Med |volume=10 |issue=2 |pages=465–467 |year=2015 |pmid=26622338 |pmc=4509005 |doi=10.3892/etm.2015.2557 |url=}}</ref> or [[meningitis]]
**[[Optic neuropathy]]
**Occlusive retinal vasculopathy
**Proliferative vitreoretinopathy<ref name="pmid16848208">{{cite journal |vauthors=Vukojević N, Popovic Suić S, Sikić J, Katusić D, Curković T, Sarić B, Jukić T |title=[Acute retinal necrosis] |journal=Acta Med Croatica |volume=60 |issue=2 |pages=145–8 |year=2006 |pmid=16848208 |doi= |url=}}</ref>
**[[Macular pucker]]<ref name="pmid1873262">{{cite journal |vauthors=McDonald HR, Lewis H, Kreiger AE, Sidikaro Y, Heckenlively J |title=Surgical management of retinal detachment associated with the acute retinal necrosis syndrome |journal=Br J Ophthalmol |volume=75 |issue=8 |pages=455–8 |year=1991 |pmid=1873262 |pmc=1042429 |doi= |url=}}</ref>
**[[Vitreous]] [[hemorrhage]]
**[[Neovascularization]]<ref name="pmid17184841">{{cite journal |vauthors=Lau CH, Missotten T, Salzmann J, Lightman SL |title=Acute retinal necrosis features, management, and outcomes |journal=Ophthalmology |volume=114 |issue=4 |pages=756–62 |year=2007 |pmid=17184841 |doi=10.1016/j.ophtha.2006.08.037 |url=}}</ref>
**[[Phthisis bulbi]]<ref name="pmid17184841">{{cite journal |vauthors=Lau CH, Missotten T, Salzmann J, Lightman SL |title=Acute retinal necrosis features, management, and outcomes |journal=Ophthalmology |volume=114 |issue=4 |pages=756–62 |year=2007 |pmid=17184841 |doi=10.1016/j.ophtha.2006.08.037 |url=}}</ref>
 
===Prognosis===
*Without treatment, the prognosis for Acute retinal necrosis (ARN) varies:<ref name="pmid25356955">{{cite journal |vauthors=Brydak-Godowska J, Borkowski P, Szczepanik S, Moneta-Wielgoś J, Kęcik D |title=Clinical manifestation of self-limiting acute retinal necrosis |journal=Med. Sci. Monit. |volume=20 |issue= |pages=2088–96 |year=2014 |pmid=25356955 |pmc=4226315 |doi=10.12659/MSM.890469 |url=}}</ref>
**Mild ARN: Usually self-limited and will resolve itself without treatment; risk of permanent [[vision loss]] is very low.
**Fulminant ARN: Will usually progress to complications such as [[progressive outer retinal necrosis]] and has a worse prognosis.
***[[Retinal detachment]] will usually occur without treatment, leading to permanent [[vision loss]].
***Spread of infection through the [[anterior]] chamber to the [[brain]] has particularly poor prognosis if [[encephalitis]] or [[meningitis]] develops.
*With treatment, the prognosis for ARN is good if the therapy is administered in the early stages and sustained until symptoms resolve.
**Uncommonly, prognosis can worsen if the patient is [[immunocompromised]] and experiences a subsequent infection due to vulnerability from prolonged topical [[corticosteroid]] use.


==Diagnosis==
==Diagnosis==
===Diagnostic Criteria===
[[Acute retinal necrosis diagnostic criteria|Diagnostic Criteria]] | [[Acute retinal necrosis history and symptoms|History and Symptoms]] | [[Acute retinal necrosis physical examination|Physical Examination]] | [[Acute retinal necrosis laboratory findings|Laboratory Findings]] | [[Acute retinal necrosis electrocardiogram|Electrocardiogram]] | [[Acute retinal necrosis chest x ray|Chest X Ray]] | [[Acute retinal necrosis CT|CT]] | [[Acute retinal necrosis MRI|MRI]] | [[Acute retinal necrosis echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Acute retinal necrosis other imaging findings|Other Imaging Findings]] | [[Acute retinal necrosis other diagnostic studies|Other Diagnostic Studies]]
The diagnosis of acute [[retinal]] [[necrosis]] is made when the following criteria are met:<ref name="pmid8172275">{{cite journal |vauthors=Holland GN |title=Standard diagnostic criteria for the acute retinal necrosis syndrome. Executive Committee of the American Uveitis Society |journal=Am. J. Ophthalmol. |volume=117 |issue=5 |pages=663–7 |year=1994 |pmid=8172275 |doi= |url=}}</ref>
*One or more discrete foci of peripheral [[retinal]] [[necrosis]], located outside of the major [[temporal]] [[vascular]] [[Arterial arcades|arcades]]
*Circumferential spread if [[Antiviral drug|antiviral therapy]] has not been administered
*[[Occlusion|Occlusive]] [[retinal]] vasculopathy
*A prominent [[vitreous]] or [[anterior chamber]] [[inflammation]]
*Rapid disease progression in the absence of therapy
 
===History===
Patient history of prior or concurrent diseases, particularly those associated with Acute retinal necrosis pathogens, or sources of [[immunocompromise]] should be considered in the diagnosis of Acute retinal necrosis:<ref name="pmid1397473">{{cite journal |vauthors=Moutschen MP, Scheen AJ, Lefebvre PJ |title=Impaired immune responses in diabetes mellitus: analysis of the factors and mechanisms involved. Relevance to the increased susceptibility of diabetic patients to specific infections |journal=Diabete Metab |volume=18 |issue=3 |pages=187–201 |year=1992 |pmid=1397473 |doi= |url=}}</ref>
*History of [[diabetes mellitus]]
*History of prolonged [[corticosteroid]] use<ref name="pmid25356955">{{cite journal |vauthors=Brydak-Godowska J, Borkowski P, Szczepanik S, Moneta-Wielgoś J, Kęcik D |title=Clinical manifestation of self-limiting acute retinal necrosis |journal=Med. Sci. Monit. |volume=20 |issue= |pages=2088–96 |year=2014 |pmid=25356955 |pmc=4226315 |doi=10.12659/MSM.890469 |url=}}</ref>
*History of [[Herpes simplex|genital or oral herpes infection]]
*History of [[Chickenpox|chickenpox]] or [[shingles]]
*History of [[Mononucleosis|mononucleosis]]
*History of [[HIV]] infection
 
===Symptoms===
Symptoms of Acute retinal necrosis include the following:<ref name="pmid17504853">{{cite journal |vauthors=Muthiah MN, Michaelides M, Child CS, Mitchell SM |title=Acute retinal necrosis: a national population-based study to assess the incidence, methods of diagnosis, treatment strategies and outcomes in the UK |journal=Br J Ophthalmol |volume=91 |issue=11 |pages=1452–5 |year=2007 |pmid=17504853 |pmc=2095441 |doi=10.1136/bjo.2007.114884 |url=}}</ref>
*[[Vision loss]]
**[[Blindness]] may be present in more severe cases
*[[Photophobia|Excessive sensitivity to light]]
*[[Ocular]] pain
*[[Flu]] symptoms
*[[Erythema|Redness]] of the affected eye
*[[Floaters]]<ref name="pmid24336545">{{cite journal |vauthors=Ford JR, Tsui E, Lahey T, Zegans ME |title=Question: Can you identify this condition? Acute retinal necrosis |journal=Can Fam Physician |volume=59 |issue=12 |pages=1307; 1308–10 |year=2013 |pmid=24336545 |pmc=3860929 |doi= |url=}}</ref>
*[[Flashes]]<ref name="urlAmerican Academy of Ophthalmology">{{cite web |url=http://www.aao.org/ |title=American Academy of Ophthalmology |format= |work= |accessdate=}}</ref>
 
===Physical Examination===
Physical examination for acute retinal necrosis is remarkable for the following:<ref name="pmid25356955">{{cite journal |vauthors=Brydak-Godowska J, Borkowski P, Szczepanik S, Moneta-Wielgoś J, Kęcik D |title=Clinical manifestation of self-limiting acute retinal necrosis |journal=Med. Sci. Monit. |volume=20 |issue= |pages=2088–96 |year=2014 |pmid=25356955 |pmc=4226315 |doi=10.12659/MSM.890469 |url=}}</ref>
*[[Erythema]] and [[hyperaemia]] of the [[retina]]<ref name="pmid17504853">{{cite journal |vauthors=Muthiah MN, Michaelides M, Child CS, Mitchell SM |title=Acute retinal necrosis: a national population-based study to assess the incidence, methods of diagnosis, treatment strategies and outcomes in the UK |journal=Br J Ophthalmol |volume=91 |issue=11 |pages=1452–5 |year=2007 |pmid=17504853 |pmc=2095441 |doi=10.1136/bjo.2007.114884 |url=}}</ref>
*White and yellow [[necrosis|necrotic]] [[lesions]] in the [[retina]]
**[[Pus|Purulent]] [[exudate]] can also be found in the periphery of the [[retina]]<ref name="pmid24385671">{{cite journal |vauthors=Flaxel CJ, Yeh S, Lauer AK |title=Combination systemic and intravitreal antiviral therapy in the management of acute retinal necrosis syndrome (an American Ophthalmological Society thesis) |journal=Trans Am Ophthalmol Soc |volume=111 |issue= |pages=133–44 |year=2013 |pmid=24385671 |pmc=3868412 |doi= |url=}}</ref>
**Opaque [[vitreous]] from the coalescence of [[necrotic]] [[tissue]]
*[[Anterior chamber]] [[inflammation]]
*[[Vitreous]] [[inflammation]]
*[[Scleritis]]
 
===Laboratory Findings===
Laboratory findings associated with Acute retinal necrosis are those used to determine the [[viral]] pathogen, obtained from [[aqueous humor]] or the [[vitreous]].<ref name="pmid25356955">{{cite journal |vauthors=Brydak-Godowska J, Borkowski P, Szczepanik S, Moneta-Wielgoś J, Kęcik D |title=Clinical manifestation of self-limiting acute retinal necrosis |journal=Med. Sci. Monit. |volume=20 |issue= |pages=2088–96 |year=2014 |pmid=25356955 |pmc=4226315 |doi=10.12659/MSM.890469 |url=}}</ref>
*Qualitative and Real-time [[Polymerase chain reaction]]<ref name="pmid25356955">{{cite journal |vauthors=Brydak-Godowska J, Borkowski P, Szczepanik S, Moneta-Wielgoś J, Kęcik D |title=Clinical manifestation of self-limiting acute retinal necrosis |journal=Med. Sci. Monit. |volume=20 |issue= |pages=2088–96 |year=2014 |pmid=25356955 |pmc=4226315 |doi=10.12659/MSM.890469 |url=}}</ref>
**PCR-tests for Acute retinal necrosis patients will produce [[genomic]] evidence of the causative [[virus]].
**It is the preferred test due to the 90% specificity in detecting [[Herpes simplex virus]] (HSV), [[Varicella-zoster virus]] (VZV), and [[Cytomegalovirus]] (CMV).
*Viral cultures may reveal positive results for HSV-1, HSV-2, VZV, or CMV.<ref name="pmid23052715">{{cite journal |vauthors=Silva RA, Berrocal AM, Moshfeghi DM, Blumenkranz MS, Sanislo S, Davis JL |title=Herpes simplex virus type 2 mediated acute retinal necrosis in a pediatric population: case series and review |journal=Graefes Arch. Clin. Exp. Ophthalmol. |volume=251 |issue=2 |pages=559–66 |year=2013 |pmid=23052715 |doi=10.1007/s00417-012-2164-8 |url=}}</ref>
*[[Immunoflourescence]] may reveal [[antibodies]] indicative of Acute retinal necrosis pathogens.<ref name="pmid18159535">{{cite journal |vauthors=Singh A, Preiksaitis J, Ferenczy A, Romanowski B |title=The laboratory diagnosis of herpes simplex virus infections |journal=Can J Infect Dis Med Microbiol |volume=16 |issue=2 |pages=92–8 |year=2005 |pmid=18159535 |pmc=2095011 |doi= |url=}}</ref>
*Detection of indicative [[antibodies]] via Goldmann-witmer coefficient.<ref name="pmid16458686">{{cite journal |vauthors=De Groot-Mijnes JD, Rothova A, Van Loon AM, Schuller M, Ten Dam-Van Loon NH, De Boer JH, Schuurman R, Weersink AJ |title=Polymerase chain reaction and Goldmann-Witmer coefficient analysis are complimentary for the diagnosis of infectious uveitis |journal=Am. J. Ophthalmol. |volume=141 |issue=2 |pages=313–8 |year=2006 |pmid=16458686 |doi=10.1016/j.ajo.2005.09.017 |url=}}</ref>
 
===Imaging Findings===
====Key CT Findings for Acute Retinal Necrosis====
CT imaging may reveal indicators of [[inflammation]] and infection by the causative pathogen for Acute retinal necrosis (ARN).<ref name="pmid15569737">{{cite journal |vauthors=Bert RJ, Samawareerwa R, Melhem ER |title=CNS MR and CT findings associated with a clinical presentation of herpetic acute retinal necrosis and herpetic retrobulbar optic neuritis: five HIV-infected and one non-infected patients |journal=AJNR Am J Neuroradiol |volume=25 |issue=10 |pages=1722–9 |year=2004 |pmid=15569737 |doi= |url=}}</ref>
*Hypoattenuation along the [[optic tract]] indicative of [[Varicella-zoster virus]] (VZV) infection.
*Hyperattenuation along the [[optic tract]], [[retina]], [[sclerae]], and [[lateral geniculate body]], indicating presence of lesions indicative of ARN.<ref name="pmid4026646">{{cite journal |vauthors=Sergott RC, Belmont JB, Savino PJ, Fischer DH, Bosley TM, Schatz NJ |title=Optic nerve involvement in the acute retinal necrosis syndrome |journal=Arch. Ophthalmol. |volume=103 |issue=8 |pages=1160–2 |year=1985 |pmid=4026646 |doi= |url=}}</ref>
*Infection-caused shrunken left globe.
 
====Key MRI Findings for Acute Retinal Necrosis====
MRI imaging may reveal the following indicators of Acute retinal necrosis:<ref name="pmid15569737">{{cite journal |vauthors=Bert RJ, Samawareerwa R, Melhem ER |title=CNS MR and CT findings associated with a clinical presentation of herpetic acute retinal necrosis and herpetic retrobulbar optic neuritis: five HIV-infected and one non-infected patients |journal=AJNR Am J Neuroradiol |volume=25 |issue=10 |pages=1722–9 |year=2004 |pmid=15569737 |doi= |url=}}</ref>
*Increased T2 signal intensity in the optic pathway: [[optic nerves]], [[optic chiasm]], [[lateral geniculate bodies]], [[optic radiations]], [[visual cortex]], [[midbrain]] structures, [[trigeminal nerves]], and [[meninges]].
**The increased intensity reveals lesions that may be indicative of [[Herpes simplex virus]] or [[Cytomegalovirus]] infection.
*[[Contrast-enhanced|Contrast enhanced CT]] T1-weighted images may reveal enhancement of [[optic nerve]], [[optic chiasm]], [[optic tracts]], [[optic radiation]], semilunar ganglion–Meckel cave, [[meninges]], and [[midbrain]].
 
====Electrocardiogram====
*There are no diagnostic electrocardiogram findings associated with Acute retinal necrosis.
 
====Chest X Ray====
*There are no diagnostic chest x ray findings associated with Acute retinal necrosis.
 
====Echocardiography or Ultrasound====
*There are no diagnositic echocardiography or ultrasound findings associated with Acute retinal necrosis.
 
====Other Imaging Findings====
=====Fundus Autoflourescence=====
Fundus Autoflourescence (FAF) is an imaging technique that examines [[flourophores]] in the [[neurosensory retina]] and the retinal pigment [[epithelium]], presenting with the following findings indicative of Acute retinal necrosis:<ref name="pmid7890502">{{cite journal |vauthors=Delori FC, Dorey CK, Staurenghi G, Arend O, Goger DG, Weiter JJ |title=In vivo fluorescence of the ocular fundus exhibits retinal pigment epithelium lipofuscin characteristics |journal=Invest. Ophthalmol. Vis. Sci. |volume=36 |issue=3 |pages=718–29 |year=1995 |pmid=7890502 |doi= |url=}}</ref>
*Hypoautoflourescence in the [[retina]], in conjunction with hyperflourescent borders, is indicative of Acute retinal necrosis and atrophy of retinal [[pigment]] [[epithelium]].<ref name="FreundMrejen2013">{{cite journal|last1=Freund|first1=K. Bailey|last2=Mrejen|first2=Sarah|last3=Jung|first3=Jesse|last4=Yannuzzi|first4=Lawrence A.|last5=Boon|first5=Camiel J. F.|title=Increased Fundus Autofluorescence Related to Outer Retinal Disruption|journal=JAMA Ophthalmology|volume=131|issue=12|year=2013|pages=1645|issn=2168-6165|doi=10.1001/jamaophthalmol.2013.5030}}</ref>
**Posterior extension of the hyperflourescent borders may be indicative of spreading [[inflammation]] and Acute retinal necrosis.
**Hyperflourescence may also be indicative of reduced ability to block flourophores into the [[retina]] due to damage and degradation.<ref name="pmid26120371">{{cite journal |vauthors=Ward TS, Reddy AK |title=Fundus autofluorescence in the diagnosis and monitoring of acute retinal necrosis |journal=J Ophthalmic Inflamm Infect |volume=5 |issue= |pages=19 |year=2015 |pmid=26120371 |pmc=4477008 |doi=10.1186/s12348-015-0042-3 |url=}}</ref>
*FAF is advantageous to color photos due to the ability to more starkly contrast lesions with unaffected [[retinal]] tissue.
 
=====Fluorescein Angiography=====
Fluorescein angiographic images may indicate evidence of Acute retinal necrosis by displaying [[retinal]] [[vasculature]] and potential [[retinal]] [[hemorrhages]], as well as white-yellow [[necrotic]] lesions.<ref name="pmid12063045">{{cite journal |vauthors=Takei H, Ohno-Matsui K, Hayano M, Mochizuki M |title=Indocyanine green angiographic findings in acute retinal necrosis |journal=Jpn. J. Ophthalmol. |volume=46 |issue=3 |pages=330–5 |year=2002 |pmid=12063045 |doi= |url=}}</ref><ref name="urlFluorescein angiography: MedlinePlus Medical Encyclopedia">{{cite web |url=https://medlineplus.gov/ency/article/003846.htm |title=Fluorescein angiography: MedlinePlus Medical Encyclopedia |format= |work= |accessdate=}}</ref>
*Fluorscein angiography can reveal [[optic nerve head]] leakage caused by intraocular [[inflammation]] from the pathogent responsible for ARN.<ref name="pmid20404987">{{cite journal |vauthors=Abu El-Asrar AM, Herbort CP, Tabbara KF |title=Differential diagnosis of retinal vasculitis |journal=Middle East Afr J Ophthalmol |volume=16 |issue=4 |pages=202–18 |year=2009 |pmid=20404987 |pmc=2855661 |doi=10.4103/0974-9233.58423 |url=}}</ref>
*Imaging may reveal [[occlusion|occlusive]] vasculopathy and periarterial vascular sheathing.
 
=====Optical Coherence Tomography=====
Optical Coherence Tomography (OCT) imaging may indicate Acute retinal necrosis with the following:<ref name="pmid16766400">{{cite journal |vauthors=Suzuki J, Goto H, Minoda H, Iwasaki T, Sakai J, Usui M |title=Analysis of retinal findings of acute retinal necrosis using optical coherence tomography |journal=Ocul. Immunol. Inflamm. |volume=14 |issue=3 |pages=165–70 |year=2006 |pmid=16766400 |doi=10.1080/09273940600672198 |url=}}</ref>
*Reflective inner layers of the [[retina]], indicative of white-yellow [[necrotic]] [[lesions]].
**Abnormalities and disorganization in the [[retinal]] structure indicative of [[inflammation]].
*[[Retinal]] [[exudate]]
*Diminished thickness of the [[retina]].
 
===Other Diagnostic Studies===
There are no other diagnostic studies associated with Acute retinal necrosis.


==Treatment==
==Treatment==
===Medical Therapy===
:*''' Empiric antimicrobial therapy'''


::*Preferred regimen: [[Acyclovir]] 10 mg/kg IV q8h for 1-2 weeks followed by [[Acyclovir]] 400 mg PO bid for chronic maintenance
[[Acute retinal necrosis medical therapy|Medical Therapy]] | [[Acute retinal necrosis surgery|Surgery]] | [[Acute retinal necrosis primary prevention|Primary Prevention]] | [[Acute retinal necrosis secondary prevention|Secondary Prevention]] | [[Acute retinal necrosis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Acute retinal necrosis future or investigational therapies|Future or Investigational Therapies]]


::*Alternative regimen (1): [[Acyclovir]] 10 mg/kg IV q8h for 1-2 weeks followed by [[Valacyclovir]] 1 g IV q8h for 6 weeks to several months followed by [[Acyclovir]] 400 mg PO bid for chronic maintenance
==Case Studies==
::*Alternative regimen (2), unresponsive: [[Foscarnet]] 1.2-2.4 mg/0.1 mL intravitreal injection 1-3 times per week {{and}} ([[Ganciclovir]] 5 mg/kg IV q12 for 2 weeks followed by 5 mg/kg q24h for 5-7 weeks {{or}} [[Foscarnet]] 60 mg/kg IV q8h for 2 weeks followed by 90-120 mg/kg IV q24h {{or}} [[Cidofovir]] 5 mg/kg IV for 2 weeks followed by 5 mg/kg IV q2weeks) followed by ([[Acyclovir]] 400 mg PO bid for chronic maintenance {{or}} [[Valganciclovir]] 900 mg PO qd for chronic maintenance)
[[Acute retinal necrosis case study one|Case #1]]


::*Note: [[Ganciclovir]] is administered for patients with suspected CMV acute retinal necrosis. Whereas [[Foscarnet]] is administered for patients who are not immunocompromised
[[Category:Ophthalmology]]
 
[[Category:Disease]]
:*''' Pathogen-directed antimicrobial therapy'''
 
::*'''HSV or VZV'''
:::*Preferred regimen: [[Acyclovir]] 10 mg/kg IV q8h for 1-2 weeks followed by [[Acyclovir]] 400 mg PO bid for chronic maintenance
:::*Alternative regimen: [[Acyclovir]] 10 mg/kg IV q8h for 1-2 weeks followed by [[Valacyclovir]] 1 g IV q8h for 6 weeks to several months followed by [[Acyclovir]] 400 mg PO bid for chronic maintenance
 
::*''' Cytomegalovirus'''
:::*Preferred regimen: [[Foscarnet]] 1.2-2.4 mg/0.1 mL intravitreal injection 1-3 times per week {{and}} [[Ganciclovir]] 5 mg/kg IV q12 for 2 weeks followed by 5 mg/kg q24h for 5-7 weeks followed by [[Valganciclovir]] 900 mg PO qd for chronic maintenance
 
===Surgery===
Surgery is not the first-line treatment option for patients with Acute retinal necrosis; it is primarily indicated when there is risk of complications, including [[retinal detachment]] and tissue atrophy.<ref name="pmid26035758">{{cite journal |vauthors=Shantha JG, Weissman HM, Debiec MR, Albini TA, Yeh S |title=Advances in the management of acute retinal necrosis |journal=Int Ophthalmol Clin |volume=55 |issue=3 |pages=1–13 |year=2015 |pmid=26035758 |pmc=4567584 |doi=10.1097/IIO.0000000000000077 |url=}}</ref>
 
====Vitrectomy====
*[[Vitrectomy]] may be indicated both before and after occurrence of [[retinal detachment]] to improve visual prognosis.<ref name="pmid22937510">{{cite journal |vauthors=Luo YH, Duan XC, Chen BH, Tang LS, Guo XJ |title=Efficacy and necessity of prophylactic vitrectomy for acute retinal necrosis syndrome |journal=Int J Ophthalmol |volume=5 |issue=4 |pages=482–7 |year=2012 |pmid=22937510 |pmc=3428546 |doi=10.3980/j.issn.2222-3959.2012.04.15 |url=}}</ref>
**[[Prophylactic]] [[vitrectomy]] can be effective in removing [[inflammation]] factors, preventing [[retinal detachment]] by removing or preventing the spread of pre-existing [[lesions]] and [[necrotic]] tissue.<ref name="pmid18584565">{{cite journal |vauthors=Kawaguchi T, Spencer DB, Mochizuki M |title=Therapy for acute retinal necrosis |journal=Semin Ophthalmol |volume=23 |issue=4 |pages=285–90 |year=2008 |pmid=18584565 |doi=10.1080/08820530802111192 |url=}}</ref>
**Remedial [[Vitrectomy]] in patients experiencing [[retinal detachment]] can lead to improved visual prognosis by [[retinal]] reattachment.<ref name="pmid1873262">{{cite journal |vauthors=McDonald HR, Lewis H, Kreiger AE, Sidikaro Y, Heckenlively J |title=Surgical management of retinal detachment associated with the acute retinal necrosis syndrome |journal=Br J Ophthalmol |volume=75 |issue=8 |pages=455–8 |year=1991 |pmid=1873262 |pmc=1042429 |doi= |url=}}</ref>
*The success of [[vitrectomy]] in improving outcomes is dependent on the onset of Acute retinal necrosis relative to the time the procedure is performed.<ref name="pmid22937510">{{cite journal |vauthors=Luo YH, Duan XC, Chen BH, Tang LS, Guo XJ |title=Efficacy and necessity of prophylactic vitrectomy for acute retinal necrosis syndrome |journal=Int J Ophthalmol |volume=5 |issue=4 |pages=482–7 |year=2012 |pmid=22937510 |pmc=3428546 |doi=10.3980/j.issn.2222-3959.2012.04.15 |url=}}</ref>
**Increased extent of [[necrosis]] and larger size, as well as posterior located, [[lesions]] were associated with worse visual prognosis despite [[prophylactic]] or remedial [[vitrectomy]].
**[[Prophylactic]] [[vitrectomy]] is often encouraged to maximize efficacy, performed in the early stages of ARN.
====Prophylactic Laser Retinopexy====
*Prophylactic laser retinopexy may be indicated to prevent [[retinal detachment]] by [[Laser photocoagulation|photocoagulation]], creating posterior chorioretinal adhesions.<ref name="pmid18723739">{{cite journal |vauthors=Park JJ, Pavesio C |title=Prophylactic laser photocoagulation for acute retinal necrosis. Does it raise more questions than answers? |journal=Br J Ophthalmol |volume=92 |issue=9 |pages=1161–2 |year=2008 |pmid=18723739 |doi=10.1136/bjo.2008.147181 |url=}}</ref>
**The procedure is contraindicated if there is [[vitreous]] [[inflammation]] or obstructed view and access to the [[posterior pole]].
*Due to reported occurrences of [[retinal detachment]] from prophylactic laser photocoagulation, more research is necessary to determine the ideal indications for the procedure.<ref name="pmid18723739">{{cite journal |vauthors=Park JJ, Pavesio C |title=Prophylactic laser photocoagulation for acute retinal necrosis. Does it raise more questions than answers? |journal=Br J Ophthalmol |volume=92 |issue=9 |pages=1161–2 |year=2008 |pmid=18723739 |doi=10.1136/bjo.2008.147181 |url=}}</ref>
**If performed on patients with excessive [[inflammation]] and [[vitreous]] opacity, there is evidence of photocoagulation worsening prognosis of Acute retinal necrosis, leading to [[retinal detachment]] and [[blindness]].<ref name="pmid18584565">{{cite journal |vauthors=Kawaguchi T, Spencer DB, Mochizuki M |title=Therapy for acute retinal necrosis |journal=Semin Ophthalmol |volume=23 |issue=4 |pages=285–90 |year=2008 |pmid=18584565 |doi=10.1080/08820530802111192 |url=}}</ref>
 
===Prevention===
====Primary Prevention====
Preventing onset of Acute retinal necrosis is dependent on preventing the causative infection from [[Herpes simplex virus]] (HSV), [[Varicella-zoster virus]] (VZV), and [[Cytomegalovirus]] (CMV). Measures to prevent viral infection include the following:<ref name="urlWHO | Herpes simplex virus">{{cite web |url=http://www.who.int/mediacentre/factsheets/fs400/en/ |title=WHO &#124; Herpes simplex virus |format= |work= |accessdate=}}</ref><ref name="Charkoudian2011">{{cite journal|last1=Charkoudian|first1=Leon D.|title=Acute Retinal Necrosis After Herpes Zoster Vaccination|journal=Archives of Ophthalmology|volume=129|issue=11|year=2011|pages=1495|issn=0003-9950|doi=10.1001/archophthalmol.2011.320}}</ref><ref name="urlCMV | Overview | Cytomegalovirus and Congenital CMV Infection | CDC">{{cite web |url=http://www.cdc.gov/cmv/overview.html |title=CMV &#124; Overview &#124; Cytomegalovirus and Congenital CMV Infection &#124; CDC |format= |work= |accessdate=}}</ref>
*Avoiding oral and genital contact with individuals infected with [[Herpes simplex virus|HSV]]
*Avoiding proximity to individuals infected with [[Varicella-zoster virus|VZV]] to avoid contact with pathogenic respiratory droplets and fluid contact
*Avoiding fluid contact with individuals infected with [[Cytomegalovirus|CMV]]
 
====Secondary Prevention====
While recurrence of Acute retinal necrosis is not completely preventable presently, administration of topical and [[intravitreal]] [[antiviral]] therapy targeted to the specific etiological cause of the disease can reduce the chance of recurrence.<ref name="pmid24385671">{{cite journal |vauthors=Flaxel CJ, Yeh S, Lauer AK |title=Combination systemic and intravitreal antiviral therapy in the management of acute retinal necrosis syndrome (an American Ophthalmological Society thesis) |journal=Trans Am Ophthalmol Soc |volume=111 |issue= |pages=133–44 |year=2013 |pmid=24385671 |pmc=3868412 |doi= |url=}}</ref>
*Application of [[antiviral]] therapy is more effective for prevention when administered as close to disease onset as possible.
**Extensive, prolonged therapy is important in preventing spread of the disease to the unaffected eye.
 
Further [[prophylactic]] measures, such as [[vitrectomy]], may be used in current Acute retinal necrosis patients to minimize the possibility of complications, including [[retinal detachment]].<ref name="pmid26035758">{{cite journal |vauthors=Shantha JG, Weissman HM, Debiec MR, Albini TA, Yeh S |title=Advances in the management of acute retinal necrosis |journal=Int Ophthalmol Clin |volume=55 |issue=3 |pages=1–13 |year=2015 |pmid=26035758 |pmc=4567584 |doi=10.1097/IIO.0000000000000077 |url=}}</ref>
 
==See also==
* [[Cytomegalovirus retinitis]]
* [[Progressive outer retinal necrosis]]
 
==External links==
* http://www.iceh.org.uk/files/tsno8/text/18.htm
* http://www.eyepathologist.org/disease.asp?IDNUM=301330
 
==References==
{{reflist|2}}


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Risk calculators and risk factors for Acute retinal necrosis

For patient information click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords:

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Acute retinal necrosis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1


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