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


==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}} {{AE}} {{LRO}}; {{Faizan}}


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}} Retinal necrosis syndrome; Acute retinal necrosis syndrome; Necrotizing herpetic retinitis; Bilateral acute retinal necrosis


==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.


==Pathophysiology==
==[[Acute retinal necrosis classification|Classification]]==
*The pathogenesis of Acute retinal necrosis is characterized by [[viral]] infection of


==Causes==
==[[Acute retinal necrosis pathophysiology|Pathophysiology]]==
The exact causes are not known, but [[varicella zoster virus]] is frequently implicated,<ref name="pmid17184841">{{cite journal |author=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=http://linkinghub.elsevier.com/retrieve/pii/S0161-6420(06)01207-3}}</ref> and other [[herpesviridae|herpesviruses]] can be involved.<ref name="pmid17264500">{{cite journal |author=Kezuka T, Atherton SS |title=Acute retinal necrosis |journal=Chem Immunol Allergy |volume=92 |issue= |pages=244–53 |year=2007 |pmid=17264500 |doi=10.1159/000099275 |url=http://content.karger.com/produktedb/produkte.asp?doi=10.1159/000099275&typ=pdf |series=Chemical Immunology and Allergy |isbn=3-8055-8187-4}}</ref>


==Differentiating {{PAGENAME}} from Other Diseases==
==[[Acute retinal necrosis causes|Causes]]==


==Epidemiology and Demographics==
==[[Differentiating Acute retinal necrosis from other diseases|Differentiating Acute retinal necrosis from other Diseases]]==


==Risk Factors==
==[[Acute retinal necrosis epidemiology and demographics|Epidemiology and Demographics]]==


==Screening==
==[[Acute retinal necrosis risk factors|Risk Factors]]==


==Natural History, Complications, and Prognosis==
==[[Acute retinal necrosis screening|Screening]]==  
===Natural History===


===Complications===
==[[Acute retinal necrosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
 
===Prognosis===


==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
 
===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]]
 
===Laboratory Findings===
 
===Imaging Findings===
 
===Other Diagnostic Studies===


==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
::*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
::*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)
::*Note: [[Ganciclovir]] is administered for patients with suspected CMV acute retinal necrosis. Whereas [[Foscarnet]] is administered for patients who are not immunocompromised
:*''' 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===
===Prevention===


==See also==
[[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]]
* [[Cytomegalovirus retinitis]]
* [[Progressive outer retinal necrosis]]


==External links==
==Case Studies==
* http://www.iceh.org.uk/files/tsno8/text/18.htm
[[Acute retinal necrosis case study one|Case #1]]
* http://www.eyepathologist.org/disease.asp?IDNUM=301330


==References==
==Source==
{{reflist|2}}
[http://eyewiki.aao.org/Acute_retinal_necrosis American Academy of Ophthalmology]
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Latest revision as of 20:16, 29 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Luke Rusowicz-Orazem, B.S.; Faizan Sheraz, M.D. [2]

Synonyms and keywords: Retinal necrosis syndrome; Acute retinal necrosis syndrome; Necrotizing herpetic retinitis; Bilateral acute retinal necrosis

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

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