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| __NOTOC__ | | __NOTOC__ |
| {{SI}} | | {{Acute retinal necrosis}} |
| {{CMG}}; {{AE}} {{Faizan}}
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| ==Overview==
| | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
| '''Acute retinal necrosis''' is a type of [[retinitis]] which can be associated with viral infections. | |
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| 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}} |
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| 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 |
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| ==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>
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| **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>
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| *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>
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| **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>
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| ***[[Periarteritis]]
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| ***Opaque, dense [[vitreous]]
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| ***Peripheral [[retinal]] [[exudates]]
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| ***[[Retinal detachment]]
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| ***[[Vision loss]]
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| ***Resistance to [[antibiotic]] therapy
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| ***Negative test results for [[bacterial]] infection
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| *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.
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| *The official diagnostic criteria for acute [[retinal]] [[necrosis]] was proposed by the American [[Uveitis]] Society in 1994.
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| ==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>
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| **'''Acute stage''': Occurs at onset of disease and usually progresses past acute classification after a few weeks.
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| ***Presents with coalescence of white, necrotic tissue in the peripheral retina.
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| ***Vaso-[[occlusion|occlusive]] retinal [[vasculitis]] is usually present.
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| ***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.
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| **'''Late stage''': Is the natural progression of the disease and will present differentiating characteristics after a few weeks up to a few months.
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| ***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
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| ***[[Retinal detachment]], severe [[vision loss]], and potential [[blindness]] in the affected eye is indicative of late stage ARN.
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| ***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.
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| *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>
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| **'''Mild''': Is used to characterize ARN that is stable and non-progressive.
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| ***There is usually no sign of [[retinal detachment]].
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| **'''Fulminant''': ARN that is progressive and will usually lead to [[retinal detachment]] and further complications if untreated.
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| ==Pathophysiology== | | ==[[Acute retinal necrosis classification|Classification]]== |
| ===Pathogenesis===
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| *The pathogenesis of Acute retinal necrosis is characterized by [[retinal]] [[inflammation]] due to [[viral]] infection:
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| ==Causes== | | ==[[Acute retinal necrosis pathophysiology|Pathophysiology]]== |
| *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>
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| **''[[Herpes simplex virus]]'' 1 (HSV-1)
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| **''[[Herpes simplex virus]]'' 2 (HSV-2)
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| **[[Varicella-zoster virus]] (VZV)
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| **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>
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| *[[Varicella-zoster virus|VZV]] and [[Herpes simplex virus|HSV-1]] are usually the causes of ARN in individuals older than 25 years.
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| **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>
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| *[[Herpes simplex virus|HSV-2]] is usually the cause of ARN in individuals younger than 25 years.
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| ==Differentiating {{PAGENAME}} from Other Diseases== | | ==[[Acute retinal necrosis causes|Causes]]== |
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| ==Epidemiology and Demographics== | | ==[[Differentiating Acute retinal necrosis from other diseases|Differentiating Acute retinal necrosis from other Diseases]]== |
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| ==Risk Factors== | | ==[[Acute retinal necrosis epidemiology and demographics|Epidemiology and Demographics]]== |
| *Risk factors for the development of Acute retinal necrosis (ARN) include the following:
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| **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>
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| **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>
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| **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>
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| **[[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>
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| **[[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
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| ==Screening== | | ==[[Acute retinal necrosis risk factors|Risk Factors]]== |
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| ==Natural History, Complications, and Prognosis== | | ==[[Acute retinal necrosis screening|Screening]]== |
| ===Natural History===
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| ===Complications=== | | ==[[Acute retinal necrosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| ===Prognosis===
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| ==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>
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| *One or more discrete foci of peripheral [[retinal]] [[necrosis]], located outside of the major [[temporal]] [[vascular]] [[Arterial arcades|arcades]]
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| *Circumferential spread if [[Antiviral drug|antiviral therapy]] has not been administered
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| *[[Occlusion|Occlusive]] [[retinal]] vasculopathy
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| *A prominent [[vitreous]] or [[anterior chamber]] [[inflammation]]
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| *Rapid disease progression in the absence of therapy
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| ===Symptoms===
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| *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>
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| **[[Vision loss]]
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| ***[[Blindness]] may be present in more severe cases
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| **[[Photophobia|Excessive sensitivity to light]]
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| **[[Ocular]] pain
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| **[[Flu]] symptoms
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| **[[Erythema|Redness]] of the affected eye
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| **[[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>
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| **[[Flashes]]<ref name="urlAmerican Academy of Ophthalmology">{{cite web |url=http://www.aao.org/ |title=American Academy of Ophthalmology |format= |work= |accessdate=}}</ref>
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| ===Physical Examination===
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| 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>
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| *[[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>
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| *White and yellow [[necrosis|necrotic]] [[lesions]] in the [[retina]]
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| **[[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>
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| **Opaque [[vitreous]] from the coalescence of [[necrotic]] [[tissue]]
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| ===Laboratory Findings===
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| ===Imaging Findings===
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| ===Other Diagnostic Studies===
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| ==Treatment== | | ==Treatment== |
| ===Medical Therapy===
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| :*''' Empiric antimicrobial therapy'''
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| ::*Preferred regimen: [[Acyclovir]] 10 mg/kg IV q8h for 1-2 weeks followed by [[Acyclovir]] 400 mg PO bid for chronic maintenance
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| ::*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
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| ::*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)
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| ::*Note: [[Ganciclovir]] is administered for patients with suspected CMV acute retinal necrosis. Whereas [[Foscarnet]] is administered for patients who are not immunocompromised
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| :*''' Pathogen-directed antimicrobial therapy'''
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| ::*'''HSV or VZV'''
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| :::*Preferred regimen: [[Acyclovir]] 10 mg/kg IV q8h for 1-2 weeks followed by [[Acyclovir]] 400 mg PO bid for chronic maintenance
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| :::*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
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| ::*''' Cytomegalovirus'''
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| :::*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
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| ===Surgery===
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| ===Prevention===
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| ==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]]
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| * [[Progressive outer retinal necrosis]]
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| ==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
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| ==References== | | ==Source== |
| {{reflist|2}} | | [http://eyewiki.aao.org/Acute_retinal_necrosis American Academy of Ophthalmology] |
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