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==Overview==
==Overview==

Revision as of 13:57, 12 August 2016

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

Overview

Acute retinal necrosis is a type of retinitis which can be associated with viral infections.

It was first characterized in 1971.[1][2]

One study indicated an incidence of 1 per 1.6 to 2.0 million.[3]

Historical Perspective

Classification

  • Acute retinal necrosis (ARN) may be classified by staging and severity into the following:[7]
    • 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-occlusive retinal vasculitis is usually present.
      • The 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:[8]
    • Mild: Is used to characterize ARN that is stable and non-progressive.
    • Fulminant: ARN that is progressive and will usually lead to retinal detachment and further complications if untreated.

Pathophysiology

Pathogenesis

Causes

Differentiating Acute retinal necrosis from Other Diseases

Epidemiology and Demographics

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.[11]
    • For Japanese populations: possessing the HLA-Aw33, HLA-B44, and HLA-DRw6 antigens are correlated to genetic predisposition to ARN.[8]
    • Experiencing encephalitis from herpes simplex virus[12]
    • Immunocompromise from prior or concurrent disease.[13]
    • Immunosuppresion from extended corticosteroid therapy.[14]

Screening

Natural History, Complications, and Prognosis

Natural History

Complications

Prognosis

Diagnosis

Diagnostic Criteria

The diagnosis of acute retinal necrosis is made when the following criteria are met:[15]

Symptoms

Physical Examination

Physical examination for acute retinal necrosis is remarkable for the following:[8]

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

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

External links

References

  1. "eMedicine - Acute Retinal Necrosis : Article by Andrew A Dahl, MD". Archived from the original on 16 February 2008. Retrieved 2008-02-05.
  2. 2.0 2.1 Urayama A, Yamada N, Sasaki T: Unilateral acute uveitis with retinal periarteritis and detachment. Jpn J Clin Ophthalmol 1971; 25: 607.
  3. 3.0 3.1 3.2 Muthiah MN, Michaelides M, Child CS, Mitchell SM (2007). "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. 91 (11): 1452–5. doi:10.1136/bjo.2007.114884. PMC 2095441. PMID 17504853.
  4. 4.0 4.1 Young NJ, Bird AC (1978). "Bilateral acute retinal necrosis". Br J Ophthalmol. 62 (9): 581–90. PMC 1043304. PMID 708676.
  5. 5.0 5.1 Flaxel CJ, Yeh S, Lauer AK (2013). "Combination systemic and intravitreal antiviral therapy in the management of acute retinal necrosis syndrome (an American Ophthalmological Society thesis)". Trans Am Ophthalmol Soc. 111: 133–44. PMC 3868412. PMID 24385671.
  6. Hayasaka S, Asano T, Yabata K, Ide A (1983). "Acute retinal necrosis". Br J Ophthalmol. 67 (7): 455–60. PMC 1040094. PMID 6860612.
  7. Gartry DS, Spalton DJ, Tilzey A, Hykin PG (1991). "Acute retinal necrosis syndrome". Br J Ophthalmol. 75 (5): 292–7. PMC 1042358. PMID 1645179.
  8. 8.0 8.1 8.2 8.3 Brydak-Godowska J, Borkowski P, Szczepanik S, Moneta-Wielgoś J, Kęcik D (2014). "Clinical manifestation of self-limiting acute retinal necrosis". Med. Sci. Monit. 20: 2088–96. doi:10.12659/MSM.890469. PMC 4226315. PMID 25356955.
  9. Pikkel YY, Pikkel J (2014). "Acute retinal necrosis in childhood". Case Rep Ophthalmol. 5 (2): 138–43. doi:10.1159/000363130. PMC 4049010. PMID 24932179.
  10. Ganatra JB, Chandler D, Santos C, Kuppermann B, Margolis TP (2000). "Viral causes of the acute retinal necrosis syndrome". Am. J. Ophthalmol. 129 (2): 166–72. PMID 10682968.
  11. Holland GN, Cornell PJ, Park MS, Barbetti A, Yuge J, Kreiger AE, Kaplan HJ, Pepose JS, Heckenlively JR, Culbertson WW (1989). "An association between acute retinal necrosis syndrome and HLA-DQw7 and phenotype Bw62, DR4". Am. J. Ophthalmol. 108 (4): 370–4. PMID 2801857.
  12. Vandercam T, Hintzen RQ, de Boer JH, Van der Lelij A (2008). "Herpetic encephalitis is a risk factor for retinal necrosis". Neurology. 71 (16): 1268–74. doi:10.1212/01.wnl.0000327615.99124.99. PMID 18852442.
  13. Moutschen MP, Scheen AJ, Lefebvre PJ (1992). "Impaired immune responses in diabetes mellitus: analysis of the factors and mechanisms involved. Relevance to the increased susceptibility of diabetic patients to specific infections". Diabete Metab. 18 (3): 187–201. PMID 1397473.
  14. Yamamoto JH, Boletti DI, Nakashima Y, Hirata CE, Olivalves E, Shinzato MM, Okay TS, Santo RM, Duarte MI, Kalil J (2003). "Severe bilateral necrotising retinitis caused by Toxoplasma gondii in a patient with systemic lupus erythematosus and diabetes mellitus". Br J Ophthalmol. 87 (5): 651–2. PMC 1771672. PMID 12714420.
  15. Holland GN (1994). "Standard diagnostic criteria for the acute retinal necrosis syndrome. Executive Committee of the American Uveitis Society". Am. J. Ophthalmol. 117 (5): 663–7. PMID 8172275.
  16. Ford JR, Tsui E, Lahey T, Zegans ME (2013). "Question: Can you identify this condition? Acute retinal necrosis". Can Fam Physician. 59 (12): 1307, 1308–10. PMC 3860929. PMID 24336545.
  17. "American Academy of Ophthalmology".