Acute retinal necrosis overview

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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

Chest X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

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Surgery

Primary Prevention

Secondary Prevention

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Case #1

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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.

Overview

Historical Perspective

Acute retinal necrosis was first discovered in 1971 by Urayama A, Yamada N, Sasaki T. Acute retinal necrosis was first officially classified as bilateral acute retinal necrosis in 1978 by N.J. Young and A.C. Bird, applied to 4 cases of bilateral necrotizing retinitis that progressed to retinal detachment and phthisis despite corticosteroid and antibiotic therapy. 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 may be classified both by staging - Acute or Late - or by severity: Mild or Fulminant.

Pathophysiology

The pathogenesis of Acute retinal necrosis is characterized by retinal inflammation due to ocular viral infection. Particles from Herpes simplex virus 1 (HSV-1), Herpes simplex virus 2 (HSV-2), and Varicella zoster virus (VZV) infiltrate the retina via various locations of epithelial penetration, including the skin, conjunctiva, cornea, and nasal cavity. 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 latency, reactivate, and cause retinal inflammation. For Caucasian populations: possessing the HLA-DQw7, HLA-Bw62, and HLA-DR4 antigens are correlated to genetic predisposition to ARN. For Japanese populations: possessing the HLA-Aw33, HLA-B44, and HLA-DRw6 antigens are correlated to genetic predisposition to ARN. Acute retinal necrosis is associated with the following ocular conditions: Progressive outer retinal necrosis, Uveitis, Cytomegalovirus retinitis, Toxoplasmic chorioretinitis, and Endophthalmitis.

Causes

Acute retinal necrosis is usually caused by reactivation of latent viruses: Herpes simplex virus 1 & 2, Varicella-zoster virus, cytomegalovirus, and Epstein-Barr virus.

Differentiating Acute retinal necrosis other Diseases

Acute retinal necrosis must be differentiated from other diseases that cause eye pain, conjunctival infection, photophobia, and vision loss.

Epidemiology and Demographics

Research in the United Kingdom resulted in an estimated incidence of approximately 6.3 per 100,000 individuals. Acute retinal necrosis (ARN) developed from Herpes simplex virus 1 and Varicella-zoster virus is most common among patients older than 50 years, while the incidence of HSV-2 caused ARN is highest in children and young adults between age 9 and 22 years. There is no racial or gender predisposition to Acute retinal necrosis.

Risk Factors

The primary risk factors for Acute retinal necrosis include Immunocompromise and immunosuppression from disease and prolonged corticosteroid use. Genetic predisposition for certain caucasian and Japanese populations heightens the possibility of developing Acute retinal necrosis.

Screening

There is no established, diagnostic screening process for Acute retinal necrosis.

Natural History, Complications and Prognosis

Diagnosis

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

References

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