Progressive outer retinal necrosis

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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.

Synonyms and Keywords: PORN syndrome

Overview

Progressive outer retinal necrosis, also known as PORN syndrome, is a form of necrotizing retinitis that primarily affects severely immunocompromised individuals. It is primarily caused by Varicella zoster virus infection, most commonly manifesting in patients who have AIDs or are undergoing chemotherapy. Symptoms of PORN syndrome primarily include vision loss—progressing to blindness if untreated—as well as other visual symptoms such as floaters and flashes. The clinical presentation of progressive outer retinal necrosis includes white-yellow necrotic lesions that coalesce to form a single film, as well as opacification of the retina and abnormal pigmentation. Unlike the associated condition acute retinal necrosis, PORN syndrome does not usually manifest with anterior chamber or vitreous inflammation, nor do patients usually experience eye pain. It is important to differentiate PORN syndrome from other ocular conditions to prevent complications, including blindness, retinal detachment, and cataracts. If left untreated, the prognosis of PORN syndrome for visual acuity is poor: approximately 67% of cases will lead to blindness in the affected eye (and 61% cases of PORN syndrome will spread to the previously unaffected eye). Medical therapy should be started as early as possible to stop the progression of symptoms. The mainstay of therapy is intravenous antimicrobial therapy, usually a combination of Ganciclovir and Foscarnet. Individual uses of the two therapies, in addition to Ancyclovir, may be indicated for less severe cases.

Historical Perspective

Classification

There is no official classification schema for progressive outer retinal necrosis.

Pathophysiology

Pathogenesis

The pathogenesis of progressive outer retinal necrosis (PORN) is characterized by retinal necrosis due to ocular viral infection.[3] Viral particles infiltrate the retina via various modes of transmission:[4]

Retinal inflammation is caused by the up-regulated production of cytokines.

Associated Conditions

Progressive outer retinal necrosis is associated with the following ocular conditions:

PORN is often associated with AIDS as a complication of immunocompromised status.[9]

Causes

Progressive outer retinal necrosis (PORN) is primarily caused by Varicella zoster virus (VZV), and is less commonly caused by Cytomegalovirus (CMV), Herpes simplex virus 1, and rarely BK Virus. PORN usually appears in immunocompromised individuals, usually as a complication of diseases such as AIDS or from chemotherapy.[10][11]

Viral

Differentiating Progressive outer retinal necrosis from Other Diseases

Epidemiology and Demographics

Epidemiological and demographic data for progressive outer retinal necrosis (PORN) are closely tied to that of AIDS, of which PORN is often a complication.

Gender

  • Females are more likely than males to develop progressive outer retinal necrosis.[19]

Age

  • Progressive outer retinal necrosis occurs more frequently in individuals over the age of 35.[19][20]

Developing countries

  • Incidences of PORN are higher in developing countries, particularly those in Africa, due to the higher local prevalence of AIDS.[19]

Risk Factors

Risk factors for progressive outer retinal necrosis include the following:

Screening

There is no established, diagnostic screening procedure for progressive outer retinal necrosis.

Natural History, Complications, and Prognosis

Natural History

  • Early clinical findings of progressive outer retinal necrosis (PORN) include white-yellow necrotic peripheral and macular retinal lesions, as well as opacification of non-necrotic tissue, indicative of the onset of disease.[3]
  • Without treatment, the necrotic lesions will rapidly coalesce into a unified film, progressing to complete retinal necrosis.[10]
  • Complete retinal detachment will usually occur between 30 days and 3 months after onset.[11]
    • Blindness usually follows between 4 weeks and 6 months after the PORN diagnosis.[23]
  • PORN will usually spread to the previously unaffected eye within 4 weeks.

Complications

The following complications of progressive outer retinal necrosis occur, if left untreated, from complete retinal necrosis:[11]

Prognosis

  • Without treatment, the prognosis for vision acuity in the affected eyes is poor and it is highly likely that it will become bilateral.[3]
    • Approximately 67% of progressive outer retinal necrosis cases will progress to blindness if left untreated.[10]
    • Approximately 70% of progressive outer retinal necrosis cases will progress to retinal detachment.
    • Approximately 61% of progressive outer retinal necrosis cases will become bilateral.
  • With treatment, the prognosis varies:[23]

Diagnosis

Diagnostic Criteria

The following standardized criteria are used to officially diagnose progessive outer retinal necrosis:[10]

  • Presence of multifocal lesions without granular borders in the deep retinal layers
  • Evidence that the infection started in the peripheral retina with or without focal involvement
  • Extremely rapid progression
  • Presence of minimal intraocular infection

History and Symptoms

History

A history of immunocompromising disease and/or therapy may be present in progressive outer retinal necrosis patients, particularly the following:[21][10][19][22]

Symptoms

Symptoms of progressive outer retinal necrosis include the following:[10]

Physical Examination

Physical examination for progressive outer retinal necrosis may be remarkable for the following:

Laboratory Findings

Laboratory findings associated with progressive outer retinal necrosis (PORN) are those used to confirm the Varicella zoster virus (VZV) infection, obtained from aqueous humor or the vitreous. Useful laboratory techniques may include:[25]

  • Qualitative and real-time polymerase chain reaction may produce genomic evidence of VZV infection with high specificity[24][26]
  • Viral cultures may reveal evidence of VZV infection indicative of PORN
    • Retinochoroidal biopsy may be performed to obtain a culture sample, in addition to direct sampling from the aqueous humor[9]
    • Diagnosis via viral culture alone is not recommended due to the low specificity and sensitivity (53.7% and 46.3%, respectively), indicating a high chance of obtaining a false-negative.[26]
  • Immunofluorescence may reveal antibodies indicative of VZV infection[27]
  • Detection of indicative Varicella zoster virus antibodies via Goldmann-Witmer coefficient[28]

Imaging Findings

Other Diagnostic Studies

There are no other diagnostic studies associated with progressive outer retinal necrosis.

Treatment

Medical Therapy

The mainstay of therapy for progressive outer retinal necrosis (PORN) is Highly Active Anti-Retroviral Therapy (HAART), consisting of the following regimens:[23][9]

  • Empiric antimicrobrial therapy
  • Note: The combination antimicrobial therapy of Ganciclovir and Foscarnet is recommended as the most effective treatment regiment for halting the progression of PORN. Single antimicrobrial therapy is not usually recommended.[32]

Surgery

Surgery is not the first-line treatment option for patients with progressive outer retinal necrosis; it is primarily indicated when there is risk of complications, including retinal detachment and tissue atrophy.[33]

Vitrectomy

Prophylactic Laser Retinopexy

Prevention

Effective measures for the prevention of progressive outer retinal necrosis include the following:

Source

American Academy of Ophthalmology

See also

References

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  2. Forster DJ, Dugel PU, Frangieh GT, Liggett PE, Rao NA (1990). "Rapidly progressive outer retinal necrosis in the acquired immunodeficiency syndrome". Am. J. Ophthalmol. 110 (4): 341–8. PMID 2220967.
  3. 3.0 3.1 3.2 3.3 3.4 Moorthy, R. S; Weinberg, D. V; Teich, S. A; Berger, B. B; Minturn, J. T; Kumar, S.; Rao, N. A; Fowell, S. M; Loose, I. A; Jampol, L. M (1997). "Management of varicella zoster virus retinitis in AIDS". British Journal of Ophthalmology. 81 (3): 189–194. doi:10.1136/bjo.81.3.189. ISSN 0007-1161.
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  10. 10.0 10.1 10.2 10.3 10.4 10.5 10.6 10.7 Engstrom RE, Holland GN, Margolis TP, Muccioli C, Lindley JI, Belfort R, Holland SP, Johnston WH, Wolitz RA, Kreiger AE (1994). "The progressive outer retinal necrosis syndrome. A variant of necrotizing herpetic retinopathy in patients with AIDS". Ophthalmology. 101 (9): 1488–502. PMID 8090452.
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  30. Al-Dhibi HA, Al-Mahmood AM, Arevalo JF (2014). "A systematic approach to emergencies in uveitis". Middle East Afr J Ophthalmol. 21 (3): 251–8. doi:10.4103/0974-9233.134687. PMC 4123279. PMID 25100911.
  31. Yeh S, Wong WT, Weichel ED, Lew JC, Chew EY, Nussenblatt RB (2010). "Fundus Autofluorescence and OCT in the Management of Progressive Outer Retinal Necrosis". Ophthalmic Surg Lasers Imaging: 1–4. doi:10.3928/15428877-20100216-14. PMC 3265678. PMID 20337261.
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  33. Shantha JG, Weissman HM, Debiec MR, Albini TA, Yeh S (2015). "Advances in the management of acute retinal necrosis". Int Ophthalmol Clin. 55 (3): 1–13. doi:10.1097/IIO.0000000000000077. PMC 4567584. PMID 26035758.
  34. Luo YH, Duan XC, Chen BH, Tang LS, Guo XJ (2012). "Efficacy and necessity of prophylactic vitrectomy for acute retinal necrosis syndrome". Int J Ophthalmol. 5 (4): 482–7. doi:10.3980/j.issn.2222-3959.2012.04.15. PMC 3428546. PMID 22937510.
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