Progressive outer retinal necrosis

Jump to navigation Jump to search

WikiDoc Resources for Progressive outer retinal necrosis

Articles

Most recent articles on Progressive outer retinal necrosis

Most cited articles on Progressive outer retinal necrosis

Review articles on Progressive outer retinal necrosis

Articles on Progressive outer retinal necrosis in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Progressive outer retinal necrosis

Images of Progressive outer retinal necrosis

Photos of Progressive outer retinal necrosis

Podcasts & MP3s on Progressive outer retinal necrosis

Videos on Progressive outer retinal necrosis

Evidence Based Medicine

Cochrane Collaboration on Progressive outer retinal necrosis

Bandolier on Progressive outer retinal necrosis

TRIP on Progressive outer retinal necrosis

Clinical Trials

Ongoing Trials on Progressive outer retinal necrosis at Clinical Trials.gov

Trial results on Progressive outer retinal necrosis

Clinical Trials on Progressive outer retinal necrosis at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Progressive outer retinal necrosis

NICE Guidance on Progressive outer retinal necrosis

NHS PRODIGY Guidance

FDA on Progressive outer retinal necrosis

CDC on Progressive outer retinal necrosis

Books

Books on Progressive outer retinal necrosis

News

Progressive outer retinal necrosis in the news

Be alerted to news on Progressive outer retinal necrosis

News trends on Progressive outer retinal necrosis

Commentary

Blogs on Progressive outer retinal necrosis

Definitions

Definitions of Progressive outer retinal necrosis

Patient Resources / Community

Patient resources on Progressive outer retinal necrosis

Discussion groups on Progressive outer retinal necrosis

Patient Handouts on Progressive outer retinal necrosis

Directions to Hospitals Treating Progressive outer retinal necrosis

Risk calculators and risk factors for Progressive outer retinal necrosis

Healthcare Provider Resources

Symptoms of Progressive outer retinal necrosis

Causes & Risk Factors for Progressive outer retinal necrosis

Diagnostic studies for Progressive outer retinal necrosis

Treatment of Progressive outer retinal necrosis

Continuing Medical Education (CME)

CME Programs on Progressive outer retinal necrosis

International

Progressive outer retinal necrosis en Espanol

Progressive outer retinal necrosis en Francais

Business

Progressive outer retinal necrosis in the Marketplace

Patents on Progressive outer retinal necrosis

Experimental / Informatics

List of terms related to Progressive outer retinal necrosis

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

Associated Conditions

Causes

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.[15]

Age

  • Progressive outer retinal necrosis occurs more frequently in individuals over the age of 35.[15][16]

Developing countries

  • Incidences of PORN are higher in developing countries, particularly those in Africa, due to the higher local prevalence of AIDS.[15]

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.[9]
  • Complete retinal detachment will usually occur between 30 days and 3 months after onset.[10]
    • Blindness usually follows between 4 weeks and 6 months after the PORN diagnosis.[19]
  • 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:[10]

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.[9]
    • 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:[19]

Diagnosis

Diagnostic Criteria

The following standardized criteria are used to officially diagnose progessive outer retinal necrosis:[9]

  • 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:[17][9][15][18]

Symptoms

Symptoms of progressive outer retinal necrosis include the following:[9]

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:[21]

  • Qualitative and real-time polymerase chain reaction may produce genomic evidence of VZV infection with high specificity[20][22]
  • 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[8]
    • 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.[22]
  • Immunoflourescence may reveal antibodies indicative of VZV infection[23]
  • Detection of indicative Varicella zoster virus antibodies via Goldmann-Witmer coefficient[24]

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:[19][8]

  • Empiric antimicrobrial therapy
  • Note: The combination antimicrobial therapy of Ganciclovir and Foscarnet is recommended as the most effective treatment regiment for halting progression of PORN. Single antimicrobrial therapy is not usually recommended.[28]

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.[29]

Vitrectomy

Prophylactic Laser Retinopexy

Prevention

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

Source

American Academy of Ophthalmology

See also

References

  1. Jabs DA, Schachat AP, Liss R, Knox DL, Michels RG (1987). "Presumed varicella zoster retinitis in immunocompromised patients". Retina (Philadelphia, Pa.). 7 (1): 9–13. PMID 3602608.
  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.
  4. 4.0 4.1 Grose C (2012). "Acute retinal necrosis caused by herpes simplex virus type 2 in children: reactivation of an undiagnosed latent neonatal herpes infection". Semin Pediatr Neurol. 19 (3): 115–8. doi:10.1016/j.spen.2012.02.005. PMC 3419358. PMID 22889540.
  5. 5.0 5.1 5.2 "Facts About Uveitis | National Eye Institute".
  6. 6.0 6.1 "CMV retinitis: MedlinePlus Medical Encyclopedia".
  7. 7.0 7.1 7.2 Davis JL (2012). "Diagnostic dilemmas in retinitis and endophthalmitis". Eye (Lond). 26 (2): 194–201. doi:10.1038/eye.2011.299. PMC 3272204. PMID 22116459.
  8. 8.0 8.1 8.2 8.3 8.4 Galindez OA, Sabates NR, Whitacre MM, Sabates FN (1996). "Rapidly progressive outer retinal necrosis caused by varicella zoster virus in a patient infected with human immunodeficiency virus". Clin. Infect. Dis. 22 (1): 149–51. PMID 8824984.
  9. 9.0 9.1 9.2 9.3 9.4 9.5 9.6 9.7 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.
  10. 10.0 10.1 10.2 Austin RB (2000). "Progressive outer retinal necrosis syndrome: a comprehensive review of its clinical presentation, relationship to immune system status, and management". Clin. Eye Vis. Care. 12 (3–4): 119–129. PMID 11137426.
  11. Dart JK (1986). "Eye disease at a community health centre". Br Med J (Clin Res Ed). 293 (6560): 1477–80. PMC 1342247. PMID 3099921.
  12. Leibowitz HM (2000). "The red eye". N Engl J Med. 343 (5): 345–51. doi:10.1056/NEJM200008033430507. PMID 10922425.
  13. University of Michigan Eyes Have it (2009)http://kellogg.umich.edu/theeyeshaveit/red-eye/
  14. Abu El-Asrar AM, Herbort CP, Tabbara KF (2009). "Differential diagnosis of retinal vasculitis". Middle East Afr J Ophthalmol. 16 (4): 202–18. doi:10.4103/0974-9233.58423. PMC 2855661. PMID 20404987.
  15. 15.0 15.1 15.2 15.3 15.4 Gore DM, Gore SK, Visser L (2012). "Progressive outer retinal necrosis: outcomes in the intravitreal era". Arch. Ophthalmol. 130 (6): 700–6. doi:10.1001/archophthalmol.2011.2622. PMID 22801826.
  16. Sittivarakul W, Aui-aree N (2009). "Clinical features, management and outcomes of progressive outer retinal necrosis (PORN) in southern Thailand". J Med Assoc Thai. 92 (3): 360–6. PMID 19301729.
  17. 17.0 17.1 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.
  18. 18.0 18.1 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.
  19. 19.0 19.1 19.2 19.3 19.4 19.5 Moorthy RS, Weinberg DV, Teich SA, Berger BB, Minturn JT, Kumar S, Rao NA, Fowell SM, Loose IA, Jampol LM (1997). "Management of varicella zoster virus retinitis in AIDS". Br J Ophthalmol. 81 (3): 189–94. PMC 1722141. PMID 9135381.
  20. 20.0 20.1 20.2 Coisy S, Ebran JM, Milea D (2014). "Progressive outer retinal necrosis and immunosuppressive therapy in myasthenia gravis". Case Rep Ophthalmol. 5 (1): 132–7. doi:10.1159/000362662. PMC 4036147. PMID 24926266.
  21. Matos K, Muccioli C, Belfort Junior R, Rizzo LV (2007). "Correlation between clinical diagnosis and PCR analysis of serum, aqueous, and vitreous samples in patients with inflammatory eye disease". Arq Bras Oftalmol. 70 (1): 109–14. PMID 17505729.
  22. 22.0 22.1 Wilson DA, Yen-Lieberman B, Schindler S, Asamoto K, Schold JD, Procop GW (2012). "Should varicella-zoster virus culture be eliminated? A comparison of direct immunofluorescence antigen detection, culture, and PCR, with a historical review". J. Clin. Microbiol. 50 (12): 4120–2. doi:10.1128/JCM.06753-11. PMC 3502980. PMID 23035203.
  23. Singh A, Preiksaitis J, Ferenczy A, Romanowski B (2005). "The laboratory diagnosis of herpes simplex virus infections". Can J Infect Dis Med Microbiol. 16 (2): 92–8. PMC 2095011. PMID 18159535.
  24. De Groot-Mijnes JD, Rothova A, Van Loon AM, Schuller M, Ten Dam-Van Loon NH, De Boer JH, Schuurman R, Weersink AJ (2006). "Polymerase chain reaction and Goldmann-Witmer coefficient analysis are complimentary for the diagnosis of infectious uveitis". Am. J. Ophthalmol. 141 (2): 313–8. doi:10.1016/j.ajo.2005.09.017. PMID 16458686.
  25. You YS, Lee SJ, Lee SH, Park CH, Kwon OW (2007). "Progressive outer retinal necrosis combined with vitreous hemorrhage in a patient with acquired immunodeficiency syndrome". Korean J Ophthalmol. 21 (1): 51–4. doi:10.3341/kjo.2007.21.1.51. PMC 2629688. PMID 17460434.
  26. 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.
  27. 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.
  28. Ciulla TA, Rutledge BK, Morley MG, Duker JS (1998). "The progressive outer retinal necrosis syndrome: successful treatment with combination antiviral therapy". Ophthalmic Surg Lasers. 29 (3): 198–206. PMID 9547773.
  29. 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.
  30. 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.
  31. 31.0 31.1 Kawaguchi T, Spencer DB, Mochizuki M (2008). "Therapy for acute retinal necrosis". Semin Ophthalmol. 23 (4): 285–90. doi:10.1080/08820530802111192. PMID 18584565.
  32. McDonald HR, Lewis H, Kreiger AE, Sidikaro Y, Heckenlively J (1991). "Surgical management of retinal detachment associated with the acute retinal necrosis syndrome". Br J Ophthalmol. 75 (8): 455–8. PMC 1042429. PMID 1873262.
  33. 33.0 33.1 Park JJ, Pavesio C (2008). "Prophylactic laser photocoagulation for acute retinal necrosis. Does it raise more questions than answers?". Br J Ophthalmol. 92 (9): 1161–2. doi:10.1136/bjo.2008.147181. PMID 18723739.