Progressive outer retinal necrosis: Difference between revisions
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===Other Diagnostic Studies=== | ===Other Diagnostic Studies=== | ||
There are no other diagnostic studies associated with Progressive outer retinal necrosis. | |||
==Treatment== | ==Treatment== | ||
Revision as of 17:17, 2 September 2016
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Luke Rusowicz-Orazem, B.S.
Overview
Historical Perspective
- A clinical presentation of necrotizing retinitis occurring specifically in immunocompromised individuals was first documented in 1987 by Jabs DA et al.[1]
- Progressive outer retinal necrosis (PORN) was first identified by Forster, DJ et al. in 1990.[2]
- It was identified and differentiated from Acute retinal necrosis by its rapid progression, unresponsiveness to antiviral therapy acyclovir, and resultant retinal detachment.
- Study of two HIV-positive individuals led to the identification of Varicella-zoster infection as the cause for PORN.
- Immunocompromise was recognized to be an important differentiation from Acute retinal necrosis patients.
Classification
There is no official diagnostic 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 from Varicella zoster virus.[3]
- Particles from Varicella-zoster virus (VZV) infiltrate the retina via various modes of transmission:[4]
- Epithelial penetration of the skin: transmitted through the ophthalmic branch of the Trigeminal nerve.
- Epithelial penetration of the conjunctiva: transmitted directly through the optic nerve.
- Epithelial penetration of the cornea: transmitted through the maxillary branch of the Trigeminal nerve.
- Epithelial penetration of the nasal cavity: transmitted through the Olfactory nerve in the Subarachnoid space.
- 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 HIV virus infection and AIDS as a complication of immunocompromise.[8]
Causes
- Progressive outer retinal necrosis (PORN) is primarily caused by Varicella zoster virus (VZV).
- PORN usually results from immunocompromised individuals infected with VZV, usually as a complication of diseases such as AIDS or from chemotherapy.[9][10]
Differentiating Progressive outer retinal necrosis from Other Diseases
- Progressive outer retinal necrosis (PORN) must be differentiated from other diseases that cause eye pain, conjunctival infection, photophobia, and vision loss. Accurate and prompt diagnosis is critical to prevent blindness and complications.[7][11][12][13]
- PORN is differentiated from Acute retinal necrosis by its occurrence primarily in immunocompromised individuals; Acute retinal necrosis will also develop in immunocompetent individuals.
Epidemiology and Demographics
Epidemiological and demographic data for Progressive outer retinal necrosis (PORN) is closely tied to that of AIDs, of which PORN is often a complication of.
Gender
- Females are more likely than males to develop Progressive outer retinal necrosis.[15]
Age
Developing countries
- Incidences of PORN are higher in developing countries due to the higher prevalence in AIDs, particularly in Africa.[15]
Risk Factors
- Risk factors for Progressive outer retinal necrosis include the following:
- Immunocompromise from prior or concurrent disease, particularly AIDs.[17][9][15]
- Immunosuppresion from extended corticosteroid or chemotherapy.[18]
- Being female
- Being older than 35 years
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 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 from onset.[10]
- PORN will usually spread to the previously-unaffected eye within 4 weeks.
Complications
Complications of Progressive outer retinal necrosis occur, if untreated, from the complete retinal necrosis:[10]
- Retinal detachment
- Cataracts
- Retinal atrophy[19]
- Permanent vision loss and blindness[19]
- Pigmentation scarring
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 lead to blindness if left untreated.[9]
- Approximately 70% of Progressive outer retinal necrosis cases will lead to retinal detachment.
- Approximately 61% of Progressive outer retinal necrosis cases will become bilateral.
- With treatment, the prognosis varies:[19]
- Foscarnet and ganciclovir antiviral intravenous therapy can improve prognosis if administered during the early stages.
- Vitrectomy can successfully prevent retinal detachment.
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 therapy may be present in Progressive outer retinal necrosis patients, particularly the following:[17][9][15]
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:
- White-yellow necrotic peripheral and macular retinal lesions, coalescing to form a single film[3]
- Deep opacification of the peripheral retina[9]
- Abnormal retinal pigmentation
- Absence or minimal presence of anterior chamber or vitreous inflammation, as well as absence of scleritis.[20]
- This is an important characteristic in differentiating a Progressive outer retinal necrosis diagnosis from Acute retinal necrosis.
Laboratory Findings
Laboratory findings associated with Progressive outer retinal necrosis (PORN) are those used to determine the Varicella zoster virus (VZV) infection, obtained from aqueous humor or the vitreous:[21]
- Qualitative and Real-time Polymerase chain reaction: may produce genomic evidence of VZV infection with high specificity.[20][22]
- Direct flourescent antibody assays may reveal
- Viral cultures may reveal evidence of VZV infection indicative of PORN.
- 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
Ophthalmoscopy is the imaging modality of choice for Progressive outer retinal necrosis and is characterized by the following findings:[20][25][8][26]
- Retinal opacity
- Superficial retinal hemorrhage
- Optic disk swelling
- Vitritis
- Retinal and choroidal detachment
- White-yellow, multifocal retinal lesions with "cherry red spot" central macula
- Absence of vitreous or anterior chamber inflammation, important in the differential diagnosis from Acute retinal necrosis
Other Diagnostic Studies
There are no other diagnostic studies associated with Progressive outer retinal necrosis.
Treatment
Medical Therapy
Surgery
Prevention
See also
- Cytomegalovirus retinitis
- List of eye diseases and disorders
- List of systemic diseases with ocular manifestations
References
- ↑ 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.
- ↑ 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.
- ↑ 3.0 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.
- ↑ 4.0 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.
- ↑ 5.0 5.1 5.2 "Facts About Uveitis | National Eye Institute".
- ↑ 6.0 6.1 "CMV retinitis: MedlinePlus Medical Encyclopedia".
- ↑ 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.
- ↑ 8.0 8.1 8.2 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.
- ↑ 9.0 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.
- ↑ 10.0 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.
- ↑ Dart JK (1986). "Eye disease at a community health centre". Br Med J (Clin Res Ed). 293 (6560): 1477–80. PMC 1342247. PMID 3099921.
- ↑ Leibowitz HM (2000). "The red eye". N Engl J Med. 343 (5): 345–51. doi:10.1056/NEJM200008033430507. PMID 10922425.
- ↑ University of Michigan Eyes Have it (2009)http://kellogg.umich.edu/theeyeshaveit/red-eye/
- ↑ 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.
- ↑ 15.0 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.
- ↑ 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.
- ↑ 17.0 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.
- ↑ 18.0 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.
- ↑ 19.0 19.1 19.2 19.3 19.4 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.
- ↑ 20.0 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.
- ↑ 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.
- ↑ 22.0 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.