Progressive outer retinal necrosis: Difference between revisions
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Revision as of 17:46, 30 August 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
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.[9]
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][10][11][12]
- 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
Risk Factors
Screening
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.[14]
- 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:[14]
- Retinal detachment
- Cataracts
- Retinal atrophy[15]
- Permanent vision loss and blindness[15]
- Pigmentation scarring
Prognosis
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
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.[16]
- This is an important characteristic in differentiating a Progressive outer retinal necrosis diagnosis from Acute retinal necrosis.
Laboratory Findings
Imaging Findings
Other Diagnostic Studies
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 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 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 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.
- ↑ 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.
- ↑ 14.0 14.1 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.
- ↑ 15.0 15.1 15.2 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.
- ↑ 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.