O OftalmopediaAn open ophthalmology reference ⌕

Progressive outer retinal necrosis

AI overview

Progressive outer retinal necrosis (PORN) syndrome is a form of chorioretinitis, an infection in the retina, the back of the eye.

Key points
  • Most commonly caused by varicella zoster virus
    source quote
    The disease is most commonly caused by the varicella zoster virus
  • Found almost exclusively in patients with HIV/AIDS
    source quote
    is found almost exclusively in patients with HIV/AIDS (acquired immunodeficiency syndrome)
  • Second most common opportunistic retinal infection in North America among people with AIDS
    source quote
    Progressive outer retinal necrosis is the second most common opportunistic retinal infection in North America among people with AIDS.
  • Falls under the umbrella of necrotizing herpetic retinopathy along with Acute retinal necrosis
    source quote
    The syndrome, PORN, falls under the umbrella of necrotizing herpetic retinopathy, along with Acute retinal necrosis
  • Hallmark feature is absence of intraocular inflammation and vascular involvement
    source quote
    this disease process has no signs of intraocular inflammation, the hallmark feature, or vascular involvement
  • Presents with multiple lesions in the peripheral retina
    source quote
    PORN is a chorioretinitis presenting with multiple lesions in the peripheral retina
Symptoms
  • Blurry vision
    source quote
    Blurry vision
  • Acute onset bilateral visual loss or painless progressive visual loss
    source quote
    Acute onset bilateral visual loss OR painless progressive visual loss
  • Decreased vision with pain
    source quote
    Decreased vision with pain
  • Floaters
    source quote
    Floaters
  • Headaches
    source quote
    Headaches
Diagnosis
  • Diagnosis is made primarily through history and physical exam
    source quote
    The diagnosis of Progressive outer retinal necrosis (PORN) syndrome is made primarily through history and physical exam.
  • Medical history of AIDS increases likelihood of PORN diagnosis
    source quote
    A patient's medical history of AIDS increases the likelihood of PORN as the diagnosis.
  • Eye exam shows opacified lesions in peripheral retina without inflammation
    source quote
    An eye exam will show specific hallmark features, including opacified lesions in the peripheral retina without inflammation.
  • Optical coherence tomography allows examination of outer and inner layers of the retina
    source quote
    Optical coherence tomography (OCT): this test allows ophthalmologists to look at outer and inner layers of the retina
  • Vitreous tap analysis can show the causative organism such as VZV
    source quote
    Vitreous Tap: Analysis of the fluid in the eye can show the causative organism, such as VZV.
Treatment
  • HAART treatment for HIV/AIDS reduced severity and frequency of chorioretinitis diseases
    source quote
    With the use of HAART (3 drug regimen standard) treatment for HIV/AIDS in the 1990s, the presentation of these chorioretinitis diseases became less severe and less frequent.
  • Treatment includes anti-viral medications Ganciclovir and Foscarnet
    source quote
    Treatment for Necrotizing Herpetic Retinopathy, including ARN and PORN, includes the anti-viral medications Ganciclovir and Foscarnet.
  • Medications are given as intra-vitreal injections
    source quote
    These medications are given as intra-vitreal injections (injected into the fluid of the eye).
  • Treatment has shown success in reversing disease process and improving vision
    source quote
    This treatment has shown success in reversing the disease process and improving vision to an extent.
Red flags
  • Retinal detachments are surgical emergencies that can lead to blindness
    source quote
    Retinal detachments are considered surgical emergencies, as they can lead to blindness if not promptly fixed.
  • If untreated, PORN can become bilateral affecting both eyes
    source quote
    If left untreated, PORN syndrome can become bilateral, affecting both eyes.
Prognosis

If left untreated, PORN can become bilateral, cause retinal atrophy, and lead to retinal detachment which can result in blindness if not promptly fixed.

AI-synthesized from the Wikipedia article “Progressive outer retinal necrosis”. Not medical advice. Verify source →
Progressive outer retinal necrosis
Other namesVaricella zoster virus retinitis (VZVR),[1]
The typical "cracked mud" appearance of the fundus in progressive outer retinal necrosis[2][3]
SpecialtyOphthalmology, infectious diseases

Progressive outer retinal necrosis (PORN) syndrome is a form of chorioretinitis, an infection in the retina, the back of the eye. The disease is most commonly caused by the varicella zoster virus and is found almost exclusively in patients with HIV/AIDS (acquired immunodeficiency syndrome). Progressive outer retinal necrosis is the second most common opportunistic retinal infection in North America among people with AIDS.[4] The reason this disease process is considered opportunistic is precisely because it only presents in patients with AIDS, a disease that attacks and weakens the immune system, making space for other infections, like Varicella zoster virus (VZV) and Herpes simplex virus (HSV), to attack the body.

The syndrome, PORN, falls under the umbrella of necrotizing herpetic retinopathy, along with Acute retinal necrosis. Although fairly similar processes, the two forms of retinopathy have different presenting symptoms. Acute Retinal Necrosis, or ARN, is damage more central in the retinal portion of the eye with dead or necrotic areas well defined. ARN also presents with inflammation within the eye and is often painful.[5] PORN is a chorioretinitis presenting with multiple lesions in the peripheral retina. Unlike ARN, this disease process has no signs of intraocular inflammation, the hallmark feature, or vascular involvement.[6][4]

The presentation of the disease is dependent on the patient's AIDS prognosis. If the immune system has been significantly weakened by AIDS, a patient will likely present with worse and faster progressing PORN.[5] Commonly presenting symptoms of PORN include:[citation needed]

  • Blurry vision
  • Acute onset bilateral visual loss OR painless progressive visual loss
  • Decreased vision with pain
  • Floaters
  • Headaches

The diagnosis of Progressive outer retinal necrosis (PORN) syndrome is made primarily through history and physical exam. A patient's medical history of AIDS increases the likelihood of PORN as the diagnosis. An eye exam will show specific hallmark features, including opacified lesions in the peripheral retina without inflammation.[citation needed]

There are medical tests that can aid in diagnosing the severity of the disease process, however are not necessary for initial diagnosis.[citation needed]

  • Imaging studies: may help diagnose the extent of retinal involvement
  • Optical coherence tomography (OCT): this test allows ophthalmologists to look at outer and inner layers of the retina
  • Fundus autofluorescence: shows areas of unhealthy cells in the retina
  • Blood work: the labs can show how immuno-compromised an individual is
  • Vitreous Tap: Analysis of the fluid in the eye can show the causative organism, such as VZV.

Other possible diseases to consider when a patient presents with symptoms similar to PORN include: CMV retinitis, ARN, Syphilitic retinitis, Toxoplasma retinochoroiditis, tuberculosis, toxocariasis, fungal or bacterial endophthalmitis Inflammatory: Sarcoidosis, Behcet's, other retinal vasculitides, Neoplastic: intraocular lymphoma, leukemia, metastasis.[6]

When AIDS first became a diagnosis, ophthalmologists began seeing patients with infectious processes such as Progressive outer retinal necrosis (PORN). With the use of HAART (3 drug regimen standard) treatment for HIV/AIDS in the 1990s, the presentation of these chorioretinitis diseases became less severe and less frequent.[7]

Treatment for Necrotizing Herpetic Retinopathy, including ARN and PORN, includes the anti-viral medications Ganciclovir and Foscarnet. These medications are given as intra-vitreal injections (injected into the fluid of the eye). This treatment has shown success in reversing the disease process and improving vision to an extent.[6]

If left untreated, PORN syndrome can become bilateral, affecting both eyes. The retina can also atrophy, or decay, over time as the blood supply decreases. This leads to retinal detachment, a process where the retina breaks off the back of the eye. Retinal detachments are considered surgical emergencies, as they can lead to blindness if not promptly fixed.[6]

  1. ↑ Moorthy RS, Weinberg DV, Teich SA, Berger BB, Minturn JT, Kumar S, Rao NA, Fowell SM, Loose IA, Jampol LM (March 1997). "Management of varicella zoster virus retinitis in AIDS". Br J Ophthalmol. 81 (3): 189–94. doi:10.1136/bjo.81.3.189. PMC 1722141. PMID 9135381.
  2. ↑ Austin, RB (December 2000). "Progressive outer retinal necrosis syndrome: a comprehensive review of its clinical presentation, relationship to immune system status, and management". Clinical Eye and Vision Care. 12 (3–4): 119–129. doi:10.1016/s0953-4431(00)00052-7. PMID 11137426.
  3. ↑ Mehrotra, Navneet; Khandelwal, Jayesh; Nagpal, Manish (2020). "Surgical management of complications in a case of progressive outer retinal necrosis". Taiwan Journal of Ophthalmology. 10 (4): 312‑314. doi:10.4103/tjo.tjo_27_19. PMC 7787098. PMID 33437609.
  4. 1 2 Austin, Robert B (2000-12-01). "Progressive outer retinal necrosis syndrome: a comprehensive review of its clinical presentation, relationship to immune system status, and management". Clinical Eye and Vision Care. 12 (3): 119–129. doi:10.1016/S0953-4431(00)00052-7. ISSN 0953-4431. PMID 11137426.
  5. 1 2 Ittner, EA; Bhakhri, R; Newman, T (Jan 2016). "Necrotising herpetic retinopathies: a review and progressive outer retinal necrosis case report". Clin Exp Optom. 99 (1): 24–9. doi:10.1111/cxo.12284. PMID 26084658.
  6. 1 2 3 4 "Progressive Outer Retinal Necrosis - EyeWiki". eyewiki.aao.org. Retrieved 2020-12-13.
  7. ↑ Pérez-Blázquez, E.; Redondo, M.I.; Gracia, T. (2008). "Sida y oftalmología: una visión actual". Anales del Sistema Sanitario de Navarra. 31. doi:10.4321/s1137-66272008000600007. ISSN 1137-6627.