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Proliferative vitreoretinopathy(VRNI; PVR)

MedGen UID:
66167
Concept ID:
C0242852
Disease or Syndrome
Synonym: Vitreoretinopathy, neovascular inflammatory
SNOMED CT: Proliferative vitreoretinopathy (232016005); PVR - Proliferative vitreoretinopathy (232016005); Fibrosis of retina (232016005); Retinitis proliferans (232016005)
 
Gene (location): CAPN5 (11q13.5)
 
Monarch Initiative: MONDO:0700115
OMIM®: 193235

Definition

Vitreoretinal membrane shrinkage or contraction secondary to the proliferation of primarily retinal pigment epithelial cells and glial cells, particularly fibrous astrocytes, followed by membrane formation. The formation of fibrillar collagen and cellular proliferation appear to be the basis for the contractile properties of the epiretinal and vitreous membranes. [from MONDO]

Clinical features

From HPO
Uveitis
MedGen UID:
52961
Concept ID:
C0042164
Disease or Syndrome
Inflammation of one or all portions of the uveal tract.
Retinal detachment
MedGen UID:
19759
Concept ID:
C0035305
Disease or Syndrome
Primary or spontaneous detachment of the retina occurs due to underlying ocular disease and often involves the vitreous as well as the retina. The precipitating event is formation of a retinal tear or hole, which permits fluid to accumulate under the sensory layers of the retina and creates an intraretinal cleavage that destroys the neurosensory process of visual reception. Vitreoretinal degeneration and tear formation are painless phenomena, and in most cases, significant vitreoretinal pathology is found only after detachment of the retina starts to cause loss of vision or visual field. Without surgical intervention, retinal detachment will almost inevitably lead to total blindness (summary by McNiel and McPherson, 1971).
Vitreous hemorrhage
MedGen UID:
12119
Concept ID:
C0042909
Pathologic Function
Bleeding within the vitreous compartment of the eye.
Vitreoretinopathy
MedGen UID:
87480
Concept ID:
C0344290
Disease or Syndrome
Ocular abnormality characterized by premature degeneration of the vitreous and the retina that may be associated with increased risk of retinal detachment.
Blindness
MedGen UID:
99138
Concept ID:
C0456909
Disease or Syndrome
Blindness is the condition of lacking visual perception defined as a profound reduction in visual perception. On the 6m visual acuity scale, blindness is defined as less than 3/60. On the 20ft visual acuity scale, blindness is defined as less than 20/400. On the decimal visual acuity scale, blindness is defined as less than 0.05. Blindness is typically characterized by a visual field of no greater than 10 degrees in radius around central fixation.
Peripheral retinal neovascularization
MedGen UID:
633873
Concept ID:
C0474355
Disease or Syndrome
A type of retinal neovascularization that affects the periphery of the retina.
Abnormal electroretinogram
MedGen UID:
96908
Concept ID:
C0476397
Finding
Any abnormality of the electrical responses of various cell types in the retina as measured by electroretinography.
Posterior retinal neovascularization
MedGen UID:
870359
Concept ID:
C4024803
Anatomical Abnormality
A type of retinal neovascularization that affects the posterior pole of the retina.
Large hyperpigmented retinal spots
MedGen UID:
870375
Concept ID:
C4024820
Anatomical Abnormality

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVProliferative vitreoretinopathy

Professional guidelines

PubMed

Shaikh N, Srishti R, Khanum A, Thirumalesh MB, Dave V, Arora A, Bansal R, Surve A, Azad S, Kumar V
Indian J Ophthalmol 2023 Jan;71(1):28-38. doi: 10.4103/ijo.IJO_928_22. PMID: 36588205Free PMC Article
Dumbrăveanu L, Cușnir V, Bobescu D
Rom J Ophthalmol 2021 Oct-Dec;65(4):315-329. doi: 10.22336/rjo.2021.66. PMID: 35087972Free PMC Article
Liu Y, Wu N
Int J Nanomedicine 2021;16:1391-1403. Epub 2021 Feb 24 doi: 10.2147/IJN.S294807. PMID: 33658779Free PMC Article

Recent clinical studies

Etiology

Wu F, Eliott D
Semin Ophthalmol 2021 May 19;36(4):218-223. Epub 2021 Feb 22 doi: 10.1080/08820538.2021.1890791. PMID: 33616467
Idrees S, Sridhar J, Kuriyan AE
Int Ophthalmol Clin 2019 Winter;59(1):221-240. doi: 10.1097/IIO.0000000000000258. PMID: 30585928Free PMC Article
Ockrim Z, Yorston D
BMJ 2010 Oct 25;341:c5400. doi: 10.1136/bmj.c5400. PMID: 20974661
Fong DS, Aiello LP, Ferris FL 3rd, Klein R
Diabetes Care 2004 Oct;27(10):2540-53. doi: 10.2337/diacare.27.10.2540. PMID: 15451934
Kruger EF, Nguyen QD, Ramos-Lopez M, Lashkari K
Int Ophthalmol Clin 2002 Summer;42(3):129-43. doi: 10.1097/00004397-200207000-00015. PMID: 12131590

Diagnosis

Liu T, Faia LJ
Ophthalmol Retina 2023 Dec;7(12):1128. Epub 2023 Oct 12 doi: 10.1016/j.oret.2023.09.013. PMID: 37831037
Shaikh N, Srishti R, Khanum A, Thirumalesh MB, Dave V, Arora A, Bansal R, Surve A, Azad S, Kumar V
Indian J Ophthalmol 2023 Jan;71(1):28-38. doi: 10.4103/ijo.IJO_928_22. PMID: 36588205Free PMC Article
Claes C, Lafetá AP
Dev Ophthalmol 2014;54:188-95. Epub 2014 Aug 26 doi: 10.1159/000360466. PMID: 25196769
B S G, Pai GS, Pai AH, Vinekar AS, Pai HS, Noronha T, Fernandes MS
Kathmandu Univ Med J (KUMJ) 2013 Jan-Mar;11(41):91-3. doi: 10.3126/kumj.v11i1.11052. PMID: 23774424
Fong DS, Aiello LP, Ferris FL 3rd, Klein R
Diabetes Care 2004 Oct;27(10):2540-53. doi: 10.2337/diacare.27.10.2540. PMID: 15451934

Therapy

Wang WX, Xing M, Apte RS
JAMA Ophthalmol 2024 Jul 1;142(7):669-670. doi: 10.1001/jamaophthalmol.2024.1093. PMID: 38780937
Shaikh N, Srishti R, Khanum A, Thirumalesh MB, Dave V, Arora A, Bansal R, Surve A, Azad S, Kumar V
Indian J Ophthalmol 2023 Jan;71(1):28-38. doi: 10.4103/ijo.IJO_928_22. PMID: 36588205Free PMC Article
Idrees S, Sridhar J, Kuriyan AE
Int Ophthalmol Clin 2019 Winter;59(1):221-240. doi: 10.1097/IIO.0000000000000258. PMID: 30585928Free PMC Article
Papakostas TD, Vavvas D
Semin Ophthalmol 2018;33(1):70-74. Epub 2017 Nov 29 doi: 10.1080/08820538.2017.1353816. PMID: 29185844
Wirostko WJ, Han DP, Perkins SL
Curr Opin Ophthalmol 2000 Jun;11(3):195-200. doi: 10.1097/00055735-200006000-00008. PMID: 10977227

Prognosis

Fu Y, Xie TH, Gu ZH, Yang N, Geng RF, Zhang YL
Int Ophthalmol 2022 Dec;42(12):3813-3820. Epub 2022 Jul 8 doi: 10.1007/s10792-022-02401-7. PMID: 35802298
Miserocchi E, Iuliano L, Fogliato G, Modorati G, Couto C, Schlaen A, Hurtado E, Llorenç V, Adan A, Bandello F
Ocul Immunol Inflamm 2019;27(7):1090-1098. Epub 2018 Jul 30 doi: 10.1080/09273948.2018.1501494. PMID: 30059636
Toygar O, Mi CW, Miller DM, Riemann CD
Graefes Arch Clin Exp Ophthalmol 2016 Nov;254(11):2111-2118. Epub 2016 Apr 19 doi: 10.1007/s00417-016-3355-5. PMID: 27094701
Fraser S, Steel D
BMJ Clin Evid 2010 Nov 24;2010 PMID: 21406128Free PMC Article
Chaum E
Int Ophthalmol Clin 1995 Winter;35(1):163-73. doi: 10.1097/00004397-199503510-00017. PMID: 7751128

Clinical prediction guides

Shaikh N, Srishti R, Khanum A, Thirumalesh MB, Dave V, Arora A, Bansal R, Surve A, Azad S, Kumar V
Indian J Ophthalmol 2023 Jan;71(1):28-38. doi: 10.4103/ijo.IJO_928_22. PMID: 36588205Free PMC Article
Fu Y, Xie TH, Gu ZH, Yang N, Geng RF, Zhang YL
Int Ophthalmol 2022 Dec;42(12):3813-3820. Epub 2022 Jul 8 doi: 10.1007/s10792-022-02401-7. PMID: 35802298
Huang CY, Mikowski M, Wu L
Graefes Arch Clin Exp Ophthalmol 2022 Mar;260(3):711-722. Epub 2021 Oct 12 doi: 10.1007/s00417-021-05448-x. PMID: 34636994
Fraser S, Steel D
BMJ Clin Evid 2010 Nov 24;2010 PMID: 21406128Free PMC Article
Steel D, Fraser S
BMJ Clin Evid 2008 Feb 15;2008 PMID: 19450333Free PMC Article

Recent systematic reviews

Xiang J, Fan J, Wang J
PLoS One 2023;18(10):e0292698. Epub 2023 Oct 30 doi: 10.1371/journal.pone.0292698. PMID: 37903162Free PMC Article
Tsui JC, Brucker AJ, Kim BJ, Kolomeyer AM
Retina 2023 Aug 1;43(8):1226-1239. doi: 10.1097/IAE.0000000000003770. PMID: 36893435
Steel D
BMJ Clin Evid 2014 Mar 3;2014 PMID: 24807890Free PMC Article
Fraser S, Steel D
BMJ Clin Evid 2010 Nov 24;2010 PMID: 21406128Free PMC Article
Steel D, Fraser S
BMJ Clin Evid 2008 Feb 15;2008 PMID: 19450333Free PMC Article

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