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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
Chronopoulos A, Hattenbach LO, Schutz JS
Surv Ophthalmol 2021 Jul-Aug;66(4):585-593. Epub 2020 Dec 24 doi: 10.1016/j.survophthal.2020.12.007. PMID: 33359545
Idrees S, Sridhar J, Kuriyan AE
Int Ophthalmol Clin 2019 Winter;59(1):221-240. doi: 10.1097/IIO.0000000000000258. PMID: 30585928Free PMC Article
Kwon OW, Song JH, Roh MI
Dev Ophthalmol 2016;55:154-62. Epub 2015 Oct 26 doi: 10.1159/000438972. PMID: 26501375
Ciprian D
Rom J Ophthalmol 2015 Apr-Jun;59(2):88-92. PMID: 26978867Free PMC Article

Diagnosis

Ferro Desideri L, Artemiev D, Zandi S, Zinkernagel MS, Anguita R
Graefes Arch Clin Exp Ophthalmol 2024 Mar;262(3):679-687. Epub 2023 Oct 16 doi: 10.1007/s00417-023-06264-1. PMID: 37843566Free PMC Article
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
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
Claes C, Lafetá AP
Dev Ophthalmol 2014;54:188-95. Epub 2014 Aug 26 doi: 10.1159/000360466. PMID: 25196769
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

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
Chronopoulos A, Hattenbach LO, Schutz JS
Surv Ophthalmol 2021 Jul-Aug;66(4):585-593. Epub 2020 Dec 24 doi: 10.1016/j.survophthal.2020.12.007. PMID: 33359545
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
Gandorfer A
Eye (Lond) 2008 Oct;22(10):1273-7. Epub 2008 Feb 22 doi: 10.1038/eye.2008.29. PMID: 18292784

Prognosis

Jin Y, Chen H, Xu X, Hu Y, Wang C, Ma Z
Retina 2017 Jul;37(7):1236-1245. doi: 10.1097/IAE.0000000000001350. PMID: 27779559
Pastor JC, Rojas J, Pastor-Idoate S, Di Lauro S, Gonzalez-Buendia L, Delgado-Tirado S
Prog Retin Eye Res 2016 Mar;51:125-55. Epub 2015 Jul 21 doi: 10.1016/j.preteyeres.2015.07.005. PMID: 26209346
Fraser S, Steel D
BMJ Clin Evid 2010 Nov 24;2010 PMID: 21406128Free PMC Article
Kroll P, Rodrigues EB, Hoerle S
Ophthalmologica 2007;221(2):78-94. doi: 10.1159/000098253. PMID: 17380062
Pastor JC
Surv Ophthalmol 1998 Jul-Aug;43(1):3-18. doi: 10.1016/s0039-6257(98)00023-x. PMID: 9716190

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
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
Chaudhary R, Scott RAH, Wallace G, Berry M, Logan A, Blanch RJ
Transl Vis Sci Technol 2020 Feb 21;9(3):23. doi: 10.1167/tvst.9.3.23. PMID: 32742753Free 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

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
Russo A, Morescalchi F, Donati S, Gambicorti E, Azzolini C, Costagliola C, Semeraro F
Int Ophthalmol 2018 Apr;38(2):855-867. Epub 2017 Mar 13 doi: 10.1007/s10792-017-0489-3. PMID: 28289950
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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