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Peripapillary chorioretinal atrophy

MedGen UID:
870322
•Concept ID:
C4024765
•
Disease or Syndrome
HPO: HP:0007950

Definition

Chorioretinal atrophy concentrated around the optic papilla (i.e., the optic nerve head). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPeripapillary chorioretinal atrophy

Conditions with this feature

Retinitis pigmentosa 27
MedGen UID:
320323
•Concept ID:
C1834329
•
Disease or Syndrome
Any retinitis pigmentosa in which the cause of the disease is a mutation in the NRL gene.
See: Condition Record
Helicoid peripapillary chorioretinal degeneration
MedGen UID:
354733
•Concept ID:
C1862382
•
Disease or Syndrome
Sveinsson chorioretinal atrophy (SCRA) is characterized by bilateral, well-defined, tongue-shaped strips of atrophic retina and choroid that extend from the optic nerve into the peripheral ocular fundus. The lesions may be evident at birth and usually progress at a variable rate, sometimes leading to central visual loss. Separate small distinct circular atrophic lesions are observed in the peripheral ocular fundus in some patients. Congenital anterior polar cataracts are found in approximately 25% of affected individuals (summary by Jonasson et al., 2007).
See: Condition Record
Helicoid peripapillary chorioretinal degeneration
Retinitis pigmentosa 27

Professional guidelines

PubMed

Diagnosis and Treatment of Myopic Maculopathy.
Yokoi T, Ohno-Matsui K
Asia Pac J Ophthalmol (Phila) 2018 Nov-Dec;7(6):415-421. Epub 2018 Sep 26 doi: 10.22608/APO.2018290. PMID: 30255668

Recent clinical studies

Etiology

IDIOPATHIC MULTIFOCAL CHOROIDITIS WITH SERPIGINOUS-LIKE PERIPAPILLARY CHORIORETINAL ATROPHY.
Moussa K, Alsberge JB, Munk MR, Vora RA, Emami-Naeini P, Yiu G, Cunningham ET Jr
Retina 2022 Aug 1;42(8):1574-1582. doi: 10.1097/IAE.0000000000003481. PMID: 35333832
Long-term follow-up of a patient with JAG1-associated retinopathy.
Cheema MR, Stone LG, Sellar PW, Quinn S, Clark SC, Martin RJ, O'Brien JM, Warriner C, Browning AC
Doc Ophthalmol 2021 Oct;143(2):237-247. Epub 2021 Apr 20 doi: 10.1007/s10633-021-09836-w. PMID: 33877487
Peripapillary Atrophy in High Myopia.
Liu W, Gong L, Li Y, Zhu X, Stewart JM, Wang C
Curr Eye Res 2017 Sep;42(9):1308-1312. Epub 2017 May 30 doi: 10.1080/02713683.2017.1307992. PMID: 28557535
Optic disc, rim and peripapillary chorioretinal atrophy in normal Japanese eyes: the Kumejima Study.
Iwase A, Sawaguchi S, Sakai H, Tanaka K, Tsutsumi T, Araie M
Jpn J Ophthalmol 2017 May;61(3):223-229. Epub 2017 Feb 9 doi: 10.1007/s10384-017-0499-8. PMID: 28185066
Clinical implications of peripapillary atrophy in glaucoma.
Jonas JB
Curr Opin Ophthalmol 2005 Apr;16(2):84-8. doi: 10.1097/01.icu.0000156135.20570.30. PMID: 15744137

Diagnosis

IDIOPATHIC MULTIFOCAL CHOROIDITIS WITH SERPIGINOUS-LIKE PERIPAPILLARY CHORIORETINAL ATROPHY.
Moussa K, Alsberge JB, Munk MR, Vora RA, Emami-Naeini P, Yiu G, Cunningham ET Jr
Retina 2022 Aug 1;42(8):1574-1582. doi: 10.1097/IAE.0000000000003481. PMID: 35333832
Long-term follow-up of a patient with JAG1-associated retinopathy.
Cheema MR, Stone LG, Sellar PW, Quinn S, Clark SC, Martin RJ, O'Brien JM, Warriner C, Browning AC
Doc Ophthalmol 2021 Oct;143(2):237-247. Epub 2021 Apr 20 doi: 10.1007/s10633-021-09836-w. PMID: 33877487
Peripapillary Atrophy in High Myopia.
Liu W, Gong L, Li Y, Zhu X, Stewart JM, Wang C
Curr Eye Res 2017 Sep;42(9):1308-1312. Epub 2017 May 30 doi: 10.1080/02713683.2017.1307992. PMID: 28557535
Optic disc, rim and peripapillary chorioretinal atrophy in normal Japanese eyes: the Kumejima Study.
Iwase A, Sawaguchi S, Sakai H, Tanaka K, Tsutsumi T, Araie M
Jpn J Ophthalmol 2017 May;61(3):223-229. Epub 2017 Feb 9 doi: 10.1007/s10384-017-0499-8. PMID: 28185066
Clinical implications of peripapillary atrophy in glaucoma.
Jonas JB
Curr Opin Ophthalmol 2005 Apr;16(2):84-8. doi: 10.1097/01.icu.0000156135.20570.30. PMID: 15744137

Therapy

Prevalence of angioid streaks and other ocular complications of Paget's disease of bone.
Dabbs TR, Skjodt K
Br J Ophthalmol 1990 Oct;74(10):579-82. doi: 10.1136/bjo.74.10.579. PMID: 2285679Free PMC Article

Prognosis

Long-term follow-up of a patient with JAG1-associated retinopathy.
Cheema MR, Stone LG, Sellar PW, Quinn S, Clark SC, Martin RJ, O'Brien JM, Warriner C, Browning AC
Doc Ophthalmol 2021 Oct;143(2):237-247. Epub 2021 Apr 20 doi: 10.1007/s10633-021-09836-w. PMID: 33877487
Progression in Open-Angle Glaucoma with Myopic Disc and Blood Flow in the Optic Nerve Head and Peripapillary Chorioretinal Atrophy Zone.
Kiyota N, Shiga Y, Takahashi N, Yasuda M, Omodaka K, Tsuda S, Kunikata H, Nakazawa T
Ophthalmol Glaucoma 2020 May-Jun;3(3):202-209. Epub 2020 Mar 23 doi: 10.1016/j.ogla.2020.03.005. PMID: 32672617
Factors associated with deep circulation in the peripapillary chorioretinal atrophy zone in normal-tension glaucoma with myopic disc.
Kiyota N, Kunikata H, Takahashi S, Shiga Y, Omodaka K, Nakazawa T
Acta Ophthalmol 2018 May;96(3):e290-e297. Epub 2017 Nov 24 doi: 10.1111/aos.13621. PMID: 29171726
Optic disc, rim and peripapillary chorioretinal atrophy in normal Japanese eyes: the Kumejima Study.
Iwase A, Sawaguchi S, Sakai H, Tanaka K, Tsutsumi T, Araie M
Jpn J Ophthalmol 2017 May;61(3):223-229. Epub 2017 Feb 9 doi: 10.1007/s10384-017-0499-8. PMID: 28185066
Clinical and Genetic Features of Choroideremia in Childhood.
Khan KN, Islam F, Moore AT, Michaelides M
Ophthalmology 2016 Oct;123(10):2158-65. Epub 2016 Aug 6 doi: 10.1016/j.ophtha.2016.06.051. PMID: 27506488

Clinical prediction guides

IDIOPATHIC MULTIFOCAL CHOROIDITIS WITH SERPIGINOUS-LIKE PERIPAPILLARY CHORIORETINAL ATROPHY.
Moussa K, Alsberge JB, Munk MR, Vora RA, Emami-Naeini P, Yiu G, Cunningham ET Jr
Retina 2022 Aug 1;42(8):1574-1582. doi: 10.1097/IAE.0000000000003481. PMID: 35333832
Long-term follow-up of a patient with JAG1-associated retinopathy.
Cheema MR, Stone LG, Sellar PW, Quinn S, Clark SC, Martin RJ, O'Brien JM, Warriner C, Browning AC
Doc Ophthalmol 2021 Oct;143(2):237-247. Epub 2021 Apr 20 doi: 10.1007/s10633-021-09836-w. PMID: 33877487
Progression in Open-Angle Glaucoma with Myopic Disc and Blood Flow in the Optic Nerve Head and Peripapillary Chorioretinal Atrophy Zone.
Kiyota N, Shiga Y, Takahashi N, Yasuda M, Omodaka K, Tsuda S, Kunikata H, Nakazawa T
Ophthalmol Glaucoma 2020 May-Jun;3(3):202-209. Epub 2020 Mar 23 doi: 10.1016/j.ogla.2020.03.005. PMID: 32672617
Fundus autofluorescence in patients with pseudoxanthoma elasticum.
Shiraki K, Kohno T, Moriwaki M, Yanagihara N
Int Ophthalmol 2001;24(5):243-8. doi: 10.1023/a:1025433431654. PMID: 14531624

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