U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Choroidal dystrophy, central areolar, 3(CACD3)

MedGen UID:
442631
•Concept ID:
C2751055
•
Disease or Syndrome
Synonyms: CACD3; CHOROIDAL DYSTROPHY, CENTRAL AREOLAR, WITH OR WITHOUT DRUSEN
 
Monarch Initiative: MONDO:0013151
OMIM®: 613144

Clinical features

From HPO
Drusen
MedGen UID:
488956
•Concept ID:
C1260959
•
Finding
Drusen (singular, 'druse') are tiny yellow or white accumulations of extracellular material (lipofuscin) that build up in Bruch's membrane of the eye.
See: Feature record | Search on this feature
Chorioretinal atrophy
MedGen UID:
884881
•Concept ID:
C4048273
•
Disease or Syndrome
Atrophy of the choroid and retinal layers of the fundus.
See: Feature record | Search on this feature

Recent clinical studies

Etiology

Longitudinal structure-function analysis of molecularly-confirmed CYP4V2 Bietti Crystalline Dystrophy.
Cheloni R, Clough N, Jackson D, Moosajee M
Eye (Lond) 2024 Apr;38(5):853-862. Epub 2023 Oct 28 doi: 10.1038/s41433-023-02791-7. PMID: 37898718Free PMC Article
Classification and Growth Rate of Chorioretinal Atrophy after Voretigene Neparvovec-Rzyl for RPE65-Mediated Retinal Degeneration.
Bommakanti N, Young BK, Sisk RA, Berrocal AM, Duncan JL, Bakall B, Mathias MT, Ahmed I, Chorfi S, Comander J, Nagiel A, Besirli CG
Ophthalmol Retina 2024 Jan;8(1):42-48. Epub 2023 Sep 3 doi: 10.1016/j.oret.2023.08.017. PMID: 37660736Free PMC Article
Pathogenesis of myopic choroidal neovascularization: A systematic review and meta-analysis.
Zhang XJ, Chen XN, Tang FY, Szeto S, Ling XT, Lin ZX, Tham CC, Pang CP, Chen LJ, Yam JC
Surv Ophthalmol 2023 Nov-Dec;68(6):1011-1026. Epub 2023 Jul 28 doi: 10.1016/j.survophthal.2023.07.006. PMID: 37517683
Solar retinopathy in India: Clinical presentation and demographic distribution in 253 patients (349 eyes).
Das AV, Tyagi M, Kadam Y, Belenje A
Indian J Ophthalmol 2023 May;71(5):2061-2065. doi: 10.4103/IJO.IJO_2620_22. PMID: 37203081Free PMC Article
Retinitis pigmentosa inversa.
Ferrucci S, Anderson SF, Townsend JC
Optom Vis Sci 1998 Aug;75(8):560-70. doi: 10.1097/00006324-199808000-00021. PMID: 9734800

Diagnosis

Classification and Growth Rate of Chorioretinal Atrophy after Voretigene Neparvovec-Rzyl for RPE65-Mediated Retinal Degeneration.
Bommakanti N, Young BK, Sisk RA, Berrocal AM, Duncan JL, Bakall B, Mathias MT, Ahmed I, Chorfi S, Comander J, Nagiel A, Besirli CG
Ophthalmol Retina 2024 Jan;8(1):42-48. Epub 2023 Sep 3 doi: 10.1016/j.oret.2023.08.017. PMID: 37660736Free PMC Article
Solar retinopathy in India: Clinical presentation and demographic distribution in 253 patients (349 eyes).
Das AV, Tyagi M, Kadam Y, Belenje A
Indian J Ophthalmol 2023 May;71(5):2061-2065. doi: 10.4103/IJO.IJO_2620_22. PMID: 37203081Free PMC Article
Outer Retinal Tubulation: A Case Series.
Rousso LA, Rodman JA, Sutton B, Shechtman DL
Optom Vis Sci 2017 Mar;94(3):423-431. doi: 10.1097/OPX.0000000000001056. PMID: 28225373
Retinitis pigmentosa inversa.
Ferrucci S, Anderson SF, Townsend JC
Optom Vis Sci 1998 Aug;75(8):560-70. doi: 10.1097/00006324-199808000-00021. PMID: 9734800
Sex-linked chorioretinal heredodegenerations.
François J
Birth Defects Orig Artic Ser 1971 Mar;7(3):99-116. PMID: 4950918

Therapy

Longitudinal structure-function analysis of molecularly-confirmed CYP4V2 Bietti Crystalline Dystrophy.
Cheloni R, Clough N, Jackson D, Moosajee M
Eye (Lond) 2024 Apr;38(5):853-862. Epub 2023 Oct 28 doi: 10.1038/s41433-023-02791-7. PMID: 37898718Free PMC Article
Classification and Growth Rate of Chorioretinal Atrophy after Voretigene Neparvovec-Rzyl for RPE65-Mediated Retinal Degeneration.
Bommakanti N, Young BK, Sisk RA, Berrocal AM, Duncan JL, Bakall B, Mathias MT, Ahmed I, Chorfi S, Comander J, Nagiel A, Besirli CG
Ophthalmol Retina 2024 Jan;8(1):42-48. Epub 2023 Sep 3 doi: 10.1016/j.oret.2023.08.017. PMID: 37660736Free PMC Article
Pathogenesis of myopic choroidal neovascularization: A systematic review and meta-analysis.
Zhang XJ, Chen XN, Tang FY, Szeto S, Ling XT, Lin ZX, Tham CC, Pang CP, Chen LJ, Yam JC
Surv Ophthalmol 2023 Nov-Dec;68(6):1011-1026. Epub 2023 Jul 28 doi: 10.1016/j.survophthal.2023.07.006. PMID: 37517683
CHOROIDAL NEOVASCULARIZATION AND CHORIORETINAL ATROPHY IN A PATIENT WITH MELANOMA-ASSOCIATED RETINOPATHY AFTER IPILIMUMAB/NIVOLUMAB COMBINATION THERAPY.
Elwood KF, Pulido JS, Ghafoori SD, Harper CA, Wong RW
Retin Cases Brief Rep 2021 Sep 1;15(5):514-518. doi: 10.1097/ICB.0000000000000882. PMID: 31246886
Retinitis pigmentosa inversa.
Ferrucci S, Anderson SF, Townsend JC
Optom Vis Sci 1998 Aug;75(8):560-70. doi: 10.1097/00006324-199808000-00021. PMID: 9734800

Prognosis

TEN-YEAR VISUAL OUTCOME AND CHANGE IN CHORIORETINAL ATROPHY AFTER INTRAVITREAL RANIBIZUMAB FOR MACULAR NEOVASCULARIZATION IN PATHOLOGIC MYOPIA.
Sakata R, Miyata M, Ooto S, Tamura H, Ueda-Arakawa N, Muraoka Y, Miyake M, Hata M, Takahashi A, Kido A, Numa S, Mori Y, Tsuda K, Uji A, Oishi A, Tsujikawa A
Retina 2023 Nov 1;43(11):1863-1871. doi: 10.1097/IAE.0000000000003869. PMID: 37339449
Four-year follow up of macular intrachoroidal cavitation and chorioretinal atrophy: A case report.
Abe S, Iwase T
Medicine (Baltimore) 2021 Dec 17;100(50):e28269. doi: 10.1097/MD.0000000000028269. PMID: 34918700Free PMC Article
CHARACTERISTICS OF PERIPAPILLARY INTRACHOROIDAL CAVITATION IN HIGHLY MYOPIC EYES: The Zhongshan Ophthalmic Center-Brien Holden Vision Institute High Myopia Cohort Study.
Liu R, Li Z, Xiao O, Zhang J, Guo X, Loong Lee JT, Wang D, Lee P, Jong M, Sankaridurg P, He M
Retina 2021 May 1;41(5):1057-1062. doi: 10.1097/IAE.0000000000002963. PMID: 32833786
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
Retinitis pigmentosa inversa.
Ferrucci S, Anderson SF, Townsend JC
Optom Vis Sci 1998 Aug;75(8):560-70. doi: 10.1097/00006324-199808000-00021. PMID: 9734800

Clinical prediction guides

TEN-YEAR VISUAL OUTCOME AND CHANGE IN CHORIORETINAL ATROPHY AFTER INTRAVITREAL RANIBIZUMAB FOR MACULAR NEOVASCULARIZATION IN PATHOLOGIC MYOPIA.
Sakata R, Miyata M, Ooto S, Tamura H, Ueda-Arakawa N, Muraoka Y, Miyake M, Hata M, Takahashi A, Kido A, Numa S, Mori Y, Tsuda K, Uji A, Oishi A, Tsujikawa A
Retina 2023 Nov 1;43(11):1863-1871. doi: 10.1097/IAE.0000000000003869. PMID: 37339449
Four-year follow up of macular intrachoroidal cavitation and chorioretinal atrophy: A case report.
Abe S, Iwase T
Medicine (Baltimore) 2021 Dec 17;100(50):e28269. doi: 10.1097/MD.0000000000028269. PMID: 34918700Free PMC Article
CHARACTERISTICS OF PERIPAPILLARY INTRACHOROIDAL CAVITATION IN HIGHLY MYOPIC EYES: The Zhongshan Ophthalmic Center-Brien Holden Vision Institute High Myopia Cohort Study.
Liu R, Li Z, Xiao O, Zhang J, Guo X, Loong Lee JT, Wang D, Lee P, Jong M, Sankaridurg P, He M
Retina 2021 May 1;41(5):1057-1062. doi: 10.1097/IAE.0000000000002963. PMID: 32833786
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
Spectral OCT analysis in Bietti crystalline dystrophy.
Gaucher D, Saleh M, Sauer A, Bourcier T, Speeg-Schatz C
Eur J Ophthalmol 2010 May-Jun;20(3):612-4. doi: 10.1177/112067211002000315. PMID: 20099228

Recent systematic reviews

Pathogenesis of myopic choroidal neovascularization: A systematic review and meta-analysis.
Zhang XJ, Chen XN, Tang FY, Szeto S, Ling XT, Lin ZX, Tham CC, Pang CP, Chen LJ, Yam JC
Surv Ophthalmol 2023 Nov-Dec;68(6):1011-1026. Epub 2023 Jul 28 doi: 10.1016/j.survophthal.2023.07.006. PMID: 37517683

Supplemental Content

Table of contents

    Clinical resources

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • OMIM
      Related records in OMIM
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...