Sveinsson chorioretinal atrophy/helicoid peripapillary chorioretinal degeneration: first histopathology report

2.50
Hdl Handle:
http://hdl.handle.net/2336/13536
Title:
Sveinsson chorioretinal atrophy/helicoid peripapillary chorioretinal degeneration: first histopathology report
Authors:
Jonasson, Fridbert; Hardarson, Sverrir; Olafsson, Bjorn Mar; Klintworth, Gordon K
Citation:
Ophthalmology 2007, 114(8):1541-6
Issue Date:
1-Aug-2007
Abstract:
PURPOSE: To report the histopathologic features in an eye with Sveinsson chorioretinal atrophy (SCRA), also termed helicoid peripapillary chorioretinal degeneration, for the first time. PARTICIPANT: An 82-year-old woman clinically and genetically confirmed to have SCRA. DESIGN: Examination of an eye obtained after death. METHOD: Light microscopic examination of an eye of an 82-year-old woman documented to have SCRA since the age of 10 years. MAIN OUTCOME MEASURE: The findings in ocular tissues were determined by light microscopy. RESULTS: In the most advanced areas of chorioretinal atrophy, the sensory retina, retinal pigment epithelium (RPE), choriocapillaris, and choroid were absent. In the transition between affected and unaffected areas, the RPE and the outer segments of the photoreceptors only were affected. The optic nerve was smaller than normal, but well myelinated. Other ocular tissues retained a relatively normal appearance for a patient who had died at this age. CONCLUSIONS: The mildest and presumably earliest morphologic changes involved the photoreceptor outer segments, the RPE, and choriocapillaris in this progressive degenerative disease of the retina and choroid.
Description:
To access publisher full text version of this article. Please click on the hyperlink in Additional Links field
Additional Links:
http://www.sciencedirect.com/science/article/B6VT2-4N6FYVV-4/2/d26c690cc1cc869188e1c045faf2fab8

Full metadata record

DC FieldValue Language
dc.contributor.authorJonasson, Fridbert-
dc.contributor.authorHardarson, Sverrir-
dc.contributor.authorOlafsson, Bjorn Mar-
dc.contributor.authorKlintworth, Gordon K-
dc.date.accessioned2007-09-11T08:29:55Z-
dc.date.available2007-09-11T08:29:55Z-
dc.date.issued2007-08-01-
dc.date.submitted2007-09-11-
dc.identifier.citationOphthalmology 2007, 114(8):1541-6en
dc.identifier.issn1549-4713-
dc.identifier.pmid17339054-
dc.identifier.doi10.1016/j.ophtha.2006.11.016-
dc.identifier.otherOPH12-
dc.identifier.urihttp://hdl.handle.net/2336/13536-
dc.descriptionTo access publisher full text version of this article. Please click on the hyperlink in Additional Links fielden
dc.description.abstractPURPOSE: To report the histopathologic features in an eye with Sveinsson chorioretinal atrophy (SCRA), also termed helicoid peripapillary chorioretinal degeneration, for the first time. PARTICIPANT: An 82-year-old woman clinically and genetically confirmed to have SCRA. DESIGN: Examination of an eye obtained after death. METHOD: Light microscopic examination of an eye of an 82-year-old woman documented to have SCRA since the age of 10 years. MAIN OUTCOME MEASURE: The findings in ocular tissues were determined by light microscopy. RESULTS: In the most advanced areas of chorioretinal atrophy, the sensory retina, retinal pigment epithelium (RPE), choriocapillaris, and choroid were absent. In the transition between affected and unaffected areas, the RPE and the outer segments of the photoreceptors only were affected. The optic nerve was smaller than normal, but well myelinated. Other ocular tissues retained a relatively normal appearance for a patient who had died at this age. CONCLUSIONS: The mildest and presumably earliest morphologic changes involved the photoreceptor outer segments, the RPE, and choriocapillaris in this progressive degenerative disease of the retina and choroid.en
dc.language.isoenen
dc.publisherElsevieren
dc.relation.urlhttp://www.sciencedirect.com/science/article/B6VT2-4N6FYVV-4/2/d26c690cc1cc869188e1c045faf2fab8en
dc.subject.meshAged, 80 and overen
dc.subject.meshAtrophyen
dc.subject.meshChoroid Diseasesen
dc.subject.meshOptic Disken
dc.subject.meshOptic Nerveen
dc.subject.meshPhotoreceptors, Vertebrateen
dc.subject.meshPigment Epithelium of Eyeen
dc.subject.meshRetinal Degenerationen
dc.titleSveinsson chorioretinal atrophy/helicoid peripapillary chorioretinal degeneration: first histopathology reporten
dc.typeArticleen
dc.identifier.journalOphthalmologyen
dc.format.digYES-

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