Universität zu Köln

Gliem, Martin, Mueller, Philipp L., Mangold, Elisabeth, Bolz, Hanno J., Stoehr, Heidi, Weber, Bernhard H. F., Holz, Frank G. and Issa, Peter Charbel ORCID: 0000-0002-0351-6673 (2015). Reticular Pseudodrusen in Sorsby Fundus Dystrophy. Ophthalmology, 122 (8). S. 1555 - 1563. NEW YORK: ELSEVIER SCIENCE INC. ISSN 1549-4713

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Abstract

Purpose: To investigate the association of reticular pseudodrusen (RPD) with Sorsby fundus dystrophy (SFD). Design: Prospective, monocenter, cross-sectional case series. Subjects: Sixteen patients of 4 unrelated families with SFD caused by mutations in TIMP3. Methods: All subjects underwent multimodal imaging including near-infrared (NIR) reflectance and fundus autofluorescence with a confocal scanning laser ophthalmoscope and spectral-domain optical coherence tomography (SD OCT). Main Outcome Measures: Prevalence, topographic distribution, and phenotype of RPD. Results: Mean age of the investigated patients was 56.8 years (range, 23-78 years). Reticular pseudodrusen were identified frequently in SFD patients in the sixth decade of life (5 of 7 [71%]) and were absent in younger (n = 3) or older (n = 6) patients. They were most abundant in the superior quadrant and spared the foveal region. Reticular pseudodrusen appeared as yellowish round to oval (dot subtype; n = 5) or confluent, wriggled (ribbon subtype; n = 3) lesions, sometimes forming irregular networks. Reticular pseudodrusen were hyporeflective on NIR reflectance and hypofluorescent on fundus autofluorescence imaging. They appeared as subretinal deposits on SD OCT imaging. Other lesions, such as peripheral pseudodrusen and soft drusen, were present less frequently. Conclusions: Reticular pseudodrusen are a frequent finding in patients with SFD. Although SFD patients with RPD are younger, distribution and phenotype of RPD are similar to those observed in patients with age-related macular degeneration. The association of RPD with SFD implicates a role of Bruch's membrane, the Bruch's membraneeretinal pigment epithelium interface, or both in the pathogenesis of RPD. (C) 2015 by the American Academy of Ophthalmology.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Gliem, Martin UNSPECIFIED UNSPECIFIED UNSPECIFIED Mueller, Philipp L. UNSPECIFIED UNSPECIFIED UNSPECIFIED Mangold, Elisabeth UNSPECIFIED UNSPECIFIED UNSPECIFIED Bolz, Hanno J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Stoehr, Heidi UNSPECIFIED UNSPECIFIED UNSPECIFIED Weber, Bernhard H. F. UNSPECIFIED UNSPECIFIED UNSPECIFIED Holz, Frank G. UNSPECIFIED UNSPECIFIED UNSPECIFIED Issa, Peter Charbel UNSPECIFIED orcid.org/0000-0002-0351-6673 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-398237
DOI:	10.1016/j.ophtha.2015.04.035
Journal or Publication Title:	Ophthalmology
Volume:	122
Number:	8
Page Range:	S. 1555 - 1563
Date:	2015
Publisher:	ELSEVIER SCIENCE INC
Place of Publication:	NEW YORK
ISSN:	1549-4713
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language SUBRETINAL DRUSENOID DEPOSITS; AGE-RELATED MACULOPATHY; MACULAR DEGENERATION; BRUCHS MEMBRANE; GEOGRAPHIC-ATROPHY; PSEUDOXANTHOMA ELASTICUM; MORPHOMETRIC-ANALYSIS; CHOROIDAL THICKNESS; TISSUE INHIBITOR; TIMP3 GENE Multiple languages Ophthalmology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/39823