Multimodal analysis of the Preferred Retinal Location and the Transition Zone in patients with Stargardt Disease

Purpose The purpose of our study was to investigate morpho-functional features of the preferred retinal location (PRL) and the transition zone (TZ) in a series of patients with recessive Stargardt disease (STGD1). Methods Fifty-two STGD1 patients with at least one ABCA4 mutation, atrophy of the cent...

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Published in:Graefe's archive for clinical and experimental ophthalmology 2017-07, Vol.255 (7), p.1307-1317
Main Authors: Verdina, Tommaso, Greenstein, Vivienne C., Sodi, Andrea, Tsang, Stephen H., Burke, Tomas R., Passerini, Ilaria, Allikmets, Rando, Virgili, Gianni, Cavallini, Gian Maria, Rizzo, Stanislao
Format: Article
Language:English
Subjects:
Adult
Aged
Atrophy
Female
Fluorescein Angiography - methods
Fundus Oculi
Humans
Macula Lutea - pathology
Macular Degeneration - congenital
Macular Degeneration - diagnosis
Macular Degeneration - physiopathology
Male
Medicine
Medicine & Public Health
Middle Aged
Multimodal Imaging - methods
Ophthalmology
Ophthalmoscopy - methods
Reproducibility of Results
Retina
Retinal degeneration
Retinal Disorders
Retinal Pigment Epithelium - pathology
Retrospective Studies
Structure-function relationships
Tomography, Optical Coherence - methods
Visual Acuity
Visual Fields
Young Adult
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Summary:Purpose The purpose of our study was to investigate morpho-functional features of the preferred retinal location (PRL) and the transition zone (TZ) in a series of patients with recessive Stargardt disease (STGD1). Methods Fifty-two STGD1 patients with at least one ABCA4 mutation, atrophy of the central macula (MA) and an eccentric PRL were recruited for the study. Microperimetry, fundus autofluorescence (FAF), spectral-domain optical coherence tomography (SD-OCT ) were performed. The location and stability of the PRL along with the associated FAF pattern and visual sensitivities were determined and compared to the underlying retinal structure. Results The mean visual sensitivity of the PRLs for the 52 eyes was 10.76 +/- 3.70 dB. For the majority of eyes, PRLs were associated with intact ellipsoid zone (EZ) bands and qualitatively normal FAF patterns. In 17 eyes (32.7%) the eccentric PRL was located at the edge of the MA. In 35 eyes (67.3%) it was located at varying distances from the border of the MA with a TZ between the PRL and the MA. The TZ was associated with decreased sensitivity values (5.92 +/- 4.69 dB) compared to PRLs ( p
ISSN:0721-832X
1435-702X
DOI:10.1007/s00417-017-3637-6