Morphology and function of the retina in children and young adults with Stargardt dystrophy
Background: The aim of our study was to evaluate retinal function in children and young adults with Stargardt dystrophy by correlating retinal morphology (autofluorescence, OCT) with functional tests (visual acuity, microperimetry) and electroretinography (full-field ERG, pattern ERG, multifoc...
Main Authors: | , , , , , |
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Format: | Article |
Language: | English |
Published: |
Slovenian Medical Association
2012-06-01
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Series: | Zdravniški Vestnik |
Online Access: | http://vestnik.szd.si/index.php/ZdravVest/article/view/930 |
Summary: | Background: The aim of our study was to evaluate
retinal function in children and young adults
with Stargardt dystrophy by correlating retinal
morphology (autofluorescence, OCT) with
functional tests (visual acuity, microperimetry)
and electroretinography (full-field ERG, pattern
ERG, multifocal ERG).
Patients and methods: 6 patients (3 F, 3 M, VA:
0.2 ± 0.1, average onset of problems at 10 yrs of
age) were included in the study. Autofluorescence
(AF) was recorded and the central 10 º
visual fields were tested by microperimetry. The
morphology of the retina was recorded by optical
coherence tomography (OCT). Full-field
ERG, pattern ERG (PERG) and multifocal ERG
(mfERG) were recorded according to ISCEV
standard in all the patients.
Results: AF showed mottled central hypo-hyperautofluorescent
areas with or without peripheral
hyperautofuorescent flecks in 4 patients and
a central hypoautofluorescent region with hyperautofluorescent
ring in 2 patients. OCT showed
transverse loss of the central photoreceptor layers
(average OCT thickness: 38.9 ± 14.8μm, average
OCT volume 6.1 ± 0.5 mm3). Microperimetry
showed superior shift of fixation to the preferred
retinal locus (PRL). PERG was abnormal in 8
eyes. Full-field ERG was normal in all except two
patients with abnormal cone and 30 Hz responses.
MfERG showed reduced responses in all five
rings (ring 1: 22.6 % of mean normal value, ring 2:
27.3 %, ring 3: 38.9 %, ring 4: 57.9 %, ring 5: 64.2 %).
Conclusions: In young patients with Stargardt
dystrophy central retinal atrophy was shown by
OCT and AF. The shift of fixation to the PRL was
seen in all the patients. MfERG showed central
cone dysfunction in all the patients. |
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ISSN: | 1318-0347 1581-0224 |