Dataset Information

Chorioretinal architecture in Aicardi syndrome: an optical coherence tomography and fluorescein angiography study.

ABSTRACT: Aicardi syndrome is a rare congenital disorder with a classic triad of infantile spasms, chorioretinal lacunae, and agenesis of the corpus callosum. We report the use of fluorescein angiography and spectral domain optical coherence tomography to examine the posterior segment structures in an 8-month-old girl with Aicardi syndrome. Most of the observed features correlated with previously published histopathological findings, but inner nuclear layer cysts have not been previously described. To our knowledge, this is the first study of the tomographic and angiographic chorioretinal features in vivo.

SUBMITTER: Martel JN

PROVIDER: S-EPMC3142350 | biostudies-literature | 2011 Jun

REPOSITORIES: biostudies-literature

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Chorioretinal architecture in Aicardi syndrome: an optical coherence tomography and fluorescein angiography study.

Martel Joseph N JN Rutar Tina T Lujan Brandon J BJ de Alba Campomanes Alejandra A

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus 20110601 3

Aicardi syndrome is a rare congenital disorder with a classic triad of infantile spasms, chorioretinal lacunae, and agenesis of the corpus callosum. We report the use of fluorescein angiography and spectral domain optical coherence tomography to examine the posterior segment structures in an 8-month-old girl with Aicardi syndrome. Most of the observed features correlated with previously published histopathological findings, but inner nuclear layer cysts have not been previously described. To our ...[more]

PMID: 21777802

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Project description:ObjectiveTo evaluate agreement between fluorescein angiography (FA) and optical coherence tomography (OCT) results for diagnosis of macular edema in patients with uveitis.DesignMulticenter cross-sectional study.ParticipantsFour hundred seventy-nine eyes with uveitis from 255 patients.MethodsThe macular status of dilated eyes with intermediate uveitis, posterior uveitis, or panuveitis was assessed via Stratus-3 OCT and FA. To evaluate agreement between the diagnostic approaches, ? statistics were used.Main outcome measuresMacular thickening (MT; center point thickness, ? 240 ?m per reading center grading of OCT images) and macular leakage (ML; central subfield fluorescein leakage, ? 0.44 disc areas per reading center grading of FA images), and agreement between these outcomes in diagnosing macular edema.ResultsOptical coherence tomography (90.4%) more frequently returned usable information regarding macular edema than FA (77%) or biomicroscopy (76%). Agreement in diagnosis of MT and ML (? = 0.44) was moderate. Macular leakage was present in 40% of cases free of MT, whereas MT was present in 34% of cases without ML. Biomicroscopic evaluation for macular edema failed to detect 40% and 45% of cases of MT and ML, respectively, and diagnosed 17% and 17% of cases with macular edema that did not have MT or ML, respectively; these results may underestimate biomicroscopic errors (ophthalmologists were not explicitly masked to OCT and FA results). Among eyes free of ML, phakic eyes without cataract rarely (4%) had MT. No factors were found that effectively ruled out ML when MT was absent.ConclusionsOptical coherence tomography and FA offered only moderate agreement regarding macular edema status in uveitis cases, probably because what they measure (MT and ML) are related but nonidentical macular pathologic characteristics. Given its lower cost, greater safety, and greater likelihood of obtaining usable information, OCT may be the best initial test for evaluation of suspected macular edema. However, given that ML cannot be ruled out if MT is absent and vice versa, obtaining the second test after negative results on the first seems justified when detection of ML or MT would alter management. Given that biomicroscopic evaluation for macular edema erred frequently, ancillary testing for macular edema seems indicated when knowledge of ML or MT status would affect management.Financial disclosure(s)Proprietary or commercial disclosure may be found after the references.

Dataset Information

Chorioretinal architecture in Aicardi syndrome: an optical coherence tomography and fluorescein angiography study.

Publications

Chorioretinal architecture in Aicardi syndrome: an optical coherence tomography and fluorescein angiography study.

Similar Datasets

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