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Imaging the cornea at Fabry�Â�´s disease using polarisation sensitive OCT

Poster Details

First Author: S.Holzer AUSTRIA

Co Author(s):    N. Pircher   F. Beer   J. Lammer   M. Pircher   C. Hitzenberger   G. Schmidinger     

Abstract Details

Purpose:

Anderson-Fabry disease is a rare x-linked recessive lysosomal storage disease. In the course of this medical condition a lack of alpha-galactosidase affects vessels and causes a wide range of systemic symptoms. Next to conjunctival and retinal vessel abnormalities, ocular involvement may early include corneal deposits and opacities at or near the level of the Bowman�Â�´s membrane. Consisting of lamellar configured glycosphingolipids these accumulations might have a depolarising or birefringent effect which could be detected by polarisation sensitive optic coherence tomography (PS-OCT). Purpose of this study is to assess these properties using a custom built PS-OCT.

Setting:

Medical University of Vienna, Dept. of Ophthalmology and Optometry Medical University of Vienna, Center for Medical Physics and Biomedical Engineering

Methods:

After an extensive ophthalmic examination, including a slit lamp grading of opacities,�Â�  the cornea of patients and healthy controls was imaged using a custom built Swept Source PS-OCT (�Î�»=1045nm, scan time <2sec).150 B-Scans consisting of 1024 A-Scans were acquired to generate a wide view volume scan of the cornea. Additionally, several parameters such as local retardation and Bowman layer thickness were assessed. Until now 2 patients and 2 healthy subjects were included in the study. A minimum number of 8 patients (including different stages of the disease) and corresponding controls are planed.

Results:

The recruitment process is ongoing, results will be presented.

Conclusions:

Anterior segment PS-OCT might support a suspected diagnosis of Fabry�Â�´s disease, a genetic test still is required for a certain diagnosis. In future PS-OCTs with an improved resolution might be of benefit for a non invasive early diagnosis of Fabry�Â�´s disease.

Financial Disclosure:

NONE

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