| Autor: |
Pippa Staps, Anita de Breuk, Johannes R.M. Cruysberg, Michèl Willemsen, Thomas Theelen |
| Jazyk: |
angličtina |
| Rok vydání: |
2022 |
| Předmět: |
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| Zdroj: |
Case Reports in Ophthalmology, Vol 13, Iss 2, Pp 341-349 (2022) |
| Druh dokumentu: |
article |
| ISSN: |
1663-2699 |
| DOI: |
10.1159/000524591 |
| Popis: |
Sjögren-Larsson syndrome (SLS) is a neurometabolic disease with a peculiar crystalline maculopathy. It is yet unclear if vascular abnormalities play a role in SLS maculopathy pathogenesis. We used optical coherence tomography angiography (OCT-A) to search for vessel abnormalities in SLS maculopathy. We performed a cross-sectional study in 4 patients (2 males, 2 females, aged 12–36 years) with various stages of SLS maculopathy. Besides OCT-A imaging, a complete ophthalmological examination and additional retinal imaging by transversal and en face spectral domain (SD) OCT were performed. OCT-A images were qualitatively assessed for vascular abnormalities, and imaging was compared to eight eyes of four healthy controls. On OCT-A, all eyes of patients with SLS showed a reduced capillary density around the fovea, and an enlarged foveal avascular zone (FAZ; SLS patients [n = 6 eyes] mean 0.70 mm2 [SD 0.18]; healthy controls [n = 8 eyes] mean 0.34 mm2 [SD 0.07], p = 0.004). In 2 patients, telangiectatic vessels were seen in the deep capillary layer. In conclusion, OCT angiography showed capillary paucity and morphological vessel abnormalities in these 4 patients with SLS. |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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