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4/2010
vol. 112 abstract:
Case report
Estimation of morphology and function of the eye in Usher’s syndrom
Małgorzata Mrugacz
1
,
Michał Szumiński
1
,
Dorota Średzińska-Kita
1
,
Alina Bakunowicz-Łazarczyk
1
Online publish date: 2010/12/22
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Purpose: Retinitis pigmentosa (RP) is a set of heterogenous retinal diseases that affect primarily photoreceptors and retinal pigmented
epithelium. The Usher’s syndrome (RP associated with hearing impairment or loss), is responsible for about 10-20% of all cases. We investigate macular morphology using spectral domain optical coherence tomography in correlation with visual function. Material and methods: Two patients with Usher syndrome: a 13 years old girl and her 9 years old sister underwent comprehensive ophthalmological examination including: BCVA testing (Snellen charts), biomicroscopy of the anterior and posterior segment of the eye, electroretinography and SD-OCT scans of the macular region. Results: BCVA was noticeable decreased in both eyes (Vod = 5/16, Vos = 5/16), of 13 years old patient, while it was slightly diminished in her younger sister (Vod = 5/6, Vos = 5/6). In patient with visual deterioration a blue cone deficiency was found. Central foveal thickness (CFT) and foveal outer segment/pigment epithelium thickness (FOSPET) was significantly reduced in 13 years old patient. Conclusions: Spectral optical coherence tomography is a useful method to monitor morphological changes of the macula and their progress in patients with retinitis pigmentosa in Usher’s syndrome. |
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