Abstract
4/2010
vol. 112
Case report
Estimation of morphology and function of the eye in Usher’s syndrom
- Klinika Okulistyki Dziecięcej z Ośrodkiem Leczenia Zeza Uniwersyteckiego Szpitala Klinicznego w Białymstoku
Online publish date: 2010/12/22
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.
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.
Integrated with
