Retinal and choroidal findings detected by swept-source optical coherence tomography in paediatric patients with usher syndrome

Session Details

Session Title: Free Paper Session 25: Imaging IV

Session Date/Time: Sunday 10/09/2017 | 10:00-11:30

Paper Time: 11:06

Venue: Room 113

First Author: : O.Subirà SPAIN

Co Author(s): :    J. Catala-Mora   N. Padronn-Perez   J. Diaz-Cascajosa                       

Abstract Details


To report the retinal and choroidal features detected by Swept-source optical coherence tomography (ss-OCT) in paediatric patients with Usher syndrome (USH).


Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Barcelona, SPAIN


ss-OCT scans of 28 eyes from 14 patients (9 male, 5 female) aged 7-16 years (mean age of 13 years), affected by USH were reviewed. 12 patients had USH type 1 and only 2 patients had USH type 2. A careful evaluation was carried out on external limiting membrane (ELM), inner limiting membrane (ILM), epiretinal membranes (ERMs), retinal micropseudocysts (MPCs) and cystoid macular edema (CME) and photoreceptor inner/outer segment (IS/OS) junction. Eventually, subfoveal retinal thickness (SFRT) and subfoveal choroidal thickness (SFCT) were measured.


The ELM was absent in 12 eyes (42,8%), whereas the ILM was found in 15 eyes (53,5%) and the presence of an ERM was detected only in 2 eyes (7.17%). On the other hand, MPCs were detected in 17 eyes (60.7%) and CME was found in 8 eyes (28.5%). Finally, the RPE disruption was only detected in 2 eyes, although the photoreceptor IS/OS junction was absent in the foveal region of 2 eyes (7.17%) and disrupted in 10 eyes (35.7%).


Our study indicates that USH is associated with alterations of many retinal layers. In particular, the vitreoretinal interface is affected in 60.7% of patients (either presence of ILM or ERM) and MPCs were detected in another 60.7%. These findings happen early in the evolution of the disease and could play a role in possible future treatments. We regard ss-OCT may contribute to understand the physiopathology involved in the first stages of USH.

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