International Journal of Ophthalmic PathologyISSN: 2324-8599

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Research Article, Int J Ophthalmic Pathol Vol: 2 Issue: 1

Retinal Nerve Fiber Layer Thickness Analysis with Optical Coherence Tomography in Wolfram Syndrome

Ágata Mota1*, Sofia Fonseca1, Carla Sofia Ferreira1, Olinda Faria1,2, Sérgio Estrela Silva1, Manuel Falcão1,2, Susana Penas1,2, Elisete Brandão1, Fernando Falcão Reis1,2
1Ophthalmology Department, Hospital de São João, Porto, Portugal
2Faculdade de Medicina, Universidade do Porto, Porto, Portugal
Corresponding author : Ágata Mota
Ophthalmology Department, Hospital de São João, Alameda Professor Hernâni Monteiro, 4200 – 319 Porto, Portugal
Tel: +351 965381831
E-mail: [email protected]
Received: November 17, 2012 Accepted: January 15, 2013 Published: January 22, 2013
Citation: Mota Á, Fonseca S, Ferreira CS, Faria O, Silva SE, et al. (2013) Retinal Nerve Fiber Layer Thickness Analysis with Optical Coherence Tomography in Wolfram Syndrome. Int J Ophthalmic Pathol 2:1. doi:10.4172/2324-8599.1000107

Abstract

Retinal Nerve Fiber Layer Thickness Analysis with Optical Coherence Tomography in Wolfram Syndrome

Purpose: To evaluate the peripapillary retinal nerve fiber layer (RNFL) in Wolfram’s Syndrome (WS) using optical coherence tomography (OCT).

Methods: The peripapillary RNFL of eighteen eyes of nine patients
with the clinical diagnosis of WS were measured using Stratus OCT. The RNFL was determined consecutively three times.

Results: The mean RNFL thickness is inferior to the 99% normal limit given by the instrument´s normative database in all patients. The superior and inferior quadrants were beneath the 99% normal limit in every patient. The nasal quadrant was at the level 1-5% in 37.0% of the measurements and at the level 5-95% in 3.7%. The temporal quadrant was at the level 1-5% in 13.0% of the measurements and at the level 5-95% in 5.5%.

Conclusion: In our series, there is an overall decrease of the RNFL thickness. The nasal and temporal quadrants were the least affected. This is the first time that this characteristic of the disease is described. Understanding the pattern of RNFL damage can be important clinically to differentiate WS from other causes of hereditary optic atrophy that have distinct patterns of RNFL loss. This provides further insight in the lesion pattern and pathophysiology of the disease.

Keywords: Wolfram syndrome; DIDMOAD; Retinal nerve fiber layer; Stratus OCT; Optic nerve atrophy

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