July 2018
Volume 59, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2018
Histopathologic Validation of Choroidal Thickness as a Biomarker for Leber's Hereditary Optic Neuropathy (LHON)
Author Affiliations & Notes
  • Fei Lin
    Ophthalmology, Doheny Eye Institute-UCLA, Los Angeles, California, United States
    Ophthalmology, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, California, United States
  • Samuel Asanad
    Ophthalmology, Doheny Eye Institute-UCLA, Los Angeles, California, United States
    Ophthalmology, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, California, United States
  • Fatemeh Darvizeh
    Ophthalmology, San Raffaele Scientific Institute, Milan, Italy
  • Fred N. Ross-Cisneros
    Ophthalmology, Doheny Eye Institute-UCLA, Los Angeles, California, United States
    Ophthalmology, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, California, United States
  • Mario Schunimann
    Ophthalmology, Doheny Eye Institute-UCLA, Los Angeles, California, United States
  • Piero Barboni
    Ophthalmology, San Raffaele Scientific Institute, Milan, Italy
  • Alfredo A Sadun
    Ophthalmology, Doheny Eye Institute-UCLA, Los Angeles, California, United States
    Ophthalmology, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, California, United States
  • Footnotes
    Commercial Relationships   Fei Lin, None; Samuel Asanad, None; Fatemeh Darvizeh, None; Fred Ross-Cisneros, None; Mario Schunimann, None; Piero Barboni, None; Alfredo Sadun, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science July 2018, Vol.59, 307. doi:
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      Fei Lin, Samuel Asanad, Fatemeh Darvizeh, Fred N. Ross-Cisneros, Mario Schunimann, Piero Barboni, Alfredo A Sadun; Histopathologic Validation of Choroidal Thickness as a Biomarker for Leber's Hereditary Optic Neuropathy (LHON). Invest. Ophthalmol. Vis. Sci. 2018;59(9):307.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : We previously demonstrated significant choroidal thinning in patients with chronically severe Leber’s hereditary optic neuropathy (LHON) using swept-source optical coherence tomography (SS-OCT). Here, we histopathologically validate the choroid as a potential in vivo biomarker for LHON using postmortem tissue. We hypothesize that the choroid will exhibit significant thinning in chronically severe LHON.

Methods : 5 LHON, 11778 mtDNA mutation, postmortem eyes (mean age: 61.4±9.91 years) were compared to 5 age-matched control eyes (mean age: 57.4± 6.23 years). Tissues were fixed in phosphate-buffered formalin, dissected horizontally through the middle of the optic nerve, embedded into paraffin, sectioned and stained with hematoxylin and eosin. Choroidal thickness was measured at 7 different points beginning from the fovea out to 2.5mm both nasally and temporally. Choroidal thickness was defined as the distance from Bruch's membrane (BM) to the inner scleral margin. Welch’s t-test was used for statistical analysis.

Results : LHON eyes exhibited a significant reduction in choroidal thickness compared to controls. In the temporal region, LHON mean total thickness was 442.7± 7.60 μm compared to 601.22 ± 4.99 μm in controls (p=0.0005). In the nasal region, LHON mean total thickness was 409.37 ± 8.27μm compared to 550.64 ± 18.51μm in controls (p=0.016). Temporal thinning was most severe in the parafoveal region, measuring 139.07±1.73 μm in LHON and 194.7±1.45 μm in controls (p=0.001). In contrast to the temporal side, choroidal thinning on the nasal side was most pronounced within the peripapillary region, measuring 127.65±1.22 μm in LHON and 163.6±1.32 μm in controls (p=0.005).

Conclusions : Histopathological measurements of choroidal thickness revealed significant thinning in both the parafoveal and peripapillary regions of the choroid in LHON similarly observed in optical coherence tomography (OCT) studies. This data can be helpful in validating the in vivo choroidal thickness measurements in LHON.

This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.

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