Choroidal thickness and the retinal ganglion cell complex in chronic Leberʼs hereditary optic neuropathy: a prospective study using swept-source optical coherence tomography

Abstract

Choroidal thinning has been suggested in Leber’s hereditary optic neuropathy (LHON). No study has been conducted of the choroid in relation to the retinal ganglion cell-inner plexiform layer (RGC-IPL). We sought to measure choroidal thickness in chronic LHON and to correlate thickness changes with the RGC-IPL. Chronic LHON, 11778 mitochondrial DNA (mtDNA) mutation, patients (26 eyes; mean age: 35.1\u2009±\u200916.1 years) were prospectively recruited at Doheny Eye Center, University of California Los Angeles from March 2016 to July 2017. Age-matched healthy controls (27 eyes; mean age: 32.4\u2009±\u200911.1 years) were enroled for comparison. Swept-source optical coherence tomography (SS-OCT) imaging was performed in chronic LHON patients and compared with age-matched healthy controls. The macular choroid was significantly thinner in chronic LHON (250.5\u2009±\u200962.2\u2009μm) compared with controls (313.9\u2009±\u200960.2\u2009μm; p\u2009<\u20090.0001). The peripapillary choroid was also significantly thinner in chronic LHON (135.7\u2009±\u200951.4\u2009μm) compared with controls (183.0\u2009±\u200961.8\u2009μm, p\u2009<\u20090.001). Choroidal thickness strongly correlated with retinal nerve fibre layer (RNFL) thickness in both the macular (R2\u2009=\u20090.72; 95% CI, 0.57–0.84) and peripapillary regions (R2\u2009=\u20090.53; 95% CI, 0.31–0.70). Choroidal thickness was also significantly correlated with macular RGC-IPL thickness (R2\u2009=\u20090.51; 95% CI, 0.26–0.73). Choroidal thinning in chronic LHON correlated strongly with both RNFL and RGC-IPL thicknesses. These findings may suggest a pathophysiological mechanism involving vascular pathology of the choroid in relation to the retinal ganglion cell complex in LHON.