The Optical Coherence Tomographic Profile of Leber Hereditary Optic Neuropathy

Thomas R Hedges; Marisa Gobuty; Richard A Manfready; Natalie Erlich-Malona; Caitlin Monaco; Carlos E Mendoza-Santiesteban

doi:10.3109/01658107.2016.1173709

The Optical Coherence Tomographic Profile of Leber Hereditary Optic Neuropathy

Neuroophthalmology. 2016 May 2;40(3):107-112. doi: 10.3109/01658107.2016.1173709. eCollection 2016 Jun.

Authors

Thomas R Hedges¹, Marisa Gobuty¹, Richard A Manfready¹, Natalie Erlich-Malona¹, Caitlin Monaco¹, Carlos E Mendoza-Santiesteban²

Affiliations

¹ New England Eye Center, Tufts Medical Center, Tufts University , Boston, Massachusetts, USA.
² New England Eye Center, Tufts Medical Center, Tufts University, Boston, Massachusetts, USA; Dysautonomia Center, Department of Neurology, New York University School of Medicine, New York, New York, USA; Department of Ophthalmology, Pontifical Catholic University of Chile, Santiago de Chile, Chile.

Abstract

The objective of this study was to describe the changes in the retinal ganglion cell complex (GCC) relative to the retinal nerve fibre layer (RNFL) over time in Leber hereditary optic neuropathy (LHON) patients. Average RNFL and GCC thickness was measured in seven patients in the early acute (123, 68.4 μm), late acute (113.5, 57.4 μm), and chronic (72.7, 50.8 μm) phases. Patients showed thinning of the GCC with RNFL swelling in the early acute phase. GCC thinning became severe within weeks and persisted. RNFL swelling normalised during the late acute phase with eventual thinning in the chronic phase. GCC changes appear at the commencement of visual loss and in some cases prior to vision loss. These findings define an optical coherence tomography (OCT) profile in LHON.

Keywords: Ganglion cell complex; Leber hereditary optic neuropathy; optical coherence tomography; retinal nerve fibre layer.

Grants and funding

This work was supported by the Massachusetts Lions Clubs/Research to Prevent Blindness Challenge Grant to the New England Eye Center, Tufts Medical Center, Tufts University.