In vivo vessel volume quantification in Leber's hereditary optic neuropathy (LHON) during the disease conversion and progression

Abstract

Purpose : Vascular changes are seen to occur in LHON. These include telangiectactic microangiopathy in the acute phase and extensive papillomacular capillary drop out in the later phase, suggesting a vascular component in the pathophysiology of this disorder. Here we analyse vascular changes using a novel imaging technique of the optic disc and peripapillary area in a patient with LHON.

Methods : A novel imaging technique characterised by three-dimensional volume rendering of optical coherence tomography angiography (3D OCTA) was used to analyse changes in the optic disc and peripapillary area during the progression and conversion of the disease. Optical coherence tomography (Cirrus™ HD-OCT Model 5000 with AngioPlex, Carl ZeissMeditec, Inc., Dublin,USA) volume scans were obtained from the optic nerve head of a 26-year-old female with acute LHON. Consequently, three-dimensional structural OCT and OCTA were analysed, enabling segmentation and spatial characterization of vascular changes seen in different stages of the disease. Clinical findings were collected during each visit.

Results : The first set of OCTA images were obtained after 106 days of rapid central vision loss in the right eye and 49 days in the left eye followed by images recorded approximately 1.5 and 4.5 months later. At the first visit we measured vessel volume at 343,49 x 10⁶ µm³ and area at 23.21 x 10⁶ µm² in the right eye; the decline in vessel measurements was noticed during the follow up visits and counted at 206,14 x 10⁶ µm³ and 13,31 x 10⁶ µm² respectively during the last visit (246 days after onset). The patient presented with a shorter disease duration in the left eye (49 days after onset) and vessel volume was 406,15 x 10⁶ µm³ and area was 28,45 x 10⁶ µm² at the first visit, followed by 421,79 x 10⁶ µm³ and 28,75 x 10⁶ µm² during the second and 187,84 x 10⁶ µm³ and 13,67 x 10⁶ µm² the last follow up visits.

Conclusions : This is the first description of 3D OCTA visualization and quantification of vascular changes in LHON patient during the disease progression. This method allows for in vivo imaging and provides crucial information and new insight on the spatio-anatomical features to better understand of the vascular component of the disease.

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

 

3D OCTA follow up images in a patient with LHON. Right eye is on the left side of the image, left eye on the right.