A retrospective analysis of characteristics of visual field damage in patients with Leber's hereditary optic neuropathy

Ruijin Ran, Shuo Yang, Heng He, Shiqi Ma, Zhiqi Chen, Bin Li, Ruijin Ran, Shuo Yang, Heng He, Shiqi Ma, Zhiqi Chen, Bin Li

Abstract

The objective of this study is to investigate the characteristics and the evolution of visual field damage caused by Leber's hereditary optic neuropathy (LHON) and to provide clinical data for the diagnosis of LHON. Parameters of visual field in 32 consecutive patients (49 eyes) with LHON who were confirmed by genetic diagnostic tests were retrospectively measured within 1 week, between three to six months, and at six months after onset. Visual field defects revealed central scotoma in 26 eyes (53.1 %), paracentral scotoma in 12 eyes (24.5 %), ceco-central defects in 6 eyes (12.2 %), blind spot enlargenment in 3 eyes (6.1 %), quadrantanopia in 2 eyes (4.1 %) within 1 week after onset. After 3 to 6 months, ceco-central defects were detected in 22 eyes (44.9 %), central isopter constriction in 10 eyes (20.4 %), hemianopia or quadrantanopia in 5 eyes (10.2 %), central scotoma in 4 eyes (8.2 %), and paracentral scotoma in 1 eye (2.0 %). After 6 months, central isopter constriction was observed in 18 eyes (36.7 %), diffuse defects in 21 eyes (42.9 %), ceco-central defects in 3 eyes (6.1 %), hemianopia or quadrantanopia in 5 eyes (10.2 %), and central scotoma in 2 eyes (4.1 %). LHON at different stages was characterized by different focal visual field defects: visual field defects in LHON patients within 1 week after onset were mostly central or paracentral scotoma, which was enlarged around the ceco-central defect, or connected to form a blind spot after 3-6 months. Diffuse and central isopter constriction defects were usually developed after 6 months. Damages firstly appeared in papillomacular bundle and gradually expanded outward. These characteristics of visual field defects reported in this study might provide a clinical basis for better diagnosis of LHON.

Keywords: Leber’s hereditary optic neuropathy; Retrospective analysis; Visual field.

Figures

Fig. 1
Fig. 1
Correlations between VFI, MD and BCVA (LogMAR):MD was negatively correlated with BCVA (LogMAR) (r = –0.613, p = 0.011) (line a). VFI was negatively correlated with BCVA (LogMAR) (r = –0.703, p = 0.000) (line c). No correlation between PSD and BCVA (Log MAR) was detected (r = 0.102, p = 0.577) (line b)
Fig. 2
Fig. 2
Visual field defects found in patients with LHON (from the first to the last): characteristics of visual field damage are blind spot enlargement (a), paracentral scotoma (b), central scotoma (c), ceco-central scotoma (d), hemianopia (e), quadrantanopia (f), central isopter constriction (g), diffuse defects (h)
Fig. 3
Fig. 3
Characteristics of fundus in LHON patients. a The early manifestation of fundus (within 1 week): retinal vein and artery expanded around the optic disk. Edema and hyperemia surrounding the optic disc were observed. b The late stages (over 6 months): retinal vein and artery have become tapered and stiff. The optic disc is pale and atrophic

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