What are the characteristics and progression of visual field defects in patients with Leber hereditary optic neuropathy: a prospective single-centre study in China

Hong-Li Liu, Jia-Jia Yuan, Zhen Tian, Xin Li, Lin Song, Bin Li, Hong-Li Liu, Jia-Jia Yuan, Zhen Tian, Xin Li, Lin Song, Bin Li

Abstract

Objective: To study the characteristics and progression of visual field defects in patients with Leber hereditary optic neuropathy.

Design: Prospective study.

Setting: 3-A-class hospital in China; single-centre study.

Participants: From 100 patients diagnosed with Leber hereditary optic neuropathy, 80 (160 eyes; 68 men and 12 women; youngest patient, 6 years; oldest patient, 35 years) were recruited.

Exposure: All patients were followed up for at least 12 months. Each patient underwent at least three visual field examinations. Patient groups 1-6 were created according to the time of visual field data acquisition. Patient group 7 included patients with a different onset of disease between eyes. Group 8 was composed of patients with a course of disease of 12-24 months when one of the examinations performed. Patients who performed the third examination made up patient group 9.

Primary outcome measures: Prevalence of the different visual field defect types on the basis of severity in groups 1-6. Mean of the difference of visual function between eyes in group 7.

Result: In groups 1-6, the prevalences of defects classified using Visual Field Index values were significantly different between groups 1 and 3. In group 7, with the prolongation of the course of the disease, the mean of the difference of visual function between eyes decreased. There was no significant correlation between age and the severity of visual field defect. There was significant correlation between visual acuity and the severity of visual field defect.

Conclusion: Visual field defects in patients with Leber hereditary optic neuropathy (G11778A) may continuously progress within 6 months of disease development, and remain stable after 9 months. With the progression of the disease, the differences in visual function between eyes may decrease. The severity of visual field defect seems to be independent of age; however, could be related to visual acuity.

Trial registration number: NCT03428178, NCT01267422.

Keywords: genetic therapy; leber hereditary optic neuropathy; visual fields.

Conflict of interest statement

Competing interests: None declared.

© Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.

Figures

Figure 1
Figure 1
Progression of visual field defects in patients with Leber hereditary optic neuropathy. All patients are stratified into groups according to the time course of the disease. (A) Proportion of patients with severe visual field defects in the different groups. (B) Classification of visual field defects according to MD. The prevalence of the different defect types based on severity is significantly different between groups 1 and 4. There are no significant differences between adjacent groups. (C) Classification of visual field defects according to VFI. The prevalence of the different defect types based on severity is significantly different between groups 1 and 3. There are no significant differences between adjacent groups. Group 1: visual field data obtained within 1 month after vision loss. Group 2: visual field data obtained between 1 and 3 months after vision loss. Group 3: visual field data obtained between 3 and 6 months after vision loss. Group 4: visual field data obtained between 6 and 9 months after vision loss. Group 5: visual field data obtained between 9 and 12 months after vision loss. Group 6: visual field data obtained between 12 and 24 months after vision loss. MD, mean deviation; VFI, Visual Field Index. *There are significant differences.
Figure 2
Figure 2
Progression of visual field defects between eyes in patients with different onset of disease. Patient group 7 is stratified into subgroups according to the time course of the disease. Subgroup 1: visual field data obtained within 1 month after vision loss. Subgroup 2: visual field data obtained between 1 and 3 months after vision loss. Subgroup 3: visual field data obtained between 3 and 6 months after vision loss. Subgroup 4: visual field data obtained between 6 and 9 months after vision loss. Subgroup 5: visual field data obtained between 9 and 12 months after vision loss. Subgroup 6: visual field data obtained between 12 and 24 months after vision loss. MD, mean deviation; VFI, Visual Field Index.

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