Secondary hyperhomocysteinemia-related occlusive retinal vasculopathy: A case report

Abstract

Purpose: To report a case of occlusive retinal vasculopathy, secondary to hyperhomocysteinemia.

Observations: A 43-year-old male was examined at the retina outpatient clinic due to complaints of bilateral decrease in visual acuity. The patient underwent a comprehensive ophthalmological examination, wide-field fundus photographs and fluorescein angiography, as well as spectral domain optical coherence tomography with enhanced-deep imaging. The patient had a significant medical history of chronic kidney disease and progressive bilateral vision loss over the last two years, which worsened in the left eye during the past 3 months. Fundus examination revealed a vitreous hemorrhage in the left eye and bilateral proliferative retinopathy. Blood glucose and systemic blood pressure were unremarkable. Plasma homocysteine was reported at >500 μmol/L, which is higher than the corrected reference range by age.

Conclusion and importance: Hyperhomocysteinemia is a rare but well-known disease, capable of accelerating atherosclerotic disease and generating a prothrombotic state that can lead to multiple systemic complications. Despite its low incidence, the disease should be part of the differential diagnosis in patients with bilateral proliferative retinopathy, in the absence of diabetes mellitus and systemic hypertension.

Keywords: Atherosclerosis; Chronic kidney disease; Hyperhomocysteinemia; Occlusive retinal vasculopathy; Proliferative retinopathy.

Fig. 1

Ultra-wide field retinal Imaging of the right eye shows a vitreous hemorrhage, with a subhyaloid hemorrhage sparing the fovea, neovascularization of the optic disk, engorged and pale arterial retinal vessels, thinning of venous vessels, hyperpigmented deposits and pigmentary changes.

Fig. 2

Ultra-wide field retinal Imaging of the left eye displays an old vitreous hemorrhage on the inferior quadrants, fibrovascular proliferation arising from the optic disk head, engorged and pale arterial retinal vessels, thinning of venous vessels, hyperpigmented deposits and pigmentary changes.

Fig. 3

A. Fluorescein angiography of the right eye shows hyperfluorescence with a leopard spot pattern in the choroid, leakage of the neovascularization of the optic disk. B. Periphery capillary dropout areas.

Fig. 4

Fluorescein angiography of the left eye shows leakage of the neovascularization of the optic disk and vitreous hemorrhage.

Fig. 5

Spectral domain optic coherence tomography of the right eye shows hyperreflective atherosclerotic vascular wall of the superior temporal arterial branch arcade.

Fig. 6

Enhanced depth imaging of the right eye shows thickening of the choroidal vessels' walls and lumen enlargement.

Fig. 7

Six month after panretinal photocoagulation. A. Fluorescein angiography of the right eye shows scars of photocoagulation, inactivation of proliferative retinopathy. B. Fluorescein angiography of the left eye shows inactivation of proliferative retinopathy, no leakage visible.

Fig. 8

A. Ultra-wide field retinal Imaging of the right eye shows the absence of retinal neovascularization and neovascularization of the optic disk. B. Ultra-wide field retinal Imaging of the left eye shows an old vitreous hemorrhage on the inferior quadrants, absence of retinal neovascularization and neovascularization of the optic disk.