Central Retinal Vein Occlusion in Young Healthy Patients and the Role of Thrombophilia in Pathogenesis

Yu Mi Lee; Myung Won Lee

doi:10.3341/jkos.2017.58.3.352

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Lee and Lee: Central Retinal Vein Occlusion in Young Healthy Patients and the Role of Thrombophilia in Pathogenesis

Case Report

Journal of the Korean Ophthalmological Society 2017; 58(3): 352-357.

Published online: 15 March 2017

DOI: https://doi.org/10.3341/jkos.2017.58.3.352

Central Retinal Vein Occlusion in Young Healthy Patients and the Role of Thrombophilia in Pathogenesis

Yu Mi Lee, MD, Myung Won Lee, MD

Department of Ophthalmology, Dankook University Hospital, Dankook University College of Medicine, Cheonan, Korea.

Address reprint requests to Myung Won Lee, MD. Department of Ophthalmology, Dankook University Hospital, #201 Manghyang-ro, Dongnam-gu, Cheonan 31116, Korea. Tel: 82-41-550-6497, Fax: 82-41-550-7050, persica79@naver.com

Received 1 September 2016 Revised 9 January 2017 Accepted 17 February 2017

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

We report two young patients who developed central retinal vein occlusion (CRVO) without any systemic disease, and various thrombophilia tests were performed to determine the etiology.

Case summary

Two young patients, a 22-year-old female and a 23-year-old male, who had acute vision loss were diagnosed with nonischemic CRVO via fluorescein angiography. They had no other disease and no common risk factors for CRVO. We performed various tests to determine the thrombophilic risk factors and discovered a transient decrease in protein S antigen and protein C antigen in the female and male patients, respectively.

Conclusions

CRVO in young patients without systemic disorders may have different mechanisms in the pathology and thus additional laboratory tests to determine thrombophilic disorders are necessary.

Keywords: Central retinal vein occlusion, Protein C deficiency, Protein S deficiency, Thrombophilic disorder

Figures and Tables

Figure 1

Color fundus photograph, fluorescein angiography (FAG), and optical coherence tomography (OCT) from right eye of a 23-year-old woman with central retinal vein occlusion and transient decrease in Protein S antigen. Color fundus photograph presented dilated retinal veins, flame-shaped hemorrhages in inferior area, multiple cotton-wool spots, and mild disk elevation (A ). FAG demonstrated disc hyperemia, leakage from retinal vein, macular edema and blocked hypofluorescence compatible with the hemorrhages (B). OCT demonstrated a significant macular edema with subretinal fluid (C) and bevacizumab was injected into vitreous at once. Following fundus photography (D) and FAG (E) of the same patient after 15 months later returned to normal. The macular edema had resorbed completely and visual acuity was also restored (F).

Figure 2

Color fundus photograph, fluorescein angiography (FAG), and optical coherence tomography (OCT) from right eye of a 22-year-old man with central retinal vein occlusion and transient decrease in Protein C antigen. Fundus photograph presented dilated retinal veins, flame-shaped retinal hemorrhages in 4 quadrant, and mild disk elevation (A). FAG demonstrated mild disc hyperemia, leakage from retinal vein, and blocked hypofluorescence compatible with the hemorrhages (B). OCT demonstrated nearly normal macular structure (C). Following fundus photography (D) and FAG (E) of the same patient after 15 months showing that almost retinal hemorrhage was absorbed but vessel tortuosity, small amount of retinal and disc hemorrhage, and leakage from capillary remained. The macula had maintained normal structure in following OCT (F) and the best corrective visual acuity was 1.0.

Table 1

Initial hematologic test for general condition in 2 CRVO patients

CRVO = central retinal vein occlusion; Hb = hemoglobin; Hct = hematocrit; WBC = white blood cell; PLT = platelet; BUN = blood urea nitrogen; AST = aspartate transaminase; ALT = alanine transaminase; Na = sodium; K = potassium; Cl = chloride; ESR = erythrocyte sedimentation rate; PT = prothrombin time; aPTT = activated partial thromboplastin time; HDL = high-density lipoprotein; LDL = low-density lipoprotein; CRP = C-reactive protein.

^*These criteria is for men and slightly different from women's. Normal range of Hb for women is from 12 to 16 g/dL, Hct from 36 to 48%.

Table 2

Coagulation profile in patients

Ag = antigen; Ab = antibody; IgG = immunoglobulin G; IgM = immunoglobulin M; GPI = glucose 6 phosphate isomerase; MPO = myeloperoxidase antibodie; P-ANCA = perinuclear antineutrophil cytoplasmic antibody; PR3 = proteinase 3 antibody; C-ANCA = cytoplasmic antineutrophil cytoplasmic antibody; IFA =immunofluorescence assay; RA = rheumatoid factor.

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