REVIEW Retinal vein occlusion: an approach to diagnosis, systemic risk factors and management J. W. Y. Yau, 1 P. Lee, 1 T. Y. Wong, 2 J. Best 1 and A. Jenkins 1 1 Department of Medicine, University of Melbourne, St Vincent’s Hospital and 2 Centre for Eye Research Australia, University of Melbourne, Melbourne, Victoria, Australia Key words retinal vein occlusion, cardiovascular disease, thrombophilia, blood coagulation disorder, risk factor. Correspondence Joanne W. Y. Yau, Department of Medicine, University of Melbourne, St Vincent’s Hospital, Fitzray Vic. 3065, Australia. Email: joanne.yau@svhm.org.au Received 18 October 2007; accepted 25 March 2008. doi:10.1111/j.1445-5994.2008.01720.x Abstract Retinal vein occlusion (RVO) is the second most common retinal vascular disease after diabetic retinopathy and is a common cause of visual morbidity and blindness in the elderly. A large proportion of patients with RVO has a history of cardiovascular disease, hypertension, diabetes mellitus or open- angle glaucoma. Although RVO is sometimes associated with thrombophilias and coagulation abnormalities, the role of coagulation factors in the develop- ment of RVO remains unclear. This review did not find strong evidence to support an extensive work-up for thrombophilic and coagulation diseases for the vast majority of patients. However, when tests for common cardiovascular risk factors for RVO are negative, evaluation for potential coagulation disorders may be indicated, particularly in young patients and in patients with bilateral RVO, a history of previous thromboses or a family history of thrombosis. Introduction Retinal vein occlusion (RVO) is the second most common retinal vascular disease after diabetic retinopathy and an important cause of visual morbidity and blindness. In Australia, the prevalence of RVO among persons aged 49 years and older is 1.6% and the 10-year incidence of new RVO cases is 1.6%. 1,2 Most patients who develop the disease are middle aged to elderly 1,2 and more than half have associated cardio- vascular disease (CVD). 3–5 Previous studies have shown an increased risk of RVO in patients with hypertension, 6–12 diabetes mellitus, 4–7,9–12 dyslipidaemia, 3,11 CVD 3,7,8 and open-angle glaucoma. 6–8,13,14 Although RVO is seen mainly in elderly persons, with 51% of cases occurring in individuals older than 65 years, 15 RVO can also occur in young adults, where the associations with systemic vas- cular disease are less common. 16 The exact pathogenesis and risk factors of RVO in younger patients, most of whom are otherwise healthy, are still poorly understood. Based on the occurrence of RVO in younger patients, risk factors that predispose to coagulation abnormalities have recently been examined in greater detail. Published work on thrombophilic abnormalities in RVO patients has accumulated recently and most of these reports consist of small studies and case reports based on retrospective infor- mation on patients retrieved from clinical databases. Cases in which deficiency of protein C, protein S and antithrom- bin III have been identified, 17,18 as well as others with Behcet disease, 19 antiphospholipid syndrome 20,21 and hyperhomocysteinaemia. 17,22 Prospective studies on the association between thrombophilic factors and RVO, how- ever, are rare. This report aims to review the association between systemic risk factors and RVO, focusing in particular on its association with cardiovascular risk factors and coagu- lation abnormalities. The pathogenesis of RVO is also dis- cussed in light of the underlying systemic disease and how it contributes to the aetiology of this retinal disorder. The key ocular signs on fundoscopy are emphasized and an ap- proach to clinical diagnosis and management is outlined. Funding: None Potential conflicts of interest: None Internal Medicine Journal 38 (2008) 904–910 904 ª 2008 The Authors Journal compilation ª 2008 Royal Australasian College of Physicians