Central retinal artery occlusion associated with patent foramen ovale

Abstracts

Central retinal artery occlusion it’s a disease most encountered in older patients, however it can be seen in children and young persons. In this situation the principal causes are cardiac abnormalities, and the patent foramen ovale is the most observed. The purpose of this study is to report a case of central retinal artery occlusion in a young patient with patent foramen ovale and, also, describe the importance of a detailed management in cases of retinal vascular occlusions.

Central retinal artery/pathology; Cardiac abnormalities; Foramen ovale, patent; Retinal artery occlusion; Young adult; Case reports

Oclusão da artéria central da retina é uma doença comumente encontrada em pacientes idosos, mas pode também ser vista em crianças e adultos jovens. Nestes, as principais causas são anomalias cardíacas, sendo o forame oval patente o mais observado. O objetivo do trabalho é relatar o caso de um paciente jovem com oclusão da artéria central da retina apresentando persistência de forame oval e, também, salientar a importância de uma propedêutica detalhada nos casos de oclusões vasculares da retina.

INTRODUCTION

Central retinal artery occlusion (CRAO) is characterised by a sudden, painless and severe loss of vision. It generally affects adults around the age of 60, and rarely below the age of 30. Men are more likely to be affected than women. In the large majority of cases, vision is worse than 20/400, but some patients present a cilioretinal artery which preserves a central vision of about 20/40 or better. Its most common cause is thrombosis due to atherosclerosis at the level of the lamina cribrosa⁽¹⁾. However, in young patients it is most frequently associated with cardiac disorders, trauma, haemoglobinopathies and ocular abnormalities such as optic disc drusen and peripapillary arterial loop⁽²⁾.

This paper describes a case of CRAO in a young patient with patent foramen ovale. To the best of our knowledge, this is the first such case report in the Brazilian literature.

CASE REPORT

RVS, a 19-year-old male student, was referred to the Santa Casa de Belo Horizonte Eye Clinic (MG) with a sudden and painless loss of visual acuity in the left eye (LE) occurring approximately 20 days earlier. He had no history of any disease, trauma, or use of medications or illegal drugs, including cigarettes. Ophthalmic examination found a visual acuity of 20/20 in the right eye (RE) and hand motion in the LE, showing no improvement with use of a pinhole. Biomicroscopy found an afferent pupillary defect in the LE and no changes in the RE. Intraocular pressure was 10 mmHg. Fundus examination showed optic disc and retinal pallor, arteriolar narrowing, flame-shaped haemorrhages and sequelae of oedema in the macular region, accumulation of hard exudates in the fovea, and mild macular star formation.

Due to suspected retinal vascular disease, the patient was referred for thromboembolism screening (Table 1), retinal fluorescein angiography, optical coherence tomography (OCT), and cardiac and haematological evaluation.

Table 1
Protocol for retinal thrombosis and vasculitis of the Retina and Vitreous Unit, Santa Casa de Misericórdia, Belo Horizonte

• Simple Retinography	• Homocysteine
• Angiography	• Protein electrophoresis
• Coagulation testing factors VII, IX, XI, fibrinogen	• Complete blood count: anemia – Hb, electrophoresis, search for sickle cells, bilirubin, Serun Fe, transderrin, ferritin, WBC/platelet count – mieloproliferative/ disorders / neoplasms
• Fasting serum glucose	• C3 - CH50
• Erytrocyte sedimentatiom rate	• Urea e creatinine
• ANF	• Urinalysis (sediments analysis)
• Lupus anticoagulant	• VDRL - FTA-Abs
• Anticardiolipin IgG/IgM	• PPD
• Antiphospholipid	• Toxoplasmosis IgM/IgG
• Antithrombin B	• Anti-HIV
• Protein C and S	• CMV
• Factor V Leiden	• Herpes virus
• Prothrombin gene mutation	• Hepatitis B, A e C

In the assessment of thromboembolic events, only the transthoracic echocardiogram was abnormal, showing an interatrial septum with a small right-to-left shunt suggestive of patent foramen ovale. Retinal fluorescein angiography (Figure 1) found hypofluorescent spots corresponding to the retinal haemorrhages and increased foveal hypofluorescence suggesting ischemia. OCT (Figure 2) found increased thickness and reflectivity of the internal layers of the retina corresponding to intracellular oedema and ischemia, attenuating the optical signals from the external retinal layers (RPE/Bruch membrane/ choriocapillaris complex). The patient is currently taking acetylsalicylic acid 100 mg/day as prescribed by his cardiologist.

Figure 1
1A a 1D: Retinography and fluorescein angiography showing signs of central retinal arteryocclusion.

Figure 2
Optical coherence tomography of patient with central retinal artery occlusion

DISCUSSION

According to the RECO (Retinal Emboli of Cardiac Origin) study, 45% of patients under 45 years of age with CRAO also present cardiac abnormalities, of whom 27% need to take anticoagulants or undergo cardiac surgery⁽³⁾.

Patent foramen ovale is the most common congenital cardiac abnormality, and it can cause cerebral and systemic emboli before the age of 55 years⁽⁴⁾. In cases of large patent foramen ovale with spontaneous right-to-left shunt, surgical treatment is recommended⁽⁵⁾. In the case presented here, the patient had a small patent foramen ovale without spontaneous shunt, being therefore eligible for medical treatment with an anticoagulant.

The occurrence of CRAO stresses the importance of a detailed systemic evaluation to identify its exact aetiology, with careful follow-up to prevent further thromboembolic events. For this reason, specialist services should have a screening protocol for cases of retinal vascular occlusion.

REFERÊNCIAS

• ¹
  Liesegng TJ, Skuta GL, Cantor LB, editors. Basic and Clinical Science Course. Section 12: Retina and vitreous. San Francisco:American Academy of Ophthalmology; 2005. p.54-79.
• ²
  Sharma S, Brown GC. Retinal artery obstruction. In: Ryan SJ. Retina, 3rd ed. St. Louis: Mosby; 2001. Vol. 1, p.1350-67.
• ³
  Clifford L, Sievers R, Salmon A, Newson RS. Central retinal artery occlusion: association with patent foramen ovale. Eye (Lond). 2006;20(6):736-8.
• ⁴
  Ho IV, Spaide R. Central retinal artery occlusion associated with a patent foramen ovale. Retina. 2007;27(2):259-60.
• ⁵
  Kramer M, Goldenberg-Cohen N, Shapira Y, Axer-Siegel R, Shmuely H, Adler Y, et al. Role of transesophageal echocardiography in the evaluation of patients with retinal artery occlusion. Ophtalmology. 2001;108(8):1461-4. Comment in Ophthalmology. 2002;109(5):829; author reply 829.

Publication Dates

• Publication in this collection
  Sep-Oct 2014

History

• Received
  20 Oct 2011
• Accepted
  27 Mar 2012