Retinal artery occlusion in an otherwise healthy, young patient is rare. In this context it is important to consider patent foramen ovale as a differential. Early referral to a cardiology specialist for diagnosis and treatment is important for preventing further ocular and non-ocular events

Arif, S

Calvert, PA

Denniston, AK

Keane, PA

Liu, X

UCL Discovery

Case reportPatent foramen ovale presenting as visual lossXiaoxuan Liu1, Patrick A Calvert2, Sayqa Arif2, Pearse A Keane3and Alastair K Denniston1,4,51Department of Ophthalmology, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust,Birmingham B15 2TH, UK2Department of Cardiology, Papworth Hospital NHS Foundation Trust, Cambridge CB23 3RE, UK3NIHR Biomedical Research Centre for Ophthalmology, Moorfields Eye Hospital NHS Foundation Trust and UCL Institute ofOphthalmology, London EC1V 2PD, UK4Academic Unit of Ophthalmology, Institute of Inflammation and Ageing, University of Birmingham, Birmingham B15 2WB, UK5Centre for Rare Diseases, Institute of Translational Medicine, Birmingham Health Partners, Birmingham B15 2TH, UKCorresponding author: Alastair K Denniston. Email: a.denniston@bham.ac.ukLessonRetinal artery occlusion in an otherwise healthy, youngpatient is rare. In this context it is important to considerpatent foramen ovale as a differential. Early referral to acardiology specialist for diagnosis and treatment is impor-tant for preventing further ocular and non-ocular events.KeywordsCardiovascular medicine, Clinical, OphthalmologyCase reportA 21-year-old man presented with an eight-day his-tory of a blurry patch of vision in the right eye. Hecould not identify any precipitants, had no signiﬁcantmedical history and did not take any regular medica-tion. His best corrected visual acuity was 6/4 unaidedin both eyes. Intraocular pressure was normal in botheyes and anterior segments appeared quiet. Ondilated fundoscopy, a ﬁlling defect was apparent ina branch of the inferotemporal retinal artery of theright eye approximately 1–2 disc diameters awayfrom the optic disc, associated with pale areas resem-bling cotton wool spots. The diagnosis of branch ret-inal artery occlusion was made. These ﬁndings werealso visualised with optical coherence tomography –Figure 1. One week later, the patient’s symptoms ofblurriness had subjectively improved, and the paleareas previously described had reduced in size.Screening for cytomegalovirus, toxoplasma, humanimmunodeﬁciency virus, vasculitis and thrombophiliawere all negative. Routine blood tests were insigniﬁ-cant with a total cholesterol of 4.6mmol/L, triglycer-ides of 1.9mmol/L, erythrocyte sedimentation rate of2 urea and normal renal and liver function.Referral to cardiology was made to investigatefor possible cardiac causes of embolism, and thepatient was empirically started on aspirin.General cardiological assessments were unremark-able with a blood pressure of 135/83mmHg, heartrate of 81 beats per minute, normal heart soundswith no murmurs or added sound and no carotidbruits were detected. Magnetic resonance imagingof the head, magnetic resonance angiogram of thehead and neck vessels (speciﬁcally looking for carotidartery dissection), and carotid doppler scans were allnormal. A 72-hour Holter monitoring test showedsinus rhythm throughout, and conventional trans-thoracic echocardiography was insigniﬁcant.However, a transthoracic echocardiographic bubblecontrast study, which showed no evidence of bubblestransiting at rest, did show a large right to left shuntat the atrial level within one beat of release of val-salva manoeuvre – Figure 2. This was strongly sug-gestive of a large patent foramen ovale. No otherpotential causes for the retinal artery embolus wereidentiﬁed at any point.In view of the potential consequences of futureemboli causing further occlusions of the retinal orcerebral circulations, percutaneous closure of thepatent foramen ovale was undertaken, alongside sixmonths of low-dose aspirin and lifelong clopidogreltherapy. This patient has had no further episodes andat 12 months since the episode he is asymptomaticwith normal visual acuity (6/6 unaided in each eye).DiscussionPatent foramen ovale is a persistent cardiac commu-nication between the left and right atria, which nor-mally closes within a year after birth. In utero, thiscommunication allows right-to-left shunting of thecirculation. Soon after birth, as the pulmonary circu-lation is established, the pulmonary pressure dropsand the left atrial pressure exceeds that of the right,! 2016 The Author(s)Creative Commons CC-BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 3.0 License (http://www.creativecommons.org/licenses/by-nc/3.0/) which permits non-commercial use, reproduction and distribution of the work without further permission providedthe original work is attributed as specified on the SAGE and Open Access page (https://uk.sagepub.com/en-us/nam/open-access-at-sage).Journal of the Royal Society of Medicine Open;8(1) 1–3DOI: 10.1177/2054270416669302causing the foramen to close. Patent foramen ovale isprevalent in 20–34% of the population1 and are gen-erally benign and asymptomatic. Occasionally, theycan give rise to systemic emboli, which can cause bothocular and cerebral ischaemic events, such as crypto-genic strokes. Reported ocular manifestations ofpatent foramen ovale include central and branch ret-inal arterial occlusion and optic neuropathy.2,3 Patentforamen ovale has also been associated with decom-pression sickness in scuba divers and recurrentmigraines.1 In our patient, diagnosis was madeusing transthoracic echocardiogram bubble study;Figure 1. Composite demonstrating partial occlusion of a branch retinal arteriole characterised by embolic material within theretinal arteriole and ‘cotton wool spots’ of the adjacent retina. Spectralis MulticolorTM images comprising multicolour andassociated infrared reflectance, green reflectance, blue reflectance images at time of presentation (a) and at six months later (b)with associated retinal thickness heat maps (c) and absolute thickness measurements in microns (d) at the same time points;change in retinal thickness is also shown (e).Figure 2. Fundus fluorescein angiogram demonstrating reduced flow through a branch macular arteriole arising from theinferotemporal arcade visualised with 55’ lens (a) and ultra-widefield lens (b) of the Heidelberg Spectralis HRA OCT. The cottonwool spots are seen as three hypofluorescent areas.2 Journal of the Royal Society of Medicine Open 8(1)however, it has been reported that transoesophagealechocardiogram is more sensitive in the detection ofcardiac sources of retinal emboli when compared totransthoracic echocardiogram.4Retinal artery occlusions encompass a group ofdiseases, whereby sudden obstruction of the arterialcirculation causes ischaemic damage to the retina.5 Itis primarily a disease of the older population and isdivided into two subtypes: branch retinal arteryocclusion and central retinal artery occlusion.Central retinal artery occlusion usually results in pro-found visual loss in the aﬀected eye, whereas branchretinal artery occlusion may not aﬀect the centralvision and instead leave a scotoma, or a visual ﬁelddefect.The commonest risk factors for branch retinalartery occlusion are hypertension, atherosclerosis,diabetes mellitus and thrombophilia, but it may alsoarise from ipsilateral carotid artery atherosclerosisand cardiogenic embolism, such as in atrial ﬁbrilla-tion, cardiac valvular disease and infectious endocar-ditis. Retinal artery occlusion in young patients withpatent foramen ovale is rare and has only beenreported in a handful of cases.2,3,6,7This case highlights the importance of consideringpatent foramen ovale in the context of retinal arteryocclusion in an otherwise healthy young patient. Suchcases beneﬁt from early referral to a cardiology spe-cialist to enable prompt diagnosis and treatment inorder to prevent further serious ocular and non-ocular events.DeclarationsCompeting Interests: The views expressed in the publication arethose of the author and not necessarily those of the Department ofHealth.Funding: PK has received a proportion of his funding from theDepartment of Health’s NIHR Biomedical Research Centre forOphthalmology at Moorﬁelds Eye Hospital and UCL Instituteof Ophthalmology.Ethical approval: Written informed consent was obtained fromthe patient for publication of this case report and accompanyingimages.Guarantor: AD.Contributorship: AD and PC were involved in patient care. XL,AD and PC drafted the manuscript. All authors critically revisedand approved the ﬁnal manuscript.Acknowledgements: None.Provenance: Not commissioned; peer-reviewed by R Ismail.References1. Calvert PA, Rana BS, Kydd AC and Shapiro LM.Patent foramen ovale: anatomy, outcomes, and closure.Nat Rev Cardiol 2011; 8: 148–160.2. Clifford L, Sievers R, Salmon A and Newsom RS.Central retinal artery occlusion: association withpatent foramen ovale. Eye (Lond) 2006; 20: 736–738.3. Nakagawa T, Hirata A, Inoue N, Hashimoto Y andTanihara H. A case of bilateral central retinal arteryobstruction with patent foramen ovale. ActaOphthalmol Scand 2004; 82: 111–112.4. Kramer M, Goldenberg-Cohen N, Shapira Y, Axer-Siegel R, Shmuely H and Alder Y. Role of transesopha-geal echocardiography in the evaluation of patients withretinal artery occlusion. Ophthalmology 2001; 108:1461–1464.5. Gass JDM. Stereoscopic atlas of macular diseases: diag-nosis and treatment, 4th ed. St Louis: Mosby, 1997.6. Morjaria R, Tsaloumas M and Shah P. An unusualpresentation of patent foramen ovale. JRSM Open2015; 6: 2054270415596320.7. Ho IV and Spaide R. Central retinal artery occlusionassociated with a patent foramen ovale. Retina 2007;27: 259–260.Liu et al. 3

Patent foramen ovale presenting as visual loss

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Patent foramen ovale presenting as visual loss

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