Monocular central retinal artery occlusion caused by bilateral internal carotid artery hypoplasia complicated with patent foramen ovale: a case report and review of literature

Abstract

Background Central retinal artery occlusion (CRAO) is an emergent ophthalmic disease which is commonly caused by atherosclerosis, thromboembolism, and arteriospasm. Here, we report a case of CRAO which is caused by extreme rare bilateral internal carotid artery (ICA) hypoplasia complicated with patent foramen ovale (PFO). The cardiogenic emboli blocked central retinal artery through unclosed foramen ovale and specific blood flow pathway. Case presentation This report describes a case of a 46-year-old woman sudden onset with amaurosis fugax for about 20 min and persistent visual impairment of left eye. Fundus fluorescein angiography shows the arm-retinal circulation time of left eye is 25 s, indicating that the occlusion occurs in the pathway from aortic arch to ophthalmic artery. The MRA and CTA examinations reveal the bilateral ICA hypoplasia and variation of Wills circle. Furthermore, transesophageal echocardiography (TEE) confirms the PFO and cardiogenic embolic event. Conclusions This work presents a CRAO case caused by rare congenital hypoplasia of ICA complicated with PFO, reminding us every single cause of vascular disease should be investigated carefully and the TOAST typing of cerebrovascular disease can be of great reference to the ocular vascular disease.

Congenital internal carotid artery hypoplasia: A systematic review

“…33,36 Vision loss, specifically amaurosis fugax due to ophthalmic artery insufficiency was found in two cases. 29,39 In all cases that presented with signs of ischemic stroke, the lateralisation of the hypoplasia corresponded with the side of ischemia. In general, older patients were more likely to present with stroke symptoms, and younger patients were more likely to present with an incidental complaint unrelated to CICAH.…”

“…Stroke-related symptoms were the most frequent reasons for an encounter with a physician (14 of 36 reports). Presentations included vision loss, 38,29,39 hemiplegia, 8,28,40,41,42 dysarthria 8,35,43,44 and TIA. 33,36 Vision loss, specifically amaurosis fugax due to ophthalmic artery insufficiency was found in two cases.…”

“…Some authors suggested a type that fitted best with their observations, and others noted that their cases had elements of more than one type. 39,40,59,60…”

“…Some authors suggested a type that fitted best with their observations, and others noted that their cases had elements of more than one type. 39,40,59,60 Zhang et al suggested a new classification based on visualisation of the ophthalmic segment. 15 They identified 20 cases of CICAH and divided them into two groups: ophthalmic segment occlusion (60%, n = 12) and nonophthalmic segment occlusion (40%, n = 8).…”

Central retinal artery occlusion as a result of symptomatic patent foramen ovale

“…They also conducted a review of literature and found seven such other cases, in which PFO has been implicated as a causative factor of CRAO. In their review, they cited transesophageal echocardiogram (TEE) as being more sensitive in diagnosing PFO as compared to TTE [ 9 , 10 ]. Our case also reported a similar occurrence of CRAO and PFO.…”