{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,5]],"date-time":"2026-03-05T10:28:48Z","timestamp":1772706528690,"version":"3.50.1"},"reference-count":19,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,6,13]],"date-time":"2021-06-13T00:00:00Z","timestamp":1623542400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,6,13]],"date-time":"2021-06-13T00:00:00Z","timestamp":1623542400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100004731","name":"Natural Science Foundation of Zhejiang Province","doi-asserted-by":"publisher","award":["LY21H090015"],"award-info":[{"award-number":["LY21H090015"]}],"id":[{"id":"10.13039\/501100004731","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Eur J Med Res"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>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.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>This report describes a case of a 46-year-old woman sudden onset with amaurosis fugax for about 20\u00a0min and persistent visual impairment of left eye. Fundus fluorescein angiography shows the arm-retinal circulation time of left eye is 25\u00a0s, 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.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>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.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s40001-021-00530-w","type":"journal-article","created":{"date-parts":[[2021,6,13]],"date-time":"2021-06-13T22:02:36Z","timestamp":1623621756000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Monocular central retinal artery occlusion caused by bilateral internal carotid artery hypoplasia complicated with patent foramen ovale: a case report and review of literature"],"prefix":"10.1186","volume":"26","author":[{"given":"Lanbing","family":"Zhu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Na","family":"Xu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2304-8982","authenticated-orcid":false,"given":"Yan","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,6,13]]},"reference":[{"issue":"3","key":"530_CR1","doi-asserted-by":"publisher","first-page":"243","DOI":"10.1590\/1677-5449.001918","volume":"17","author":"AC Guimaraes","year":"2018","unstructured":"Guimaraes AC, Pessoa TDB, Moreira RH, de Araujo WJB. Agenesis of the internal carotid artery. J Vasc Bras. 2018;17(3):243\u20137.","journal-title":"J Vasc Bras"},{"issue":"9","key":"530_CR2","first-page":"e163","volume":"51","author":"YS Luk","year":"2010","unstructured":"Luk YS, Man EM, Sy AN. Bilateral hypoplasia of the internal carotid arteries. Singapore Med J. 2010;51(9):e163-165.","journal-title":"Singapore Med J"},{"issue":"2","key":"530_CR3","doi-asserted-by":"publisher","first-page":"186","DOI":"10.1177\/1971400917692162","volume":"30","author":"S Li","year":"2017","unstructured":"Li S, Hooda K, Gupta N, Kumar Y. Internal carotid artery agenesis: a case report and review of literature. Neuroradiol J. 2017;30(2):186\u201391.","journal-title":"Neuroradiol J"},{"issue":"12","key":"530_CR4","doi-asserted-by":"publisher","first-page":"105342","DOI":"10.1016\/j.jstrokecerebrovasdis.2020.105342","volume":"29","author":"S Giragani","year":"2020","unstructured":"Giragani S, Kumar K, Kasireddy AR, Alwala S. Bilateral internal carotid artery agenesis and posterior circulation stroke: a rare association. J Stroke Cereb Dis. 2020;29(12):105342.","journal-title":"J Stroke Cereb Dis"},{"issue":"2","key":"530_CR5","doi-asserted-by":"publisher","first-page":"133","DOI":"10.1007\/BF01773165","volume":"6","author":"P Lasjaunias","year":"1984","unstructured":"Lasjaunias P, Santoyo-Vazquez A. Segmental agenesis of the internal carotid artery: angiographic aspects with embryological discussion. Anat Clin. 1984;6(2):133\u201341.","journal-title":"Anat Clin"},{"issue":"2","key":"530_CR6","doi-asserted-by":"publisher","first-page":"477","DOI":"10.1148\/radiology.182.2.1732967","volume":"182","author":"DJ Quint","year":"1992","unstructured":"Quint DJ, Silbergleit R, Young WC. Absence of the carotid canals at skull base CT. Radiology. 1992;182(2):477\u201381.","journal-title":"Radiology"},{"issue":"2","key":"530_CR7","first-page":"283","volume":"42","author":"TA Lie","year":"1968","unstructured":"Lie TA. J H: Congenital anomalies of the carotid arteries. Plast Reconstr Surg. 1968;42(2):283.","journal-title":"Plast Reconstr Surg"},{"issue":"4","key":"530_CR8","doi-asserted-by":"publisher","first-page":"352","DOI":"10.1016\/S0090-3019(97)00491-6","volume":"50","author":"T Hattori","year":"1998","unstructured":"Hattori T, Kobayashi H, Inoue S, Sakai N. Persistent primitive trigeminal artery associated with absence of internal carotid artery. Surg Neurol. 1998;50(4):352\u20135.","journal-title":"Surg Neurol"},{"issue":"10","key":"530_CR9","doi-asserted-by":"publisher","first-page":"1027","DOI":"10.1179\/016164110X12767786356273","volume":"32","author":"JE Cohen","year":"2010","unstructured":"Cohen JE, Gomori JM, Leker RR. Internal carotid artery agenesis: diagnosis, clinical spectrum, associated conditions and its importance in the era of stroke interventions. Neurol Res. 2010;32(10):1027\u201332.","journal-title":"Neurol Res"},{"issue":"9","key":"530_CR10","doi-asserted-by":"publisher","first-page":"2339","DOI":"10.1007\/s00330-001-1262-5","volume":"12","author":"A Uchino","year":"2002","unstructured":"Uchino A, Sawada A, Hirakawa N, Totoki T, Kudo S. Congenital absence of the internal carotid artery diagnosed during investigation of trigeminal neuralgia. Eur Radiol. 2002;12(9):2339\u201342.","journal-title":"Eur Radiol"},{"issue":"4","key":"530_CR11","doi-asserted-by":"publisher","first-page":"483","DOI":"10.1097\/WNO.0000000000000666","volume":"38","author":"R Dhiman","year":"2018","unstructured":"Dhiman R, Gorimanipalli B, Swamy DR, Sharma S, Garg A, Saxena R. Congenital Third nerve palsy associated with midbrain hypoplasia due to bilateral segmental internal carotid artery agenesis. J Neuro-Ophthalmol. 2018;38(4):483\u20135.","journal-title":"J Neuro-Ophthalmol"},{"issue":"6","key":"530_CR12","first-page":"566","volume":"23","author":"G Medhi","year":"2017","unstructured":"Medhi G, Pendharkar H, terBrugge K, Prasad C, Gupta AK. Trigeminal neuralgia caused by a new variant of persistent trigeminal artery associated with agenesis of internal carotid artery. Interv Neuroradiol J Peritherapeutic Neuroradiol Surg Proced Related Neurosci. 2017;23(6):566\u201370.","journal-title":"Interv Neuroradiol J Peritherapeutic Neuroradiol Surg Proced Related Neurosci"},{"issue":"10","key":"530_CR13","first-page":"2635","volume":"26","author":"YC Weon","year":"2005","unstructured":"Weon YC, Chung JI, Kim HJ, Byun HS. Agenesis of bilateral internal carotid arteries and posterior fossa abnormality in a patient with facial capillary hemangioma: presumed incomplete phenotypic expression of PHACE syndrome. AJNR Am J Neuroradiol. 2005;26(10):2635\u20139.","journal-title":"AJNR Am J Neuroradiol"},{"issue":"3","key":"530_CR14","doi-asserted-by":"publisher","first-page":"509","DOI":"10.1016\/j.ijporl.2006.11.003","volume":"71","author":"G Ottaviano","year":"2007","unstructured":"Ottaviano G, Calzolari F, Martini A. Goldenhar syndrome in association with agenesia of the internal carotid artery. Int J Pediatr Otorhinolaryngol. 2007;71(3):509\u201312.","journal-title":"Int J Pediatr Otorhinolaryngol"},{"issue":"20","key":"530_CR15","doi-asserted-by":"publisher","first-page":"E740","DOI":"10.1097\/BRS.0b013e3181b41181","volume":"34","author":"M Braga","year":"2009","unstructured":"Braga M, Pederzoli M, Beretta S, Ferrarini M, Canovaro P, Arpaia G, Crespi V. Agenesis of the right internal carotid artery and Klippel-Feil syndrome: case report. Spine. 2009;34(20):E740-742.","journal-title":"Spine"},{"issue":"11","key":"530_CR16","doi-asserted-by":"publisher","first-page":"1805","DOI":"10.1007\/s003300000500","volume":"10","author":"HJ Jaeger","year":"2000","unstructured":"Jaeger HJ, Mehring UM, Gissler HM, Mathias KD. Congenital absence of the internal carotid artery and the basilar artery with persistent trigeminal artery associated with coarctation of the aorta. Eur Radiol. 2000;10(11):1805\u20139.","journal-title":"Eur Radiol"},{"issue":"2","key":"530_CR17","doi-asserted-by":"publisher","first-page":"138","DOI":"10.1007\/s002340100678","volume":"44","author":"WJ Moon","year":"2002","unstructured":"Moon WJ, Porto L, Lanfermann H, Weis R, Zanella FE. Agenesis of internal carotid artery associated with congenital anterior hypopituitarism. Neuroradiology. 2002;44(2):138\u201342.","journal-title":"Neuroradiology"},{"key":"530_CR18","doi-asserted-by":"publisher","first-page":"58","DOI":"10.1186\/s12902-015-0037-y","volume":"15","author":"S Stagi","year":"2015","unstructured":"Stagi S, Traficante G, Lapi E, Pantaleo M, Becciani S, Mortilla M, Seminara S, de Martino M. Agenesis of internal carotid artery associated with isolated growth hormone deficiency: a case report and literature review. BMC Endocr Disord. 2015;15:58.","journal-title":"BMC Endocr Disord"},{"key":"530_CR19","doi-asserted-by":"publisher","first-page":"128","DOI":"10.1016\/j.jocn.2020.11.009","volume":"83","author":"A MacDonald","year":"2020","unstructured":"MacDonald A, Alvaro A. CADASIL in a patient with bilateral internal carotid artery agenesis. J Clin Neurosci. 2020;83:128.","journal-title":"J Clin Neurosci"}],"container-title":["European Journal of Medical Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40001-021-00530-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s40001-021-00530-w\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40001-021-00530-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,6,13]],"date-time":"2021-06-13T22:02:57Z","timestamp":1623621777000},"score":1,"resource":{"primary":{"URL":"https:\/\/eurjmedres.biomedcentral.com\/articles\/10.1186\/s40001-021-00530-w"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,6,13]]},"references-count":19,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["530"],"URL":"https:\/\/doi.org\/10.1186\/s40001-021-00530-w","relation":{},"ISSN":["2047-783X"],"issn-type":[{"value":"2047-783X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,6,13]]},"assertion":[{"value":"20 May 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"29 May 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"13 June 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This study was performed in accordance with the tenets of the Declaration of Helsinki and with the ethical standards of the Ethics committee of the second affiliated hospital and Yuying children hospital of Wenzhou Medical University. Consent to participate was not applicable due to the retrospective design of this study.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the patient for the publication of this report and any accompanying images.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"55"}}