Background: Agenesis of the internal carotid artery (ICA) is a rare anatomic variation of the cerebral circulation. A malfunction during the embryonic peri-od is at stake. It is very often asymptomatic and therefore discovered inci-dentally. Association with ischemic stroke is exceptional. Physiopathology is uncertain; the hypothesis of a blood flow reversal is reported in the literature. The diagnosis is based on the absence of a carotid canal as revealed by CT scans at skull base level with bone settings. Other non-invasive techniques, in-cluding echo-Doppler of the neck vessels, can now make the diagnosis. Objec-tive: Discuss the association between occurrence of an ischemic stroke in the left posterior sylvian territory and discovery of ipsilateral carotid agenesis. Case report: A 24-year-old female patient was prospectively recruited at DAX hospital center (France). Vascular risk factors involved active smoking of 8 pack-years and a combined estrogen/progestin contraceptive. Her family history included an uncle with heterozygote Factor V Leiden mutation. She presented a left upper limb sensorimotor deficit. Nuclear Magnetic Resonance Angiography (MRA) revealed a right superficial sylvian ischemic stroke asso-ciated with agenesis of the right internal carotid artery. CT scan at skull base level with bone settings showed absence of a right carotid canal, thus confirm-ing agenesis. An opinion was then requested for an arteriography which showed an operative polygon of Willis, a sylvian artery or middle cerebral ar-tery (MCA) irrigated by a very large right posterior communicating artery (PCOM) and the left anterior cerebral artery (ACA) supplied by the anterior communicating artery (ACOM), through the left carotid system. The right syl-vian territory where stroke occurred was therefore supplied by the functional vertebrobasilar system. ECG and four-day meticulous monitoring detected no cardiac rhythm disorders. Transesophageal echocardiography was normal. Blood tests results showed normal blood coagulation with glycohemoglobin level at 4.6%. Total cholesterol was 1.42 g/l including LDL-C at 0.82 g/l and HDL-C at 0.36 g/l; triglycerides levels were 1.23 g/l. VDRL-TPHA serodiagnosis for syphilis, and HIV serology were all negative. Thrombophilia testing was performed and showed absence of Factor V Leiden mutation. Homocyste-inemia level was normal. The patient was discharged under KARDEGIC 160 mg, one daily with home rehabilitation. Currently she’s doing well and no re-currence is observed.
Cite this paper
Jennifer, M. N. , Martinez, M. , Desbordes, P. , Diawara, A. , Houeze, R. , Laurent, C. and Jacquin, J. (2018). Agenesis of Internal Carotid Artery and Ischemic Stroke, One Case Report: A Review of Literature. Open Access Library Journal, 5, e4947. doi: http://dx.doi.org/10.4236/oalib.1104947.
Okawa, M., Higashi, T., Komiyama, M., Fukuda, K., Abe, H. and Inoue, T. (2015) Left Internal Carotid Artery Agenesis with Trans-Sellar Collateral and a Right Aortic Arch: Case Report. Interventional Neuroradiology, 21, 759-764. https://doi.org/10.1177/1591019915609130
Kiritsi, O., Noussios, G., Tsitas, K. and Lappas, D. (2012) Unilateral Agenesis of the Internal Carotid Artery Presented as Transient Ischaemic Attack: A Case Report. Surgical and Radiologic Anatomy, 34, 475-477. https://doi.org/10.1007/s00276-011-0901-z
Li, S., Hooda, K., Gupta, N. and Kumar, Y. (2017) Internal Carotid Artery Agenesis: A Case Report and Review of Literature. The Neuroradiology Journal, 30, 186-191. https://doi.org/10.1177/1971400917692162
Tode. Medizinisch chirurgische Bibliothek (Kopenhgen) 1787; 10: 408. In: Die, K.W., der Arterien, V. und Venen, H.J., Eds., Handbuch der System Anatomie des Menschen, Vol. 3, Part 1, 2nd Edition, Vieweg, Brunswick, 1876.
Claros, P., Bandos, R., Gilea, I., et al. (1999) Case Report: Major Con-Genital Anomalies of the Internal Carotid Artery—Agenesis, Aplasia, and Hypoplasia. International Journal of Pediatric Otorhinolaryngology, 49, 69-76. https://doi.org/10.1016/S0165-5876(99)00012-9
Lie, T. and Hage, J. (1968) Congenital Anomalies of the Carotid Arteries. Plastic and Reconstructive Surgery, 42, 283. https://doi.org/10.1097/00006534-196809000-00046
Sarcelle, J.S., Naheedy, M.H. and Hasso, A.N. (1980) Agénésie totale de l’artère carotide interne. AJNR American Journal of Neuroradiology, 1, 435-442.
Given, C.A. II, Huang-Hellinger, F., Baker, M.D., et al. (2001) Congenital Absence of the Internal Carotid Artery: Case Reports and Review of the Collateral Circulation. American Journal of Neuroradiology, 22, 1953-1959.
David, H., Dubayle, P., Girodeau, A., Sarrazin, J.L. and Boyer, B. (2000) Agénésie de la carotid interne à propos d’un cas. Journal de radiologie, 82, 147-150.
Handa, J., Matsuda, I., Nakasu, S. and Nakano, Y. (1980) Agenesis of the Internal Carotid Artey: Angiographic, Tomographic and Computed Tomographic Correlation. Neuroradiology, 19, 207-211. https://doi.org/10.1007/BF00376709
Pascaud, J.L., Vigneu, P., Hummel, P., Bouchet, J.B., Rihollet, J. and Pascaud-Ged, E. (1982) Absence partielle de carotide interne avec anastomose intercarotidienne transsellaire. Nouvelle observation et revue de la littérature. Journal of Radiology, 63, 37-40.
Elefante, R., Fucci, G., Granata, F., Graziussi, G. and Smaltino, F. (1983) Agenesis of the Right Internal Carotid Artery with an Unusual Transsellar Intracavernous Intercarotid Connection. American Journal of Neuroradiology, 4, 88-89.
Faivre, J., Vallée, B., Carsin, M., Scarabin, J.M. and Simon, J. (1978) Agénésie des portions cervicale et pétreuse de la carotide interne gauche. Revascularisation de sa portion caverneuse par une anastomose intercarotidienne transsphénoidale. Journal of Neuroradiology, 5, 133-138.
Lasjaunias, P. and Santoyo-Vazquez, A. (1984) Agenesis of the Internal Carotid Artery: Angiographic Aspects with Embryological Discussion. Clinical Anatomy, 6, 133-141. https://doi.org/10.1007/BF01773165
Tasar, M., Yetiser, S., Tasar, A., et al. (2004) Congenital Absence or Hypoplasia of the Carotid Artery: Radioclinical Issues. American Journal of Otolaryngology, 25, 339-349. https://doi.org/10.1016/j.amjoto.2004.04.008
Florio, F., Balzano, S., Nardella, M., et al. (1999) Congenital Absence of the Internal Carotid Artery. CardioVascular and Interventional Radiology, 22, 74-78. https://doi.org/10.1007/s002709900334
Kahraman, A.S., Kahraman, B., Ozdemir, Z.M., Dogan, M., Kaya, M., Gormeli, C.A. and Durak, M.A. (2016) Congenital Agenesis of Right Internal Carotid Artery: A Report of Two Cases. Journal of the Belgian Society of Radiology, 100, 48.
Leys, D., Lucas, C., Gautier, C., Hachulla, E. and PruvoLes, J.-P. (2004) Accidents is chémiques cérébraux du sujet jeune. Mini-revueSang Thrombose Vaisseaux, 16, 43-50.
Ottaviano, G., Calzolari, F. and Martini, A. (2007) Goldenhar Syndrome in Association with Agenesia of the Internal Carotid Artery. International Journal of Pediatric Otorhinolaryngology, 71, 509-512. https://doi.org/10.1016/j.ijporl.2006.11.003
Afifi, A.K., Godersky, J.C., Menezes, A., Smoker, W.R. and Bell, W.E. (1987) Cerebral Hemiatrophy, Hypoplasia of Internal Carotid Artery, and Intracranial Aneurysm. A Rare Association Occurring in an Infant. Archives of Neurology, 44, 232-235.
Xie, T., Zhang, X.B., Li, Q.P., et al. (2010) Hemifacial Spasm Patient with Ipsilateral Total Absence of Common Carotid Artery, Vertebra-Lartery and Aneurysm of the Contralateral Internal Carotid Artery. Surgical and Radiologic Anatomy, 32, 707-710. https://doi.org/10.1007/s00276-010-0660-2