RIGHT AORTIC ARCH ASSOCIATED WITH AGENESIS OF THE LEFT INTERNAL CAROTID ARTERY – AN EXTREMELY RARE CASE REPORT
Keywords:Carotid artery, Agenesis, Intracranial aneurysm, Right aortic arch
Introduction: A right aortic arch and agenesis of internal carotid artery (ICA) are both extremely rare vascular devel- opment anomalies. Etiology of the both anomalies might be associated with the abnormal regression of the dorsal aorta. Most cases of ICA are asymptomatic due to sufficient collateral circulation and it is usually an incident finding on head and neck imaging by color Doppler ultrasonography, computed tomography (CT) or magnetic resonance imaging (MRI).
ICA agenesis has a significant association – 24-67% – with intracranial aneurysms and their early detection can spare the patient serious complications.
Clinical case: A 28-year-old male had a single episode of hypertension that motivated the realization of several tests. During the investigation he was submitted to a duplex ultrasonography that revealed a diffuse narrowing of the left common carotid artery (CCA), with a markedly decrease in the peak systolic velocity and the absence of the left internal carotid artery (ICA) was suspected. Contrast-enhanced computed tomography (CT) demonstrated no abnormalities, such as cerebral infarc- tion or intracranial vascular malformations, but confirmed a right-sided aortic-arch, with anomalous origin of the left subcla- vian artery with a common origin of both CCAs and the absence of the left ICA. Examination of the head CT in bone window demonstrated an absence of the left internal carotid canal.
Conclusion: This clinical case emphasizes the importance of recognizing this condition due to the associated hemody- namic changes and in order to discover and evaluate other additional vascular malformations (aneurysms, collateral channels) and their life threatening potential risks (subarachnoid hemorrhage or ischemia). Also, it has a special importance in case of planning carotid or trans-sphenoidal hypophyseal surgery. To our knowledge, only 8 cases have been reported right aortic arch associated with agenesis of the left internal carotid artery.
Florio F, Balzano S, Nardella M, Strizzi V, Cammisa M, Bozzini V, Catapano G, D’Angelo V. Congenital absence of the internal carotid artery. Cardiovasc Interv Radiol. 1999;22:74-78
Kaya O, Yilmaz C, Gulek B, et al. An important clue in the sonographic diagnosis of internal carotid artery agenesis: ipsilateral common carotid artery hypopla- sia. Case Rep Radiol. 2014;2014:516456. http://dx.doi. org/10.1155/2014/516456. PMid:25097789
Dainese L, Spirito R, Barili F, et al. Right aortic arch relat- ed to Kommerell diverticulum and internal carotid artery agenesis. Circ Cardiovasc Imaging 2: e6–e7, 2009
Given CA 2nd, Huang-Hellinger F, Baker MD, Chepuri NB, Morris PP. Congenital absence of the internal carotid artery: case reports and review of the collateral circulation. AJNR Am J Neuroradiol. 2001;22(10):1953-9. PMid:11733331
Graham CB III, Wippold FJ II, Capps GW. Magnetic resonance imaging in internal carotid
artery agenesis with computed tomography and angiographic correlation: case reports. Angiology. 1999;50:847– 853 Slankamenac P, Jesic A, Zivanovic Z, Gebauer-Bukurov K, Bozic K. Agenesis of internal carotid artery with unusual fetal collateral circulation. Arch Neurol. 2010;67:1280- 1281.
Farhat W, Ahdab R, Hosseini H. Congenital agenesis of internal carotid artery with ipsilateral Horner presenting as focal neurological symptoms. Vasc Health Risk Manag, 2011, 7:37–40
Ito S, Miyazaki H, Iino N, Shiokawa Y, Saito I. Unilateral agenesis and hypoplasia of the internal carotid artery: a report of three cases. Neuroradiology 2005; 47: 311-15
Cali RL, Berg R, Rama K. Bilateral internal carotid artery agenesis: a case study and review of the literature. Surgery. 1993;113(2):227-33. PMid:8430372
Tao F, Heiden RA, Konkus CJ, Solano JP, Yuppa F. Congenital absence of left internal carotid artery associated with aberrant intracranial arterial circulation. Appl Radiol, 2005, 34(6):44–46
Bhat DI, Somanna S, Kovoor JM. ME. Bilateral hypoplasia of the internal carotid artery. Indian J Radiol Imaging, 2011, 21(4):257–260
Lie TA, Hage J. Congenital anomalies of the carotid arteries. Plast Reconstr Surg. 1968;42:35-51
Teal JS, Naheedy MH, Hasso AN. Total agenesis of the internal carotid artery. AJNR Am J Neuroradiol. 1980;1:435-42
Afifi AK, Godersky JC, Menezes A, Smoker WR, Bell WE, Jacoby CG. Cerebral hemiatrophy, hypoplasia of internal carotid artery, and intracranial aneurysm. A rare association occurring in an infant. Arch Neurol 1987;44:232-5
Paredes AM, Caspi J, Lilje C. Tetralogy of Fallot and anomalous origin of the left pulmonary artery from the ascending aorta associated with absent right internal carotid artery. World J Pediatr Congenit Heart Surg 6: 324–327, 20157)
Alexander MW, Chui MC, Gray T. Transellar intercavernous carotid anastomosis with agenesis of internal carotid artery and right-sided descending aorta: case report. J Can Assoc Radiol 35: 92–95, 19848
Okawa M, Higashi T, Komiyama M, Fukuda K, Abe H, Inoue T. Left internal carotid artery agenesis with trans-sellar collateral and a right aortic arch: Case report. Interv Neuroradiol 21: 759–764, 20159
Suzuki N, Tanaka N, Hirata K, Yamashita Y, Shiozawa T. Dysgenesis of the bilateral internal carotid arteries. Case report. Neurol Med Chir (Tokyo) 24: 881–885, 1984
Rumbolt Z, Castillo M, Solander S. Bilateral congenital absence of the internal carotid artery. Eur Radiol 13: L130– L132, 200311
Yamasaki K, Yokogami K, Ohta H, Yamashita S, Kawasoe T, Takeshima H. Agenesis of the left internal carotid artery in the right aortic arch with isolation of the left innominate artery associated with ruptured cerebral aneurysm: case re- port. NMC Case Rep J 2: 46–48, 2015
Iida Y, Nagamine H, Nomura M. Right Aortic Arch Associated with Left Internal Carotid Artery Agenesis. NMC Case Report Journal 2017; 4: 79–82
How to Cite
Copyright (c) 2022 Portuguese Journal of Cardiac Thoracic and Vascular Surgery
This work is licensed under a Creative Commons Attribution 4.0 International License.