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Table of Contents
CASE REPORT
Year : 2022  |  Volume : 71  |  Issue : 1  |  Page : 71-73

A rare anomalous origin of the right vertebral artery from the right aortic arch with the left aberrant subclavian artery arising from kommerell's diverticulum


1 Department of Anatomy, Meram Faculty of Medicine, Necmettin Erbakan University, Konya, Turkey
2 Department of Radiology, Faculty of Medicine, Selcuk University, Konya, Turkey

Date of Submission23-Jan-2021
Date of Acceptance27-Oct-2021
Date of Web Publication17-Mar-2022

Correspondence Address:
Dr. Gulay Acar
Department of Anatomy, Meram Faculty of Medicine, Necmettin Erbakan University, Yunus Emre Mh. Unzile Sk., 42090 Meram, Konya
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jasi.jasi_17_21

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  Abstract 


Right-sided aortic arch (RAA) with left aberrant subclavian artery (LASA) is a rare vascular variant due to the failure in regression process during embryologic development of the aortic arch. The prevalence of it ranges from 0.04% to 0.1% in radiology series. We report the case of a 44-year-old female shown to have the presence of a RAA with LASA arising from the Kommerell's diverticulum, and also in association with an aberrant aortic origin of the right vertebral artery using computed tomography angiography. Considering the diameter of the diverticulum <5 cm and the absence of severe external esophageal or tracheal compression, thoracic surgery was not indicated and it was decided to keep the patient under clinical follow-up at regular intervals. Based upon this present case and literature review, the knowledge of this anomalous anatomy and its embryologic basis appear to be important for diagnostic endovascular interventions and planning thoracic surgery.

Keywords: Aberrant left subclavian artery, computed tomography angiography, Kommerell's diverticulum, right-sided aortic arch, right vertebral artery


How to cite this article:
Acar G, Koplay M. A rare anomalous origin of the right vertebral artery from the right aortic arch with the left aberrant subclavian artery arising from kommerell's diverticulum. J Anat Soc India 2022;71:71-3

How to cite this URL:
Acar G, Koplay M. A rare anomalous origin of the right vertebral artery from the right aortic arch with the left aberrant subclavian artery arising from kommerell's diverticulum. J Anat Soc India [serial online] 2022 [cited 2022 May 24];71:71-3. Available from: https://www.jasi.org.in/text.asp?2022/71/1/71/339874




  Introduction Top


Right-sided aortic arch (RAA) is a rare and mostly asymptomatic variant of the aortic arch (AA) with a frequency of <0.1% in adult population. Type II RAA associated with the left aberrant subclavian artery (LASA) arising from the Kommerell's diverticulum (KD) is observed in <50% of the RAA.[1],[2],[3] We reported this case with an anomalous aortic origin of the right vertebral artery (RVA) using computed tomography angiography (CTA). An aberrant origin of vertebral artery (VA) observed mostly on the left side may cause vertebrobasilar insufficiency.[4] To the best of our knowledge, this is the second documented report of Type II RAA with an aberrant aortic RVA in the literature.


  Case Report Top


A 44-year-old woman with a history of systemic hypertension presented at the emergency department with severe headache, dizziness, and dyspnea. Furthermore, she had complaints of anxiety episodes, dyspnea on exertion, and no other cardiovascular symptoms. The amplitude of arterial pulse and systolic blood pressure value were found lower in the left upper limb than right on physical examination. Thoracic CTA (128-slice CT scanner; Siemens Somatom Definition Flash, Munich, Germany) was performed to rule out pulmonary embolism after written informed consent was obtained from the patient. Evaluation of CTA images revealed an incidental finding of the RAA with the LASA arising from the KD with a diameter of 3.9 cm, as well as an anomalous aortic origin of the RVA [Figure 1].
Figure 1: Computed tomography angiography volume-rendered reconstruction showing Type II RAA with aberrant RVA. (a) Anterolateral and (b) posterolateral aspect. RCCA: Right common carotid artery, LCCA: Left common carotid artery, RSA: Right subclavian artery, ALSA: Left aberrant subclavian artery, RVA: Right vertebral artery, KD: Kommerell's diverticulum

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The KD has a mild mass effect on the trachea, and the LASA forms a retroesophageal vascular ring. The RVA was found to arise directly from the AA between the right common carotid artery (RCCA) and the right subclavian artery (RSA), and extend upward to the transverse process of cervical vertebrae to pass through the foramen of C4 and all of the upper transverse processes [Figure 2]. Considering her imaging findings and medical history with the absence of dysphagia, the patient was interpreted as unsuitable for surgical intervention. Regular follow-up without medication was recommended to the patient. Furthermore, the patient confirmed written informed consent for publication of this case report with images.
Figure 2: Thoracic computed tomography angiography sagittal maximum intensity projection image showing an aberrant origin of the right vertebral artery from the aortic arch. RCCA: Right common carotid artery, RSA: Right subclavian artery, RVA: Right vertebral artery

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  Discussion Top


The classic left-sided AA (LAA) gives rise to three branches from right to left: the brachiocephalic trunk, the left common carotid artery (LCCA), and finally, the left subclavian artery (LSA). Normally, the VA is originated as the first branch of the ipsilateral subclavian artery. In the literature, the AA branching variations are a common finding with a diversity of frequencies ranging from 5% to 35%.[1],[3] RAA anomaly has three subtypes based on Edwards classification: mirror image RAA (Type I, 59%), RAA with LASA (Type II, 39.5%), and RAA with direct communication between isolated LSA and pulmonary artery (Type III, 1.5%) [Figure 3].[5],[6] The KD is a bulbous dilatation of an anomalous origin of the LASA which leads to a relatively loose vascular ring mostly behind the esophagus. The LASA coexists with congenital heart diseases in 5%–10% of cases. Ichikawa et al. reported the prevalence of KD as 100% in Type II RAA, while it was found in 62% in the study by Tyczyński et al.[1],[7],[8] Owing to compression of the esophagus or trachea, the KD can cause miscellaneous symptoms such as dyspnea and dysphagia and rarely results in an aortic dissection or ruptured aneurysm. Considering the diameter (≥5 cm) of the KD and severe symptoms such as an aortic aneurysm and a cerebrovascular insufficiency, surgical intervention can be required.[1],[2],[9]
Figure 3: Schematic diagram showing the left-sided aortic arch and subtypes of the right-sided aortic arch. Type I; Mirror image right-sided aortic arch, Type II; Right-sided aortic arch with left aberrant subclavian artery, Type III; Right-sided aortic arch with direct communication between the isolated left subclavian artery and left pulmonary artery

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Embryology

During the 4th and 8th weeks of intrauterine life, a series of aortic regression and reformation result in the formation of the LAA, which was reported in about 74%–89.4% of cases. Initially, the primitive AA consists of ventral and dorsal aortic trunks. Between these trunks, six paired branchial arch arteries develop and seven cervical intersegmental arteries (CIAs) arise from the dorsal aorta at different stages of the organogenesis. Normally, the LAA is formed by the regression of the dorsal aortic trunk and ductus arteriosus on the right side. In contrast, the failure of this involution results in RAA, which is subdivided into three types.[5],[6],[9] First, the RAA was identified by Fioratti and Aglietti in 1763. Due to the persistence of the right fourth branchial arch and involution or regression of the left fourth dorsal aortic segment between the LCCA and LSA, the RAA with LASA is formed, and also, the KD develops as a remnant of the fourth primitive dorsal arch during embryonic period. Type II RAA is a rare (0.05%–0.1%) and mostly an asymptomatic incidental finding unless the KD becomes a compressive mass or ruptured.[1],[2],[3] After the obliteration of six CIAs, the seventh CIA converts into the proximal subclavian artery giving origin to the VA, which is the further development of the intercostal longitudinal anastomosis. The failure of involution in these developmental stages results in different anomalous origins of the VA. If the first or the second CIA does not regress, the VA arises from the external or internal carotid artery, whereas the persistence of other CIAs (3–6) causes an anomalous origin of the VA from the AA or common carotid artery.[5],[9],[10],[11] Hence, in this case, we suggested that the right anastomosis between the sixth and seventh CIAs may not occur and the sixth CIA fails to regress.

We presented a rare case of Type II RAA having branches, in a proximal to distal progression, with the following order: LCCA, RCCA, RVA, RSA, and LASA arising from the KD. A review of the literature from 2011 to date showed that 32 case reports of Type II were reported. Concerning the higher incidence of ruptured aneurysm or dissection associated with the KD, the prevalence of it was reported by Cinà et al. as 53%, by Kouchoukos and Masetti as 20%, and by Austin and Wolfe as 19%.[5],[8],[12],[13],[14] Detailed identification of this aberrant vascular anatomy is crucial for accurate surgical and endovascular intervention planning and to prevent intraoperative vascular complications during surgery and diagnostic cerebral angiography.[1],[2],[5]

In the recent literature, an aberrant origin of the RVA is less common than the LVA, whereas the prevalence of the aortic origin of the RVA associated with Type II RAA is even more the rarest of all. Generally, an anomalous aortic origin of the RVA most commonly occurs as the last branch of the AA distal to the origin of the LSA.[1],[9],[10] To our knowledge, an anomalous origin of the RVA from the RAA, with the LCCA as the first branch of the AA followed by RCCA, RSA, and LASA, has been presented only in the study by Tyczyński et al.[1] An aberrant origin of the RVA directly from the arch may lead to an increased risk of spinal and right upper limb ischemia.[1],[4]

Although this anomaly is found incidentally and mostly asymptomatic, preoperative evaluation using CTA is essential for planning endovascular intervention and surgical approach and avoidance of unexpected outcomes. Identification of this case report with the review of the literature has significant clinical implications in thoracic surgery and endovascular procedures.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Tyczyński P, Michałowska I, Wolny R, Dobrowolski P, Łazarczyk H, Rybicka J, et al. Left aberrant subclavian artery. Systematic study in adult patients. Int J Cardiol 2017;240:183-6.  Back to cited text no. 1
    
2.
Zhyvotovska A, Yusupov D, Abdul R, Chandrakumar H, Hartt A, Akter K, et al. Right-sided aortic arch with aberrant left subclavian artery in a pregnant female: A case report and literature review. Am J Med Case Rep 2020;8:143-7.  Back to cited text no. 2
    
3.
Oztas DM, Umutlu M, Ertan M, Beyaz MO, Badem S, Erdinc I, et al. Brief review of right aortic arch with aberrant left subclavian artery. Aorta (Stamford) 2019;7:179-80.  Back to cited text no. 3
    
4.
Lazaridis N, Piagkou M, Loukas M, Piperaki ET, Totlis T, Noussios G, et al. A systematic classification of the vertebral artery variable origin: Clinical and surgical implications. Surg Radiol Anat 2018;40:779-97.  Back to cited text no. 4
    
5.
Edwards JE. Anomalies of the derivates of the aortic arch system. Med Clin North Am 1948;32:925-49.  Back to cited text no. 5
    
6.
Tong E, Rizvi T, Hagspiel KD. Complex aortic arch anomaly: Right aortic arch with aberrant left subclavian artery, fenestrated proximal right and duplicated proximal left vertebral arteries-CT angiography findings and review of the literature. Neuroradiol J 2015;28:396-403.  Back to cited text no. 6
    
7.
Ichikawa T, Koizumi J, Tanno K, Okochi T, Nomura T, Shimura S, et al. Kommerell diverticulum in adults: Evaluation of routine CT examinations. Tokai J Exp Clin Med 2016;41:65-9.  Back to cited text no. 7
    
8.
Silveira JV, Junqueira FP, Silveira CG, Consolim-Colombo FM. Kommerell diverticulum: Right aortic arch with anomalous origin of left subclavian artery and duplicity of right vertebral artery in a 16-year-old girl. Am J Case Rep 2019;20:228-32.  Back to cited text no. 8
    
9.
Morishita A, Tomioka H, Katahira S, Hoshino T, Hanzawa K. Surgical treatment for kommerell's diverticulum associated with a right-sided aortic arch and an aberrant left subclavian artery: Endovascular or hybrid. Ann Vasc Dis 2019;12:228-32.  Back to cited text no. 9
    
10.
Vitošević F, Vitošević Z, Rasulić L. The right vertebral artery arising from the right common carotid artery: Report of a rare case. Surg Radiol Anat 2020;42:1263-6.  Back to cited text no. 10
    
11.
Maiti TK, Konar SK, Bir S, Nanda A, Cuellar H. Anomalous origin of the right vertebral artery: Incidence and significance. World Neurosurg 2016;89:601-10.  Back to cited text no. 11
    
12.
Cinà CS, Althani H, Pasenau J, Abouzahr L. Kommerell's diverticulum and right-sided aortic arch: A cohort study and review of the literature. J Vasc Surg 2004;39:131-9.  Back to cited text no. 12
    
13.
Kouchoukos NT, Masetti P. Aberrant subclavian artery and Kommerell aneurysm: Surgical treatment with a standard approach. J Thorac Cardiovasc Surg 2007;133:888-92.  Back to cited text no. 13
    
14.
Austin EH, Wolfe WG. Aneurysm of aberrant subclavian artery with a review of the literature. J Vasc Surg 1985;2:571-7.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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