ARTERIA LUSORIA : A CASE REPORT

1. ARTERIA LUSORIA : A CASE REPORT M. BOUSSALAH, N. TOUIL, S. HABCHAOUI, O. KACIMI, N. CHIKHAOUI Emergency Radiology Department, IbnRoch University Hospital, Casablanca, Morroco VARIOUS : VR 8

2. The aberrant right subclavian artery (ARSA) is the most common anomaly of the aortic arch, occurring in 0.5 to 2.5% of individuals [1]. • It is the first arch anomaly to have been described in 1735 by Hunauld [2]. • David Bayford was the first to describe dysphagia caused by an aberrant right subclavian artery, calling the clinical syndrome dysphagia lusoria and the aberrant artery causing it arteria lusoria [3]. INTRODUCTION : VARIOUS VR : 8

3. With the advent and widespread use of precise non-invasive imaging techniques, such as computed tomography and magnetic resonance angiography, this arch anomaly is recognized more frequently. • Objectives : We aim to provide a concise overview of the epidemiology, development, anatomy, clinical presentation, imaging and management of arteria lusoria for the clinician confronted with a patient with this anomaly. INTRODUCTION : VARIOUSVR : 8

4. We describe findings in a patient, in whom angiographic investigation, for subarachnoid hemorrhage, incidentally revealed a complex anomaly of supra aortic vessels : an arteria lusoriaarising from a common trunk between the subclavian arteries associated to a truncus bicaroticus. MATERIELS AND METHODS : VARIOUS VR : 8

5. Mr L. M • Thirty-fouryears-old, white man, • Withoutmedicalhistory of dysphagia, dyspnea or coughing, • Admitted for subarachnoidhemorrhage, • The angiographic investigation showed incidentally an aberrant right subclavian artery arising from a common trunk between the subclavian arteries associated to a truncus bicaroticus ( in the anteroposterior projection digital subtraction aortogram) [Figure. 1]. A CASE REPORT VARIOUS VR : 8

6. 6 RCCA LSCA ARSA LCCA Trunk Truncbic A CASE REPORT ArcusAo Figure. 1 :Antero-posterior projection digital substraction aortogram demonstrating an ARSA arising from a common trunk between the subclavian arteries, and associated to a truncus bicaroticus. ArcusAo : Aorticarch, ARSA : aberrant right subclavian artery, LCCA : leftcommoncarotidartery, LSCA : left subclavian artery, RCCA : right cammoncarotidartery, Truncbic : truncus bicaroticus. VARIOUS : VR 8

7. The multi-detector row computed tomography (MDCT) confirmed the arteria lusoria, by showing its posterior course between the esophagus and spine, to perfuse the right upper extremity [Figure. 2] A CASE REPORT VARIOUS VR : 8

8. C A ARSA ARSA B T E ArcusAo A CASE REPORT Figure. 2 :Conrast-enhance MDCT showing arteria lusoria : Axial (A and B) and sagittal (C) images show aberrant right subclavian artery (ARSA) compressing esophagus (E) through a posterior course (black arow). ArcusAo : Aortic arch. E: esophagus, T : trachea VARIOUS : VR 8

9. A non operative treatment was chosen for this complex anomaly of the supra aortic vessels, based on its asymptomatic character. A CASE REPORT VARIOUS VR : 8

10. The proximal subclavian artery derives from a complex succession of vascular segments resulting from the transformation of the six primordial paired branchial arches (Figure. 3a) [4-5]. • The subclavian artery is normally derived from the remodeling of the right fourth branchial arch and the segment of the right dorsal aorta distal to this arch, as well as the sixth cervical inter segmental artery (Figure. 3b). DEVELOPMENTAL ANATOMY : VARIOUS VR : 8

11. The precise mechanisms responsible for this complex remodeling are poorly understood. • An arteria lusoria results from an interruption in this complex remodeling of the branchial arch system, typically of the right dorsal aorta distal to the sixth cervical inter segmental artery[5]. • The aberrant right subclavian artery is thus not connected to the ascending aorta or proximal aortic arch, but to the descending aorta through remnants of the right dorsal aorta (Figure. 3 c). DEVELOPMENTAL ANATOMY : VARIOUS VR : 8

12. Fig. 3 : Developmental anatomy underlying arteria lusoria; a :embryological development of the aortic arches according to Rathke’s representation [5-6]; b : normal adult situs; c : arteria lusoria situs. I: first branchial arch. II: second branchial arch. III: third branchial arch. IV: fourth branchial arch. V: fifth branchial arch. VI: sixth branchialarch. 5th: fifth cervical intersegmental artery. 6th: sixth cervical intersegmentalartery. AA: aortic arch. AS: aortic sinus. ARSA: aberrant right subclavianartery. DA: dorsal aorta DC: ductuscaroticus. ECA: external carotid artery. ICA: internal carotid artery. LSCA: left subclavian artery. LVA: left vertebral artery. RSCA: right subclavian artery. RVA: right vertebral artery. VPA: ventral pharyngeal artery. DEVELOPMENTAL ANATOMY : VARIOUS VR : 8

13. In the arteria lusoria configuration, four vessels arise sequentially from a left aortic arch: the right common carotid artery, the left common carotid artery, the left subclavian artery, and the aberrant right subclavian artery (Figure 1-2 ). • The latter arises from the proximal descending aorta, on the left side of the thorax and has to cross upwards and to the right either behind the esophagus (80–84%), between the esophagus and the trachea (12.7–15%), or in front of the trachea (4.2–5%) [5-7]. • In up to 60% of patients, the origin of this artery is wider than the rest of the thoracic subclavian artery, forming an infundibulum. This was first described by BurckhardF.Kommerellas an aortic diverticulum in the first clinically diagnosed arteria lusoria, giving rise to the term “ Kommerell’s diverticulum ” [5]. • The rest of the aortic arch is usually normal. • Cardiac anomalies may be also associated with arteria lusoria. ANATOMY : VARIOUSVR : 8

14. Arteria lusoria is usually asymptomatic, and is most often discovered during the course of evaluation of other mediastinal anomalies. • There are three settings in which an aberrant right subclavian artery becomes symptomatic : • when the esophagus and trachea are hemmed in between the lusorian artery dorsally and anteriorly by a truncus bicaroticus [8]; • from aberrant subclavian artery aneurysm; • with age, possibly from atherosclerotic hardening or fibro muscular dysplasia of arteries [5]. • When symptomatic, the ARSA most often produces dysphagia lusoria from esophageal compression, or dyspnea and chronic coughing from tracheal compression. • Other symptoms are much more rare and are signs of aneurysmal dilatation of the proximal lusorian artery, a lethal condition. CLINICAL PICTURE : VARIOUS VR : 8

15. CHEST RADIOGRAPHY – BARIUM STUDIES : • The diagnosis based primarily on findings at chest radiography in association with those at esophagography. • The lateral projection chest radiography can show the aberrant artery as a round, localized density continuous with the superior margin of the aortic arch. • The anteroposterior projection can demonstrate a density in the mediastinum ascending obliquely from the superior margin of the aortic arch[9]. • Arteria lusoria was first described radiologically by Kommerell on barium studies of the esophagus. It is characterized by an oblique defect about 5mm in width, on the posterior aspect of the esophagus, passing upwards from left to right just above the level of the aortic arch, with an abnormal degree of pulsation [5,10] (Figure. 4). IMAGING : VARIOUS VR : 8

16. CHEST RADIOGRAPHY – BARIUM STUDIES : c IMAGING : a b Fig. 4. Arteria lusoria: Small right posterior defect in oesophagus caused by the anomalous subclavian artery (white arrow); a :Postero-anterior view; b : Left anterior oblique; c : Lateral. [10] VARIOUS VR : 8

17. Multi-detector row computed tomography (MDCT) : • A non invasive imaging modality of choice in vascular anomaly visualization increasingly used. • MDCT Angiography allows the evaluation of the vascular structures and the lung parenchyma as well. • The fast data acquisition of the spiral CT allows acquisition of volumetric data sets during a single breath-hold. Furthermore, MDCTprovides nearly isotropic (4–8-channel scanners) [case report] or isotropic (16-channel scanners) spatial resolution [11]. IMAGING : VARIOUSVR : 8

18. Multi-detector row computed tomography (MDCT) : • Axial image presentation (Figure. 2 a-b) is often not suitable for demonstration purposes. The high spatial resolution of modern MDCT scanners, however, encourages the application of alternative image presentation techniques based on two dimensional (2D) image reconstruction (multi-planar reformation (MPR)) (Figure. 2c). IMAGING : VARIOUSVR : 8

19. Conventional catheter-based angiography : • Digital subtraction angiography (DSA) gives valuable information regarding arteria lusoria [Figure. 1]. • It is an invasive procedure and has the disadvantage in showing extravascular structures such as esophagus. • It has also been shown that the effective radiation doses in MDCT angiography studies are moderate and even lower in comparison with DSA in a comparable patient group [12]. IMAGING : VARIOUSVR : 8

20. Magnetic Resonance angiography (MRA) :( Figure. 5) • A non invasive imaging modality. • The most-suited technique currently used in the evaluation of thoracic aortic anatomy and disease is dynamic subtraction MRA, using Gadolinium as intravenous contrast agent [13,14]. IMAGING : b a Fig. 5. Aberrant right subclavian artery a :Coronal image showing the SCA (25’dA) originating from the isthmus of the aorta (7) and coursing to the right arm. Left common carotid artery (23g); b: Sagittal image: the aberrant artery crosses posterior to the trachea (T) whereas as the right common carotid artery (23d) is in front [14] VARIOUS VR : 8

21. Most patients with an aberrant right subclavian artery are asymptomatic and rarely warrant any treatment. • It is indicated for symptomatic relief of dysphagia lusoria, and also for prevention of complications due to aneurysmal dilatation of the lusorian artery. • Robert E. Gross performed the first successful surgical repair of arteria lusoria in 1946, ligating and sectioning the lusorian artery, eliminating esophageal compression and improving dysphagia [15]. • Many techniques for division of the ARSAand reconstitution of blood flow to the right arm have been described, with mortality ranging between 9% and 50% [16]. TREATMENT : VARIOUSVR : 8

22. Several reports describe individual cases of aberrant RSA aneurysms treated using covered stents deployed in the descending aorta over the origin of the left subclavian artery [17,18]. • Conservative treatment of aneurysmal aberrant right subclavian artery is associated with high mortality and morbidity rates, with 44 to 57% evolving towards rupture or fistulization, which was fatal in nearly all reported cases. Aggressive management should thus be proposed[5]. TREATMENT : VARIOUS VR : 8

23. Arteria lusoria is the most common aortic arch anomaly. • The diagnosis and differentiation of arch anomalies is based on findings at chest radiography in association with those at esophagography. • The vascular anatomy and the relationship with the surrounding structures may be demonstrated with echocardiography, CT or MR angiography, although the performance of these different imaging techniques at reaching the diagnosis has not been evaluated. Occasionally, angiography may be required. CONCLUSION : VARIOUSVR : 8

24. A symptomatic aberrant right subclavian artery can be safely repaired through minimally invasive surgery and endovascular techniques. • Aggressive treatment of an aneurysmal lusorian artery should be proposed, given the rapid natural evolution towards rupture and high mortality of this complication, despite high operative mortality associated with this elective procedure. CONCLUSION : VARIOUSVR : 8

25. Zapata H, Edwards JE, Titus JL. Aberrant right subclavian arterywithleftaorticarch: associatedcardiac anomalies. PediatrCardiol 1993;14(3):159–61. Hunauld PM. Examen de quelques parties d’un singe. HistAcad Roy Sci 1735;2:516–23. Bayford D. An account on a singular case of obstructed deglutition. Memoirs Med Soc London 1794;2:271–82. Congdon ED. Transformation of the aortic arch system during the development of the human embryo. Contributions Embryol 1922;14(68):47–110. Myers P.O, FaselJ.H.D, Kalangos A, Gailloud P, Arteria lusoria: Developmentalanatomy, clinical, radiological and surgical aspects. AnCard59 (2010) 147–154. Rathke H. Ueber die Entwickelung der Arterien, welche bei den Saugethierenvon den Bogen der Aorta ausgehen. Arch F Anat1843: 270–302. Holzapfel G. Ungewöhnlicher Ursprung und Verlauf der Arteria subclaviadextra. Anat Hefte 1899;12:369–523. van Son JA, Konstantinov IE, Kommerell BF. Kommerell’sdiverticulum. Tex HeartInst J 2002;29(2):109–12 BranscomJJ, Austin JH. Aberrant right subclavian artery. Findingsseen on plain chestroentgenograms. Am J Roentgenol Radium TherNuclMed 1973;119(3):539–42. Felson B, Cohen S, et al. Anomalous right subclavian artery. Radiology 1950;54(3):340–9. REFERENCES : VARIOUS VR : 8

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27. Objectives :Aberrant right subclavian artery or arteria lusoria, originating as the last vessel from the aortic arch, is one of the commonest anomalies of great vessels. We aim to provide a concise overview of the epidemiology, clinical presentation, imaging and management of arteria lusoria confronted with a patient with this anomaly. • Materials and methods : We describe findings in a patient, in whom angiographic investigation, for subarachnoid hemorrhage, incidentally revealed an arteria lusoria. ABSTRACT : VARIOUS VR : 8

28. Results :We report the case of a 34-year-old white man with a subarachnoid hemorrhage. The angiographic investigation showed a right aortic arch with an aberrant right subclavian artery in the anteroposterior projection digital subtraction aortogram. The Multi Director Computed Tomography (MDCT) angiography revealed an aberrant right subclavian artery wish originates from the aortic arch and crosses the midline between the spine and the esophagus to reach the right side. • Conclusion :Arteria lusoria is the mostcommonaorticarchanomaly. It canrarely cause dysphagia. Most patients remainasymptomatic and for thosewithsymptoms, choice of treatmentrequiresdeliberationbased on variousfactors. ABSTRACT : VARIOUS VR : 8