ARTERIA LUSORIA : A CASE REPORT

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ARTERIA LUSORIA : A CASE REPORT
M. BOUSSALAH, N. TOUIL, S. HABCHAOUI, O. KACIMI, N. CHIKHAOUI
Emergency Radiology Department, Ibn Roch University Hospital, Casablanca, Morroco
VARIOUS : VR 8
INTRODUCTION :
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].
VARIOUS VR : 8
INTRODUCTION :
3
• With the advent and widespread use of precise noninvasive 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.
VARIOUS VR : 8
4
MATERIELS AND METHODS :
We describe findings in a patient, in whom
angiographic
investigation,
for
subarachnoid
hemorrhage, incidentally revealed a complex anomaly
of supra aortic vessels : an arteria lusoria arising from
a common trunk between the subclavian arteries
associated to a truncus bicaroticus.
VARIOUS VR : 8
5
A CASE REPORT
• Mr L. M
• Thirty-four years-old, white man,
• Without medical history of dysphagia, dyspnea or
coughing,
• Admitted for subarachnoid hemorrhage,
• 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].
VARIOUS VR : 8
A CASE REPORT
6
RCCA
LSCA
ARSA
LCCA
Trunk
Trunc bic
Arcus Ao
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.
Arcus Ao : Aortic arch, ARSA : aberrant right subclavian artery, LCCA : left common carotid artery, LSCA : left subclavian artery, RCCA :
right cammon carotid artery, Trunc bic : truncus bicaroticus.
VARIOUS : VR 8
7
A CASE REPORT
• 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]
VARIOUS VR : 8
8
A CASE REPORT
C
A
ARSA
ARSA
B
T
E
Arcus Ao
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). Arcus Ao : Aortic arch. E: esophagus, T : trachea
VARIOUS : VR 8
9
A CASE REPORT
• A non operative treatment was chosen for this
complex anomaly of the supra aortic vessels, based
on its asymptomatic character.
VARIOUS VR : 8
DEVELOPMENTAL ANATOMY :
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).
VARIOUS VR : 8
DEVELOPMENTAL ANATOMY :
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).
VARIOUS VR : 8
12
DEVELOPMENTAL ANATOMY :
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 branchial arch. 5th: fifth cervical intersegmental artery. 6th: sixth cervical
intersegmental artery.
AA: aortic arch. AS: aortic sinus. ARSA: aberrant right subclavian artery. DA: dorsal aorta DC: ductus caroticus. 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.
VARIOUS VR : 8
ANATOMY :
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
Burckhard F.Kommerell as 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.
VARIOUS VR : 8
CLINICAL PICTURE :
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.
VARIOUS VR : 8
IMAGING :
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).
VARIOUS VR : 8
IMAGING :
 CHEST RADIOGRAPHY – BARIUM STUDIES :
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
16
IMAGING :
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, MDCT provides nearly isotropic (4–8-channel
scanners) [case report] or isotropic (16-channel scanners)
spatial resolution [11].
VARIOUS VR : 8
IMAGING :
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 (multiplanar reformation (MPR)) (Figure. 2c).
VARIOUS VR : 8
IMAGING :
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].
VARIOUS VR : 8
IMAGING :
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].
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
TREATMENT :
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 ARSA and reconstitution
of blood flow to the right arm have been described, with
mortality ranging between 9% and 50% [16].
VARIOUS VR : 8
TREATMENT :
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].
VARIOUS VR : 8
23
CONCLUSION :
• 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.
VARIOUS VR : 8
24
CONCLUSION :
• 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.
VARIOUS VR : 8
REFERENCES :
25
1. Zapata H, Edwards JE, Titus JL. Aberrant right subclavian artery with left aortic
arch: associated cardiac anomalies. Pediatr Cardiol 1993;14(3):159–61.
2. Hunauld PM. Examen de quelques parties d’un singe. Hist Acad Roy Sci
1735;2:516–23.
3. Bayford D. An account on a singular case of obstructed deglutition. Memoirs Med
Soc London 1794;2:271–82.
4. Congdon ED. Transformation of the aortic arch system during the development of
the human embryo. Contributions Embryol 1922;14(68):47–110.
5. Myers P.O, Fasel J.H.D, Kalangos A, Gailloud P, Arteria lusoria: Developmental
anatomy, clinical, radiological and surgical aspects. AnCard 59 (2010) 147–154.
6. Rathke H. Ueber die Entwickelung der Arterien, welche bei den Saugethieren von
den Bogen der Aorta ausgehen. Arch F Anat 1843: 270–302.
7. Holzapfel G. Ungewöhnlicher Ursprung und Verlauf der Arteria subclavia dextra.
Anat Hefte 1899;12:369–523.
8. van Son JA, Konstantinov IE, Kommerell BF. Kommerell’s diverticulum. Tex Heart
Inst J 2002;29(2):109–12
9. Branscom JJ, Austin JH. Aberrant right subclavian artery. Findings seen on plain
chest roentgenograms. Am J Roentgenol Radium Ther Nucl Med
1973;119(3):539–42.
10. Felson B, Cohen S, et al. Anomalous right subclavian artery. Radiology
1950;54(3):340–9.
VARIOUS VR : 8
REFERENCES :
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11. Schertler T, Wildermuth S, Teodorovic N, Mayer d, Marincek B, Boehm T, Visualization
of congenital thoracic vascular anomalies using multi-detector row computed
tomography and two- and three-dimensional post-processing, European Journal of
Radiology 61 (2007) 97–119
12. Alper F, Akgun M, Kantarci M, Eroglu A, Ceyhan E, Onbas O, Duran C, Okur A,
Demonstration of vascular abnormalities compressing esophagus by MDCT: Special
focus on dysphagia lusoria. EJR 2006; 59: 82-87.
13. Van den Berg Jos C, Imaging of the thoracic aorta , Presse Med. 2011; 40: e391–e412
14. Kastler B, Livolsi A, Germain P, Bernard Y, Michalakis D, Rodiere E, Louis G, Litzler JF,
Vignaux O, Apport de l’IRM dans l’exploration des anomalies cardiaques congénitales
et des gros vaisseaux. J Radiol 2004; 85: 1821- 1850.
15. Gross RE. Surgical treatment for dysphagia lusoria. Ann Surg 1946;124:532–4.
16. Von Saegesser L, Faidutti B. Symptomatic aberrant retroesophageal subclavian artery:
considerations about the surgical approach, management and results. Thorac
Cardiovasc Surg 1984;32: 307-10.
17. Kopp R, Wizgall I, Kreuzer E, Meimarakis G, Weidenhagen R, Kuhnl A, et al. Surgical
and endovascular treatment of symptomatic aberrant right subclavian artery (arteria
lusoria). Vascular 2007;15:84-91.
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VARIOUS VR : 8
ABSTRACT :
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.
VARIOUS VR : 8
ABSTRACT :
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 most common aortic
arch anomaly. It can rarely cause dysphagia. Most
patients remain asymptomatic and for those with
symptoms, choice of treatment requires deliberation
based on various factors.
VARIOUS VR : 8
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