CASE 4319 Published on 13.12.2005
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Incidentally detection of a right-sided aortic arch with an aberrant left sided aberrant subclavian artery originating from a Kommerell’s diverticulum

Section

Cardiovascular

Case Type

Clinical Cases

Authors

Lothar Albrecht, Sophie Pabela, Soenke Langner, Andreas Hoehnea, Claus-Peter Froehlich, Jens-Peter Kuehn, Ulrich Silla, Norbert Hosten aKlinik und Poliklinik für Chirurgie der Ernst-Moritz-Arndt-Universität Greifswald, Abteilung für Allgemein-, Viszeral-, Thorax- und Gefäßchirurgie, Friedrich-Loeffler-Str. 23b, 17475 Greifswald

Connected authors
Patient

40 years, male

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Clinical History
A 40 year old men with a gastric ulcer perforation was incidentally diagnosed to have a right-sided aortic arch with an aberrant left subclavian artery (ALSA) and diverticulum of Kommerell (DOK). This development disorder and its general treatment are discussed.
Imaging Findings
A man 40 years of age presented with acute upper upper abdominal pain in our surgical emergency department. A perforated gastric ulcer was diagnosed and surgery performed successfully. During the clinical course he developed fever (up to 39° C) and leucocytosis. A CT Scan of the body including lungs with a Siemens Sensation 16 Scanner (1,5 mm collimation, 6 mm reconstruction, table feed 30 mm/s; 100 ml Accupaque 350 iv, Flow 3.0 ml/s) could exclude septic foci. Incidentally, a development disorder of the Aorta was detected as descriced here: There is no aortic knob visible on chest x-ray (Figure 1). Axial images read a right sided aortic arch with a Kommerell’s diverticulum and a retroesophageal aberrant left subclavian artery (Figure 2). MIP and VRT show furthermore a right- sided descending aorta and several projections of the diverticulum (Figure 3, 4 and 5). When our patient is scheduled for dismission from our hospital a further cardiosurgical consultation is planned that will discuss necessity of treatment. As far as we know he has not been suffering from any symptoms by the diverticulum.
Discussion
Aberrant left subclavian artery (ALSA) are rare and generally associated with a right-sided aorta. Coincidence with a diverticulum of Kommerell (DOK) is often found (1). Right-sided aortic arches occur approximately in 0.1% of patients. [2,3]. Only 50 to 70% present with an associated ALSA (1, 4, 5). Not all of them originate from a DOK (1). If they do, the left subclavian artery arises from the DOK at the junction of the right aortic arch and the right descending aorta and passes behind the esophagus towards the left arm. The diverticulum is a broad saccular aneurysmal dilation because the fetal ductus arteriosus, at the origin of the aberrant left subclavian artery, carries a large volume of blood (6). In a series of 2418 angiograms 22 cases had aberrant subclavian arteries (0,91%) and only three of them were ALSAs, originating from a DOK (0,12%). One of the latter was complicated by an aneurysm (7). Aneurysms are more often reported in aberrant right subclavian arteries (ARSA) with DOKs (8, 9). Coincidence with cardiac development disorders as VSD, ASD or Fallot’s Tetralogy is observed in 10% and usually does not require any specific treatment (10). Kommerell’s diverticulum with ALSAs can be asymptomtic (95%) (8). If they are symptomatic dysphagia is the most common presentation by esophageal compression (2, 11). Respiratory disorders can arise as well if the trachea is affected (3, 12). If just a mild dysphagia lusoria is the leading symptom this can satisfactorily be managed by dietary modification for a certain time (3). But once diagnosed, the diverticulum should be surgically removed regardless of size because risk of rupture is likely. Rupture occurred in 6 of 20 patients (30%) with a Kommerell’s diverticulum (two of them smaller than 5 cm in diameter) and an aberrant subclavian artery and was fatal despite of emergency surgery for repair (13). Mortality in these cases is described as 100%. Thus DOK means a fatal congenital vascular anomaly(13, 14).
Differential Diagnosis List
Aberrant left sided subclavian artery originating from a Komerrells diverticulum.
Final Diagnosis
Aberrant left sided subclavian artery originating from a Komerrells diverticulum.
Case information
URL: https://www.eurorad.org/case/4319
DOI: 10.1594/EURORAD/CASE.4319
ISSN: 1563-4086
Figure 1

Plain chest X-ray.

Plain chest X-ray.
Pa projection: There\\\'s no aortic knob visible in typical location. Upper mediastinum is prominent on the right side.
Figure 2

Axial 6 mm CT scan: A right-sided aortic arch with a diverticulum.

Axial 6 mm CT scan: A right-sided aortic arch with a diverticulum.
Arrow shows diverticulum.
Figure 3

Coronal 5 mm MIP: An abberrant left subclavian artery originating from the diverticulum.

Coronal 5 mm MIP: An abberrant left subclavian artery originating from the diverticulum.
Arrow shows diverticulum.
Figure 4

Oblique coloured VRT with Bone:

Oblique coloured VRT with Bone:
Location of descending aorta right sided to thoracic vertebras.