Abstract
Neuhauser syndrome is a rare vascular anomaly characterized by the esophagus and trachea circling via the ligamentum arteriosum and right aortic arch. Kommerell’s diverticulum have the same characteristic by an outpouch at the onset of an aberrant left subclavian artery worsening this compression. The association between these pathologies is very rare and the operative strategy is unclear. We describe a case with the association with a computed tomography scanner aortic reconstruction and a repair’s operative strategy.
Keywords: Neuhauser syndrome, Kommerell’s diverticulum, Retro-tracheal left subclavian artery pinching
A 25-year-old woman presented with exertional inspiratory dyspnea. Her history included surgical ligamentum arteriosum section through left thoracotomy at 11 years of age. Contrast-enhanced computed tomography (CT) scan with three-dimensional reconstruction suggested a Neuhauser anomaly [1], Kommerell’s diverticulum [2] (arrow in Fig. 1A), and a retro-tracheal left subclavian artery pinching of both the trachea (arrow in Fig. 1B) and esophagus. Fiberoptic bronchoscopy revealed an extrinsic compression of the lower third of the trachea with no sign of tracheomalacia (arrow in Fig. 2).
Fig. 1.
A, B CT scan aortic reconstruction before surgery
Fig. 2.
Fiberoptic bronchoscopy showing extrinsic compression
Surgery was carried out by median sternotomy, as the Kommerell’s diverticulum is difficult to access through either right or left thoracotomy. Under cardiopulmonary bypass, a complete transection of the aorta was performed in order to control the Kommerell’s diverticulum, under moderately hypothermic (26 °C) circulatory arrest and antegrade selective cerebral perfusion. Hence, Kommerell’s diverticulum was excluded by Blalock’s suture at its base. Then, the left subclavian artery was directly anastomosed on the left common carotid artery and the aortic continuity was reestablished.
A month after surgery, CT angiogram showed absence of pinch appearance on the trachea (arrow in Fig. 3A), no aortic aneurysm, and the left subclavian artery anastomosed on the left common carotid artery (arrow in Fig. 3B).
Fig. 3.
A, B CT scan aortic reconstruction after surgery and left subclavian reimplantation
Funding
None.
Declarations
Ethical approval was waived by the local Ethics Committee of University A in view of the retrospective nature of the study and all the procedures being performed were part of the routine care. Human right was respected. We do not want our data to be used for commercial purposes.
Informed consent
Obtained.
Conflict of interest
The authors declare no competing interests.
Footnotes
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References
- 1.Neuhauser Eb. The roentgen diagnosis of double aortic arch and other anomalies of the great vessels. Am J Roentgenol Radium Ther Nucl Med. 1946;56:1. [PubMed]
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