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The Clinical Spectrum of Kommerell’s Diverticulum in Adults with a Right-Sided Aortic Arch: A Case Series and Literature Overview
Background: Kommerell’s diverticulum is a rare vascular anomaly characterized as an outpouch at the onset of an aberrant subclavian artery. In the variant of a right-sided aortic arch, the trachea and esophagus are enclosed dorsally by the arch. In the configuration of an aberrant left subclavian ar...
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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MDPI
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7996811/ https://www.ncbi.nlm.nih.gov/pubmed/33652796 http://dx.doi.org/10.3390/jcdd8030025 |
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author | van Rosendael, Philippe J. Stöger, J. Lauran Kiès, Philippine Vliegen, Hubert W. Hazekamp, Mark G. Koolbergen, David R. Lamb, Hildo J. Jongbloed, Monique R. M. Egorova, Anastasia D. |
author_facet | van Rosendael, Philippe J. Stöger, J. Lauran Kiès, Philippine Vliegen, Hubert W. Hazekamp, Mark G. Koolbergen, David R. Lamb, Hildo J. Jongbloed, Monique R. M. Egorova, Anastasia D. |
author_sort | van Rosendael, Philippe J. |
collection | PubMed |
description | Background: Kommerell’s diverticulum is a rare vascular anomaly characterized as an outpouch at the onset of an aberrant subclavian artery. In the variant of a right-sided aortic arch, the trachea and esophagus are enclosed dorsally by the arch. In the configuration of an aberrant left subclavian artery, a Kommerell’s diverticulum and persisting ductus arteriosus or ductal ligament enclose the lateral side, forming a vascular ring which may result in (symptomatic) esophageal or tracheal compression. Spontaneous rupture of an aneurysmatic Kommerell’s diverticulum has also been reported. Due to the rarity of this condition and underreporting in the literature, the clinical implications of a Kommerell’s diverticulum are not well defined. Case summary: We describe seven consecutive adult patients with a right-sided aortic arch and an aberrant course of the left subclavian artery (arteria lusoria), and a Kommerell’s diverticulum, diagnosed in our tertiary hospital. One patient had severe symptoms related to the Kommerell’s diverticulum and underwent surgical repair. In total, two of the patients experienced mild non-limiting dyspnea complaints and in four patients the Kommerell’s diverticulum was incidentally documented on a computed tomography (CT) scan acquired for a different indication. The size of the Kommerell’s diverticulum ranged from 19 × 21 mm to 30 × 29 mm. In the six patients that did not undergo surgery, a strategy of periodic follow-up with structural imaging was pursued. No significant growth of the Kommerell’s diverticulum was observed and none of the patients experienced an acute aortic syndrome to date. Discussion: Kommerell’s diverticulum in the setting of a right-sided aortic arch with an aberrant left subclavian artery is frequently associated with tracheal and esophageal compression and this may result in a varying range of symptoms. Guidelines on management of Kommerell’s diverticulum are currently lacking. This case series and literature overview suggests that serial follow-up is warranted in adult patients with a Kommerell’s diverticulum with small dimensions and no symptoms, however, that surgical intervention should be considered when patients become symptomatic or when the diameter exceeds 30 mm in the absence of symptoms. |
format | Online Article Text |
id | pubmed-7996811 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-79968112021-03-27 The Clinical Spectrum of Kommerell’s Diverticulum in Adults with a Right-Sided Aortic Arch: A Case Series and Literature Overview van Rosendael, Philippe J. Stöger, J. Lauran Kiès, Philippine Vliegen, Hubert W. Hazekamp, Mark G. Koolbergen, David R. Lamb, Hildo J. Jongbloed, Monique R. M. Egorova, Anastasia D. J Cardiovasc Dev Dis Case Report Background: Kommerell’s diverticulum is a rare vascular anomaly characterized as an outpouch at the onset of an aberrant subclavian artery. In the variant of a right-sided aortic arch, the trachea and esophagus are enclosed dorsally by the arch. In the configuration of an aberrant left subclavian artery, a Kommerell’s diverticulum and persisting ductus arteriosus or ductal ligament enclose the lateral side, forming a vascular ring which may result in (symptomatic) esophageal or tracheal compression. Spontaneous rupture of an aneurysmatic Kommerell’s diverticulum has also been reported. Due to the rarity of this condition and underreporting in the literature, the clinical implications of a Kommerell’s diverticulum are not well defined. Case summary: We describe seven consecutive adult patients with a right-sided aortic arch and an aberrant course of the left subclavian artery (arteria lusoria), and a Kommerell’s diverticulum, diagnosed in our tertiary hospital. One patient had severe symptoms related to the Kommerell’s diverticulum and underwent surgical repair. In total, two of the patients experienced mild non-limiting dyspnea complaints and in four patients the Kommerell’s diverticulum was incidentally documented on a computed tomography (CT) scan acquired for a different indication. The size of the Kommerell’s diverticulum ranged from 19 × 21 mm to 30 × 29 mm. In the six patients that did not undergo surgery, a strategy of periodic follow-up with structural imaging was pursued. No significant growth of the Kommerell’s diverticulum was observed and none of the patients experienced an acute aortic syndrome to date. Discussion: Kommerell’s diverticulum in the setting of a right-sided aortic arch with an aberrant left subclavian artery is frequently associated with tracheal and esophageal compression and this may result in a varying range of symptoms. Guidelines on management of Kommerell’s diverticulum are currently lacking. This case series and literature overview suggests that serial follow-up is warranted in adult patients with a Kommerell’s diverticulum with small dimensions and no symptoms, however, that surgical intervention should be considered when patients become symptomatic or when the diameter exceeds 30 mm in the absence of symptoms. MDPI 2021-02-26 /pmc/articles/PMC7996811/ /pubmed/33652796 http://dx.doi.org/10.3390/jcdd8030025 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) ). |
spellingShingle | Case Report van Rosendael, Philippe J. Stöger, J. Lauran Kiès, Philippine Vliegen, Hubert W. Hazekamp, Mark G. Koolbergen, David R. Lamb, Hildo J. Jongbloed, Monique R. M. Egorova, Anastasia D. The Clinical Spectrum of Kommerell’s Diverticulum in Adults with a Right-Sided Aortic Arch: A Case Series and Literature Overview |
title | The Clinical Spectrum of Kommerell’s Diverticulum in Adults with a Right-Sided Aortic Arch: A Case Series and Literature Overview |
title_full | The Clinical Spectrum of Kommerell’s Diverticulum in Adults with a Right-Sided Aortic Arch: A Case Series and Literature Overview |
title_fullStr | The Clinical Spectrum of Kommerell’s Diverticulum in Adults with a Right-Sided Aortic Arch: A Case Series and Literature Overview |
title_full_unstemmed | The Clinical Spectrum of Kommerell’s Diverticulum in Adults with a Right-Sided Aortic Arch: A Case Series and Literature Overview |
title_short | The Clinical Spectrum of Kommerell’s Diverticulum in Adults with a Right-Sided Aortic Arch: A Case Series and Literature Overview |
title_sort | clinical spectrum of kommerell’s diverticulum in adults with a right-sided aortic arch: a case series and literature overview |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7996811/ https://www.ncbi.nlm.nih.gov/pubmed/33652796 http://dx.doi.org/10.3390/jcdd8030025 |
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