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...
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doaj-347be1f97e8f48d7b2733e98391a5f0a2021-02-27T00:04:35ZengMDPI AGJournal of Cardiovascular Development and Disease2308-34252021-02-018252510.3390/jcdd8030025The Clinical Spectrum of Kommerell’s Diverticulum in Adults with a Right-Sided Aortic Arch: A Case Series and Literature OverviewPhilippe J. van Rosendael0J. Lauran Stöger1Philippine Kiès2Hubert W. Vliegen3Mark G. Hazekamp4David R. Koolbergen5Hildo J. Lamb6Monique R. M. Jongbloed7Anastasia D. Egorova8Center for Congenital Heart Disease Amsterdam-Leiden (CAHAL), Leiden University Medical Center, 2333 ZA Leiden, The NetherlandsDepartment of Radiology, Leiden University Medical Center, 2333 ZA Leiden, The NetherlandsCenter for Congenital Heart Disease Amsterdam-Leiden (CAHAL), Leiden University Medical Center, 2333 ZA Leiden, The NetherlandsCenter for Congenital Heart Disease Amsterdam-Leiden (CAHAL), Leiden University Medical Center, 2333 ZA Leiden, The NetherlandsCenter for Congenital Heart Disease Amsterdam-Leiden (CAHAL), Leiden University Medical Center, 2333 ZA Leiden, The NetherlandsCenter for Congenital Heart Disease Amsterdam-Leiden (CAHAL), Leiden University Medical Center, 2333 ZA Leiden, The NetherlandsDepartment of Radiology, Leiden University Medical Center, 2333 ZA Leiden, The NetherlandsCenter for Congenital Heart Disease Amsterdam-Leiden (CAHAL), Leiden University Medical Center, 2333 ZA Leiden, The NetherlandsCenter for Congenital Heart Disease Amsterdam-Leiden (CAHAL), Leiden University Medical Center, 2333 ZA Leiden, The NetherlandsBackground: 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.https://www.mdpi.com/2308-3425/8/3/25Kommerell’s diverticulumright sided aortic archanomalous left subclavian arteryarteria lusoriatracheal compressionesophageal compression |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Philippe J. van Rosendael J. Lauran Stöger Philippine Kiès Hubert W. Vliegen Mark G. Hazekamp David R. Koolbergen Hildo J. Lamb Monique R. M. Jongbloed Anastasia D. Egorova |
spellingShingle |
Philippe J. van Rosendael J. Lauran Stöger Philippine Kiès Hubert W. Vliegen Mark G. Hazekamp David R. Koolbergen Hildo J. Lamb Monique R. M. Jongbloed Anastasia D. Egorova The Clinical Spectrum of Kommerell’s Diverticulum in Adults with a Right-Sided Aortic Arch: A Case Series and Literature Overview Journal of Cardiovascular Development and Disease Kommerell’s diverticulum right sided aortic arch anomalous left subclavian artery arteria lusoria tracheal compression esophageal compression |
author_facet |
Philippe J. van Rosendael J. Lauran Stöger Philippine Kiès Hubert W. Vliegen Mark G. Hazekamp David R. Koolbergen Hildo J. Lamb Monique R. M. Jongbloed Anastasia D. Egorova |
author_sort |
Philippe J. van Rosendael |
title |
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_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_sort |
clinical spectrum of kommerell’s diverticulum in adults with a right-sided aortic arch: a case series and literature overview |
publisher |
MDPI AG |
series |
Journal of Cardiovascular Development and Disease |
issn |
2308-3425 |
publishDate |
2021-02-01 |
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. |
topic |
Kommerell’s diverticulum right sided aortic arch anomalous left subclavian artery arteria lusoria tracheal compression esophageal compression |
url |
https://www.mdpi.com/2308-3425/8/3/25 |
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