Abstract
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.
Highlights
From Embryology to Clinical PresentationA Kommerell’s diverticulum is a rare vascular anomaly and is characterized by an aneurysmatic onset of an aberrant left or right subclavian artery [1]
During development of a normal left aortic arch (Figure 1B), the right alpha-segment regresses [10]
The right 4th pharyngeal arch arteries (PAA) shows an abnormal regression, this connection cannot be established resulting in an aberrant, more distal origin of the right subclavian artery from the descending aorta, via a persisting right alpha-segment [3] (Figure 1C)
Summary
Due to its incidental nature and presumed underreporting in the literature, the natural history and exact clinical consequences of Kommerell’s diverticulum are unknown. Kim and colleagues recommend surgical resection of a Kommerell’s diverticulum even in asymptomatic patients and reported the retrospective results of 19 adult patients who underwent surgical correction independent of the size of the Kommerell’s diverticulum, that ranged from 15–45 mm [16] In this series, the surgical procedure was complicated by an perioperative type A dissection (5.3%), laryngeal and phrenic nerve injury (10.5%), and transient neurologic dysfunction (5.3%). The configuration of a Kommerell’s diverticulum from an aberrant left subclavian artery from a right-sided aorta is often associated with some degree of tracheal and esophageal compression, and this may result in a range of associated symptoms.
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