Congenital vascular ring
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REVIEW ARTICLE
Congenital vascular ring Naoki Yoshimura1 · Kazuaki Fukahara1 · Akio Yamashita1 · Toshio Doi1 · Shigeyuki Yamashita1 · Takahiro Homma1 · Shigeki Yokoyama1 · Masaya Aoki1 · Akihiko Higashida1 · Yoshifumi Shimada1 · Daisuke Toritsuka1 · Kanetsugu Nagao1 · Yuki Ikeno2 Received: 1 August 2019 / Accepted: 13 September 2019 © Springer Nature Singapore Pte Ltd. 2019
Abstract A vascular ring is a rare congenital cardiovascular anomaly, which encircles and compresses the trachea or esophagus, or both. In this review we discuss the pathophysiology, theoretical embryopathogenesis, diagnostic modalities, and surgical treatment of the different types of vascular ring. Knowledge of the normal embryonic development of the aortic arch and related structures is important for understanding and classifying the various forms of vascular ring. The development of a vascular ring begins with the embryonic aortic arch system. The persistence, involution, or regression of the arches determines the multiple variations of vascular ring. With the development of new technologies, multi-detector computed tomography (MDCT) has become a good diagnostic modality for pre- and postoperative evaluation. MDCT provides an excellent image of aortic arch abnormalities and the related anatomy, as well as the tracheal pathology. For patients with symptoms, surgical division of the vascular ring usually achieves excellent outcomes with marked resolution of symptoms and a low risk of morbidity and mortality. Symptomatic vascular rings require early surgical intervention to prevent prolonged vascular compression of the airway and serious complications. Keywords Vascular ring · Double aortic arch · Tracheoesophageal compression · Multidetector computed tomography · Thoracoscopic surgery
Introduction A vascular ring is a congenital anomaly in which the aortic arch and its branches completely or incompletely, encircle and compress the trachea or esophagus, or both [1]. It is a rare malformation, accounting for only about 1% of congenital cardiovascular anomalies. A vascular ring may be asymptomatic or cause respiratory and/or gastrointestinal symptoms, depending on the degree of tightness of the ring and on subsequent tracheoesophageal compression [2, 3]. Frequent symptoms include noisy breathing and a barking This review was submitted at the invitation of the editorial committee. * Naoki Yoshimura [email protected]‑toyama.ac.jp 1
First Department of Surgery, University of Toyama, Graduate School of Medicine, 2630 Sugitani, Toyama 930‑0194, Japan
Department of Cardiothoracic Surgery, University of Colorado, Aurora, CO, USA
2
cough, recurrent upper respiratory infections, wheezing, exertional dyspnea, and dysphagia. Patients with severe compression suffer respiratory distress requiring intubation. Dysphagia is not usually a symptom until the child is old enough to be introduced to solid food [2, 3]. Better diagnostic modalities, especially multi-detector computed tomography (MDCT), can define the exact anatomy of an aortic arch malformatio
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