Vascular rings, including double aortic arch and right aortic arch with aberrant left subclavian and left ligamentum, are part of a larger group of vascular-related aerodigestive compression syndromes that also includes innominate artery compression syndrome, dysphagia lusoria, aortic arch anomalies, and aneurysms of either the aorta or pulmonary artery. Additionally, post-surgical airway compression is a distinct entity in itself. The approach to the diagnosis and management of these varied phenomena has been streamlined by the multidisciplinary team at Boston Children\'s Hospital. Echocardiography, computed tomographic angiography, esophagram, and three-phase dynamic bronchoscopy are routinely performed in these patients in order to produce a comprehensive understanding of the unique anatomic challenges that each patient presents. Adjunctive diagnostic techniques include modified barium swallow, routine preoperative and postoperative screening of the vocal cords, and radiographic identification of the artery of Adamkiewicz. Beyond the vascular reconstruction, which ranges from subclavian-to-carotid transposition to descending aortic translocation, we liberally apply tracheobronchopexy and rotational esophagoplasty to relieve respiratory and esophageal symptoms. Due to the heightened risk for recurrent laryngeal nerve injury, intraoperative recurrent laryngeal nerve monitoring has become routine in these cases. The comprehensive care of these patients requires the coordinated efforts of a large team of dedicated personnel in order to achieve the optimal result.

OBJECTIVE: Vascular rings may cause tracheal and/or oesophageal compression. For many patients, symptoms/signs have been present for a long period before diagnosis. However, in the era of prenatal diagnosis, some units advocate universal early surgery. The risks and efficacy of surgery must be known to adequately counsel for the operation. This meta-analysis sought to define the morbidity and mortality associated with surgical correction, and persistent post-operative symptoms.
METHODS: PubMed, Cochrane Library and CINAHL databases were searched for studies that described the outcome of patients undergoing surgery for a double or right aortic arch (DAA or RAA). Non-comparative and random effects model-based meta-analyses were conducted to calculate the pooled rates of mortality, surgical complications, reintervention, and persistent follow-up symptoms.
RESULTS: Nineteen eligible studies were included comprising 18 studies describing outcomes for DAA surgery and 15 for RAA surgery. For DAA surgery, overall mortality rate was 0% [95% confidence interval (CI) 0.0-1.0], post-surgical complication rate 18% [95% CI: 12.0-23.0], prevalence of reintervention 3% [95% CI: 1.0-5.0] and prevalence of symptoms at last follow-up was 33% [95% CI: 17.0-52.0]. For RAA surgery, overall pooled mortality was 0% [95% CI: 0.0-0.0], prevalence of post-surgical complications was 15% [95% CI: 8.0-23.0], reintervention rate was 2% [95% CI: 0.0-4.0], prevalence of symptoms at last follow-up was 40% [95% CI: 26.0-55.0].
CONCLUSIONS: While surgery to correct a vascular ring is safe, the rate of persistent symptoms is high and further strategies must be sought to reduce this burden.

Abnormalities in position and/or branching of the aortic arch can lead to vascular rings that may cause narrowing of the tracheal lumen due to external compression, or constriction of the oesophagus, causing symptoms that vary in relation to the anatomical vascular pattern and the relationship between these structures. Respiratory morbidity related to external airways compression is a major concern in children affected by vascular rings. Clinical presentation depends on the severity of the tracheal lumen reduction and the presence of associated tracheomalacia. Recurrent respiratory infections, wheezing, atelectasis, and hyperinflation are mostly reported. As they are nonspecific and therefore difficult to recognize, attention should be given to all children with history of respiratory distress, extubation failure, noisy breathing, and recurrent respiratory infections. Early diagnosis and referral to specialized centres can prevent the long-term complications and improve the respiratory outcomes of these patients.

Thoracic vascular malformations are a set of complex congenital abnormalities that result in major alterations of the patient\'s anatomy. It can be discovered incidentally or the anomalies may generate heart failure with a right-to-left shunt, dysphagia, dyspnea, stridor, cough, recurrent bronchopulmonary infections, hypoxemia, paradoxical emboli, and occasionally apneas and massive hemothorax. The knowledge of the embryology, and the normal development of the vascular structures of the thorax, allows to understand these abnormalities-and thus helps in making an accurate diagnosis-with its possible complications, symptoms, and treatments for the patient. The following is a review of the most common thoracic vascular malformations and their imaging findings.

This article reviews the physiology of the ductus arteriosus, the pathophysiology of the patent ductus arteriosus (PDA), and the role advanced imaging such as computed tomography (CT) and magnetic resonance imaging (MRI) can play in guiding diagnosis and percutaneous or surgical intervention. A PDA can have variable clinical and radiologic presentations and can be important to characterize in patients with vascular rings, aortic maldevelopment and congenital heart disease. An understanding of the PDA and the application of CT and MRI can allow the radiologist to provide key information to physicians who plan to close a PDA or maintain PDA patency in the setting of ductal-dependent congenital heart disease.

Vascular rings are congenital abnormalities of the aortic arch vascular system that compress the trachea and esophagus. A review of long-term outcomes suggests that chronic feeding difficulties can persist following surgical repair of vascular rings. Previous reports of postoperative vascular ring division outcomes indicate that chronic esophageal symptoms may persist following repair, though most available data focuses on persistent respiratory symptoms. It is therefore the aim of this article to summarize and organize recent evidence reporting the frequency, presentation, and management of feeding difficulties following vascular ring repair in pediatric patients. Pathophysiologic mechanisms for postoperative esophageal symptoms may include residual compression from an unresected diverticulum of Kommerell or delayed repair leading to chronic esophageal dysmotility despite correction of esophageal compression. Guidance on the management of feeding difficulties following vascular ring repair is limited. The authors describe success in one case with nasogastric tube feeding and interdisciplinary evaluation. Consensus regarding the management of feeding difficulty following vascular ring repair is needed.

Compression of the airway, esophagus or both by aortic and pulmonary vessels can be caused by a variety of anatomical situations. Vascular rings are the most commonly encountered entity; however, compression can also occur from less common anomalies such as a left pulmonary artery sling or innominate artery compression. Vascular rings and other vascular compression abnormalities can be challenging to visualize and image and often require advanced imaging by CT or MRI to better understand the cause and extent of compression. Atretic vascular structures, such as the ligamentum arteriosum or atretic arch, play a key role in creating a vascular ring and do not enhance with contrast agent in a typical fashion. Despite these imaging challenges, classic and useful signs can indicate the presence or absence of a vascular ring or compression.

BACKGROUND: Double aortic arch (DAA) is an extremely rare vascular malformation, even more so when coexisting with esophageal cancer.
METHODS: We report a new case of DAA with esophageal cancer recently seen at our Thoracic Tumor Clinic and review cases of DAA coexisting with esophageal cancer reported in the literature of English language from 2010 to 2020. The purposes of our literature review were to explore how to best achieve radical esophagectomy while reducing postoperative complications. The clinical manifestations, diagnostic method, surgical approach, reconstruction route, and the extent of lymphadenectomy of esophageal cancer with DAA were analyzed in detail.
CONCLUSIONS: For such patients, 3D computed tomography is necessary for preoperative diagnosis. The surgical approach should consider factors such as the location of the tumor in the esophagus and whether the tumor is surrounded by DAA, as well as the position of the descending aorta and the requirements for the surgical field for lymphadenectomy. If esophageal reconstruction is required, the retrosternal route is preferred. We recommend that only patients with positive results of intraoperative frozen biopsy of recurrent laryngeal nerve lymph nodes should undergo three-field lymphadenectomy, which may be the best method to achieve radical esophagectomy for middle and lower esophageal cancers with DAA while minimizing postoperative complications.

This review aims at presenting and summarizing the current state of literature on the presentation and surgical management of a right-sided aortic arch with a left-sided ligamentum forming a complete vascular ring around the oesophagus and trachea.
A systematic database search for appropriate literature was conducted on PubMed/MEDLINE. Articles were considered relevant when providing details on the presentation, diagnosis and surgical treatment of this specific congenital arch anomaly in human beings.
Affected patients present with respiratory and/or oesophageal difficulties due to tracheoesophageal compression. Conservative treatment might be reasonable in asymptomatic or mildly symptomatic cases; however, once moderate-to-severe symptoms develop, surgical intervention is definitely indicated. Surgery is commonly performed through a left thoracotomy or median sternotomy and includes the division of the left ductal ligamentum; if a Kommerell\'s diverticulum is present that is >1.5 times the diameter of the subclavian artery, then concomitant resection of the large diverticulum and translocation of the aberrant left subclavian artery is also conducted. Postoperative morbidity and mortality are low and are rather related to concomitant intracardiac and extracardiac anomalies than to the procedure itself. In a majority of patients, full resolution of symptoms is seen within months to years from the surgery. Nevertheless, there is also a subset of patients who remain with some tracheobronchial narrowing, sometimes even requiring reintervention during follow-up due to persisting or recurring symptoms.
Overall, the surgical management of a right aortic arch forming a true vascular ring in infancy, childhood and adulthood seems relatively safe and effective in providing symptomatic relief despite some persistent tracheobronchial and/or oesophageal narrowing in some cases.

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