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Seminars in Pediatric Surgery Dec 2021Vascular rings are congenital aortic arch anomalies that lead to compression of the trachea or esophagus. The goal of this review is to summarize our current... (Review)
Review
Vascular rings are congenital aortic arch anomalies that lead to compression of the trachea or esophagus. The goal of this review is to summarize our current recommendations for the management of patients with a diagnosis of a vascular ring. We review the history, classification methods, and epidemiology of the various types of vascular rings. We then propose a management strategy for the relatively new paradigm of fetal diagnosis, including the management of asymptomatic vascular rings. Finally, we finish with a review of the operative techniques and outcomes for the four main categories of vascular rings.
Topics: Aorta, Thoracic; Esophagus; Heart Defects, Congenital; Humans; Subclavian Artery; Vascular Ring
PubMed: 34930596
DOI: 10.1016/j.sempedsurg.2021.151128 -
Surgery Today Oct 2020A vascular ring is a rare congenital cardiovascular anomaly, which encircles and compresses the trachea or esophagus, or both. In this review we discuss the... (Review)
Review
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.
Topics: Aorta, Thoracic; Deglutition Disorders; Dyspnea; Humans; Multidetector Computed Tomography; Vascular Ring
PubMed: 31676999
DOI: 10.1007/s00595-019-01907-5 -
Seminars in Pediatric Surgery Jun 2016The term vascular ring refers to congenital vascular anomalies of the aortic arch system that compress the esophagus and trachea, causing symptoms related to those two... (Review)
Review
The term vascular ring refers to congenital vascular anomalies of the aortic arch system that compress the esophagus and trachea, causing symptoms related to those two structures. The most common vascular rings are double aortic arch and right aortic arch with left ligamentum. Pulmonary artery sling is rare and these patients need to be carefully evaluated for frequently associated tracheal stenosis. Another cause of tracheal compression occurring only in infants is the innominate artery compression syndrome. In the current era, the diagnosis of a vascular ring is best established by CT imaging that can accurately delineate the anatomy of the vascular ring and associated tracheal pathology. For patients with a right aortic arch there recently has been an increased recognition of a structure called a Kommerell diverticulum which may require resection and transfer of the left subclavian artery to the left carotid artery. A very rare vascular ring is the circumflex aorta that is now treated with the aortic uncrossing operation. Patients with vascular rings should all have an echocardiogram because of the incidence of associated congenital heart disease. We also recommend bronchoscopy to assess for additional tracheal pathology and provide an assessment of the degree of tracheomalacia and bronchomalacia. The outcomes of surgical intervention are excellent and most patients have complete resolution of symptoms over a period of time.
Topics: Aorta, Thoracic; Aortic Diseases; Brachiocephalic Trunk; Bronchoscopy; Child; Echocardiography; Esophageal Stenosis; Humans; Infant; Pulmonary Artery; Subclavian Artery; Tomography, X-Ray Computed; Tracheal Stenosis; Vascular Malformations; Vascular Surgical Procedures
PubMed: 27301603
DOI: 10.1053/j.sempedsurg.2016.02.009 -
Otolaryngologic Clinics of North America Apr 2024Swallowing is an elaborate process that requires neuromuscular coordination. Pediatric esophageal dysphagia is broadly categorized into structural and nonstructural... (Review)
Review
Swallowing is an elaborate process that requires neuromuscular coordination. Pediatric esophageal dysphagia is broadly categorized into structural and nonstructural causes. The structural causes of pediatric esophageal dysphagia are related to processes that narrow the lumen of the esophagus. Esophageal strictures are the result of scar tissue formation within the lumen of the esophagus, leading to stenosis. Vascular rings and slings cause external compression of the esophagus. Diagnosis requires an esophagram and computed tomography or magnetic resonance imaging. Treatment is guided by the patient's symptoms and underlying diagnosis, although it often requires surgical intervention when symptomatic.
PubMed: 38575487
DOI: 10.1016/j.otc.2024.02.015 -
Pediatric Pulmonology May 2023Pulmonary artery sling is a rare childhood vascular tracheobronchial compression syndrome that is frequently associated with tracheal stenosis. Consequently, neonates or... (Review)
Review
Pulmonary artery sling is a rare childhood vascular tracheobronchial compression syndrome that is frequently associated with tracheal stenosis. Consequently, neonates or infants may present with critical airway obstruction if there is long segment airway narrowing and complete rings. Rapid diagnosis of this cardiac vascular malformation and evaluation of the extent and severity of airway involvement is essential to plan surgery, typically a slide tracheoplasty to relieve critical airway obstruction. Long term outcome can be excellent following surgical repair of the stenosed airway and reimplantation of the left pulmonary artery. In this review we focus on the embryology, diagnostic workup, airway investigations and management for this rare but challenging congenital condition.
Topics: Infant; Infant, Newborn; Humans; Child; Pulmonary Artery; Treatment Outcome; Retrospective Studies; Heart Defects, Congenital; Vascular Malformations; Airway Obstruction; Trachea
PubMed: 36790334
DOI: 10.1002/ppul.26345 -
The Journal of Thoracic and... Sep 2020
Topics: Aorta; Humans; Vascular Ring
PubMed: 32616348
DOI: 10.1016/j.jtcvs.2020.03.160 -
Asian Cardiovascular & Thoracic Annals Feb 2022Vascular rings are aortic arch anomalies with a spectrum of manifestations ranging from asymptomatic lesions detected incidentally to an acute presentation secondary to... (Review)
Review
BACKGROUND
Vascular rings are aortic arch anomalies with a spectrum of manifestations ranging from asymptomatic lesions detected incidentally to an acute presentation secondary to tracheo-esophageal compression. Circumflex retro-esophageal aortic arch is an extreme form of true vascular ring. It remains an uncharted territory to many surgeons.
METHODS
A comprehensive search of peer reviewed journals was completed based on the key words, "Circumflex aorta," "Circumflex retro-esophageal aorta" and "circumflex arch" using Google scholar, Scholars Portal Journals and PubMed. The reference section for each article found was searched to obtain additional articles. Literature on the circumflex aorta was reviewed starting from the embryogenesis to the latest management strategies.
RESULTS
Right circumflex aorta is more prevalent compared to left circumflex aorta. It can occur in isolation or in association with other intracardiac lesions. Mainly presents in children, however reported in adults too. The presentation may vary from asymptomatic lesion to acute respiratory distress secondary to airway compression. Computerized tomography (CT) and magnetic resonance imaging (MRI) are important tools in delineating the vascular anatomy. Aortic uncrossing is the definitive procedure. However, the role of concomitant tracheobronchopexy is emerging. Native tissue-to-tissue anastomosis is commonly preferred, but cases of extra-anatomic grafts are reported.
CONCLUSION
Circumflex aorta is amenable to complete repair. Preoperative delineation of anatomy is important for successful surgical outcome. Division of the retro-esophageal segment is crucial in relieving the compressive symptoms. In addition, tracheobronchopexy is helpful in addressing residual tracheomalacia but this accounts for a high-risk surgery.
Topics: Adult; Aorta; Aorta, Thoracic; Child; Heart Defects, Congenital; Humans; Trachea; Treatment Outcome; Vascular Ring
PubMed: 33957784
DOI: 10.1177/02184923211015092 -
Archives of Disease in Childhood.... Aug 2021A 12-year-old girl was referred with a 7-month history of episodes of dyspnoea, stridor and a sense of chest constriction during physical exercise. These were...
A 12-year-old girl was referred with a 7-month history of episodes of dyspnoea, stridor and a sense of chest constriction during physical exercise. These were self-limiting and never occurred at night. Physical examination was normal. An initial diagnosis of vocal cord dysfunction was made.Spirometry showed a plateau of forced expiratory flow, with a truncated aspect of the expiratory phase (figure 1). Six weeks later she described occasional dysphagia with solid foods. The barium swallow, performed only in anteroposterior projection, did not show oesophageal dilation. A bronchoscopy showed extrinsic compression of the middle lower third of the trachea and the right main bronchus. A chest CT scan was performed (figures 2 and 3).
Topics: Airway Obstruction; Child; Exercise; Female; Humans; Respiratory Sounds; Spirometry; Subclavian Artery
PubMed: 31653663
DOI: 10.1136/archdischild-2019-317625 -
European Journal of Cardio-thoracic... May 2022
Topics: Child; Diverticulum; Female; Humans; Pregnancy; Prenatal Diagnosis; Subclavian Artery; Treatment Outcome; Vascular Ring
PubMed: 35024831
DOI: 10.1093/ejcts/ezac007