Vascular ring double aortic arch12/9/2023 Respiratory symptoms are more common in infancy and early childhood, while adult patients tend to present more with dysphagia rather than stridor. ĭouble aortic arch is most diagnosed in childhood due to symptoms related to oesophageal and/or tracheal compression and obstruction. It is sometimes associated with chromosome 22q11 deletion and trisomy 21 in up to 20% of the cases. An associated cardiac anomaly is prevalent in up to 12.6%, including ventricular septal defect and Fallot’s tetralogy. The prevalence of vascular rings is estimated to be ~1% of which 55% are double aortic arch. They then rejoin as a single descending aorta. Double aortic arch results from failed regression of the right fourth arch during embryonic development, resulting in the formation of a vascular ring from the splitting of the ascending aorta into two limbs that pass on either side of the trachea and oesophagus, encircling and compressing them. The aortic arch normally develops from the left fourth arch, while the right fourth arch becomes the right innominate artery. Vascular ring malformations are relatively rare, and double aortic arch is their most common type. Bronchoscopy revealed no foreign body throughout the tracheobronchial tree, but evidence of pulsatile circumferential constriction of the lower trachea was noted, with tendency of the tracheal wall to collapse upon withdrawal of the bronchoscope ( Fig. Suspicion of unwitnessed foreign body aspiration arose, and an elective diagnostic bronchoscopy was arranged. No lung crepitations were appreciated.Ī chest X-ray was normal. Chest auscultation revealed bilaterally reduced, yet equal, air entry with transmitted stridor. Oxygen saturations on room air were 94% and the infant was afebrile. The infant was born full-term and had no significant medical or surgical history.Ī moderate degree of biphasic stridor, with both suprasternal and intercostal recessions, was noted on examination. They did not recall a specific chocking event or a recent respiratory tract infection. Symptomatic vascular rings require early surgical intervention to prevent prolonged vascular compression of the airway and serious complications.ĭouble aortic arch Multidetector computed tomography Thoracoscopic surgery Tracheoesophageal compression Vascular ring.A 1-month-old infant presented with progressive stridor that was noticed by the parents to have got worse for 2 months. 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. MDCT provides an excellent image of aortic arch abnormalities and the related anatomy, as well as the tracheal pathology. With the development of new technologies, multi-detector computed tomography (MDCT) has become a good diagnostic modality for pre- and postoperative evaluation. The persistence, involution, or regression of the arches determines the multiple variations of vascular ring. The development of a vascular ring begins with the embryonic aortic arch system. 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. In this review we discuss the pathophysiology, theoretical embryopathogenesis, diagnostic modalities, and surgical treatment of the different types of vascular ring. A vascular ring is a rare congenital cardiovascular anomaly, which encircles and compresses the trachea or esophagus, or both.
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