The presentation and management of vascular rings: An otolaryngology perspective

Presented as a poster at the American Broncho-Esophageal Association Annual Meeting, Scottsdale, Arizona 2004
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Summary

Objective

To review the presentation and natural history of children with vascular rings and present management guidelines.

Methods

Retrospective study of tertiary care pediatric medical center charts from 1991 to 2002.

Results

There were 37 males and 27 females with a diagnosis of vascular rings. At presentation, 91% of patients had airway symptoms and 47% had esophageal symptoms. Airway symptoms included stridor (63%), recurrent respiratory infections (47%), respiratory distress (19%), and cough (17%). The most common esophageal symptom was dysphagia (27%). Pre-operative studies included: echocardiography (96%), chest X-ray (93%), barium swallow (75%), magnetic resonance imaging (MRI) (60%), and computerized tomography (CT) scan of the chest (59%). Surgical management included open (n = 25) and thoracoscopic (n = 39) approach. Complications included recurrent laryngeal nerve injury in five patients (8%).

Conclusion

Children with vascular rings present with respiratory and/or feeding difficulty. The evaluation should include chest X-ray, echocardiography, and barium swallow. Direct laryngoscopy and bronchoscopy are recommended to assess the degree of compression of the airway and/or esophagus, tracheomalacia, and vocal fold motion prior to intervention. Indication for surgical release is given when the diagnosis is made and can be assisted by advanced radiology studies. Surgical options include minimally invasive techniques involving either thoracoscopic or robotic-assisted repairs, as well as open procedures involving thoracotomy.

Introduction

Vascular rings are congenital vascular anomalies of the aortic arch complex that result in compression of the trachea and/or esophagus. These lesions often manifest with airway compromise due to extrinsic tracheal compression. Patients with airway symptoms usually present earlier, while those with esophageal symptoms present later in childhood, often associated with the onset of solid feeds. Congenital vascular anomalies can be broadly categorized as anatomically complete rings (as is seen in double aortic arch patients) or anatomically incomplete rings (as is seen in the remainder of vascular rings) [1], [2], [3], [4].

As the airway and esophagus may be significantly compressed in patients with vascular rings, the otolaryngologist is often called upon to assist in the evaluation of these patients. Technological advances have led to changes in the evaluation and management of vascular rings. This study was designed to review our institutional experience with the presentation, evaluation, and management of vascular rings over the last decade and to present guidelines for the care of these patients.

Section snippets

Methods

A retrospective chart review was performed of all patients undergoing surgical division of vascular rings at the Children's Hospital Boston, covering the period 1991–2002. This study was approved by the Committee for Clinical Investigation at Children's Hospital Boston. Complete patient records were reviewed for this study. Pertinent data extracted from the patient's charts included: demographic information, presenting symptoms, pre-operative imaging studies, intra-operative findings, outcomes,

Results

From 1991 to 2002, 64 patients (37 males, 27 females) underwent vascular ring division at the Children's Hospital Boston. The mean age at the time of surgery was 3.0 years (standard deviation 5.5 years). The mean weight at the time of surgery was 15.3 kg (standard deviation 20.4 kg). The most common presenting symptoms were related to the airway (92%) and the esophagus (45%). Presenting airway symptoms included stridor (63%), recurrent respiratory infections (47%), respiratory distress (19%), and

Discussion

The term vascular ring denotes a congenital anomaly of the aortic arch system that results in compression of the trachea and/or esophagus with resulting symptoms.

An understanding of the embryology of the great vessels is imperative to understanding the aberrant development that leads to vascular rings. During embryogenesis, the human aortic arch system develops with six pairs of aortic arches connecting two ventral and dorsal aortae [1], [2]. A significant portion of the first, second, and

Conclusion

Children with vascular rings present with respiratory and/or feeding difficulty. The symptoms of the patient can help guide the differential diagnosis regarding which type of vascular ring the patient may have. Evaluation of patients with a suspected vascular ring should include chest X-ray, echocardiography, and barium swallow. Direct laryngoscopy and bronchoscopy are recommended to assess the degree of compression, tracheomalacia, and vocal fold motion prior to intervention. Surgical options

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