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The presentation and management of vascular rings: an otolaryngology perspective
Rahul K Shah1, Bassem N Mora, Emile Bacha
1Division of Otolaryngology, Children's National Medical Center, 111 Michigan Avenue, NW, Washington, DC 20010, USA. reza.rahbar@childrens.harvard.edu
Insights
Vascular rings in children commonly cause airway and feeding problems. Early diagnosis with imaging and bronchoscopy guides surgical release, with minimally invasive options available.
Area of Science:
- Pediatric Surgery
- Cardiovascular Surgery
- Thoracic Surgery
Background:
- Vascular rings are congenital anomalies where the great arteries form a ring around the trachea and esophagus.
- These anomalies can lead to significant respiratory and feeding difficulties in infants and children.
Purpose of the Study:
- To review the clinical presentation and natural history of pediatric patients diagnosed with vascular rings.
- To establish management guidelines for children with vascular rings.
Main Methods:
- Retrospective chart review of patients diagnosed with vascular rings at a tertiary care pediatric medical center.
- Data collected from 1991 to 2002, including patient demographics, symptoms, diagnostic studies, and surgical interventions.
Main Results:
- 37 males and 27 females were diagnosed with vascular rings.
- 91% of patients presented with airway symptoms (e.g., stridor, recurrent infections) and 47% with esophageal symptoms (e.g., dysphagia).
- Common diagnostic tools included echocardiography (96%), chest X-ray (93%), and barium swallow (75%). Surgical management involved open (n=25) and thoracoscopic (n=39) approaches, with an 8% rate of recurrent laryngeal nerve injury.
Conclusions:
- Children with vascular rings typically present with respiratory and/or feeding issues.
- Diagnostic workup should involve chest X-ray, echocardiography, barium swallow, and direct laryngoscopy/bronchoscopy.
- Surgical intervention is indicated upon diagnosis, with both open and minimally invasive (thoracoscopic, robotic-assisted) options available.
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.
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