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Surgical Management and follow-up of vascular rings
F Rivilla1, J G Utrilla, F Alvarez
1Department of Surgery, La Paz Children's Hospital, Madrid, Spain.
Insights
Aortic arch anomalies cause significant airway and esophageal issues in children. Surgical correction leads to good outcomes, with most patients becoming asymptomatic, though severe tracheomalacia can be fatal.
Area of Science:
- Cardiology
- Pediatric Surgery
- Thoracic Surgery
Background:
- Aortic arch anomalies are critical causes of upper respiratory and esophageal obstruction in infants and children.
- Symptoms often manifest early in life, but surgical intervention is frequently delayed.
Purpose of the Study:
- To review the surgical treatment and outcomes of pediatric patients with aortic arch anomalies causing tracheo-esophageal compression.
Main Methods:
- Retrospective analysis of 30 patients treated between 1966 and 1987.
- Diagnosis confirmed via chest roentgenogram, barium esophagogram, and angiography.
- Surgical approach involved left thoracotomy, anomaly identification, and division of the constricting ring.
Main Results:
- Common anomalies included double aortic arch (43%), aberrant right subclavian artery (30%), and right aortic arch with ductus/ligamentum arteriosum (20%).
- Associated malformations were present in 27% of cases.
- 85% of patients were asymptomatic post-surgery; persistent stridor was minimal to moderate.
- Severe tracheomalacia accounted for the two deaths.
Conclusions:
- Surgical correction of aortic arch anomalies is effective in relieving tracheo-esophageal compression.
- Early diagnosis and intervention are crucial, though delays occurred in this series.
- While outcomes are generally favorable, severe tracheomalacia remains a significant risk factor for mortality.
Abstract:
Thirty patients with aortic arch anomalies resulting in tracheo-oesophageal compression were treated during the period 1966 through 1987. These anomalies are important causes of upper respiratory and oesophageal obstruction in babies and small children. Although symptoms started within the first months of life in most cases, only 15 underwent surgery before six months and a delay of more than one year occurred in 5. Diagnosis was established by chest roentgenogram, barium oesophagogram and angiography. Thirteen (43%) patients had a double aortic arch, 9 (30%) cases had aberrant right subclavian artery and 6 (20%) patients had right aortic arch with ductus or ligamentum arteriosum. One (3.5%) patient had pulmonary artery sling and 1 (3.5%) case had right aortic arch and ductus arteriosum and aberrant right subclavian artery. Associated malformations were seen in 8 (27%) cases, (4 ventricular septal defects, 1 atrial septal defect, 1 coarctation of the aorta, 1 hypoplasia of left pulmonary artery, 1 left diaphragmatic eventration). Basic surgical procedure includes exposure through a left thoracotomy, complete identification of the anomaly and division of the constricting ring. 85% of the patients are asymptomatic and minimal to moderate stridor persists among the remainder. Severe tracheomalacia was responsible for the only two deaths in the series.