Congenital aortic arch anomalies and vascular rings
Vincent, J.; Lacquet, L.K.; Kuijpers, P.J.; Brom, A.G.
Acta Chirurgica Belgica 74(3): 305-316
1975
ISSN/ISBN: 0001-5458 PMID: 1224893 Document Number: 89204
The anomalies of the aortic arch are consequences of malformations in the first twelve weeks of the intrauterine life. Embryologically it is possible to explain and classify all of them. Most remain asymptomatic and do not need treatment. About one third of all anomalies are causing tracheo-esophageal compression with consequent complaints. In most cases the symptomatology starts in the first days of life. Some of them can be temporarily treated conservatively. The prognosis of patients, who need surgery is good, even in small infants. Surgery consists mainly in section of the atretic or hypoplastic part of the double aortic arch, section of the aberrant subclavian artery, section of the Botal ligament or duct and liberation of the trachea and esophagus. A normal configuration of trachea and esophagus is reached after several months or even more than a year. There are no reports on reoperations for secondary strictures. In the University Hospital of Nijmegen and Leiden we operated on 17 patients. There were 8 patients of group I A --- Edwards classification -- double aortic arch with both aortic arches open and left Botal duct or ligament; 4 patients of group I B, double aortic arch with one atretic aortic arch with a left Botal ligament; 3 patients of group II B, with a left aortic arch and aberrant right subclavian artery and a left Botal ligament; 2 patients of group III B with a right aortic arch and an aberrant left subclavian artery and a left Botal duct or ligament. Of our 17 patients one died during the operation because of a haemorrhage and overtransfusion; an other one died three weeks postoperatively from an endotracheal bleeding. All the other patients are without complaints and remained so.