Types of tracheal and esophageal constriction due to arterial anomalies of the aortic arch, with suggestions as to treatment.

H OMMEL [1,2] or HunauId [3] is credited with having described the first recorded instance of a doubIe aortic arch some 220 years ago. About fifty years Iater a persisting double aorta was found by MaIacarne in Torino. In 1793 Bayford [4] reported the case of a sixty year oId man, dysphagic a11 his Iife, in whom an aberrant right subcIavian artery arising from the descending part of a Ieft-sided aorta and coursing between the esophagus and trachea was found at death. Bayford beIieved the anomaIous position of this vesse1 may have produced these symptoms, and coined the term “dysphagia Iusoria” (from the Latin, “Iusor,” a deceiver) to identify this syndrome. These anomaIies remained as anatomic curiosities unti1 1926, when Jex-BIake [5] observed a puppy with stridor and dysphagia, in which autopsy reveaIed a constricting ring due to a right arch and a Ieft ligamentum arteriosum. He may have been the first to suggest that surgica1 correction of this was feasible if the diagnosis couId be made. Gross [6] stated that he began considering this in 1931 when the problem appeared in a postmortem examination, and Wolman [7] again suggested it in 1939. However, it was aImost exactly a century and a haIf after Bayford, unti1 Gross [6] in June, 1945, recorded the successfu1 division of an aortic ring constricting both the trachea and esophagus, and then the foIIowing year [8] surgica1 section of an aberrant right subcIavian artery causing dysphagia Iusoria. Since then severa discussions [I I-r5J of these embryoIogic deviations have appeared and certain facts concerning their recognition, significance and treatment are emerging. It is the purpose of this report to present some of these, as we11 as the experience of severa individuals in this regard at the ChiIdren’s Orthopedic HospitaI in SeattIe.* AIthough doubIe aortas are norma in certain reptiIes and right-sided aortas persist in birds, the definitive pattern of a singIe Ieft aortic arch and its typica major branches in man is estabIished very earIy. According to Congdon’s [16] cIassic studies the arteria1 arcades in the giII arches begin appearing in the third week of embryonic deveIopment. And they have evoIved to their ad& form, incIuding the disappearance of the right aortic arch and the fusion of the two dorsa1 aortas beyond this, some six weeks Iater. MaternaI iIInesses which might influence the course of these metamorphoses therefore must presumably be sought in the very earIy stage of pregnancy. The evolution of these vesseIs in relation to the foregut and deveIoping trachea and lung buds is compIex and diffIcuIt to expIain in writing. Two animated movies, pubIished by Dr. George Humphreys, show these processes cIearIy. The study of Edwards [13] gives their patterns an orderIiness adapted to cIassifying the anomaIies actuaIIy found and those that are possibIe. Inasmuch as it is not the primary purpose of this essay to discuss these, only variations of more common cIinicaI interest wiII be presented. These variations faII into three genera1 categories: (I) those in which the trachea and esophagus are actualIy encircIed by a double aortic arch, part or all of which may be patent