Persistent double dorsal aorta: A systematic review of the literature

Our discovery of a case of persistent double dorsal aorta prompted us to systematically review the literature of all previously reported cases of this anomaly. For our case, we present a completely separated double dorsal aorta, with the right accessory aorta arising from the abdominal aorta and ascending through the aortic hiatus to supply posterior intercostal arteries (PIAs) to the 9th-3rd spaces bilaterally. We examined and compiled data from the 10 previously reported cases, along with our observations from our cadaver, into a systematic review of all known cases of persistent double dorsal aorta. In addition to our case report and systematic review, we investigated the literature focusing on formation of the dorsal aorta in the embryo in order to postulate potential mechanisms for formation of this anomaly. Two variants of persistent double dorsal aorta have been reported in the literature. The first type is characterized by a double-lumen descending aorta with a central dividing septum, and the second features complete separation of the two dorsal aortae. The completely separated variant shows further heterogeneity in the origins of the PIAs and the iliac arteries, and the majority of the reported cases also demonstrate additional anatomical anomalies. We outline the events in embryonic dorsal aorta formation as well as discuss several potential mechanisms that could underlie persistent double dorsal aorta formation. Clin. Anat. 30:517-524, 2017. © 2017 Wiley Periodicals, Inc.