Persistent double dorsal aorta: A systematic review of the literature

Abstract

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.

Case report: persistent double dorsal aorta

“…Persistence of a double dorsal aorta is an extremely rare condition, with just 12 cases described to date. 3 , 5 A bibliographic review conducted by Mills and Gest 3 compiled 11 cases from 1975 to 2016 and just one other case was reported in 2022, by Hurtado et al 5 …”

“…This fusion of the dorsal aortas starts anteriorly and propagates in the posterior direction. 3 , 6 , 7 …”

“…In the other variant, the descending aorta has a double lumen because of the presence of central dividing septum. 3 …”

Histologic and morphologic character of pediatric abdominal aortic developmental coarctation and hypoplasia

“…It is believed that the first description of aortic duplication was by von Kolliker in 1880, 33 and reports of this entity are rare. 34 Surprisingly, true aortic duplications may be asymptomatic and are often unanticipated autopsy findings. The vascular anomaly affecting case 6 of the current study mimics an abdominal aortic duplication, having one channel with lumbar artery branches before terminating at the origin of the common iliac arteries and a second channel from which the CA, SMA, and RAs arose.…”

Middle Aortic Syndrome: Pathogenesis, Visceral Manifestations, and Management

“…Failed lateral-to-medial migration and fusion with the persistence of both double dorsal aortae is rarely reported. 12 Both failed migration with unilateral obliteration and the persistence of a single atretic dorsal aorta, as well as "overfusion," have been postulated as potential mechanisms behind idiopathic MAS. 2,4,5,13 Pathological assessment reveals marked subendothelial fibroplasia without inflammatory changes similar to fibromuscular dysplasia.…”