Unusual Congenital Aortic Anomaly with Rare Common Celiamesenteric Trunk Variation: MR Angiography and Digital Substraction Angiography Findings

Abstract

Magnetic resonance angiography and digital substraction angiography (DSA) findings in a case with a rare congenital thoracoabdominal aortic hypoplasia and common celiamesenteric trunk variation with occlusion of infrarenal abdominal aorta are described here. To our knowledge, this aortic anomaly has not been previously described in the English literature. DSA is the optimum imaging modality for determination of aortic hypoplasia, associated vascular malformations, collateral vessels, and direction of flow within vessels.

Severe diffuse hypoplasia of the aorta associated with multiple vascular abnormalities

“…5,10 Untreated patients frequently die in their early thirties from cardiac failure or cerebral hemorrhage, and aggressive medical management and often operative intervention become necessary in the second or third decade of life. 14 Medical control of the typically severe hypertension in these patients is often difficult.…”

“…Overfusion of the two embryonic dorsal aortas or their failure to fuse is thought to be the basis for most aortic narrowings. 7,10 Growth of a developmentally normal aorta may be disturbed by an acquired insult, either in utero or during the early life. Certain viruses, particularly rubella, may interfere with cell growth and may have direct cytopathic effects on a developing tissue.…”

“…14 The abdominal organs are supplied by the celiac artery, SMA and IMA, which usually originate separately from the abdominal aorta. 10 Variations in these arteries are very rare. Incomplete CT is found in 9%, and common origin of the CT and SMA is found in 7% of persons.…”

“…1,5,12 It is slightly more common among women than men. 5,10 The etiology of aortic hypoplasia is poorly understood. Developmental defects, a response to infection and inflammation have been proposed as possible mechanisms.…”

“…5,9 The etiology of this condition is still controversial, and both congenital and acquired etiologies have been proposed. 5,10 It is often associated with a narrowing or hypoplasia of the renal arteries (33–81%), celiac trunk (CT, 9%), superior mesenteric artery (SMA, 4%) and inferior mesenteric artery (IMA, 1.3%). 8,11 In the present study, we report a case of diffuse vascular hypoplasia, involving both the thoracic and abdominal aorta, associated with an atypical anatomical variation of the CT and SMA, and with multiple changes on the visceral and other arteries of the body.…”