A Fatal Aortoesophageal Fistula Caused by Critical Combination of Double Aortic Arch and Nasogastric Tube Insertion for Superior Mesenteric Artery Syndrome

Abstract

Double aortic arch (DAA) is a rare vascular congenital abnormality. Since a vascular ring surrounds bronchus and esophagus, any oral or nasal intubation can physically cause fatal aortoesophageal fistula (AEF). We report herein the first case of association of DAA and superior mesenteric artery (SMA) syndrome and the second case of AEF caused by nasogastric intubation in an adult with DAA. A 19-year-old woman visited our hospital for nausea and vomiting. She was diagnosed with SMA syndrome by computed tomography (CT). Nasogastric intubation relieved her symptoms in 4 days. Extramural compression with top ulceration was found in esophagogastroduodenoscopy on the 5th hospital day. She suddenly showed massive hematemesis on the 12th hospital day. AEF was found by CT. Soon, she died despite of intensive care. Retrospective interview disclosed the fact that DAA was pointed out in her childhood. We conclude that intubation must be avoided in DAA and a detailed clinical interview about DAA is mandatory to avoid AEF.

Clinical value of multi-slice spiral computed tomography angiography and three-dimensional reconstruction in the diagnosis of double aortic arch

“…DAA entirely or partially surrounds the trachea and esophagus and causes compression and symptoms, including wheezing and difficulties in respiration and swallowing. However, unclear clinical features lead to misdiagnosis or even fatal aorta-tracheal fistula due to tracheal cannula ( 2 ). Therefore, early accurate diagnosis is critical ( 3 ).…”

Primary aortoesophageal fistula: A rare but fatal cause of upper gastrointestinal bleeding

“…Infection contributes to about 25% of all aortoenteric fistulas before the 1970s, whereas an aneurysmal aorta is the most common cause today . Besides aneurysms and infection, other causes of aortoenteric fistula include tumor and foreign bodies …”

“…The first diagnostic tool for upper gastrointestinal bleeding is EGD, but its detection rate for AEF is low . The endoscopic findings include mild elevated or polypoid lesions with overlying clots or fibrins at the upper or middle esophagus . A pulsatile bulging lesion with or without adherent blood clots is rarely detected.…”

“…8 Besides aneurysms and infection, 9,10 other causes of aortoenteric fistula include tumor 11 and foreign bodies. 12,13 The first diagnostic tool for upper gastrointestinal bleeding is EGD, but its detection rate for AEF is low. 8 The endoscopic findings include mild elevated or polypoid lesions with overlying clots or fibrins at the upper or middle esophagus.…”

“…8 The endoscopic findings include mild elevated or polypoid lesions with overlying clots or fibrins at the upper or middle esophagus. 2,[13][14][15] A pulsatile bulging lesion with or without adherent blood clots is rarely detected. Therefore, a high index of suspicion is essential for a prompt diagnosis of AEF.…”