Primary aorto-enteric fistula as a rare cause of massive gastrointestinal haemorrhage.

The incidence of primary aorto-enteric fistula (PAEF) is low with only few case reports and case series published. Depending on the location of the PAEF, the perforation leads to upper or lower gastrointestinal haemorrhages. We conducted a MEDLINE search according to the PRISMA statement. Articles with publication dates from 2000 to 2016 were included and present an own case report. We considered all case reports and series reporting on PAEF and identified 85 individual patients from 32 case reports and five case series. The majority of PAEF is associated with atherosclerotic or aneurysmatic findings of the aorta and in particular with inflammatory aortic diseases. Most commonly, the duodenum (64 %) was mentioned as location of the perforation. Other cases involved the jejunum (< 10 %) and the colon (5 %). Almost all patients were diagnosed either with gastrointestinal haemorrhage, abdominal or back pain, or anaemia due to bleeding. The immediate and correct diagnosis of this entity remains difficult. Therefore, treatment is delayed leading to an extraordinary high mortality of almost 100 % in untreated cases. Duplex ultrasound and contrast-enhanced CT angiography have high diagnostic sensitivity and specificity to rule out acute abdominal aortic pathologies. New endovascular approaches can help to lower mortality.