Primary aorto-esophageal fistula due to Takayasuʹs aortitis

Background: Aneurysmal dilatation in Takayasuʹs arteritis is a recognized complication; however, fistula formation, especially to the esophagus, is very rare. Methods: A 22-year-old male presented with severe hematemesis. Investigation by means of esophagogastroscopy and CT scan revealed a saccular aneurysm in the proximal descending aorta with communication to the esophagus. The patient was taken to theater, the aneurysm excised and replaced by a graft. Results: Gross examination of the aneurysm showed multiple points of outpouching from the aneurysm. Histopathological examination of the showed marked intimal fibromyxoid thickening, loss of outer medial muscle and elastic fibers and marked fibrosis of the adventitial layer. The histological features were in keeping with Takayasuʹs arteritis. No evidence of tuberculosis was noted. Conclusions: This case illustrates an unusual complication of Takayasuʹs arteritis, in the form of a fistula between the aorta and the esophagus, which resulted in massive hematemesis and the ultimate demise of the patient.