Aorta-vena cava fistula

: In 1831 James Syme described the unusual occurrence of an aortocaval fistula in a 22-year-old man with luetic aortitis. This initial report was followed by illustration of this phenomenon in Rokitanski's Book of Pathologic Anatomy in 1841 and by Ryle's delineation of an aortocaval fistula on a pathologic specimen placed in Guy's Hospital Museum in 1892. The first series of aortocaval fistulas, cited by Rudolf Matas in 1909, consisted of a collection of 20 cases gathered by Boinet 10 years earlier. Several later reports, including those by Reid in 1925 and by Lehman in 1938, failed to add any additional cases. It was not until 1955 that Boffi presented an additional six patients who had this disorder, none of whom survived. Since that time, more than 100 cases of spontaneous aortocaval fistulas have been documented. This increasing experience has resulted in improved understanding and surgical treatment of these large-vessel arteriovenous communications. Nevertheless, lack of awareness and failure of recognition of this problem continue to impede its successful management. In this review we present two additional illustrative cases, summarize the clinical and pathophysiologic features of aortocaval fistulas, and outline present approaches to treatment.