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An unusual case of abdominal aortic aneurysm with simultaneous rupture in the retroperitoneum and in the inferior vena cava is reported. The patient presented with  clinical signs of hemorrhagic shock, peripheral venous congestion and acute renal failure. An urgent contrast-enhanced computed tomography  was performed , leading to an accurate diagnosis , and  straight to surgery.
Simultaneous rupture of an abdominal aortic aneurysm (AAA) in the retroperitoneum and into  the inferior vena cava(IVC), with a subsequent aorto-caval fistula (ACF), is absolutely rare and it is not clearly reported in the Medical Literature. The clinical presentation is dramatic and demands urgent diagnostic and therapeutic interventions (1,2,3).
A 67 years old man, heavy cigarette smoker, with a history of controlled hypertension, not complicated by symptoms of peripheral vascular diseases, was admitted to the hospital for acute onset of abdominal pain , followed by hypovolemic shock, with arterial pression 85/50 mmHg. The physical examination showed cyanosis of patientâs inferior extremities and back , and diffuse tenderness in the lower abdominal quadrants and in the left flank; a systolo-diastolic murmur was audible in the periumbelical region. Among blood tests, we remark Ht: 33%; blood urea: 121 mg%; creatinine: 6 mg%, while bilirubin, GOT , GPT and coagulation tests were normal.The abdomen ultrasound revealed an enlarged abdominal aorta with a periaortic hematoma. A subsequent multidetector row-angio computed tomography (CT) demonstrated a calcified fusiform infrarenal AAA, 8.7 cm in diameter, long 14 cm, starting just below the renal arteries, and ending at the iliac bifurcation. It appeared ruptured in the right retroperitoneum , whith a large hematoma extending from the ileo-psoas muscle to the Gerota fascia (Illustration 1,2). During the same arterial phase, the IVC appeared dilated and opacified simultaneously to the aorta , through an abnormal communication , 3 cm in diameter, with the adjacent left wall of the AAA (Illustration 3). The same phase of CT study demonstrated other characteristic aspects of the ACF, interesting from a hemodynamic point of view. The IVC and the iliac veins were dilated , as well as the right renal vein , with absence of perfusion of both the kidneys(Illustration 4,5) ; the same congestion could be observed in the hepatic veins (Illustration 6).A prompt laparotomy confirmed the presence of an infrarenal AAA ruptured in the left retroperitopneum. Its sac was incised anteriorly, the inner thrombus carefully removed, and an ACF 3 cm in diameter was visualized. It was repaired with interrupted stitches from within the aorta. An aorto-bis-iliac prosthetic Dacron graft was inserted. The post-operative course was uneventful, with complete resolution of the acute renal failure , and without persisting signs of venous insufficiency or pelvic congestion.
Our observation permits to outline the leading symptoms of this syndrome : acute abdomen and hemorrhagic shock, typical of rupture of an AAA, and peripheral venous congestion and hypertension in the IVC characteristic of an ACF. The systemic complications of this latter can express as heart failure (4,5,6), necrotic hepatitis (7), or more commonly as acute renal failure. Its sudden onset and rapid development can be referred to the circulatory shock and to hypertension in the IVC and both renal veins. Of course, the size of the ACF and the different volume of the shunt influence the severity of all the possible complication.From a diagnostic point of view, we can remark that, although these signs can alert toward the recognition of this syndrome, a contrast enhanced CT is essential to make an accurate diagnosis, to plan an urgent surgical treatment and to avoid intraoperative complications, such as dislodgement of mural thrombi, inadvertent laceration of the IVC, too vigorous fluid infusion with secondary worsening of cardiac failure(8,9,10,11).Considering the etiology, an atherosclerotic AAA can simply ulcerate into the adherent IVC, or more rarely, it can also simultaneously rupture in the retroperitoneum.Our therapeutic approach consisted in an immediate open surgery. Nevertheless , in case of large size ACF, an endovascular venous technique can be considered , in order to obviate the hemodynamic consequences of the ACF before an open surgical treatment of the AAA (12). On the other hand, it must be observed that an endovascular aortic reconstruction, if complicating later with an endoleak , can reactivate and progressively worsen the pre-esisting ACF (12).
AAA: Abdominal Aortic AneurysmIVC: Inferior Vena CavaACF: Aorto-Caval FistulaCT: Computer Tomography
1.Burke A.M., Jamieson G.G. Aortocaval fistula associated with ruptured aortic aneurysm. Br.J.Surg. 1983;70:431-433.2.Cinara I.S., Davidovic L.B.,Kostic D.M. et al. Aorto-caval fistula:a review of eighteen years experience. Acta Chir. Belg. 2005;105:616-620.3.Davidovic L., Dragas M., Cvetkovic S. et al. Twenty years of experience in the treatment of spontaneous aorto-venous fistulas in a developing country. World J.Surg. on line 30.4.2011.4.Houben P..F.Bollen E.C., Nuyens C.M. âAsymptomaticâ ruptured aneurysms: a report of two cases of aortocaval fistula presenting with cardiac failure.Eur.J.Vasc.Surg.1993;7:352-354.5.Sadraoui A., Philip I., Debauchez M.,et al. Diagnostic hémodynamique dâune fistule aortocave compliquant un anévrysme de lâaorte abdominale. Ann.Fr.Anest.Réan. 1994;123:403-406.  6.Leigh-Smith S., Smith R.C. Aorto caval fistula-the âbursting heart syndromeâ. J.Accid.Emerg.Med.2000;17:223-225.7.Sobrihno G., Ferreira M.E., Albino J.P. et al. Acute ischemic hepatitis in aortocaval fistola. Eur.J.Vasc.Endovasc.Surg.2005;29:239-243.8.Bednarkiewicz M., Prete R., Kalangos A. et al. Aortocaval fistula associated with abdominal aortic aneurysm:a diagnostic challange. Ann.Vasc.Surg.1997;11:464-466.9.Frauenfelder T., Wildermuth S., Marincek B., Boehm T. Nontraumatic emergent abdominal vascular conditions:advantages of multi-detector row CT and three-dimensional imaging. Radiographics 2004;24:481-496.10.Davidovic L.B., Markovic M.D., Jakovlievic N.S. et al. Unusual forms of ruptured abdominal aortic aneurysms. Vascular 2008;16:17-24.11.Peve W.C., Lee E.S., Lamba R. Symptomatic, acute aortocaval fistula complicating an infrarenal aortic aneurysm. J.Vasc.Surg.2010;51:475.12.Siepe M., Koeppe S., Euringer W., Schlensak C. Aorto-caval fistula from acute rupture of an abdominal aortic aneurysm with a hybrid approach. J.Vasc.Surg 2009;49:1574-1576.