Int Surg 2006;91:5-8 Hypovolemic Shock Caused by a True Aneurysm of the Pancreatico-Duodenal Artery: Case Report and Review of the Literature Domenico Iusco, Leopoldo Sarli, Enrico Donadei, Luigi Roncoroni Surgical Department, Section of Surgical Clinic and Surgical Therapy, Parma University, Parma, Italy True pancreatico-duodenal artery aneurysm (PDAA) is a rare condition that since 1973 has been described in only 54 cases. It is frequently associated with celiac axis stenosis and often present with rupture. Even if most PDAAs that are not ruptured are asymptomatic and are diagnosed during investigation for other diseases, they may have some symptoms, such as chronic abdominal discomfort and an abdominal pulsating mass, that can be helpful for diagnosis. The treatment of this condition has evolved in time from a merely surgical one to an angiographic noninvasive approach. We present a case of a PDAA that manifested with sudden hypovolemic shock requiring an emergency operation, and through a review of the literature, we discuss the different diagnostic/therapeutic proto- cols to use in different situations. Key words: Aneurysm - Hemoperitoneum — Pancreatico-duodenal artery mong vascular diseases of the abdomen, aneu- rysms of visceral arteries present a real diag- nostic and therapeutic challenge. They are rare affec- tions (0.2% to 0.7% of the population) that may present with a wide range of symptoms ranging from persistent abdominal discomfort to hypovole- mic shock caused by rupture. These diseases require an aggressive surgical approach or a conservative angiographic one, depending on the situation. The incidence of this kind of aneurysm is on the increase, especially because of incidental diagnosis during ex- aminations, which are more commonly performed today, such as angiography, angio-nuclear magnetic resonance (NMR), and computed tomography (CT). Pancreatico-duodenal artery aneurysm (PDAA) is rare. As with other visceral aneurysms, it is very Reprint requests: Iusco Domenico, MD, Istituto Clinica Chirurgia Generale e Terapia Chirurgica, Azienda Ospedale, Via Gramsci n. 14, 43100 Parma, Italy. Tel +0039 521 991156; Fax: +0039 521 940125; E-mail: minguccio73@hotmail.com Int Surg 2006;91difficult to have an early diagnosis of PDAA; most of the patients reported presented after rupture, which carries a high mortality rate. The increased use of visceral angiography, and, more recently, the intro- duction of transcatheter arterial embolization, have helped to reduce this rate.2* True and false PDAAs should be distinguished from each other, the latter resulting from pancreati- tis, abdominal trauma, or septic emboli, whereas the former is frequently associated with a stenosis of the celiac axis.‘ We present a case of true PDAA that allows us to underscore some peculiarities of this disease and to seek, through data analysis, useful information concerning the diagnostic therapeutic protocol to follow in these cases. Case Report ES., male, 60 years of age, without any significant disease in the anamnesis, complained of the sudden onset of a belt-like pain in the epigastric-mesogastric abdominal region accompanied by an episode of syncope, which passed after a few minutes. Because of the persistence of the abdominal pain, the patient decided to drive himself to the emergency room, where he suffered sudden hypovolemic shock. His blood pressure was 80/60 mmHg, the skin was sweaty, and femoral pulses were present. Blood tests showed anemia (hemoglobin = 6.3 g/dl; hematocrit (HCT) = 19.1%), leucocytosis (18 x 10°/,l), and an increase in total bilirubin (1.5 mg/dl). Ultrasound sonography showed an echogenic mass of the head of the pancreas; CT performed with contrast showed a large hypodense mass (maximum diameter, 17 cm) occupying the mesogastric region and the region under the liver, with hemorrhagic spilling of con- trast: the pancreas was thickened and pushed for- ward. This hemorrhagic mass was situated at the Gerota fascia and around the right kidney (Fig. 1). The patient, in a state of hypovolemic shock, was transferred to the emergency surgical service for emergency surgery. The laparotomy showed signif- icant hemoperitoneum with imposing paraduode- nal, retropancreatic (head), and right renal loggia hematoma; 3.5 1 of blood was suctioned, and a retroduodenopancreatic arterial hemorrhage caused by an aneurysm of the upper pancratico-duodenal arteries 1.5 cm in diameter was detected. Hemostasis was achieved by clamping the source of bleeding and by ligation of the gastroduodenal artery. During surgery, 12 units of red blood cells, 5 units of Beet Fig. 1 CT scan showing the hemorrhagic mass. platelets, and 9 units of plasma were transfused; the level of hemoglobin was raised from 2.8 to 5.5 g/dl. ‘The postoperative course was uneventful. During the hospital stay, the patient underwent a panangiography that showed ligature of the gastro- duodenal artery, considerable stenosis on the origin of the celiac axis, probably caused by tendon fibers of the arcuate median ligament, no aneurysmatic dila- tation of the splanchnic arteries, and upper mesen- teric artery and venous porto-spleno-mesenteric axis patency (Fig. 2). Fig, 2 Postoperative angiography showing stenosis of the origin celiac axis. Int Surg 200691The patient is in good condition after a 1-year follow-up. Discussion PDAAs are particularly uncommon, representing only 2% to 2.5% of all visceral aneurysms.? The in- cidence of true PDAA is extremely low, but remains unknown because no large series have yet been pub- lished. It is noteworthy that, in contrast to large vessel aneurysms, atherosclerosis may not be the primary causative factor. Evidence suggests that lo- cal hemodynamic events play an important role in the development of most visceral aneurysms. In fact, a higher incidence of splenic artery aneurysm is associated with multiparity and portal hypertension because of the increased arterial blood peculiar to these two conditions. Similarly, Sutton and Louton* postulated that the stenosis of the celiac axis, also found in our case report, results in an increased retrograde flow through the pancreatico-duodenal arteries, which favors the development of PDAA. Of the 52 cases of true PDAA reported in the literature, 33 were found in association with a steno- sis of the celiac axis, whereas only 9 were reported with local atherosclerotic lesions. The remaining cases were caused by congenital aberration of the ves- sel wall or medial fibrodysplasia®.” The etiology of the stenosis of the celiac axis is quite variable: the most frequent cause is the compression of the axis by the arcuate median ligament of the diaphragm, whereas celiac axis thrombosis or agenesia are rarer." Only 10% of the true PDAAs described in the literature were asymptomatic and were found by chance during instrumental examination performed for other reasons; 35% were symptomatic (chronic abdominal discomfort and abdominal pulsating mass); and 55% manifested with a rupture. As our case report shows, true PDAAs more fre- quently rupture in the retroperitoneal space, causing acute abdominal pain that may simulate a gastrodu- odenal, biliary, or pancreatic disease. Unlike aneurysms of the splenic artery, which rarely rupture when they are <2 cm/ true PDAAs have not shown a clear correlation between size and propensity to rupture. In the literature, 10 cases of ruptured PDAAs with a diameter of <1.5 cm are described (four of these were <1 cm in diameter).! This peculiarity makes the treatment of this aneu- rysm strongly recommended every time it is diag- nosed, because in the case of rupture, there is a mortality rate ranging from 26% to 37%, in contrast Int Surg 200691 to a 0% to 4% rate in patients with a nonruptured aneurysm. In the case of hypovolemic shock with unstable hemodynamic parameters, after immediate resusci- tation and intensive treatment, an emergency celiot- omy should not be delayed. When critical but stable hemodynamic conditions prevail, it is recommended to perform an angiography, not only to study the vascular anatomy, but also because it is possible to treat these diseases with embolization, which may result as being therapeutic or at least may allow for the stabilization of the bleeding and for the time needed for definitive surgery.!? The failures of this diagnostic-therapeutic tech- nique are described as being caused by particular anatomic conditions or technical difficulties. In ad- dition, aneurysm rupture during attempts at trans- catheter embolization and ischemic damage caused by the lack of major collateral arterial branches have been reported.!? Frequently, an immediate surgical approach does not reveal the exact location of the aneurysm, because 70% of PDAAs are located be- hind the pancreatic parenchyma; therefore, after a ruptured aortic aneurysm has been ruled out, it is acceptable merely to perform only a general control of the hemorrhage and to have a surgical first look. In the immediate postoperative period, an angiogra- phy is mandatory because it shows the aneurysm, which will be treated either with a surgical second look or with angiographic embolization.>4 The surgical treatment of PDAA suggested in the literature differs according to whether the aneurysm has ruptured or not. When the aneurysm has rup- tured, the main surgical goal is to stop of the bleed- ing immediately, and so an excluding vascular liga- ture was performed in 60% of the cases; in the rest of the cases, it was possible to perform a resection of the aneurysm, either associated or not to an anasto- mosis of the pancreatico-duodenal artery with the upper mesenteric artery. Furthermore, in some cases of emergency surgery, a cephalo-pancreatic resection was required to dominate the retroperitoneal hema- toma for its retro-pancreatic localization.15. When the operation was not performed in an emergency situation, the operation most performed was aneurysmectomy in 53% of the reported cases, associated with anastomosis between the pancre- atico-duodenal artery and upper mesenteric artery in 23% and aorto-hepatic bypass in 12% of the cases. Quandelle et al recommended, in the case of coex- isting aneurysm and celiac axis stenosis, the revas- cularization of the celiac territory to preclude recur-qusco. rence by restabilizing normal circulation, in addition to local treatment of the aneurysm. Mora’? and Proud and Chamberlain’ described two cases in which celiac axis decompression alone was sufficient for the spontaneous remission of the aneurysm at an angiographic follow-up. This was probably caused by thrombosis of the aneurysmatic sac. In conclusion, PDDAs are rare events that, when not diagnosed by chance, often present with dra- matic hypovolemic shock that should be treated in the most appropriate way according to the situation. Because there is no direct correlation between aneu- rysm size and its rupture, its treatment by means of interventional radiology or surgery is always recom- mended. 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