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Hepatic Artery Pseudoaneurysm Rupture: A Case Report and Review of the Literature
The Journal of Emergency Medicine, Vol. 44, No. 1, pp. 100–103, 2013 Copyright Ó 2013 Elsevier Inc. Printed in the USA. All rights reserved 0736-4679/$ - see front matter
doi:10.1016/j.jemermed.2011.08.021
Clinical Communications: Adults HEPATIC ARTERY PSEUDOANEURYSM RUPTURE: A CASE REPORT AND REVIEW OF THE LITERATURE Dena A. Reiter, MD,* Aaron M. Fischman, MD,† and Bradley D. Shy, MD* *Department of Emergency Medicine and †Department of Interventional Radiology, Mount Sinai School of Medicine, New York, New York Reprint Address: Dena A. Reiter, MD, Department of Emergency Medicine, Mount Sinai School of Medicine, One Gustave L. Levy Place, Box 1620, New York, NY 10029
, Abstract—Background: Ruptured hepatic artery pseudoaneurysm, a type of visceral artery aneurysm, is a rare condition that is life threatening if not diagnosed and treated rapidly in the emergency department (ED). Patients presenting with this condition require aggressive resuscitation. Endovascular embolization is the first-line treatment option. Objectives: We present a case of spontaneously ruptured hepatic artery pseudoaneurysm and provide a review of the current literature on this topic, focusing on appropriate ED management. Case Report: A 41-year-old woman with a history of systemic lupus erythematosus and multiple hepatic bilomas presented to the ED in critical condition with sudden onset of severe abdominal pain and hemodynamic instability. She was found to have a ruptured hepatic artery pseudoaneurysm with marked hemoperitoneum on computed tomography angiography. She was aggressively resuscitated and successfully managed via endovascular embolization. Conclusion: Ruptured hepatic artery pseudoaneurysm is a life-threatening condition that must be rapidly diagnosed and managed in the ED. Visceral artery aneurysm rupture is a diagnosis that should be considered in any patient presenting to the ED with hemodynamic instability and abdominal pain. Definitive management is with endovascular embolization. Ó 2013 Elsevier Inc.
of all hepatic artery aneurysms is estimated at approximately 0.002%, and approximately 50% of hepatic artery aneurysms are pseudoaneurysms (1,2). Such aneurysms are at high risk of spontaneous rupture, resulting in abdominal vascular catastrophe. Reported mortality rates of any visceral artery aneurysm rupture is estimated between 25% and 70% (1,3). We present a case of ruptured right hepatic artery pseudoaneurysm associated with systemic lupus erythematosus and multiple hepatic bilomas. To our knowledge, no such case has ever been reported in the medical literature.
CASE REPORT A 41-year-old woman with a history of systemic lupus erythematosus, autoimmune hepatitis, primary sclerosing cholangitis, antiphospholipid antibody syndrome, and pulmonary embolus (receiving warfarin therapy) presented to the Emergency Department (ED) reporting sudden-onset severe diffuse abdominal pain. At triage, her vital signs were: temperature 36.0 C, blood pressure 114/71 mm Hg, heart rate 123 beats/min, respiratory rate 20 breaths/min, and oxygen saturation 100% on room air. Shortly after arrival in the ED, the patient had a syncopal episode. After her collapse, we found her minimally responsive with agonal breathing and weak femoral pulses. She quickly regained consciousness with brief bag mask ventilation, at which point she became very agitated, reporting unbearable abdominal and chest pain.
, Keywords—pseudoaneurysm; hemoperitoneum; embolization; resuscitation; critical care
INTRODUCTION Hepatic artery pseudoaneurysm is a rare condition infrequently reported in the medical literature. The incidence
RECEIVED: 10 March 2011; FINAL SUBMISSION RECEIVED: 1 July 2011; ACCEPTED: 28 August 2011 100
Hepatic Artery Pseudoaneurysm Rupture
101
Figure 1. Computed tomography demonstrating right hepatic artery pseudoaneurysm with hepatic capsular rupture (arrow) and extravasation into the peritoneal cavity.
The patient was ill-appearing, agitated, confused, and diaphoretic. Her post-syncope vital signs were: heart rate 126 beats/min, blood pressure 66/43 mm Hg, respiratory rate 25 breaths/min, and oxygen saturation of 100% on an oxygen non-rebreather mask. Her abdomen was nondistended and diffusely tender to palpation. Bedside pregnancy test was negative and initial bedside ultrasound examination did not reveal intraperitoneal free fluid. Fluid resuscitation was started and the patient was hemodynamically stabilized. Immediate computed tomography (CT) angiogram imaging was obtained as there was concern for aortic dissection or rupture; it demonstrated multiple bilomas within the liver, a ruptured right hepatic artery pseudoaneurysm (4.7 cm by 4.8 cm), rupture of the lateral hepatic capsular wall with active extravasation into the biloma and into the peritoneal cavity, and marked hemoperitoneum (Figures 1, 2). Laboratory analyses were notable for a hemoglobin level of 5.9 g/dL, an international normalized ratio of 2.5, and a lactate of 13.3 mmol/L. Blood products (uncross-matched packed red blood cells, fresh frozen plasma, and prothrombin complex concentrate) were started immediately. She was taken to the interventional radiology suite for emergent embolization. A replaced right hepatic artery with descending intrahepatic branch feeding a large pseudoaneurysm with active extravasation was visualized (Figure 3A). She successfully underwent embolization of the right hepatic artery using n-butyl cyanoacrylate glue (Figure 3B). The patient was transferred to the intensive care unit. She remained hemodynamically stable. Her hospital course was complicated by bacteremia. She was discharged home after 28 days. Her warfarin therapy was stopped. At routine follow-up 4 months after discharge, she was alive and well.
Figure 2. Three-dimensional reconstructed image of computed tomography angiography demonstrating right hepatic artery pseudoaneurysm (arrow).
DISCUSSION Ruptured hepatic artery aneurysm is a rare condition with very high mortality. It was first described in 1809 by James Wilson, who conducted an autopsy on a clergyman who had died after its rupture. It was noted to have ‘‘the color, shape and size of the heart’’ (4). Incidence of false aneurysms may be increasing since the advent of percutaneous diagnostic and therapeutic biliary procedures, and detection may also be rising since the advent of CT scanning after trauma (5). Additionally, patients who have undergone liver transplant are at increased risk. There has been an increase in number of reported hepatic artery aneurysms, which may be attributed to increased awareness, improved imaging modalities, and increase in number of percutaneous biliary procedures, as well as liver biopsy and drainage (1,5,6). Overall, there have been fewer than 400 cases reported in the literature, with 80% presenting with rupture as the initial clinical event (5). Of all visceral arterial aneurysms, hepatic aneurysms have the highest reported rate of rupture at 44% (7). Of those that rupture, 50% rupture into the biliary tract, resulting in gastrointestinal hemorrhage (8). Another 20– 30% rupture into the peritoneal cavity (1,2). Although the frequency of death from aneurysmal rupture in humans is unknown, the reported mortality rate once rupture into the peritoneal cavity has occurred is 82% (9).
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visceral organ structure and may not manifest with intraperitoneal free fluid. Additionally, certain visceral aneurysm ruptures may bleed into the retroperitoneum and be undetectable on bedside ultrasound. In an ED setting involving a critically ill patient, CT angiography is rapidly attainable and provides accurate information regarding the anatomy of visceral aneurysms. It also delineates the extent of extravasation and hemoperitoneum as well as demonstrates any associated intraperitoneal or retroperitoneal pathology. CT angiography may be followed by conventional angiography if a therapeutic intervention is planned. As patients with visceral artery aneurysms are frequently presenting in hemorrhagic shock, aggressive resuscitation with blood products as well as reversal of any underlying coagulopathies is critical. Hemorrhage control is definitive. Presently, endovascular repair is considered first-line treatment of visceral artery aneurysms (13). It allows for more precise location of the aneurysm, accurate assessment of collateral flow, targeting of smaller, more distal vessels, and is minimally invasive (13). Arterial ligation is considered a last resort when embolization fails, as it has a limited success rate with higher morbidity. At one institution, 48 consecutive patients with visceral artery aneurysm (VAA) who underwent endovascular repair were identified between 1997 and 2005. Low morbidity and mortality were noted in the periprocedural period; however, these numbers were higher among patients undergoing emergent repair (8.3% mortality) vs. elective repair (0% mortality) (14). Another institution reported 6% mortality in the endovascular repairs of 65 consecutive patients who presented with VAA (15). Selection bias toward healthier patients may have affected these low mortality numbers; thus, it is important to include both interventional radiology and surgical consultation in determining the best management approach in the ED. Figure 3. (A) Pre-embolization angiogram demonstrating a replaced right hepatic artery with descending intrahepatic branch feeding a large pseudoaneurysm with active extravasation (arrow). (B) Post-embolization angiogram.
There are several imaging modalities available to aid in the diagnosis of hepatic artery aneurysms and pseudoaneurysms. They may be identified using ultrasound with the addition of color Doppler (10–12). However, there are no studies examining the use of bedside ultrasound in the ED to detect such lesions. ED providers may not be sufficiently trained or have resources available at the bedside to perform such studies. Bedside ultrasound is insufficient to detect all ruptured visceral aneurysms. As demonstrated in this case, bleeding may be initially contained within the
CONCLUSION We presented a case of hepatic artery pseudoaneurysm rupture in a patient with a history of systemic lupus erythematosus and multiple hepatic bilomas. We believe this to be the first such case reported in the medical literature. Hepatic artery pseudoaneurysm rupture not associated with trauma or iatrogenic complication is a rare lifethreatening diagnosis that must be identified and managed quickly in the ED. The diagnosis of visceral artery aneurysm rupture should be considered in any patient presenting with abdominal pain and hemodynamic compromise. CT angiography is the diagnostic modality of choice, as it is rapidly attainable and provides detailed information regarding the anatomy and underlying pathology that exists. Early aggressive resuscitation and
Hepatic Artery Pseudoaneurysm Rupture
coagulopathy reversal with blood products is essential, and definitive management should be primarily with endovascular embolization. REFERENCES 1. Abbas MA, Fowl RJ, Stone WM, et al. Hepatic artery aneurysm: factors that predict complications. J Vasc Surg 2003;38:41–5. 2. Shanley CJ, Shah NL, Messina LM. Common splanchnic artery aneurysms: splenic, hepatic, and celiac. Ann Vasc Surg 1996;10:315–22. 3. Pasha SF, Gloviczki P, Stanson AW, Kamath PS. Splanchnic artery aneurysms. Mayo Clin Proc 2007;82:472–9. 4. Shannon R. Hepatic artery aneurysm. Med J Aust 1991;154:773–4. 5. Arneson MA, Smith RS. Ruptured hepatic artery aneurysm: case report and review of literature. Ann Vasc Surg 2005;19:540–5. 6. Lumsden AB, Mattar SG, Allen RC, Bacha EA. Hepatic artery aneurysms: the management of 22 patients. J Surg Res 1996;60:345–50. 7. Zachary K, Geier S, Pellecchia C, Irwin G. Jaundice secondary to hepatic artery aneurysm: radiological appearance and clinical features. Am J Gastroenterol 1986;81:295–8.
103 8. Wagner WH, Allins AD, Treiman RL, et al. Ruptured visceral artery aneurysms. Ann Vasc Surg 1997;11:342–7. 9. Rogers DM, Thompson JE, Garrett WV, Talkington CM, Patman RD. Mesenteric vascular problems. A 26-year experience. Ann Surg 1982;195:554–65. 10. Zajko AB, Tobben PJ, Esquivel CO, Starzl TE. Pseudoaneurysms following orthotopic liver transplantation: clinical and radiologic manifestations. Transplant Proc 1989;21:2457–9. 11. Worthy SA, Olliff JF, Olliff SP, Buckels JA. Color flow Doppler ultrasound diagnosis of a pseudoaneurysm of the hepatic artery following liver transplantation. J Clin Ultrasound 1994;22:461–5. 12. Falkoff GE, Taylor KJ, Morse S. Hepatic artery pseudoaneurysm: diagnosis with real-time and pulsed Doppler US. Radiology 1986; 158:55–6. 13. Gabelmann A, Gorich J, Merkle EM. Endovascular treatment of visceral artery aneurysms. J Endovasc Ther 2002;9:38–47. 14. Tulsyan N, Kashyap VS, Greenberg RK, et al. The endovascular management of visceral artery aneurysms and pseudoaneurysms. J Vasc Surg 2007;45:276–83. discussion 283. 15. Saltzberg SS, Maldonado TS, Lamparello PJ, et al. Is endovascular therapy the preferred treatment for all visceral artery aneurysms? Ann Vasc Surg 2005;19:507–15.
doi:10.1016/j.jemermed.2011.08.021
Clinical Communications: Adults HEPATIC ARTERY PSEUDOANEURYSM RUPTURE: A CASE REPORT AND REVIEW OF THE LITERATURE Dena A. Reiter, MD,* Aaron M. Fischman, MD,† and Bradley D. Shy, MD* *Department of Emergency Medicine and †Department of Interventional Radiology, Mount Sinai School of Medicine, New York, New York Reprint Address: Dena A. Reiter, MD, Department of Emergency Medicine, Mount Sinai School of Medicine, One Gustave L. Levy Place, Box 1620, New York, NY 10029
, Abstract—Background: Ruptured hepatic artery pseudoaneurysm, a type of visceral artery aneurysm, is a rare condition that is life threatening if not diagnosed and treated rapidly in the emergency department (ED). Patients presenting with this condition require aggressive resuscitation. Endovascular embolization is the first-line treatment option. Objectives: We present a case of spontaneously ruptured hepatic artery pseudoaneurysm and provide a review of the current literature on this topic, focusing on appropriate ED management. Case Report: A 41-year-old woman with a history of systemic lupus erythematosus and multiple hepatic bilomas presented to the ED in critical condition with sudden onset of severe abdominal pain and hemodynamic instability. She was found to have a ruptured hepatic artery pseudoaneurysm with marked hemoperitoneum on computed tomography angiography. She was aggressively resuscitated and successfully managed via endovascular embolization. Conclusion: Ruptured hepatic artery pseudoaneurysm is a life-threatening condition that must be rapidly diagnosed and managed in the ED. Visceral artery aneurysm rupture is a diagnosis that should be considered in any patient presenting to the ED with hemodynamic instability and abdominal pain. Definitive management is with endovascular embolization. Ó 2013 Elsevier Inc.
of all hepatic artery aneurysms is estimated at approximately 0.002%, and approximately 50% of hepatic artery aneurysms are pseudoaneurysms (1,2). Such aneurysms are at high risk of spontaneous rupture, resulting in abdominal vascular catastrophe. Reported mortality rates of any visceral artery aneurysm rupture is estimated between 25% and 70% (1,3). We present a case of ruptured right hepatic artery pseudoaneurysm associated with systemic lupus erythematosus and multiple hepatic bilomas. To our knowledge, no such case has ever been reported in the medical literature.
CASE REPORT A 41-year-old woman with a history of systemic lupus erythematosus, autoimmune hepatitis, primary sclerosing cholangitis, antiphospholipid antibody syndrome, and pulmonary embolus (receiving warfarin therapy) presented to the Emergency Department (ED) reporting sudden-onset severe diffuse abdominal pain. At triage, her vital signs were: temperature 36.0 C, blood pressure 114/71 mm Hg, heart rate 123 beats/min, respiratory rate 20 breaths/min, and oxygen saturation 100% on room air. Shortly after arrival in the ED, the patient had a syncopal episode. After her collapse, we found her minimally responsive with agonal breathing and weak femoral pulses. She quickly regained consciousness with brief bag mask ventilation, at which point she became very agitated, reporting unbearable abdominal and chest pain.
, Keywords—pseudoaneurysm; hemoperitoneum; embolization; resuscitation; critical care
INTRODUCTION Hepatic artery pseudoaneurysm is a rare condition infrequently reported in the medical literature. The incidence
RECEIVED: 10 March 2011; FINAL SUBMISSION RECEIVED: 1 July 2011; ACCEPTED: 28 August 2011 100
Hepatic Artery Pseudoaneurysm Rupture
101
Figure 1. Computed tomography demonstrating right hepatic artery pseudoaneurysm with hepatic capsular rupture (arrow) and extravasation into the peritoneal cavity.
The patient was ill-appearing, agitated, confused, and diaphoretic. Her post-syncope vital signs were: heart rate 126 beats/min, blood pressure 66/43 mm Hg, respiratory rate 25 breaths/min, and oxygen saturation of 100% on an oxygen non-rebreather mask. Her abdomen was nondistended and diffusely tender to palpation. Bedside pregnancy test was negative and initial bedside ultrasound examination did not reveal intraperitoneal free fluid. Fluid resuscitation was started and the patient was hemodynamically stabilized. Immediate computed tomography (CT) angiogram imaging was obtained as there was concern for aortic dissection or rupture; it demonstrated multiple bilomas within the liver, a ruptured right hepatic artery pseudoaneurysm (4.7 cm by 4.8 cm), rupture of the lateral hepatic capsular wall with active extravasation into the biloma and into the peritoneal cavity, and marked hemoperitoneum (Figures 1, 2). Laboratory analyses were notable for a hemoglobin level of 5.9 g/dL, an international normalized ratio of 2.5, and a lactate of 13.3 mmol/L. Blood products (uncross-matched packed red blood cells, fresh frozen plasma, and prothrombin complex concentrate) were started immediately. She was taken to the interventional radiology suite for emergent embolization. A replaced right hepatic artery with descending intrahepatic branch feeding a large pseudoaneurysm with active extravasation was visualized (Figure 3A). She successfully underwent embolization of the right hepatic artery using n-butyl cyanoacrylate glue (Figure 3B). The patient was transferred to the intensive care unit. She remained hemodynamically stable. Her hospital course was complicated by bacteremia. She was discharged home after 28 days. Her warfarin therapy was stopped. At routine follow-up 4 months after discharge, she was alive and well.
Figure 2. Three-dimensional reconstructed image of computed tomography angiography demonstrating right hepatic artery pseudoaneurysm (arrow).
DISCUSSION Ruptured hepatic artery aneurysm is a rare condition with very high mortality. It was first described in 1809 by James Wilson, who conducted an autopsy on a clergyman who had died after its rupture. It was noted to have ‘‘the color, shape and size of the heart’’ (4). Incidence of false aneurysms may be increasing since the advent of percutaneous diagnostic and therapeutic biliary procedures, and detection may also be rising since the advent of CT scanning after trauma (5). Additionally, patients who have undergone liver transplant are at increased risk. There has been an increase in number of reported hepatic artery aneurysms, which may be attributed to increased awareness, improved imaging modalities, and increase in number of percutaneous biliary procedures, as well as liver biopsy and drainage (1,5,6). Overall, there have been fewer than 400 cases reported in the literature, with 80% presenting with rupture as the initial clinical event (5). Of all visceral arterial aneurysms, hepatic aneurysms have the highest reported rate of rupture at 44% (7). Of those that rupture, 50% rupture into the biliary tract, resulting in gastrointestinal hemorrhage (8). Another 20– 30% rupture into the peritoneal cavity (1,2). Although the frequency of death from aneurysmal rupture in humans is unknown, the reported mortality rate once rupture into the peritoneal cavity has occurred is 82% (9).
102
D. A. Reiter et al.
visceral organ structure and may not manifest with intraperitoneal free fluid. Additionally, certain visceral aneurysm ruptures may bleed into the retroperitoneum and be undetectable on bedside ultrasound. In an ED setting involving a critically ill patient, CT angiography is rapidly attainable and provides accurate information regarding the anatomy of visceral aneurysms. It also delineates the extent of extravasation and hemoperitoneum as well as demonstrates any associated intraperitoneal or retroperitoneal pathology. CT angiography may be followed by conventional angiography if a therapeutic intervention is planned. As patients with visceral artery aneurysms are frequently presenting in hemorrhagic shock, aggressive resuscitation with blood products as well as reversal of any underlying coagulopathies is critical. Hemorrhage control is definitive. Presently, endovascular repair is considered first-line treatment of visceral artery aneurysms (13). It allows for more precise location of the aneurysm, accurate assessment of collateral flow, targeting of smaller, more distal vessels, and is minimally invasive (13). Arterial ligation is considered a last resort when embolization fails, as it has a limited success rate with higher morbidity. At one institution, 48 consecutive patients with visceral artery aneurysm (VAA) who underwent endovascular repair were identified between 1997 and 2005. Low morbidity and mortality were noted in the periprocedural period; however, these numbers were higher among patients undergoing emergent repair (8.3% mortality) vs. elective repair (0% mortality) (14). Another institution reported 6% mortality in the endovascular repairs of 65 consecutive patients who presented with VAA (15). Selection bias toward healthier patients may have affected these low mortality numbers; thus, it is important to include both interventional radiology and surgical consultation in determining the best management approach in the ED. Figure 3. (A) Pre-embolization angiogram demonstrating a replaced right hepatic artery with descending intrahepatic branch feeding a large pseudoaneurysm with active extravasation (arrow). (B) Post-embolization angiogram.
There are several imaging modalities available to aid in the diagnosis of hepatic artery aneurysms and pseudoaneurysms. They may be identified using ultrasound with the addition of color Doppler (10–12). However, there are no studies examining the use of bedside ultrasound in the ED to detect such lesions. ED providers may not be sufficiently trained or have resources available at the bedside to perform such studies. Bedside ultrasound is insufficient to detect all ruptured visceral aneurysms. As demonstrated in this case, bleeding may be initially contained within the
CONCLUSION We presented a case of hepatic artery pseudoaneurysm rupture in a patient with a history of systemic lupus erythematosus and multiple hepatic bilomas. We believe this to be the first such case reported in the medical literature. Hepatic artery pseudoaneurysm rupture not associated with trauma or iatrogenic complication is a rare lifethreatening diagnosis that must be identified and managed quickly in the ED. The diagnosis of visceral artery aneurysm rupture should be considered in any patient presenting with abdominal pain and hemodynamic compromise. CT angiography is the diagnostic modality of choice, as it is rapidly attainable and provides detailed information regarding the anatomy and underlying pathology that exists. Early aggressive resuscitation and
Hepatic Artery Pseudoaneurysm Rupture
coagulopathy reversal with blood products is essential, and definitive management should be primarily with endovascular embolization. REFERENCES 1. Abbas MA, Fowl RJ, Stone WM, et al. Hepatic artery aneurysm: factors that predict complications. J Vasc Surg 2003;38:41–5. 2. Shanley CJ, Shah NL, Messina LM. Common splanchnic artery aneurysms: splenic, hepatic, and celiac. Ann Vasc Surg 1996;10:315–22. 3. Pasha SF, Gloviczki P, Stanson AW, Kamath PS. Splanchnic artery aneurysms. Mayo Clin Proc 2007;82:472–9. 4. Shannon R. Hepatic artery aneurysm. Med J Aust 1991;154:773–4. 5. Arneson MA, Smith RS. Ruptured hepatic artery aneurysm: case report and review of literature. Ann Vasc Surg 2005;19:540–5. 6. Lumsden AB, Mattar SG, Allen RC, Bacha EA. Hepatic artery aneurysms: the management of 22 patients. J Surg Res 1996;60:345–50. 7. Zachary K, Geier S, Pellecchia C, Irwin G. Jaundice secondary to hepatic artery aneurysm: radiological appearance and clinical features. Am J Gastroenterol 1986;81:295–8.
103 8. Wagner WH, Allins AD, Treiman RL, et al. Ruptured visceral artery aneurysms. Ann Vasc Surg 1997;11:342–7. 9. Rogers DM, Thompson JE, Garrett WV, Talkington CM, Patman RD. Mesenteric vascular problems. A 26-year experience. Ann Surg 1982;195:554–65. 10. Zajko AB, Tobben PJ, Esquivel CO, Starzl TE. Pseudoaneurysms following orthotopic liver transplantation: clinical and radiologic manifestations. Transplant Proc 1989;21:2457–9. 11. Worthy SA, Olliff JF, Olliff SP, Buckels JA. Color flow Doppler ultrasound diagnosis of a pseudoaneurysm of the hepatic artery following liver transplantation. J Clin Ultrasound 1994;22:461–5. 12. Falkoff GE, Taylor KJ, Morse S. Hepatic artery pseudoaneurysm: diagnosis with real-time and pulsed Doppler US. Radiology 1986; 158:55–6. 13. Gabelmann A, Gorich J, Merkle EM. Endovascular treatment of visceral artery aneurysms. J Endovasc Ther 2002;9:38–47. 14. Tulsyan N, Kashyap VS, Greenberg RK, et al. The endovascular management of visceral artery aneurysms and pseudoaneurysms. J Vasc Surg 2007;45:276–83. discussion 283. 15. Saltzberg SS, Maldonado TS, Lamparello PJ, et al. Is endovascular therapy the preferred treatment for all visceral artery aneurysms? Ann Vasc Surg 2005;19:507–15.