Ruptured Abdominal Aortic Aneurysm in a Patient with an AortoeLeft Renal Vein Fistula Vincent van Weel,1 Alex Watson,2 and Bram Fioole,1 Rotterdam, The Netherlands, and Newcastle upon Tyne, United Kingdom
The combination of an aortoeleft renal vein fistula (ALRVF) and a ruptured abdominal aortic aneurysm is extremely rare, with only 9 cases having been reported. We present such a case and discuss the previously reported cases and treatment strategies.
A 71-year-old woman with a history of hypertension presented to our emergency department with a ruptured abdominal aortic aneurysm (AAA). On arrival, she was hypotensive and complained of sudden-onset back pain. Physical examination revealed a pulsatile expansile abdominal mass with palpable femoral pulses. Laboratory test results included hemoglobin of 7.2 mmol/L (reference 7.5e10 mmol/L) and elevated serum creatinine (142 mmol/L, reference 49e90 mmol/L) with a renal clearance of 32 mL/min (reference >90 mL/min). A subsequent computed tomography angiogram (CTA) revealed a contained rupture of an infrarenal AAA, maximum diameter of 85 mm, with an associated retroperitoneal hematoma. In addition, there was an aortoeleft renal vein fistula (ALRVF) clearly visible by contrast within the retroaortic left renal vein (LRV), vena cava, ovarian veins, and venous uterine plexus. Furthermore, contrast enhancement was diminished in the left kidney (Fig. 1 and 2). The aneurysm neck was conical with a diameter at the level of the renal arteries of 24 mm, increasing 1 Department of Vascular Surgery, Maasstad Hospital, Rotterdam, The Netherlands. 2 Department of Vascular Surgery, Freeman Hospital, Newcastle upon Tyne, United Kingdom.
Correspondence to: Vincent van Weel, MD, Maasstad Hospital, Maasstadweg 21, P.O. Box 9100, 3007 AC Rotterdam, The Netherlands; E-mail: [email protected] Ann Vasc Surg 2014; 28: 493.e11–493.e13 http://dx.doi.org/10.1016/j.avsg.2013.03.021 Ó 2014 Published by Elsevier Inc. Manuscript received: January 14, 2013; manuscript accepted: March 6, 2013; published online: December 20, 2013.
to 31 mm within the first 10 mm, contraindicating endovascular repair. Due to increasing patient instability an emergency open aortic repair was immediately undertaken. Major venous bleeding was encountered after opening of the aneurysmal sac from the retroaortic LRV fistula, which was eventually controlled by extensive under-running from within the sac. A separate point of rupture into the left retroperitoneal space was also demonstrated. Aortic continuity was restored with a bifurcated Dacron graft (16 8 mm; Vascutek, Renfrewshire, Scotland, UK) with end-to-end anastomoses onto the common iliac arteries. Total blood loss was 8 L (2.2 L returned with cell saver) and the postoperative course was complicated by sigmoidal ischemia, requiring Hartmann’s procedure. Unfortunately, the patient died 10 days after the first operation due to multiorgan failure. An ALRVF is extremely rare. Only 30 cases have been reported in the English literature,1,2 including 9 patients with a ruptured AAA (Table I).3e10 In >90% of cases a retroaortic LRV was observed. Retroaortic LRV is present in 2e4% of the population.11 During embryonic development of the caval and renal system a circumaortic venous collar develops. Most commonly, subsequent disintegration of the postaortic component of this renal collar results in the usual preaortic LRV; however, disintegration of the preaortic component results in a retroaortic LRV. Even rarer, both pre- and postaortic components of the collar may persist or two preaortic LRVs may develop. As observed in our case, a retroaortic LRV has a unique downward course joining the vena cava 493.e11
493.e12 Case reports
Annals of Vascular Surgery
Fig. 1. CTA, transverse image: ruptured abdominal aortic aneurysm (1) with retroperitoneal hematoma and opacification of the retroaortic left renal vein (2); aortoeleft renal vein fistula (3); and vena cava (4). Note the reduced contrast enhancement of the left kidney (5).
significantly lower than the right renal vein.12 Some investigators proposed a mechanism of spontaneous rupture of the aneurysm into the venous system4; however, in the patient described in this study, the fistula and rupture occurred in two separate locations. Moreover, most patients with an ALRVF do not present with a ruptured aneurysm and it is our opinion that the fistula and rupture are two separate entities. An ALRVF may present with hematuria, renal impairment, abdominal or left flank pain, and reduced enhancement of the left kidney on CT angiographic images (‘‘silent left kidney’’). This is all due to the effect of venous hypertension on the left kidney.11 In male patients, scrotal edema or left varicocele may result from venous overload in the scrotal pampiniform venous plexus via the left gonadal vein. In the literature to date, AAAs combined with an ALRVF were most commonly treated with open aortic repair. Investigators have described control of bleeding from the ALRVF by digital compression of the LRV and suturing of the fistula from within the aneurysm. We found that this can be technically challenging in patients with a ruptured AAA due to the edematous and brittle retroperitoneal tissues resulting from compression-related erosion and retroperitoneal hematoma. In our patient this resulted in major blood loss. Similar technical difficulties have been described by others.5 The dorsal course of the LRV makes it difficult to control the LRV prior to opening the aneurysmal sac, especially in cases of ruptured aneurysm with retroperitoneal hematoma. In an elective setting, obtaining control
Fig. 2. CTA, three-dimensional reconstruction. Opacification of the retroaortic left renal vein (1), vena cava (2), ovarian veins (3), and venous uterine plexus (4).
of the LRV either by vessel loops or endovascular balloon occlusion may be favorable. In our patient, endovascular repair was not performed due to an unfavorable proximal sealing zone (short, conical aneurysm neck). If anatomically suitable, endovascular repair may provide an alternative to prevent bleeding complications. This has been described in 2 patients with a ruptured AAA and ALRVF.6,7 Although initially technically successful, both cases required reintervention due to type 2 endoleaks. Gabrielli and colleagues suggested that proximity of the arteriovenous fistula to the renal arteries increases the risk of type 1 endoleak because it could narrow the sealing and fixation zone of the stent.9 However, to the best of our knowledge, no such case has been described. Due to the downward course of a retroaortic LRV, persistent leakage from the fistula is more likely to result in a type 2 endoleak, as described elsewhere.6,7 The type 2 endoleaks in these cases were
Vol. 28, No. 2, February 2014
Case reports 493.e13
Table I. Published cases of aortoeleft renal vein fistula and ruptured AAA and the treatment administered First No investigator Year cases Treatment
Hoballah 1993 1 et al.3 Thompson 1993 1 et al.4
Reintervention
Open repair None Open repair None (preaortic LRV) Open repair Patient died
Masood et al.5 Sultan et al.6
1994 1
Resnick et al.7
2006 1
Gabrielli et al.8 Gabrielli et al.9 Istvanic et al.10
2009 1
Conversion to open repair (type 2 endoleak) Translumbar EVAR coiling of IMA (Gore and AV fistula Excluder) (type 2 endoleak) Open repair None
2011 2
Open repair None
2012 1
Not reported Not reported
1999 1
If anatomically and hemodynamically suitable, endovascular repair is an attractive and feasible alternative.
EVAR (Talent)
IMA, inferior mesenteric artery; AV, arteriovenous.
successfully treated by delayed embolization of the lumbar arteries, inferior mesenteric artery, and the flow channel within the aneurysmal sac,7 and by conversion to open repair in an elective setting.6 In a recent systematic review, the outcome of endovascular stent-graft repair of major abdominal arteriovenous fistulas (communication with caval, iliac, or renal segments) was evaluated.13 It showed that the most common procedure-related complication was a type 2 endoleak, which was found in 22% (5 of 23) of patients. This condition was either self-limiting or required minimal percutaneous intervention. In conclusion, AAA rupture associated with ALRVF is extremely rare. Preoperative recognition of ALRVF by the vascular surgeon is of the utmost importance as controlling bleeding from the ALRVF from within the aneurysmal sac may be challenging.
REFERENCES 1. Dragas M, Davidovic L, Pejkic S, et al. Aorto-left renal vein fistula is a rare complication of abdominal aortic aneurysm with unique clinical presentation. J Vasc Surg 2010;52: 1658e61. 2. Barrier P, Otal P, Garcia O, et al. Aorta-left renal vein fistula complicating an aortic aneurysm: preoperative and postoperative multislice CT findings. Cardiovasc Intervent Radiol 2007;30:485e7. 3. Hoballah JJ, Chalmers RT, Sharp WJ, et al. Aortic aneurysm rupture into a retroaortic left renal vein. Ann Vasc Surg 1993;7:363e7. 4. Thompson RW, Yee LF, Natuzzi ES, et al. Aorta-left renal vein fistula syndrome caused by rupture of a juxtarenal abdominal aortic aneurysm: novel pathologic mechanism for a unique clinical entity. J Vasc Surg 1993;18:310e5. 5. Masood T, Naylor AR, Edge JM, et al. Double aortovenous fistula: a unique presentation of a ruptured abdominal aortic aneurysm. Eur J Vasc Surg 1994;8:107e9. 6. Sultan S, Madhavan P, Colgan MP, et al. Aorto-left renal vein fistula: is there a place for endovascular management? J Endovasc Surg 1999;6:375e7. 7. Resnick S, Rome V, Eskandari M, et al. Translumbar repair of a type II endoleak associated with an aorta-to-left renal vein fistula after aortic stent-graft placement. J Vasc Interv Radiol 2006;17:557e61. 8. Gabrielli R, Rosati MS, Irace L, et al. Rupture of abdominal aortic aneurysm into retro-aortic left renal vein: a case report. Eur J Vasc Endovasc Surg 2009;18:21e3. 9. Gabrielli R, Rosati MS, Siani A, et al. Preoperative evaluation of retroperitoneal venous system anomalies during abdominal aortic aneurysm rupture. Interact Cardiovasc Thorac Surg 2011;12:278e80. 10. Istvanic T, Vizjak V, Saric G, et al. Rupture of abdominal aortic aneurysm with fistula between the aorta and the confluence of the left renal vein into the vena cava. Images in cardiovascular medicine. Tex Heart Inst J 2012;39:148e9. 11. Mansour MA, Rutherford RB, Metcalf RK, et al. Spontaneous aorto-left renal vein fistula: the ‘‘abdominal pain, haematuria, silent left kidney’’ syndrome. Surgery 1991;109:101e6. 12. Skandalakis JE, Colborn GL, Weidman TA, et al. Surgical Anatomy. The Embryologic and Anatomic Basis of Modern Surgery. Vol 2. Athens, Greece: Paschalidis, 2004. pp 1307e10. 13. Antoniou GA, Koutsias S, Karathanos C, et al. endovascular stent-graft repair of major abdominal arteriovenous fistula: a systematic review. J Endovasc Ther 2009;16:514e23.
The combination of an aortoeleft renal vein fistula (ALRVF) and a ruptured abdominal aortic aneurysm is extremely rare, with only 9 cases having been reported. We present such a case and discuss the previously reported cases and treatment strategies.
A 71-year-old woman with a history of hypertension presented to our emergency department with a ruptured abdominal aortic aneurysm (AAA). On arrival, she was hypotensive and complained of sudden-onset back pain. Physical examination revealed a pulsatile expansile abdominal mass with palpable femoral pulses. Laboratory test results included hemoglobin of 7.2 mmol/L (reference 7.5e10 mmol/L) and elevated serum creatinine (142 mmol/L, reference 49e90 mmol/L) with a renal clearance of 32 mL/min (reference >90 mL/min). A subsequent computed tomography angiogram (CTA) revealed a contained rupture of an infrarenal AAA, maximum diameter of 85 mm, with an associated retroperitoneal hematoma. In addition, there was an aortoeleft renal vein fistula (ALRVF) clearly visible by contrast within the retroaortic left renal vein (LRV), vena cava, ovarian veins, and venous uterine plexus. Furthermore, contrast enhancement was diminished in the left kidney (Fig. 1 and 2). The aneurysm neck was conical with a diameter at the level of the renal arteries of 24 mm, increasing 1 Department of Vascular Surgery, Maasstad Hospital, Rotterdam, The Netherlands. 2 Department of Vascular Surgery, Freeman Hospital, Newcastle upon Tyne, United Kingdom.
Correspondence to: Vincent van Weel, MD, Maasstad Hospital, Maasstadweg 21, P.O. Box 9100, 3007 AC Rotterdam, The Netherlands; E-mail: [email protected] Ann Vasc Surg 2014; 28: 493.e11–493.e13 http://dx.doi.org/10.1016/j.avsg.2013.03.021 Ó 2014 Published by Elsevier Inc. Manuscript received: January 14, 2013; manuscript accepted: March 6, 2013; published online: December 20, 2013.
to 31 mm within the first 10 mm, contraindicating endovascular repair. Due to increasing patient instability an emergency open aortic repair was immediately undertaken. Major venous bleeding was encountered after opening of the aneurysmal sac from the retroaortic LRV fistula, which was eventually controlled by extensive under-running from within the sac. A separate point of rupture into the left retroperitoneal space was also demonstrated. Aortic continuity was restored with a bifurcated Dacron graft (16 8 mm; Vascutek, Renfrewshire, Scotland, UK) with end-to-end anastomoses onto the common iliac arteries. Total blood loss was 8 L (2.2 L returned with cell saver) and the postoperative course was complicated by sigmoidal ischemia, requiring Hartmann’s procedure. Unfortunately, the patient died 10 days after the first operation due to multiorgan failure. An ALRVF is extremely rare. Only 30 cases have been reported in the English literature,1,2 including 9 patients with a ruptured AAA (Table I).3e10 In >90% of cases a retroaortic LRV was observed. Retroaortic LRV is present in 2e4% of the population.11 During embryonic development of the caval and renal system a circumaortic venous collar develops. Most commonly, subsequent disintegration of the postaortic component of this renal collar results in the usual preaortic LRV; however, disintegration of the preaortic component results in a retroaortic LRV. Even rarer, both pre- and postaortic components of the collar may persist or two preaortic LRVs may develop. As observed in our case, a retroaortic LRV has a unique downward course joining the vena cava 493.e11
493.e12 Case reports
Annals of Vascular Surgery
Fig. 1. CTA, transverse image: ruptured abdominal aortic aneurysm (1) with retroperitoneal hematoma and opacification of the retroaortic left renal vein (2); aortoeleft renal vein fistula (3); and vena cava (4). Note the reduced contrast enhancement of the left kidney (5).
significantly lower than the right renal vein.12 Some investigators proposed a mechanism of spontaneous rupture of the aneurysm into the venous system4; however, in the patient described in this study, the fistula and rupture occurred in two separate locations. Moreover, most patients with an ALRVF do not present with a ruptured aneurysm and it is our opinion that the fistula and rupture are two separate entities. An ALRVF may present with hematuria, renal impairment, abdominal or left flank pain, and reduced enhancement of the left kidney on CT angiographic images (‘‘silent left kidney’’). This is all due to the effect of venous hypertension on the left kidney.11 In male patients, scrotal edema or left varicocele may result from venous overload in the scrotal pampiniform venous plexus via the left gonadal vein. In the literature to date, AAAs combined with an ALRVF were most commonly treated with open aortic repair. Investigators have described control of bleeding from the ALRVF by digital compression of the LRV and suturing of the fistula from within the aneurysm. We found that this can be technically challenging in patients with a ruptured AAA due to the edematous and brittle retroperitoneal tissues resulting from compression-related erosion and retroperitoneal hematoma. In our patient this resulted in major blood loss. Similar technical difficulties have been described by others.5 The dorsal course of the LRV makes it difficult to control the LRV prior to opening the aneurysmal sac, especially in cases of ruptured aneurysm with retroperitoneal hematoma. In an elective setting, obtaining control
Fig. 2. CTA, three-dimensional reconstruction. Opacification of the retroaortic left renal vein (1), vena cava (2), ovarian veins (3), and venous uterine plexus (4).
of the LRV either by vessel loops or endovascular balloon occlusion may be favorable. In our patient, endovascular repair was not performed due to an unfavorable proximal sealing zone (short, conical aneurysm neck). If anatomically suitable, endovascular repair may provide an alternative to prevent bleeding complications. This has been described in 2 patients with a ruptured AAA and ALRVF.6,7 Although initially technically successful, both cases required reintervention due to type 2 endoleaks. Gabrielli and colleagues suggested that proximity of the arteriovenous fistula to the renal arteries increases the risk of type 1 endoleak because it could narrow the sealing and fixation zone of the stent.9 However, to the best of our knowledge, no such case has been described. Due to the downward course of a retroaortic LRV, persistent leakage from the fistula is more likely to result in a type 2 endoleak, as described elsewhere.6,7 The type 2 endoleaks in these cases were
Vol. 28, No. 2, February 2014
Case reports 493.e13
Table I. Published cases of aortoeleft renal vein fistula and ruptured AAA and the treatment administered First No investigator Year cases Treatment
Hoballah 1993 1 et al.3 Thompson 1993 1 et al.4
Reintervention
Open repair None Open repair None (preaortic LRV) Open repair Patient died
Masood et al.5 Sultan et al.6
1994 1
Resnick et al.7
2006 1
Gabrielli et al.8 Gabrielli et al.9 Istvanic et al.10
2009 1
Conversion to open repair (type 2 endoleak) Translumbar EVAR coiling of IMA (Gore and AV fistula Excluder) (type 2 endoleak) Open repair None
2011 2
Open repair None
2012 1
Not reported Not reported
1999 1
If anatomically and hemodynamically suitable, endovascular repair is an attractive and feasible alternative.
EVAR (Talent)
IMA, inferior mesenteric artery; AV, arteriovenous.
successfully treated by delayed embolization of the lumbar arteries, inferior mesenteric artery, and the flow channel within the aneurysmal sac,7 and by conversion to open repair in an elective setting.6 In a recent systematic review, the outcome of endovascular stent-graft repair of major abdominal arteriovenous fistulas (communication with caval, iliac, or renal segments) was evaluated.13 It showed that the most common procedure-related complication was a type 2 endoleak, which was found in 22% (5 of 23) of patients. This condition was either self-limiting or required minimal percutaneous intervention. In conclusion, AAA rupture associated with ALRVF is extremely rare. Preoperative recognition of ALRVF by the vascular surgeon is of the utmost importance as controlling bleeding from the ALRVF from within the aneurysmal sac may be challenging.
REFERENCES 1. Dragas M, Davidovic L, Pejkic S, et al. Aorto-left renal vein fistula is a rare complication of abdominal aortic aneurysm with unique clinical presentation. J Vasc Surg 2010;52: 1658e61. 2. Barrier P, Otal P, Garcia O, et al. Aorta-left renal vein fistula complicating an aortic aneurysm: preoperative and postoperative multislice CT findings. Cardiovasc Intervent Radiol 2007;30:485e7. 3. Hoballah JJ, Chalmers RT, Sharp WJ, et al. Aortic aneurysm rupture into a retroaortic left renal vein. Ann Vasc Surg 1993;7:363e7. 4. Thompson RW, Yee LF, Natuzzi ES, et al. Aorta-left renal vein fistula syndrome caused by rupture of a juxtarenal abdominal aortic aneurysm: novel pathologic mechanism for a unique clinical entity. J Vasc Surg 1993;18:310e5. 5. Masood T, Naylor AR, Edge JM, et al. Double aortovenous fistula: a unique presentation of a ruptured abdominal aortic aneurysm. Eur J Vasc Surg 1994;8:107e9. 6. Sultan S, Madhavan P, Colgan MP, et al. Aorto-left renal vein fistula: is there a place for endovascular management? J Endovasc Surg 1999;6:375e7. 7. Resnick S, Rome V, Eskandari M, et al. Translumbar repair of a type II endoleak associated with an aorta-to-left renal vein fistula after aortic stent-graft placement. J Vasc Interv Radiol 2006;17:557e61. 8. Gabrielli R, Rosati MS, Irace L, et al. Rupture of abdominal aortic aneurysm into retro-aortic left renal vein: a case report. Eur J Vasc Endovasc Surg 2009;18:21e3. 9. Gabrielli R, Rosati MS, Siani A, et al. Preoperative evaluation of retroperitoneal venous system anomalies during abdominal aortic aneurysm rupture. Interact Cardiovasc Thorac Surg 2011;12:278e80. 10. Istvanic T, Vizjak V, Saric G, et al. Rupture of abdominal aortic aneurysm with fistula between the aorta and the confluence of the left renal vein into the vena cava. Images in cardiovascular medicine. Tex Heart Inst J 2012;39:148e9. 11. Mansour MA, Rutherford RB, Metcalf RK, et al. Spontaneous aorto-left renal vein fistula: the ‘‘abdominal pain, haematuria, silent left kidney’’ syndrome. Surgery 1991;109:101e6. 12. Skandalakis JE, Colborn GL, Weidman TA, et al. Surgical Anatomy. The Embryologic and Anatomic Basis of Modern Surgery. Vol 2. Athens, Greece: Paschalidis, 2004. pp 1307e10. 13. Antoniou GA, Koutsias S, Karathanos C, et al. endovascular stent-graft repair of major abdominal arteriovenous fistula: a systematic review. J Endovasc Ther 2009;16:514e23.
