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Leriche Syndrome in a Patient with Situs Inversus Totalis
Leriche Syndrome in a Patient with Situs Inversus Totalis Nelson Oliveira, Fernando Oliveira, Emanuel Dias, Lisa Borges, and Isabel Cassio, S~ao Miguel Azores, Portugal
Situs inversus is a rare congenital defect defined by a mirror image anatomic variation of the thoracic and abdominal organs. In this situation, abdominal aortic surgery may become particularly challenging. This is the case of a 51-year-old man, who presented with an incapacitating lower limb claudication. On his workup, a complete occlusion of the infrarenal aorta requiring surgical revascularization was found. Additionally, a situs inversus totalis was identified along with a single horseshoe kidney. The patient underwent uneventfully a surgical aortobifemoral interposition. The sole modification to the standard procedure regarded the graft limbs tunneling, which was performed in a prerenal pathway in the retroperitoneal space, thus avoiding iatrogenic injury to the anteriorly positioned iliac veins. Although challenging, conventional aortic surgery can be safely accomplished in patients with situs inversus totalis.
Situs inversus is a rare congenital defect with a prevalence of 0.01% which arises when malrotation of the thoracic and abdominal viscera occurs. Consequently, these viscera become disposed in a mirrored position compared to normal anatomy. Additionally, this condition may be associated with other anatomic variations including horseshoe kidney.1,2 Generally, situs inversus is only identified when a clinical or imaging workup is performed for unrelated reasons. Seldom has situs inversus been reported in patients with aortic pathology requiring intervention, and conventional surgery may be challenging because of the altered anatomy.
CASE REPORT This is the case of a 51-year-old man with a previous medical history notorious for smoking habits,
Conflicts of Interest: The authors have no conflicts of interest to report. Department of Angiology and Vascular Surgery, Divino Espı´rito Santo HospitalePonta Delgada, S~ao Miguel Azores, Portugal. Correspondence to: Nelson Oliveira, MD, Hospital do Divino Espı´rito Santo, Rua D. Manuel I, 9500 Ponta Delgada, S~ao Miguel Azores, Portugal; E-mail: [email protected] Ann Vasc Surg 2015; 29: 1317.e5–1317.e8 http://dx.doi.org/10.1016/j.avsg.2015.02.023 Ó 2015 Elsevier Inc. All rights reserved. Manuscript received: November 2, 2014; manuscript accepted: February 1, 2015; published online: May 29, 2015.
hypercholesterolemia, systemic hypertension, and a previous embolic stroke without sequelae, presumably related to a patent foramen ovale. For this reason, the patient was receiving oral anticoagulation with a vitamin K antagonist (warfarin) and followed by cardiology. The patient was referred to our department with an incapacitating bilateral gluteal and calf claudication (Rutherford class 3). Noteworthy on the physical examination was the absence of lower limb pulses. Thermal gradients, cyanosis, or trophic lesions such as ulcers or necrosis were not present. On the imaging workup, a computerized tomography angiography identified an infrarenal aortic thrombosis extending to the common and external iliac arteries bilaterally. Additionally, a situs inversus totalis and a horseshoe kidney (Figs. 1 and 2) were also found. The initial approach consisted of conservative medical treatment and cardiovascular risk factor correction. However, after a year of follow-up, the patient was incapable of answering to his professional demands, and therefore, a conventional surgical revascularization was offered. A digital subtraction angiography was performed preoperatively confirming the previously described arterial findings. Both common femoral arteries were patent, and no significant infrainguinal occlusive disease was noted (Fig. 3). After obtaining informed consent, the patient underwent an aortobifemoral interposition through a standard transperitoneal approach under full-dose 1317.e5
1317.e6 Case reports
Fig. 1. Computerized tomography angiography reconstruction demonstrating an infrarenal aortic thrombosis in association to situs inversus totalis. Note that the inferior vena cava and the iliac veins assume an anterior position relatively to the aorta and iliac arteries (white arrow).
anticoagulation with a low-weight heparin. Because of the anterior position of the inferior vena cava along with the iliac veins relatively to the arterial axis (Fig. 1, white arrow) and the presence of the horseshoe kidney, the graft limbs were tunneled anteriorly to the kidney and followed a more superficial pathway in the retroperitoneal space, anterior to the ureter (Fig. 4). No hemorrhagic complications occurred, and no blood transfusions were required during hospitalization. After 6 days of hospitalization, the patient was discharged with palpable pedal and posterior tibial pulses. After 3-month follow-up, the patient has resumed his professional activity without any restrains and remains with all lower limb pulses palpable and symmetrical.
DISCUSSION Situs inversus is a rare congenital defect which has been reported in 1 in every 8,000e25,000 births.3
Annals of Vascular Surgery
Although in this anomaly all internal organs are reversely disposed in a mirror image to normal anatomy, this entity only becomes clinically relevant when other complications occur. Other congenital anomalies associated to situs inversus include cardiac abnormalities (more commonly atrioventricular discordance, transpositions complexes), Kartagener syndrome (immobile cilia syndrome) and related upper and lower respiratory tract defects (bronchiectasis and abnormal paranasal sinuses),4 urinary anomalies (dysplasia, hypoplasia, agenesis, ectopia, horseshoe kidney, polycystic kidney),5 and venous anomalies (anomalous inferior vena cava and azygos vein continuation, inferior vena cava coarctation, renal vein abnormalities).6e8 The association of situs inversus totalis and abdominal aortic pathology has rarely been reported. Most cases refer to patients presenting with aortic aneurysms,9e12 but a seldom report of aortic occlusive disease has also been published.13 Open aortic surgery has been feared in patients with situs inversus as the unfamiliar anatomy may be associated to an increased risk of intraoperative complications.9 Alternative approaches have been preferred by some authors as reported by Chan et al.11 who chose to perform an endovascular aneurysm repair in their patient because of the presence of situs inversus. However, an increased risk of perioperative complications is not found among the literature when adequate preoperative planning is performed.9e12,14,15 Open surgery remains the gold standard for the treatment of Leriche syndrome, and anatomic revascularization has proven to have increased patency compared to extra-anatomic bypasses. Despite requiring systemic anticoagulation throughout the perioperative period and presenting unfamiliar anatomic features, an aortobifemoral interposition was the preferred treatment for our patient. In our view, a correct preoperative planning of both the surgical approach and pathway for graft tunneling were the key to avoiding any intraoperative complications with the consequent favorable outcome that our patient presented. Open surgery is the gold standard for the treatment of infrarenal aortic thrombosis and can be safely performed in patients with situs inversus totalis. The association of abdominal aortic thrombosis with situs inversus has seldom been reported.
Vol. 29, No. 6, August 2015
Case reports 1317.e7
Fig. 2. (A, D) Computerized tomography angiography demonstrating the infrarenal aortic thrombosis in association to situs inversus totalis. A single horseshoe kidney located over the aortic bifurcation can be depicted (C, D).
REFERENCES
Fig. 3. Digital subtraction angiography confirming the aortic occlusion. A single renal artery is depicted and an exuberant Roland arch originating from the superior mesenteric artery.
1. Yokoyama T, Copeland NG, Jenkins NA, et al. Reversal of left-right asymmetry: a situs inversus mutation. Science 1993;260:679e82. 2. Matsusshita K, Veda S, Kegami K. Horseshoe kidney in a patient with situs inversus totalis. J Urol 1982;128: 604e5. 3. Belmonte JC. How the body tells left from right. Sci Am 1999;280:46e51. 4. Gutgesell HP. Cardiac malposition and heterotaxia. In: Garson A, Jr, Bricker JT, McNamara DG eds. The Science and Practice of Pediatric Cardiology. Philadelphia: Lea & Febiger, 1990. pp 1280e303. 5. Treiger BFG, Khazan R, Goldman SM, et al. Renal cell carcinoma with situs inversus totalis. Urology 1993;41: 455e7. 6. Beppu H, Matsushita S, Kimata S, et al. A case of anomalous inferior vena cava with azygos continuation associated with situs inversus totalis and acquired mitral valvular disease. Jpn Heart J 1967;8:428e32. 7. Victor S, Jayanthi V, Vallal TT, et al. Coarctation of inferior vena cava in situs inversus totalis. Indian J Gastroenterol 1992;11:89. 8. Dupont JR. Isolated left-sided vena cava and abdominal aortic aneurysm. Arch Surg 1971;102:211e2.
1317.e8 Case reports
Annals of Vascular Surgery
Fig. 4. (A) Intraoperative field with after aortic bifemoral interposition with a woven-polyester bifurcated graft (Unigraft 16 8mm). (B) Detail of the prerenal course of the graft in the retroperitoneum. The graft
limbs were tunneled in a superficial pathway in the retroperitoneal space avoiding iatrogenic injury to the iliac veins which were found in an anterior position relatively to the iliac arteries.
9. Kato T, Takagi H, Sekino S, et al. Abdominal aortic aneurysm in situs inversus totalis. Ann Vasc Surg 2006;20:267e70. 10. Baccellieri D, Mirenda F, Mandolfino T, et al. Symptomatic aorto-iliac aneurysm and situs viscerum inversus: case report. Chir Ital 2006;58:113e6. 11. Chan YC, Ting AC, Qing KX, et al. Successful endovascular infrarenal aneurysm repair in a patient with situs inversus totalis. Ann Vasc Surg 2010;24:1134.e5e7. 12. Huston I, Nicholas G. Situs inversus totalis and an infrarenal abdominal aortic aneurysm: case report. Vasc Endovascular Surg 1990;24:372e4.
13. Rapovka G. Occlusion of the infrarenal segment of the aorta in a patient with total situs inversus of the internal organs. Khirurgiia (Mosk) 1989;137e8. 14. Kimura N, Kawahito K, Ito S. Rupture of an abdominal aortic aneurysm in a patient with situs inversus totalis: report of a case. Surg Today 2008;38: 249e52. 15. Occhionorelli S, Navarra G, Santini M, et al. Symptomatic abdominal aortic aneurysm and ‘‘situs viscerum inversus.’’ Diagnostic and therapeutic approach. J Cardiovasc Surg (Torino) 1998;39:31e3.
Situs inversus is a rare congenital defect defined by a mirror image anatomic variation of the thoracic and abdominal organs. In this situation, abdominal aortic surgery may become particularly challenging. This is the case of a 51-year-old man, who presented with an incapacitating lower limb claudication. On his workup, a complete occlusion of the infrarenal aorta requiring surgical revascularization was found. Additionally, a situs inversus totalis was identified along with a single horseshoe kidney. The patient underwent uneventfully a surgical aortobifemoral interposition. The sole modification to the standard procedure regarded the graft limbs tunneling, which was performed in a prerenal pathway in the retroperitoneal space, thus avoiding iatrogenic injury to the anteriorly positioned iliac veins. Although challenging, conventional aortic surgery can be safely accomplished in patients with situs inversus totalis.
Situs inversus is a rare congenital defect with a prevalence of 0.01% which arises when malrotation of the thoracic and abdominal viscera occurs. Consequently, these viscera become disposed in a mirrored position compared to normal anatomy. Additionally, this condition may be associated with other anatomic variations including horseshoe kidney.1,2 Generally, situs inversus is only identified when a clinical or imaging workup is performed for unrelated reasons. Seldom has situs inversus been reported in patients with aortic pathology requiring intervention, and conventional surgery may be challenging because of the altered anatomy.
CASE REPORT This is the case of a 51-year-old man with a previous medical history notorious for smoking habits,
Conflicts of Interest: The authors have no conflicts of interest to report. Department of Angiology and Vascular Surgery, Divino Espı´rito Santo HospitalePonta Delgada, S~ao Miguel Azores, Portugal. Correspondence to: Nelson Oliveira, MD, Hospital do Divino Espı´rito Santo, Rua D. Manuel I, 9500 Ponta Delgada, S~ao Miguel Azores, Portugal; E-mail: [email protected] Ann Vasc Surg 2015; 29: 1317.e5–1317.e8 http://dx.doi.org/10.1016/j.avsg.2015.02.023 Ó 2015 Elsevier Inc. All rights reserved. Manuscript received: November 2, 2014; manuscript accepted: February 1, 2015; published online: May 29, 2015.
hypercholesterolemia, systemic hypertension, and a previous embolic stroke without sequelae, presumably related to a patent foramen ovale. For this reason, the patient was receiving oral anticoagulation with a vitamin K antagonist (warfarin) and followed by cardiology. The patient was referred to our department with an incapacitating bilateral gluteal and calf claudication (Rutherford class 3). Noteworthy on the physical examination was the absence of lower limb pulses. Thermal gradients, cyanosis, or trophic lesions such as ulcers or necrosis were not present. On the imaging workup, a computerized tomography angiography identified an infrarenal aortic thrombosis extending to the common and external iliac arteries bilaterally. Additionally, a situs inversus totalis and a horseshoe kidney (Figs. 1 and 2) were also found. The initial approach consisted of conservative medical treatment and cardiovascular risk factor correction. However, after a year of follow-up, the patient was incapable of answering to his professional demands, and therefore, a conventional surgical revascularization was offered. A digital subtraction angiography was performed preoperatively confirming the previously described arterial findings. Both common femoral arteries were patent, and no significant infrainguinal occlusive disease was noted (Fig. 3). After obtaining informed consent, the patient underwent an aortobifemoral interposition through a standard transperitoneal approach under full-dose 1317.e5
1317.e6 Case reports
Fig. 1. Computerized tomography angiography reconstruction demonstrating an infrarenal aortic thrombosis in association to situs inversus totalis. Note that the inferior vena cava and the iliac veins assume an anterior position relatively to the aorta and iliac arteries (white arrow).
anticoagulation with a low-weight heparin. Because of the anterior position of the inferior vena cava along with the iliac veins relatively to the arterial axis (Fig. 1, white arrow) and the presence of the horseshoe kidney, the graft limbs were tunneled anteriorly to the kidney and followed a more superficial pathway in the retroperitoneal space, anterior to the ureter (Fig. 4). No hemorrhagic complications occurred, and no blood transfusions were required during hospitalization. After 6 days of hospitalization, the patient was discharged with palpable pedal and posterior tibial pulses. After 3-month follow-up, the patient has resumed his professional activity without any restrains and remains with all lower limb pulses palpable and symmetrical.
DISCUSSION Situs inversus is a rare congenital defect which has been reported in 1 in every 8,000e25,000 births.3
Annals of Vascular Surgery
Although in this anomaly all internal organs are reversely disposed in a mirror image to normal anatomy, this entity only becomes clinically relevant when other complications occur. Other congenital anomalies associated to situs inversus include cardiac abnormalities (more commonly atrioventricular discordance, transpositions complexes), Kartagener syndrome (immobile cilia syndrome) and related upper and lower respiratory tract defects (bronchiectasis and abnormal paranasal sinuses),4 urinary anomalies (dysplasia, hypoplasia, agenesis, ectopia, horseshoe kidney, polycystic kidney),5 and venous anomalies (anomalous inferior vena cava and azygos vein continuation, inferior vena cava coarctation, renal vein abnormalities).6e8 The association of situs inversus totalis and abdominal aortic pathology has rarely been reported. Most cases refer to patients presenting with aortic aneurysms,9e12 but a seldom report of aortic occlusive disease has also been published.13 Open aortic surgery has been feared in patients with situs inversus as the unfamiliar anatomy may be associated to an increased risk of intraoperative complications.9 Alternative approaches have been preferred by some authors as reported by Chan et al.11 who chose to perform an endovascular aneurysm repair in their patient because of the presence of situs inversus. However, an increased risk of perioperative complications is not found among the literature when adequate preoperative planning is performed.9e12,14,15 Open surgery remains the gold standard for the treatment of Leriche syndrome, and anatomic revascularization has proven to have increased patency compared to extra-anatomic bypasses. Despite requiring systemic anticoagulation throughout the perioperative period and presenting unfamiliar anatomic features, an aortobifemoral interposition was the preferred treatment for our patient. In our view, a correct preoperative planning of both the surgical approach and pathway for graft tunneling were the key to avoiding any intraoperative complications with the consequent favorable outcome that our patient presented. Open surgery is the gold standard for the treatment of infrarenal aortic thrombosis and can be safely performed in patients with situs inversus totalis. The association of abdominal aortic thrombosis with situs inversus has seldom been reported.
Vol. 29, No. 6, August 2015
Case reports 1317.e7
Fig. 2. (A, D) Computerized tomography angiography demonstrating the infrarenal aortic thrombosis in association to situs inversus totalis. A single horseshoe kidney located over the aortic bifurcation can be depicted (C, D).
REFERENCES
Fig. 3. Digital subtraction angiography confirming the aortic occlusion. A single renal artery is depicted and an exuberant Roland arch originating from the superior mesenteric artery.
1. Yokoyama T, Copeland NG, Jenkins NA, et al. Reversal of left-right asymmetry: a situs inversus mutation. Science 1993;260:679e82. 2. Matsusshita K, Veda S, Kegami K. Horseshoe kidney in a patient with situs inversus totalis. J Urol 1982;128: 604e5. 3. Belmonte JC. How the body tells left from right. Sci Am 1999;280:46e51. 4. Gutgesell HP. Cardiac malposition and heterotaxia. In: Garson A, Jr, Bricker JT, McNamara DG eds. The Science and Practice of Pediatric Cardiology. Philadelphia: Lea & Febiger, 1990. pp 1280e303. 5. Treiger BFG, Khazan R, Goldman SM, et al. Renal cell carcinoma with situs inversus totalis. Urology 1993;41: 455e7. 6. Beppu H, Matsushita S, Kimata S, et al. A case of anomalous inferior vena cava with azygos continuation associated with situs inversus totalis and acquired mitral valvular disease. Jpn Heart J 1967;8:428e32. 7. Victor S, Jayanthi V, Vallal TT, et al. Coarctation of inferior vena cava in situs inversus totalis. Indian J Gastroenterol 1992;11:89. 8. Dupont JR. Isolated left-sided vena cava and abdominal aortic aneurysm. Arch Surg 1971;102:211e2.
1317.e8 Case reports
Annals of Vascular Surgery
Fig. 4. (A) Intraoperative field with after aortic bifemoral interposition with a woven-polyester bifurcated graft (Unigraft 16 8mm). (B) Detail of the prerenal course of the graft in the retroperitoneum. The graft
limbs were tunneled in a superficial pathway in the retroperitoneal space avoiding iatrogenic injury to the iliac veins which were found in an anterior position relatively to the iliac arteries.
9. Kato T, Takagi H, Sekino S, et al. Abdominal aortic aneurysm in situs inversus totalis. Ann Vasc Surg 2006;20:267e70. 10. Baccellieri D, Mirenda F, Mandolfino T, et al. Symptomatic aorto-iliac aneurysm and situs viscerum inversus: case report. Chir Ital 2006;58:113e6. 11. Chan YC, Ting AC, Qing KX, et al. Successful endovascular infrarenal aneurysm repair in a patient with situs inversus totalis. Ann Vasc Surg 2010;24:1134.e5e7. 12. Huston I, Nicholas G. Situs inversus totalis and an infrarenal abdominal aortic aneurysm: case report. Vasc Endovascular Surg 1990;24:372e4.
13. Rapovka G. Occlusion of the infrarenal segment of the aorta in a patient with total situs inversus of the internal organs. Khirurgiia (Mosk) 1989;137e8. 14. Kimura N, Kawahito K, Ito S. Rupture of an abdominal aortic aneurysm in a patient with situs inversus totalis: report of a case. Surg Today 2008;38: 249e52. 15. Occhionorelli S, Navarra G, Santini M, et al. Symptomatic abdominal aortic aneurysm and ‘‘situs viscerum inversus.’’ Diagnostic and therapeutic approach. J Cardiovasc Surg (Torino) 1998;39:31e3.