Repair of a juxtarenal abdominal aortic aneurysm in a patient with situs inversus totalis using a retroperitoneal approach Matt A. Cwinn, MD,a Alex Morzycki, MSc,b Min Lee, MD,c and Peter Midgley, MD,c Halifax, Nova Scotia, Canada Situs inversus totalis (SIT) is a rare condition characterized by the mirror image location of all of the thoracic and abdominal organs. There are only a handful of reports documenting the presence and repair of an abdominal aortic aneurysm in the setting of SIT. Here, we present a rare case of a juxtarenal abdominal aortic aneurysm repaired through a retroperitoneal approach in a patient with SIT. We demonstrate that the retroperitoneal approach is a safe and effective method to manage complex aortic aneurysm disease in a patient with SIT. (J Vasc Surg Cases 2016;2:92-4.)
Situs inversus totalis (SIT) is a rare condition characterized by the mirror image location of the thoracic and abdominal organs. It is a rare autosomal recessive disorder that is estimated to exist in approximately 0.3/10,000 live births.1 SIT exists within a spectrum of laterality disorders. Situs solitus refers to the normal configuration of the thoracic and abdominal organs. SIT refers to the complete mirror image location of all of the thoracic and abdominal organs. In some patients, SIT is discovered incidentally and is not associated with any adverse consequences. However, congenital cardiac defects are reported to occur in up to 40% to 60% of patients with SIT.1,2 Situs ambiguus refers to a scenario in which the arrangement of thoracic and abdominal organs cannot be categorized as either situs solitus or SIT. Patients with situs ambiguus often have a host of cardiac, pulmonary, and intra-abdominal anomalies.3 To our knowledge, neither situs ambiguus nor SIT is typically associated with the development of an abdominal aortic aneurysm (AAA). The rarity of SIT and the inverse anatomy provide obvious challenges to the surgeon. Here, we present a rare case of a juxtarenal AAA in a patient with SIT that was repaired using a retroperitoneal approach. Consent was obtained from the patient to write and to publish the case report before submission. CASE REPORT An 82-year-old man with known SIT (Fig 1) and no known concomitant cardiac defects presented to the clinic with an From the Department of General Surgery,a Dalhousie Medical School,b and Department of Vascular Surgery,c Dalhousie University. Author conflict of interest: none. Correspondence: Matt A. Cwinn, MD, MSc, 1276 South Park St, 8-813 VG Site, Halifax, NS B3H 2Y9, Canada (e-mail:
[email protected]). The editors and reviewers of this article have no relevant financial relationships to disclose per the Journal Policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. 2468-4287 Ó 2016 Published by Elsevier Inc. on behalf of Society for Vascular Surgery. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/). http://dx.doi.org/10.1016/j.jvscit.2016.04.002
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Fig 1. Chest radiograph demonstrating the presence of dextrocardia. L denotes left side. asymptomatic 9-cm juxtarenal AAA (Fig 2). His past medical history was relevant for hypertension and a remote smoking history. His family history was relevant for a first-degree male family member without SIT who previously underwent AAA repair. The patient was consented for aneurysm repair through a right retroperitoneal approach. After induction of anesthesia and placement of a central venous catheter and arterial line, the patient was placed in a left lateral decubitus position. An incision was made from the tip of the right eleventh rib to the umbilicus. The retroperitoneum was entered, and the aorta was exposed proximally above the renal arteries and distally to the common iliac arteries. The unilateral nature of the retroperitoneal approach simplified what would have otherwise been a challenging exposure as it allowed us to expose the aorta without contending with mirror image anatomy. Other than positioning the patient on the opposite side, we were able to perform the dissection in the usual fashion, without encountering any anomalous structures.
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Fig 2. Computed tomography demonstrating the presence of a large abdominal aortic aneurysm (AAA) as well as situs inversus totalis (SIT). A, Coronal image demonstrating the maximum diameter of the AAA. B and C, Axial images of the right (B) and left (C) iliac arteries. D and E, Coronal (D) and axial (E) images demonstrating aneurysmal degeneration at the level of the renal arteries. The arrow in E indicates the origin of the left renal artery. Note the presence of dextrocardia and the inverse location of the liver, stomach, and spleen in images (B) and (D).
Proximal and distal control of the aorta was obtained, and a suprarenal aortic clamp was placed. The aorta was repaired with an 18- 9-mm bifurcated graft. The proximal anastomosis incorporated both renal arteries and was completed with a suprarenal clamp time of 24 minutes. Distally, both iliac arteries were heavily
calcified, and bilateral endarterectomies were performed before conducting the anastomoses. A paravertebral nerve catheter was then placed intraoperatively, as is routine at our institution after AAA repair using the retroperitoneal approach. The technique for a surgeon-placed
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paravertebral catheter after retroperitoneal exposure has been described previously and is a safe and effective means of providing postoperative analgesia.4 Briefly, the parietal pleura was dissected free from the tenth and eleventh ribs and the extrapleural space was entered. Blunt dissection was carried out to the paravertebral space. A Tuohy needle was used to introduce the nerve catheter through the skin. The catheter was then placed in the paravertebral space under direct vision. Overlying tissues were then closed in layers. The incision was closed and the skin approximated with clips. Before completion of the case, the paravertebral catheter is injected with 0.25% bupivacaine and connected to a pump for continuous postoperative analgesia, which was managed by the anesthesiologist postoperatively. The patient had an uneventful recovery and was discharged to his home hospital to undergo additional rehabilitation on postoperative day 7.
DISCUSSION The presence of AAA in situs inversus is uncommon, with only a small number of case reports describing this condition.5-9 Consequently, the ideal surgical approach in a patient with SIT and AAA is unknown. Kimura et al described performing a right-sided thoracotomy with proximal aortic cross-clamping to gain vascular control in a patient with SIT and aneurysmal rupture. Another report demonstrated that transperitoneal repair can be performed using a minilaparotomy without evisceration.9 Recently, Chan et al reported a case of endovascular AAA repair in a patient with SIT, demonstrating the feasibility of an endovascular approach.6 We believe our report to be the first to document the repair of a juxtarenal AAA in SIT through a retroperitoneal approach. The retroperitoneal approach provides the surgeon with a more substantial exposure of the proximal aorta to facilitate suprarenal cross-clamping. In the case described here, the retroperitoneal approach provided an additional benefit as it obviated the need to contend with the mirror image positions of the abdominal viscera. In fact, the only major change to the usual operative technique was placement of the patient in the left rather than right decubitus position. Kimura et al provided similar justification for performing a right thoracotomy with cross-clamping of the descending thoracic aorta in the setting of rupture.8 In an acute situation, this was deemed to be faster and safer than contending with mirror image anatomy while gaining supraceliac control through a transperitoneal approach.8 Other anatomic and physiologic considerations must be taken into account in approaching a patient with SIT. Patients with Kartagener syndrome have chronic sinusitis, bronchiectasis, and SIT.10 Some series have also noted a number of cardiac defects in patients with SIT, ranging from isolated atrial septal defects to complex cardiac
anomalies.1 However, the frequency with which congenital cardiac anomalies are reported to occur in the setting of SIT varies considerably from 5% to >40%.1,3,11 Patients who suffer from Kartagener syndrome would be at increased risk for perioperative pulmonary complications. Patients with congenital cardiac anomalies could be at risk for a variety of complications, including an inability to tolerate aortic cross-clamping and sensitivity to perioperative fluid shifts and volume status. Thus, it would be prudent to thoroughly investigate any patient who presents with SIT and an AAA for concomitant pulmonary and cardiac conditions. CONCLUSIONS This case demonstrates the feasibility of a retroperitoneal approach for patients with SIT and more complex aneurysmal disease. Prior reports have demonstrated the feasibility of open transperitoneal repair and endovascular aneurysm repair in patients with infrarenal AAA and SIT. REFERENCES 1. Lin AE, Krikov S, Riehle-Colarusso T, Frias JL, Belmont J, Anderka M, et al. Laterality defects in the national birth defects prevention study (1998-2007): birth prevalence and descriptive epidemiology. Am J Med Genet A 2014;164A:2581-91. 2. Lee SE, Kim HY, Jung SE, Lee SC, Park KW, Kim WK. Situs anomalies and gastrointestinal abnormalities. J Pediatr Surg 2006;41:1237-42. 3. Wolla CD, Hlavacek AM, Schoepf UJ, Bucher AM, Chowdhury S. Cardiovascular manifestations of heterotaxy and related situs abnormalities assessed with CT angiography. J Cardiovasc Comput Tomogr 2013;7:408-16. 4. Langille GM, Launcelott GO, Rendon RA. Access to the extrapleural space at the time of surgery for continuous paravertebral block after flank incision: description of the technique and case series. Urology 2013;81:675-8. 5. Baccellieri D, Mirenda F, Mandolfino T, La Spada M, Stilo F, Spinelli F. [Symptomatic aorto-iliac aneurysm and situs viscerum inversus: case report]. Chir Ital 2006;58:113-6. 6. Chan YC, Ting AC, Qing KX, Cheng SW. Successful endovascular infrarenal aneurysm repair in a patient with situs inversus totalis. Ann Vasc Surg 2010;24:1134.e5-7. 7. Kato T, Takagi H, Sekino S, Manabe H, Matsuno Y, Umemoto T. Abdominal aortic aneurysm in situs inversus totalis. Ann Vasc Surg 2006;20:267-70. 8. Kimura N, Kawahito K, Ito S. Rupture of an abdominal aortic aneurysm in a patient with a situs inversus totalis: report of a case. Surg Today 2008;38:249-52. 9. Riera Hernandez C, Perez Ramirez P, Esteban Gracia C, Jimenez Olivera MA, Llagostera Pujol S. Situs inversus totalis and abdominal aortic aneurysm: surgical repair of an extremely uncommon association. Int J Surg Case Rep 2015;10:216-8. 10. Fretzayas A, Moustaki M. Clinical spectrum of primary ciliary dyskinesia in childhood. World J Clin Pediatr 2016;5:57-62. 11. Ellis K, Fleming RJ, Griffiths SP, Jameson AG. New concepts in dextrocardia. Angiocardiographic considerations. Am J Roentgenol Radium Ther Nucl Med 1966;97:295-313. Submitted Jan 23, 2016; accepted Apr 9, 2016.
