Robotic pancreaticoduodenectomy in a patient with situs inversus totalis and variant celiacomesenteric trunk

Bor-Shiuan Shyr; Bor-Uei Shyr; Shih-Chin Chen; Yi-Ming Shyr; Shin-E Wang

doi:10.4103/fjs.fjs_173_21

CASE REPORT

Year : 2022 | Volume : 55 | Issue : 2 | Page : 56-59

Robotic pancreaticoduodenectomy in a patient with situs inversus totalis and variant celiacomesenteric trunk

Bor-Shiuan Shyr, Bor-Uei Shyr, Shih-Chin Chen, Yi-Ming Shyr, Shin-E Wang
Division of General Surgery, Department of Surgery, Taipei Veterans General Hospital, National Yang Ming Chiao Tung University, Taipei, Taiwan

Date of Submission	18-Aug-2021
Date of Decision	18-Jan-2022
Date of Acceptance	21-Jan-2022
Date of Web Publication	25-Apr-2022

Correspondence Address:
Shin-E Wang
Division of General Surgery, Department of Surgery, Taipei Veterans General Hospital, 201, Section 2 Shi-Pai Road, Taipei 112
Taiwan

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/fjs.fjs_173_21

Abstract

Situs inversus totalis (SIT) is a rare congenital anomaly characterized by right–left reversal of visceral organs and dextrocardia; the use of robotic pancreaticoduodenectomy (RPD) may be challenging in these patients. A male patient presented with jaundice; imaging studies revealed a bile duct tumor and SIT with variant celiacomesenteric trunk and multiple vascular anomalies. RPD was carried out cautiously and successfully. The trocar port design mirrored that of a normal patient. Pathological examination revealed extrahepatic bile duct adenocarcinoma, classified as pT3N1M0, stage IIB. The patient recovered uneventfully. Although difficult, RPD is technically feasible in an SIT patient with cholangiocarcinoma.

Keywords: Cholangiocarcinoma, pancreaticoduodenectomy, robotic, situs inversus totalis

How to cite this article:
Shyr BS, Shyr BU, Chen SC, Shyr YM, Wang SE. Robotic pancreaticoduodenectomy in a patient with situs inversus totalis and variant celiacomesenteric trunk. Formos J Surg 2022;55:56-9

How to cite this URL:
Shyr BS, Shyr BU, Chen SC, Shyr YM, Wang SE. Robotic pancreaticoduodenectomy in a patient with situs inversus totalis and variant celiacomesenteric trunk. Formos J Surg [serial online] 2022 [cited 2022 May 26];55:56-9. Available from: https://www.e-fjs.org/text.asp?2022/55/2/56/343815

Introduction

Situs inversus totalis (SIT) is a rare autosomal recessive congenital anomaly characterized by the reversal of right–left polarity of the thoracic and abdominal visceral organs with dextrocardia.^[1],[2] The incidence of SIT is about 1/8000–1/25,000 in the general population.^[1],[2] Although patients with SIT have a high incidence of cardiac, splenic, and hepatobiliary anomalies, these malformations are not considered to be premalignant, and SIT in itself does not seem to affect health or life expectancy.^[2] Nevertheless, the diagnosis and surgery for abdominal pathologies in patients with SIT are technically more complicated.^[2]

Pancreaticoduodenectomy, also known as the Whipple procedure, is a complex and time-consuming procedure. The use of open pancreaticoduodenectomy in patients with SIT has been reported.^{[1],[2],[3],[4]} However, MIS might be challenging in these patients not only because of the reversed anatomy but also due to the other possible associated anomalies that may not have been expected before surgery.^[5] So far, there has only been one report of laparoscopic pancreaticoduodenectomy in a patient with SIT.^[5]

With the introduction of the da Vinci Robotic Surgical System (Intuitive Surgical, Inc., Sunnyvale, CA, USA), several limitations related to the laparoscopic approach have been overcome.^[6],[7] To the best of our knowledge, the use of robotic pancreaticoduodenectomy (RPD) in patients with SIT has not been previously reported. Here, we report the use of RPD for the management of extrahepatic bile duct cancer in a patient with SIT.

Case Report

This case report was approved by the Institutional Review Board of Taipei Veterans General Hospital (IRB-TPEVGH No. 2021–03-003BC). The patient provided informed consent for the use of this de-identified, retrospective case study.

A 70-year-old man with known dextrocardia for 50 years presented with a 1-month history of progressive, persistent, and painless jaundice accompanied by poor appetite, nausea, and weight loss of 3 kg in the absence of fever. Abdominal computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP) revealed SIT with a right–left reversal of abdominal visceral organs, polysplenia, and an increased soft-tissue density from the common hepatic duct to common bile duct, causing bilateral intrahepatic duct dilatation [Figure 1]. Due to progressive jaundice, the patient underwent percutaneous transhepatic cholangial drainage (PTCD) due to obstruction at the level of hepatic hilum. Cytologic analysis of the bile collected via PTCD was consistent with the diagnosis of adenocarcinoma. Multidetector-row CT angiography with three-dimensional (3D) angiographic reconstruction showed SIT with multiple vascular anomalies [Figure 2], including variant celiacomesenteric trunk, replaced common hepatic artery originating from the superior mesenteric artery (SMA), early bifurcation of the gastroduodenal artery, and splenic artery originating from the SMA. Electrocardiogram and chest radiograph showed dextrocardia. Laboratory examination showed elevated total serum bilirubin level of 8.14 g/dL (reference value <1.2 g/dL), increased cancer antigen 19–9 (CA 19–9) level of 704 U/mL (reference value <37 U/mL), and normal carcinoembryonic antigen level of 2.15 ng/mL (reference value <5 ng/mL). Based on the above evaluations, a diagnosis of extrahepatic bile duct adenocarcinoma with SIT was made.

Figure 1: (a) Situs inversus totalis, with the cardiac apex, stomach, and superior mesenteric artery on the right, and the liver, pancreatic head, duodenum, and superior mesenteric vein on the left; (b) Mirror image of situs inversus totalis in the same patient

Click here to view

Figure 2: (a) Three-dimensional angiography reconstruction, and (b) multidetector-row computed tomography angiography shows multiple vascular anomalies, including variant celiacomesenteric trunk, replaced common hepatic artery originating from the superior mesenteric artery, early bifurcation of the gastroduodenal artery, and splenic artery originating from the superior mesenteric artery

Click here to view

RPD with mesopancreas level 2 dissection along the SMA was carried out cautiously. The trocar port design mirrored that of a normal patient without SIT. The robotic camera port was placed at the left of the umbilicus to visualize the superior mesenteric vessels clearly during the pancreatic head separation [Figure 3]. Despite the reversed anatomy, the patient's position and the minimally invasive techniques applied were similar to those with normal anatomy.^[7] The operation was uneventful with a total console time of 10.5 h and a total blood loss of 300 mL.

Figure 3: Trocar port design for robotic pancreaticoduodenectomy in situs inversus totalis. The trochar port sites were designed according to the patient's abdomen status after full CO₂ insufflation to 12–15 mmHg

Click here to view

A polypoid tumor measuring 3.5 cm × 2.2 cm × 1.8 cm in the common hepatic and common bile duct was collected for histopathologic analysis. Microscopic examination revealed moderately differentiated adenocarcinoma of the bile duct with perineural and lymphovascular invasion in the absence of lymph node metastasis. Based on the American Joint Committee on Cancer staging system (8^th Edition), the tumor was classified as pT3N1M0, Stage IIB. The postoperative course was uneventful, and the patient was discharged 15 days after surgery.

Discussion

The term “situs” refers to the position of the heart and viscera relative to the midline. In general, this can be further subclassified into situs solitus which is the normal position, situs inversus referring to the mirror image location of the viscera, and situs ambiguus describing a not clearly lateralized arrangement of the thoracoabdominal organs. Situs inversus, often called as SIT, is more common and is characterized by having the cardiac apex, spleen, stomach, and aorta on the right, whereas the liver and inferior vena cava are on the left.^[8] In situs ambiguus, on the other hand, the liver may be in the midline, but the atrial morphology is ill defined and the bowel is malrotated. The incidence of situs ambiguus is approximately 1 in 24,000–40,000 live births.^[9]

Despite many reports describing several malignancies (i.e., biliary duct, pancreas, ampulla of Vater, liver, stomach, colon, ovary, and lung) among patients with SIT, there is no known causal link between SIT and malignancy.^[1] Only five cases of cholangiocarcinoma in SIT have been reported,^[10] and herein we describe this association for the sixth time in literature. Moreover, this is the first report of the use of RPD in a patient with SIT.

Evidently, SIT makes the surgical procedure more challenging because of the difficulty in following standard surgical protocols. Advanced diagnostic imaging techniques, such as MD-CTA with 3D reconstruction and MRCP, if available, are highly recommended for detecting vascular anomalies as seen in our patient. Careful preoperative planning with these techniques is crucial to understand the regional anatomic abnormalities in order to avoid major vessel injuries and reduce unexpected complications.

This report highlights that although RPD is relatively difficult in patients with SIT, the use of this technique is feasible. Extensive preoperative assessment with advanced imaging techniques is necessary to fully understand not only the right–left reversal of the visceral organs but also the multiple vascular anomalies. With this surgical planning, MIS in patients with SIT may be feasible.

Ethical approval and declaration of patient consent

This study was approved by the Institutional Review Board of Taipei Veterans General Hospital (approval number: 2021-03-003BC).

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

This study is financially supported by Taipei Veterans General Hospital (V110B-023, V110C-010 and V110C-011), the Ministry of Science and Technology (MOST 108-2314-B-075 -051 -MY3), and the Ministry of Health and Welfare (MOHW107-TDU-B-212-114026A).

Conflicts of interest

Prof. Yi-Ming Shyr, an editorial board member at Formosan Journal of Surgery, had no role in the peer review process of or decision to publish this article. The other authors declared no conflicts of interest in writing this paper.

References

1.	Chen C, Yi X, He Y, Cai S, Gu G, Sun C, et al. Pancreatic head cancer involving variant common hepatic artery with situs inversus totalis. ANZ J Surg 2018;88:506-8.
2.	Kyuno D, Kimura Y, Imamura M, Uchiyama M, Ishii M, Meguro M, et al. Pancreaticoduodenectomy for biliary tract carcinoma with situs inversus totalis: Difficulties and technical notes based on two cases. World J Surg Oncol 2013;11:312.
3.	Zheng Z, Xiao Y, Zhang S, Pu G. A patient with situs inversus totalis and pancreatic head cancer. Dig Liver Dis 2013;45:e11.
4.	Sakaguchi O, Kamio H, Sakurai H, Kumagai K, Kobayashi E, Kakita N, et al. Pancreas head carcinoma associated with situs inversus viscerum totalis. Nihon Geka Gakkai Zasshi 1985;86:111-5.
5.	Guo CX, Chen W, Yao WY, Li GG, Zhang Q, Chen YW, et al. The first report of laparoscopic pancreaticoduodenectomy for primary duodenal carcinoma in a patient with situs inversus totalis: Report of a rare case. Surg Laparosc Endosc Percutan Tech 2019;29:e29-33.
6.	Shyr YM, Wang SE, Chen SC, Shyr BU. Robotic pancreaticoduodenectomy in the era of minimally invasive surgery. J Chin Med Assoc 2020;83:639-43.
7.	Shyr BU, Shyr BS, Chen SC, Shyr YM, Wang SE. Mesopancreas level 3 dissection in robotic pancreaticoduodenectomy. Surgery 2021;169:362-8.
8.	Macafee DA, Armstrong D, Hall RI, Dhingsa R, Zaitoun AM, Lobo DN. Pancreaticoduodenectomy with a “twist”: The challenges of pancreatic resection in the presence of situs inversus totalis and situs ambiguus. Eur J Surg Oncol 2007;33:524-7.
9.	Lim HK, Choi YS, Lee SE, Kang H. Pancreaticoduodenectomy performed in a patient with situs ambiguous accompanied with isolated levocardia, malrotation, and normal spleen. Ann Surg Treat Res 2014;87:340-4.
10.	Coronel M, Lanke G, Cambell D, Coronel E, Tzeng CD, Foo W, et al. Performing endoscopic retrograde cholagiopancreatpgraphy and endoscopic ultrasound for management of malignant bile duct obstruction in a patient with a situs inversus totalis. ACG Case Rep J 2020;7:e00483.