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A case report of perihilar cholangiocarcinoma in a patient with situs inversus totalis

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Abstract

Situs inversus totalis is a rare congenital malformation in which organs are positioned in a mirror-image relationship to normal conditions. It often presents with vascular and biliary malformations. Only a few reports have pointed out the surgical difficulties in patients with situs inversus totalis, especially in those with perihilar cholangiocarcinoma. This report describes a 66-year-old male patient who underwent left hemihepatectomy (S5, 6, 7, and 8) with combined resection of the caudate lobe (S1), extrahepatic bile duct, and regional lymph nodes for perihilar cholangiocarcinoma with situs inversus totalis. Cholangiocarcinoma was mainly located in the perihilar area and progressed extensively into the bile duct. Surgery was performed after careful evaluation of the unusual anatomy. Although several vascular anomalies required delicate manipulation, the procedures were performed without major intraoperative complications. Postoperatively, bile leakage occurred, but the patient recovered with drainage treatment. The patient was discharged on the 29th postoperative day. Adjuvant chemotherapy with S-1 was administered for approximately 6 months. There was no recurrence 15 months postoperatively. Appropriate imaging studies and an understanding of unusual anatomy make surgery safe and provide suitable treatment for patients with situs inversus totalis.

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Abbreviations

CCA:

Cholangiocarcinoma

CT:

Computed tomography

ERCP:

Endoscopic retrograde cholangiopancreatography

EUS:

Endoscopic ultrasonography

IVC:

Inferior vena cava

LGA:

Left gastric artery

PTPE:

Percutaneous transhepatic portal vein embolization

SA:

Splenic artery

SIT:

Situs inversus totalis

SMA:

Superior mesenteric artery

US:

Ultrasonography

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Authors and Affiliations

  1. Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, 980-8574, Japan

    Naohiro Hirano, Masahiro Iseki, Kei Nakagawa, Masamichi Mizuma, Takashi Kamei & Michiaki Unno

  2. Division of Gastroenterology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, 980-8574, Japan

    Ryotaro Matsumoto, Shin Miura, Kiyoshi Kume & Atsushi Masamune

Authors
  1. Naohiro Hirano
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  2. Masahiro Iseki
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  3. Kei Nakagawa
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  4. Masamichi Mizuma
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  5. Takashi Kamei
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  6. Ryotaro Matsumoto
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  7. Shin Miura
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  8. Kiyoshi Kume
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  9. Atsushi Masamune
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  10. Michiaki Unno
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Contributions

NH drafted the manuscript; MI revised the manuscript; KN was the main surgeon; all authors reviewed and approved the final manuscript.

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Correspondence to Michiaki Unno.

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Written informed consent was obtained from the patient for the publication of this case report and the accompanying images.

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Hirano, N., Iseki, M., Nakagawa, K. et al. A case report of perihilar cholangiocarcinoma in a patient with situs inversus totalis. Clin J Gastroenterol (2024). https://doi.org/10.1007/s12328-024-01940-z

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  • DOI: https://doi.org/10.1007/s12328-024-01940-z

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