Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Jan 27, 2021; 13(1): 151-161
Published online Jan 27, 2021. doi: 10.4254/wjh.v13.i1.151
Living-donor liver transplantation in Budd-Chiari syndrome with inferior vena cava complete thrombosis: A case report and review of the literature
Vinicius Rocha-Santos, Daniel Reis Waisberg, Rafael Soares Pinheiro, Lucas Souto Nacif, Rubens Macedo Arantes, Liliana Ducatti, Rodrigo Bronze Martino, Luciana Bertocco Haddad, Flavio Henrique Galvao, Wellington Andraus, Luiz Augusto Carneiro-D'Alburquerque
Vinicius Rocha-Santos, Daniel Reis Waisberg, Rafael Soares Pinheiro, Lucas Souto Nacif, Rubens Macedo Arantes, Liliana Ducatti, Rodrigo Bronze Martino, Luciana Bertocco Haddad, Flavio Henrique Galvao, Wellington Andraus, Luiz Augusto Carneiro-D'Alburquerque, Department of Gastroenterology, Abdominal Organs Transplantation Division, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo (HC-FMUSP), Sao Paulo 05403900, SP, Brazil
Flavio Henrique Galvao, Wellington Andraus, Luiz Augusto Carneiro-D'Alburquerque, Department of Gastroenterology, Laboratory of Medical Investigation 37 (LIM-37), Faculdade de Medicina da Universidade de Sao Paulo (FMUSP), Sao Paulo 01246903, Brazil
Author contributions: Rocha-Santos V, Carneiro-D'Alburquerque LA, Nacif LS and Waisberg DR were the patient’s surgeons; Andraus W, Pinheiro RS and Ducatti L performed the donor operation; Rocha-Santos V, Waisberg DR, Pinheiro RS and Nacif LS drafted the manuscript; Arantes RM, Ducatti L, Martino RB and Haddad LB performed the literature review and contributed to manuscript drafting; Galvao FH, Andraus W and Carneiro-D'Alburquerque LA were responsible for revision of the manuscript and for important intellectual content; all authors issued final approval for the version to be submitted.
Informed consent statement: The patient provided written consent for publication of this manuscript.
Conflict-of-interest statement: The authors declare that they have no conflict of interest regarding the content of this manuscript.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Vinicius Rocha-Santos, MD, PhD, Adjunct Professor, Attending Doctor, Department of Gastroenterology, Abdominal Organs Transplantation Division, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo (HC-FMUSP), Av. Dr Eneas de Carvalho Aguiar, nº255, 9th Floor, Room 9114, Sao Paulo 05403900, SP, Brazil. dr_vinicius@uol.com.br
Received: September 23, 2020
Peer-review started: September 23, 2020
First decision: October 21, 2020
Revised: November 12, 2020
Accepted: November 28, 2020
Article in press: November 28, 2020
Published online: January 27, 2021
Abstract
BACKGROUND

Budd-Chiari syndrome (BCS) is a challenging indication for liver transplantation (LT) due to a combination of massive liver, increased bleeding, retroperitoneal fibrosis and frequently presents with stenosis of the inferior vena cava (IVC). Occasionally, it may be totally thrombosed, increasing the complexity of the procedure, as it should also be resected. The challenge is even greater when performing living-donor LT as the graft does not contain the retrohepatic IVC; thus, it may be necessary to reconstruct it.

CASE SUMMARY

A 35-year-old male patient with liver cirrhosis due to BCS and hepatocellular carcinoma beyond the Milan criteria underwent living-donor LT with IVC reconstruction. It was necessary to remove the IVC as its retrohepatic portion was completely thrombosed, up to almost the right atrium. A right-lobe graft was retrieved from his sister, with outflow reconstruction including the right hepatic vein and the branches of segment V and VIII to the middle hepatic vein. Owing to massive subcutaneous collaterals in the abdominal wall, venovenous bypass was implemented before incising the skin. The right atrium was reached via a transdiaphragramatic approach. Hepatectomy was performed en bloc with the retrohepatic vena cava. It was reconstructed with an infra-hepatic vena cava graft obtained from a deceased donor. The patient remains well on outpatient clinic follow-up 25 mo after the procedure, under an anticoagulation protocol with warfarin.

CONCLUSION

Living-donor LT in BCS with IVC thrombosis is feasible using a meticulous surgical technique and tailored strategies.

Keywords: Liver transplantation, Living donors, Budd-Chiari syndrome, Hepatic veno-occlusive disease, Inferior vena cava, Case report

Core Tip: A right-lobe living-donor liver transplantation (LT) with inferior vena cava (IVC) resection and reconstruction was performed in a patient with liver cirrhosis due to Budd-Chiari syndrome and hepatocellular carcinoma beyond the Milan criteria. It was necessary to remove the IVC because its retrohepatic portion was completely thrombosed, up to almost the right atrium. It was reconstructed with an infra-hepatic vena cava graft obtained from a deceased donor. The patient remains well 25 mo after the procedure. This case highlights the meticulous surgical technique and tailored strategies required for dealing with these challenging procedures in living-donor LT.