Xiang W, Wang H, Si ZZ, Chen GS, Wang GW, Li T. Type I congenital extrahepatic portosystemic shunt treated by orthotopic liver transplantation: A case report. World J Clin Cases 2019; 7(7): 903-907 [PMID: 31024963 DOI: 10.12998/wjcc.v7.i7.903]
Corresponding Author of This Article
Ting Li, MD, PhD, Associate Research Scientist, Attending Doctor, Surgeon, Organ Transplantation Center, The Second Xiang-Ya Hospital, Central South University, No. 139 Middle Renmin Road, Changsha 410011, Hunan Province, China. liting001@csu.edu.cn
Research Domain of This Article
Transplantation
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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/
Wen Xiang, Hao Wang, Zhong-Zhou Si, Guang-Shun Chen, Guan-Wu Wang, Ting Li, Organ Transplantation Center, The Second Xiang-Ya Hospital, Central South University, Changsha 410011, Hunan Province, China
Author contributions: All authors contributed to the acquisition of data and the writing and revision of the manuscript.
Supported bythe Natural Science Foundation of Hunan Province, No. 2016JJ3165 and No. 2015JJ2169; and the Fundamental Research Funds for the Central Universities of Central South University, No. 2017zzts902.
Informed consent statement: The patient provided informed written consent.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
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 which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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: Ting Li, MD, PhD, Associate Research Scientist, Attending Doctor, Surgeon, Organ Transplantation Center, The Second Xiang-Ya Hospital, Central South University, No. 139 Middle Renmin Road, Changsha 410011, Hunan Province, China. liting001@csu.edu.cn
Telephone: +86-731-85295108 Fax: +86-731-85295808
Received: December 19, 2018 Peer-review started: December 19, 2018 First decision: January 6, 2019 Revised: January 24, 2019 Accepted: February 26, 2019 Article in press: February 26, 2019 Published online: April 6, 2019 Processing time: 108 Days and 1.8 Hours
Abstract
BACKGROUND
Congenital extrahepatic portosystemic shunt, also known as Abernethy deformation, is a rare malformation caused by dysplasia in the portal vein system. There are few reports of liver transplantation as a treatment for Abernethy deformation, and our report is the first case in China. This is the second reported case with congenital extrahepatic portosystemic shunt combined with focal nodular hyperplasia and hepatopulmonary treated with liver transplantation.
CASE SUMMARY
The patient was a 14-year-old girl, diagnosed preoperatively as type Ib Abernethy deformation, intrahepatic multiple space-occupying lesion, and hepatopulmonary syndrome. The patient recovered well after undergoing classic orthotopic liver transplantation. Liver function, pulmonary function, and portal vein computed tomography angiography imaging were reexamined 20 mo postoperatively, and no abnormality was observed.
CONCLUSION
Liver transplantation is an effective treatment for type I Abernethy deformation combined with focal nodular hyperplasia and hepatopulmonary syndrome.
Core tip: Congenital extrahepatic portosystemic shunt is a rare congenital abnormal vascular anastomosis between the portal vein and vena cava. This report discusses clinical, angiographic diagnostic and histopathological features in one patient with congenital extrahepatic portosystemic shunt, as well as its surgical strategy. Liver transplantation appears to be a feasible treatment option for patients with congenital extrahepatic portosystemic shunt because it achieves optimal treatment effectiveness.
