Zhang DY, Weng SQ, Dong L, Shen XZ, Qu XD. Portal hypertension induced by congenital hepatic arterioportal fistula: Report of four clinical cases and review of the literature. World J Gastroenterol 2015; 21(7): 2229-2235 [PMID: 25717263 DOI: 10.3748/wjg.v21.i7.2229]
Corresponding Author of This Article
Ling Dong, Professor, Department of Gastroenterology of Zhongshan Hospital, Fudan University, 180 Fenglin Road, Xuhui District, Shanghai 200032, China. dong.ling@zs-hospital.sh.cn
Research Domain of This Article
Gastroenterology & Hepatology
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/
Dan-Ying Zhang, Shu-Qiang Weng, Ling Dong, Department of Gastroenterology of Zhongshan Hospital, Fudan University, Shanghai 200032, China
Xi-Zhong Shen, Department of Gastroenterology of Zhongshan Hospital, Fudan University, Shanghai 200032, China
Xi-Zhong Shen, Shanghai Institute Of Liver Diseases ; Key Laboratory of Medical Molecule Virology, Ministry of Education and Health, Shanghai 200032, China
Xu-Dong Qu, Department of Interventional Radiology, Zhongshan Hospital, Fudan University, Shanghai 200032, China
Author contributions: Zhang DY and Qu XD performed the majority of the analyses of the clinical case; Zhang DY and Dong L acquired the data, performed the analysis and interpreted the data; Zhang DY and Weng SQ drafted the article; Shen XZ critically revised the manuscript for important intellectual content; Zhang DY and Weng SQ designed the study and wrote the manuscript; Zhang DY and Weng SQ contributed equally to this work and should be regarded as co-first authors.
Supported by National Nature Science Foundation of China No. 81000968, No. 81101540, No. 81101637, No. 81172273, No. 81272388, No. 81301820 and No. 81472673; Doctoral Fund of Ministry of Education of China No. 20120071110058, The National Clinical Key Special Subject of China.
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/
Correspondence to: Ling Dong, Professor, Department of Gastroenterology of Zhongshan Hospital, Fudan University, 180 Fenglin Road, Xuhui District, Shanghai 200032, China. dong.ling@zs-hospital.sh.cn
Telephone: +86-21-64041990 Fax: +86-21-63148437
Received: July 15, 2014 Peer-review started: July 16, 2014 First decision: August 27, 2014 Revised: September 11, 2014 Accepted: October 20, 2014 Article in press: October 21, 2014 Published online: February 21, 2015 Processing time: 210 Days and 22.2 Hours
Abstract
Intrahepatic arterioportal fistula (IAPF) can be caused by many secondary factors. We report four cases of portal hypertension that were eventually determined to be caused by congenital hepatic arterioportal fistula. The clinical manifestations included ascites, variceal hemorrhage and hepatic encephalopathy. Computed tomography scans from all of the patients revealed the early enhancement of the portal branches in the hepatic arterial phase. All patients were diagnosed using digital subtraction angiography (DSA). DSA before embolization revealed an arteriovenous fistula with immediate filling of the portal venous radicles. All four patients were treated with interventional embolization. The four patients remained in good condition throughout follow-up and at the time of publication. IAPF is frequently misdiagnosed due to its rarity; therefore, clinicians should consider IAPF as a potential cause of non-cirrhotic portal hypertension.
Core tip: In Western countries, less than 10% of portal hypertension cases are caused by non-cirrhotic portal hypertension, including intrahepatic arterioportal fistula (IAPF). IAPF is a condition characterized by abnormal communication between the portal vein and the hepatic artery that most often occurs secondary to surgery, trauma, transhepatic intervention or biopsy. Currently, only 35 cases of congenital IAPF have been reported. To better understand the clinicopathological presentation of this type of portal hypertension, IAPF-induced PH confirmed by angiography was studied in four patients. Because congenital hepatic arterioportal fistulae are rare, they are often misdiagnosed.
