Published online Feb 21, 2015. doi: 10.3748/wjg.v21.i7.2229
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
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.