Catheter Embolization of Type II Congenital Extrahepatic Portosystemic Shunt with Hematochezia: A Case Series and Review of the Literature

Abstract

Hematochezia is a rare clinical presentation of congenital extrahepatic portosystemic shunt (CEPS). We describe a series of three patients with type II CEPS presenting as hematochezia that were treated by catheter embolization, followed by a brief review of published articles. Hematochezia of the patients was due to the giant inferior mesenteric vein, superior rectal vein and colonic varices. The catheter embolization was successfully accomplished in all of the patients. After a mean follow-up of 27 months, no serious adverse effects were observed. For unexplained massive hematochezia, CEPS needs to be considered as a differential diagnosis. Based on our present results and the review of the literature, transcatheter permanent embolization of the giant inferior mesenteric vein might be an effective and safe treatment for type II CEPS.Level of Evidence Level 4, case series.

Comparison of Surgical Ligation and Endovascular Embolization in the Management of Type II Congenital Extrahepatic Portosystemic Shunt: A Retrospective Clinical Study

“…In combination with other congenital malformations, patients with CEPS present with hypergalactosemia, high bile acid, high serum ammonia, and hepatic encephalopathy caused by the portal and vena cava shunt. It has been reported that 66-100% of the patients with CEPS have hyperammonemia and 17-30% have hepatic encephalopathy [7][8][9].…”

Efficacy and Safety of Surgical Ligation versus Endovascular Embolization for Type II Congenital Extrahepatic Portosystemic Shunt

“…Angiography is the gold standard for diagnosing CEPS and identifying the type of CEPS present [ 8 , 9 ]. While shunt anatomy and flow dynamics can be determined by angiography and/or direct venography, balloon shunt occlusion venography is critical for detecting intrahepatic portal vein branches.…”

“…Portal vein and mesenteric venous thrombosis is a common complication after shunt ligation that can lead to portal hypertension; therefore, postprocedural anticoagulation therapy is very essential [ 4 , 8 ]. Heparin and warfarin have been used to prevent thrombosis in previous studies; however, the activated partial thromboplastin time and international normalized ratio should be closely monitored to adjust drug dosage [ 14 , 17 ].…”

The Characteristics and Outcomes of Abernethy Syndrome in Korean Children: A Single Center Study

“…Owing to the severity of complications and their potential reversibility after suppression of the shunts, closure or ligation of a shunt in type 2 CEPS is necessary [7]. It is performed either surgically or with percutaneous transcatheter embolization [20]. Various studies have strongly recommended that, when a shunt is detected with no clinically significant complications and is confirmed to be permanent, it should be closed before a significant complication occurs if a closure can be performed satisfactorily [821].…”