Management and classification of type II congenital portosystemic shunts

Congenital portosystemic shunts with and without gastrointestinal bleeding – case series

“…In the majority of congenital portosystemic shunt patients with gastrointestinal bleeding, the shunt drains the portal blood into the iliac vein via the inferior mesenteric vein, and Kobayashi et al [6] identified this type of shunt as "type C." Of note, the Kobayashi Classification does not differentiate [3]. There were six cases of extrahepatic congenital portosystemic shunt that presented with lower gastrointestinal bleeding in our study.…”

“…We reviewed the literature and found nine cases of congenital portosystemic shunt presenting with gastrointestinal bleeding [3,[12][13][14][15][16][17][18][19]. Of the eight patients with extrahepatic congenital portosystemic shunts, seven had a type I shunt and one had a type II shunt [3,[12][13][14][15][16][17]19].…”

“…Of the eight patients with extrahepatic congenital portosystemic shunts, seven had a type I shunt and one had a type II shunt [3,[12][13][14][15][16][17]19]. In these eight patients, the shunt drained the portal blood into the unilateral and/or bilateral internal iliac veins via the inferior mesenteric vein, two into the bilateral internal iliac veins, two into the left internal iliac vein and two into the right internal iliac vein; for the other two cases, the specific vein(s) that received the shunted portal blood were not mentioned in the literature [3,[12][13][14][15][16][17]19]. It was speculated that the abundant developing collaterals, such as the superior hemorrhoidal plexus, connected the inferior mesenteric and iliac veins [15].…”

“…The first patient with congenital portosystemic shunt was reported by John Abernethy in 1793; autopsy revealed that the patient had an extrahepatic portosystemic shunt and lacked the portal vein [2]. To date, several classification systems for congenital portosystemic shunt have been proposed [3][4][5][6]. Congenital portosystemic shunt is typically classified as extrahepatic (Abernethy, type I and type II) [4] or intrahepatic (Park, types I-IV) [5], based on the anatomical morphology.…”

TIPS treatment in a patient with severe lower gastrointestinal bleeding with a misdiagnosis of cirrhotic portal hypertension

“…Lautz et al established two groups type I CEPS without intrahepatic portal flow and type II CEPS preservation of at least some hepatic portal flow. They have subclassified type II as: Type IIa (the shunt arises from the portal vein), type IIb (the shunt arises from main portal vein, bifurcation or splenoportal confluence), and type IIc (the shunt arises from mesenteric, splenic or gastric veins) (3).…”