Abstract
Congenital extrahepatic portosystemic shunt (CEPS) is a rare disorder characterised by partial or complete diversion of portomesenteric blood into systemic veins via congenital shunts. Type I is characterised by complete lack of intrahepatic portal venous blood flow due to an end to side fistula between main portal vein and the inferior vena cava. Type II on the other hand is characterised by partial preservation of portal blood supply to liver and side to side fistula between main portal vein or its branches and mesenteric, splenic, gastric, and systemic veins. The presentation of these patients is variable. Focal liver lesions, most commonly nodular regenerative hyperplasia, are an important clue to the underlying condition. This pictorial essay covers imaging characteristics in abdominopelvic region.
Highlights
Abernethy described the first case of congenital extrahepatic portosystemic shunt on autopsy on a 10-month-old female who died of unknown cause [1]
Type II congenital extrahepatic portosystemic shunt is characterised by partial diversion of the portal blood flow into the systemic veins [3]
An absence of portal vein is detected on US in type I congenital extrahepatic portosystemic shunt
Summary
Abernethy described the first case of congenital extrahepatic portosystemic shunt on autopsy on a 10-month-old female who died of unknown cause [1]. He demonstrated the absence of portal vein and existence of a mesentericocaval shunt. Type II shunts are more varied in their anatomy and are characterised by partial interruption of portal venous flow to the liver caused by portocaval, gastrorenal, mesenterico-renal, splenorenal, or mesenterico-iliac shunts [3]. The embryogenesis of this congenital anomaly is complex. Type II malformations can be corrected by surgical ligation or endovascular occlusion [7]
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