Congenital portosystemic shunts with and without gastrointestinal bleeding - case series.

BACKGROUND: The clinical presentation of congenital portosystemic shunt is variable and gastrointestinal bleeding is an uncommon presentation.

OBJECTIVE: To describe the imaging features of congenital portosystemic shunt as it presented in 11 children with (n = 6) and without gastrointestinal bleeding (n = 5).

MATERIALS AND METHODS: We performed a retrospective study on a clinical and imaging dataset of 11 children diagnosed with congenital portosystemic shunt.

RESULTS: A total of 11 children with congenital portosystemic shunt were included in this study, 7 with extrahepatic portosystemic shunts and 4 with intrahepatic portosystemic shunts. Six patients with gastrointestinal bleeding had an extrahepatic portosystemic shunt, and the imaging results showed that the shunts originated from the splenomesenteric junction (n = 5) or splenic vein (n = 1) and connected to the internal iliac vein. Among the five cases of congenital portosystemic shunt without gastrointestinal bleeding, one case was an extrahepatic portosystemic shunt and the other four were intrahepatic portosystemic shunts.

CONCLUSION: Most congenital portosystemic shunt patients with gastrointestinal bleeding had a shunt that drained portal blood into the iliac vein via an inferior mesenteric vein. This type of shunt was uncommon, but the concomitant rate of gastrointestinal bleeding with this type of shunt was high.