Pictorial review. Hepatic vascular shunts: embryology and imaging appearances

Abstract

The purpose of this pictorial review is to understand the embryological basis of the development of congenital hepatic vascular shunts and to review the multimodality imaging appearances of congenital and acquired hepatic vascular shunts. Hepatic vascular shunts are commonly seen in imaging. Familiarity with their characteristic appearances is important in order to accurately characterise these shunts and diagnose the underlying disorders.

Figure 1

Schematic images showing development of the hepatic venous system, which starts to develop in the fifth week of intrauterine life. Vitelline veins form the hepatic sinusoids. Left umbilical vein connects to the right vitelline vein via the ductus venosus. Anastomotic network around the duodenum forms the portal vein. LCV, left cardinal vein; RCV, right cardinal vein; RUV, right umbilical vein; LUV, left umbilical vein; SC, superior communication; MC, middle communication; IC, inferior communication.

Figure 2

Hepatic venous system changes at birth. (a) Schematic diagram showing antenatal circulation with direct communication between the umbilical vein and inferior vena cava (IVC) through the ductus venosus. (b) Schematic diagram showing post-natal circulation with inflow into liver through the hepatic artery and portal vein, and outflow through the hepatic veins.

Figure 3

A 23-year-old male with Osler-Weber-Rendu syndrome. (a) Axial arterial-phase CT image showing multiple arterioportal shunts in both lobes of liver (arrows). (b) Selective coeliac arteriogram showing multiple arterioportal shunts (arrows).

Figure 4

A 55-year-old female with known hepatocellular carcinoma. Selective hepatic arteriogram showing a hypervascular tumour in the right lobe (arrow) with arterioportal shunting. Note the early opacification of portal vein, which shows a “thread and streak” sign from tumour thrombus (double arrow).

Figure 5

A 65-year-old man with a large arterioportal shunt after percutaneous liver biopsy. (a) Axial colour Doppler image of the right lobe of liver at the site of cystic lesion seen on a greyscale sonogram (not shown), showing increased colour flow (arrow) at the site of percutaneous liver biopsy. (b) Axial spectral Doppler image of the right portal vein, showing arterialisation with hepatofugal flow due to large arterioportal shunt in the right lobe. (c) Anterior volume-rendered CT image confirming presence of a large arterioportal shunt (arrow) in the right lobe. (d) Selective right hepatic artery angiogram showing a large arterioportal shunt. Note the enlarged portal vein (double arrow) due to shunting into the right portal vein (arrow).

Figure 6

A 17-year-old male with fragile X syndrome and Park Type I portosystemic shunt. (a) Oblique sagittal greyscale sonogram showing a large shunt (arrowhead) between the right portal vein (arrow) and the inferior vena cava (IVC; double arrow). (b) Contrast-enhanced CT axial and (c) coronal images showing a large shunt (black arrow) between the right portal vein (arrow) and the IVC (double arrow).

Figure 7

A 76-year-old female with a history of alcohol abuse and Park Type II portosystemic shunt. (a) Axial contrast-enhanced CT image showing a small shunt (white arrow) involving portal venous branch. (b) Another image 5 mm superior to this level showing the shunt (black arrow) connecting to the right hepatic vein. Bilateral pleural effusions, left larger than right, are also seen.

Figure 8

A 50-year-old male scheduled for liver transplantation for end-stage liver disease secondary to hepatitis C with Park Type III portosystemic shunt. Contrast-enhanced CT (a) axial and (b) sagittal images showing a shunt (white arrows) connecting the main portal vein to the inferior vena cava (black arrows) via a small varix (arrowhead).

Figure 9

A 48-year-old male with cirrhosis and Park Type IV portosystemic shunts. Axial contrast-enhanced CT images showing small shunts (arrows) between the portal vein and hepatic veins in (a) the left lobe and (b) the right lobe. Small right pleural effusion is also present.

Figure 10

A 46-year-old female with a history of heavy alcohol use, transient ischaemic attacks, pulmonary arteriovenous malformations and a large portal vein to inferior vena cava (IVC) shunt (patent ductus venosus). Contrast-enhanced CT coronal image showing a large shunt (double arrow) originating from the bifurcation of the portal vein communicating with the IVC (arrow). Multiple hepatocellular carcinomas were also identified on the same CT examination (not shown).

Figure 11

A 48-year-old male with transjugular intrahepatic portosystemic shunt (TIPS) performed for cirrhosis and portal hypertension. Angiogram showing a patent TIPS shunt in the liver between the right hepatic vein and the right portal vein. Coil embolisation of the varices was performed. The proximal end of the stent extends into the right atrium in this patient, who was not a suitable candidate for liver transplantation. Such extension into the right atrium in a potential liver transplantation candidate may make subsequent liver transplantation difficult.

Figure 12

A 50-year-old male with a surgically created portocaval shunt for cirrhosis and portal hypertension. (a) Oblique coronal colour Doppler sonogram showing a shunt (arrow) seen connecting the inferior vena cava (IVC) to the main portal vein (PV). (b) Axial contrast-enhanced CT image showing the shunt (arrow). Multiple foci of hepatocellular carcinomas are also present.

Figure 13

A 32-week-old foetus with a vascular liver mass. Post-natal resection revealed infantile haemangioendothelioma. Axial colour Doppler image of the foetal abdomen shows a heterogeneous mass in the foetal liver with increased vascularity from arteriosystemic venous shunts.

Figure 14

A 24-year-old female with Budd-Chiari syndrome. (a) Sagittal colour Doppler image showing narrowing in the inferior vena cava (IVC; arrows) with aliasing consistent with stenosis. (b) Oblique transverse colour Doppler image through the liver showing hepatic vein to hepatic vein collaterals (arrows). (c) Venogram through caudate lobe vein (white arrow) showing intrahepatic venovenous collaterals with occlusion (arrowhead) of the terminal right hepatic vein (black arrow). Also note truncation (double arrow) of the intrahepatic segment of the right hepatic vein, as evidenced by non-opacification of the side branches. (d) Right hepatic venogram showing a narrowed and truncated hepatic vein (black arrow) with intrahepatic venous collateral (white arrowhead) drainage through the caudate lobe (white arrow) and an accessory right hepatic vein (black arrowhead).

Figure 15

A 54-year-old male with cirrhosis, portal hypertension and main portal vein to umbilical vein shunt. Axial contrast-enhanced CT image showing a collateral from the main portal vein to the patent umbilical vein (arrow). Moderate ascites and small splenic infarcts are also seen.