Ann Hepatobiliary Pancreat Surg.  2023 Aug;27(3):307-312. 10.14701/ahbps.22-126.

Spontaneous hepatic arterioportal fistula in extrahepatic portal vein obstruction: Combined endovascular and surgical management

• Panda A
• Narayanan D
• Netaji A
• Varshney VK
• Agarwal L
• Garg PK

• KMID: 2545778
• DOI: http://doi.org/10.14701/ahbps.22-126

Abstract

Hepatic arterioportal fistulae are abnormal communications between the hepatic artery and portal vein. They are reported to be congenital or acquired secondary to trauma, iatrogenic procedures, hepatic cirrhosis, and hepatocellular carcinoma, but less likely to occur spontaneously. Extrahepatic portal venous obstruction (EHPVO) can lead to pre-hepatic portal hypertension. A spontaneous superimposed hepatic arterioportal fistula can lead to pre-sinusoidal portal hypertension, further exacerbating its physiology. This report describes a young woman with long-standing EHPVO presenting with repeated upper gastrointestinal variceal bleeding and symptomatic hypersplenism. Computed tomography scan demonstrated a cavernous transformation of the portal vein and a macroscopic hepatic arterioportal fistula between the left hepatic artery and portal vein collateral in the central liver. The hepatic arterioportal fistula was associated with a flow-related left hepatic artery aneurysm and a portal venous collateral aneurysm proximal and distal to the fistula, respectively. Endovascular coiling was performed for the hepatic arterioportal fistula, followed by proximal splenorenal shunt procedure. This case illustrates an uncommon association of a spontaneous hepatic arterioportal fistula with EHPVO and the utility of a combined endovascular and surgical approach for managing multifactorial non-cirrhotic portal hypertension in such patients.

Keyword

Arterioportal fistulas; Extrahepatic portal venous obstruction; Embolization; Endovascular; Portal hypertension

Figure

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  Fig. 1 (A) Coronal maximum intensity projection image from arterial phase computed tomography (CT) demonstrating a left hepatic artery aneurysm (solid white arrow) with a large branch leading to early arterial opacification of portal venous collateral (dashed white arrow) suggestive of hepatic arterioportal fistula (HAPF). (B) Axial thin section arterial phase CT at the level of portal venous collateral demonstrating early arterial opacification of portal venous collateral (arrow) before opacification of splenic and superior mesenteric veins consistent with HAPF. (C) Axial thin section venous phase CT at the level of portal venous collateral (same as B) demonstrating a portal collateral vein aneurysm (solid white arrow) distal to the HAPF. Also shown is a cavernous transformation of extrahepatic portal vein (dashed white arrow) consistent with extrahepatic portal venous obstruction (EHPVO). (D) Coronal multiplanar reformat image from venous phase CT demonstrating additional findings of EHPVO such as portosystemic collaterals (black arrow) and massive splenomegaly. The superior mesenteric vein (dashed white arrow) and splenic vein (solid white arrow) were patent in this case.

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  Fig. 2 Volume rendered technique image from arterial phase computed tomography demonstrating the spectrum of arterial findings, including left hepatic artery (LHA) aneurysm and macroscopic hepatic arterioportal fistula (HAPF) with arterial opacification of portal vein collateral. Also note the replaced right hepatic artery (RHA) arising from superior mesenteric artery (SMA) coursing posterior to the HAPF and the markedly ectatic splenic artery with small aneurysm.

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  Fig. 3 Selected digital subtraction angiogram images demonstrating endovascular management of hepatic arterioportal fistula (HAPF). Pre-embolization images from common hepatic artery angiogram (A) and left hepatic artery angiogram (B) demonstrating the left hepatic artery aneurysm (solid black arrow in A), shunting of arterial blood into a portal venous channel (dashed black arrows in A, B), and the macroscopic fistulous connection between left hepatic artery and the portal venous collateral (solid black arrow in B). (C) Post- embolization image from left hepatic artery angiogram showing complete obliteration of both left hepatic artery aneurysm and HAPF with no residual flow.

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  Fig. 4 (A) Coronal maximum intensity projection image from portal venous phase computed tomography (CT) demonstrating endovascular coil in left hepatic artery (solid black arrow). The splenorenal shunt is patent (black arrow with white outline). Proximal splenic vein (solid white arrow) and left renal vein (dashed white arrow) draining into the inferior vena cava are expected to be prominent. The replaced right hepatic artery is denoted by a dashed black arrow. Axial (B) and coronal (C) thin section portal venous phase CT at the level of porta demonstrating resolution of portal cavernoma without any residual portal vein collateral aneurysm (white arrows). The peribiliary venous plexus has also regressed with resolved central intrahepatic biliary dilation. (D) Coronal multiplanar reformat image from venous phase CT demonstrating expected changes of proximal splenorenal shunt with splenectomy, resolution of portosystemic collaterals and dilation of splenic vein (solid white arrow), and superior mesenteric vein (dashed white arrow) due to diversion of portal circulation.

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