Giant Intrahepatic Portal Vein Aneurysm: Leave it or Treat it?

Abstract

Portal vein aneurysm (PVA) is a rare vascular dilatation of the portal vein. It is a rare vascular anomaly representing less than 3% of all visceral aneurysms and is not well understood. Usually, PVA are incidental findings, are asymptomatic, and clinical symptoms are proportionally related to size. Patients present with nonspecific epigastric pain or gastrointestinal bleeding with underlying portal hypertension. PVA may be associated with various complications such as biliary tract compression, portal vein thrombosis/rupture, duodenal compression, gastrointestinal bleeding, and inferior vena cava obstruction. Differential diagnoses of portal vein aneurysms are solid, cystic, and hypervascular abdominal masses, and it is important that the radiologists be aware of their multi-modality appearance; hence, the aim of this article was to provide an overview of the available literature to better simplify various aspects of this rare entity and diagnostic appearance on different modality with available treatment options. In our case, a 55-year-old male patient came to the gastroenterology OPD for further management of pancreatitis with portal hypertension and biliary obstruction with plastic stents in CBD and PD for the same. In this article, we have reported a case of largest intrahepatic portal vein aneurysm and its management by endovascular technique. As per our knowledge, this is the largest intrahepatic portal vein aneurysm and first case where the endovascular technique was used for the treatment of the same.

Unusual discovery in the liver: intrahepatic portal vein aneurysm

“…On US, it is seen as an anechoic lesion on greyscale, with colour flow and monophasic waveform on duplex Doppler US. Computed tomography/magnetic resonance imaging may be helpful in distinguishing PVA from a cystic/hypervascular mass . Asymptomatic patients such as ours can be observed with regular surveillance US scans.…”

“…PVA is very rare vascular anomaly with an incidence of 0.067–0.43%, first described by Barzilai and Kleckner in 1956 . Most (63%) are extrahepatic.…”

“…Acquired causes include chronic liver disease, pancreatitis, trauma, invasive malignancies or previous surgery. Congenital aneurysms are thought to arise due to failure of regression of the right primitive vitelline vein . Most patients are asymptomatic, but some may experience epigastric or right hypochondriac pain, gastrointestinal bleeding from portal hypertension, obstructive jaundice or gastric outlet obstruction due to a large PVA causing compression of the bile ducts or duodenum, or fever and pain due to thrombosis of the aneurysm.…”

“…Most patients are asymptomatic, but some may experience epigastric or right hypochondriac pain, gastrointestinal bleeding from portal hypertension, obstructive jaundice or gastric outlet obstruction due to a large PVA causing compression of the bile ducts or duodenum, or fever and pain due to thrombosis of the aneurysm. Spontaneous rupture has been reported in only two cases . On US, it is seen as an anechoic lesion on greyscale, with colour flow and monophasic waveform on duplex Doppler US.…”

“…Intervention is indicated mainly for complicated PVA such as those with bothersome symptoms, expansion over time, thrombosis, mass effect or rupture or for non‐thrombotic PVA >3 cm . Therapeutic modalities include aneurysmorrhaphy or aneurysmectomy, and shunt procedures or liver transplantation in the setting of portal hypertension . Endovascular techniques have also been employed in certain cases.…”