Portal vein aneurysm: What to know

Abstract

Portal vein aneurysm is an unusual vascular dilatation of the portal vein, which was first described by Barzilai and Kleckner in 1956 and since then less than 200 cases have been reported. The aim of this article is to provide an overview of the international literature to better clarify various aspects of this rare nosological entity and provide clear evidence-based summary, when available, of the clinical and surgical management. A systematic literature search of the Pubmed database was performed for all articles related to portal vein aneurysm. All articles published from 1956 to 2014 were examined for a total of 96 reports, including 190 patients. Portal vein aneurysm is defined as a portal vein diameter exceeding 1.9 cm in cirrhotic patients and 1.5 cm in normal livers. It can be congenital or acquired and portal hypertension represents the main cause of the acquired version. Surgical indication is considered in case of rupture, thrombosis or symptomatic aneurysms. Aneurysmectomy and aneurysmorrhaphy are considered in patients with normal liver, while shunt procedures or liver transplantation are the treatment of choice in case of portal hypertension. Being such a rare vascular entity its management should be reserved to high-volume tertiary hepato-biliary centres.

Intrahepatic Portal Vein Aneurysm with Concurrent Hepatocellular Carcinoma

“…The most common cause of acquired PVA is portal hypertension related to chronic liver disease, such as cirrhosis. [3][4][5] Long-standing portal hypertension causes intimal thickening with compensatory medial hypertrophy of the portal vein. 3 With time, the medial hypertrophy is replaced with fibrous tissue leading to a weakening of the vein wall, thus making it susceptible to aneurysmal dilatation.…”

“…Other secondary causes of PVA include pancreatitis, trauma and invasive malignancy. 1,5 To the best of the authors' knowledge, no data yet exist in the available English scientific literature evidencing a direct relationship between HCC and PVA. As such, this remains an exciting avenue to be explored.…”

“…1 Contrast-enhanced CT allows multi-planar 3D image reconstruction and can clearly demonstrate the size, location and extent of an aneurysm. 5 Within the PVA, CT or magnetic resonance imaging differentiate slow-flowing blood from thromboses. 1 More invasive imaging techniques such as direct or indirect portography are other potential confirmatory investigations, particularly if a portocaval fistula is suspected.…”

“…1 In general, PVAs require no treatment and careful follow-up is adequate. 2,5 The decision to treat a PVA depends on its size and location, the existence of symptoms and the presence of thrombosis. 2 It is important to be familiar with the imaging appearance and nature of PVAs to allow for appropriate management of the condition.…”

Klinefelter Syndrome with Portal Vein Aneurysm: Case Report

“…Portal vein aneurysm (PVA) is the most common site of visceral VA, representing fewer than 3% of all VAs (2-4). Less than 200 cases of PVAs have been reported since first discovered in 1956 by Barzilai and Kleckner (5,6). PVA is usually detected incidentally on imaging studies.…”