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Review
. 2024 May 27;16(5):751-765.
doi: 10.4254/wjh.v16.i5.751.

Current concepts in the management of non-cirrhotic non-malignant portal vein thrombosis

Adam J Willington  1 , Dhiraj Tripathi  2
Affiliations
Review

Current concepts in the management of non-cirrhotic non-malignant portal vein thrombosis

Adam J Willington et al. World J Hepatol. .

Abstract

Non-cirrhotic non-malignant portal vein thrombosis (NCPVT) is an uncommon condition characterised by thrombosis of the portal vein, with or without extension into other mesenteric veins, in the absence of cirrhosis or intra-abdominal malignancy. Complications can include intestinal infarction, variceal bleeding and portal biliopathy. In this article, we address current concepts in the management of NCPVT including identification of risk factors, classification and treatment, and review the latest evidence on medical and interventional management options.

Keywords: Mesenteric veins; Non-cirrhotic portal vein thrombosis; Portal hypertension; Portal vein; Venous thrombosis.

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Conflict of interest statement

Conflict-of-interest statement: Adam J Willington – no conflicts of interest. Dhiraj Tripathi – Speaker honorarium from WL Gore & Associates.

Figures

Figure 1
Figure 1
Cross-sectional images of recent portal vein thrombosis and subsequent cavernoma formation. A: Axial computed tomography demonstrating acute portal vein thrombosis (Orange arrow) with altered hepatic parenchymal attenuation secondary to ischaemia; there is an incidental large splenic cyst; B: Coronal computed tomography demonstrating acute portal vein thrombosis (Orange arrow) with altered hepatic parenchymal attenuation secondary to ischaemia; there is an incidental large splenic cyst; C: Axial computed tomography 6 months later in the same patient, demonstrating formation of portal vein cavernoma (Orange arrow); D: Coronal computed tomography 6 months later in the same patient, demonstrating formation of portal vein cavernoma (Orange arrow).
Figure 2
Figure 2
Suggested management algorithm for recent non-cirrhotic non-malignant portal vein thrombosis. 1Major risk factors include: Myeloproliferative neoplasms, antiphospholipid syndrome, paroxysmal nocturnal haemoglobinuria, homozygous or compound heterozygous factor V Leiden or prothrombin G20210A gene mutations, personal or first-degree familial history of unprovoked deep vein thrombosis. 2Consider measuring D-dimer. If D-dimer > 500 ng/mL at 1 month following anticoagulation discontinuation consider long-term anticoagulation. NCPVT: Non-cirrhotic non-malignant portal vein thrombosis.
Figure 3
Figure 3
Suggested management algorithm for chronic non-cirrhotic non-malignant portal vein thrombosis. 1Major risk factors include: Myeloproliferative neoplasms, antiphospholipid syndrome, paroxysmal nocturnal haemoglobinuria, homozygous or compound heterozygous factor V Leiden or prothrombin G20210A gene mutations, personal or first-degree familial history of unprovoked deep vein thrombosis. 2Consider long-term anticoagulation on a case-by-case basis. Where a decision is made to discontinue existing anticoagulation consider measuring D-dimer. If D-dimer > 500 ng/mL at 1 month following anticoagulation discontinuation consider long-term anticoagulation. NCPVT: Non-cirrhotic non-malignant portal vein thrombosis; PVR: Portal vein recanalization; TIPS: Transjugular intrahepatic portosystemic shunt.
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