Causes and Management of Non-cirrhotic Portal Hypertension​

Abstract

Purpose of the Review Non-cirrhotic portal hypertension (NCPH) includes a heterogeneous group of conditions. The aim of this paper is to make an overview on the denominations, diagnostical features and management of porto-sinusoidal vascular disease (PSVD) and chronic portal vein thrombosis (PVT) being the main causes of NCPH in the Western world. Recent Findings The management of NCPH consists in the treatment of associated diseases and of portal hypertension (PH). PH due to PSVD or PVT is managed similarly to PH due to cirrhosis. TIPS placement and liver transplantation are considerable options in patients with refractory variceal bleeding/ascites and with progressive liver failure. Anticoagulation is a cornerstone both in the treatment of thrombosis in PSVD and in the prevention of thrombosis recurrence in patients with portal cavernoma. Summary Physicians should be aware of the existence of PSVD and chronic PVT and actively search them in particular settings. To now, the management of portal hypertension-related complications in NCPH is the same of those of cirrhosis. Large cooperative studies on the natural history of NCPH are necessary to better define its management.

Idiopathic Non-Cirrhotic Portal Hypertension and Porto-Sinusoidal Vascular Disease: Review of Current Data

“…Therefore, in the absence of specific and universally implemented guidelines, the screening frequency and primary and secondary prophylaxis for variceal bleeding in INCPH are similar to those recommended for cirrhotic PH. The prophylactic measures include the use of non-specific beta-adrenergic blockage and endoscopic band ligation [ 2 , 56 , 80 , 113 ]. Similarly, the management for ascites and hepatic encephalopathy is identical to those in cirrhotic patients.…”

A success story of modified Sugiura technique surgery in a non-cirrhotic portal hypertension (NCPH)

“…The presence of low liver stiffness (<10 kPa) in patients with clinically evident portal hypertension may exclude liver cirrhosis diagnosis. 18 In a study, the median hepatic venous pressure gradient in NCPH patients was 5.02-10 mmHg, and LS of 6.6± 2.7 kPa. 19 On endoscopic examination, our patient revealed grade IV esophageal varices GOV-1, and mild portal hypertensive gastropathy at admission.…”

Report of a case of cirrhotic portal hypertension with ectopic varices in the bilateral pulmonary hilar

“…4 The causes of portal hypertension-derived ectopic varices are mainly as follows: (1) intrahepatic portal hypertension, for example, hepatic cirrhosis, tumor invasion combined with the blockage of portal vein branches, hepatic vein thrombosis (with hypersplenism in some cases), and so forth; (2) extrahepatic portal hypertension, including portal veins (thrombosis or cancer embolus formation, spongelike changes, extrinsic compression) and splenic veins (thrombosis, extrinsic compression). 5 These types of portal hypertension often cause duodenal varices, most commonly in the bulbous part, followed by the descending part. The mechanism of abdominal adhesion is that the formation of adhesions between the intestinal tract and the abdominal wall or other structures after abdominal surgery leads to the formation of collateral branches between the portal and systemic circulation.…”

“…The pathogenesis of ectopic varices mainly includes portal hypertension and abdominal adhesions 4 . The causes of portal hypertension‐derived ectopic varices are mainly as follows: (1) intrahepatic portal hypertension, for example, hepatic cirrhosis, tumor invasion combined with the blockage of portal vein branches, hepatic vein thrombosis (with hypersplenism in some cases), and so forth; (2) extrahepatic portal hypertension, including portal veins (thrombosis or cancer embolus formation, spongelike changes, extrinsic compression) and splenic veins (thrombosis, extrinsic compression) 5 . These types of portal hypertension often cause duodenal varices, most commonly in the bulbous part, followed by the descending part.…”