From the Guidelines
Severe biliary pancreatitis is a risk factor for the development of chronic portal vein thrombosis (PVT) with cavernous transformation and non-cirrhotic portal hypertension (NCPH).
Pathogenesis
The pathogenesis of PVT and NCPH in the context of severe biliary pancreatitis involves the formation of thrombi in the portal vein, which can be triggered by local factors such as pancreatitis, as well as underlying prothrombotic disorders 1.
- The thrombi can lead to the obstruction of the portal vein, resulting in the formation of numerous, serpiginous vascular channels in the porta hepatis, a condition known as cavernous transformation 1.
- This obstruction can cause increased pressure in the portal vein, leading to portal hypertension, which can manifest as gastrointestinal bleeding, splenomegaly, and portosystemic collaterals 1.
- The severity of portal hypertension can contrast with mild or absent liver dysfunction, and normal levels of transaminases, alkaline phosphatase, and gamma-glutamyl transferase 1.
Relationship between Severe Biliary Pancreatitis and PVT
Severe biliary pancreatitis can lead to the development of PVT and NCPH through several mechanisms:
- Portal cholangiopathy: Severe biliary pancreatitis can cause compression and deformation of intra- and extrahepatic bile ducts by the collateral veins constituting the cavernoma, leading to biliary symptoms such as biliary pain and cholecystitis 1.
- Local factors: Severe biliary pancreatitis can trigger the formation of thrombi in the portal vein, which can lead to PVT and NCPH 1.
- Underlying prothrombotic disorders: Underlying prothrombotic disorders, such as myeloproliferative neoplasm, antiphospholipid syndrome, and inherited thrombophilic factors, can increase the risk of developing PVT and NCPH in patients with severe biliary pancreatitis 1. The diagnosis of EHPVO should be considered in patients with features of portal hypertension or hypersplenism, and in patients affected with a condition associated with a risk for PVT, such as severe biliary pancreatitis 1.
From the Research
Pathogenesis of Severe Biliary Pancreatitis and Chronic Portal Vein Thrombosis
- The pathogenesis of severe biliary pancreatitis and its relationship to chronic portal vein thrombosis (PVT) with cavernous transformation and non-cirrhotic portal hypertension (NCPH) is complex and involves multiple factors 2, 3, 4.
- Portal vein thrombosis can lead to cavernous transformation, which can cause chronic incomplete biliary obstruction due to portal biliary ductopathy 3.
- The development of portal biliary ductopathy is thought to be caused by induction of fibrosis in the biliary tract, loss of biliary motility, chronic cholestasis, and increased formation of cholelithiasis 3.
Relationship between Severe Biliary Pancreatitis and Chronic Portal Vein Thrombosis
- Severe biliary pancreatitis can lead to chronic pancreatitis, which can cause portal vein stenosis or thrombosis, resulting in cavernous transformation and NCPH 5, 4.
- A fistula between a pancreatic pseudocyst and the splenic vein can also contribute to the development of PVT 4.
- The management of cholelithiasis in cases with portal vein cavernous transformation requires special attention due to the risk of complications such as cholangitis, biliary strictures, and hemobilia 3.
Clinical Presentation and Management
- Patients with NCPH and portal cavernomatosis can present with symptomatic biliary disease, including obstructive biliary disease and severe biliary tract abnormalities 6.
- Endoscopic retrograde cholangiopancreatography (ERCP) and palliative treatment with small papillotomy and placement of a plastic biliary endoprosthesis can be effective in managing symptomatic biliary disease 6.
- The treatment of portal cholangiopathy should be individualized, and endoscopic therapy is the preferred choice in symptomatic biliary disease 6.