Natural history of spontaneous pancreatic-portal vein fistulae: A case report

PPVF is a rare complication of acute and chronic pancreatitis, often associated with alcohol-related disease. Pathogenesis may involve enzymatic erosion of the portal vein or secondary fistulization following PVT. Clinical recognition is difficult due to variable presentations and limited data on natural history. We report a 55-year-old man with chronic calcifying alcoholic pancreatitis and a history of bilateral segmental pulmonary embolism. He presented with 3 days of worsening epigastric pain and fever, in shock with tachycardia, hypotension, and peripheral hypoperfusion. Laboratory tests revealed marked inflammation, metabolic acidosis, acute kidney injury, and hepatic cytolysis with cholestasis. Contrast-enhanced CT demonstrated acute-on-chronic pancreatitis complicated by PVT extending into the superior mesenteric and splenic veins, peri-pancreatic fluid collections, and findings suggestive of a pseudocysto–pancreatico–portal fistula, along with hepatic abscesses, venous intestinal ischemia, and SMA stenosis. Despite aggressive management with broad-spectrum antibiotics, therapeutic anticoagulation, and intensive supportive care, the patient developed multiorgan failure with progressive ischemia and shock. Exploratory laparotomy revealed extensive intestinal ischemia precluding surgical intervention. The patient subsequently died from combined arterial and venous hepatic and intestinal ischemia. PPVF is a rare but severe complication, with variable clinical presentations ranging from incidental findings to catastrophic multiorgan involvement. MRCP is the preferred diagnostic modality, while ERCP may be considered for equivocal cases. Management is highly individualized, considering comorbidities and procedural risks. This case highlights that even with maximal supportive care, PPVF may rapidly progress to multiorgan failure. PPVF in acute-on-chronic pancreatitis can result in devastating outcomes. High clinical suspicion, early imaging, and prompt individualized intervention are essential, but prognosis may remain poor in severe presentations.