Pancreaticoportal Fistula Formation as a Consequence of Recurrent Acute Pancreatitis: Clinical and Imaging Considerations

Abstract

A pancreatic-portal vein fistula (PPVF) is an extremely rare complication of pancreatitis. In this report, we present a patient with a PPVF in the setting of recurrent pancreatitis and portal vein thrombosis diagnosed by cross-sectional imaging and endoscopic retrograde cholangio-pancreatography, who responded to medical management and anticoagulation. A critical review of this disease process is explored to highlight pathology, imaging characteristics, and essential alternative diagnostic considerations. We also discuss potential complications and current treatment strategies. PPVFs pose a unique diagnostic challenge and awareness of its clinical presentation can further improve patient outcomes.

Spontaneous Pancreatic Pseudocyst-Superior Mesenteric Vein Fistula

“…1,[3][4][5][6] It is also possible that the fistula develops first because of pancreatic enzymes destroying the pseudocyst and vein walls and inflammation from the pancreatic enzymes within the vein leads to thrombosis. 1,3,[5][6][7][8] Several cases that involved serial imaging documented the presence of fistula before thrombosis, suggesting that at least in some cases, the second theory seems more likely. 4,8 CT is a common initial imaging modality for patients with PP-PV fistulas.…”

Spontaneous pancreatic pseudocyst – superior mesenteric vein fistula: A rare complication of chronic pancreatitis

“…Vascular complications of chronic pancreatitis are also relatively common, occurring in up to 12% of patients [3] . While portal vein thrombosis and arterial pseudoaneurysms are well described in the literature, pancreatic pseudocyst - portal vein fistula (PPVF) is an extremely rare complication of chronic pancreatitis, with only a few cases reported till date [4] . The majority of these cases are alcohol-related and more often seen in men [4 , 5] .…”

“…Serious complications include septic shock, systemic lipolysis, hemorrhage, and death. Systemic lipolysis (Weber-Christian disease) is a dreaded complication of PPVF due to release of pancreatic contents into the systemic circulation through the fistula, associated with a high mortality rate [3] , [4] , [5] . Clinically, patients present with polyarthritis, polyserositis, necrotic bone lesions, and purpuric nodules on the extremities.…”

“…Endoscopic ultrasound may be useful both for diagnosis and intervention, demonstrating complex fluid within the portal vein with absent flow on color Doppler. An important imaging pitfall is differentiating PPVFs from bland portal vein thrombosis due to hypercoagulable state or extrinsic compression, and pancreatic malignancy with tumor thrombus invasion of the portal venous system [4] . Unlike pancreatic neoplasms, pseudocysts demonstrate central fluid signal with only peripheral wall enhancement after contrast administration.…”

Vascular complications of pancreatitis

“…CT with contrast generally demonstrates a fluid-attenuated portal vein with possible collateral periportal vessels and pseudocyst. Magnetic resonance cholangiopancreatography findings on T2 weighted sequences include; hyperintense portal vein and fluid signal; occasionally, visualization of the hyperintense fistulous tract could be seen[ 32 ]. In contrast, in cases of portal vein thrombosis only, ultrasonography would show decreased or absent flow on the doppler without complex fluid collection.…”