Fistula of a pancreatic pseudocyst into the superior mesenteric and portal veins causing erythema nodosum and aseptic polyarthritis - case report and review of literature

Introduction Extra-pancreatic complications of acute and chronic pancreatitis that do not relate to vital organs are rare. The most common include subcutaneous paniculitis, arthritis, bone marrow fat necrosis, and vasculitis. These associated conditions have been termed pancreatic disease syndrome (PDS), which can occur not only with pancreatitis but also in other pancreatic diseases. PDS is believed to be caused by circulating pancreatic enzymes, which can occur when the pancreas is in direct communication with the circulation. Pancreatic pseudocyst erosion into the superior mesenteric and portal veins is extremely rare; and there have only been 22 previously reported cases in literature. The authors endeavoured to describe a manifestation of PDS with formation of a pseudocystic-portal fistula, its complications, and propose adequate surgical management. Case outline We present a 37-year-old man with chronic alcoholic pancreatitis and a pancreatic pseudocyst within the head of the pancreas which communicated with the main pancreatic duct on one side and eroded into the superior mesenteric and portal veins on the other, causing erythema nodosum-like vasculitis, and polyarthritis. The patient was initially treated conservatively, but subsequently required multiple arthrotomies and finally underwent pylorus preserving duodenopancreatectomy and direct repair of the affected veins. Conclusion The majority of cases required aggressive surgical intervention due to heightened risk of hemorrhage. In patients who develop disseminated fat necrosis, an earlier surgical intervention can be justified. The authors would recommend that, where practical, a pylorus-preserving pancreaticoduodenectomy should be performed.