Disconnected pancreatic duct syndrome: Complete pancreas transection secondary to acute pancreatitis

Abstract

Disconnected pancreatic duct syndrome: complete pancreas transection secondary to acute pancreatitisKey words: Pancreas. Pancreatitis. Disconnected pancreatic duct syndrome. Dear Editor,Disconnected pancreatic duct syndrome (DPDS) is defined by a complete discontinuity of the pancreatic duct, such that a viable side of the pancreas remains isolated from the gastrointestinal tract. The most frequent causes are acute pancreatitis and pancreatic trauma, with a 10-30% incidence in acute pancreatitis (1,2). However, there is a lack of reliable epidemiologic data. This pancreatic disruption is infrequent in clinical practice and its diagnostic and therapeutic management are controversial. We present an extreme case of disconnected pancreatic duct syndrome with complete duct disruption and pancreatic transection following acute pancreatitis, as well as the diagnostic and therapeutic processes carried out. Case reportA 56-year-old female with a medical history of dyslipidemia and hypothyroidism was admitted to a regional hospital for severe acute idiopathic pancreatitis. During hospitalization she developed a large pseudocyst in the pancreatic neck region. The patient underwent 3 unsuccessful attempts to cannulate the pancreatic duct by endoscopic retrograde cholangiopancreatography (ERCP) as it was not possible to identify the pancreatic duct distal to the pseudocyst. Percutaneous drainage was then indicated, which resolved the pseudocyst but led to an external pancreatic fistula. The patient was discharged after conservative management of the fistula for three months, and referred to our hospital for reconsideration of the therapeutic management.By that time, the patient was asymptomatic and the pancreatic fistula remained closed. However, she presented an exocrine pancreatic insufficiency treated with pancreatic enzyme replacement therapy. The patient underwent conservative management and close follow-up with physical examination and radiologic imaging with CT-scan or MRI alternatively with endoscopic ultrasound every 6 months.After two years of uneventful follow-up the patient developed progressive abdominal pain. Radiologic imaging demonstrated a marked dilation of distal pancreatic duct ( Fig. 1 A and B). This finding was confirmed by endoscopic ultrasound suggesting an intraductal papillary mucinous neoplasm (IPMN).Due to the above findings, the patient underwent surgery. Intraoperatively, a complete transection throughout the pancreas parenchyma with duct disruption at the neck was found, with a space between both pancreatic edges of about 2 cm, where portomesenteric axis was located. Distal pancreatectomy involving isolated pancreatic side and splenectomy was carried out.The patient evolved without complications after surgery and was discharged on the 7 th post-operative day. Histopathologic examination revealed chronic pancreatitis without any characteristic suggestive of IPMN. DiscussionDisconnected pancreatic duct syndrome (DPDS) is characterized by a discontinuity of the pancreatic duct and consequent lack ...