| Summary: | In a subset of patients with acute necrotizing pancreatitis, segmental necrosis affecting the main pancreatic duct may result in a discontinuity between the left-sided pancreas and the duodenum. Such an interruption in the setting of a viable upstream portion of the gland can give rise to the disconnected pancreatic duct syndrome (DPDS). By maintaining its secretory function, the disconnected segment may lead to persistent external pancreatic fistulae, recurrent pancreatic fluid collections, and/or obstructive recurrent acute or chronic pancreatitis of the isolated parenchyma. There are currently no universally accepted guidelines for the diagnosis or treatment of DPDS, and because the condition is underrecognized, the diagnosis is often delayed. DPDS is associated with a prolonged disease course and poses a burden on patients' quality of life as well as high health care resource utilization. The aim of our review is to summarize current knowledge, discuss diagnostic approaches, outline management options, and raise awareness of this challenging complication of necrotizing pancreatitis.
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