Disconnected pancreatic duct syndrome: disease classification and management strategies.

BACKGROUND: Disconnected pancreatic duct syndrome (DPDS) typically complicates acute necrotizing pancreatitis (ANP) and presents as a pseudocyst months after the initial episode of pancreatitis. However, our experience suggests that the presentation of DPDS may be quite varied and might require significant evaluation and judgment before surgical intervention. We sought to determine the presentations of DPDS and assess the management of the various forms of presentation.

STUDY DESIGN: A retrospective review of all patients with DPDS between July 2005 and June 2011 was performed. Patients were included when CT scan demonstrated a clear disconnected pancreas that was confirmed at operation. Medical records were reviewed in detail to determine clinical presentation, management, and outcomes.

RESULTS: Of the 50 patients identified, 66% were male, with a mean age of 53 ± 16 years. Mortality was 2% and 3 patients (6%) required late reoperation. The DPDS presented in 3 forms: diagnosed concurrently with ANP (concurrent DPDS; n = 28); delayed presentation with a pseudocyst (delayed DPDS; n = 15); and as a consequence of chronic pancreatitis (CP) (CP DPDS; n = 7). Concurrent DPDS was treated with necrosectomy including body/tail resection within 60 days of onset and complicated by a grade B/C fistula in 36%. Delayed DPDS required distal pancreatectomy 440 days after diagnosis, with a 7% fistula rate. Chronic pancreatitis DPDS was treated with lateral pancreatojejunostomy at 417 days with no fistulas.

CONCLUSIONS: Disconnected pancreatic duct syndrome presents concurrently with ANP, in a delayed fashion, or infrequently in the setting of CP. Prompt recognition and classification with appropriate operative therapy results in low mortality and nonoperatively managed pancreatic fistulas.