Total pancreatectomy involves the resection of the whole pancreas, the common bile duct, the gallbladder, the duodenum, a short segment of small intestine beyond the duodenum, the pylorus (outlet of the stomach), the distal (lower) part of the stomach, the spleen, and regional lymph nodes (Figure 1A).

After resection, the end of the remaining bile duct and the stomach are connected to the small bowel to ensure flow of bile and food into the intestines (Figure 2). Two anastomoses are constructed (Figure 1B): 

• Biliary anastomosis. Hepatico-jejunostomy is performed between common hepatic duct remnant and a site on the jejunum.
• Enteric anastomosis. An antecolic anastomosis is constructed between the stomach and the jejunum; distal to the hepatico-jejunostomy.

Indications to single-stage elective total pancreatectomy include the presence of a multifocal neoplasm (intraductal papillary mucinous neoplasia, pancreatic metastases from clear cell renal carcinoma, neuroendocrine tumor in the setting of MEN1), or the presence of an intraductal papillary mucinous neoplasm involving the entire main pancreatic duct. Single-stage unplanned total pancreatectomy may be necessary after an initial partial pancreatectomy because of positive resection margins on intraoperative frozen section. Two-stage total pancreatectomy (completion pancreatectomy) is performed because of severe postoperative complications or neoplastic recurrence in the pancreatic remnant after previous pancreatic resection. 

Total pancreatectomy is invariably associated with the development of exocrine insufficiency (inability to properly digest food) and endocrine insufficiency (diabetes mellitus). Management of pancreatic insufficiency include pancreatic enzyme replacement therapy and insulin therapy. Exocrine insufficiency and diabetes may be particularly hard to control in the first months after the operation, but studies have indicated that quality of life in the long term is satisfactory.

Figure 1. A: resection phase; B: reconstruction.