Pylorus-preserving pancreaticoduodenectomy for advanced duodenal disease in familial adenomatous polyposis.
BACKGROUND: Although only 5 per cent of patients with familial adenomatous polyposis (FAP) die from duodenal cancer, a recent study indicated that the mortality rate is much higher in patients with Spigelman stage IV disease. This has prompted an increased rate of referral for excisional surgery and an analysis of the results. METHODS: Between January 1994 and June 2002, 16 patients with FAP (mean age 55 years; eight men) were referred to a single surgeon for pylorus-preserving pancreaticoduodenal resection for Spigelman stage IV duodenal adenomatosis. RESULTS: One patient died from multiple organ failure after relaparotomy for haemorrhage and a jejunal perforation; other major complications included anastomotic leak (one), primary haemorrhage (one), lymphatic leak (one), chylous ascites (one), pulmonary embolus (two) and prolonged delayed gastric emptying that required total parenteral nutrition (three). Overall there were 11 major complications in eight patients. Two patients developed insulin-dependent diabetes and one postprandial dumping. Postoperative histological examination revealed five unsuspected cancers, which led to four deaths within 3 years of surgery. One patient died 2 months after surgery from pulmonary thromboembolism and another at 5 months from an inoperable brain tumour. Nine of the 16 patients were alive and well at a mean of 38 months after surgery. CONCLUSION: The choice between continued endoscopic surveillance and excisional surgery for Spigelman stage IV duodenal disease remains finely balanced.