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A regular systematic large bowel screening programme, managed by a Polyposis Registry, significantly improves the prognosis of FAP.

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Congenital duodenal obstruction (DO) has a well-known association with Down syndrome (DS) and other congenital malformations. Previously reported series on DO have not examined the influence of DS on associated congenital malformations and postoperative morbidity and mortality. We report on a retrospective study of all children born with DO over an 11-year period to investigate this. A total of 79 patients with DO were studied: group 1 consisted of 51 (64.6%) children without DS, and group 2 consisted of 28 (35.4%) children with DS. There was no significant difference in mean gestational age and birth weight between groups 1 and 2. A coexisting congenital malformation was found in 68% of patients. Gastrointestinal malformations were the most common in group 1 (71.1%), and cardiac malformations were the most common in group 2 (81.5%). The mean time to reach full intragastric feeds was 12 days, with no significant difference between the two groups ( p=0.383). Seven (8.9%) patients developed a postoperative complication, with no significant difference between both groups ( p=0.853). A total of 11 patients died: six (12%) in group 1 and five (28%) in group 2, with no significant difference between the groups ( p=0.454). DS does not influence the morbidity and mortality of DO but does carry a higher incidence of congenital cardiac abnormalities. Delayed mortality was a result of coexisting congenital cardiac and respiratory disease.

Thirty-five consecutive cases of adenocarcinoma of the ampulla of Vater seen over the past 36 years were reviewed. The introduction of new diagnostic techniques over the course of this study improved the accuracy of preoperative diagnosis but did not lead to earlier diagnosis. The surgical resectability rate was 88%, and 53% of postoperative survivors were free of disease at 5 years. Of the 14 patients with metastases to regional lymph nodes, 27% survived disease-free for 5 years. Surgical mortality was 25% for the entire series but has been reduced to 6.6% over the past decade. Surgical mortality was primarily due to leakage of the pancreaticojejunostomy; the risk of pancreaticojejunostomy leak correlated inversely with the degree of chronic pancreatitis in the pancreatic remnant. In 35% of resected cases, a benign adenomatous component was contained within the cancer of the ampulla of Vater. Cure rates are good for this lesion. The most important factor in maximizing cure rate is careful attention to the technical details of pancreaticojejunostomy in order to minimize surgical mortality. Benign adenomas appear to be a frequent precursor of carcinoma of the ampulla of Vater.