Prophylactic pylorus preserving pancreatoduodenal resection for advanced duodenal adenomatosis in FAP-results from the ST Mark's hospital register

Abstract

tVackground Ibe majority of patients with Familial Adenomatous Polyposis (FAP) develop duodenal adenomas and these lesions carry significant malignant potential. Disease severity may be assessed by an endoscopic and histological staging system the Spigdman Classification ~*ith advanced (Stage IV disease) carrying a significant risk of rnalignant progression) ,2 Aims To assess the outcome of prophylactic pyiorus preserving pancreatoduodenal resection performed fbr Stage IV duodenal polyposis detected in an endoscopic surveillance programme Methods Surveillance endoscopy was performed with a side viewing duodenoscope to a set protocol. Patients with advanced disease reterred for surgery were included in this study. Data were cdlated prospectively on the Polyposis Register and the case notes reviewed retrospectively,. Results Between 1994 and 2002, hifteen patients (seven male, average age 54 },ears 4 months) underwent prophylactic PPPDR. Eight sulti~red major post operative complications (53%). Although the histology of the resected specimen revealed less severe changes in two patients, fwe with Stage IV disease showed adenocarcmoma. One patient died from multi-organ failure in the post-operative period, and a second patient suffered a fatal pulmonary embolns shortly after hospital discharge. Four of five patients with ampullary adenocareinoma have dmd (10-36 months post operatively) and one further pattern has died from a brain tumour. Thie remaioder are alive at a mean of 36 months (3-103). Conclusion Large ampullary adenomas are most likely to harbour carcinoma in situ. PPPDR is currently the only method of permanently ablating abnormal duodenal mucosa in FAP, but the progmosis is gloomy in the presence of established cancer. However the pmceedure is associated with a significant mortally and morbidity and management decisions are finely halaoced.