Abstract
Multiple colonic polyps, almost guaranteed colorectal cancer by the age of forty-five and an increased risk of non-colonic cancers characterise the autosomal dominant condition Familial Adenomatous Polyposis (FAP) [1]. The patients and families faced with such a diagnosis present many difficult management challenges, both surgical and non-surgical. We discuss the current surgical options for treatment of the more significant manifestations of FAP arising in the colorectum and duodenum as well as desmoid disease
Highlights
Of the many adenomatous polyps which develop in the colon and rectum of patients with Familial Adenomatous Polyposis (FAP), “...one or more of the adenomata form... a malignant adenocarcinoma” [2]
Most patients should have been offered a colectomy by the start of their third decade of life, as 15% of FAP patients will present with colorectal cancer before the age of 25 [5]; surveillance usually starts from the age of 10 [6]
The former involves a permanent ileostomy and will not be discussed in detail. The latter, first described in 1978 [13], avoids a permanent stoma by constructing a new reservoir which is re-anastomosed to the anal canal. This panproctocolectomy with ileal pouch-anal anastomosis (IPAA) requires the whole colon and rectum to be mobilised and the large bowel removed by dividing the intestine at the ileocaecal and anorectal junctions
Summary
The latter, first described in 1978 [13], avoids a permanent stoma by constructing a new reservoir which is re-anastomosed to the anal canal This panproctocolectomy with ileal pouch-anal anastomosis (IPAA) requires the whole colon and rectum to be mobilised and the large bowel removed by dividing the intestine at the ileocaecal and anorectal junctions. Patients subjected to a hand-sewn anastomosis are more likely to experience a poorer functional outcome [14, 29], with increased bowel action and faecal incontinence more common [29] This may be a consequence of the anal stretch required to facilitate the anastomosis, or the removal of the anal transitional zone during the mucosectomy [26]. Often seen in IPAA procedures carried out for inflammatory bowel disease [32], is very unusual in FAP patients [17, 29]
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