Familial adenomatous polyposis (FAP) is a colonic cancer syndrome which runs in families. It presents as mutliple adenomas in the colon which will become cancer of colon unless the colon is removed surgically.Though the cancer will present in the thirties, we have seen this presenting even at an age of 9 or 10.This runs in families caused by adenomatous polyposis coli (APC) gene, located on chromosome 5q21.
Patients present with hundreds of adenomatous polyps in the colon and rectum usually in adolescent phase which become cancer of colon by the thirties. Thought the polyps are found throughout the colon, more are found on the left half of colon making then easily diagnosable.
The upper gastrointestinal tract too can have its share of duodenal polyps which can become cancerous. They are mostly around the ampulla of Vater found in the second part of the duodenum.Stomach too can have multiple polyps.
Gardener's syndrome is a condition characterized by gastrointestinal polyps , osteomas , desmoid tumors of skin and soft tissues and thyroid tumors.
Definitive treatment consists of subtotal colectomy with ileorectal anastomosis,total proctocolectomy with a continent ileostomy, or proctocolectomy with ileoanal pouch.
Out of all these methods, proctocolectomy with an ileal pouch–anal anastomosis
is surgical treatment of choice,.Here, there is complete resection of colorectal mucosa with preservation of anal defecation.After definitive surgery,endoscopic surveillance of the
ileoanal anastomosis is useful as chances of polyps exist in the subsequent years.
Medical treatment consists of Sulindac, a nonsteroidal antiinflammatory drug.Celecoxib decreases duodenal polyposis.
Endoscopic methods include polypectomy, thermal ablation using monopolar,bipolar cautery or argon plasma coagulation, and laser coagulation.
Genetic counselling is useful.
A technique of the surgery of total colectomy using laparoscopic technique is outlined as a single incision surgery at http://www.youtube.com/watch?v=Wq4FFIbNiPg
Dr John AC Thanakumar
www.lapsurge.org
Dr John AC Thanakumar
www.lapsurge.org
