{"id":2815,"date":"2010-02-15T22:46:49","date_gmt":"2010-02-16T03:46:49","guid":{"rendered":"http:\/\/insidesurgery.com\/?p=2815"},"modified":"2013-08-02T22:13:12","modified_gmt":"2013-08-03T02:13:12","slug":"gardners-syndrome","status":"publish","type":"post","link":"https:\/\/insidesurgery.com\/2010\/02\/gardners-syndrome\/","title":{"rendered":"Gardner’s Syndrome"},"content":{"rendered":"
Pathophysiology<\/strong><\/p>\n 1)<\/strong> hereditary polyposis coli syndrome with large and small bowel carpeted by adenomatous polyps 2)<\/strong> occurs in association with other tumors and hypertrophic tissue 1)<\/strong> thousands of adenomatous polyps in large and small bowel (usually do not develop until after puberty) 2)<\/strong> mesenteric desmoid tumors 3)<\/strong> epidermoid inclusion cysts (sebaceous cysts) especially on face 4)<\/strong> soft tissue tumors (lipomas and fibromas) 5)<\/strong> congenital retinal hypertrophy 6)<\/strong> cancer of ampulla of Vater<\/p>\n Histology\/Gross Pathology<\/strong><\/p>\n polyps are adenomas<\/p>\n Associated Conditions<\/strong><\/p>\n 100% of patients develop adenocarcinoma of bowel before age 40 years<\/p>\n Inheritance\/Epidemiology<\/strong><\/p>\n 1)<\/strong> autosomal dominant (occasionally occurs as sporadic condition) 2)<\/strong> deletion of APC gene on long arm of chromosome 5<\/p>\n Treatment<\/strong><\/p>\n total colectomy with ileoanal anastomosis<\/p>\n Tips for USMLE<\/strong><\/p>\n if a 6 year old girl develops multiple epidermoid inclusion cysts on her face, has hypertrophic renal tissue, but has no polyps on upper and lower barium studies, think Gardner’s<\/p>\n
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\nSigns and Symptoms<\/strong><\/p>\n