{"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
\n
\nSigns and Symptoms<\/strong><\/p>\n

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

Related Posts<\/strong><\/p>\n

Colon Cancer<\/a><\/p>\n","protected":false},"excerpt":{"rendered":"

Pathophysiology 1) hereditary polyposis coli syndrome with large and small bowel carpeted by adenomatous polyps 2) occurs in association with other tumors and hypertrophic tissue Signs and Symptoms 1) thousands of adenomatous polyps in large and small bowel (usually do not develop until after puberty) 2) mesenteric desmoid tumors 3) epidermoid inclusion cysts (sebaceous cysts) […]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"_genesis_hide_title":false,"_genesis_hide_breadcrumbs":false,"_genesis_hide_singular_image":false,"_genesis_hide_footer_widgets":false,"_genesis_custom_body_class":"","_genesis_custom_post_class":"","_genesis_layout":"","footnotes":""},"categories":[5126,63],"tags":[1347,1345,1344,1346,1213],"yoast_head":"\nGardner's Syndrome - 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