الفهرس 
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Abstract
FAP is characterized by the development of innumerable colorectal adenomatous polyps, often exceeding effective endoscopic management. Patients with FAP may present with extracolonic manifestations depending on the specific gene mutation involved. These manifestations include duodenal adenomas, desmoid tumors (DTs), thyroid cancer, osteomas; lipomas; and congenital hypertrophy of the retinal pigment epithelium (CHRPE). Colorectal cancer is the leading cause of death in undiagnosed FAP patients. This study was conducted at GISC, Mansoura University, aiming to assess the outcome of different surgical management options regarding disease free survival, quality of life, and evaluation of the effectiveness of screening programs. We retrospectively reviewed the data of consecutive 35 patients diagnosed with FAP who underwent surgical intervention during the duration between January 2000 and December 2018. All cases were subjected to complete history taking, thorough physical examination and routine laboratory investigations. Other investigations included colonoscopy, upper endoscopy and pelviabdominal US. CT was requested only in doubtful cases of colorectal masses. Any postoperative complications were noted and recorded. Post-operative gastro-intestinal and sexual function were assessed. The study showed the following results the mean age of the included cases was 36.3 (±12.7) years. The male to female ratio was 1:1.9. The most common symptoms were diarrhea (n=24 , 68.6%) and bleeding per rectum (n=23 , 65.7%). Other presentations included abdominal pain (n=14 , 40%), tenesmus(n=3 , 8.6%), constipation (n=7 , 20%), altered bowel habits (n=2 , 5.7%) and melena (n=1 , 2.9%). Positive family history was present in 19 patients (54.3%). Colorectal masses were diagnosed and biopsied on preoperative colonoscopy in eight patients (22.9%). Twenty-nine patients (82.9%) were operated through the open approach and six cases (17.1%) via laparoscopy. Surgical procedures were; TPC, IPAA and ileostomy (n=26 , 83.9%), TPC with IPAA (n=3 , 8.6%), TC with IRA (n=5 , 14.2%) and TPC with terminal ileostomy (n=1 , 2.9%). The only significant predictor factor for progression to adenocarcinoma on top of FAP was preoperative constipation (P = 0.048). Complications were encountered in five cases (14.3%) in the form of leakage (2 cases , 5.7%), pelvic collection (n=2 , 5.7%) and subacute intestinal obstruction (2.9%). Fourteen patients completed their follow up visits (40%), while five cases died during follow up. Sexual dysfunction was reported by two female patients (14.3%) in the form of dyspareunia. Mortality was encountered in five cases (14.3%). The cause of death was intra-abdominal desmoid tumor infiltrating aorta (2.9%), brain metastasis (2.9%), ovarian tumor (2.9%), chemotherapy side effects (5.7%). Candidates for screening were 350 family members. After family screening for FAP patients, it showed that eight of the family members diagnosed as FAP, while seven was free. Six of the patients whose diagnosis was FAP proceeded for prophylactic TPC and IPAA, while the remaining two members refused the procedure