Surgery For Familial Adenomatous Polyposis Research Articles

Results of surgical treatment for familial adenomatous polyposis

Aim: to analyze the results of surgery for familial adenomatous polyposis (FAP). Patients and methods: the case series study included 20 patients with FAP, 85 % of procedures with anastomosis and 15 % with a permanent ileostomy. Laparoscopic approach was used in 35 %. Results: the mean time of operation time was 243 minutes, the mean intraoperative blood loss was 244 ml, and the mean hospital stay was 17.2. Three (15.0 %) patients developed postoperative complications. Laparoscopic pro­cedures were advantageous in terms of intraoperative blood loss and faster recovery. The first polyps were detected in the rectal stump within 6-8 months after surgery, desmoid tumors within 24.3 months. Most patients had an acceptable quality of life with an mean number of stools per day 11.1. Conclusions: FAP is a complex problem of modern medicine requiring the teamwork of various medical specialists. Minimally invasive interventions for FAP have advantages over open procedures.

Therapeutic approaches for patients with coexisting familial adenomatous polyposis and colorectal cancer.

Colorectal cancer is a major cause of death in patients with familial adenomatous polyposis. Despite evidence for prophylactic colectomy, there is no ideal therapy for patients with coexisting familial adenomatous polyposis and colorectal cancer. We evaluated the correlation between surgery for familial adenomatous polyposis and multimodal treatment for colorectal cancer, and clarified prognosis of Japanese patients with familial adenomatous polyposis and colorectal cancer. We retrospectively reviewed data from 303 patients who underwent colorectal surgery for familial adenomatous polyposis between 2000 and 2012. Overall, 172 patients had colorectal cancer. The most common procedure for familial adenomatous polyposis was restorative proctocolectomy with ileal pouch anal anastomosis, irrespective of colorectal cancer. Partial colectomy was more frequent in patients with than without colorectal cancer (8.7% and 0%, respectively). Ileal pouch anal anastomosis was frequently (60.6%) performed in patients with Stage I-III colorectal cancer. Overall, 12 of 20 patients with Stage IV colorectal cancer underwent metastasectomy; six patients simultaneously and six metachronously. There were fewer cases of ileal pouch anal anastomosis, but more total colectomy with ileorectal anastomosis was performed metachronously, compared with simultaneous metastasectomy (P = 0.006). More cytotoxic (P = 0.006) and molecular (P = 0.03) agents were administered to the ileorectal anastomosis/partial colectomy patients, compared with total proctocolectomy/ileal pouch anal anastomosis patients. A 5-year overall survival was 100% in Stage 0/I, 89.8% in Stage II, 87.9% in Stage III and 48.4% in Stage IV. In patients with familial adenomatous polyposis and colorectal cancer, primary surgery, metastasectomy and chemotherapy could be compatible with standard surgical approaches for familial adenomatous polyposis . However, modifying surgical procedures for familial adenomatous polyposis might help multimodality therapy for Stage IV colorectal cancer to prolong survival.

Childbirth after surgery for familial adenomatous polyposis in Japan.

Familial adenomatous polyposis (FAP) is a genetic disorder. Some female patients with FAP can become pregnant. However, the current state of childbirth after surgery for FAP is unclear in Japan. The study investigated 303 patients (147 female) who had undergone surgery for FAP at the 23 institutions between 2000 and 2012. Eighty female patients had information available on childbirth after surgery for FAP. Eight patients (10%) gave birth after surgery. The mean age at surgery for FAP was 27 (range 20-41) years and 37years in patients with and without childbirth after surgery, respectively (P=0.044). The rate of childbirth after surgery was 17% in women ≤30years of age and 13% in those ≤40years of age. Although only one patient with invasive cancer (2.9%) gave childbirth after surgery, seven patients without cancer (15.6%) gave birth (P=0.045). This study clarified the current state of childbirth after surgery for FAP in Japan. It is important to use these data to determine the best therapeutic approach for female FAP patients.

The Evolution of Prophylactic Colorectal Surgery for Familial Adenomatous Polyposis

Over the past 50 years, prophylactic colorectal surgery for patients with familial adenomatous polyposis has evolved as new technologies and ideas have emerged. The aim of this study was to review all the index surgeries for familial adenomatous polyposis performed at our institution to assess the changes in surgical techniques. All index abdominal surgeries for polyposis from 1950 to 2007 were identified through the Polyposis Registry Database. We assigned the patients to prepouch (before 1983), pouch (after 1983), and laparoscopic (after 1991) eras, and analyzed the changes in prophylactic surgery. Four hundred twenty-four patients were included; 51% were male. Median age at surgery was 26 (range, 9-66) years. In the prepouch era, 97% (66 of 68) of all surgeries and 100% of restorative surgeries were ileorectal anastomosis. After 1983, 70% (54 of 77) of patients with a severe phenotype had an ileal pouch-anal anastomosis. After 1991, 110 operations (43%) were laparoscopic (88 ileorectal and 22 ileal pouch-anal anastomosis). Colon surgery for familial adenomatous polyposis has evolved as advances in surgical technique have created more options to reduce the risk of cancer. Current strategy uses polyposis severity and distribution to decide on the surgical option, and laparoscopy to minimize morbidity.

Is Internet Information Adequate to Facilitate Surgical Decision-Making in Familial Adenomatous Polyposis?

Prophylactic surgery decisions are difficult. Supplemental information improves patients' knowledge, promoting active participation in decision-making. Our objective was to examine internet information regarding prophylactic surgery for familial adenomatous polyposis to determine its adequacy in facilitating patient participation in surgical decision-making. We searched the internet for information on surgery for familial adenomatous polyposis, using an intentionally simple strategy to represent patients' searches. We examined the first 50 sites from each search, assessing each for content by using predefined criteria. Every site was evaluated by two investigators (kappa 0.71) by using the DISCERN criteria, a tool for evaluating quality of health information. Search-efficiency was calculated. Searches revealed 307,138 "hits"; 20 sites met inclusion criteria. GOOGLE demonstrated the highest search-efficiency (28 percent). Sites were maintained by general health pages (35 percent), hospitals (30 percent), professional organizations (15 percent), familial adenomatous polyposis registries (10 percent), and government (10 percent). Only 40 percent had been developed and/or updated within two years. Most included basic information regarding risk, symptoms, diagnosis, as well as discussion of familial adenomatous polyposis-associated diseases and surveillance (80-100 percent). Although 90 percent of sites presented surgical treatment options, only 60 percent provided details. Few provided information regarding postoperative bowel function (40 percent), sexual function (20 percent), or fertility (5 percent). Seven (35 percent) were identified as being of "good/excellent" quality. Only four were patient-oriented; variable information was provided. Internet information regarding surgical treatment of familial adenomatous polyposis is sparse, and insufficient to support active patient participation in decision-making. Despite the time and financial commitment required, development of high-quality internet resources may be justified given the lack of adequate patient-oriented information currently available.

Significance of incidental desmoids identified during surgery for familial adenomatous polyposis.

The behavior of intra-abdominal desmoids in familial adenomatous polyposis is incompletely understood. Findings range from typical mass lesions to flat sheets, termed the desmoid reaction or desmoid precursor lesion. The latter often are incidental findings of uncertain significance. The study was designed to describe the natural history of incidental intra-abdominal desmoid tumors with particular reference to the desmoid reaction. Patients who underwent laparotomy for familial adenomatous polyposis at the Cleveland Clinic Foundation were identified. The incidence of incidental intra-abdominal desmoid tumors was determined by review of operative records. Intra-abdominal desmoid tumors were classified as mass lesions if three-dimensional or desmoid reaction if two-dimensional. The incidence of clinically apparent intra-abdominal desmoid tumors (typical mass lesions on physical examination or cross-sectional imaging in symptomatic patients) was determined by chart review. The incidence of clinical intra-abdominal desmoid tumors between groups was compared by Fisher's exact test. A total of 266 patients (153 females; median age, 26 (range, 9-63) years) underwent abdominal surgery for familial adenomatous polyposis. Incidental intra-abdominal desmoid tumors were identified in 34 patients: 8 at the index surgery and 26 at relaparotomy. These lesions influenced the planned procedure in eight cases (26 percent), including preventing ileoanal pouch in 3 of 19 patients in whom this was intended. The median follow-up from the time of identification of intra-abdominal desmoids was 42 (range, 2-178) months at which point four patients (11 percent) had developed clinical intra-abdominal desmoid tumors. There was no significant difference in incidence of clinical intra-abdominal desmoid tumors between mass and desmoid reaction groups ( P = 0.27). Incidental intra-abdominal desmoid tumors are a common finding at relaparotomy in patients with familial adenomatous polyposis. These lesions influence planned surgery in a minority of cases. Desmoid reaction may have little bearing on the subsequent development of clinically significant intra-abdominal desmoid tumors.