Hereditary polyposis syndromes remain a challenging disease entity: Old dilemmas and new insights.

In this editorial we present an overview and insights of the management of hereditary polyposis syndromes. The primary focus was on familial adenomatous polyposis, juvenile polyposis syndrome and Peutz-Jegher syndrome. Genetic testing has become increasingly available and is easier than ever to integrate into clinical practice. Furthermore, several genes have been added to the expanding list of genes associated with hereditary polyposis syndromes, allowing for precise diagnostics and tailored follow-up. Endoscopic evaluation of patients with hereditary polyposis syndromes is paramount in the surveillance strategies. Current endoscopic procedures include both diagnostic procedures and surveillance as well as therapeutic interventions. Recommendations for endoscopic procedures in the upper and lower gastrointestinal canal were described. Surgery is still a key component in the management of patients with hereditary polyposis syndromes. The increased cancer risk in these patients often render prophylactic procedures or intended curative procedures in the case of cancer development. Surgical interventions in the upper and lower gastrointestinal canal were described with relevant considerations. Development of chemopreventive medications is ongoing. Few drugs have been investigated, including nonsteroidal anti-inflammatory drugs. It has been demonstrated that cyclooxygenase-2 inhibitors may lower the number of polyps. Other medications are currently under investigation, but none have, to date, consistently been able to prevent development of disease.

Functional outcome and quality of life after transanal minimal invasive pouch surgery.

PURPOSE: To investigate functional outcomes and quality of life (QoL) after restorative proctocolectomy (RPC) using transanal minimal invasive surgery (TAMIS). METHOD: The study consists of two sub-studies. A cohort study comprised 98 consecutive patients, who underwent TAMIS RPC. These patients were the first at our department to undergo TAMIS RPC. We collected information about surgery, complications, postoperative morbidity and mortality ≤ 30 days, and pouch problems. Patients were also invited to participate in a case-control study in which the patients would respond to three different questionnaires, the Inflammatory Bowel Disease Questionnaire (IBDQ), the Short Form-36 General Health Questionnaire (SF-36), and questions from the Pouch Dysfunction Score. We compared the responding TAMIS RPC patients to a Danish national cohort (0-10 years from RPC, n = 514) of patients having RPC between 1980 and 2010. We compared functional outcomes and QoL. RESULTS: Four (4%) of the TAMIS patients had an anastomotic leak; none of these required re-operation with removal of the pouch. Anastomotic leak was treated with antibiotics and drain. Out of the four leaks, only one ended up with a permanent stoma; all others had their stoma reversed successfully. The TAMIS patients had the same number of bowel movements as the patients in the Danish national cohort study. The same was seen with regard to incontinence. We had no conversions in our series of TAMIS procedures. CONCLUSION: The TAMIS technique shows acceptable outcomes, both in regard to postoperative complications and also functional outcome and QoL.

Short-term Outcome of Robot-assisted and Open IPAA: An Observational Single-center Study.

BACKGROUND: The potential advantages of robot-assisted laparoscopy are being increasingly investigated, although data on its efficacy in benign colorectal surgery are scarce. OBJECTIVE: We compared the early postoperative outcome in robot-assisted IPAA with open surgery procedures. DESIGN: This was an observational study based on prospectively collected data obtained from chart reviews. SETTING: The single-center data set covers patients operated on from January 13, 2004, to September 16, 2014, at a specialist center. PATIENTS: Patients with ulcerative colitis undergoing IPAA surgery were included. MAIN OUTCOME MEASURES: Study end points included the duration of operation, admission length, complications (Clavien-Dindo), reoperations, and readmissions. RESULTS: Eighty-one robot-assisted and 170 open IPAA procedures were performed. The duration of operation was significantly longer for robot-assisted laparoscopic procedures (mean difference, 154 minutes; CI, 140-170). During a mean follow-up of 102 days, no significant differences in the distribution of complications were found (Spearman p = 0.12; p = 0.07), and no postoperative deaths occurred in either group. Postoperative admission length was shorter following robot-assisted procedures (mean difference, -1.9; CI, -3.5 to -0.3), whereas 40% of patients were readmitted, compared with 26% of patients who had open surgery (OR, 1.9; CI, 1.1-3.4). Pouch failure occurred in 3 patients (1 following robot-assisted laparoscopy; 2 following open surgery). On multivariate regression analyses, robot-assisted laparoscopy was associated with a significantly longer duration of operation (mean difference, 159 minutes; CI, 144-174), and more readmissions for any cause (OR, 2; CI, 1.1-3.7). LIMITATIONS: This was a nonrandomized, single-center observational study. CONCLUSION: In this implementation phase, robot-assisted IPAA surgery offers acceptable short-term outcomes. The limitations of this observational study call for randomized controlled trials with long-term follow-up and exploration of functional results.