Prophylactic colectomy and rectal preservation in FAP: systematic endoscopic follow-up and adenoma destruction changes natural history of polyposis

Background and study aims Prophylactic surgery of familial adenomatous polyposis (FAP) includes total colectomy with ileorectal anastomosis (IRA) to proctocolectomy with ileoanal anastomosis (IAA). Surgical guidelines rely on studies without systematic endoscopic follow-up and treatment. Our aim was to report our experience based on a different approach: therapeutic follow-up, comparing in this setting IRA and IAA in terms of oncological safety and quality of life. Patients and methods Between January 1965 and November 2015, all patients who underwent prophylactic surgery for FAP with therapeutic endoscopic follow-up in Lyon University hospital: systematic endoscopic treatment of adenomas, were retrospectively and prospectively (since 2011) included. Results A total of 296 patients were analyzed: 92 had proctocolectomy with IAA (31.1 %), 197 total colectomy with IRA (66.5 %), and seven abdominoperineal resections (2.4 %). Median follow-up was 17.1 years (range, 0–38.1). Incidence of secondary cancer (IR vs. IAA) was 6.1 % vs. 1.1 % (P = 0.06; 95 %CI 0.001–0.36). The 15-year cancer-free and overall survival (IR vs. IAA) were 99.5 % vs 100 % (P = 0.09) and 98.9 % vs. 98.8 % (P = 0.82), respectively. Postoperative morbidity occurred in 44 patients: 29 (14.7 %) in the IRA and 15 (16.3 %) in the IAA group (P = 0.72). The mean number of stools per day in the respective groups were 4.4 (2.5) vs. 5.5 (2.6) (P = 0.001). Fecal incontinence occurred in 14 patients (7.1 %) in the IRA vs. 16 (17.4 %) in the IAA group (P = 0.03). Conclusions A combination of therapeutic endoscopic treatment and extended rectal preservation appears to be a safe alternative to ileoanal J-pouch anastomosis.

A173 RECOGNIZING RARE PRESENTATIONS OF POLYPOSIS SYNDROMES AND THEIR ASSOCIATED MALIGNANCIES IN PEDIATRIC PATIENTS

Background There is a range of polyposis syndromes and presentations in pediatrics. There are also associated extra-colonic malignancies of which to be cognizant when orchestrating the initial work-up of the various polyposis syndromes. Aims To use case review to highlight the importance of recognizing the breadth of presentations of polyposis syndromes in pediatrics. Methods Two recent pediatric presentations of polyposis with extra-intestinal manifestations were identified, chart review completed, and compared with newly published ESPGHAN guidelines. Results Two patients with intestinal polyposis are presented, in which extra-colonic malignancies and genetic mutations were identified. The first patient presented at age 16 with a history of fatigue and abdominal pain, and was found to have pancytopenia and splenomegaly. Initial work up included a bone marrow biopsy that was normal. The patient then underwent upper and lower endoscopic evaluation for increasing abdominal pain and persistent anemia and was found to have polymorphic polyps in the duodenum, sigmoid and rectum. On pathology, polyps were mostly inflammatory, but one was found to be hamartomatous. Additional screening revealed a thyroid nodule, found to be follicular carcinoma, requiring hemithyroidectomy. The patient was confirmed to have a PTEN mutation and was diagnosed with Cowden syndrome; following this diagnosis proceeded with a prophylactic bilateral mastectomy. The second patient was referred at age 15 with a strong family history of APC-associated FAP. At the time of consultation she was asymptomatic and she remained so throughout her work-up. Screening endoscopy revealed 70–90 recto-sigmoid adenomatous polyps as well as scattered gastric and duodenal polyps. Her initial work-up also uncovered an early papillary thyroid carcinoma. Her treatment included a total thyroidectomy and total proctocolectomy with J-pouch and ileoanal anastomosis. Follow-up endoscopy continues for surveillance of numerous gastric adenomas which to this point have not progressed to high-grade dysplasia or malignancy. Conclusions These 2 cases highlight the importance of recognizing that neoplastic conditions typically diagnosed in adulthood can also present in the pediatric age group. Ideally, further guidelines in pediatrics would be beneficial to ensure a consistent approach to investigating polyposis and associated malignancies. Pertaining to our specific patients, each had identification of a thyroid malignancy before the recommended screening age of 18 as per the currently accepted guideline, and neither were symptomatic. More cases are needed to establish if this earlier recognition of disease is meaningful in postulating potential mortality associated with a later diagnosis. Funding Agencies None

P126 EPIDEMIOLOGY AND RISK FACTORS OF BREAST CANCER IN INFLAMMATORY BOWEL DISEASE IN THE UNITED STATES BETWEEN 2014 AND 2019: A POPULATION-BASED STUDY

Background The risk of extra-colonic cancer in IBD is unclear. While thiopurines and tumour necrosis factor-α antagonists (anti-TNFs) are associated with increased risk of skin cancer and lymphoma in IBD, there is scant data on breast cancer in IBD. We evaluated the prevalence of breast cancer in IBD and investigated the role of biologics, immunomodulators, and clinical characteristics of IBD in patients with IBD and breast cancer. Methods We queried a commercial database (Explorys Inc), an aggregate of Electronic Health Record data from 26 major integrated healthcare systems in the US from 1999 to 2019. We identified a cohort of eligible patients with a diagnosis of “Crohn’s disease” (CD) and “Ulcerative Colitis” (UC) between June 2014 and 2019, based on Systemized Nomenclature Of Medicine – Clinical Terms. We calculated the prevalence of “primary malignant neoplasm of breast” in IBD overall, and among different sub-groups and identified risk factors for breast cancer in IBD utilizing linear regression. Results Of the 35,521,930 individuals in the database from October 2014–2019, we identified 165,750 and 140,640 individuals with a diagnosis of CD and UC with an overall prevalence rate of 0.47% and 0.4% respectively. Out of these, 3,160 individuals with CD and 3,340 individuals with UC had a co-diagnosis of breast cancer. The prevalence of breast cancer in individuals without IBD was 1.1%. Compared to individuals with breast cancer without IBD, the prevalence of breast cancer in CD was increased at 1.9% [OR: 1.79, 95% CI: 1.73–1.85, p<0.0001] and in UC was 2.3% [OR: 2.24, 95% CI: 2.17–2.32, p<0.0001]. Compared to individuals with IBD and no malignancy, individuals with IBD and breast cancer were predominantly females, elderly (>65yo), Caucasians, had history of tobacco use and appendectomy (Table 1). Anti-TNFs and vedolizumab were associated with increased risk of breast cancer in CD (ORs 1.82 and 1.38) but not in UC. Azathioprine was associated with increased risk of breast cancer in both CD and UC (ORs 1.9 and 1.29). For UC, history of colectomy was associated with an increased risk of breast cancer with OR 1.51. For CD, history of total abdominal colectomy (TAC) with ileostomy or with ileoanal anastomosis, partial resection of the colon and small intestinal surgery were all associated increased risk of breast cancer. However, fistulizing disease, peri-anal disease, and incision and drainage of perineal or perirectal abscess were not associated with increased risk of breast cancer (Table 1). Conclusion We found a two-fold higher prevalence of breast cancer in patients with IBD compared to individuals without IBD. We identified tobacco use, appendectomy and surrogate markers of luminal inflammation (use of immunomodulators, biologic agents and history of intestinal surgery) to increase risk of breast cancer. Further prospective studies are needed to confirm these findings which have implications on agressive screening of breast cancer in females with IBD.

Safety of coloanal/ileoanal anastomosis during cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for peritoneal carcinomatosis: results of 20 consecutive patients

Objective No studies to date have focused on the safety of coloanal/ileoanal anastomosis (CAIAA) in cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS + HIPEC), which is associated with severe morbidity and mortality. We herein present the outcomes of patients with peritoneal carcinomatosis (PC) who underwent CAIAA. Methods We evaluated the prospectively collected data from 20 patients with PC who underwent CRS + HIPEC with respect to the primary disease, synchronous resections, intraoperative chemotherapy regimen, timing of protective ileostomy closure, and overall postoperative complications. Results Most patients underwent CRS + HIPEC and CAIAA for PC due to colorectal cancer. Coloanal anastomosis was performed in 15 (75%) patients, and J-pouch ileoanal anastomosis was performed in 5 (25%) patients. No anastomosis-related complications occurred in any patients who underwent CAIAA; however, one patient died of pulmonary embolism on postoperative day 7. Conclusions CAIAA is associated with serious complications even after performing benign colorectal surgery. However, it may be challenging for surgeons to simultaneously perform CAIAA in patients with PC who undergo CRS + HIPEC. We emphasize that this procedure can be safely performed with experienced surgical teams by using a multidisciplinary approach.

WOUND INFECTIONS

Background: An intestinal stoma is a surgically created opening of the bowelonto the body surface. Temporary ostomies can be created from small or large bowel in a varietyof manners and serves a valuable role in persons undergoing surgery for acute infectious events,malignancy or trauma. Temporary faecal diversion is recommended with a low colorectal,coloanal or ileoanal anastomosis. Objective: To find the frequency of wound infection followingintestinal stoma closure. Materials and methods: The case series study was conducted insurgical unit Hayatabad medical complex Peshawar over 139 patients between March 2008 toAugust 2009. Closure was done on the next day of admission. Patients were advised to report toOPD if they develop wound infection in between follow up visits. Data were collected using aspecially designed proforma. Results: Out of 139 patients, 104(74.82%) were male and35(25.18%) were female. Colostomy was done in 78(56.1%) patients and ileostomy in 61(43.9%)of patients. Average age was 35.69 years ±16.5SD. There are 11(7.9%) wound infectionobserved during the hospital while at 30th day of post-op follow up decreased to just 3(2.2%).Average hospital stay was 4.96 days ± 2.06SD with a range of 3-10 days. Conclusions: Woundinfection was observed as compared to other national and international studies. Male were threetimes more than females and wound infection was seen more in males.

Pitfalls in Diagnosis of Early-Onset Inflammatory Bowel Disease

Aim The onset of inflammatory bowel disease (IBD) in patients younger than the age of 5 years is rare. Moreover, when there is exclusive colonic inflammation, diagnosis can be challenging. Our aim was to describe the difficulties and pitfalls in the diagnosis of early-onset IBD (eoIBD) and their repercussions in treatment decisions. Patients and Methods Patients with eoIBD (Crohn's disease [CD], ulcerative, and unclassified colitis) treated at our center between 1990 and 2016 were studied retrospectively. Demographic, clinical, medical, and surgical treatment data were analyzed. Results A total of 19 patients were diagnosed with eoIBD at 21 (1–46) months of age. Bloody diarrhea, growth failure, and abdominal pain were present in 90, 42, and 16%, respectively. After 9 (1–62) months from the onset, patients were classified as ulcerative colitis (nine), CD (two), and unclassified colitis (eight). Unresponsiveness to medical treatment was observed in 10 and prompted surgical assessment. A partial colectomy was performed in one, and nine underwent a total colectomy (one end stoma and eight ileoanal anastomosis [IAA]). At least one surgical complication occurred in 80% and ultimately six patients with an IAA required an end stoma. Overtime, final diagnosis of 5 out of the 10 surgical patients changed due to biopsy findings, unresponsiveness to medical treatment, or extraintestinal and perianal manifestations. After a 12-year (1–22) follow-up, 57.9% of the diagnoses of all patients were modified. Conclusion EoIBD poses a challenge due to ambiguous presentation and absence of specific diagnostic tests. Surgical evaluation is often needed and surgeons must be mindful of possible initial misdiagnosis, in addition to short- and long-term outcomes before deciding aggressive surgical measures as well as intestinal reconstruction.