Proctocolectomy Research Articles

Surgical Interventions, Malignancies, and Causes of Death in a FAP Patient Registry

BackgroundFamilial adenomatous polyposis (FAP) patients are at risk for numerous malignancies. Multiple surgeries exist to mitigate the risk of colorectal cancer. Surgeons must weigh future quality of life versus the risk of dysplasia. As FAP patient longevity increases, there remains a risk of other malignancies. This study examines surgical interventions, development of cancers, and causes of mortality in a FAP registry. MethodsPatients with FAP or attenuated FAP (aFAP) were identified by linking the Hereditary Gastrointestinal Cancer Registry with University of Utah’s medical records. Patients without sufficient information were excluded. Patient demographics, surgical histories, cancer diagnoses, and causes of death were extracted. Logrank and Fisher’s exact tests were employed to detect significant differences between groups. ResultsAfter exclusion criteria, 140 patients were analyzed. Sixty patients (42.9%) underwent total proctocolectomy with ileal pouch-anal anastomosis (IPAA) followed by 50 (35.7%) having total colectomy with ileorectal anastomosis (IRA). IPAA patients were more likely female (p = 0.01) and have FAP (p < 0.01) versus IRA patients. Nineteen patients (15.0%) required additional colorectal surgeries; however, no differences were based on initial surgery. Colorectal cancer was diagnosed in 22 patients (15.7%), while 7 (5.0%) developed gastric cancer. Of the 15 deceased patients, 6 (40%) died due to gastric adenocarcinoma. DiscussionThis study suggests that aFAP and FAP patients are undergoing appropriate colorectal interventions to reduce colorectal cancer mortality; however, repeat interventions are frequent. Gastric malignancy is common and represents the leading cause of death. Further studies are needed to determine appropriate surveillance protocols to reduce this risk of malignancy.

P002 Endoscopic Characteristics and Prognosis of Pouchitis Involving the Pouch Inlet in Patients With Inflammatory Bowel Disease

BACKGROUND: In patients with inflammatory bowel disease (IBD), surgical intervention is sometimes required due to medically refractory colitis or the development of neoplasia, and restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the most common procedure for those with colitis. However, pouchitis can develop in up to 80% of patients after the surgery and significantly impairs quality of life. Despite the high prevalence of pouchitis, endoscopic phenotypes have not been clarified. This study assessed the frequency of and risk factors for inflammation involving the pouch inlet. METHODS: This is a retrospective single-center study of IBD patients treated by total proctocolectomy with IPAA and who subsequently underwent pouchoscopies at the University of Chicago between January 2007 and September 2019. We reviewed the endoscopic findings in different anatomic areas of the pouch: the pre-pouch ileum, inlet, “tip of the J”, proximal and distal pouch, anastomosis, rectal cuff, anal canal, and perianal area. This analysis evaluated all available pouchoscopies and included any patient who had inlet inflammation noted at least once. Demographic and clinical data were also assessed. Log-rank test was used for a univariate analysis to assess factors contributing to pouch excision. Cox proportional hazard regression analysis was performed as a multivariate analysis including univariate variables with a p-value < 0.10. RESULTS: We reviewed 1,081 pouchoscopies from 426 IBD patients who underwent proctocolectomy with IPAA and identified 188 patients (44%) with pouchitis involving the inlet. Of these patients, 62% were men, 89% were Caucasian, mean age at diagnosis (SD) was 24 ± 11 years, and mean BMI 26 ± 5. The included patients had pre-pouch diagnoses of ulcerative colitis (165; 89%), indeterminate colitis (14; 7.5%), and Crohn’s disease (7; 3.8%) as the diagnosis before surgery. Twenty-five percent (47/188) had inlet stenosis and 33% (62/188) had pouch-anal anastomotic stenosis. Compared with the cuff, the rate of ulcerations was higher in the pre-pouch ileum and inlet (27.1% vs 42% and 66%, respectively). After a mean of 12 years of follow-up, the overall prevalence of pouch excision was 14% (25/188). Multivariate analysis identified that inlet stenosis significantly predicted pouch excision (HR = 2.92; 95% CI = 1.24-6.86; p = 0.014), whereas anastomotic stenosis was not a significant predictor (HR = 1.48; 0.64-3.42; p = 0.36). CONCLUSION(S): Inlet inflammation and stenosis is present in a significant number of IBD patients with pouchitis, and stenosis in particular is associated with pouch excision. There is a pressing need to understand the causes and develop treatments of this complication.

Distribution of Elective Ileal Pouch-Anal Anastomosis Cases for Ulcerative Colitis: a Study Utilizing the University Health System Consortium Database

BackgroundTrends and distribution of ileal pouch-anal anastomosis (IPAA) procedures for patients with ulcerative colitis (UC) are unknown. We examined the frequency, distribution, and volume-outcome relationship for this relatively infrequent procedure using a large national data source. MethodsData were obtained from the University HealthSystem Consortium (UHC) for patients with a primary diagnosis of UC admitted electively and who underwent surgical intervention between 2012 and 2015. ResultsThe mean age of the study population (n = 6875) was 43 years and 57% were men. Among these, one-third (n = 2307) underwent an IPAA, while 24% (n = 1160) underwent total abdominal colectomy, 16% (n = 1134) underwent proctectomy, and 2% (n = 108) underwent total proctocolectomy with end ileostomy. The frequency of IPAA cases among all elective surgical cases was relatively stable at 33–35% over the study period. A total of 131 hospitals, out of 279 hospitals participating in the UHC (47%), performed IPAA. UHC contains all inpatient data on more than 140 (> 90%) academic medical centers in the US and their affiliates. Most hospitals (101) performed < 5 cases annually. The median number of IPAA cases performed annually was 1.8 [IQR 0.8 – 4.3]. The top 10 hospitals performed one-half (48%) of IPAA cases, but only 18% of another type of complex pelvic dissection cases such as low anterior resection. Short-term postoperative complications after IPAA, however, were similar regardless of IPAA volume. ConclusionsNearly one-half of IPAA cases were performed at only 10 hospitals out of the 131 hospitals performing IPAA in the study. IPAA procedures are infrequently performed by most academic medical centers in the US. The redistribution of IPAA procedures, likely a result of previously established referral patterns and centralization, has a potential impact on the training of future colorectal fellows as well as access to care.

Incidence and risk factors of portomesenteric venous thrombosis after colorectal surgery for cancer in the elderly population

BackgroundAlthough it is known that portomesenteric venous thrombosis (PMVT) is associated with total colectomy and proctocolectomy in young patients with inflammatory bowel disease, little is known about incidence and risk factors of PMVT among the elderly population undergoing colorectal surgery for cancer.MethodsData of elderly patients (> 70 years) undergoing surgery for colorectal cancer were retrospectively registered. The occurrence of PMVT was correlated with the patients’ characteristics and operative variables. Data collected included age, sex, obesity, ASA score, tumor degree, type of surgical resection, surgical approach (laparoscopic or open), and duration of surgery (from skin incision to the application of dressings).ResultsA total of 137 patients > 70 years who underwent surgery for colorectal cancer and developed an acute intraabdominal process with suggestive symptoms, needing a CT scan, were included. Three of these patients (2.1%) had portomesenteric venous thrombosis during the study period, which was proved with CT scan. There were no significant patients’ characteristics or operative variables between patients with or without the occurrence of PMVT after surgery. Of interest, only operative time was significantly higher in patients with PMVT after surgery (256 ± 40 vs 140 ± 41, p < 0.001).ConclusionsPMVT as a cause of abdominal pain after colorectal surgery for cancer in the elderly population is uncommon. An index of suspicion for PMVT in an elderly postoperative colorectal cancer patient with sudden onset of abdominal pain must be maintained.

102P - Clinical features of anorectal cancer in patients with Crohn’s disease: Japanese single center study

BackgroundPatients with long-standing Crohn’s disease (CD) are at a high risk of development of anorectal cancer (ARC), however, the clinical features of ARC complicating CD are still uncertain. The aim of the present study was to clarify the clinical features of ARC complicating CD. MethodsFrom 1995 to 2018, 483 patients with CD underwent surgery in our hospital. Of the 483 patients, 14 patients (2.9%) were diagnosed with ARC. A detailed review of the medical records of the patients was undertaken. ResultsThe median age at diagnosis of ARC was 45 years (25-72 yrs). The median time interval between the diagnosis of CD and ARC was 21 years (7-40 yrs). Eight patients had fecal diversion because of their perianal disease. Ten patients had cancer-related symptoms. The other 4 patients had no cancer-related symptom, but serum CEA was elevated in the 2 of the 4 patients. The 4 patients were diagnosed by cancer surveillance biopsy and 3 of the 4 patients (75%) underwent R0 resection. On the other hand, only 2 of the 10 patients (20%) underwent R0 resection in patients who had cancer-related symptom at the diagnosis of ARC. The surgical procedures were as follows: abdominoperineal resection (n = 6), total pelvic exenteration (n = 3), total proctocolectomy (n = 1), loop ileostomy (n = 3), and exploratory laparotomy (n = 1). UICC staging was as follows: stage I;2. stageII;3, stageIII;4, stageIV;5. After the surgery, chemoradiotherapy was performed in 4 patients, chemotherapy in 4, and radiotherapy in 1. All the patients who underwent R0 resection achieved 5-year relapse free survival, while the 1-year / 2-year overall survival rate of the patients’ group who underwent R1 and R2 resection was 75%/50% (R1) and 40%/0% (R2), respectively. ConclusionsARC was not rare in the patients with long-standing CD. The prognosis is poor in patients’ group with R1/R2 resection, while, patients’ group who achieved R0 resection is good. There is a pressing need to develop strategies for the early detection of ARC. Legal entity responsible for the studyThe authors. FundingHas not received any funding. DisclosureAll authors have declared no conflicts of interest.

Recommendations of the Spanish Working Group on Crohn’s Disease and Ulcerative Colitis (GETECCU) on pouchitis in ulcerative colitis. Part 1: Epidemiology, diagnosis and prognosis

Pouchitis is a common complication in ulcerative colitis patients after total proctocolectomy. This is an unspecific inflammation of the ileo-anal pouch, the aetiology of which is not fully known. This inflammation induces the onset of symptoms such as urgency, diarrhoea, rectal bleeding and abdominal pain. Many patients suffering from pouchitis have a lower quality of life. In addition to symptoms, an endoscopy with biopsies is mandatory in order to establish a definite diagnosis. The recommended index to assess its activity is the Pouchitis Disease Activity Index (PDAI), but its modified version (PDAIm) can be used in clinical practice. In accordance with the duration of symptoms, pouchitis can be classified as acute ( 4 weeks), and, regarding its course, pouchitis can be infrequent ( 4 episodes per year) or continuous.

Long-Term Outcomes after Endoscopic Submucosal Dissection for Ulcerative Colitis-Associated Dysplasia

Introduction: The Surveillance for Colorectal Endoscopic Neoplasia Detection and Management in Inflammatory Bowel Disease Patients: International Consensus Recommendations guidelines recommend surveillance colonoscopy instead of colectomy after the complete removal of “endoscopically resectable” dysplastic lesions in ulcerative colitis (UC). There are no studies on long-term outcomes of endoscopic submucosal dissection (ESD) for UC-associated neoplasia (UCAN). We aimed to evaluate the clinical outcomes of ESD for UC-associated dysplasia (UCAD) during long-term follow-up. Methods: We retrospectively enrolled 17 consecutive UC patients with 22 UCADs, who underwent initial ESD or total proctocolectomy at the Hiroshima University Hospital. The clinicopathological features of the patients and neoplasias and clinical outcomes of ESD were evaluated and compared with those of total proctocolectomy. Results: UCAD in the ESD and total proctocolectomy groups was mostly noted on the left side of the colon, and most lesions were superficial macroscopic lesions. In the ESD group, en bloc resection and histological complete resection rates were 83 and 67%, respectively. One patient died of malignant melanoma; however, none of the patients died of UC-associated carcinoma in both groups. Metachronous neoplasias developed in 5 of the 7 patients in the ESD group. Among the 5 patients with metachronous UCAN, 4 finally underwent total proctocolectomy and 1 underwent additional ESD. Conclusions: ESD for UCAD is a useful method for total excisional biopsy. UC patients with UCAD resected by ESD have a high risk of developing metachronous UCAN during the follow-up period.

Predominantly Antibiotic-resistant Intestinal Microbiome Persists in Patients With Pouchitis Who Respond to Antibiotic Therapy

Pouchitis that develops in patients with ulcerative colitis after total proctocolectomy and ileal pouch anal anastomosis is usually treated with antibiotics. Some patients have recurrence of flares, or become antibiotic-dependent, and require repeated courses or prolonged periods of antibiotic therapy. We investigated microbial factors associated with response to antibiotic treatment and development of antibiotic dependence in patients with pouchitis. We performed a prospective study of 49 patients who had undergone pouch surgery at a tertiary center. Disease activity was determined based on clinical, endoscopic, and histologic criteria. Pouch phenotype was defined as recurrent-acute pouchitis (n= 6), chronic pouchitis and Crohn's-like disease of the pouch (n= 27), normal pouch from patient with ulcerative colitis (n= 10), and normal pouch from patient with familial adenomatous polyposis (n= 6). Fecal samples (n= 234) were collected over time during or in the absence of antibiotic treatment (ciprofloxacin and/or metronidazole). Thirty-three patients were treated with antibiotics, for a median of 425 days of cumulative antibiotic therapy, during follow-up. Calprotectin was measured and fecal DNA was sequenced using shotgun metagenomics and analyzed with specifically designed bioinformatic pipelines. Bacterial strains were isolated from fecal samples. We assessed their ciprofloxacin resistance and ability to induce secretion of inflammatory cytokines by HT-29 intestinal epithelial cells. Most antibiotic-treated patients (79%) had a clinical response to each course of antibiotics; however, 89% of those who completed a 4-week course relapsed within 3 months. Median calprotectin levels decreased by 40% in response to antibiotics. Antibiotic treatment reduced disease-associated bacteria such as Clostridium perfringens, Ruminococcus gnavus, and Klebsiella pneumoniae, but also beneficial species, such as Faecalibacterium prausnitzii. The microbiomes of antibiotic-responsive patients were dominated by facultative anaerobic genera (Escherichia, Enterococcus, and Streptococcus), with multiple ciprofloxacin-resistance mutations in drug target genes and confirmed drug resistance. However, these strains had lower potential for virulence and did not induce secretion of inflammatory cytokines by epithelial cells. After antibiotic cessation, patients had an abrupt shift in microbiome composition, with blooms of oral and disease-associated bacteria. In addition, antibiotic treatment enriched for strains that acquired multidrug resistance loci, encoding enzymes that confer resistance to nonrelated antibiotics, including extended-spectrum beta-lactamases. The efficacy of antibiotic treatment of pouchitis might be attributed to the establishment of an antibiotic-resistant microbiome with low inflammatory potential. This microbiome might provide resistance against colonization by bacteria that promote inflammation. To avoid progression to antibiotic-dependent disease and its consequences, strategies such as short-term alternating antibiotics and nutrition- and microbiome-based interventions should be considered.

Endoscopic activity in asymptomatic patients with an ileal pouch is associated with an increased risk of pouchitis.

The significance of endoscopic activity in asymptomatic ulcerative colitis (UC) patients with an ileal pouch is unknown. To investigate the association of endoscopic pouch activity in asymptomatic patients with the subsequent development of pouchitis. We analyzed a retrospective cohort of patients with UC or IBD-unspecified who underwent a total proctocolectomy with ileal pouch anal anastomosis (IPAA). Asymptomatic patients with a Pouchitis Disease Activity Index (PDAI) symptom sub-score of zero who underwent an index surveillance pouchoscopy were included. Endoscopic pouch body activity was graded as 0: normal, 1: mucosal inflammation, or 2: mucosal breaks (ulcers and/or erosions). The primary outcome was primary acute idiopathic pouchitis defined as PDAI score≥7 with symptoms lasting less than four weeks and responsive to standard antibiotics, not otherwise meeting criteria for secondary pouchitis. The secondary outcome was chronic idiopathic pouchitis defined as PDAI score≥7 with symptoms lasting greater than four weeks despite standard antibiotics. Predictors of pouchitis were analyzed using Kaplan-Meier and Cox regression methods with hazard ratios (HR) and 95% confidence intervals (CI) reported. 143 asymptomatic pouch patients were included. Index endoscopic pouch body activity was 0 in 86 (60.1%) patients, 1 in 26 (18.2%) and 2 in 31 (21.7%). The median length of follow-up after index surveillance pouchoscopy was 3.03 [IQR 1.24-4.60] years. Primary acute idiopathic pouchitis occurred in 44 (31%) patients and chronic idiopathic pouchitis in 12 (8.4%). Grade 2 endoscopic pouch activity was associated with the development of acute pouchitis (HR 2.39, 95% CI 1.23-4.67), although not chronic pouchitis (HR 1.76, 95% CI 0.53-5.87). Histologic inflammation in endoscopically normal pouch mucosa was not associated with acute or chronic pouchitis. Mucosal breaks are present in nearly a quarter of asymptomatic patients with IPAA and are associated with an increased risk of acute pouchitis.

1878 Migration of Over-the-Scope Clip (OTSC) Resulting in Intestinal Obstruction

INTRODUCTION: Iatrogenic perforations are a known and feared complication of diagnostic and therapeutic colonoscopy. Over-the-scope clips (OTSCs) have recently been utilized for management of these perforations. We present the case of a 40-year-old male patient treated with over-the-scope (Ovesco®) clip for an iatrogenic post-polypectomy perforation with subsequent intestinal obstruction occurring as a result of migration of the clip. CASE DESCRIPTION/METHODS: A 40-year-old male with familial adenomatous polyposis and total proctocolectomy, ileoanal anastomosis and a J pouch had an ileoscopy performed for polyp surveillance. A 3 cm flat polyp was removed with Eleview® injection lift technique. A hole like defect was noted in the postpolypectomy site that developed into an obvious small 2-3 mm perforation. To repair the defect, a one 14/6 mm type gc over-the-scope clip (Ovesco®) and a 12/6 mm type gc over-the-scope clip (Ovesco®) was successfully placed. Four months later the patient presented to the emergency department with severe anal pain. He felt a hard metallic object in the anal canal. He denied any fevers, chills, nausea, vomiting, or abdominal pain. Rectal tenderness was found on digital rectal examination. A CT abdomen and pelvis showed an object of metallic density at the anal verge with nonobstructive bowel gas pattern. Colonoscopy revealed two over-the-scope clips approximately 2 cm from the anal verge embedded in the mucosa (Figure 1) which were removed. The patient did well post procedure and was discharged home the next day. DISCUSSION: The Ovesco® clip (Ovesco, Tübingen, Germany) is a novel endoscopic method to achieve mechanical compression of gastrointestinal tissue with current indications including closure of fistulas, iatrogenic perforations, hemostasis and non-variceal gastrointestinal bleeding. Iatrogenic perforations during colonoscopy have an incidence ranging from 0.1% to 0.3% of cases. These complications carry a high mortality rate and in the past warranted surgical intervention. However, with the advances in therapeutic endoscopy many perforations can be managed effectively with endoclips, OTSCs and/or endosuturing. These new techniques do come with device-specific risks. Fischer et al. reported the first case of OTSC-induced bowel obstruction. Here we present another case of bowel obstruction as a result of OTSC placement for a perforation, treated with surgical intervention. All gastroenterologists should be aware of clinical scenarios in which the Ovesco clip can be used.

657 Ulceration of the Pre-Pouch Ileum in Patients With Ulcerative Colitis Following Proctocolectomy With Ileal Pouch-Anal Anastomosis: An Analysis of Pouchoscopies From 272 Patients

INTRODUCTION: Colectomy is required in up to 30% of patients with ulcerative colitis (UC) due to medically refractory disease or development of dysplasia/cancer. In such cases, restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the surgery of choice. However, pouchitis may occur in up to 80% of patients and significantly impairs quality of life. Pouchitis usually spares the pre-pouch ileum, however, a subset of patients develops discrete ulcerations in the pre-pouch ileum despite a firm preoperative UC diagnosis. The prevalence of such findings and predictive factors have not been adequately studied. We report a large cohort of patients undergoing pouchoscopies and describe the prevalence of pre-pouch ileal ulcerations. METHODS: This is a retrospective single-center study of adult patients with UC who had a total proctocolectomy with IPAA and subsequently underwent pouchoscopy between January 2007 and August 2018. At our institution, pouchoscopies are performed using a standard operating protocol. These reports include detailed descriptions of the mucosa as well as high definition images of the different areas of the pouch, the pre-pouch ileum, the pouch inlet, forward view of the pouch, a retroverted view of the pouch, and the rectal cuff. Based on these images and descriptions, we characterized pouch phenotypes and ulcer locations. Patient demographic and clinical data were also assessed. RESULTS: We reviewed the pouchoscopies of 272 patients who underwent proctocolectomy with IPAA for a pre-operative diagnosis of UC. Characteristics of the patients included median age 39 years (IQR 30.2-50.7), median BMI 25 (IQR 22.5-28.6), 90% Caucasian, 59% men, 23% ex-smokers and 83% extensive colitis prior to colectomy. 18% of our patients had discrete ulcerations in their pre-pouch ileum. In a multivariate analysis including age, sex, BMI, disease extent, race, ex-smoking status, prior treatment with anti TNFs or immunomodulators, history of PSC and previous infection with C. diff, none of these factors were associated with pre-pouch ileal ulcers (Table 1). CONCLUSION: Despite a firm diagnosis of UC prior to colectomy, 18% of patients at our tertiary center were found to have discrete ulcers in their pre-pouch ileum. We did not identify predictive factors in this patient cohort, but the potential impact of these findings suggests that proactive post-operative monitoring may be helpful.

1515 Portal Vein Thrombosis After Laparoscopic Sigmoid Colon Resection for Recurrent Diverticulitis

INTRODUCTION: Portal vein thrombosis (PVT) following laparoscopic surgery is a rare complication. PVT after laparoscopic colorectal surgery is usually associated with inflammatory conditions like IBD and is more likely to occur after total proctocolectomy and in presence of postoperative intra-abdominal infection. We report a case of PVT in a patient with no identifiable hypercoagulable disorder who underwent laparoscopic sigmoid colectomy for recurrent diverticulitis. CASE DESCRIPTION/METHODS: A 54 year old male with a past medical history of gout and no known history of thrombosis had recurrent diverticulitis. He also had Clostridium difficile infection secondary to antibiotic use. CT showed diverticulosis, but no portal vein thrombosis. He underwent robotic sigmoid colon resection with an uneventful postoperative course. Six months later he started noticing right upper quadrant abdomen pain and diarrhea that went on for five weeks. He denied confusion, ascites and GI bleed. His PCP ordered a CT abdomen in that showed right hepatic lobe atrophy and non-opacification of branches of right portal vein consistent with prior intrahepatic portal vein thrombosis. Lab work revealed normal CBC, negative stool studies, slightly elevated alkaline phosphatase of 137, AST of 31, ALT of 28, total bilirubin of 1. He was referred to our hepatology clinic. We repeated MR abdomen with and without contrast that confirmed chronic non-occlusive thrombus in the right portal vein, left portal vein and main portal vein were widely patent. Further lab work by hematology showed no evidence of thrombophilic condition. Repeat labs after three months showed normal LFTS and intact liver function. DISCUSSION: PVT as a complication of laparoscopic surgery is uncommon. Local and systemic factors contribute to development of post-op PVT. Thrombosis related to CO2pneumoperitoneum may be caused by hemodynamic changes like decrease in superior mesenteric arterial and portal venous blood flow. Issues with PVT have been seen related to splenectomy, bariatric procedures, and right hemicolectomy. Pneumoperitoneum induced hemodynamic changes and local inflammation due to diverticulitis might have contributed to PVT in our case.

1477 A Rare Case of Successful Endoscopic Decompression of Ileal-Pouch Volvulus

INTRODUCTION: Ileal-pouch anal anastomosis is a common surgery to restore bowel continence after total proctocolectomy. Obstruction is the most common late complication and occurs in up to 25% of cases. However, Ileal-pouch volvulus is an exceedingly rare complication of this procedure with only 16 published cases. All but one were treated with emergent surgical intervention. We present the second case of successful endoscopic decompression and the first reported in a patient with familial adenomatous polyposis (FAP). CASE DESCRIPTION/METHODS: A 33-year-old female with FAP status-post total proctocolectomy with ileoanal anastomosis and J-pouch formation complicated by history of ventral hernia repair and pouchitis presented with one week of severe left sided abdominal pain and emesis. On the day of presentation she noted new onset of abdominal distension causing her to present to the emergency room. The patients work up was notable for urosepsis, leukocytosis, AKI, hyperkalemia and a metabolic acidosis. A CT showed dilated small bowel loops swirled around a transition point in the left lower quadrant. A barium enema confirmed the diagnosis with barium that did not pass the distal rectum but was not therapeutic. Flexible sigmoidoscopy was then performed and showed twisted bowel with stenosis at 20 cm proximal to the anus. The endoscope was gently advanced with minimal CO2 insufflation and the stenosis re-opened, revealing dark gray, edematous mucosa with black spots. Proximally and distally, the mucosa appeared healthy. A colonic decompression tube was inserted to prevent future volvulus. The patient underwent exploratory laparotomy seven days later. No volvulus or bowel ischemia was found at that time and the pouch appeared viable. Extensive lysis of adhesions in the right upper quadrant and reduction of an internal hernia were performed. A small segment of small bowel was resected due to serosal injury. The patient then completed a course of antibiotics and was discharged home. DISCUSSION: This is a unique case of an extremely rare complication of an Ileal-pouch anal anastomosis. Additional abdominal surgeries, pouchitis and sepsis have been described as risk factors for pouch complications, all of which occurred in our patient and may have contributed to this rare presentation. Endoscopic decompression, if done expeditiously, may prevent loss of the pouch and need for surgical repair. This demonstrates the need for high clinical suspicion and awareness of this rare complication.

782 Pathological Findings of Resected Inflow Limbs in Patients With Pouches for Ulcerative Colitis

INTRODUCTION: Approximately 30% of ulcerative colitis (UC) patients require surgery during their disease course. Patients will commonly undergo total proctocolectomies with subsequent creation of a “pouch,” such as a J-pouch (pelvic pouch with ileal pouch-anal anastomosis) or a Kock pouch. A small portion of patients undergoing “pouch” procedures for UC will develop severe symptoms related to the inflow limb of the pouch requiring operative intervention. A clear understanding has not been established as to the pathological mechanism by which these pouch inflow problems develop and what pathologic entity they belong. METHODS: Methods: A retrospective chart review was conducted at a single tertiary medical center. A database was created of all patients who underwent subsequent pouch-related surgical procedures between 2006 and 2018. Patients with requiring operative intervention of the inflow limb were identified among this cohort. Operative and pathological data were collected. RESULTS: 178 patients were identified who underwent procedures yielding a surgical pathologic specimen related to their pouch. 15 of these procedures reported significant pathology of the inflow limb to the original pouch (9 J-pouches and 6 Kock pouches), occurring on average of 19.6 years after the creation of the original pouch. The most common diagnosis requiring reoperation was obstruction due to stricture formation (n = 11). Reoperations for inflow limb pathology included pouch excision (n = 3), inflow limb resection with ileo-pouch anastomosis (n = 9), stricturoplasty (n = 1), and bypass of the inflow limb (n = 1). In addition, 3 patients required a temporary, diverting loop ileostomy as part of their reoperation. The pathologic findings of the inflow limb were consistent with Crohn's ileitis in 6 patients (40%). 3 other patients (20%) were diagnosed with Crohn's disease and had diagnostic findings suspicious of Crohn's on inflow limb pathology. Remaining patients (n = 6) were found to have chronic, nonspecific enteritis/serositis. There was one case of poorly differentiated adenocarcinoma. CONCLUSION: A small proportion of patients who have undergone a “pouch” procedure require eventual surgery for a problem with the inflow limb of the pouch. These patients were found to have a high rate of Crohn's disease of the inflow limb and overall change in diagnosis to Crohn's disease.

1651 Colon, Urothelial and Sebaceous Malignancy All in One: A Rare Presentation of Muir-Torre Syndrome

INTRODUCTION: Muir-Torre syndrome (MTS) is a rare autosomal-dominant condition and phenotypic variant of Lynch syndrome caused by mutations in DNA mismatch repair genes MSH2, MSH6, and MLH1. Clinical diagnostic criteria include the presence of at least one sebaceous gland neoplasm and one visceral malignancy, often colonic or genitourinary. Compared to Lynch syndrome, the prevalence of MTS is likely underreported. We present a unique case of a patient with incidental findings of multiple visceral malignancies associated with skin cancer. CASE DESCRIPTION/METHODS: A 57-year-old Caucasian man presented for surveillance colonoscopy. His family history was significant for father with colon cancer at age 50, lung, and renal cancer. The patient was diagnosed with sebaceous adenocarcinoma, invasive squamous cell, and basal cell carcinomas requiring excisional surgery at age 46. Screening colonoscopy at that time was normal, with repeat after 7 years noting diminutive sigmoid tubular adenomas that were resected. Current colonoscopy revealed 14 colon polyps, multiple with high grade dysplasia, and an invasive sigmoid adenocarcinoma. Staging CT A/P was negative for metastatic disease. CEA was normal. The patient opted for left hemicolectomy instead of total proctocolectomy. Tumor pathology revealed moderately differentiated adenocarcinoma with negative margins (pT4N0M0). Colon cancer genetic testing showed lack of expression of MSH2/MSH6 and germline MSH2 mutation, confirming the diagnosis of MTS. A month later he developed hematuria and acute kidney injury that culminated in a new diagnosis of invasive high-grade urothelial carcinoma. Neoadjuvant chemotherapy was started with plan for cystectomy. DISCUSSION: MTS is a rare condition that warrants early recognition, regular follow-up and observation for new malignancies. Our case highlights the importance of awareness and the need for early, universal screening in high-risk individuals. MTS patients should undergo screening colonoscopy at ages 20–25 with surveillance every year thereafter to offer mortality benefit. If colon cancer is diagnosed, total rather than partial colectomy is preferred due to a high metachronous cancer rate of 25%. Patients and relatives should be counseled on increased risk of future malignancies. Reconstruction of family history may allow for the identification and selection of patients in whom to proceed with clinical, molecular, and immunohistochemical analysis. Clinicians must therefore be cognizant of this disease and its enduring effects.

A Case of Pseudomelanosis Duodeni: Striking Endoscopic Features With Subtle but Characteristic Pathologic Findings

Abstract A 72-year-old man was referred to the inflammatory bowel disease clinic for evaluation for possible Crohn disease. The patient was diagnosed with pan-ulcerative colitis in the late 1960s. He had medically refractory disease and required a total proctocolectomy with end ileostomy in the early 1970s. Since surgery, the patient has done well with his end ileostomy and has not required any inflammatory bowel disease medications. Over the past 14 months, he has developed intermittent foul-smelling serosanguinous drainage from his previous drained pilonidal cyst. His local gastroenterologist raised the concern for possible Crohn disease with perianal involvement. He has a significant family history of Crohn disease in his father. Past medical history includes chronic kidney disease, hypertension, diabetes, coronary artery disease, gout, and arthritis. Esophagogastroduodenoscopy (EGD) revealed diffuse brown to black speckled/tigroid spots in the entire examined duodenum. Duodenal biopsy showed scattered clusters of macrophages containing dark pigmented granules within the apical tips of the lamina propria. These pigmented granules were focally positive with iron staining. No villous blunting, intraepithelial lymphocytosis, granuloma, or significant inflammation was identified. The distinctive endoscopic and histologic features are diagnostic of pseudomelanosis duodeni. Pseudomelanosis duodeni is a rare benign entity characterized by diffuse black tigroid spots and aggregates of pigment-laden macrophages in the lamina propria. Results of iron staining are characteristically variable. Pseudomelanosis duodeni is seen in middle-aged to elderly adults. Most of the patients with pseudomelanosis duodeni are asymptomatic. Although the exact etiology remains unknown, pseudomelanosis duodeni has been reported to be associated with several medical conditions such as end-stage renal disease, hypertension, diabetes, heart failure, gastrointestinal hemorrhage, and medications, including oral iron therapy, furosemide, hydralazine, thiazide, and iron sulfate. Pathologists should be aware of this rare condition of the duodenum to make an accurate diagnosis of this rare entity.

Laparoscopic Surgery for Afferent Limb Syndrome after Total Proctocolectomy and Ileal Pouch Anal Anastomosis Occurring after Pregnancy Delivery

Laparoscopic Surgery for Afferent Limb Syndrome after Total Proctocolectomy and Ileal Pouch Anal Anastomosis Occurring after Pregnancy Delivery

Long-term survival between total colectomy versus proctocolectomy in patients with FAP: a registry-based, observational cohort study.

The best surgical choice for patients with familial adenomatous polyposis (FAP) is still debated. No prospective trials have been carried out to evaluate the pros and cons of the recommended procedures: total colectomy (ileorectal anastomosis [IRA]) vs restorative proctocolectomy (ileal pouch-anal anastomosis [IPAA]). The aim of this study was to provide a basis for tailored precision surgery in patients with FAP. We conducted a retrospective review of patients with FAP who underwent surgery and were registered in a dedicated database in Milan, Italy. Twenty-year survival related to surgical approach and prognostic factors were investigated using a Cox regression model. A total of 925 patients underwent surgery between 1947 and 2015: 340 (36.8%) IPAA and 585 (63.2%) IRA. Colorectal cancer (CRC) at surgery was diagnosed in 28.6% of patients and a pathogenic APC variant was identified in 88%. During a median follow-up of 129 months, 150 patients died. The survival probability was significantly higher in the IRA than the IPAA group: 0.82 vs 0.75 (hazard ratio [HR] 0.6, 95% confidence interval [CI] 0.42-0.84). Multivariable regression modeling adjusted for propensity scores showed a similar difference, although no longer significant. Multivariable analysis indicated as independent risk factors CRC (HR 4.68, 95% CI 3.04-7.20) and age at surgery (HR 1.03, 95% CI 1.02-1.06). Among patients without cancer, the main risk factor for shorter survival was older age (HR 1.06, 95% CI 1.04-1.09). The study confirms excellent long-term results of surgical approaches with IRA and IPAA, suggesting that the best surgical choice may be an individually and clinically tailored approach, preferably at a young age.

Early Experience with Variant Two-Stage Approach in Surgical Management of Inflammatory Bowel Disease Colitis in the Pediatric Population.

Introduction: Multistaged surgical management of inflammatory bowel disease (IBD), culminating in ileal pouch-anal anastomosis (IPAA), can provide cure for refractory IBD symptoms while maintaining fecal continence. Surgical approaches to IPAA historically included a three-stage approach done by subtotal colectomy (STC) followed by IPAA with diversion. Recently, a variant two-stage approach without diversion at IPAA has become increasingly utilized, yet evidence of the efficacy of this approach is limited. Methods: Retrospective review of patients aged 5-21 years who underwent initial STC, followed by a total proctocolectomy with IPAA +/- diversion for medically refractory IBD from January 2010 to August 2018 (n = 25). Results: Majority of IPAA procedures were done laparoscopically (88.5%). Thirteen patients (52%) underwent two-stage variant IPAA. There were no differences in readmission rates (66.7% versus 53.8%, P = .5) or reoperation rates (50% versus 30.8%, P = .3) between groups. Forty percent of patients experienced a complication after IPAA. Complication rates were similar between two-stage and three-stage IPAA groups (38.5% versus 50%, P = .33). Complications within the two-stage group included anastomotic leak, pouchitis, wound infection, anastomotic stricture, and incarcerated hernia. Complications within the three-stage group included bloody ostomy output, dehydration, anastomotic stricture, small bowel obstruction, and pouch volvulus. Conclusions: Treatment of refractory IBD in children remains challenging, but STC followed by IPAA is an approach that provides symptom relief and preserves continence. Complication rates remained unchanged regardless of whether IPAA was conducted with or without diversion, demonstrating that the two-stage variant approach is a safe and feasible treatment that may reduce subsequent anesthesia exposure and trips to the operating room.

Intestinal Contents Stayed After Discharge as Low Anterior Resection Syndrome

Background: The lower anastomosis after low anterior resection (LAR) is associated with the development of low anterior resection syndrome (LARS). However, pathophysiology of fragmentation of defecation which is one of LARS remains unknown. Patients and Methods: Between January 2015 and December 2017, eight patients with temporary ileostomy who underwent LAR with double stapling technique (DST) for rectal cancer at our hospital were studied. As the control group, nine patients with temporary ileostomy who underwent proctocolectomy (PC) with DST for ulcerative colitis in the same period were recruited. Defecography was performed two weeks after closure of the ileostomy. The defecation of all patients was evaluated by defecography. Results: In all patients, the descent of the perineum and linearization of the anorectal angle were observed. In patients after PC, all barium could be discharged by some abdominal pressures. On the other hand, in all patients after LAR, barium located anal to the anastomosis could be discharged through the anus, while barium located oral to the anastomosis stayed after discharge, which could not be expelled by maximal abdominal pressures more than 5 minutes after starting evaluation. Conclusion: Remnant intestinal contents after defecation seems to be one of disturbance for defecation after LAR.