Clinical outcomes and learning curve for ERCP during advanced endoscopy training: a comparison of supine versus prone positioning.

BACKGROUND AND AIMS: No studies have evaluated trainees' outcomes when learning ERCP with patients in the supine and prone positions simultaneously. We aimed to assess whether patient position impacts procedural outcomes and learning curve. METHODS: We prospectively evaluated patients undergoing ERCP by a supervised advanced endoscopy trainee (AET) at a tertiary care center. Adult patients with native papillae were included. The AET was universally given 5 attempts per cannulation. Outcomes were evaluated quarterly. RESULTS: Successful cannulation was achieved in 44 supine (69%) and 17 prone (68%) patients (P = .95). Although mean time to reach the papilla was shorter in the supine patient position, time to biliary cannulation (7.8 vs 9.4 minutes, P = .53) and number of attempts were similar. A stepwise increase was seen in cannulation rates throughout the academic year (P < .01) and increased more in supine patients (P = .01). Procedure and total room times were shorter in supine patients. CONCLUSIONS: Shorter procedure and room turnover times and a comparable cannulation rate were found for supine versus prone ERCP.

Endoscopic ultrasound-guided gallbladder drainage as a rescue therapy for unresectable malignant biliary obstruction: a multicenter experience.

BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP) is often unsuccessful in patients with duodenal stenosis or malignant ampullary infiltration. While endoscopic ultrasound-guided biliary drainage (EUS-BD) has been proposed as an alternative, EUS-guided gallbladder drainage (EUS-GBD) is an attractive option when both approaches fail. We aimed to assess the effectiveness and safety of EUS-GBD as rescue therapy for malignant distal bile duct obstruction. METHODS: A multicenter retrospective study was performed on patients with unresectable malignant distal bile duct obstruction who underwent EUS-GBD between 2014 and 2019 after unsuccessful ERCP and EUS-BD. Clinical success was defined as a decrease in serum bilirubin of > 50 % within 2 weeks. RESULTS: 28 patients were included, with a lumen-apposing metal stent used in 26 (93 %) and a self-expandable metal stent in two (7 %). The technical success rate was 100 %. The clinical success rate was 93 %, with an improvement in bilirubin (7.3 [SD 5.4] pre-procedure vs. 2.8 [SD 1.1] post-procedure; P = 0.001). Delayed adverse events included food impaction of the stent (n = 3), with a further two patients developing cholecystitis and bleeding. CONCLUSION: This study demonstrates the feasibility of gallbladder drainage to relieve malignant distal bile duct obstruction in patients with failed ERCP and EUS-BD.

Risk of Pancreatic Cancer in Patients With Pancreatic Cysts and Family History of Pancreatic Cancer.

BACKGROUND & AIMS: A diagnosis of pancreatic cancer in a first-degree relative increases an individuals' risk of this cancer. However, it is not clear whether this cancer risk increases in individuals with pancreatic cystic lesions who have a first-degree relative with pancreatic cancer. The Fukuoka criteria are used to estimate risk of pancreatic cancer for patients with pancreatic cystic lesions: individuals with cysts with high risk or worrisome features (Fukuoka positive) have a higher risk of pancreatic cancer than individuals without these features (Fukuoka negative). We aimed to compare the risk of pancreatic cancer and surgery based on presence or absence of pancreatic cystic lesions and a first-degree relative with pancreatic cancer. METHODS: We performed a retrospective study of patients seen at the Mayo Clinic in Rochester, Minnesota, from January 1, 2000, through December 31, 2012. We identified individuals with: pancreatic cystic lesions and first-degree relative with pancreatic cancer (group 1, n = 269), individuals with pancreatic cystic lesions but no first-degree relative with pancreatic cancer (group 2, n = 1195), and individuals without pancreatic cystic lesions but with a first-degree relative with pancreatic cancer (group 3, n = 720). We compared, among groups, as well among patients with cysts classified according to Fukuoka criteria, proportions of individuals who developed pancreatic cancer or underwent pancreatic surgery within a 5-year period. RESULTS: A significantly higher proportion of individuals in group 1 developed pancreatic cancer during the 5-year period than in group 3 (6.64% vs 1.69%; P = .03); there was no significant difference between the percentage of individuals in group 1 vs group 2 who developed pancreatic cancer (6.64% vs 4.05%; P = .41). There was no significant difference in pancreatic cancer development among individuals with Fukuoka-positive cysts with vs without a family history of pancreatic cancer (P = .39). There was no significant difference in the proportion of patients in group 1 vs group 2 who underwent pancreatic surgery for their pancreatic cyst over the 5-year period (14.37% vs 11.80%; P = .59). Among patients with Fukuoka-negative cysts, a significantly higher proportion underwent surgery in group 1 than in group 2 (10.90% vs 5.90%; P = .03). However, among patients with Fukuoka-positive cysts, there was no difference in proportions of patients who underwent surgery between groups 1 and 2 (P = .66). CONCLUSIONS: In a retrospective study of patients with pancreatic cysts and/or cancer, we found that a family history of pancreatic cancer does not affect 5-year risk of pancreatic cancer in patients with pancreatic cystic lesions. Despite this, among patients with Fukuoka-negative cysts, a higher proportion of those with a family history of pancreatic cancer undergo surgery than patients without family history of pancreatic cancer.

Large-caliber metal stents versus plastic stents for the management of pancreatic walled-off necrosis.

BACKGROUND AND AIMS: Symptomatic pancreatic walled-off necrosis (WON) may be managed by endoscopic transmural drainage and endoscopic transmural necrosectomy, with stent placement at endoscopic drainage sites. The optimal stent choice is yet to be determined. We compared outcomes after endoscopic management of WON using either large-caliber fully covered self-expandable metal stents (LC-SEMSs) or double-pigtail plastic stents (DPPSs). METHODS: We performed a retrospective comparison of outcomes among patients who received LC-SEMSs or DPPSs before endoscopic transmural necrosectomy for WON. RESULTS: Among 94 patients included, WON resolution rates did not differ between the DPPS (36 patients) and LC-SEMS (58 patients) groups, whether concomitant percutaneous drainage was considered a failure (75% vs 82.8%; P = .36) or not (91.7% vs 94.8%; P = .55). Of 75 patients (80%) successfully treated without percutaneous drainage, 37 (49%) underwent endoscopic transmural drainage without subsequent endoscopic transmural necrosectomy. WON was more likely to resolve without subsequent endoscopic transmural necrosectomy in the LC-SEMS group than the DPPS group (60.4% vs 30.8%; P = .01). WON resolution without subsequent endoscopic transmural necrosectomy remained more likely with LC-SEMSs (odds ratio, 4.5 [95% confidence interval, 1.5-15.5]) after adjusting for patient age and size and location of WON. Rates of adverse events were similar except for clinically significant bleeding requiring endoscopic intervention, which was higher with DPPSs than LC-SEMSs (14% vs 2%; P = .02). CONCLUSION: Management of pancreatic WON with LC-SEMSs appears to decrease both the need for repeated necrosectomy procedures and the risk of intervention-related hemorrhage.

Fukuoka criteria accurately predict risk for adverse outcomes during follow-up of pancreatic cysts presumed to be intraductal papillary mucinous neoplasms.

OBJECTIVE: Fukuoka consensus guidelines classify pancreatic cystic lesions (PCLs) presumed to be intraductal papillary mucinous neoplasms (IPMNs) into Fukuoka positive (FP) (subgroups of high-risk (HR) and worrisome features (WFs)) and Fukuoka negative (FN) (non-HR feature/WF cysts). We retrospectively estimated 5-year risk of pancreatic cancer (PC) in FN, WF and HR cysts of patients with PCL-IPMN. DESIGN: From Mayo Clinic databases, we randomly selected 2000 patients reported to have a PCL; we excluded inflammatory or suspected non-IPMN cysts and those without imaging follow-up. We re-reviewed cross-sectional imaging and abstracted clinical and follow-up data on PCL-IPMNs. The study contained 802 patients with FN cysts and 358 with FP cysts. RESULTS: Patients with PCL-IPMN had median (IQR) follow-up of 4.2 (1.8-7.1) years. Among FN cysts, 5-year PC risk was low (2-3%) regardless of cyst size (p=0.67). After excluding events in the first 6 months, 5-year PC risk remained low (0-2%) regardless of cyst size (p=0.61). Among FP cysts, HR cysts (n=66) had greater 5-year PC risk than WF cysts (n=292) (49.7% vs 4.1%; p<0.001). In HR cysts, 3-year PC risk was greatest for obstructive jaundice versus enhancing solid component or main pancreatic duct >10 mm (79.8% vs 37.3% vs 39.4%, respectively; p=0.01). CONCLUSIONS: Fukuoka guidelines accurately stratify PCL-IPMNs for PC risk, with FN cysts having lowest and HR cysts having greatest risk. After 6-month follow-up, WF and FN cysts had a low 5-year PC risk. Surveillance strategies should be tailored appropriately.

Clinical, Biochemical, and Histopathology Features of Patients With Glycogenic Hepatopathy.

BACKGROUND & AIMS: Glycogenic hepatopathy, a syndrome characterized by hepatomegaly and increased liver transaminases in patients with type 1 diabetes, has not been well characterized in adults. We describe the clinical, biochemical, and histopathology profile of a cohort of patients with glycogenic hepatopathy. We also examined differences between patients with type 1 diabetes with versus without glycogenic hepatopathy. METHODS: We performed a case-control study of patients with type 1 diabetes diagnosed with glycogenic hepatopathy and patients with type 1 diabetes without glycogenic hepatopathy (control subjects). Cases were identified in the database of electronic medical records at Mayo Clinic, Rochester from January 1, 1998, through January 1, 2014. Age- and sex-matched control subjects were identified from a Mayo Clinic registry of patients with type 1 diabetes who had normal levels of liver enzymes. Demographic, clinical, laboratory, and histopathology data were collected and compared between cases and control subjects. The primary outcome was difference in frequency of diabetic ketoacidosis episodes and hemoglobin (Hb) A1c levels between cases and control subjects. RESULTS: Among the 36 patients diagnosed with glycogenic hepatopathy, 20 had undergone liver biopsy analysis. Most cases were female (n = 28; 77.8%). Abdominal pain was the most common symptom (n = 23; 63.9%); 28 patients (77.8%) had hepatomegaly. All patients had poor control of diabetes (mean HbA1c level, 11.2 ± 2.4%). A higher proportion of cases had recurrent episodes of diabetic ketoacidosis (61%) than control subjects (9%) (P = .009), and cases had a higher mean level of HbA1c (11.2 ± 2.4% vs 9.0 ± 2.2% in control subjects; P = .0004). Adult cases had higher levels of aspartate transaminase (312.5 IU/L; range, 245.5-775 IU/L) than pediatric cases (157; range, 104-267 IU/L; P = .02) and lower serum levels of albumin (3.7 ± 0.5 g/dL vs 4.3 ± 0.4 g/dL for pediatric cases; P = .008). Only 16.7% of pediatric patients with glycogenic hepatopathy had growth retardation. Levels of liver transaminases were normalized at follow-up examinations of 18 of 21 adult or pediatric patients with glycogenic hepatopathy. CONCLUSIONS: More than half of patients with glycogenic hepatopathy and type 1 diabetes have recurrent episodes of diabetic ketoacidosis, and these patients have higher levels of HbA1c than patients with type 1 diabetes without glycogenic hepatopathy. We observed growth retardation in only about 17% of pediatric patients with glycogenic hepatopathy.

Open-Capsule Budesonide for Refractory Celiac Disease.

OBJECTIVES: Refractory celiac disease (RCD) is a rare condition often associated with poor prognosis. Various immunosuppressive medications (IMs) have been used with modest success. We describe outcomes in patients treated with open-capsule budesonide (OB), including those for whom IM treatment failed. METHODS: We identified RCD patients treated with OB at Mayo Clinic, Rochester, Minnesota from 2003 to 2015. Demographic, serologic, and clinical variables were analyzed. RESULTS: We identified 57 patients who received OB for suspected RCD. Based on clonal T-cell receptor gamma gene rearrangement or aberrant phenotype of intraepithelial lymphocytes (IELs), 13 patients (23%) were classified as having RCD-2 and 43 (75%) as RCD-1. In one patient (2%) TCR gene rearrangement status was unknown. Most patients were women (69%), mean (s.d.) age was 60.5 (3.5) years and body mass index was 28.4 (4.5) kg/m2. The majority had diarrhea (72%), with median of 6 bowel movements per day (range, 4-25). IM treatment (azathioprine, systemic corticosteroids, or regular budesonide) had failed in nearly half. Twenty-four patients (42%) had anemia and 12 (21%) had hypoalbuminemia. All had Marsh 3 lesions on biopsy: 3a (19%), 3b (46%), and 3c (35%). After OB therapy, the majority had clinical (92%) and histologic (89%) improvement. Follow-up biopsy in 7 out of 13 patients with RCD-2 (53%) showed an absence of clonal TCR gamma gene rearrangement/aberrant IEL phenotype previously seen. On follow-up, 2 patients (4%) died of enteropathy-associated T-cell lymphoma. CONCLUSIONS: Most patients with RCD show clinical and histopathologic improvement with OB therapy, including those with failure of IMs. OB is a promising therapeutic option for management of RCD.

Effects of carbon dioxide insufflation during direct cholangioscopy on biliary pressures and vital parameters: a pilot study in porcine models.

BACKGROUND AND AIMS: Direct per-oral cholangioscopy allows endoscopic visualization of the biliary tract. Insufflation with carbon dioxide (CO2) is an alternative to saline solution irrigation during direct cholangioscopy. There are no data on maximal CO2 insufflation in direct cholangioscopy without causing biliary barotrauma or fatal gas embolism. We aimed to evaluate the safety of increasing CO2 insufflation in direct cholangioscopy without causing biliary barotrauma or fatal gas embolism. METHODS: This was an in vivo animal study. Four domestic pigs, under general endotracheal anesthesia, were used. The first animal was used to validate the feasibility of direct cholangioscopy and biliary pressure measurements, after which all animals underwent laparotomy, insertion of a pressure transducer in the cystic duct, and direct transpapillary placement of the cholangioscope. The common bile duct (CBD) and cystic duct were ligated to contain the instilled gas and exclusively expose the biliary tree. Insufflation of CO2 started at 200 mL/min and was continuously increased until there was evidence of bile duct rupture (as measured by a drop in intraductal pressures) or instability of vital signs (hypotension, bradycardia, bradypnea, O2 desaturation). Necropsy was performed on all animals to assess the liver and biliary system for evidence of barotrauma. RESULTS: CO2 was insufflated up to 8 L/min without causing bile duct rupture or instability in vital signs despite increasing CBD pressure with insufflation. There was significant correlation between CO2 flow with partial pressure of CO2 in arterial blood (PaCO2) (coefficient, 0.96-1.00; P < .01) and end tidal expired CO2 (EtCO2) (coefficient, 0.94-1.00; P < .01). However, the pulse rate, respiratory rate, arterial blood pressure, and O2 did not correlate with the amount of CO2 flow. There was no evidence of hepatic or biliary barotrauma on necropsy. CONCLUSIONS: This pilot experience in porcine models suggests that CO2 insufflation is safe for direct cholangioscopy and does not result in biliary barotrauma or vital signs instability.

Clinical Features and Outcomes of Gastric Ischemia.

BACKGROUND AND AIMS: Gastric ischemia is a rare condition associated with poor prognosis. Our study aim was to highlight the clinical features and outcomes of patients with gastric ischemia. METHODS: A retrospective review of patients diagnosed with isolated gastric ischemia at our institution from January 1, 2000, to May 5, 2016, was performed. Demographic, clinical, endoscopic, radiologic, and outcome variables were abstracted for analysis. RESULTS: Seventeen patients (65% men) with mean age of 69.3 ± 11.3 years and body mass index of 28.8 ± 11.1 were identified. The etiologies for gastric ischemia included local vascular causes (n = 8), systemic hypoperfusion (n = 4), and mechanical obstruction (n = 5). The most common presenting symptoms were abdominal pain (65%), gastrointestinal bleeding (47%), and altered mental status (23%). The typical endoscopic appearance was mucosal congestion and erythema with or without ulceration. Gastric pneumatosis and portal venous air were more commonly seen on CT imaging. Radiologic and/or surgical intervention was needed in 9 patients, while the remaining 8 patients were managed conservatively with acid suppression, antibiotics, and nasogastric tube decompression. The median duration of hospital stay was 15 days (range 1-36 days). There were no cases of rebleeding and the mortality rate as a direct result of gastric ischemia was 24% within 6 months of diagnosis. CONCLUSION: Although uncommon, gastric ischemia is associated with significant mortality. Endoscopy and CT imaging play an important role in its diagnosis. The management of gastric ischemia is dictated by its severity and associated comorbidities.

Safety and efficacy of fistula closure by endoscopic suturing: a multi-center study.

Background and aim: Endoscopic closure of fistulas is desirable but remains a challenge, especially for gastrogastric fistulas. The aim of this study was to assess the success rate for the closure of gastrointestinal fistulas using an endoscopic suturing device. Patients and methods: Electronic records at three medical centers were reviewed to identify patients who underwent sutured fistula closure as recorded on a central database. Demographic details, clinical variables, and details of the endoscopic procedure were recorded. Results: A total of 56 patients (mean age 54 ± 12.7 years) underwent fistula closure. Gastrogastric fistulas were most common (n = 29; 51.8 %). Sixteen patients (28.6 %) had undergone previous failed attempts at closure. Immediate success was universal (n = 56; 100 %). Thirteen patients had persistent closure without any need for further therapies, while 17 underwent additional endoscopic procedures, with successful closure in 4 /17. Only one patient had abdominal pain requiring hospital admission. Conclusion: Sutured closure of fistulas in a single session is safe and can be expected, even in what are known to be challenging cases. Sequential closure for recurrences is sometimes successful in the longer term.

Endoscopically inserted nasobiliary catheters for high dose-rate brachytherapy as part of neoadjuvant therapy for perihilar cholangiocarcinoma.

BACKGROUND AND AIM: Selected patients with unresectable perihilar cholangiocarcinoma can undergo neoadjuvant chemoradiotherapy followed by liver transplantation, which has been shown to improve survival. The aim of this study was to determine the feasibility and safety of endoscopic transpapillary insertion of nasobiliary tubes (NBTs) and brachytherapy catheters for high dose-rate (HDR) brachytherapy as part of this neoadjuvant chemoradiotherapy. PATIENTS AND METHODS: Medical records of patients undergoing biliary brachytherapy for hilar cholangiocarcinoma at the Mayo Clinic, Rochester were reviewed. Patients were treated with curative intent using external beam radiotherapy (4500 cGy), chemotherapy (5-FU or capecitabine), and HDR brachytherapy (930 - 1600 cGy in one to four fractions delivered over 1 - 2 days) prior to planned liver transplantation. RESULTS: Between 2009 and 2013, 40 patients underwent biliary HDR brachytherapy via endoscopically placed NBTs (8.5 - 10 Fr). Patients had a median age of 55 years (range 28 - 68); 25 patients (62.5 %) had primary sclerosing cholangitis. Prior to therapy, 29 patients (72.5 %) had plastic stents, two (5 %) had metal stents, and nine (22.5 %) had no stents. Bilateral NBTs were placed in five patients (12.5 %). NBT/brachytherapy catheter displacement was seen in eight patients (20 %) - five intraprocedure and three post-procedure. A radiotherapy error and NBT kinking each occurred once. Post-procedure adverse events included: cholangitis (n = 5; 12.5 %), severe abdominal pain (n = 3; 7.5 %), duodenopathy (n = 3; 7.5 %), gastropathy (n = 3; 7.5 %), and both duodenopathy and gastropathy (n = 2; 5 %). CONCLUSION: HDR biliary brachytherapy administered via endoscopically placed NBTs and brachytherapy catheters is technically feasible and appears reasonably safe in selected patients with unresectable perihilar cholangiocarcinoma.

Comparable pouch retention rate between pediatric and adult patients after restorative proctocolectomy and ileal pouches.

BACKGROUND & AIMS: We compared long-term outcomes between adult and pediatric patients with inflammatory bowel disease (IBD) who underwent restorative proctocolectomy with ileal pouch-anal anastomosis. METHODS: We performed a retrospective study that analyzed data from consecutive patients with ileal pouches who presented to the subspecialty Pouch Center at the Cleveland Clinic from 2002-2011. Pouch outcomes of 104 pediatric patients (having pouch surgery at age <18 years; 53 male) were compared with those of 1135 adults (having pouch surgery at an age 18 years or older; 632 male). RESULTS: Pediatric patients had a shorter duration from time of IBD diagnosis to colectomy than adult patients. Fewer pediatric than adult patients had a history of smoking, concomitant extraintestinal manifestations, or dysplasia as the indication for colectomy. However, pediatric patients had higher rates of pouch procedure-related complications, postoperative pouch-associated hospitalization, and postoperative use of anti-tumor necrosis factor (TNF) agents. In multivariate analysis, risk factors for pouch failure included preoperative use of anti-TNF agents (hazard ratio [HR], 1.81; 95% confidence interval [CI], 1.05-3.13; P = .032), postoperative use of anti-TNF agents (HR, 2.07; 95% CI, 1.31-3.27; P = .002), Crohn's disease of the pouch (HR, 2.21; 95% CI, 1.28-3.82; P = .005), pouch procedure-related complications (HR, 2.68; 95% CI, 1.55-4.64; P < .001), and postoperative pouch-associated hospitalization (HR, 25.20; 95% CI, 14.44-43.97; P < .001). Being a pediatric patient was not significantly associated with pouch failure in univariate or multivariate analyses (HR, 0.6; 95% CI, 0.32-1.16; P = .13). CONCLUSIONS: On the basis of an analysis of patients with IBD who underwent restorative proctocolectomy and presented at a subspecialized Pouch Center, patients who had the surgery at a pediatric age tend to have a higher incidence of postoperative pouch complications than adults. However, long-term rates of pouch retention were comparable.

Long per-rectal endoscopic myotomy for a case of Hirchsprung's disease.

Video 1The colonoscopy showed a nondilated rectum, distal sigmoid, and a roomy, dilated proximal colon, with the transition zone at 23 cm from the squamo-columnar junction.

Progressive primary sclerosing cholangitis requiring liver transplantation is associated with reduced need for colectomy in patients with ulcerative colitis.

BACKGROUND & AIMS: We investigated the association between the severity of primary sclerosing cholangitis (PSC) and clinical outcomes of patients with ulcerative colitis (UC) on the basis of need for colectomy. METHODS: We analyzed data from 167 patients with PSC and UC who were followed from 1985 to 2011. Patients with PSC and UC were divided into groups that received orthotopic liver transplantation (OLT) (n = 86) or did not (non-OLT, n = 81). Clinical and demographic variables were obtained, and patients were followed until they received OLT or the date of their last clinical visit. RESULTS: The OLT group had significantly more subjects with less severe symptoms of UC (59, 68.6%) than the non-OLT group (12, 14.8%; P < .001). The subjects in the OLT group had a median of 0 UC flares compared with 3 in the non-OLT group (P < .001); fewer subjects in the OLT group required use of azathioprine or mercaptopurine (1, 1.2%), compared with the non-OLT group (14, 17.3%; P = .006). More subjects in the non-OLT group required colectomies (61, 75.3%) than in the OLT group (23, 26.7%; P < .001). On the basis of Cox regression analysis, OLT for PSC independently reduces the need for colectomy (hazard ratio [HR], 0.43; 95% confidence interval [CI], 0.25-0.75; P = .003), as does a high Mayo risk score at diagnosis (HR, 0.52; 95% CI, 0.37-0.72; P < .001). Development of colon neoplasia increased the risk for colectomy (HR, 2.47; 95% CI, 1.63-3.75; P < .001). CONCLUSIONS: Severe progressive PSC that requires liver transplantation appears to reduce the disease activity of UC and the need for colectomy.