Role of video capsule endoscopy in patients with constitutional mismatch repair deficiency (CMMRD) syndrome: report from the International CMMRD Consortium.

BACKGROUND AND STUDY AIMS: Constitutional mismatch repair deficiency (CMMRD) syndrome, also known as biallelic mismatch repair deficiency (BMMRD) syndrome is a rare autosomal-recessive genetic disorder that has a high mortality due to malignancy in childhood and early adulthood. The small bowel phenotype in CMMRD is not well described and surveillance protocols for small bowel cancer have not been well established. This study was conducted to evaluate the usefulness and clinical impact of video capsule endoscopy (VCE) for small bowel surveillance. PATIENTS AND METHODS: We retrospectively reviewed the prospectively maintained International CMMRD Consortium database. Treating physicians were contacted and VCE report data were extracted using a standardized template. RESULTS: Among 58 patients included in the database, 38 VCE reports were collected from 17 patients. Polypoid lesions were first detected on VCE at a median age of 14 years (range: 4 - 17). Of these, 39 % in 7 patients (15/38) showed large polypoid lesions (> 10 mm) or multiple polyps that prompted further investigations. Consequently, three patients were diagnosed with small bowel neoplasia including one patient with adenocarcinoma. Small bowel neoplasia and/or cancer were confirmed histologically in 35 % of the patients (6/17) who had capsule surveillance and the lesions in half of these patients were initially visualized on VCE. Multiple polyps were identified on eight VCEs that were completed on three patients. Ten VCEs (28 %) were incomplete due to slow bowel transit; none required capsule removal. CONCLUSIONS: Small bowel surveillance in patients with CMMRD should be initiated early in life. VCE has the potential to detect polyps; however, small bowel neoplasias are often proximal and can be missed, emphasizing the importance of concurrent surveillance with other modalities. MEETING PRESENTATIONS: Digestive Disease Week 2017 and World Congress of Pediatric Gastroenterology, Hepatology and Nutrition 2016.

'Short' double-balloon enteroscope endoscopic retrograde cholangiopancreatography in patients with a surgically altered upper gastrointestinal tract.

BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP) remains a challenge for endoscopists in patients with surgically altered anatomy of the upper gastrointestinal tract. Double-balloon enteroscopes (DBEs) have revolutionized the ability to access the small bowel. The indication for its therapeutic use is expanding to include ERCP for patients who have undergone small bowel reconstruction. Most of the published experiences in DBE-assisted ERCP have used conventional double-balloon enteroscopes that are 200 cm in length, which do not permit use of the standard ERCP accessories. The authors report their experience with DBE-assisted ERCP using a 'short' DBE in patients with surgically altered anatomy. METHODS: A retrospective review of patients with previous small bowel reconstruction who underwent ERCP with a 'short' DBE at the Centre for Therapeutic Endoscopy and Endoscopic Oncology (Toronto, Ontario) between February 2007 and November 2008 was performed. RESULTS: A total of 20 patients (10 men) with a mean age of 57.9 years (range 26 to 85 years) underwent 29 sessions of ERCP with a DBE. Six patients underwent Billroth II gastroenterostomy, seven patients Roux-en-Y hepaticojejunostomy, five patients Roux-en-Y gastrojejunostomy, one patient Roux-en-Y esophagojejunostomy and one patient a Whipple's operation with choledochojejunostomy. Some patients (n=12 [60%]) underwent previous attempts at ERCP in which the papilla of Vater or bilioenteric anastomosis could not be reached with either a duodenoscope or pediatric colonoscope. All procedures were performed with a commercially available DBE (working length 152 cm, distal end diameter 9.4 mm, channel diameter 2.8 mm). The procedures were performed under conscious sedation with intravenous midazolam, fentanyl and diazepam, except in one patient in whom general anesthesia was administered. Either the papilla of Vater or bilioenteric anastomosis was reached in 25 of 29 cases (86.2%) in a mean duration of 20.8 min (range 5 min to 82 min). Bile duct cannulation was successful in 24 of 25 cases in which the papilla or bilioenteric anastomosis was reached. Therapeutic interventions were successful in 15 patients (24 procedures) including sphincterotomy (n=7), stone extraction (n=9), biliary dilation (n=8), stent placement (n=9) and stent removal (n=8). The mean total duration of the procedures was 70.7 min (range 30 min to 117 min). There were no procedure-related complications. CONCLUSION: DBEs enable successful diagnostic and therapeutic ERCP in patients with a surgically altered anatomy of the upper gastrointestinal tract. It is a safe, feasible and less invasive therapeutic option in this group of patients. Standard 'long' DBEs have limitations of long working length and the need for modified ERCP accessories. 'Short' DBEs are equally as effective in reaching the target limb as standard 'long' DBEs, and overcomes some limitations of long DBEs to result in high success rates for endoscopic therapy.

Length of Barrett's segment predicts success of extensive endomucosal resection for eradication of Barrett's esophagus with early neoplasia.

BACKGROUND: Although the efficacy and safety of extensive endomucosal resection (EMR) in eradicating Barrett's esophagus (BE) harbouring early neoplasia have been established, factors predicting efficacy remains unclear. AIM: To determine the complete eradication rate of Barrett's esophagus with high-grade intraepithelial neoplasia (HGIN) or intramucosal carcinoma (IMC), safety, and factors predicting complete eradication by EMR. METHODS: Patients with histological confirmation of Barrett's HGIN/IMC were prospectively identified. EMR was performed using Duette multiband ligator or cap technique by a single operator (NEM). RESULTS: 99 patients (81 males) with median age 67 years [interquartile range (IQR) 60-77 years] and median Barrett's length 4 cm (IQR 2-6 cm) were included. Of 628 index EMRs [mean 6.3, median 5 (IQR 3-8)], 23% showed IMC, 58.5% showed HGIN, and 16% showed low-grade dysplasia only. A median of 8 EMR resections per patient (IQR 6-16, 1,064 resections in 89 patients) resulted in complete eradication of BE harboring neoplasia in 49.4% and eradication of HGIN/IMC in 81% (BE <5 cm subgroup: 65% complete eradication and 91% HGIN eradication) at median follow-up of 18 months (range 6-27 months). On univariate analysis, focal dysplasia (P = 0.003) and Barrett's length <5 cm (P = 0.001) were predictors of complete BE eradication. Barrett's length <5 cm was the only significant predictor [odds ratio (OR) 3.4, standard error (SE) 0.11, P = 0.0006] on multiple logistic regression analysis. Strictures developed in 27% and major bleeding in 2% with no procedure-related perforations or mortality. CONCLUSIONS: Extensive EMR for removal of BE with early neoplasia is safe. Outcomes for complete BE eradication are modest at 49.4% and eradication of high-grade dysplasia at 81%. Barrett's length <5 cm is the only significant predictor of complete response.

Fluticasone propionate for treatment of esophageal lichen planus. A case series.

Esophageal lichen planus is a rare condition, and although the majority of cases occur in conjunction with lichen planus at other sites, the endoscopic features are often misinterpreted resulting in a delay in diagnosis. We report a series of five patients presenting to our unit between 2005 and 2009. All five patients were female and presented with dysphagia. Endoscopy demonstrated proximal esophageal stricturing in four patients. Characteristic histological findings were found in four patients. Lichen planus was diagnosed at other sites, and preceded gastrointestinal symptoms, in all patients; five had oral involvement, two had genital involvement, and one had dermal involvement. All patients received proton pump inhibitor therapy without demonstrable benefit. Administration of oral fluticasone proprionate resulted in symptomatic improvement in three patients.

Unsedated transnasal endoscopy: a Canadian experience in daily practice.

BACKGROUND: Esophagogastroduodenoscopy (EGD) is the most frequently performed diagnostic procedure for upper gastrointestinal disorders. The procedure is routinely performed under conscious sedation in North America. A significant proportion of morbidity and mortality associated with EGD is related to hypoxia due to conscious sedation. The use of sedation is also associated with an increase in cost, loss of work on the day of endoscopy and the need for the patient to be accompanied home after the procedure. Transnasal endoscopy has advantages such as no sedation and less patient monitoring, nursing time and expenses than conventional per oral EGD. OBJECTIVES: To assess the feasibility and acceptability of unsedated transnasal EGD in daily practice. METHODS: Patients due to undergo EGD were given a choice of either unsedated transnasal EGD or per oral EGD with sedation. Patients who chose unsedated transnasal EGD had the procedure performed in the office by a senior gastroenterologist with experience in transnasal EGD. All procedures were performed using a small-calibre esophagogastroduodenoscope. All patients were surveyed using a patient satisfaction questionnaire, and were asked to give specific scores in terms of choking sensation, sore throat, nasal discomfort and abdominal discomfort. All variables were assessed by scores between 0 and 10, with 10 indicating the most severe degree of each variable. Any complications were also recorded. RESULTS: Between March 2002 and August 2003, 231 patients underwent transnasal EGD. The median age of the patients was 57 years (range 15 to 87 years). Complete examinations were possible in 98% of patients. Patients reported a high degree of acceptability (mean score 6.6, range 1 to 10) and low degrees of choking sensation (mean 1.8, range 0 to 10), nasal discomfort (mean 1.7, range 0 to 10), sore throat (mean 0.8, range 0 to 9) and abdominal discomfort (mean 1.1, range 0 to 10). The only complications reported by the patients were epistaxis (n=2, 0.9%) and sinusitis (n=1, 0.4%). Some patients also reported transient light-headedness (n=12, 5%) and mucous discharge (n=2, 0.9%). When asked, 185 patients (88%) stated that they were willing to undergo the same procedure in the future if medically indicated. Of the 84 patients who had conventional EGD under conscious sedation in the past, 52 patients (62%) preferred transnasal EGD without sedation. CONCLUSIONS: Transnasal EGD is generally well tolerated, feasible and safe. It can be performed with topical anesthesia in an outpatient setting. The low complication rate, high patient satisfaction and potential cost savings make transnasal endoscopy an attractive alternative to conventional EGD to screen patients for upper gastrointestinal tract diseases.

Reproducibility of wireless capsule endoscopy in the investigation of chronic obscure gastrointestinal bleeding.

BACKGROUND: Capsule endoscopy (CE) is a valuable tool in the diagnostic evaluation of obscure gastrointestinal bleeding, but limited information is available on the reproducibility of CE findings. OBJECTIVE: To compare two successive CE studies with push enteroscopy (PE) in patients presenting with chronic obscure gastrointestinal bleeding. METHODS: A prospective study was conducted. Ten patients (seven men and three women) with chronic obscure gastrointestinal bleeding and no contraindications for CE were eligible and completed the trial. For each patient, the first capsule was administered on day 1, the second capsule was administered on day 2 and PE was performed on day 3. Endoscopists were blinded to the capsule findings. Capsule findings were assessed independently by two investigators blinded to PE findings. RESULTS: A potential small intestinal bleeding source was found in 60% of the patients when all the studies were combined. A bleeding source was found in four patients in both CE studies. The second CE also identified a bleeding source in a fifth patient. Interobserver agreement by kappa analysis was 0.642 to 1.000 (P < or 05) for the CE studies. PE identified a potential small bowel bleeding site in four patients, including one patient who had negative CE studies. CONCLUSIONS: This study confirmed the reproducibility of CE findings on successive studies. Some patients did not have a source of bleeding in the small intestine, and all studies found this.

Precut papillotomy versus persistence in difficult biliary cannulation: a prospective randomized trial.

BACKGROUND AND STUDY AIMS: Failed biliary cannulation occurs in up to 10% of patients undergoing ERCP. There is some controversy as to the safety and efficacy of using precut techniques to achieve biliary cannulation in difficult cases. To date, no randomized trial has compared the success and complication rates of precut with the rates for persistence when biliary cannulation is difficult. The aim of this study was to compare the success rates and complication rates of precut with the success rates and complication rates of persistence in cases of difficult biliary cannulation. PATIENTS AND METHODS: Patients without prior sphincterotomy who required biliary cannulation were screened. A "difficult biliary cannulation" was arbitrarily defined as failed cannulation after 12 minutes. These patients were then randomized to continue treatment by needle-knife cut over the roof of the papilla or by persistence with a non-wire-guided, single-lumen papillotome. "Primary" success was defined as deep cannulation within 15 minutes of randomization. Primary and final success rates and complication rates within 30 days after ERCP were compared. RESULTS: Over a 38-month period a total of 642 patients were screened. Patients in whom biliary cannulation was successful within a time period of 12 minutes or less formed the reference group (n = 580). The remainder of the patients were randomly assigned to the "precut" arm (n = 32) or to the "persistence" arm (n = 30). Primary success rates and complication rates were similar in the precut and persistence arms (75% and 4% respectively for the precut arm vs. 73% and 9% for the persistence arm). The final successful cannulation rate in the entire group of 642 patients was 99.5%. CONCLUSIONS: In experienced hands, precut papillotomy and persistence in cannulation are equally effective in cases of difficult cannulation, with a similar complication rate.

Sphincterotomy-associated biliary strictures: features and endoscopic management.

BACKGROUND: "Sphincterotomy stenosis" is a recognized late complication of endoscopic biliary sphincterotomy. The narrowing is limited to the biliary orifice and can be managed simply by repeat sphincterotomy. A similar but poorly characterized post-sphincterotomy complication involves narrowing that extends from the biliary orifice for a variable distance along the bile duct, beyond the duodenal wall. This lesion cannot be managed by repeating the sphincterotomy. METHODS: Six patients (3 men) are described with sphincterotomy associated biliary strictures, all smooth and high grade, presenting at a median of 19 months (range 8 to 60 months) after sphincterotomy. Further sphincterotomy was not possible as an intra-duodenal segment of bile duct was no longer visible. Endoscopic management consisted of serial incremental stent exchange at 2- to 4-month intervals. The goal of therapy was to place two 11.5F stents side-by-side. RESULTS: Stricture resolution was documented by cholangiography in all patients. One patient with a stricture resistant to treatment required three 10F stents side-by-side, and another underwent treatment to a maximum of adjacent 11.5F and 7F stents. Two 11.5F stents were eventually placed in the other four patients. Overall median duration of stent placement was 12.5 months. At a median of 26.5 months of stent-free follow-up, all patients remain asymptomatic. CONCLUSION: Sphincterotomy-associated biliary strictures are a distinct late complication of biliary sphincterotomy. These recalcitrant lesions are not amenable to repeat sphincterotomy; however, the results of this study suggest that they may be managed successfully by serial placement of stents of incrementally increasing diameter.

A randomized, double-blind study of the use of droperidol for conscious sedation during therapeutic endoscopy in difficult to sedate patients.

BACKGROUND: Droperidol has been used in combination with narcotics and benzodiazepines to achieve conscious sedation. We performed a randomized, double-blind, study of droperidol in patients at risk for difficult sedation scheduled for therapeutic endoscopy. METHODS: Patients with regular ethanol, narcotic, or benzodiazepine usage, suspected sphincter of Oddi dysfunction, or a history of difficult sedation were eligible for the study. Patients were randomized to receive either droperidol or placebo along with midazolam and meperidine as preprocedure sedation. Time to achieve sedation, interruptions due to undersedation, medication dosages, recovery time, and subjective assessments of sedation were recorded. RESULTS: One hundred one patients were randomized. The droperidol group had significantly fewer procedure interruptions and observer ratings of difficulty with sedation and required significantly less midazolam (23%) and meperidine (16%) than the placebo group. There were no significant differences in time to achieve sedation, incomplete procedures, procedure length, recovery room time, or complications. There were significantly higher observer ratings of the quality of sedation for patients who received droperidol. CONCLUSIONS: Droperidol is a useful adjunct to conscious sedation in patients who are difficult to sedate. Its use results in significantly fewer interruptions due to poor sedation and improved sedation ratings compared with sedation using midazolam and meperidine alone.

Achalasia developing years after surgery for reflux disease: case reports, laparoscopic treatment, and review of achalasia syndromes following antireflux surgery.

Two case reports demonstrate the paradoxical occurrence of achalasia many years after the successful surgical treatment of gastroesophageal reflux disease (GERD). These patients had remedial surgery laparoscopically. The three types of achalasia syndromes that can follow antireflux surgery are discussed. In type 1, primary achalasia is misdiagnosed as GERD and inappropriate antireflux surgery causes worsening dysphagia immediately after surgery without any symptom-free interval. In type 2, secondary iatrogenic achalasia is seen early after antireflux surgery and is characterized by the presence of stenosis and scar formation at the site of the fundic wrap. Although the motility studies resemble achalasia, the repair needs only to be taken down and refashioned when there is no response to balloon dilatation. In type 3, illustrated by the case reports, primary achalasia follows antireflux surgery after a significant symptom-free interval. There is complete absence of any stenosis or fibrosis of the esophagus and periesophageal tissues at remedial surgery. Moreover, surgical treatment of this condition needs to include esophageal myotomy.

Same-day discharge after endoscopic biliary sphincterotomy: observations from a prospective multicenter complication study. The Multicenter Endoscopic Sphincterotomy (MESH) Study Group.

BACKGROUND: Same-day discharge after endoscopic biliary sphincterotomy (ES) is a common clinical practice, but there have been few data to guide appropriate selection of patients. Using a prospective, multicenter database of complications, we examined outcomes after same-day discharge as it was practiced by a variety of endoscopists and evaluated the ability of a multivariate risk factor analysis to predict which patients would require readmission for complications. METHODS: A 150-variable database was prospectively collected at time of ES, before discharge and again at 30 days in consecutive patients undergoing ES at 17 centers. Complications were defined by consensus criteria and included all specific adverse events directly or indirectly related to ES requiring more than 1 night of hospitalization. RESULTS: Six hundred fourteen (26%) of 2347 patients undergoing ES were discharged on the same day as the procedure, ranging from none at 6 centers to about 50% at 2 centers. After initial observation and release, readmission to the hospital for complications occurred in 35 (5.7%) of 614 same-day discharge patients (20 pancreatitis and 15 other complications, 3 severe). Of the same-day discharge patients, readmission was required for 14 (12.2%) of 115 who had at least one independently significant multivariate risk factor for overall complications (suspected sphincter of Oddi dysfunction, cirrhosis, difficult bile duct cannulation, precut sphincterotomy, or combined percutaneous-endoscopic procedure) versus 21 (4.2%) of 499 without a risk factor (odds ratio 3.1: 95% confidence interval [1.6, 6.3], p < 0.001). Of complications presenting within 24 hours after ES, only 44% presented within the first 2 hours, but 79% presented within 6 hours. CONCLUSIONS: Same-day discharge is widely utilized and relatively safe but results in a significant number of readmissions for complications. For patients at higher risk of complications, as indicated by the presence of at least one of five independent predictors, observation for 6 hours or overnight may reduce the need for readmission.

Outcome in patients with bifurcation tumors who undergo unilateral versus bilateral hepatic duct drainage.

BACKGROUND: There is much controversy as to the importance of establishing drainage of both liver lobes in malignant hilar obstruction. The purpose of the present study was to compare survival data in patients with malignant hilar obstruction, stratified according to the Bismuth classification, who had cholangiography with filling of one or both hepatic ducts and subsequently endoscopic or percutaneous drainage of one or both ducts. METHODS: A retrospective review was performed for the time period from July 1990 to July 1995, and 224 patients were identified with a presumed diagnosis of a bifurcation tumor. All x-ray films were reviewed and 150 patients finally diagnosed as hilar tumor were classified according to Bismuth type I, II, or III. Type II and III patients were further subclassified with respect to contrast injection into a single or both hepatic duct systems and whether one or both sides were eventually drained. RESULTS: Data were obtained in 141 patients (4 patients still alive); there were 43 type I, 58 type II, and 40 type III. Type II and III patients were divided into three groups: group A, one lobe opacified with same lobe drained; group B, both lobes opacified with both lobes drained; and group C, both lobes opacified with one lobe drained. Overall median survival for type I, II, and III patients was 160, 131, and 62 days, respectively. Among type II and III patients the median survivals of groups A, B, and C were 145, 225, and 46 days, respectively. Survival was significantly longer in group A vs. group C (p < 0.001), group B vs. group C (p < 0.001, and group A + B (165 days) vs. group C p < 0.001). There was no difference in group A + B versus type I (p=0.90). In addition, when comparing single drain only (group A + C, 80 days) versus double drains (group B, 225 days), there was a significant survival advantage (p < 0.0001). CONCLUSION: In bifurcation tumors the best survival was noted in those with bilateral drainage, and the worst survival in those with cholangiographic opacification of both lobes but drainage of only one.

Use of the 25 mm flanged esophageal Z stent for malignant dysphagia: a prospective multicenter trial.

BACKGROUND: An initial multicenter study using a 21 mm flanged esophageal Z stent demonstrated excellent palliation but an 11% immediate complication rate at placement and a 27% migration rate at 1 month. This North American multicenter trial prospectively studied a 25 mm flanged Z stent to define its palliative ability and whether the increased diameter affected placement or migration problems. METHODS: Fifty patients who had esophageal Z stents at seven university or regional referral hospitals were prospectively studied. Indications for prosthesis placement, previous therapy, patient demographics, incidence of concomitant tracheoesophageal fistula, and degree of dysphagia were defined, as were procedural and subsequent stent-related problems, survival times, the ability to occlude a tracheoesophageal fistula, and subsequent degree of dysphagia. RESULTS: Twenty-four patients had infiltrating malignancy (16 exophytic and 10 extrinsic), 9 of whom had concomitant tracheoesophageal fistulas. Ten patients (20%) had misplaced stents requiring retrieval and replacement, 12 patients (24%) had subsequent stent-related problems including exsanguination (2), aspiration (3), tumor overgrowth (3), and postplacement migration (4) (8%). There was statistically significant improvement in prestent versus poststent dysphagia and two thirds of patients had complete occlusion of their tracheoesophageal fistula. CONCLUSIONS: Redesign of the esophageal Z stent has decreased the migration rate without increasing placement or subsequent erosion problems. Its efficacy appears comparable to the currently marketed Z stent for the palliation of malignant dysphagia and occlusion of tracheoesophageal fistula.