Cholestatic pruritus is a debilitating symptom associated with many liver diseases and is often refractory to medical management. Nasobiliary drainage is a relatively safe and effective method for treating intractable cholestatic pruritus. It should be considered for patients with refractory cholestatic pruritus who have failed or have contraindications to medical therapy as a predictor of response before surgical fistula creation.
Pruritus is a debilitating symptom that affects up to 80% of patients with acute and chronic cholestatic liver disease.1 It is caused by an increase in enterohepatic circulation of pruritogens including bile acids, which activate a complex neural network.2 Cholestatic pruritus is typically treated with 4 classes of medications that target different components of this neural pathway: anion exchange resins (cholestyramine), enzyme inducers (rifampin), opioid antagonists (naltrexone), and sertraline.1 However, medications are often ineffective and many patients trial other therapeutic modalities including ultraviolet therapy, plasmapheresis, extracorporeal albumin dialysis, ileal bile acid transporter inhibitors, and biliary removal via nasobiliary drainage (NBD) or external biliary diversion. Liver transplant is the last resort for severe symptoms.2,3
NBD via endoscopic retrograde cholangiopancreatography (ERCP) is a method of relieving pruritus by reducing circulating pruritogens through trans-nasal drainage of bile.4 We present refractory cholestatic pruritus secondary to congenital hepatic fibrosis in a patient with Joubert syndrome (JS) that used preoperative NBD as a predictor of success before surgical fistula creation.
by
Shayan S. Irani;
Neil R. Sharma;
Andrew C. Storm;
Raj J. Shah;
Prabhleen Chahal;
Field Willingham;
Lee Swanstrom;
Todd H. Baron;
Eran Shlomovitz;
Richard A. Kozarek;
Joyce A. Peetermans;
Edmund McMullen;
Evelyne Ho;
Schalk W. van der Merwe
Objective: To evaluate the safety and efficacy of endoscopic ultrasound-guided gallbladder drainage (EUS-GBD) using a lumen-apposing metal stent (LAMS). Background: For patients with acute cholecystitis who are poor surgical candidates, EUS-GBD using a LAMS is an important treatment alternative to percutaneous gallbladder drainage. Methods: We conducted a regulatory-compliant, prospective multicenter trial at 7 tertiary referral centers in the United States of America and Belgium. Thirty consecutive patients with mild or moderate acute cholecystitis who were not candidates for cholecystectomy were enrolled between September 2019 and August 2021. Eligible patients had a LAMS placed transmurally with 30 to 60-day indwell if removal was clinically indicated, and 30-day follow-up post-LAMS removal. Endpoints included days until acute cholecystitis resolution, reintervention rate, acute cholecystitis recurrence rate, and procedure-related adverse events (AEs). Results: Technical success was 93.3% (28/30) for LAMS placement and 100% for LAMS removal in 19 patients for whom removal was attempted. Five (16.7%) patients required reintervention. Mean time to acute cholecystitis resolution was 1.6±1.5 days. Acute cholecystitis symptoms recurred in 10.0% (3/30) after LAMS removal. Five (16.7%) patients died from unrelated causes. Procedure-related AEs were reported to the FDA in 30.0% (9/30) of patients, including one fatal event 21 days after LAMS removal; however, no AEs were causally related to the LAMS. Conclusions: For selected patients with acute cholecystitis who are at elevated surgical risk, EUS-GBD with LAMS is an alternative to percutaneous gallbladder drainage. It has high technical and clinical success, with low recurrence and an acceptable AE rate. Clinicaltrials.gov, Number: NCT03767881.
Background and study aims: Peroral endoscopic myotomy (POEM) is a time-consuming and challenging procedure. Traditionally, the myotomy is done after the submucosal tunnel has been completed. Starting the myotomy earlier, after submucosal tunneling is half completed (concurrent myotomy and tunneling), may be more efficient. This study aims to assess if the method of concurrent myotomy and tunneling may decrease the procedural time and be efficacious.
Patients and methods: This is a retrospective case series of patients who underwent modified POEM (concurrent myotomy and tunneling) or traditional POEM at a tertiary care medical center. Modified POEM or traditional POEM was performed at the discretion of the endoscopist in patients presenting with achalasia. The total procedural duration, myotomy duration, myotomy length, and time per unit length of myotomy were recorded for both modified and traditional POEM.
Results: Modified POEM was performed in 6 patients whose mean age (± standard deviation [SD]) was 58 ± 13.3 years. Of these, 5 patients had type II achalasia and 1 patient had esophageal dysmotility. The mean Eckardt score (± SD) before the procedure was 8.8 ± 1.3. The modified technique was performed in 47 ± 8 minutes, with 6 ± 1 minutes required per centimeter of myotomy and 3 ± 1 minutes required per centimeter of submucosal space. The Eckardt score was 3 ± 1.1 at 1 month and 3 ± 2.5 at 3 months. The procedure time for modified POEM was significantly shorter than that for traditional POEM.
Conclusions: Modified POEM with short submucosal tunneling may be more efficient than traditional POEM with long submucosal tunneling, and outcomes may be equivalent over short-term follow-up. Long-term data and randomized controlled studies are needed to compare the clinical efficacy of modified POEM with that of the traditional method.
OBJECTIVES: To evaluate the effect of solid pancreatic masses on the pancreatic duct (PD) at the endoscopic ultrasound (EUS) and the relationship of the location/size of a mass and PD dilation.
MATERIALS AND METHODS: Patients who underwent EUS for pancreatic indications from 2011 to 2013 at a single center were retrospectively identified. Those with biopsies that revealed adenocarcinoma or neuroendocrine tumors in the pancreas were identified and PD size was ascertained from EUS, computed tomography, or magnetic resonance imaging.
RESULTS: Of the 475 patients who had a pancreatic EUS, 239 had a dilated PD and 236 had a normal PD. Patients with a dilated PD had a significantly higher incidence of pancreatic malignancy than those with a normal PD diameter (106/239, 44.4% vs. 32/236, 13.6%, P< 0.001). Of the 138 patients with a pancreatic malignancy, 106 (76.8%) had a dilated PD at some location in the pancreas. Over 80% of patients with a mass within the head, neck, or body had a dilated PD. For a mass located at the uncinate process or the tail, PD dilation was 65% and 23%, respectively. Fifty-six (80.0%) of the masses in the head, 11 (78.6%) masses in the neck, and 16 (76.2%) masses in the body had a dilated PD upstream of the mass. In addition, a step-wise increase in the incidence of PD dilation was correlated with an increase in mass size. About 67.6% of patients with masses measuring in the 1st quartile had dilated a PD, while 77.8%, 91.0%, and 71.4% of those with masses measuring in the 2nd, 3rd, and 4th quartiles, respectively, had a dilated PD.
CONCLUSION: PD dilation is a warning sign for pancreatic malignancies, however, small masses or masses at the uncinate process or the tail of the pancreas may not affect the size of the PD.
Esophageal cancer has a poor overall prognosis and is frequently diagnosed at a late stage. Conventional treatment for metastatic esophageal cancer involves chemotherapy and radiation. Local disease control plays a significant role in improving survival. Endoscopic spray cryotherapy is a novel modality that involves freezing and thawing to produce local ablation of malignant tissue via ischemic mechanisms. Spray cryotherapy has been shown to be effective, particularly for early T-stage, superficial esophageal adenocarcinomas. We present the case of a 72-year-old-male with locally recurrent stage IV esophageal adenocarcinoma and long-term survival of 7 years to date, with concurrent chemoradiation and serial cryoablation. He remains asymptomatic and continues to undergo chemotherapy and sequential cryoablation. The findings highlight the long-term safety and efficacy of cryotherapy in combination with chemoradiation, and suggest that cryoablation may have an additive role in the treatment of advanced stage esophageal adenocarcinoma.
Background and Aims: Patients with chronic constipation or motility disorders may be referred for rectal suction biopsy (RSB) to rule out Hirschsprung's disease (HD). RSB may not be successful beyond infancy because of the increased thickness of the rectal mucosa. EMR could improve the diagnostic yield for HD when compared with traditional RSB because larger and deeper samples are acquired for analysis. Methods: In this prospective, single-center study, patients referred for RSB were offered enrollment for concurrent EMR. Specimens were analyzed pathologically for size, submucosal ganglionic tissue, and acetylcholinesterase or calretinin staining. Biopsy results were compared with transit studies, anorectal manometry, and constipation severity through validated questionnaires. Results: Seventeen patients (2 male, 15 female; mean age, 35.8 years; range, 22-61 years) were enrolled in the study from 2008 to 2014. All patients underwent anorectal manometry (88% with anorectal dysfunction, 68% with outlet obstruction) and transit studies (41% with delayed transit). There were no reports of adverse events from the RSB and EMR procedures. The RSB sample volumes were significantly lower than the EMR sample volumes (0.023 cm3 vs 0.26 cm3, P =.001). There was diagnostic tissue for submucosal visualization by RSB in 53% (9/17) of cases compared with 100% (17/17) with EMR (P =.003). No cases of HD were diagnosed by RSB; one patient had rare ganglions observed by EMR. Conclusions: EMR provides greater tissue volume and can improve the characterization of ganglion cells in rectal tissue compared with RSB in patients with moderate to severe constipation with suspected HD.
Natural orifice translumenal endoscopic surgery (NOTES) had its origins in numerous small animal studies primarily examining safety and feasibility. In human trials, safety and feasibility remain at the forefront; however, additional logistic, practical, and regulatory requirements must be addressed. The purpose of this paper is to evaluate and summarize published studies to date of NOTES in humans. The literature review was performed using PUBMED and MEDLINE databases. Articles published in human populations between 2007 and 2011 were evaluated. A review of this time period resulted in 48 studies describing procedures in 916 patients. Transcolonic and transvesicular procedures were excluded. The most common procedure was cholecystectomy (682, 75%). The most common approach was transvaginal (721, 79%). 424 procedures (46%) were pure NOTES and 491 (54%) were hybrid NOTES cases. 127 (14%) were performed in the United States of America and 789 (86%) were performed internationally. Since 2007, there has been major development in NOTES in human populations. A preponderance of published NOTES procedures were performed internationally. With further development, NOTES may make less invasive surgery available to a larger human population.
Objectives: Tobacco exposure is an established risk factor for pancreatic cancer and chronic pancreatitis; however, its role in pancreatic insufficiency is not clear. Methods: This controlled, cross-sectional study examined smokers and nonsmokers with no history of pancreatic disease. Histories and validated inventories of alcohol and tobacco use were obtained, and pancreatic insufficiency was assessed using the fecal elastase-1 assay. Results: Of 7854 patients approached, 226 were interviewed and 200 enrolled. The rates of pancreatic insufficiency [18% (18/100)] and severe pancreatic insufficiency [10% (10/100)] were significantly higher in smokers than in controls [6% (6/100), P = 0.009 and 1% (1/100), P = 0.010, respectively]. On multivariate logistic regression, the risk of pancreatic insufficiency in smokers was significantly increased [odds ratio, 4.34 (1.37-13.75); P = 0.012], controlling for alcohol use and relevant covariates. Tobacco exposure was associated with the highest odds ratio for pancreatic insufficiency. Alcohol consumption was strongly associated with tobacco exposure (P < 0.001), but not with pancreatic insufficiency by multivariate analysis (P = 0.792). Conclusions: This study suggests that tobacco exposure is independently associated with pancreatic exocrine insufficiency in patients without a prior diagnosis of pancreatic disease. Tobacco exposure seems to have greater detrimental effects on pancreatic function than alcohol in this population.
Pancreatic cyst detection is increasing largely due to increasing use of cross-sectional imaging. The management of pancreatic cysts differs for true cysts, pseudocysts, mucinous cysts, nonmucinous cysts, and malignant lesions. Depending on the setting, diagnostic tests, such as cross-sectional imaging, endoscopic ultrasound, cyst fluid chemistry, and cytology, have moderate accuracy in characterizing the cyst subtype. Molecular analysis of cyst fluid aspirates has shown promise in preliminary studies and may require smaller fluid volumes than is needed for carcinoembryonic antigen level and cytology. This article reviews published studies in which molecular analysis was performed in the evaluation of pancreatic cysts. The molecular studies are compared with the conventional tests. Most studies have had moderate sample sizes (16-124) and have characterized a high proportion of patients with malignant cysts. Evaluation of molecular analysis as a diagnostic tool merits larger prospective trials with long-term follow-up of patients who are not sent to surgery. Larger cysts may meet size criteria for resection, and it is the smaller cysts for which molecular analysis may be of benefit if additional molecular testing results in a change in management.
Background/Aims: There are conflicting opinions regarding the management of recurrent acute pancreatitis (RAP). While some physicians recommend endoscopic retrograde cholangiopancreatography (ERCP) in this setting, others consider it to be contraindicated in patients with RAP. The aim of this study was to assess the practice patterns and clinical features influencing the management of RAP in the US. Methods: An anonymous 35-question survey instrument was developed and refined through multiple iterations, and its use was approved by our Institutional Review Board. The survey was distributed via email to 408 gastroenterologists to assess the practice patterns in the management of RAP in multiple clinical scenarios. Results: The survey was completed by 65 participants representing 36 of the top academic/tertiary care centers across the country. Approximately 90.8% of the participants indicated that they might offer or recommend ERCP in the management of RAP. Multinomial logistic regression analysis revealed that ductal dilatation and presence of symptoms were the most predictive variables (p<0.001) for offering ERCP. Conclusions: A preponderance of the respondents would consider ERCP among patients with RAP presenting to tertiary care centers in the US. Ductal dilatation, presence of symptoms, and pancreas divisum significantly increased the likelihood of a recommendation for ERCP.