Search

Background: Despite advancements in technology and expertise, ERCP carries risks of significant complications, such as pancreatitis, bleeding, and perforation. Post-ERCP pancreatitis is the most common and important complication following ERCP. In our study, we aimed to examine the relationship between patient and procedure-related parameters and the development of pancreatitis., Methods: Four hundred patients who underwent ERCP between January 1, 2019 and December 31, 2020, at the General Surgery Clinic of the Bakirköy Dr. Sadi Konuk Health Application and Research Center of the University of Health Sciences Faculty of Medicine were retrospectively evaluated. Patient and procedure-related factors were analyzed statistically through univariate and multivariate analyses., Results: Age, urgent indication, ERCP history, cholangitis, precut sphincterotomy, common bile duct diameter, pancreatic cannulation, pancreatic stent, and bleeding were statistically significant risk factors for post-ERCP pancreatitis (+). In multivariate analysis, the effects of urgent indication, ERCP history, cholangitis, precut sphincterotomy, common bile duct diameter, and pancreatic cannulation on post-ERCP pancreatitis were statistically significant risk factors ( P <0.05)., Discussion: This study demonstrates that emergency indication, ERCP history, cholangitis, precut sphincterotomy, and pancreatic cannulation are significant risk factors for the development of post- ERCP pancreatitis. One notable contribution of our study to the existing literature is the unique analysis of post-sphincterotomy bleeding as an independent factor., Competing Interests: The authors declare no conflict of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Rationale: A chronic autoimmune liver disease known as primary biliary cholangitis (PBC) that selectively destructs small intrahepatic biliary epithelial cells and may result in biliary cirrhosis and eventually liver transplantation or death. PBC is associated with various other extrahepatic autoimmune diseases; however, the combination of PBC with ankylosing spondylitis has been rarely reported in the literature. Here, we reported a case of PBC with ankylosing spondylitis to improve our understanding of such coexistence and provide new ideas for the treatment of such patients., Patient Concerns: A 54-year-old man was presented to the Department of Rheumatology because of an abnormal liver function test for 7 years, chest and back pain for 1 year, and low back pain for 2 months., Diagnoses: Primary biliary cholangitis, ankylosing spondylitis, and old pulmonary tuberculosis., Interventions: The patient refused to use nonsteroidal anti-inflammatory drugs, conventional synthetic disease-modifying antirheumatic drugs, and biologic disease-modifying antirheumatic drugs; thus, he was treated with methylenediphosphonate (99Tc-MDP) and ursodeoxycholic acid (UDCA)., Outcomes: The patient achieved remission with UDCA and 99Tc-MDP therapy., Lessons: In the treatment of PBC combined with other disorders, the characteristics of different diseases should be considered. The patient reported herein was treated with 99Tc-MDP and UDCA, and his condition improved; thus, we consider 99Tc-MDP to be an effective treatment. Furthermore, in line with the current understanding of the pathogenesis of PBC and ankylosing spondylitis, we hypothesize that interleukin-17 inhibitor is an effective treatment for such patients., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Rationale: Chronic graft versus host disease (cGVHD) is a systemic immune-mediated complication that occurs in approximately half of patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HCT), and remains the leading cause of late morbidity and mortality. cGVHD involves a heterogeneous group of organic manifestations, many of which mimic autoimmune diseases such as scleroderma, primary biliary cholangitis, Sjögren syndrome and polymyositis., Patient Concerns: A 60-years-old female with a history of allo-HCT developed de novo cGVHD 11 months after allo-HCT with isolated liver involvement. The patient presented with jaundice, cytolysis, cholestasis and concomitant acute digital ischemia. Liver biopsy and autoimmunity tests were performed and were found to be compatible with immune-mediated liver damage. Nailfold capillaroscopy revealed microangiopathy, characterized by avascular areas and some enlarged capillaries resembled an early systemic sclerosis pattern., Diagnosis: Biliary cholangitis-like and digital ischemia related to cGVHD., Interventions: The patient was treated with high-dose prednisone and ursodeoxycholic acid, and extracorporeal photopheresis. The patient required hospital admission for administration of intravenous prostacyclin due to refractory Raynaud syndrome., Outcomes: After 6 to 8 weeks, the patient achieved a good response, with evident clinical improvement and progressive normalization of liver function., Lessons: cGVHD is a multiorgan pathological condition, and this case emphasizes that a multidisciplinary team, including rheumatologists, should be involved in the follow-up of allo-transplant patients to ensure that the clinical complications are adequately addressed. Early intervention is critical for improving patient' prognosis.In addition, we performed a systemic literature review based on published case articles on hepatic cGVHD and digital ischemia published up to August 2022. To the best of our knowledge, this is the first reported case of such an association., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: The optimal timing for endoscopic retrograde cholangiopancreatography (ERCP) for acute cholangitis (AC) has not been unequivocally established., Aims: To perform a meta-analysis of the outcomes associated with particular timings of ERCP for AC., Methods: A systematic literature search was conducted for studies of ERCP for AC, and then a meta-analysis of the in-hospital mortality (IHM), 30-day mortality, and length of hospital stay (LHS) was performed., Results: Seven non-randomized studies of 88,562 patients were considered appropriate for inclusion. Compared with performing ERCP more than 24 hours after admission, ERCP within 24 hours was associated with lower IHM ( P <0.0004), but no difference in 30-day mortality ( P =0.38) was found between the 2 groups. ERCP performed <48 hours after admission was associated with a lower IHM and 30-day mortality ( P <0.00001 and P =0.03) than ERCP performed >48 hours after admission. In addition, ERCP performed within 24 or 48 hours was associated with a shorter LHS ( P <0.00001 and P <0.00001, respectively)., Conclusion: ERCP within 48 hours of admission is superior to subsequent ERCP with respect to IHM, 30-day mortality, and LHS, and ERCP performed within 24 hours is associated with lower IHM and LHS., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc.)

Background: The purpose of this prospective randomized study was to compare the nosocomial biliary tract infection rate of biliary stent implantation with a biliary stent loaded with radioactive 125 I seeds (radioactive biliary stent, RBS) and conventional biliary stent (CBS); additionally, to preliminary discuss the causes of postoperative cholangitis. Moreover, the results will provide clinical evidence for the prevention of postoperative biliary tract infection., Materials and Methods: We prospectively analyzed the nosocomial infection rate of the distal malignant biliary obstruction (MBO) treatment by stent implantation with RBS and CBS. All MBO patients who initially visited our tertiary hospital between July 2015 and December 2019 (n= 196) were evaluated, enrolled, and randomly divided into 2 groups, RBS (n=97) and CBS (n=99) group. χ 2 test was used to evaluate the categorical data, and t test was used to evaluate the numerical data., Results: Our analysis of the study showed the incidence of postoperative infections of a biliary tract of the RBS group (23.7%) was significantly higher than the CBS group (11.1%). The difference was statistically significant (χ 2 =5.425, P =0.020). Our study also showed the most common pathogenic bacteria after surgery was Escherichia coli (26.5%)., Conclusion: Treatment for distal MBO with biliary stent loaded with radioactive 125 I seeds had a higher nosocomial infection rate, and the most common pathogenic bacteria was E coli. , Supplemental Digital Content 1, http://links.lww.com/sle/A350., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc.)

Background: New hypoglycemic agents include sodium-glucose cotransporter-2 inhibitors (SGLT2is), glucagon-like peptide 1 receptor agonists (GLP1RAs), and dipeptidyl peptidase-4 inhibitors (DPP4is). The association between each class of these new hypoglycemic drugs and the risks of various digestive system diseases is unknown. We aimed to explore this relationship by performing a meta-analysis., Methods: We included large randomized trials of SGLT2is, GLP1RAs, and DPP4is. Outcomes of interest were 91 kinds of digestive diseases including 75 kinds of gastrointestinal disorders and 16 kinds of hepatobiliary disorders. Meta-analysis was done to generate pooled risk ratio (RR) and 95% confidence interval (CI). Subgroup analysis was conducted according to 3 different drug classes., Results: We included 21 large trials in this meta-analysis. Compared with placebo, GLP1RAs were associated with the higher risks of gastric ulcer hemorrhage (RR 2.68, 95% CI 1.07-6.68; Pdrug = .035; I2 = 0), pancreatitis (RR 1.48, 95% CI 1.02-2.15; Pdrug = .041; I2 = 0), cholangitis acute (RR 5.96, 95% CI 1.04-34.08; Pdrug = .045; I2 = 0), and cholecystitis acute (RR 1.52, 95% CI 1.08-2.15; Pdrug = .017; I2 = 1.5%), but were not significantly associated with the occurrences of the other 87 kinds of digestive diseases (Pdrug ranged from .064 to .999). SGLT2is versus placebo were not significantly associated with the occurrences of 91 kinds of digestive diseases (Pdrug ranged from .077 to .995). DPP4is versus placebo were not significantly associated with the occurrences of 91 kinds of digestive diseases (Pdrug ranged from .085 to .999)., Conclusions: Neither SGLT2is nor DPP4is are associated with the occurrences of various kinds of digestive diseases, whereas GLP1RAs are associated with the higher risks of 4 kinds of digestive diseases, namely, gastric ulcer hemorrhage, pancreatitis, cholangitis acute, and cholecystitis acute. These findings seem to suggest that GLP1RAs are not applicable for patients at high risk of 4 specific digestive diseases, whereas SGLT2is and DPP4is are safe for patients susceptible to digestive diseases. However, our findings require to be further verified by future studies with sufficient statistical power., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Abstract: Common bile duct (CBD) stone is a commonly encountered disease that is associated with various clinical presentations ranging from the mild form of biliary colic to the severe complication of acute cholangitis. Recently, diabetes mellitus (DM) has been linked to the development of biliary diseases; however, no data regarding the association of DM with acute cholangitis development in the setting of CBD stone exist. The aim of the current study was to investigate whether DM represents a risk factor for acute cholangitis in patients with CBD stone. We performed a retrospective cross-sectional study from January 1, 2010 till June 1, 2020 of all patients presenting to Galilee Medical Center with various clinical presentations of documented CBD stone, including cholangitis, biliary pancreatitis, and biliary colic with abnormal liver enzymes. Overall, 687 patients were included in the final analysis. Among them, 101 patients (14.7%) had CBD stone associated with acute cholangitis (group A), as compared to 586 patients (85.3%) without acute cholangitis (group B). The average ages in groups A and B were 77.7 ± 13.6 and 62.5 ± 20.5 years, respectively (P < .0001). The prevalence of DM was significantly higher in group A as compared to group B (52.5% vs 36.3%, P = .001). On univariate analysis, age (odds ratio [OR] 1.05, P < .0001), male gender (OR 1.54, P = .04), and DM (OR 1.92, P = .002) were associated with acute cholangitis development, and on multivariate logistic regression analysis, the correlation was preserved for DM (OR 1.93, 95% confidence interval 1.26-2.96, P = .002). DM showed a significant association with acute cholangitis development among patients with CBD stone. Identification of bile duct stones in diabetic patients is of paramount importance since early diagnosis and treatment might prevent further life-threatening complications., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Rationale: Intermittent combined pancreaticobiliary obstruction may lead to multiple episodes of ascending cholangitis and pancreatitis, usually due to choledocholithiasis or periampullary mass. However, one of the rare causes is periampullary or juxtapapillary duodenal diverticulum. Although duodenal diverticula are relatively common in the general population, the overwhelming majority are asymptomatic. Duodenal diverticula can cause combined pancreaticobiliary obstruction through multiple mechanisms such as stasis-induced primary choledocholithiasis, stasis-induced intradiverticular enterolith, or longstanding diverticulitis, causing stenosing fibrosing papillitis or a combination of more than one of these mechanisms. Herein, I report a case of Lemmel syndrome due to a combination of multiple mechanisms and review the available literature on the epidemiology, pathogenesis, clinical presentation, diagnostic work-up, and management of juxtapapillary duodenal diverticulum., Patient Concerns: Multiple episodes of abdominal pain, jaundice, anorexia, fever, and significant unintentional weight loss., Diagnoses and Interventions: Primary choledocholithiasis, recurrent ascending cholangitis, recurrent acute pancreatitis, and pancreatic atrophy due to giant juxtapapillary duodenal diverticulum, with unsuccessful endoscopic retrograde cholangiopancreatography that was completely resolved after open transduodenal sphincteroplasty and septoplasty, transampullary and transcystic common bile duct exploration and stone extraction, and duodenal diverticular inversion., Outcome: Complete resolution of combined pancreaticobiliary obstruction without recurrence for 2 years after surgery., Lessons: Surgeons should be aware of such rare syndromes to avoid misdiagnosis and delayed or inappropriate management. Furthermore, they should understand the different available operative options for cases that are refractory to endoscopic approach.

The aim of the study was to develop a new early noninvasive diagnostic model for primary biliary cholangitis (PBC).A total of 118 PBC patients who had undergone a liver biopsy were enrolled in the study, and were randomized into a model group (78 patients) and a validation group (40 patients). The patients' histological stages were based on the classifications of the Scheuer's stage. All common parameters and liver pathological results were analyzed. And total bile acid to platelet ratio, aspartate aminotransferase to platelet ratio index, fibrosis index based on 4 factors and red cell distribution width to platelet ratio were calculated.There were 106 (89.8%) women and 12 men in this study, and the number of patients in Scheuer stage I, II, III, and IV hepatic fibrosis was 52 (44.1%), 36 (30.5%), 26 (22.0%), and 4 (3.4%), respectively. The areas under the receiver operating characteristic curves of the total bile acid to platelet ratio (TPR), the aspartate aminotransferase to platelet ratio index, the fibrosis index based on 4 factors , and the red cell distribution width to platelet ratio for predicting advanced liver fibrosis were 0.771, 0.715, 0.618, and 0.517 respectively. The areas under the receiver operating characteristic curves of the TPR was higher than other non-invasive serological models.As a simple, inexpensive and easily accessible non-invasive liver fibrosis diagnostic model, the TPR may be a new noninvasive marker for predicting histologic severity of PBC.

Background: The aim of this study was to compare the clinical outcomes between patients with preoperative cholangitis and noncholangitis patients to determine whether the preoperative cholangitis would be able to serve as an independent predictive factor on hilar cholangiocarcinoma (HCC) outcomes., Methods: A systematic literature search for reported preoperative cholangitis in patients with hilar cholangiocarcinoma was performed in 4 databases: PubMed, Web of Science, Embase, and the Cochrane Library, published from 1979 to 2017., Results: In total, the initial search identified 1228 articles. Of these studies only 9 studies met the inclusion criteria and were included in this analysis. Differences between preoperative cholangitis existing and noncholangitis patients were observed in terms of mortality (RR = 2.29; 95% CI = 1.48-3.52; P = .0002), overall morbidity (RR = 1.15;95% CI = 1.00-1.32; P = .04), Liver failure (RR = 1.15;95% CI = 1.00-1.32; P = .04), Infection (RR = 1.52;95% CI = 1.16-2.00; P = .003), sepsis (RR = 2.40;95% CI = 1.25-4.5; P = .008)., Conclusions: The results lend support to the notion that in hilar cholangiocarcinoma patients, the existence of preoperative cholangitis is statistically associated with the higher postoperative mortality and morbidity. Also that it increases the risk of liver failure and infection. therefore, it is very important to properly control the preoperative cholangitis before surgery.

Rationale: Tigecycline is the first member of the glycylcycline family. There are rarely reports of tigecycline causing coagulopathy and hypofibrinogenemia until now. We report a case on tigecycline-associated coagulopathy and hypofibrinogenemia and discuss the characteristics of the adverse reaction., Patient Concerns: A 47-year-old male patient with severe acute cholangitis who developed sepsis was treated with a high dosage (100 mg twice daily) of tigecycline. He experienced coagulopathy and hypofibrinogenemia as substantiated by increased levels of prolonged prothrombin time (PT), the international normalized ratio (INR) and activated partial thromboplastin time (APTT), and in particular, the fibrinogen (FIB) levels obviously decreased., Diagnoses: Coagulopathy and hypofibrinogenemia., Interventions: We discontinued tigecycline and gave the patient several blood products to prevent spontaneous bleeding., Outcomes: The adverse reaction disappeared after the withdrawal of tigecycline. After 30 days of hospitalization, the patient discharged with symptom free., Lessons: We suggest that coagulation parameters should be closely monitored in patients treated with tigecycline, specifically in patients who may be renal insufficiency, female or use the high-dose.

Patient Concerns: A 63-year-old man was hospitalized with history of abdominal pain since more than 1 year, and that of fever with chills since 2 weeks., Diagnoses: Based on the laboratory investigations and radiologic findings, a preliminary diagnosis of pancreatic intraductal papillary mucinous neoplasm (IPMN) with recurrent cholangitis and severe jaundice was made., Interventions: An initial attempt at endoscopic and image-guided drainage proved unsuccessful. Due to cholangitis, liver dysfunction, and hypoalbuminemia, the patient was deemed to be medically unfit for radical surgery. Therefore we considered a novel strategy of associating pancreaticostomy and biliary-irrigation for staged pancreaticoduodenectomy (APBSP). In the first stage, biliary tract double irrigation (endoscopic nasobiliary drainage and T-tube) in combination with pancreaticostomy was performed, which alleviated the symptoms and helped improve the general condition of the patient. In the second stage, radical pancreaticoduodenectomy was performed., Outcomes: Over a follow-up period of 23 months, no recurrence occurred., Lessons: In this report, we present a previously unreported treatment strategy for pancreatic IPMN with recurrent cholangitis and jaundice. The innovative treatment approach may help advance the understanding and management of this condition., Competing Interests: The authors have no conflicts of interest to disclose.

Background: Gallbladder toxicity was reported in most motesanib studies with varying frequency and at variable times after initiation of treatment., Method and Results: A 44-year-old man was admitted due to severe epigastric pain. The patient was diagnosed with non-small cell lung cancer 9 months ago and received 6 cycles of chemotherapy with motesanib, paclitaxel, and carboplatin. Ultrasonography showed a large amount of sludge within gallbladder. Computed tomography scan demonstrated diffuse dilatation of biliary tree with distended gallbladder without evidence of stone and mild pancreatic swelling. Endoscopic retrograde cholangiopancreatography showed yellowish viscous mucoid plug impacting ampullary orifice and dilated bile duct with amorphous filling defect at distal half of common duct. Endoscopic sphincterotomy was performed to prevent biliary obstruction and recurrent pancreatitis after removal of mucoid material., Conclusion: To the best of our knowledge, this is the first report of obstructive cholangitis and acute pancreatitis associated with sludge formation during motesanib therapy. Endoscopic sphincterotomy appears to be useful to treat and prevent biliary obstruction caused by motesanib-induced biliary sludge.

Objectives: Clinically relevant postoperative pancreatic fistula (POPF) after pancreatoduodenectomy is often accompanied by bacterial infection. To elucidate the mechanism of bacterial infection associated with POPF, we investigated the relationship between POPF and bacteria isolated from ascitic fluid and removed drains., Methods: Subjects were 101 patients who had undergone pancreatoduodenectomy. Microbial culture was performed using ascitic fluid obtained from drains. We also compared the isolated bacteria from removed drains on postoperative day (POD) 4 and after POD 7., Results: In 23 patients (22.8%), microbial cultures were already positive on POD 1, although purulent discharge was not observed. Among patients with grade B/C POPF, bacteria were detected on POD 1 in 53.8%; these isolated bacteria were the same as those isolated after POPF formation. In contrast, only 7.7% of patients with grade A POPF were positive on POD 1. The number of bacteria isolated from drains removed after POD 7 significantly increased compared with those isolated from drains removed on POD 4., Conclusions: Bacterial contamination in ascitic fluid may be an initiating event that leads to the development of clinically relevant POPF. Therefore, it is important to perform both the administration of the appropriate antibiotics and early drain removal.

Objectives: The published natural history of congenital hepatic fibrosis (CHF) was examined to inform clinical decision making in autosomal recessive polycystic kidney disease (ARPKD)., Methods: A systematic literature search of the data on CHF, ARPKD, Caroli disease, Caroli syndrome, and type V choledochal cyst was performed to extract data related to portal hypertension, infection, malignancy, mortality, and transplantation., Results: Information related to 1230 patients with CHF was extracted from 155 articles. Median and mean age at diagnosis were 2 and 11.2 years, respectively. Median and mean time followed after diagnosis were 5.0 and 7.5 years, respectively (range 0-38 years). Sequelae of portal hypertension (n = 409), cholangitis (n = 152), and malignancy (n = 21) were noted. The nature of the portal hypertension was similar to that in other pediatric conditions (164 with varices, 74 bleeding varices, 81 underwent portosystemic shunting). Documented cholangitis was fatal in 3 of 23 children who were infected after renal transplantation. Twenty-one patients developed hepatobiliary cancer, with the majority having cholangiocarcinoma (n = 19). Cholangiocarcinoma (CCA) was predominant in individuals older than 40 years with either Caroli syndrome or isolated CHF, not ARPKD (median and mean age at CCA diagnosis were 70.3 and 60.1 years, respectively; range 33-75 years). There was a relative paucity of data on combined liver-kidney transplantation., Conclusions: Clinical decision making in ARPKD should reflect an understanding of the potential issues emanating from CHF. Accepted pediatric specific approaches to portal hypertension are warranted but must take into consideration the stage of renal insufficiency and potential plans for renal transplantation. Cholangitis is a major issue and necessitates anticipatory guidance and awareness. CCA, although a dreaded complication, does not appear to be a major issue during childhood. The indications for liver and combined liver-kidney transplantation are controversial and warrant further analysis.

Objective: The aim of this analysis was to report a multidisciplinary series comparing choledochoceles to Todani Types I, II, IV, and V choledochal cysts., Summary Background Data: Choledochoceles have been classified as Todani Type III choledochal cysts. However, most surgical series of choledochal cysts have reported few choledochoceles because they are managed primarily by endoscopists., Methods: Surgical, endoscopic, and radiologic records were reviewed at the Riley Children's Hospital and the Indiana University Hospitals to identify patients with choledochal cysts. Patient demographics, presenting symptoms, radiologic studies, associated abnormalities, surgical and endoscopic procedures as well as outcomes were reviewed., Results: A total of 146 patients with "choledochal cysts" including 45 children (31%) and 28 with choledochoceles (18%) were identified, which represents the largest Western series. Patients with choledochoceles were older (50.7 vs. 29.0 years, P < 0.05) and more likely to be male (43% vs. 19%, P < 0.05), to present with pancreatitis (48% vs. 24%, P < 0.05) rather than jaundice (11% vs. 30%, P < 0.05) or cholangitis (0% vs. 21%, P < 0.05), to have pancreas divisum (38% vs. 10%, P < 0.01), and to be managed with endoscopic therapy (79% vs. 17%, P < 0.01). Two patients with choledochoceles (7%) had pancreatic neoplasms., Conclusions: Patients with choledochoceles differ from patients with choledochal cysts with respect to age, gender, presentation, pancreatic ductal anatomy, and their management. The association between choledochoceles and pancreas divisum is a new observation. Therefore, we conclude that classifications of choledochal cysts should not include choledochoceles.

Objectives: Our objectives were to determine the clinical significance of transient arterial enhancement (TAE) of the hepatic parenchyma in patients with suspected biliary disease and to investigate the relationship between the pattern and extent of TAE and acute cholangitis., Methods: A retrospective review of dynamic computed tomographic scans in 76 patients with suspected biliary disease was performed. Patterns of TAE were classified as polymorphous, sectorial, peribiliary, diffuse, and a combination of features. The extent of TAE was evaluated by counting the involved liver segments. The extent of TAE was correlated with laboratory and clinical findings. Patients were also divided into 2 groups: cholangitis group (n = 40) and noncholangitis group (n = 36). The pattern and extent of TAE were compared between these 2 groups., Results: Among the laboratory findings that showed significant correlation with the extent of TAE, the white blood cell count and C-reactive protein concentration were found to have a relatively high correlation (r = 0.540 and 0.514, respectively). The presence of abdominal pain, fever, and cholangitis showed a statistically significant difference with the extent of TAE (P < 0.01). Statistically significant differences in the pattern and extent of TAE between the 2 groups were found (P < 0.001). Diffuse or diffuse heterogeneous TAE patterns that involve more than 5 liver segments, such as a polymorphous combined with a peribiliary pattern and a diffuse pattern, had a high association with acute cholangitis., Conclusions: Transient arterial enhancement in patients with suspected biliary disease is well correlated with clinical parameters suggestive of inflammation. Transient arterial enhancement provides useful complementary information for biliary infection in the computed tomographic diagnosis of acute cholangitis.

Background: Early endoscopic retrograde cholangiopancreatography (ERCP) should be performed in all patients with acute biliary pancreatitis (ABP) and coexisting acute cholangitis. In patients without cholangitis and predicted mild ABP it is generally accepted that early ERCP should not be performed. Nevertheless, there is a controversy regarding the role of early ERCP in the treatment of patients with predicted severe ABP without cholangitis. We reviewed randomized trials on early ERCP versus conservative management in patients with ABP without acute cholangitis., Methods: Relevant publications in 3 electronic databases (MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials) were systematically reviewed and meta-analyzed., Results: Seven randomized trials on ERCP in acute pancreatitis were found, of which 3 including a total of 450 patients (230 in the invasive arm and 220 in the control arm) qualified for a meta-analysis according to predefined criteria. In all patients with ABP (predicted mild and severe), early ERCP was associated with a nonsignificant reduction in overall complications [risk ratio (RR) 0.76; 95% confidence interval (CI) 0.41-1.04; P = 0.38] and a nonsignificant increase in mortality (RR 1.13; 95% CI 0.23-5.63; P = 0.88). Subgroup analysis based on predicted severity did not affect these outcomes (overall complications: predicted mild: RR 0.86; 95% CI 0.62-1.19; P = 0.36; predicted severe: RR 0.82; 95% CI 0.32-2.10; P = 0.68; mortality: predicted mild: RR 1.90; 95% CI 0.25-14.55; P = 0.53; predicted severe: RR 1.28; 95% CI 0.20-8.06; P = 0.80)., Conclusion: In this meta-analysis, early ERCP in patients with predicted mild and predicted severe ABP without acute cholangitis did not lead to a significant reduction in the risk of overall complications and mortality.

Objective: To analyze the short-term surgical outcome of hepatobiliary resections for perihilar cholangiocarcinoma in the last 5 years., Summary Background Data: Hepatobiliary resection for perihilar cholangiocarcinoma remains a technically demanding procedure, calling for a high level of expertise in biliary and hepatic surgery, and is still associated with significant morbidity or mortality., Methods: Between 2000 and 2004, we surgically treated 102 consecutive patients with perihilar cholangiocarcinoma with a management strategy consisting of preoperative biliary drainage, portal vein embolization (for right-sided and extended left-sided resections), and major hepatobiliary resection. The data on all of the patients were analyzed retrospectively to identify the factors that might significantly affect the postoperative mortality and morbidity., Results: There were no cases of in-hospital mortality or postoperative liver failure. Major complications were encountered in 7 patients (6.9%), and the overall morbidity rate was 50%. Reoperation was required in 2 patients (2%). The overall median length of postoperative hospital stay was 26 days (range, 13-119 days). Univariate analysis in relation to the postoperative morbidity showed significant differences in the preoperative occurrence of segmental cholangitis or cholecystitis (P = 0.015), the severity of postoperative hyperbilirubinemia (P < 0.001), and the total amount of fresh frozen plasma administered (P = 0.002). Multivariate analysis revealed a single independent significant predictive factor for postoperative morbidity, namely, preoperative cholangitis or cholecystitis (odds ratio, 9.08; 95% confidence interval, 1.05-78.56, P = 0.045)., Conclusions: Our experience indicates that hepatobiliary resections for perihilar cholangiocarcinoma can be conducted safely, without a single case of postoperative liver failure or mortality. Occurrence of preoperative cholangitis or cholecystitis is a significant indicator for morbidity of major hepatobiliary resection.

Esophageal perforation can occur during blind intubation with a side-viewing duodenoscope during endoscopic retrograde cholangiopancreatogram (ERCP) in patients with pharyngeal or esophageal anomalies. We describe a case of difficult intubation during an ERCP due to an asymptomatic and unsuspected pharyngeal pouch (Zenker's diverticulum). The side-viewing duodenoscope was withdrawn once resistance was encountered during intubation, and a forward-viewing gastroscope was inserted carefully under direct vision to evaluate the upper esophagus. After the diagnosis was made, intubation of the duodenoscope was performed by exchanging scopes over a guidewire. Subsequent ERCP with sphincterectomy and stone removal was uneventful. We caution that a side-viewing duodenoscope should be withdrawn once resistance is encountered during blind intubation during ERCP. Our technique minimizes patient discomfort and is rapid and easy to perform. In addition, no extra device such as an overtube is required.

Routine laparoscopic cholangiography and sonography have been recommended for identification of unsuspected bile duct stones in laparoscopic cholecystectomy. The increased prevalence of retained stones seems, however, to confirm that cholangiography has been used rather selectively. The aim of this study was to investigate the kind and extent of a possible correlation between inflammatory changes in the bile duct mucosa and the hepatoduodenal ligament in patients with and without unsuspected bile duct stones. Sixty-eight patients, without symptoms or signs of bile duct stones in their case histories, laboratory findings, and preoperative sonography results, underwent laparoscopic cholecystectomy for symptomatic gallstone disease and diagnostic transcystic cholangiography and cholangioscopy. The initial step at laparoscopy revealed edema of the periductal loose tissue, vascular dilation, and petechiae on the external surface of the distal portion of the extrahepatic bile duct in 47 of 68 patients. Cholangioscopy revealed inflammatory changes in the mucosa of the distal and sphincteric portions of the extrahepatic bile duct in 45 of 47 patients, and unsuspected ductal stones were identified in 41 of these 45 patients. Neither the external surface of the bile duct nor its mucosa exhibited any signs of inflammation in the 21 remaining patients (controls), all of whom showed stone-free bile ducts. Inflammatory changes in the bile duct mucosa, occurring along with unsuspected mobile ductal stones in this study, were reflected on the external surface of the bile duct with a specificity of 87%. The changes in the external surface of the bile duct can be recognized at the initial inspection in laparoscopic cholecystectomy, as in this study, and indicate that more accurate diagnostic procedures to identify unsuspected ductal stones should be used.

This study was performed to quantify the impact of Haldane effect (HE) on the relationship between O2 extraction (O2Ex; mL O2/dL blood) and venous pH in 247 measurements performed in 91 septic patients (73 patients with intra-abdominal sepsis, 11 with retroperitoneal abscesses, 6 with severe cholangitis, and 1 with gangrenous fasclitis). The severity of sepsis varied from relatively compensated to extremely diseased conditions. This allowed a detailed assessment of the impact of HE over a wide range of cardiorespiratory and metabolic abnormalities. A recently developed model was used to quantify blood CO2 exchange and Haldane relationships and, in particular, the buffering of venous pH allowed by O2Ex (O2-linked H+ binding) on the basis of arterial and mixed venous blood gas measurements. Arterio-venous pH difference (a-vDpH) was .033 +/- .024 (mean +/- SD). It increased with venoarterial CO2 concentration difference (v-aDCO2; mL CO2/dL blood), but the increase was moderated by a simultaneous increase in O2Ex, as the likely consequence of HE: a-vDpH = .006 + .017 (v-aDCO2) - .009 (O2Ex) [r2 = .96, p < < .001]. To confirm this, the moderation of a-vDpH allowed by the HE (DpH) was calculated. A first component, due to O2-linked H+ binding, had a value of .016 +/- .012, and a second component, due to the Haldane-mediated reduction in venous CO2 tension and plasma carbonic acid concentration, had a value of .019 +/- .006. Their sum (total DpH) was .033 +/- .017 and was related directly and strongly to O2Ex: DpH = -.002 + .009 (O2Ex) [r2 = .85, p < < .001], thus confirming the quantitative impact of HE in moderating the decrease in venous pH relative to arterial pH. The loss of this effect was responsible for the larger decreases in venous pH observed in hypodynamic patients developing impaired O2Ex. These results allow an easy quantification of the Haldane component, separated from the other components affecting pH, and are also useful for assessing the protective role exerted by HE against excessive decreases in venous pH in circulatory failure.

Objective: To determine more precisely the clinical and biological characteristics of AIDS-related cholangitis, and to investigate prognostic variables of this disease., Design: Retrospective clinical and prognostic study., Setting: Biliary unit, Bicêtre Hospital, France., Patients: HIV-positive patients (n = 52) referred to the unit between December 1986 and June 1993 for biliary symptoms leading to the suspicion of AIDS-related cholangitis, (42 men; 10 women; mean age, 37 +/- 8 years)., Intervention: Endoscopic retrograde cholangiopancreatography (ERCP) was performed in order to determine the cause of the biliary symptoms., Main Outcome Measure: Clinical features and evolution of the cholangitis., Results: Among the 52 patients, 45 met the ERCP criteria of AIDS-related cholangitis (36 men; nine women). The diagnosis of cholangitis was strongly suggested by abdominal ultrasonography in 47% of the cases. ERCP showed papillary stenosis, diffuse cholangitis, extrahepatic cholangitis alone, and intrahepatic cholangitis alone in 60, 67, 7 and 27%, respectively. Endoscopic sphincterotomy was performed in 28 patients. Pain was relieved by sphincterotomy in nine patients, but the other clinical or biological features were not influenced. One-year and 2-year survival rates were 41 +/- 7% and 8 +/- 4%, respectively. Multidimensional analysis using a Cox model showed that a lymphocyte count > 500 x 10(6)/l was the only independent predictive factor of better survival., Conclusion: AIDS-related cholangitis is a disease which leads preferentially to papillary stenosis or diffuse abnormalities of the biliary tract. Prognostic factors depend on the stage of the HIV infection. Another diagnosis of cholestasis was found in approximately 15% of the patients who showed biliary symptoms.

Emergency surgery for patients with severe acute cholangitis carries formidable postoperative morbidity and mortality rates. A retrospective study was conducted on 86 consecutive patients who had exploration for the calculous obstructions to identify the high-risk population to guide better management. Septicemic shock was present in 55 patients before surgery. All patients had ductal exploration under general anesthesia. Additional procedures included cholecystectomy (n = 55), cholecystostomy (n = 5), and transhepatic intubation (n = 2). Complications and deaths occurred in 43 (50%) and 17 (20%) patients, respectively. Multivariate analysis on the 25 clinical (n = 14) and biochemical (n = 11) parameters evaluated yield the following five predictive factors (relative risk): the presence of concomitant medical problems (4.5); pH less than 7.4 (3.5); total bilirubin more than 90 mumol/l (3.1); platelet less than 150 x 10(9)/l (2.9), and serum albumin less than 30 g/L (2.9). In the presence of three or more albumin less than 30 g/L (2.9). In the presence of three or more risk factors, postoperative morbidity and mortality rates were 91% and 55%, respectively, which were significantly higher than those with two or less risk factors (34% and 6%, respectively). As thrombocytopenia developed even with transient hypotension, timely ductal decompression would improve outcome of these patients after surgery. For the high-risk population, application of nonoperative biliary drainage might be considered.

Pyogenic liver abscesses in 106 adult patients at The New York Hospital were reviewed to define optimum treatment. Mortality in the surgically treated patients was 26% (17/65), while those treated nonsurgically had a fatality rate of 95% (39/41). Multiple abscesses treated surgically had a surprisingly low mortality of 29% (5/17). Modern noninvasive tests are highly sensitive in diagnosing liver lesions greater than 2 cm. Difficulty remains in identifying small hepatic abscesses and differentiating large abscesses from tumor. Most liver abscesses have an identifiable source outside the liver. The most common source (31%) was cholangitis secondary to extrahepatic biliary obstruction. Multiple abscesses, mixed organisms, hyperbilirubinemia, and abscess complications are all associated with a significantly increased mortality. However, the lethality of the primary disease process was the most important factor determining survival. Most patients who have the underlying pathogenesis of the abscess controlled will survive surgical treatment. Transperitoneal surgical drainage and antibiotics remain the mainstay of treatment. Percutaneous drainage is recommended for high risk patients only.

Forty-six necropsy cases of recurrent pyogenic cholangitis (RPC) among Chinese in Hong Kong were reviewed. Intrahepatic biliary pigment stones or bile sludge were present in all cases and liver abscesses were common (80%). Histologically the early lesion was marked by acute inflammation in portal tracts with frequent pylethrombophlebitis and the advanced chronic lesion by periductal fibrosis. Most patients died from chronic persistent hepatic suppuration or disseminated infection. The pathogenesis is not fully understood; the present study favours the view that a protein-deficient diet initiates intrahepatic stone or sludge formation with biliary obstruction; subsequent bacterial infection probably originates from the portal system.

Traditional management of patients with primary sclerosing cholangitis (PSC) has included steroids and/or choledochotomy plus prolonged T-tube drainage. The authors have not been convinced, however, that either of these therapies significantly influence the course of the disease. Therefore, a more aggressive surgical approach was adopted that included performance of a choledochoenteric anastomosis in all patients with PSC who had either (1) a major area of extrahepatic blockage, or (2) primary involvement of the extrahepatic bile ducts. Using this approach, 17 of 22 patients (77%) managed surgically at the UCLA Medical Center from 1974 through 1980 have undergone a choledochoenteric anastomosis. Of these 17 patients, 13 (77%) have had an excellent or good result following surgery. Four patients whose disease was confined to the common bile duct have all had excellent results (mean follow-up 49.3 months since surgery). In addition, 18 of the entire group of 22 patients (82%) are still alive, a mean of 52.2 months after operation and 64.7 months following establishment of the diagnosis. These results in 22 patients encourage the authors to continue to pursue an aggressive surgical approach for patients with primary sclerosing cholangitis.

The clinical course and management of 40 patients who underwent operation for acute cholecystitis developing as a postoperative complication were reviewed. Of note was the mortality of 47%, the high incidence of gangrene, perforation, empyema, and cholangitis, and the atypical clinical presentation of acute cholecystitis under these conditions. Awareness of this possible complication, knowledge of its clinical features, and early surgical intervention are important facets of successful management.

In order to identify risk factors in patients with acute cholangitis, 140 clinical, biochemical, etiologic, and pathologic variables of 449 attacks of acute cholangitis seen in one center over a 20-year period were analyzed. Simple regression revealed 24 factors with prognostic significance, but multivariate analysis detected only seven factors with independent significance in predicting mortality (acute renal failure, cholangitis associated with liver abscesses or liver cirrhosis, cholangitis secondary to high malignant biliary strictures or after percutaneous transhepatic cholangiography, female gender, and age). When the presence of each of these factors is weighted proportional to its coefficient of regression, patients with cholangitis could be scored on a scale of 0-27. A score of seven was clinically the most useful cut off--388 attacks of cholangitis associated with a score of less than 7 had a mortality rate of only 1.8%, whereas 61 attacks associated with a score greater than or equal to 7 had a mortality rate of 49%. The value of this scoring system needs to be confirmed in prospective studies, but it may prove useful, for example, in selecting a group of high-risk patients for urgent biliary decompression in an attempt to reduce the mortality associated with this pathology.

Profuse hemorrhage into the biliary tract--major hemobilia,--is an alarming condition which attracts much attention. Minor hemobilia, often caused by gallstones or operative injury, is much more frequent, yet often neglected. Clinical observations indicate that minor hemobilia is not always an innocent condition with the blood remaining fluid and unobtrusively flowing into the intestine. Examples are given where clots from occult hemobilia have caused diagnostic errors or obstructed the bile flow, thus imitating gallstones for which they may be mistaken. Experiments have been performed to elucidate the pathophysiology of this clot formation: 1) A model of the biliary tract was constructed with bile flowing through it. When blood was injected forcefully to simulate a major hemorrhage, mixed clots of blood and bile were formed. When introduced gently, as in minor hemobilia, the blood flowed immiscibly to the lowest level where it formed a clot of pure blood. The clots dissolved under the influence of flowing bile, but remained intact when protected from the flow. 2) Mixed clots were mushy and dissolved spontaneously, while pure clots remained solid and stable. When clots containing increasing amounts of bile were incubated, increasing amounts of cleavage products of fibrinogen and fibrin were formed. 3) Blood clots were produced in gallbladders of 33 dogs. Pure clots remained solid and floating while mixed clots formed a mushy layer, strongly adherent to the mucosa. Both kinds of clots disappeared after two-four weeks, but remained if bile flow was diverted. These findings explain why under certain circumstances minor hemobilia acquires clinical significance by forming clots that may obstruct the flow or cause diagnostic errors. Successful dissolution of "retained stones" may occasionally have the simple explanation that blood clots, mistaken for calculi, have been fibrinolysed. Consequently, in biliary obstruction or when defects are found on cholangiography, the possibility of blood clots in the ducts should be considered even in the absence of overt gastrointestinal hemorrhage.

Thirty patients were identified as having primary common duct stones. Criteria for diagnosis included at least a two year symptom free interval following cholecystectomy; soft, light brown stones or sludge present in the common duct; and the absence of a long cystic duct remnant or a biliary stricture from the previous surgery. The average age of the 30 patients was 66 years. The interval between cholecystectomy and the diagnosis of primary common duct stones averaged 12 years. Acute cholangitis was a frequent mode of presentation. At the time of surgery the bile duct was often dilated out of proportion to the serum bilirubin. In only one of the 30 patients was ampullary stenosis present. Twenty-six of the 30 patients had only stone extraction and insertion of a T tube for treatment of their primary common duct stones. Twenty-two of the 26 were followed for an average of four years and nine months with no evidence of recurrent stones in 82% (18/22). Four developed recurrent primary common duct stones one, five, five, and 7 years later. It is concluded that most patients with primary common duct stones do well after stone extraction alone.

Since 1974, 16 consecutive infants with biliary atresia have been treated by hepatic portoenterostomy employing an exteriorized Roux-en-Y intestinal segment (Miluliez). Simultaneous, sequential analyses of bile pigments and lipids in serum and biliary drainage were performed. In the 11 patients with sustained bile drainage, progressive increases in bile volume, bilirubin and biliary lipid concentrations correlated well with their subsequent return toward normal in the serum. Despite relief of biliary obstruction, four patients have had progressive liver cirrhosis. The other 7 have residual liver damage which has been stable, or in two instances, improved, at late biopsy. The clinical and biochemical results suggest that both obstructive and parenchymal factors are operative in infants with biliary atresia.

Two patients with primary sclerosing cholangitis developed perforating lesions in the skin of the extremities. Clinically and histologically, the cutaneous condition showed the features of perforating folliculitis with superimposed lesions of prurigo nodularis. The course of the eruption paralleled the severity of the biliary disease. A theory of pathogenesis for the development of the perforating folliculitis in these patients is suggested. To the best of our knowledge, this is the first report of the coexistence of both conditions.

Although gallbladder hydrops occurs frequently in patients with mucocutaneous lymph node syndrome (MCLS), the etiology of the gallbladder lesion is unclear. Recently we performed a liver biopsy on a child with severe MCLS and demonstrated selective invasion of biliary ductular epithelial cells by polymorphonuclear leukocytes with sparing of the hepatocytes. The possible role of the selective destruction of biliary cells in the pathogenesis of biliary disease in MCLS is discussed.

We present two cases, a 32-year-old mother and her 10-year-old son, both of whom had ulcerative colitis and sclerosing cholangitis. The mother had radiologic as well as pathologic evidence of both diseases, while the child's disease was demonstrated pathologically. A review of the literature indicates the rarity of sclerosing cholangitis in childhood, with no reported instances of a parent-child affliction. The hypothesis that there is a genetic component to the development of sclerosing cholangitis may be supported by this family.

Obstructive cholangitis with acute renal failure is a dramatic syndrome which merits individual definition. Twenty-one patients with acute suppurative cholangitis complicated by rapidly developing renal insufficiency were studied, and the severity of the renal failure, an acute interstitial tubulopathy, bore no significant relationship to the serum bilirubin level. The mechanism of renal damage was clearly related to episodes of septicemia. Increasing experience has modified the approach to treatment. The dominant septic problem can often be controlled by vigorous antibiotic and fluid therapy, allowing time for spontaneous improvements in renal function. All patients thus operated at a distance from the septic episode survived. If emergency operation is required because of persistent or recrudescnet sepsis, the necessity for dialysis should be considered first; the circumstances demanding dialysis are defined. The priorities in therapy are then: 1) treatment of the infection, 2) treatment of the renal failure, and finally 3) operation. The amount of the operation depends on the evolution of the sepsis, but should be preceded by dialysis when required.

The clinical, radiologic and pathologic features of 37 patients diagnosed as having primary sclerosing cholangitis (PSC) were reviewed. Sixty-two per cent were men, and 35% had ulcerative colitis. The patients demonstrated considerable variability in their natural history and pathology. It appeared that they could be divided into four fairly distant groups (1) sclerosing cholangitis affecting primarily the distal common bile duct; (2) sclerosing cholangitis occurring soon after an attack of acute necrotizing cholangitis; (3) chronic diffuse sclerosing cholangitis; and (4) chronic diffuse sclerosing cholangitis associated with inflammatory bowel disease. It is suggested that the patients in these groups may have different etiologies, may respond to different treatment regimes, and may have different prognoses.

The cholangiograms of 36 patients with sclerosing cholangitis were reviewed. The mean age of the patient group was 43 years, and the mean disease duration was 4.5 years. Seventeen of the patients had associated inflammatory bowel disease. The mean serum bilirubin was 6.8 mg/dl, the mean SGOT was 105 IU/L, the mean SGPT was 108 IU/L, and the mean serum alkaline phosphatase was 534 IU/L. The cholangiograms demonstrated involvement of the extrahepatic bile ducts in 33 patients, involvement of the hepatic duct bifurcation in 33 patients, and involvement of the intrahepatic bile ducts in 35 patients. The cholangiograms were graded as to the areas of the most severe obstructive involvement. In 24 patients the area of most severe involvement was the hepatic duct bifurcation. In eight additional patients the hepatic duct bifurcation, along with the extrahepatic ducts and/or the intrahepatic ducts, were felt to be the areas most severely affected. This predilection for severe obstructive disease at the hepatic duct bifurcation in sclerosing cholangitis held for both patients with and without inflammatory bowel disease. Thus, most patients with sclerosing cholangitis have cholangiographic evidence of diffuse extrahepatic and intrahepatic biliary tract disease, with the hepatic duct bifurcation being the area generally most severely affected.