What are the new contents of the guideline since 2010?A.Patients with primary and non-primary sclerosing cholangitis (PSC) are included in these guidelines for the diagnosis and management of cholangiocarcinoma.B.Define "related stricture" as any biliary or hepatic duct stricture accompanied by the signs or symptoms of obstructive cholestasis and/or bacterial cholangitis.C.Patients who have had an inconclusive report from MRI and cholangiopancreatography should be reexamined by high-quality MRI/cholangiopancreatography for diagnostic purposes. Endoscopic retrograde cholangiopancreatography should be avoided for the diagnosis of PSC.D. Patients with PSC and unknown inflammatory bowel disease (IBD) should undergo diagnostic colonoscopic histological sampling, with follow-up examination every five years until IBD is detected.E. PSC patients with IBD should begin colon cancer monitoring at 15 years of age.F. Individual incidence rates should be interpreted with caution when using the new clinical risk tool for PSC for risk stratification.G. All patients with PSC should be considered for clinical trials; however, if ursodeoxycholic acid (13-23 mg/kg/day) is well tolerated and after 12 months of treatment, alkaline phosphatase (γ- Glutamyltransferase in children) and/or symptoms are significantly improved, it can be considered to continue to be used.H. Endoscopic retrograde cholangiopancreatography with cholangiocytology brushing and fluorescence in situ hybridization analysis should be performed on all patients suspected of having hilar or distal cholangiocarcinoma.I.Patients with PSC and recurrent cholangitis are now included in the new unified network organ sharing policy for the end-stage liver disease model standard.J. Liver transplantation is recommended after neoadjuvant therapy for patients with unresectable hilar cholangiocarcinoma with diameter < 3 cm or combined with PSC and no intrahepatic (extrahepatic) metastases.
Child ; Humans ; Cholangitis, Sclerosing/diagnosis* ; Constriction, Pathologic/complications* ; In Situ Hybridization, Fluorescence ; Cholangiocarcinoma/therapy* ; Liver Diseases/complications* ; Cholestasis ; Inflammatory Bowel Diseases/therapy* ; Bile Ducts, Intrahepatic/pathology* ; Bile Duct Neoplasms/therapy*

Objective: To explore disease characteristics of primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) and compare the differences between PSC with and without IBD. Methods: Study design was cross sectional. Forty-two patients with PSC who were admitted from January 2000 to January 2021 were included. We analyzed their demographic characteristics, clinical manifestations, concomitant diseases, auxiliary examination, and treatment. Results: The 42 patients were 11-74(43±18) years of age at diagnosis. The concordance rate of PSC with IBD was 33.3%, and the age at PSC with IBD diagnosis was 12-63(42±17) years. PSC patients with IBD had higher incidences of diarrhea and lower incidences of jaundice and fatigue than in those without IBD (all P<0.05). Alanine aminotransferase, total bilirubin, direct bilirubin, total bile acid and carbohydrate antigen 19-9 levels were higher in PSC patients without IBD than in those with IBD (all P<0.05). The positive rates for antinuclear antibodies and fecal occult blood were higher in PSC patients with IBD than in those without IBD (all P<0.05). Patients with PSC complicated with ulcerative colitis mainly experienced extensive colonic involvement. The proportion of 5-aminosalicylic acid and glucocorticoid application in PSC patients with IBD was significantly increased compared with that of PSC patients without IBD (P=0.025). Conclusions: The concordance rate of PSC with IBD is lower at Peking Union Medical College Hospital than in Western countries. Colonoscopy screening may benefit PSC patients with diarrhea or fecal occult blood-positive for early detection and diagnosis of IBD.
Humans ; Child ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Cholangitis, Sclerosing/therapy* ; Cross-Sectional Studies ; Inflammatory Bowel Diseases/diagnosis* ; Colitis, Ulcerative/complications* ; Diarrhea

Anastomosis, Surgical/adverse effects* ; Cholangitis/surgery* ; Gastroenterostomy/adverse effects* ; Humans ; Pancreatitis/complications* ; Suppuration/surgery*

BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP) is the endoscopic modality of choice for the treatment of biliary and pancreatic diseases. However, patients with cirrhosis, particularly those with decompensated cirrhosis, are believed to be at increased risk for complications associated with ERCP. There is a paucity of literature describing the outcomes of ERCP for patients with cirrhosis. This study aimed to investigate the outcomes of ERCP for cirrhosis patients, especially adverse events, and evaluated its safety and efficacy. METHODS: We performed a multicenter, retrospective study of all patients at Beijing Friendship Hospital affiliated to Capital Medical University, Xijing Hospital affiliated to Air Force Military Medical University, Beijing Youan Hospital affiliated to Capital Medical University, and the Fifth Medical Center of the People's Liberation Army General Hospital from June 2003 to August 2019. The adverse events of inpatient ERCP for patients with ( n  = 182) and without liver cirrhosis (controls; n  = 385) were compared. RESULTS: A total of 567 patients underwent ERCP between January 2003 and December 2019 were enrolled in this study. Compared to patients without cirrhosis, patients with cirrhosis were at higher risk for postoperative complications (odds ratio [OR], 4.172; 95% confidence interval [CI], 1.232-7.031; P  < 0.001) such as postoperative pancreatitis (OR, 2.026; 95% CI, 1.002-4.378; P  = 0.001) and cholangitis (OR, 3.903; 95% CI, 1.001-10.038; P  = 0.036). The main indications for ERCP for patients with cirrhosis in this study included choledocholithiasis (101 cases; 55.5%), benign bile duct strictures (46 cases; 25.3%), and malignant bile duct strictures (28 cases; 15.4%). Among them, 23 patients (12.6%) underwent balloon dilation and 79 patients (43.4%) underwent sphincterotomy. Of the patients with cirrhosis, delayed bleeding occurred in ten patients (5.5%), postoperative pancreatitis occurred in 80 patients (44.0%), and postoperative cholangitis occurred in 25 patients (13.7%). An additional multivariate analysis showed that the total bilirubin (TBIL) level (OR, 4.58; 95% CI, 2.37-6.70) and Child-Pugh score of C (OR, 3.11; 95% CI, 1.04-5.37) were risk factors for postoperative complications in patients with cirrhosis. CONCLUSIONS Compared with the general population of patients undergoing ERCP, patients with cirrhosis were more prone to postoperative pancreatitis and cholangitis. TBIL levels and Child-Pugh scores were risk factors for postoperative complications in patients with cirrhosis.
Humans ; Cholangiopancreatography, Endoscopic Retrograde/adverse effects* ; Retrospective Studies ; Constriction, Pathologic ; Risk Factors ; Liver Cirrhosis/complications* ; Pancreatitis/etiology* ; Postoperative Complications/epidemiology* ; Cholangitis

BACKGROUND/AIMS: The clinical impact of single-stage endoscopic stone extraction by endoscopic retrograde cholangiopancreatography (ERCP) and cholecystectomy during the same hospitalization remains elusive. This study aimed to determine the efficacy and safety of single-stage ERCP and cholecystectomy during the same hospitalization in patients with cholangitis. METHODS: We retrospectively reviewed the medical records of 166 patients who underwent ERCP for mild to moderate cholangitis due to choledocholithiasis secondary to cholecystolithiasis from 2012 to 2016. RESULTS: Complete stone extraction was accomplished in 92% of patients (152/166) at the first ERCP. Among 152 patients who underwent complete stone extraction, cholecystectomy was scheduled for 119 patients (78%). Cholecystectomy was performed during the same hospitalization in 89% of patients (106/119). We compared two groups of patients: those who underwent cholecystectomy during the same hospitalization (n=106) and those who underwent cholecystectomy during a subsequent hospitalization (n=13). In the delayed group, cholecystectomy was performed about three months after the first ERCP. There were no significant differences between the groups in terms of operative time, rate of postoperative complications, and interval from cholecystectomy to discharge. CONCLUSIONS: Single-stage endoscopic stone extraction is recommended in patients with mild to moderate acute cholangitis due to choledocholithiasis. The combination of endoscopic stone extraction and cholecystectomy during the same hospitalization is safe and feasible.
Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis ; Cholecystectomy ; Cholecystectomy, Laparoscopic ; Cholecystolithiasis ; Choledocholithiasis ; Hospitalization ; Humans ; Medical Records ; Operative Time ; Patient Outcome Assessment ; Postoperative Complications ; Retrospective Studies

To systematically evaluate the risks of cardiocerebral vascular events in patients with primary biliary cholangitis(PBC). Methods We carried out a Meta analysis by RevMan 5.3 software to investigate literatureon the risk of cardiocerebral vascular events in patients with PBC and controls. Results Compared with non-PBC controls,PBC patients had significantly higher risk of coronary events(=1.56,=0.0002);however,the risk of cerebrovascular events showed no significant difference between these two groups(=1.01,=0.94).Subgroup analysis demonstrated a significantly lower risk of transient ischemic attack or carotid stenosis in PBC patients(=0.63,=0.03);however,there was no significant difference in the risk of stroke(=1.11,=0.40). Conclusion Patients with PBC have an increased risk of coronary events but may have a lower risk of transient ischemic attack or carotid stenosis.
Carotid Stenosis ; etiology ; Cholangitis ; complications ; Coronary Disease ; etiology ; Humans ; Ischemic Attack, Transient ; etiology ; Liver Cirrhosis, Biliary ; complications ; Risk Factors ; Stroke ; etiology

PURPOSE: Improvements in surgical techniques and a better understanding of the unique anesthetic requirements in neonates undergoing laparoscopy have suggested that laparoscopic surgery may be effective in newborns. This study therefore evaluated the safety and feasibility of laparoscopic excision of the cyst (LEC) in neonates. METHODS: This retrospective study included 43 neonates who underwent excision of choledochal cysts between November, 2001, and January, 2018, including 21 who underwent open excision and 22 who underwent LEC. Their perioperative and surgical outcomes were reviewed. The patients were followed up for a median 37 months (range, 3–141 months). RESULTS: Baseline characteristics did not differ significantly in the open and LEC groups. Mean intraoperative peak partial pressure of arterial CO2 (PaCO2) (45.5 mmHg vs. 48.0 mmHg) and total operation time (208.3 ± 71.0 minutes vs. 235.0 ± 47.2 minutes) were similar in both groups. Parents of the patients in the LEC group provided a more positive evaluation of scar scale and greater satisfaction with wound. No patient in either group experienced any critical complications. Three patients in the open excision group required readmission for cholangitis and 2 patients had ileus. No patient in the laparoscopic excision group experienced any postoperative complications during follow-up. CONCLUSION: Despite difficulties performing laparoscopic surgery in neonates, LEC was safe and feasible when intraperitoneal peak pressure was maintained under 10 mmHg and PaCO₂ was closely monitored by a pediatric anesthesiologist. Compared with open excision, LEC provided improved cosmetic outcomes without severe complications. Prospective randomized studies with large numbers of patients are warranted.
Cholangitis ; Choledochal Cyst ; Cicatrix ; Follow-Up Studies ; Humans ; Ileus ; Infant ; Infant, Newborn ; Laparoscopy ; Minimally Invasive Surgical Procedures ; Parents ; Partial Pressure ; Postoperative Complications ; Prospective Studies ; Retrospective Studies ; Wounds and Injuries

BACKGROUND/AIMS: Controversy remains over the optimal approach to preoperative biliary drainage in patients with resectable perihilar cholangiocarcinoma. We compared the clinical outcomes of endoscopic biliary drainage (EBD) with those of percutaneous transhepatic biliary drainage (PTBD) in patients undergoing preoperative biliary drainage for perihilar cholangiocarcinoma. METHODS: A total of 106 consecutive patients who underwent biliary drainage before surgical treatment were divided into two groups: the PTBD group (n=62) and the EBD group (n=44). RESULTS: Successful drainage on the first attempt was achieved in 36 of 62 patients (58.1%) with PTBD, and in 25 of 44 patients (56.8%) with EBD. There were no significant differences in predrainage patient demographics and decompression periods between the two groups. Procedure-related complications, especially cholangitis and pancreatitis, were significantly more frequent in the EBD group than the PTBD group (PTBD vs EBD: 22.6% vs 54.5%, p<0.001). Two patients (3.8%) in the PTBD group experienced catheter tract implantation metastasis after curative resection during the follow-up period. CONCLUSIONS: EBD was associated with a higher risk of procedure-related complications than PTBD. These complications were managed properly without severe morbidity; however, in the PTBD group, there were two cases of cancer dissemination along the catheter tract.
Aged ; Aged, 80 and over ; Bile Duct Neoplasms/*surgery ; Bile Ducts/surgery ; Cholangitis/etiology ; Drainage/adverse effects/*methods ; Endoscopy, Gastrointestinal/adverse effects/*methods ; Female ; Humans ; Klatskin Tumor/*surgery ; Liver/surgery ; Male ; Middle Aged ; Pancreatitis/etiology ; Postoperative Complications/etiology ; Preoperative Care/adverse effects/*methods ; Treatment Outcome

Ulcerative colitis is an idiopathic chronic inflammatory bowel disease and its incidence in Korea has rapidly increased over the past two decades. Since ulcerative colitis is associated with increased risk for colorectal cancer, annual or biannual colonoscopy with four quadrant random biopsies at every 10 cm segments has been recommended for surveillance of colitic cancer in patients with long standing left-sided or extensive colitis. Recent epidemiologic data and meta-analysis suggest that the increment of colorectal cancer risk in ulcerative colitis was not larger than that of previous studies. Moreover, in addition to the extent and duration of colitis, other risk factors such as family history of colorectal cancer, primary sclerosing cholangitis, stricture, pseudopolyps, and histologic severity of inflammation have been recognized. As a result, updated guidelines provide surveillance strategies adjusted to the individual patient's risk for colitic cancer. Regarding surveillance method, target biopsy under panchromoendoscopy is preferentially recommended rather than random biopsy.
Cholangitis, Sclerosing/complications ; Colitis, Ulcerative/*complications ; Colon/pathology ; Colorectal Neoplasms/epidemiology/*etiology ; Humans ; Inflammatory Bowel Diseases/complications ; Polyps ; Risk Factors

Duodenal diverticula are detected in up to 27% of patients undergoing upper gastrointestinal tract evaluation with periampullary diverticula (PAD) being the most common type. Although PAD usually do not cause symptoms, it can serve as a source of obstructive jaundice even when choledocholithiasis or tumor is not present. This duodenal diverticulum obstructive jaundice syndrome is called Lemmel's syndrome. An 81-yr-old woman came to the emergency room with obstructive jaundice and cholangitis. Abdominal CT scan revealed stony opacity on distal CBD with CBD dilatation. ERCP was performed to remove the stone. However, the stone was not located in the CBD but rather inside the PAD. After removal of the enterolith within the PAD, all her symptoms resolved. Recognition of this condition is important since misdiagnosis could lead to mismanagement and therapeutic delay. Lemmel's syndrome should always be included as one of the differential diagnosis of obstructive jaundice when PAD are present.
Abdominal Pain ; Aged, 80 and over ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangiopancreatography, Magnetic Resonance ; Cholangitis/complications ; Diverticulum ; Duodenal Diseases/complications/*diagnosis ; Female ; Fluoroscopy ; Gallstones/diagnosis/therapy ; Humans ; Jaundice, Obstructive/*complications ; Tomography, X-Ray Computed