Sclerosing cholangitis is a spectrum of chronic progressive cholestatic liver disease characterized by inflammation, fibrosis, and stricture of the bile ducts, which can be classified as primary and secondary sclerosing cholangitis. Primary sclerosing cholangitis is a chronic progressive liver disease of unknown cause. On the other hand, secondary sclerosing cholangitis has identifiable causes that include immunoglobulin G4-related sclerosing disease, recurrent pyogenic cholangitis, ischemic cholangitis, acquired immunodeficiency syndrome-related cholangitis, and eosinophilic cholangitis. In this review, we suggest a systemic approach to the differential diagnosis of sclerosing cholangitis based on the clinical and laboratory findings, as well as the typical imaging features on computed tomography and magnetic resonance (MR) imaging with MR cholangiography. Familiarity with various etiologies of sclerosing cholangitis and awareness of their typical clinical and imaging findings are essential for an accurate diagnosis and appropriate management.
Adult ; Aged ; Aged, 80 and over ; Bile Ducts/*pathology ; Cholangiography/*methods ; Cholangitis/diagnosis/*pathology ; Cholangitis, Sclerosing/*diagnosis/pathology ; Cholestasis/diagnosis/*pathology ; Chronic Disease ; Constriction, Pathologic/diagnosis ; Diagnosis, Differential ; Female ; Humans ; Immunoglobulin G/immunology ; Liver/pathology ; Magnetic Resonance Imaging/methods ; Male ; Middle Aged ; Tomography, X-Ray Computed/methods

Although portobiliary fistula is a recognised complication of percutaneous transhepatic biliary drainage, it is extremely uncommon and can result in haemobilia. Herein, we present a case of complicated transhepatic biliary drainage catheter insertion in a patient with underlying hepatitis B liver cirrhosis, which resulted in a portobiliary fistula. The patient had a preoperative transhepatic biliary drainage procedure done prior to a Whipple's operation for a large, obstructive, gastrointestinal stromal tumour of the duodenum. To the best of our knowledge, this is the first case in the English medical literature reporting the successful treatment of portobiliary fistula by embolisation of its tract with embolisation coils, without compromising the portal vein or bile ducts.
Anemia ; complications ; Bile Ducts ; pathology ; Catheterization ; Cholangiography ; methods ; Embolization, Therapeutic ; methods ; Fistula ; etiology ; therapy ; Hepatitis B ; complications ; Humans ; Liver ; pathology ; Liver Cirrhosis ; complications ; Male ; Middle Aged ; Portal Vein ; pathology ; Tomography, X-Ray Computed ; Treatment Outcome

OBJECTIVETo characterize the clinical, laboratory, imaging and pathological features of primary sclerosing cholangitis (PSC) and investigate the impact of ursodeoxycholic acid (UDCA) therapy on patient prognosis.

METHODSThe medical records of 22 patients diagnosed with PSC between 2002 and 2011 were retrospectively reviewed. The PSC diagnosis had been made in patients with suspect biochemical abnormalities following evaluation by magnetic resonance cholangiopancreatography (MRCP) and/or endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC). Fibrosis and inflammation were assessed by immunohistochemical analyses of tissue biopsies. Outcome of patients treated with UDCA (13-15 mg/kg/day, oral) were compared to that of patients without UDCA treatment by the X2 or corrected X2 tests.

RESULTSAmong the 22 PSC patients, the majority was male (n=15) and presented with fatigue, dark urine, and body weight loss (n=15). Four cases had ulcerative colitis. At admission, all 22 cases showed elevated levels of alkaline phosphatase[ALP: (348+/-184) U/L], 19 cases showed elevated alanine aminotransferase [ALT: (94.0+/-67.0) U/L] and aspartate aminotransferase [AST: (98.0+/-67.0) U/L], and 15 cases showed elevated levels of total bilirubin (99.0+/-115.0) mumol/L and direct bilirubin (74.4+/-92.4 mumol/L. ERCP examination showed segmental intrahepatic bile duct stenosis with expansion, and stiff and enlarged gallbladder bile ducts, but unclear findings for the common bile ducts and pancreatic ducts. MRCP showed beading of the intrahepatic bile duct, stiffness of the bile duct wall, and dilation of the common bile duct. Fibrosis and inflammation were observed in the bile ducts, along with hyperplasia and the typical features of "onion skin" fibrosis and fibrous obliterative cholangitis. Five of the 10 patients treated with UDCA improved, and seven of the 12 patients in the non-UDCA treatment group improved. There was no statistically significant difference in outcome between the groups (paired X2=0.333, corrected X2=0.083, P more than 0.05).

CONCLUSIONPSC patients were predominantly male and the common clinical manifestations were fatigue, dark urine, and body weight loss. At admission, serum biochemical indicators of cholangitis were increased significantly and subsequent imaging studies confirmed the suspected diagnosis by showing obvious characteristic changes. UDCA treatment did not significantly improve patient prognosis.

Adult ; Cholangiography ; methods ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis, Sclerosing ; diagnostic imaging ; pathology ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Young Adult

BACKGROUNDIntra-operative cholangiography has been shown to be a sensitive and specific method of demonstrating bile duct stones. This study investigated the feasibility, safety, and clinical value of selective trans-cystic intra-operative cholangiography in primary suture following three-port laparoscopic common bile duct exploration, and identified the factors that positively predict the presence of common bile duct stones.

METHODSFrom January 2008 to January 2011, 252 of 1013 patients undergoing laparoscopic cholecystectomy received selective trans-cystic intra-operative cholangiography and primary suture following three-port laparoscopic common bile duct exploration. Their clinical data were analyzed retrospectively.

RESULTSAll operations were successful and none was converted to open surgery. The intra-operative cholangiography time was (8.3 ± 2.5) minutes, and the operative duration was (105.4 ± 23.1) minutes. According to selective intra-operative cholangiography, the positive predictive values of current jaundice, small gallstones (< 0.5 cm) and dilated cystic duct (> 0.3 cm), dilated common bile duct (> 0.8 cm), history of jaundice or gallstone pancreatitis, abnormal liver function test, and preoperative demonstration of suspected common bile duct stones on imaging were 87%, 25%, 42%, 15%, 32%, and 75% for common bile duct stones, respectively. Patients with several factors suggestive of common bile duct stones yielded higher numbers of positive cholangiograms. Unexpected stones were found in 13 patients (5.2%) by intra-operative cholangiography. The post-operative hospital stay was (4.7 ± 2.2) days. Post-operative bile leakage occurred in two cases, and these patients recovered by simple drainage for 3 - 7 days without re-operation. Of the 761 patients who underwent laparoscopic cholecystectomy alone, 5 (0.7%) presented with a retained common bile duct stone requiring intervention. The median follow-up was 12 months, and only one patient who once suffered from bile leakage presented with obstructive jaundice due to bile duct stenosis 6 months postoperatively. The other patients recovered without any serious complications.

CONCLUSIONSSelective intra-operative cholangiography yields acceptably high positive results. It is a safe, effective, and minimally invasive approach in patients with suspected choledocholithiasis and primary suture following three-port laparoscopic common bile duct exploration.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Cholangiography ; methods ; Cholecystectomy, Laparoscopic ; methods ; Choledocholithiasis ; diagnostic imaging ; surgery ; Common Bile Duct ; diagnostic imaging ; surgery ; Female ; Gallstones ; diagnostic imaging ; surgery ; Humans ; Male ; Middle Aged ; Young Adult

An obstructed biliary system is usually drained by endoscopic retrograde cholangiopancreatography, and when this is unsuccessful, the standard alternative technique is percutaneous transhepatic biliary drainage. Surgical biliary bypass may also be required. In recent years, endosonography has transformed from a solely diagnostic procedure to one with therapeutic capabilities. Endosonography-guided biliary drainage is now being performed as an alternative to percutaneous transhepatic biliary drainage. This is an evolving field, with challenges that must be addressed before it can become a routine clinical practice. This review summarises the current status of endosonography-guided biliary drainage.
Bile Ducts ; diagnostic imaging ; pathology ; Biliary Tract ; physiology ; Biliary Tract Diseases ; diagnostic imaging ; surgery ; Cholangiography ; methods ; Cholangiopancreatography, Endoscopic Retrograde ; methods ; Cholestasis ; diagnostic imaging ; surgery ; Drainage ; methods ; Endosonography ; methods ; Humans ; Models, Biological ; Stents

Acute obstructive cholangitis due to the migration of necrotized tumor fragment is a rare complication occurring after a transarterial chemoembolization. The percutaneous tumor removal procedure following percutaneous transhepatic biliary drainage is an appropriate treatment over endoscopic removal for the relief of acute cholangitis in this case. Following this serial management, no invasive hepatocellular carcinoma of the bile duct recurred after two years of follow-up.
Acute Disease ; Bile Duct Neoplasms/pathology/therapy ; Bile Ducts, Intrahepatic ; Carcinoma, Hepatocellular/pathology/therapy ; *Catheterization ; Chemoembolization, Therapeutic/*adverse effects/methods ; Cholangiography ; Cholangitis/*etiology/therapy ; Drainage ; Humans ; Jaundice, Obstructive/*etiology/therapy ; Liver Neoplasms/pathology/therapy ; Male ; Middle Aged ; Neoplasm Invasiveness ; Neoplasm, Residual

OBJECTIVE: To evaluate the feasibility of CT fluoroscopy (CTF)-guided percutaneous transhepatic biliary drainage (PTBD) in emergency patients with acute obstructive cholangitis. MATERIALS AND METHODS: The study included 28 patients admitted to the emergency center due to obstructive jaundice and found to require urgent biliary drainage, as well as judged to have a suitable peripheral bile duct for a CTF-guided puncture (at least 4 mm in width). Prior to the CTF-guided puncture, a CT scan was performed to evaluate bile duct dilatation and the underlying causes of biliary obstruction. If the patient was judged to be a suitable candidate, a CTF-guided PTBD was performed in the same CT unit without additional fluoroscopic guidance. Technical feasibility of the procedure was investigated with the evaluation of overall success rate and causes of failure. RESULTS: A hepatic puncture was attempted at the left lobe in 23 patients and right lobe in five patients. The procedure was successful in 24 of 28 patients (86%) Successful biliary puncture was achieved on the first attempt in 16 patients, the second attempt in five patients, and the third attempt in three patients. The causes of failure included guide wire twisting in one patient, biliary puncture failure in two patients, and poor visualization of the guide wire in one patient. There were no significant procedure-related complication. CONCLUSION: The CTF-guided PTBD is technically feasible and highly successful in patients judged to have a suitable indication. Moreover, although the procedure is unfamiliar and inconvenient to interventionalists, it has economical advantages in that it saves time and manpower. We believe this method can be used in the emergency patients requiring urgent biliary drainage as an alternative for the fluoroscopy-guided PTBD.
Acute Disease ; Aged ; Aged, 80 and over ; Cholangiography ; Cholangitis/*therapy ; Drainage/*methods ; Emergencies ; Feasibility Studies ; Female ; *Fluoroscopy ; Humans ; Jaundice, Obstructive/*therapy ; Male ; Middle Aged ; Prospective Studies ; Radiography, Interventional ; *Tomography, X-Ray Computed

Choledochal cyst is a cystic or fusiform dilatation of the extra- or intrahepatic bile duct that has rarely been reported in prenatal cases. Here we report a fetus with choledochal cyst diagnosed prenatally by three-dimensional (3-D) ultrasonography at 22 weeks of gestation. We demonstrated an image of choledochal cyst by using a new ultrasound technique, a 3-D multislice view. After close intrauterine followup, surgery was successfully performed and postoperative course was uneventful.
Anastomosis, Roux-en-Y ; Cholangiography/methods ; Choledochal Cyst/*diagnosis/*ultrasonography ; Female ; Fetal Diseases/*diagnosis/*ultrasonography ; Humans ; Image Processing, Computer-Assisted ; Imaging, Three-Dimensional ; Infant ; Male ; Pregnancy ; Pregnancy Trimester, Second ; Prenatal Diagnosis ; Treatment Outcome ; Ultrasonography, Prenatal/*methods

Benign schwannomas arise in neural crest-derived Schwann cells. They can occur almost anywhere in the body, but their most common locations are the central nervous system, extremities, neck, mediastinum, and retroperitoneum. Schwannomas occurring in the biliary tract are extremely rare and mostly present with obstructive jaundice. We recently experienced a case of extrahepatic biliary schwannomas in a 64-yr-old female patient who presented with intra- and extrahepatic bile duct and gallbladder stones during a screening program. To the best of our knowledge, extrahepatic biliary schwannomas associated with bile duct stones have not been reported previously in the literature.
Adult ; Bile Ducts/pathology ; Bile Ducts, Extrahepatic/*metabolism/*pathology ; Central Nervous System/pathology ; Cholangiography/methods ; Endoscopy ; Female ; Humans ; Male ; Middle Aged ; Neurilemmoma/*diagnosis/*pathology ; Tomography, X-Ray Computed

OBJECTIVE: We wanted to evaluate the therapeutic efficacy of the percutaneous balloon dilatation and large profile catheter maintenance method for the management of patients with anastomotic biliary strictures following liver transplant. MATERIALS AND METHODS: From May 1999 to June 2003, 12 patients with symptomatic benign biliary stricture complicated by liver transplantation were treated with the percutaneous balloon dilatation and large profile catheter maintenance method (1-6 months). The patients were eight males and four females, and their ages ranged from 20 to 62 years (mean age: 44 years). Ten patients underwent living donor liver transplantation and two underwent cadaveric liver transplantation. Postoperative biliary strictures occurred from two to 21 months (mean age: 18 months) after liver transplantation. RESULTS: The initial technical success rate was 92%. Patency of the bile duct was preserved for eight to 40 months (mean period: 19 months) in 10 of 12 (84%) patients. When reviewing two patients (17%), secondary balloon dilatations were needed for treating the delayed recurrence of biliary stricture. In one patient, no recurrent stenosis was seen during the further 10 months follow-up after secondary balloon dilatation. Another patient did not response to secondary balloon dilatation, and he was treated by surgery. Eleven of 12 patients (92%) showed good biliary patency for 8-40 months (mean period: 19 months) of follow-up. CONCLUSION: The percutaneous balloon dilatation and large profile catheter maintenance method is an effective therapeutic alternative for the treatment of most biliary strictures that complicate liver transplantation. It has a high success rate and it should be considered before surgery.
Treatment Outcome ; Middle Aged ; Male ; Liver Transplantation/*adverse effects ; Humans ; Hepatic Artery/ultrasonography ; Female ; Constriction, Pathologic/etiology/therapy ; Cholangiography ; Bile Duct Diseases/etiology/*therapy ; Balloon Dilatation/*methods ; Adult