We report 13 children fulfilling criteria of Alagille syndrome. All had chronic cholestasis secondary to paucity of intrahepatic bile ducts and triangular facies. Eight children had associated congenital heart disease (six pulmonic stenosis, one each tetralogy of Fallot and patent ductus arteriosus), seven with butterfly vertebrae and one with posterior embryotoxon. Seven of the 13 children are alive and jaundice-free but three with concomitant hypercholesterolemia; the six other children died of liver-related complications.
Butterflies ; Bile Ducts, Intrahepatic ; Jaundice ; Musculoskeletal Abnormalities ; Spine

BACKGROUND/AIMS: Despite improvements in surgical techniques and postoperative patient care, bile leakage can occur after hepatobiliary surgery and may lead to serious complications. The aim of this retrospective study was to evaluate the efficacy of endoscopic treatment of bile leakage after hepatobiliary surgery. METHODS: The medical records of 20 patients who underwent endoscopic retrograde cholangiopancreatography because of bile leakage after hepatobiliary surgery from August 2009 to September 2014 were reviewed retrospectively. Endoscopic treatment included insertion of an endoscopic retrograde biliary drainage stent after endoscopic sphincterotomy. RESULTS: Most cases of bile leakage presented as percutaneous bile drainage through a Jackson-Pratt bag (75%), followed by abdominal pain (20%). The sites of bile leaks were the cystic duct stump in 10 patients, intrahepatic ducts in five, liver beds in three, common hepatic duct in one, and common bile duct in one. Of the three cases of bile leakage combined with bile duct stricture, one patient had severe bile duct obstruction, and the others had mild strictures. Five cases of bile leakage also exhibited common bile duct stones. Concerning endoscopic modalities, endoscopic therapy for bile leakage was successful in 19 patients (95%). One patient experienced endoscopic failure because of an operation-induced bile duct deformity. One patient developed guidewire-induced microperforation during cannulation, which recovered with conservative treatment. One patient developed recurrent bile leakage, which required additional biliary stenting with sphincterotomy. CONCLUSIONS: The endoscopic approach should be considered a first-line modality for the diagnosis and treatment of bile leakage after hepatobiliary surgery.
Abdominal Pain ; Bile Ducts ; Bile* ; Catheterization ; Cholangiopancreatography, Endoscopic Retrograde ; Cholestasis ; Common Bile Duct ; Congenital Abnormalities ; Constriction, Pathologic ; Cystic Duct ; Diagnosis ; Drainage ; Hepatic Duct, Common ; Humans ; Liver ; Medical Records ; Patient Care ; Retrospective Studies ; Sphincterotomy, Endoscopic ; Stents

BACKGROUND/AIMS: Chronic liver disease leads to liver fibrosis, and although the liver does have a certain regenerative capacity, this disease is associated with dysfunction of the liver vessels. C-reactive protein (CRP) is produced in the liver and circulated from there for metabolism. CRP was recently shown to inhibit angiogenesis by inducing endothelial cell dysfunction. The objective of this study was to determine the effect of CRP levels on angiogenesis in a rat model of liver dysfunction induced by bile duct ligation (BDL). METHODS: The diameter of the hepatic vein was analyzed in rat liver tissues using hematoxylin and eosin (H&E) staining. The expression levels of angiogenic factors, albumin, and CRP were analyzed by real-time PCR and Western blotting. A tube formation assay was performed to confirm the effect of CRP on angiogenesis in human umbilical vein endothelial cells (HUVECs) treated with lithocholic acid (LCA) and siRNA-CRP. RESULTS: The diameter of the hepatic portal vein increased significantly with the progression of cirrhosis. The expression levels of angiogenic factors were increased in the cirrhotic liver. In contrast, the expression levels of albumin and CRP were significantly lower in the liver tissue obtained from the BDL rat model than in the normal liver. The CRP level was correlated with the expression of albumin in hepatocytes treated with LCA and siRNA-CRP. Tube formation was significantly decreased in HUVECs when they were treated with LCA or a combination of LCA and siRNA-CRP. CONCLUSION: CRP seems to be involved in the abnormal formation of vessels in hepatic disease, and so it could be a useful diagnostic marker for hepatic disease.
Angiogenic Proteins/genetics/metabolism ; Animals ; Bile Ducts/surgery ; C-Reactive Protein/*analysis/genetics/metabolism ; Cells, Cultured ; Disease Models, Animal ; Hepatic Veins/abnormalities ; Hepatocytes/cytology/metabolism ; Human Umbilical Vein Endothelial Cells ; Humans ; Lithocholic Acid/pharmacology ; Liver/metabolism/pathology ; Liver Cirrhosis/etiology ; Liver Diseases/metabolism/*pathology ; Male ; Microscopy, Fluorescence ; Mitochondria/drug effects/metabolism ; RNA Interference ; RNA, Small Interfering/metabolism ; Rats ; Rats, Sprague-Dawley ; Real-Time Polymerase Chain Reaction ; Serum Albumin/genetics/metabolism

Adolescent ; Bile Ducts, Intrahepatic ; abnormalities ; Cholestasis, Intrahepatic ; etiology ; Humans ; Male

Adolescent ; Bile Duct Diseases ; congenital ; Bile Ducts, Intrahepatic ; abnormalities ; Dilatation, Pathologic ; Female ; Humans

Anatomic variation in the hepatobiliary tract is relatively common, although bile duct duplication is rare. Detection of biliary tract variation is important because it is closely related to complications such as cholangitis, cholecystitis, pancreatitis, stone formation, and gastrointestinal tract malignancy. Therefore, preoperative imaging and a high index of suspicion are required to make an accurate diagnosis of a biliary tract anomaly. A 44-year-old woman presented with a 2-year history of epigastric pain after eating a meal and gallbladder sludge with chronic cholecystitis. The presumed diagnosis was gallbladder duplication. However, during surgery it was observed that the tubular structure was parallel with the fissure for the ligamentum venosum and ended at the lesser curvature of the stomach. The pathologic diagnosis was bile duct duplication. We report a case of a unique variant of bile duct duplication arising from the hepatic hilum.
Adult ; Anatomic Variation ; Bile ; Bile Ducts ; Biliary Tract ; Biliary Tract Diseases ; Cholangitis ; Cholecystectomy, Laparoscopic ; Cholecystitis ; Digestive System Abnormalities ; Eating ; Female ; Gallbladder ; Gastrointestinal Tract ; Humans ; Meals ; Pancreatitis ; Sewage ; Stomach

BACKGROUND/AIMS: An ectopic opening of the common bile duct (CBD) into the duodenal bulb is known to cause biliary tract diseases or peptic ulcer. Yet such a case is extremely rare and the clinical significance of this malformation has not yet been clarified. METHODS: Ten patients with an ectopic opening in the duodenal bulb and who were treated at 4 hospitals in the Chungcheong province area were enrolled. Their clinical and radiographic features and the treatment for their combined biliary diseases were retrospectively analysed. RESULTS: The mean age of the ten patients was 73 years (range: 38~84 years, 8 men, 2 women) Eight of 10 patients had clinical signs and symptoms (RUQ abdominal pain: 5, epigastric pain: 3, fever: 3, Melena: 1). The two patients without symptoms were incidentally found to have an ectopic orifice during the evaluation for bile duct dilatation and a regular health check-up, respectively. Nine had duodenal ulcer scars or bulb deformities. Four had active duodenal ulcers and one of them had bleeding from the active duodenal ulcer, which was treated by endoscopic sclerotheraphy. Six of the 10 patients (60%) had biliary tract diseases (CBD stones: 4, cholangitis without CBD stone: 1, IHD stone: 1). Cholangiography was used for evaluation in 9 patients, and it showed dilatation of the CBD or IHD in 7 (78%), a tapered common bile duct at the distal CBD in 7 (78%) and a hook-shaped distal CBD in 8 (89%). Six patients' biliary tract diseases were treated endoscopically (ERCP: 4, PTCS: 2). Duodenal perforation occurred in 1 (10%), who was successfully managed by medical treatment. CONCLUSIONS: An ectopic opening of the CBD in the duodenal bulb is frequently associated with recurrent duodenal ulcer and biliary tract disease. Combined biliary tract disease can be successfully treated endoscopically.
Bile Ducts ; Biliary Tract Diseases ; Cholangiography ; Cholangitis ; Cicatrix ; Common Bile Duct ; Congenital Abnormalities ; Dilatation ; Duodenal Ulcer ; Hemorrhage ; Humans ; Male ; Peptic Ulcer ; Retrospective Studies

The most common causes of acute pancreatitis are microlithiasis and alcohol. In pediatrics, anomalies in pancreaticobiliary system should be considered as possible causes. Among many anomalies, pancreas divisum associated with anomalous pancreaticobiliary ductal union (APBDU) is very rare. APBDU is associated with acute pancreatitis, choledochal cyst, and gallbladder cancer. Pancreas divisum is also a well known cause of acute recurrent pancreatitis. In adult cases with such conditions, the role of endoscopic management including sphincterotomy or stenting through the Santorini duct is well documented. However, it is still controversial to perform endoscopic retrograde cholangiopancreatography in pediatrics. Herein, we experienced a case of 4 year 7 month old female patient suffered from recurrent attacks of acute pancreatitis, which were caused by APBDU and incomplete pancreas divisum. She was treated by endoscopic sphincteretomy of both openings to the Santorini's and Wirsung's ducts. Thus, we report this interesting case with literature review.
Abnormalities, Multiple ; Acute Disease ; Child, Preschool ; Cholangiopancreatography, Endoscopic Retrograde ; Common Bile Duct/*abnormalities/surgery ; Female ; Humans ; Pancreas/*abnormalities ; Pancreatic Ducts/*abnormalities/surgery ; Pancreatitis/*diagnosis/etiology/surgery ; Sphincterotomy, Endoscopic ; Tomography, X-Ray Computed

We report here on an extremely rare case of duplicated extrahepatic bile ducts that was associated with choledocholithiasis, and this malady was visualized by employing the minimum intensity projection images with using multi-detector row CT. The presence of duplicated extrahepatic bile ducts with a proximal communication, and the ducts were joined distally and they subsequently formed a single common bile duct, has not been previously reported.
Aged, 80 and over ; Bile Ducts, Extrahepatic/*abnormalities/pathology/radiography ; Choledocholithiasis/*complications/radiography ; Female ; Humans

Ectopic opening of the common bile duct (CBD) in the duodenal bulb is not an incidental finding, but a pathologic condition that can be associated with clinical entities, such as recurrent duodenal ulcer, choledocholithiasis. or cholangitis. We report a case of ectopic CBD with hematemesis in a 61 year-old male patient. Esophagogastroduodenoscopy showed several active ulcers with a fresh blood clot at the duodenal bulb, which had a mucosal deformity and a small opening with suspicious drainage of bile, and the second part of the duodenum had no papilla-like structure. MRCP showed a trident-like pancreatobiliary tree. The biliary tree was visualized via a suspicious ectopic opening of the bile duct at the duodenal bulb. The distal CBD looked hook-shaped and had no filling defect. We report a unique case of ectopic opening of the CBD in the duodenal bulb, which presented as hematemesis.
Bile ; Bile Ducts ; Biliary Tract ; Cholangitis ; Choledocholithiasis ; Common Bile Duct ; Congenital Abnormalities ; Drainage ; Duodenal Ulcer ; Duodenum ; Endoscopy, Digestive System ; Hematemesis ; Humans ; Incidental Findings ; Male ; Ulcer