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

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

Intrahepatic stones are frequently associated with recurrent pyogenic cholangiohepatitis and complete surgical removal of the stones is almost always difficult because of the large number of stones and associated bile duct strictures. One hundred and seventy-nine patients with retained intrahepatic stones underwent percuttaneous stone removal utilizing a preshaped angulated catheter and a Dormia basket under fluoroscopy with a combination of techniques including irrigation-suction. crushing of large stones. balloon dilatation of strictures and extracorporenal shock wave lithotripsy. The procedure was performed through a mature T-tube tract (177 patients) and a mature transhepatic tube tract (two patients). Stones were exclusively intrahepatic in 130 patients. whereas 49 patients also had stones in the common bile duct. In 91 (50.8%) of 179 patients. the stones were completely removed and in 36 (20.1%) patients most of the stones were removed. The overall success rate was 70.9% ngulation deformity. stricture of bile ducts and impacted stones were the factor most often responsible for failure, No significant complications were observed. Fluoroscopicalyy-guided percutaneous interventional procedures with a preshaped angulated catheter is an useful complementary procedure to surgery for patients with intrahepatic stones. the major benefits of an individually fitted angulated catheter are its safety and easy access to small peripheral bile ducts.
Bile Ducts ; Catheters* ; Common Bile Duct ; Congenital Abnormalities ; Constriction, Pathologic ; Dilatation ; Fluoroscopy ; Humans ; Lithotripsy ; Shock

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

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

Adult ; Bile Ducts, Intrahepatic ; abnormalities ; Cholestasis, Intrahepatic ; diagnosis ; etiology ; therapy ; 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

BACKGROUND : Anomalous pancreaticobiliary ductal union (APBDU) is a congenital defect defined as a union of the pancreatic and the biliary ducts located outside the duodenal wall. Accordingly, this anomalous connection between the choledochus and the pancreatic duct is considered to be a factor in the development of biliary tract diseases such as choledochal cysts, pancreatitis, cholangitis, gallbladder cancer, and bile duct cancer. Our purpose is to analyze combined disease, especially biliary neoplasm, and to evaluate biliary tract microscopic changes in the biliary tract. METHODS : To study the clinical characteristics of APBDU, we reviewed 21 APBDU cases which were treated from June 1994 to January 1998. We studied the associated diseases, the surgical treatment, histological findings and the PCNA expression. We also analyzed the gallbladder epithelium in 10 control patients without APBDU. RESULTS : Among the 21 cases, 12 cases were accompanied by a choledochal cyst. A gallbladder carci noma occurred in 5 cases. Of the 13 operations, 4 involved a cholecystectomy only, and 9 involved a cholecystectomy, bile duct resection and hepaticojejunostomy. The histologic findings were reviewed in 13 cases. Bile duct metaplasia was found in 8 of the 9 cases with a bile duct resection, and gallbladder metaplasia was found in 8 of the 12 cases whereas metaplasia was detected in 1 of the 10 control patients. The PCNA expression was significantly high in APBDU group. CONCLUSIONS : The patients with APBDU showed a high incidence of gallbladder carcinoma and meta plasia in the epithelium of the gallbladder and the bile duct. This metaplasia in the gallbladder and the bile duct was thought to be related to the cancerous change.
Bile Duct Neoplasms ; Bile Ducts* ; Bile* ; Biliary Tract ; Biliary Tract Diseases ; Cholangitis ; Cholecystectomy ; Choledochal Cyst ; Common Bile Duct ; Congenital Abnormalities ; Epithelium ; Gallbladder Neoplasms ; Gallbladder* ; Humans ; Incidence ; Metaplasia ; Noma ; Pancreatic Ducts ; Pancreatitis ; Proliferating Cell Nuclear Antigen

Congenital hepatic fibrosis is an inherited, congenital disorder of the liver, and is occasionally associated with cystic disease of the liver and kidney. We present a case of congenital hepatic fibrosis with Caroli's disease. A 21-year-old woman had suffered from an episodic fever with headaches for 3 years. In laboratory examination, the liver function test was within the normal limits. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and multiple dilated bile ducts were seen by abdominal CT scanning. An orthotopic whole liver transplantation was done. The liver was fibrotic and enlarged. Multiple cystically dilated intrahepatic ducts were noted. Microscopically, diffuse portal fibrosis and widening with proliferation of bile ductules were seen. Intrahepatic bile ducts were markedly dilated and tortuous. The liver cell cords were well preserved.
Bile ; Bile Ducts ; Bile Ducts, Intrahepatic ; Caroli Disease* ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Esophageal and Gastric Varices ; Female ; Fever ; Fibrosis* ; Headache ; Humans ; Kidney ; Liver ; Liver Function Tests ; Liver Transplantation ; Tomography, X-Ray Computed ; Young Adult

OBJECTIVE: Since choledochal cyst is frequently associated with the anomalous union of pancreaticobiliary duct (AUPBD), AUPBD has been regarded to be the etiologic factor of choledochal cyst. However, the clinical significance of AUPBD an patients with choledochal cyst has not been clearly defined. Therefore, to clarify the significance of AUPBD in choledochal cyst patients, we compared the clinical features of patients with choledochal cyst according to the presence or absence of AUPBD. METHODS: Among 52 cases which were diagnosed as choledochal cyst out of 5,037 ERCP referrals between August 1990 and December 1996, we selected 44 cases, in which the pancreaticobiliary junction was clearly visualized on cholangio-pancreaticography. These cases were divided into AUPBD-present group (n = 28) and AUPBD-absent group (n = 16). Clinical features were compared between the two groups. Furthermore, in AUPBD-present group, clinical data were also analyzed according to Kimura's classification of AUPBD. RESULTS: In our study, AUPBD was associated with choledochal cyst in 28 (64%) cases. AUPBD was found only in type I and IV according to Todani's classification of choledochal cyst. There were no significant differences between the AUPBD-present group and the AUPBD-absent group in the incidence of gallstone disease, while the incidence of acute inflammation was 93% (26/28) in the AUPBD-absent group (p < 0.01). Carcinoma developed only in the AUOBD-present group (9/28, 32%) (p < 0.05). Pancreatic disorders (i.e. pancreatic stone, pancreatitis or pancreatic cancer) occurred in 12 of 28 cases in the AUPBD-present group (43%), while only in 1 of 16 cases in the AUPBD-absent group (6%) (p < 0.05). CONCLUSION: AUPBD associated with choledochal cyst may have implications not only as a possible etiologic factor but also as an important factor that may affect the clinical course, surgical planning and prognosis. In cases with choledochal cyst, we should make an effort to evaluate the presence of AUPBD.
Adolescence ; Adult ; Aged ; Bile Ducts/abnormalities* ; Calculi/complications ; Cholangiography ; Choledochal Cyst/radiography ; Choledochal Cyst/pathology ; Choledochal Cyst/complications* ; Female ; Human ; Inflammation/complications ; Male ; Middle Age ; Neoplasms/complications ; Pancreatic Ducts/radiography ; Pancreatic Ducts/abnormalities* ; Prognosis