Hepatocellular carcinoma presenting as a tumour of the hilar and extrahepatic bile ducts

A case of hepatocellular carcinoma extending within the large extra- and intrahepatic bile ducts is reported. No primary tumour was found in the liver parenchyma by abdominal ultrasound, spiral computed tomography or magnetic resonance, but transduodenal cholangioscopy showed tumour in the common hepatic ducts and the two main branches. Endoscopic biopsy showed highly differentiated hepatocellular carcinoma. The patient was treated with endoscopic biliary drainage and died at home 7 months after admittance.     

Percutaneous transhepatic cholangiography utilizing the Chiba University needle

Percutaneous transhepatic cholangiography with the Chiba University needle has been performed in 42 patients with cholestasis. The examination was successful in 100% of patients with dilated intrahepatic bile ducts and in 89% of those with normal-size ducts. Complications were minimal. The results of this study suggest that PTC with the Chiba needle is superior to the current conventional methods. The patients with intrahepatic cholestasis, which often simulates extrahepatic biliary obstruction, were spared an unnecessary laparotomy.     

Acquired biliary atresia

Three infants are described in whom acquired biliary atresia developed during the perinatal period. In two cases this was related to a spontaneous perforation of the bile duct, and in the other it probably was related to previous surgery for duodenal and ileal atresias. Clinically, the symptoms in these patients differed from the congenital forms of biliary atresia; two of the infants had dilated intrahepatic ducts on ultrasonography, and all had restriction of disease to the extrahepatic bile ducts and an excellent response to surgery.     

Case report: secondary biliary cirrhosis possibly related to congenital hepatic fibrosis. Evidence for decreased number of portal branch veins and hypertrophic peribiliary vascular plexus

A 30-year-old man with presinusoidal portal hypertension was transplanted for cryptogenic cirrhosis. On the explanted liver, few intrahepatic stones, biliary cirrhosis, chronic cholangitis of the large bile ducts and a peculiar proliferation of small dilated bile ducts at the periphery of the portal tracts led to the diagnosis of secondary biliary cirrhosis and cholangitis, possibly linked to ductal plate malformation, including congenital hepatic fibrosis associated with a minor form of Caroli's disease. Ex vivo portogram and histology showed the paucity of portal vein branches and the hypertrophy of the peribiliary vascular plexus. This hypertrophy, which has been reported in livers with presinusoidal hypertension, is another indirect argument to suggest the diagnosis of congenital hepatic fibrosis.     

A case of Caroli's disease: usefulness of color Doppler sonography for evaluating the malformation of the intrahepatic bile ducts

Caroli's disease is a rare congenital disorder with multiple cystic dilatations of the intrahepatic bile ducts. In addition to the nonobstructive localized dilatation of the bile ducts, intraluminar bulbar protrusions of the wall and bridge formation across dilated lumina have been demonstrated on sonography and computed tomography. We present a case in which color Doppler sonography clearly showed a blood flow signal toward the periphery of the liver in the protrusion of the dilated intrahepatic bile ducts. Color Doppler sonography is a useful noninvasive method for evaluating the malformation of Caroli's disease.     

Mast cell cholangiopathy: another cause of sclerosing cholangitis

A 75-year-old woman with known systemic mastocytosis presented with abdominal pain, ascites, and bile duct thickening on computed tomography and ultrasonography. A liver biopsy specimen showed infiltration with mast cells. Endoscopic retrograde cholangiography showed ductal changes compatible with those found in primary sclerosing cholangitis. Brush cytology of the intrahepatic bile ducts confirmed mast cell infiltration. Systemic mastocytosis can infiltrate the biliary system, producing a cholangiopathy radiographically similar to primary sclerosing cholangitis.     

New frontiers in the management of obstructive sleep apnoea syndrome

BACKGROUND: To report the dynamic magnetic resonance (MR) imaging findings of hepatolithiasis. METHODS: Dynamic MR images (fast spoiled gradient echo sequence with intravenous injection of gadopentate dimeglumine) and computed tomography, cholangiography, or angiography of nine patients with hepatolithiasis are analyzed. RESULTS: All affected hepatic segments showed atrophic changes and contained dilated intrahepatic ducts. These segments showed either iso- or hypointensity on T1-weighted imaging and hyperintensity on T2-weighted imaging. Preferential enhancement was noted throughout all phases of the dynamic study and persisted to delayed T1-weighted imaging in seven patients. In the last two patients, severe atrophic changes made evaluating signal intensity differences and enhancement patterns difficult. CONCLUSION: In addition to intrahepatic stones and biliary dilatation, segmental atrophy, signal intensity differences, and preferential and persistent enhancement are important MR findings of hepatolithiasis.     

Spiral computed tomography for bilioenteric anastomotic stricture

Spiral computed tomography scanning after intravenous cholangiography (IVC-SCT) was used to assess the anatomy of the biliary tree and anastomosis in a 7-year-old girl with recurrent cholangitis 5 years after surgical excision of a choledochal cyst. The technique clearly visualized the stenosis of the hepaticojejunostomy and hepatic duct and the dilated intrahepatic ducts. IVC-SCT provides a precise, prompt, and accurate image with minimal invasiveness and is especially suitable for the preoperative evaluation of pediatric patients.     

Biliary tract abnormalities in patients with arteriohepatic dysplasia

Arteriohepatic dysplasia is a congenital syndrome associated with life-long cholestasis. Because of symptoms suggesting extrahepatic biliary tract obstruction, we studied three patients with this syndrome by endoscopic retrograde cholangiopancreatogram (ERCP). All patients showed a decrease in the number of intrahepatic ducts. In addition, the intrahepatic ducts show attenuation with focal areas of dilatation. In one subject, this latter finding appeared to be a localized process. The extrahepatic ducts were also narrowed. One patient in this series was found to have gallstones and another cirrhosis. Although the relationship of these anomalies to the cholestasis seen in these patients is unclear, arteriohepatic dysplasis can be added to the list of processes associated with biliary tract abnormalities.     

Caroli''s disease: a central dot means a lot

We present here a patient of congenital non-obstructive focal dilatation of intrahepatic biliary radicles (Caroli's disease). The peculiar configuration of dilated intrahepatic biliary channels with central portal vein radicles was picked up sonologically as the 'central dot' sign. Awareness of this sign allows an accurate diagnosis without resorting to more invasive and expensive investigations.     

A microcholangiographic study of liver disease models in rats

RATIONALE AND OBJECTIVES: Rats develop hepatobiliary injury due to small bowel bacterial overgrowth (SBBO) that, at specimen, resembles cholangiography sclerosing cholangitis. To better visualize the smaller bile ducts, we used microcholangiography to determine the spectrum of biliary lesions in this and five other models of liver disease. METHODS: The models studied were as follows: (1) Surgically created jejunal, self-filling blind loops induce SBBO. (2) Intraperitoneal injection of a bacterial cell wall polymer, peptidoglycan-polysaccharide (PG-PS), causes granulomatous hepatitis. (3) Intraperitoneal injection of endotoxin (lipopolysaccharide) causes sinusoidal congestion and shock. (4) Bile duct ligation induces bile duct proliferation. (5) Alpha-naphthyl-isothiocyanate (ANIT) induces bile duct proliferation. (6) Carbon tetrachloride (CCl4) causes fibrosis and cirrhosis. Warmed barium sulfate, gelatin, and saline were injected in the extrahepatic bile duct. Liver slices (2 mm) underwent microradiographic techniques, and images were correlated with histology. RESULTS: Rats with SBBO had irregular and dilated extrahepatic bile ducts with thickened walls. Rats treated with endotoxin and CCl4 had normal microcholangiograms. Bile duct proliferation was identified following ANIT and bile duct ligation. Rats given PG-PS demonstrated irregular intrahepatic bile ducts. Microcholangiograms following SBBO and PG-PS showed similarities including focal ductal dilatation, narrowing, proliferation, and destruction. CONCLUSION: Various models of liver injury induce characteristic cholangiographic appearances. Microcholangiography is useful in examining biliary tract lesions and complements histology.     

Optic neuritis with residual tunnel vision in perchloroethylene toxicity

BACKGROUND: To identify and differentiate agenesis and severe atrophy of the right hepatic lobe on computed tomography (CT). METHODS: The CT examinations of three cases of agenesis and 11 cases of severe atrophy of the right hepatic lobe were reviewed. We evaluated visibility of the three hepatic veins, the two main portal veins (including their branches if necessary), the dilated intrahepatic ducts, enlargement of the medial and lateral segments of the left lobe and caudate lobe of the liver, presence of a retrohepatic gallbladder, hyperattenuation of the atrophic liver parenchyma, posterolateral interposition of the hepatic flexure of the colon, and upward migration of the right kidney. RESULTS: In the three cases of agenesis, no structure can be recognized as the right hepatic vein, right portal vein, or dilated right intrahepatic ducts. In the 11 cases of severe lobar atrophy, the right portal vein (or its branches) was recognized in eight cases, the right hepatic vein in four cases, and the dilated right intrahepatic ducts in 11 cases. The degree of enlargement of the lateral segment does not necessarily change inversely with the size of the medial segment and the caudate lobe. The retrohepatic gallbladder is present in eight cases (two in agenesis and six in atrophy). The phenomenon of hyperattenuation of the atrophic liver parenchyma was noted in six cases. CONCLUSION: Even though a retrohepatic gallbladder and a severely distorted hepatic morphology due to compensatory hypertrophy of the left and caudate lobes may raise a suspicion of agenesis of the right lobe of the liver, absence of visualization of all of the right hepatic vein, right portal vein and its branches, and dilated right intrahepatic ducts is a prerequisite of the diagnosis of agenesis of the right hepatic lobe on CT. In severe lobar atrophy, at least one of these structures is recognizable.     

Intra-bile duct growth of hepatocellular carcinoma: value of biliary dilatation on CT

In order to evaluate the usefulness of CT in demonstrating biliary invasion by hepatocellular carcinoma, 191 surgically proved cases were studied. Among 191 CT scans performed before surgery, six (3%) showed biliary dilatation. Pathological biliary invasion was found in eight cases (4%). Of these eight cases, four cases (50%) showed biliary dilatation on CT. In six cases with biliary dilatation on CT, pathological biliary invasion was revealed in four cases (67%). In two cases, the large (> or = 6cm) encapsulated tumors located in the hepatic hilum dilated the intrahepatic bile duct without intraductal tumor growth. We concluded that biliary dilatation on CT cannot be a sign of biliary invasion by hepatocellular carcinoma.     

A case of intrahepatic stones with an anomalous intrahepatic portal venous system

A 64-year-old woman who had undergone surgery for gallstones twice was admitted with epigastric pain and fever. Ultrasonography revealed gallstones in the dilated left lateral intrahepatic bile ducts. Abdominal computed tomography disclosed atrophy of the left lateral segment and the right lobe of the liver, and hypertrophy of the left medial segment and the caudate lobe. Percutaneous transhepatic cholangiography demonstrated that the stones were localized in the left lateral intrahepatic bile ducts, and that the choledochoduodenostomy previously established was patent. Percutaneous transhepatic portography revealed that the left portal vein was absent and the left medial segment was supplied by the right portal vein. Left lateral hepatic segmentectomy and release of the choledochoduodenostomy were performed. Postoperative recovery was good, and the patient is now well without any complaint. This report discusses the surgical treatment for intrahepatic stones under very complicated conditions and the relationship between intrahepatic stones and anomalous portal venous system.     

An approach for treating the hepatobiliary disease of cystic fibrosis by somatic gene transfer

Cystic fibrosis (CF) is an inherited disease of epithelial cell ion transport that is associated with pathology in multiple organ systems, including lung, pancreas, and liver. As treatment of the pulmonary manifestations of CF has improved, management of CF liver disease has become increasingly important in adult patients. This report describes an approach for treating CF liver disease by somatic gene transfer. In situ hybridization and immunocytochemistry analysis of rat liver sections indicated that the endogenous CFTR (cystic fibrosis transmembrane conductance regulator) gene is primarily expressed in the intrahepatic biliary epithelial cells. To specifically target recombinant genes to the biliary epithelium in vivo, recombinant adenoviruses expressing lacZ or human CFTR were infused retrograde into the biliary tract through the common bile duct. Conditions were established for achieving recombinant gene expression in virtually all cells of the intrahepatic bile ducts in vivo. Expression persisted in the smaller bile ducts for the duration of the experiment, which was 21 days. These studies suggest that it may be feasible to prevent CF liver disease by genetically reconstituting CFTR expression in the biliary tract, using an approach that is clinically feasible.     

Primary lithiasis of the bile ducts

An analysis is made of 24 cases of primitive lithiasis of the biliary tracts, viz; 10 of diffuse intrahepatic lithiasis, 9 of intrahepatic lithiasis localized in one of the ducts; and 5 of extrahepatic lithiasis. The most common cause were stenosis (14 cases) generally of idiopathic or congenital nature, followed by those of iatrogenic or tumoral origin. There were also cases of isolated or diffuse anomalus dilatation of the biliary ducts. A lithogenetic bile as well as infectious processes are rather considered secondary factors. The diagnosis of the real nature of the condiction was usually established in the postoperative period, this being an explanation why repeated surgery was so common (75%). There was a high postoperative mortality in the diffuse forms owing to severe manifestations of biliary sepsis perpetuated by the stones. Depending upon the individual cases the following procedures are proposed: 1) Biliodigestive anastomosis 2) Sphinteropapilloplasty 3) Dilatation and calibration of the stenosis 4) Postoperative instrumental extraction of calculi. The last-named procedure was carried out in 58% of the cases and result in an improvement in the serious prognosis. Primitive lithiasis should always be borne in mind when undertaking surgery of the biliary tracts in order to avoid additional surgery.     

Magnetic resonance cholangiography for evaluation of cholestatic jaundice in neonates and infants

Distinguishing extrahepatic biliary atresia from other causes of cholestasis in neonates and infants is important because surgical intervention before 2 months of age allows for long-term survival. The purpose of this prospective study was to evaluate the usefulness of magnetic resonance (MR) cholangiography in differentiating biliary atresia from other causes of cholestatic jaundice in neonates and infants. Nine anicteric infants (control group) aged 10 to 224 days (mean +/- SD, 8 +/- 65 days) and 15 neonates and infants with cholestatic jaundice, aged 22 to 142 days (mean +/- SD, 71 +/- 37) underwent MR cholangiography. The final diagnosis of extrabiliary atresia (6 patients) was based on laparotomy findings (4 patients) or autopsy (2 patients), while neonatal hepatitis (9 patients) was diagnosed according to the liver biopsy findings and clinical recovery during follow-up. Percutaneous liver biopsies were performed in all 15 patients. Results showed that the gall bladder and common bile duct (CBD) could be visualized using MR cholangiography in all patients in the control group. Nonvisualization of the CBD (6/6 patients) and demonstration of a small gall bladder (6/6 patients) characterized MR cholangiography findings in patients with biliary atresia. MR cholangiography failed to depict the CBD in one infant with hepatitis. We conclude that demonstration of the CBD by MR cholangiography in neonates and infants with cholestasis can be used to exclude the diagnosis of biliary atresia. In patients with cholestatic jaundice considered for exploratory laparotomy, preoperative MR cholangiography is recommended to avoid unnecessary surgery.     

Biliary malignancies

Biliary malignancies, including cancers of the intrahepatic and extrahepatic bile ducts, gallbladder and ampulla, should be considered in the differential diagnosis of patients with obstructive jaundice. Cancers of the intrahepatic bile ducts and ampulla are managed as liver and peri-ampullary tumours respectively. Extrahepatic bile duct cancers are diagnosed by cholangiography and evaluated for resectability by imaging and angiography. Vascular infiltration is the main contra-indication for resection, which may also involve the liver. Every attempt must be made to achieve curative resection, but local resection may be justified even if non-curative. Gallbladder cancers are usually advanced at the time of diagnosis and are unresectable--surgical palliation improves the quality of life by relieving biliary and gastric outlet obstruction. Long-term survival is possible after curative resection in early lesions that are usually diagnosed as an incidental finding after cholecystectomy for presumed gallstone disease. The role of adjuvant therapy in biliary malignancies needs further evaluation.     

Enhanced apoptosis relates to bile duct loss in primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is characterized by an immune-mediated destruction of intrahepatic small bile ducts. Apoptosis, a unique pattern of cell death, has been suggested to be responsible for the biliary destruction in PBC. To address this issue, we attempted to detect the apoptosis of biliary epithelial cells by in situ nick-end labeling and by the expression of apoptosis-related proteins using immunohistochemistry in patients with various hepatobiliary diseases, including PBC. The data was noteworthy for several reasons. First, apoptosis was occasionally detected on biliary cells in all liver specimens; however, the positive rate was high in PBC and relatively low in other livers. Strong expression of CD95 was frequently observed in the epithelial cells of the injured bile ducts of PBC, which accompanied high intensity CD95 ligand-expressing mononuclear cells. Perforin and granzyme B immunoreactivities were occasionally found on the bile ducts in control liver diseases as well as PBC, but granzyme B-positive biliary cells were prominent in PBC. In contrast, Lewis Y expression, as detected using BM-1 antibody, was consistently present in the injured bile ducts of PBC. These data suggest that apoptosis, via the perforin/granzyme B pathway, may be associated with the degrading fraction of cell cycle regulation in the small-sized biliary tree under physiological and pathological liver conditions. Moreover, enhanced apoptosis, mediated by CD95/CD95 ligand interaction, may contribute to the bile duct injury and loss observed in PBC.     

Use of the appendix to replace the choledochus

Ten cases of choledochal cyst (CC) were treated by biliary-appendicoduodenostomy. The follow-up comprised a patient interview, ultrasonography (US), and single-proton ejected computerized tomography (SPECT) scanning. In all cases an anti-reflux submucosal tunnel was added to the distal appendico-duodenostomy; all showed an uneventful postoperative course. All the dilated intrahepatic bile ducts had normalized on B-US postoperatively. Four children under went SPECT examination; all of them had patent neo-bile ducts. In the authors' opinion: (1) Anastomosing the cecal end of the appendix to the common hepatic duct seemed more favorable than the other way around, because the cecal end could be easily trimmed to the size of the common hepatic duct, which was more or less dilated in the presence of a CC; (2) It is necessary to add a submucosal tunnel to the distal appendicoduodenostomy to achieve a more reliable anti-reflux effect; and (3) Transposing the vascularized appendix through the retro-transverse colon simplified the procedure and might reduce the risk of retroperitoneal complications if bile leakage should occur.