Massive lithiasis of the intra and extrahepatic bile ducts. Presentation of a clinical case]

A case of massive intra and extrahepatic lithiasis in a 52 year-old-man is presented. Diagnosis was confirmed by ultrasonography, CT and percutaneous cholangiography. The value of the different diagnostic procedures is analyzed. The need for elective surgical treatment is insisted on in order to remove the majority of the calculi, treatment of stenosis and adequate biliary drainage. Our case was treated with an hepatico-jejuno-duodenostomy (end to side) on an isolated jejunal loop with sphincteroplasty for drainage of the distal choledochus. In the postoperative period the patient was treated with Methyl-tert-butyl-ether for dissolution of the remaining calculi.     

Pedicled fibroma of the extrahepatic bile ducts

It is reported on the very rare occurrence of a benign tumor in common hepatic bile duct of a 33-year-old woman, associated with obstructive jaundice. Clinical diagnosis and treatment are described, histological typing like fibroblastic tumor with neurogenic parts are discussed and a short review of the literature is given.     

Variations of intrahepatic and proximal extrahepatic bile ducts

BACKGROUND/AIMS: The modern segmental concept of the liver with a segmental bile duct distribution is of practical importance for biliary specialists and should include information on variations of intrahepatic and extrahepatic bile ducts. The aim of this study was to find biliary variations, especially on a segmental and sectorial level, and to try to arrange them into types. METHODOLOGY: We analyzed 51 corrosion casts of the human liver, which enabled the three-dimensional study of extrahepatic, sectorial, segmental and subsegmental bile ducts, their variations in course and confluencing, and the relationship between the structures of the portal pedicle. RESULTS: The types of confluence and their frequency were determined separately for the left, right, right anterior sectorial, right posterior sectorial and common hepatic ducts. "Normal" left confluence was formed in 82% and three variations in 18%. "Normal" right confluence was formed in 75% and four variations in 25%. A complete ("normal") anterior sectorial duct was present in 35% and four variations in 65%. A complete ("normal") posterior sectorial duct was present in 61% and four variations in 39%. CONCLUSIONS: The study showed that the biliary tree variations are quite frequent, and therefore clinically important, and that they could be arranged into several types.     

Alpha-fetoprotein-producing clear cell carcinoma of the extrahepatic bile ducts

A 79-year-old woman visiting our hospital with chief complaints of epigastric pain and jaundice was emergently admitted. Her alpha-fetoprotein (AFP) level was as high as 2265 ng/mL at admission. Her abdominal computed tomography scan revealed dilation of the intrahepatic bile duct and a tumorlike lesion protruding into the cystic duct and gallbladder from the junction between the middle portion of the bile duct and the right and left hepatic ducts. Surgery revealed a tumor extending from the extrahepatic bile duct (EHBD) to the cystic duct, with no intrahepatic tumor components. The tumor was histologically diagnosed as an AFP-producing cholangiocarcinoma of the clear cell type, originating from the EHBD. None of the previously reported cholangiocarcinomas of the AFP-producing clear cell type have been confined to the EHBD or have been resectable in a curative manner without hepatectomy.     

Biliary atresia and congenital disorders of the extrahepatic bile ducts

Biliary atresia (BA) and choledochal cysts are diseases of the intrahepatic and extrahepatic biliary tree. While their exact etiopathogeneses are not known, they should be treated promptly due to the potential for irreversible parenchymal liver disease. A diagnosis of BA may be easy or complicated, but should not be delayed. BA is always treated surgically, and performing the surgery before the age of 2 mo greatly increases its effectiveness and extends the time until the need for liver transplantation arises. While the more common types of choledochal cysts require surgical treatment, some can be treated with endoscopic retrograde cholangiopancreatography. Choledochal cysts may cause recurrent cholangitis and the potential for malignancy should not be ignored.     

Congenital cystic dilatation of the common bile duct, anomaly of the biliopancreatic junction and cancer of the bile ducts

The choledochus and pancreatic duct ordinarily enter the duodenum either separately or via a common channel located in the duodenal wall. The usual maximal length of the common channel is approximately 0.5 cm. An anomalous junction of the pancreaticobiliary tract is defined by the presence of an unusually long common channel whose length is 1 cm or longer. During the last 8 years, we studied 9 cases of congenital dilatation of the bile duct. Besides the choledochal cyst, all patients had a common channel which measured 1.5 cm or longer. The mean age of patients (6 females, 3 males) was 36.5 years. Three patients had extrahepatic bile tract carcinoma. One patient, a 51-year old man, had gallbladder carcinoma without stones while the two other patients, a 43-year old woman and a 32-year old man, had carcinoma arising from the wall of the cystic dilatation. These two patients had undergone a simple internal drainage procedure 16 and 8 years before, respectively. Our study suggests that the anomalous junction of the pancreaticobiliary tract, an embryonic anomaly which allows the reflux of pancreatic juice into the bile duct and gallbladder, may be responsible for congenital bile duct dilatation and biliary tract carcinoma. Total excision of the cystic dilatation and the gallbladder with hepaticojejunostomy is the appropriate surgical treatment.     

Diagnostic and therapeutic strategy in carcinoma of the extrahepatic bile ducts

The preoperative diagnostic approach in 25 patients with bile duct carcinoma was studied. Investigation procedures included gray-scale ultrasonography with guided fine needle biopsy of focal lesions, percutaneous transhepatic or retrograde cholangiography, transhepatic biliary drainage with brush and forceps biopsy of biliary stenosis as well as selective angiography for feasibility of surgical resection. Diagnosis was ensured in 23 patients by cytologic or histologic tissue specimens. Twenty-one lesions were judged unresectable on the basis of bile duct or vascular tumor extension and patient status. These patients were treated either by iridium 192 wire radiation therapy (6 cases) or by nonsurgical biliary drainage. Four patients with a potentially resectable tumor underwent surgery. This preoperative diagnostic approach can be recommended for selecting appropriate therapy in patients with bile duct carcinoma.     

Neuromas of the extrahepatic bile ducts as a cause of obstructive jaundice

We present four cases of extrahepatic bile duct neuromas causing obstructive jaundice. Two patients with neuromas localized in the hepatic duct had no evidence of gallstones or history of previous surgery. In these subjects, partial resection of the common bile duct and cholecystectomy was followed by hepatico-jejunostomy with Roux-en-Y reconstruction. In another patient, 7 years after cholecystectomy, a neuroma localized in the hepatic duct was treated by insertion of a T-tube. In the fourth patient, 9 years after laparoscopic cholecystectomy, partial common bile duct resection and Roux-en-Y hepatico-jejunostomy was performed. In all cases, the correct diagnosis of this very rare cause of extrahepatic bile duct obstruction was established postoperatively.     

Interstitial cells of Cajal are present in human extrahepatic bile ducts

Background and Aims: Interstitial cells of Cajal (ICC) are distributed with smooth muscle throughout the gastrointestinal tract and are involved in regulating motility. ICC were recently discovered in the wall of the human gallbladder. This study sought to determine whether ICC are present in human bile ducts. Methods: Biliary tract samples were obtained from several sources: surgical specimens (n = 16, 11 women, mean age 61 years); archival post‐mortem specimen (n = 1, 86 years, man); and cadavers (n = 2, 68 and 80 years, men). Paraffin‐embedded sections (3 µm) from the gallbladder (fundus, body and neck) and both extrahepatic and intrahepatic bile ducts were investigated. A double immunofluorescence protocol using polyclonal and monoclonal c‐kit antibodies and mast cell tryptase was used to distinguish c‐kit‐positive cells with typical ICC morphology from c‐kit‐positive mast cells. Small bowel samples were used as positive controls. ICC in the gallbladder were confirmed by ultrastructural study. Results: c‐kit‐positive cells with characteristic ICC morphology were identified in the subepithelial and muscular layers of the gallbladder and extrahepatic bile ducts. They were most prominent within the muscle layer of the extrahepatic bile ducts where they were organized into loosely arranged laminae running parallel to circular smooth muscle fibers. ICC were not found in intrahepatic bile ducts. Conclusion: This study demonstrates for the first time that ICC are present in human extrahepatic bile ducts where they are more densely aggregated than in the gallbladder. This cellular network is likely to be involved in biliary tract motility and its related disorders.