Neuroendocrine tumors of the extrahepatic biliary tree are extremely rare malignancies accounting for 0.2-2.0% of all gastrointestinal carcinoid tumors. Neuroendocrine tumors obstructing the biliary tree are extremely difficult to diagnose preoperatively and nearly impossible to differentiate from cholangiocarcinoma. Statistically, the most common anatomic location in the biliary tree is the common bile duct, followed by the perihilar region. Herein, we present a case of a small cell neuroendocrine carcinoma of the hilum in a 79-year-old man following laparotomy. To our knowledge, this is the first case of small cell type neuroendocrine carcinoma of hilar bile duct reported in Korea.
Aged ; Bile Duct Neoplasms/*diagnosis/pathology/surgery ; Cholangiopancreatography, Endoscopic Retrograde ; Diagnosis, Differential ; Hepatic Duct, Common/pathology ; Humans ; Male ; Neuroendocrine Tumors/*diagnosis/pathology/surgery ; Tomography, X-Ray Computed

BACKGROUND: Knowledge of bile duct anatomy is largely obtained through cholangiography. However, it is sometimes difficult to follow the intrahepatic segmental bile ducts and to define the extent of pathology precisely. Basically, the images on cholangiography are projected ones, so they do not provide the spatial concept. In contrast, sequential slices of CT contain information on the threedimensional structure. Purpose : We aimed to assess the intrahepatic bile duct anatomy by examining the CT. METHODS: The spiral CT images of 42 patients with bile duct dilatation were examined serially from above downward. Intrahepatic bile ducts were followed up to the third-order branches, classified according to the level of branching and compared with those on cholangiography. Further, the relationship between the segmental bile ducts and portal veins were evaluated. RESULTS: At or below the level of convergence of the ventral(V) and dorsal(D) branches of VIII segment, the posterior bile duct(Bp) arises from right or common hepatic duct(VDP, or VD-P). Below this level, the Bp descends more than 8mm and branches into VI segmental branch(B6) and the posterior trunk(Pr), or it branches directly into B6 and Pr without definite descending portion(Pd). VDP-Pd- Pr,B6(high level of branching of Bp with Pd) was observed in 22, VDP-Pr,B6(high level of branching of Bp without Pd) in 3, VD-P-Pd-Pr,B6(low level of branching of Bp with Pd) in 12, and VD-P-Pr,B6(low level of branching of Bp without Pd) in 4. These findings were closely correlated with those on cholangiography. Regarding the relationship of the segmental bile ducts and portal veins(PV), all right anterior bile ducts were located superior-medial to the PV, and the trunk of right posterior bile duct(Pr) ran superior-lateral to PV in 38 and superior-medial in 4 patients. Most segmental branches of VI and VII segments ran anterior-lateral-superior to PV(VI:39/40, VII:17/18). The Bp coursed above(and behind) the right anterior portal vein(APV) in 39, and below(and in front of) the APV in 3, of which 2 cases had separate and low origin of Bp from common hepatic duct. CONCLUSION: CT was useful to understand intrahepatic bile duct anatomy.
Bile ; Bile Ducts ; Bile Ducts, Intrahepatic* ; Cholangiography ; Dilatation ; Hepatic Duct, Common ; Humans ; Pathology ; Portal Vein ; Tomography, Spiral Computed

Caroli's syndrome is a rare congenital disorder that involves intrahepatic bile duct ectasia and congenital hepatic fibrosis, frequently seen with concomitant autosomal recessive polycystic kidney disease (ARPKD). Literature on infants with ARPKD is rare. Here, we present a case of a two month old boy who was diagnosed with Caroli's syndrome and ARPKD.
Polycystic Kidney, Autosomal Recessive/complications/*diagnosis/pathology ; Male ; Kidney/ultrasonography ; Infant ; Humans ; Hepatic Duct, Common/pathology/ultrasonography ; Caroli Disease/complications/*diagnosis/pathology

As recent advances in chemotherapy and surgical treatment have improved outcomes in patients with biliary cancers, the search for an optimal strategy for relief of their obstructive jaundice has become even more important. Without satisfactory relief of biliary obstruction, many patients would be ineligible for treatment. We review all prospective randomized trials and recent retrospective non-randomized studies for evidence that would support such a strategy. For distal malignant biliary obstruction, an optimal strategy would be insertion of metallic stents either endoscopically or percutaneously. Evidence shows that a metallic stent inserted percutaneously has better outcomes than plastic stents inserted endoscopically. For malignant hilar obstruction, percutaneous biliary drainage with or without metallic stents is preferred.
Bile Duct Neoplasms/pathology/surgery ; Biliary Tract Diseases/pathology/*surgery ; Cholangiocarcinoma/pathology/surgery ; Decompression, Surgical ; Drainage/methods ; Endoscopy ; *Evidence-Based Medicine ; Hepatic Duct, Common ; Humans ; Jaundice, Obstructive/pathology/*surgery ; Klatskin's Tumor/pathology/surgery ; Stents

We have reported a case of Mirizzi syndrome preoperatively diagnosed using MR cholangiopancreatography. MRCP and T2-weighted image using a single-shot fast spin-echo sequence accurately depicted all components of Mirizzi syndrome, including impacted stone in the neck of the gallbladder compressing the common hepatic duct and wall-thickening of the gallbladder without any evidence of malignancy. The combination of MRCP and T2-weighted image can be counted on to replace conventional modalities of diagnosing Mirizzi syndrome without any loss of diagnostic accuracy.
Bile Duct Diseases/etiology* ; Bile Duct Diseases/diagnosis ; Case Report ; Cholangiopancreatography, Endoscopic Retrograde ; Cholelithiasis/diagnosis ; Cholelithiasis/complications* ; Constriction, Pathologic/etiology ; Gallbladder/pathology ; Hepatic Duct, Common* ; Human ; Magnetic Resonance Imaging* ; Male ; Middle Age ; Syndrome

Obstructive jaundice by vascular compression is rare. The causative arteries were identified as the right hepatic artery, gastroduodenal artery, cystic artery, proper hepatic artery, and an unspecified branch of the common hepatic artery. Also the venous system, such as enlarging collateral veins in cases of portal hypertension was a causative vessel. Herein, we describe a case of a proximal choledocholithiasis due to compression of the common bile duct by right hepatic artery originated from gastroduodenal artery. Final diagnosis and treatment were achieved through an operation.
Cholangiography ; Common Bile Duct/blood supply/*pathology/surgery ; Diagnosis, Differential ; *Hepatic Artery ; Humans ; Jaundice, Obstructive/*diagnosis/etiology/radiography ; Male ; Middle Aged ; Tomography, X-Ray Computed

A 50-year-old woman complained of jaundice and dyspepsia that started 2 weeks prior to consultation. Abdomen-pelvic computed tomography showed a 3 cm mass in the right hepatic duct with central calcification, which was spreading into the second branch. Repeated biopsies through endoscopic retrograde cholangiopancreatography were needed for pathology, which was consistent with an adenocarcinoma. Imaging studies including positron emission tomography showed no evidence of distant metastasis. The patient underwent right lobectomy with bile duct resection. The final diagnosis was intrahepatic cholangiocarcinoma with central calcification. We reported a very rare case of centrally calcified mass growing in the second branch of the right hepatic duct. The possibility of intrahepatic cholangiocarcinoma with central calcification should be considered for differential diagnosis of intrahepatic calcification.
Adenocarcinoma ; Bile Ducts ; Biopsy ; Cholangiocarcinoma* ; Cholangiopancreatography, Endoscopic Retrograde ; Diagnosis ; Diagnosis, Differential ; Dyspepsia ; Female ; Hepatic Duct, Common ; Humans ; Jaundice ; Middle Aged ; Neoplasm Metastasis ; Pathology ; Positron-Emission Tomography

Hepatocelluar carcinoma presenting as a biliary duct tumor thrombus is a relatively rare entity, with poor prognosis. The primary clinical manifestation of this disease is obstructive jaundice, which can often be misdiagnosed. A 59-year-old female patient was admitted with sudden onset of abdominal pain. Laboratory tests suggested obstructive jaundice, and enhanced magnetic resonance imaging of the upper abdomen did not show obvious biliary dilatation. Endoscopic ultrasound and endoscopic retrograde cholangiopancreatography suggested an occupying lesion in the upper bile duct. SpyGlass and biopsy finally confirmed hepatocellular carcinoma with right hepatic duct tumor thrombus hemorrhage. The SpyGlass Direct Visualization System, as an advanced biliary cholangioscopy device, showed the advantages of single-person operation as well as easy access to and visualization of the lesion.
Female ; Humans ; Middle Aged ; Carcinoma, Hepatocellular/diagnostic imaging* ; Jaundice, Obstructive/etiology* ; Liver Neoplasms/diagnostic imaging* ; Hepatic Duct, Common/pathology* ; Thrombosis/complications* ; Hemorrhage/complications*

OBJECTIVETo explore the role of extrahepatic control on blood flow of hepatic vein and inferior vena cava in hepatectomy, and observe its effect on minimizing hemorrhage.

METHODSFrom 2001 to April 2003, 33 patients who had liver tumors involving segment IV, VII, VIII or half liver underwent major hepatectomies that required exposure of the inferior vena cava and main trunks of hepatic veins, during which the major hepatic veins and inferior vena cava were isolated and taped to control blood flow when necessary.

RESULTSIn 33 attempts, 32 were successful and all tumors were resected successfully. The placement of occlusion tape was unsuccessful in 1 case. 7 cases did not need blood transfusion during operation. The amount of blood transfusion for other cases were form 0 to 1 600 ml. there was no operative mortality.

CONCLUSIONSAppropriate control of main truck of hepatic vein and inferior vena cava is effective in reducing blood loss during hepatectomies. It is also very helpful for performing difficult hepatectomies.

Adult ; Aged ; Carcinoma, Hepatocellular ; surgery ; Female ; Hepatectomy ; adverse effects ; methods ; Hepatic Duct, Common ; surgery ; Hepatic Veins ; surgery ; Humans ; Liver ; blood supply ; pathology ; Liver Cirrhosis ; etiology ; Liver Neoplasms ; surgery ; Male ; Middle Aged ; Treatment Outcome ; Vena Cava, Inferior ; surgery

We report here on a case of extrahepatic biliary cystadenoma arising from the common hepatic duct. A 42-year-old woman was evaluated by us to find the cause of her jaundice. Ultrasonography and CT showed a cystic dilatation of the common hepatic duct and also marked dilatation of the intrahepatic duct. Direct cholangiography demonstrated a large filling defect between the left hepatic duct and the common hepatic duct; dilatation of the intrahepatic duct was also demonstrated. Following excision of the cystic mass, it was pathologically confirmed as a unilocular biliary mucinous cystadenoma arising from the common hepatic duct.
Adult ; Bile Duct Neoplasms/*diagnosis ; Cholangiopancreatography, Magnetic Resonance ; Choledochal Cyst/*diagnosis ; Cystadenoma/*diagnosis ; Diagnosis, Differential ; Female ; Hepatic Duct, Common/*pathology/radiography/ultrasonography ; Humans ; Tomography, X-Ray Computed ; Ultrasonography, Interventional