A rare surgical case of metachronous double carcinoma of the biliary tract

We report on a rare case of metachronous double carcinoma of the biliary tract, occurring in a 65-year-old male. The patient was admitted to the hospital with jaundice in March 2004. Ultrasonography, computed tomography (CT) and magnetic resonance imaging (MRI) scans of the abdomen showed a minimally dilated intrahepatic biliary tree with normal-appearing choledocus. Obstruction of the common hepatic duct was revealed by endoscopic retrograde cholangiopancreatography (ERCP). The patient underwent a resection of the middle third of the extrahepatic duct and cholecystectomy (cholangiocarcinoma, pT1N0M0), with the surgical margins of resection showing as negative. After 2 years, during follow-up, the findings of a positron emission tomography (PET)-CT scan suggested a possible cholangiocarcinoma of the distal part of the biliary tract; CT and MRI scanning of the abdomen showed mild dilatation of the distal common hepatic duct; an ERCP showed mild dilatation of the retropancreatic remnant of the biliary tree with endoluminal defects. Eventually the patient underwent pancreaticoduodenectomy. The histopathological diagnosis of the resected specimen confirmed a cholangiocarcinoma; 10 lymph nodes were negative (pT1N0M0). At 6 months post-op after the second operation the patient is progressing well with no signs of recurrence. Patients with cholangiocarcinoma – in whom survival is prolonged with surgical resection – should undergo careful follow-up for both recurrence and second primary cancer. PET scanning seems to play the most important diagnostic role.     

Duct-to-duct biliary reconstruction after radical resection of Bismuth Ⅲ a hilar cholangiocarcinoma

At present, radical resection remains the only effective treatment for patients with hilar cholangiocarcinoma. The surgical approach for R0 resection of hilar cholangiocarcinoma is complex and diverse, but for the biliary reconstruction after resection, almost all surgeons use Roux-en-Y hepaticojejunostomy. A viable alternative to Roux-en-Y reconstruction after radical resection of hilar cholangiocarcinoma has not yet been proposed. We report a case of performing duct-to-duct biliary reconstruction after radical resection of Bismuth Ⅲa hilar cholangiocarcinoma. End-to-end anastomosis between the left hepatic duct and the distal common bile duct was used for the biliary reconstruction, and a singlelayer continuous suture was performed along the bile duct using 5-0 prolene. The patient was discharged favorably without biliary fistula 2 wk later. Evidence for tumor recurrence was not found after an 18 mo follow- up. Performing bile duct end-to-end anastomosis in hilar cholangiocarcinoma can simplify the complex digestive tract reconstruction process.     

Bile duct disease: prospective comparison of ERCP, CT, and fat suppression MRI.

The authors compared computed tomography (CT) and endoscopic retrograde cholangiopancreatography (ERCP), techniques commonly used to study the biliary tree, with pre- and post-Gd-DTPA breath-hold fast low angle shot (FLASH) and fat suppressed spin-echo in 28 consecutive patients with bile duct abnormalities detected on ERCP, including 11 patients with malignant disease and 17 patients with benign disease. ERCP, CT, and magnetic resonance (MR) images were prospectively interpreted in a blinded fashion and reviewed by consensus. ERCP characterized all cases of malignant disease by the presence of a narrowed bile duct lumen with irregular margins. CT and MRI detected all cases of malignant disease and characterized nine of 11 as malignant. In seven of these cases, CT and MRI showed thickening of extrahepatic bile duct walls greater than 5 mm. MRI images showed intrahepatic-enhancing periportal tissue in four cases, which was not seen on CT images, and which was biopsy-proven tumor extension. Benign disease was characterized on ERCP images by the demonstration of smooth tapered narrowings in 16 cases, whereas on CT and MR images it was characterized by mild to moderate dilatation of the intrahepatic bile ducts and wall thickness less than 5 mm in 13 cases. Overall ERCP correctly characterized 27 cases as benign or malignant and CT and MRI both characterized 25. The results of this study show a trend that ERCP is superior to CT and MRI for characterizing bile duct disease.     

Adenomyoma of the common hepatic duct

BackgroundAdenomyoma occurs most commonly in the fundus of the gallbladder, seldom in other parts of the gallbladder and rarely in the extrahepatic biliary tree, where most lesions are localised to the common bile duct or papilla of Vater. Adenomyoma of the common hepatic duct is extremely rare. To the best of our knowledge, only three cases have been reported so far.Case outlineA 51-year-old woman was admitted with a three month history of attacks of right upper abdominal pain, nausea, vomiting and fever. Laboratory data, ultrasonography, ERCP and CT confirmed slight cholestasis and proximal bile duct dilatation due to a tumour within the common hepatic duct. Cholecystectomy was performed with excision of the suprapancreatic common bile duct including the convergence of the hepatic ducts plus lymphadenectomy and Roux-en-Y hepaticojejunostomy. Frozen section histology showed the benign nature of the lesion and a tumour-free resection line. Final histology showed adenomyoma. The patient has remained symptomfree for more than 30 months.DiscussionAlthough adenomyoma is a benign lesion and the surgical strategy has not been established, complete excision with frozen section is recommended to exclude small malignant foci and local recurrence as well as to avoid surgical over-treatment.     

Extracystic biliary carcinoma associated with anomalous pancreaticobiliary junction and cysts

Anomalous pancreaticobiliary junction with cystic dilatation of the biliary tract is usually associated with carcinoma arising from the cyst wall. We report an extracystic location of biliary carcinoma in the presence of anomalous pancreaticobiliary junction and cysts in a patient with obstruction of the origin of the left hepatic duct who underwent hepatobiliary resection. Cholangiocarcinoma was found to have arisen in a noncystic left hepatic duct, in conjunction with cystic dilatation involving both the cystic and common bile ducts. The present case supports a relationship between anomalous pancreaticobiliary junction and biliary carcinogenesis that may affect the extracystic biliary tree.     

Biliary cystadenocarcinoma associated with atrophy of the left hepatic lobe and hepatolithiasis mimicking intrahepatic cholangiocarcinoma: a case report

Biliary cystadenocarcinoma and its benign counterpart, biliary cystadenoma, are rare hepatic cystic tumors arising from the hepatobiliary epithelium. We report the case of a 68-year-old Taiwanese woman who presented initially with acute cholangitis. A series of imaging studies including abdominal ultrasound, computerized tomography, endoscopic retrograde cholangiopancreatography, and percutaneous transhepatic cholangiography showed bilateral intrahepatic duct (IHD) and common bile duct (CBD) stones with IHD and CBD dilatation, and an ill-defined tumor within the atrophied left hepatic lobe. The patient underwent surgical resection of the tumor and choledocholithotomy. The pathologic diagnosis was biliary cystadenocarcinoma. We review this rare disease entity and discuss its unusual radiologic features mimicking intrahepatic cholangiocarcinoma.     

Surgical management of cholangiocarcinoma

Biliary tract cancer affects approximately 7500 Americans each year. Tumors arising from the gallbladder are the most common; those of bile duct origin, or cholangiocarcinoma, are less frequently encountered, constituting approximately 2% of all reported cancers. Although cholangiocarcinoma can arise anywhere within the biliary tree, tumors involving the biliary confluence (i.e., hilar cholangiocarcinoma) represent the majority, accounting for 40 to 60% of all cases. Twenty to 30% of cholangiocarcinomas originate in the lower bile duct, and approximately 10% arise within the intrahepatic biliary tree and will present as an intrahepatic mass. Complete resection remains the most effective and only potentially curative therapy for cholangiocarcinoma. For all patients with intrahepatic cholangiocarcinoma and nearly all patients with hilar tumors, complete resection requires a major partial hepatectomy. Distal cholangiocarcinomas, on the other hand, are treated like all periampullary malignancies and typically require pancreaticoduodenectomy. Most patients with cholangiocarcinoma present with advanced disease that is not amenable to surgical treatment, and even with a complete resection, recurrence rates are high. Adjuvant therapy (chemotherapy and radiation therapy) has not been shown clearly to reduce recurrence risk.     

Obstructive jaundice after cholecystectomy

In this article we present the case of a 72 year-old woman who three years after laparoscopic cholecystectomy develops obstructive jaundice. An MRI of the liver and biliary system revealed an hiliar mass that caused dilatation of the biliary tree. The patient underwent hepatic duct resection and reconstruction via hepaticojejunostomy. The histological examination of the surgical specimen identified an intramural biliary neuroma with no evidence of malignancy.     

A case of asymptomatic intraductal papillary neoplasm of the bile duct without hepatolithiasis

A 65-year-old woman was found to have dilatation of the intrahepatic bile duct in the right anterior segment during a general health. Laboratory data were within normal ranges and no solid mass was detected in her abdominal computer tomography （CT） or nuclear magnetic resonance imaging （MRI）. However, endoscopic retrograde cholangiopancreatography （ERCP） demonstrated an obstruction of the right bile duct. Intraoperative cholangiography showed stenosis of the intrahepatic bile duct in the anterior inferior segment （B5） and narrowness of the intrahepatic bile duct in the anterior superior segment （B8）, so that we strongly suspected intrahepatic cholangiocarcinoma （ICC）. Histologically, surgically resected liver specimens, without tumor mass by macroscopic observation, showed intraductal papillary proliferation with fibrovascular cores and intraductal spreading of carcinoma in situ throughout a considerable area, especially in bile ductules around the peripheral small portal area. Furthermore, the immunohistochemical profile of the tumor （MUC5AC＋/CK7＋） was compatible with an intraductal papillary neoplasm of the bile duct （IPN-B）. Consequently, this case was diagnosed as IPN-B with spreading CIS, stageⅠ（pT1, pN0, P0, H1, M0）. We report a case of IPN-B with interesting histopathologicalfindings and emphasize that cholangiography is especially helpful for the diagnosis of bile duct dilatation due to infiltration of carcinoma cells.     

Flowcharts for the management of biliary tract and ampullary carcinomas

No strategies for the diagnosis and treatment of biliary tract carcinoma have been clearly described. We developed flowcharts for the diagnosis and treatment of biliary tract carcinoma on the basis of the best clinical evidence. Risk factors for bile duct carcinoma are a dilated type of pancreaticobiliary maljunction (PBM) and primary sclerosing cholangitis. A nondilated type of PBM is a risk factor for gallbladder carcinoma. Symptoms that may indicate biliary tract carcinoma are jaundice and pain in the upper right area of the abdomen. The first step of diagnosis is to carry out blood biochemistry tests and ultrasonography (US) of the abdomen. The second step of diagnosis is to find the local extension of the carcinoma by means of computed tomography (CT), magnetic resonance imaging (MRI), magnetic resonance cholangiopancreatography (MRCP), percutaneous transhepatic cholangiography (PTC), and endoscopic retrograde cholangiopancreatography (ERCP). Because resection is the only way to completely cure biliary tract carcinoma, the indications for resection are determined first. In patients with resectable disease, the indications for biliary drainage or portal vein embolization (PVE) are checked. In those with nonresectable disease, biliary stenting, chemotherapy, radiotherapy, and/or best supportive care is selected.     

Bronchobiliary fistula and cholangiocarcinoma: a case report and principles of management

A 64-year-old woman was admitted with fever and cough. At admission, she had jaundice, hepatomegaly, and green-stained sputum. Computed tomography (CT) showed an intrahepatic abscess located near the dome, multiple hepatic metastases, biliary tract dilatation, and a right pleural effusion. Percutaneous transhepatic cholangiography demonstrated a communication between the intrahepatic biliary ducts and the bronchial tree. The patient was treated with antibiotic therapy, pleural and biliary drainages and a percutaneous drainage of the hepatic abscess.     

Biliary tract schwannoma: A rare cause of obstructive jaundice in a young patient

Schwannoma is a tumor derived from Schwann cells which usually arises in the upper extremities, trunk, head and neck, retroperitoneum, mediastinum, pelvis, and peritoneum. However, it can arise in the gastrointestinal tract, including biliary tract. We present a 24-year-old male patient with obstructive jaundice, whose investigation with computed tomography abdomen showed focal wall thickening in the common hepatic duct, difficult to differentiate with hilar adenocarcinoma. He was diagnosed intraoperatively schwannoma of common bile duct and treated with local resection. The patient recovered well without signs of recurrence of the lesion after 12 mo. We also reviewed the common bile duct schwannoma related in the literature and evaluated the difficulty in pre and intraoperative differential diagnosis with adenocarcinoma hilar. Resection is the treatment of choice for such cases and the tumor did not recur in any of the resected cases.     

Endoscopic retrograde cholangiopancreatography in infants and children.

BACKGROUND: Although many reports describe the use of diagnostic endoscopic retrograde cholangiopancreatography (ERCP) in children, few mention therapeutic application of this technique in pediatric patients with pancreatic or biliary disease. METHODS: We report our 4-year experience of 80 ERCPs performed in 59 children for obstructive jaundice or cholestasis with dilatation of the biliary tree (32 children), biliary atresia (11), recurrent pancreatitis (8), and blunt trauma to the abdomen (8). RESULTS: The patients' ages ranged from 5 weeks to 18 years. The appropriate duct was cannulated in 94% of cases. Common bile duct sphincterotomy was performed in 35 patients and pancreatic duct sphincterotomy in one. Multiple procedures were done in 16 patients where biliary stents were inserted; in one patient with chronic pancreatitis and pancreaticolithiasis, pancreatic stent was inserted. Four patients developed mild pancreatitis, one had moderate pancreatitis and one had leak of contrast, which was treated by administration of clear fluids orally for one day. One patient with benign stenosis of the hepatic duct developed cholangitis after migration of the stent into the bowel lumen. CONCLUSION: Diagnostic and therapeutic ERCP can be done as safely and effectively in pediatric patients as in adults.     

ERCP in the diagnosis of extrahepatic biliary atresia

This study assessed the usefulness of ERCP in the diagnosis of biliary atresia. We evaluated 57 infants with prolonged cholestasis with abdominal ultrasound, liver biopsy, and ERCP. Using clinical observations alone, 22 infants were thought to have biliary atresia; whereas 35 children were thought to have neonatal hepatitis. The ERCP was performed with a prototype duodenoscope and was successful in all infants except two with biliary atresia. In 20 infants three types of radiological findings consistent with biliary atresia were seen: type 1, no visualization of biliary tree (35%); type 2, opacification of the distal common duct and gallbladder without visualization of the main hepatic duct (35%); and type 3, opacification of the distal common duct, the gallbladder, and a segment of the main hepatic duct with biliary lakes at the porta hepatis (30%). Twenty-five of 35 infants with suspected neonatal hepatitis were excluded because of a liver biopsy that was diagnostic. In the remaining 10 infants the liver biopsy had some features of extrahepatic biliary atresia and ERCP was performed prior to surgery. A normal extrahepatic biliary tree was obtained in all of them. In conclusion, ERCP permits the visualization of the biliary tree in young infants and is useful in selecting those infants who should be considered for exploratory laparotomy.     

SUPINE POSITION ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY IN A PATIENT WITH SITUS AMBIGUOUS WITH POLYSPLENIA

A 58‐year‐old woman complained of painless jaundice. The serology showed total bilirubin 10.6 mg/dL with direct bilirubin of 7.0 mg/dL. Abdominal computed tomography (CT) scan disclosed an abnormal arrangement of the abdominal viscera and dilation of the biliary tree. A nearly 1.4 cm‐sized periampullary mass was seen. These findings are compatible with situs ambiguous with polysplenia and were suggestive of a periampullary tumor. Due to her unusual anatomical features, the patient underwent an endoscopic retrograde cholangiopancreatography (ERCP) in the supine position instead of in the conventional prone position. ERCP showed that the common bile duct (CBD) diameter was increased to 20 mm. Microscopic findings of the biopsy specimen of papillary mass were compatible with an adenocarcinoma of the ampulla of Vater. The clinical stage was stage IA (T1N0M0). Eight days later, a papillectomy was carried out by endoscopic snare resection. Six months later, follow‐up studies, including ERCP, abdominal CT and 18‐fluorodeoxyglucose positron emission tomography (¹⁸‐FDG PET)‐CT scan, showed no evidence of recurrence. Although the success rate of supine position ERCP may be influenced by the extent of the intestinal malrotation and the position of the duodenum, we conclude that supine position ERCP can be carried out effectively in a patient with situs anomaly.     

Fascioliasis: a report of five cases presenting with common bile duct obstruction

Fascioliasis is a zoonotic infection caused by Fasciola hepatica. It is rarely seen with icterus caused by obstruction of the common bile duct. We report five patients with obstructive jaundice due to Fasciola hepatica, who were diagnosed and managed with endoscopic retrograde cholangiopancreatography (ERCP). All cases were admitted to hospital with complaints of icterus and pain in the right upper quadrant of the abdomen; their biochemical values were interpreted as obstructive jaundice. Ultrasound and computer tomography (CT) revealed biliary dilatation in the common bile duct, but did not help to clarify the differential diagnosis. ERCP showed the presence of Fasciola hepatica in the common bile duct. After removing the flukes, the symptoms disappeared and the biochemical values returned to normal. Biliary fascioliasis should be considered in the differential diagnosis of obstructive jaundice. This report confirms the diagnostic and therapeutic role of ERCP in patients with obstructive jaundice caused by biliary fascioliasis.     

Endoscopic retrograde cholangiopancreatography in hepatic alveolar echinococcosis

Background and Aim:  Hepatic alveolar echinococcosis (HAE) involves both the vascular and biliary structures of the liver. Endoscopic retrograde cholangiopancreatography (ERCP) is said to be an alternative for the diagnosis and treatment of biliary complications of HAE. We present here our experience with ERCP in HAE.
Methods:  We followed 13 patients who underwent ERCP for the treatment of biliary complications of HAE in the endoscopy unit of our clinic at Ataturk University School of Medicine, Erzurum between January 2002 and June 2008.
Results:  Eight men and five women were followed up. Mean age was 43.2 (24–64 years). All patients had non-resectable HAE. Indications for ERCP were biliary fistula in seven patients, obstructive jaundice in five patients and cholangitis in one patient. Endoscopic sphincterotomy (ES) was carried out in 12 patients, and in one patient with biliary leakage, a stent was inserted into the right hepatic branch. ERCP findings were dilated common bile duct, irregular narrowing and distortion of the common bile duct and common hepatic duct, communication with the cystic cavity or biliocutaneous fistula and complete disappearance of the biliary tree above the level of the common hepatic duct or hepatic bifurcation. In patients with biliary leakage, biliary drainage decreased only in two patients after ERCP and in patients with obstructive jaundice, the high bilirubin levels decreased in only one patient.
Conclusion:  ERCP showed structural changes of the external biliary tract and ES has a limited effect on these changes and stents can be used in selected cases.     

Pseudomonas aeruginosa liver abscesses following endoscopic retrograde cholangiography

Summary We report a case ofPseudomonas aeruginosa liver abscesses following endoscopic retrograde cholangiopancreatography (ERCP) in a patient without evidence of biliary tract disease and of any known cause of hepatic infection. Computer tomography (CT) scan was the best method of diagnosis, allowing, through guided percutaneous puncture of the abscesses, isolation of the organism, which was sensitive to carbenicillin. One month of antibiotherapy with repeated aspirations of the largest abscesses was successful. This report suggests that ERCP may induce cholangitic sepsis by inoculating pathogens in the biliary tree even in the absence of extrahepatic obstruction.     

25例肝包虫囊肿破入胆道的诊治体会

目的探讨肝包虫囊肿破入胆道的诊断及治疗方式。方法回顾分析我院2001年～2011年行手术治疗的25例肝包虫囊肿破入胆道患者的临床表现、实验室检验、影像学检查、手术方式及治疗效果。结果超声、CT、磁共振胰胆管成像(MRCP)及内镜逆行胰胆管造影(ERCP)对于肝包虫囊肿破入胆道均具有良好的诊断价值,其中ERCP诊断价值最高,确诊率可达100%。25例患者中22例手术方式为胆囊切除、胆总管探查、T管引流+肝包虫残腔引流,其余3例行胆囊切除、胆总管探查、T管引流+肝包虫病灶根治性切除。所有患者均痊愈出院。结论超声因普及易行,应作为诊断肝包虫囊肿破入胆道的首选辅助检查,MRCP检查具有诊断准确率高和无创等优点,ERCP则对肝包虫囊肿破入胆道诊断率最高。胆囊切除、胆总管探查、T管引流+肝包虫残腔引流应作为肝包虫囊肿破入胆道首选手术方式,对于部分复杂病例可行胆囊切除、胆总管探查、T管引流+肝包虫病灶根治性切除,效果良好。     

Metachronous double cancer of the gallbladder and pancreas associated with pancreaticobiliary maljunction

A 50-year-old Japanese woman complained of abdominal and back pain. Ten years previously she had undergone cholecystectomy, choledochectomy, and Roux-en-Y choledochojejunostomy for gallbladder cancer associated with pancreaticobiliary maljunction without bile duct dilatation. On the present admission, ultrasonography (US) and computed tomography (CT) demonstrated a large mass, 60 mm in size, in the pancreatic tail. Endoscopic retrograde cholangiopancreatography (ERCP) showed obstruction of the main pancreatic duct in the tail of the pancreas and revealed that the pancreatic duct was joined to the bile duct 25 mm above the papilla of Vater. The patient underwent distal pancreatectomy, splenectomy, left adrenalectomy, and partial gastrectomy. Histological examination revealed moderately differentiated ductal adenocarcinoma that had invaded to the proper muscle of the stomach. Double cancer of the gallbladder and pancreas in a patient with pancreaticobiliary maljunction is rare. Although the etiology of cancer of the pancreas associated with pancreaticobiliary maljunction is unclear, we should pay close attention to the pancreas as well as the biliary tract during the long-term follow-up of patients with pancreaticobiliary maljunction after they have undergone a choledochojejunostomy.