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.     

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.     

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.     

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.     

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.     

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.     

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.     

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。     

Endoscopic ultrasound‐guided biliary drainage of hilar biliary obstruction

Only 20–30% of patients with hilar cholangiocarcinoma (CC) are candidates for potentially curative resection. However, even after curative (R0) resection, these patients have a disease recurrence rate of up to 76%. The prognosis of hilar cholangiocarcinoma (CC) is limited by tumor spread along the biliary tree leading to obstructive jaundice, cholangitis, and liver failure. Therefore, palliative biliary drainage may be a major goal for patients with hilar CC. Endoscopic retrograde cholangiopancreatography (ERCP) with stent placement is an established method for palliation of patients with malignant biliary obstruction. However, there are patients for whom endoscopic stent placement is not possible because of failed biliary cannulation or tumor infiltration that limits transpapillary access. In this situation, percutaneous transhepatic biliary drainage (PTBD) is an alternative method. However, PTBD has a relatively high rate of complications and is frequently associated with patient discomfort related to external drainage. Endoscopic ultrasound‐guided biliary drainage has therefore been introduced as an alternative to PTBD in cases of biliary obstruction when ERCP is unsuccessful. In this review, the indications, technical tips, outcomes, and the future role of EUS‐guided intrahepatic biliary drainage, such as hepaticogastrostomy or hepaticoduodenostomy, for hilar biliary obstruction will be summarized.     

Implantation metastasis along the percutaneous transhepatic biliary drainage sinus tract

We describe herein the case of a 75-year-old man with metastatic tumor seeding at the percutaneous transhepatic biliary drainage tract that occurred following a pylorus-preserving pancreatoduodenectomy for carcinoma of the distal common bile duct. On postoperative day 30, the catheter was removed and ethanol was injected into the percutaneous transhepatic biliary drainage sinus tract to prevent cancer implantation. One year and 3 months after the initial operation, abdominal computed tomography showed dilation of the left lateral segmental bile ducts and a 2-cm mass. The location of this mass corresponded to the puncture point from the previously performed percutaneous transhepatic biliary drainage. Implantation of the bile duct carcinoma at the percutaneous transhepatic biliary drainage sinus tract was diagnosed, and the recurrent tumor was successfully resected by performing a left hepatic lobectomy. Currently, 1 year after the second operation, the patient is in good health without any signs of recurrence. This case report demonstrates the importance of resecting the percutaneous transhepatic biliary drainage sinus tract during the initial surgery. If left in place, careful follow-up and awareness of this mode of tumor recurrence may lead to a timely resection, with preservation of a good quality of life and long-term survival.     

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.     

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.     

全腹部薄层冠状面单次激发快速自旋回波序列MRI检查诊断小儿胆管疾病的价值分析

目的 探讨全腹部薄层冠状面单次激发快速自旋回波序列MRI在小儿肝内外胆管成像中的应用。方法 2014年10月~2016年9月收治的26例胆道系统疾病患儿（胆道闭锁症8例,胆道扩张症患儿14例,胆囊结石2例,胆管结石2例）,所有患儿均采取全腹部薄层冠状面单次激发快速自旋回波序列扫描,其中9例患儿在延迟15~20 min后采取MRI增强扫描。观察所有患儿的肝内外胆道和各级分支的解剖全貌,分析其走行分布和通畅情况等。结果 胆道闭锁症患儿肝内胆道均能够正常显示,而其中胆囊形态细小并且未见到肝外胆道显影者2例,肝外胆道和胆囊无显影6例。4例患儿肝脾肿大,2例患儿肝门区有三角形长T₂信号影;在胆道扩张症患儿中有11例患儿出现胆总管梭形扩张,有1例患儿表现为Caroli病,有2例患儿出现肝门区囊性包块,有4例患儿出现有胆囊增大和肝内胆道扩张;在胆系结石患儿中有3例患儿表现为胆总管局部管腔信号不均匀、短T₂信号的充盈缺损,胆囊结石患者表现为胆囊颈部短T₂信号的充盈缺损;与相同层面的扫描图像相比,延迟增强MRI扫描显示出肝脏、胰腺实质信号都有所减低,与胆胰管内长T₂信号的组织对比度则有所增加,其胆道系统显示更为清晰。结论 相对于常规腹部MRI扫描而言,全腹部薄层冠状面单次激发快速自旋回波序列能够更加细致地观察患者胆道系统解剖结构,更为全面地了解患者病变全貌和是否存在其他脏器异常,临床应用具有很大的诊断意义。     

Endoscopic ultrasound-guided transesophageal cholangiodrainage and consecutive endoscopic transhepatic Wallstent insertion into a jejunal stenosis

In cases where the papilla of Vater is unreachable because of pyloric/duodenal stenosis, or a catheter cannot be introduced into the papilla, or with recurrent tumor growth, or after previous gastrointestinal surgery, percutaneous transhepatic cholangiodrainage (PTCD) is considered to be the therapeutic alternative in cholestasis. The purpose of this report was to demonstrate that endoscopic ultrasound (EUS)-guided transesophageal cholangiodrainage is a feasible alternative in patients who decline to undergo PTCD. A 67-year-old female patient with recurrent tumor growth at the hepaticojejunostomy 17 months after a formerly resected cholangiocarcinoma (pT3, pN0 (0/2), M0, G2, R0; extended right hemihepatectomy), cholangitis, and peritoneal carcinomatosis underwent an EUS-guided transesophageal procedure to obtain cholangiodrainage by (i) puncture of a branch of the biliary tree at the left hepatic site, (ii) insertion of a guide wire into the bile duct and the anastomosed jejunum using the rendezvous technique with endoscopic retrograde cholangiopancreatography (ERCP)/conventional endoscopy, (iii) transesophagohepatic placement of an 8.5-Fr. double pigtail catheter, and (iv) transhepatic placement of a Wallstent through the jejunal stenosis, resulting in complete alleviation of the biliary and jejunal obstruction. There were no severe complications such as perforation or bleeding and no stent occlusion within the patient's lifetime of more than 3 months. Death was related to progressive tumor growth. EUS-guided transesophageal cholangiodrainage, here described in combination with Wallstent placement, is a reasonable, feasible, and encouraging treatment alternative in selected patients where conventional ERCP or PTCD is not an option.