Hepatic portocholecystostomy for biliary atresia.

Four infants with biliary atresia had gross obliteration of the common hepatic duct but residual patency of the gallbladder, cystic duct and common bile duct. The patients were treated by hepatic portocholecystostomy utilizing the extant bile ducts for biliary reconstruction. Bile drainage was achieved in all four infants. There was a conspicuous absence of postoperative cholangitis. Subsequent obstruction of the distal ducts in two patients necessitated reoperation and construction of a standard biliointestinal conduit. The other two children are surviving, jaundice-free, 5 1/2 and 5 years after operation with minimal sequelae of biliary atresia. Hepatic portocholecystostomy is a feasible surgical alternative to intestinal reconstruction in patients with biliary atresia in whom the disease is limited to the proximal extrahepatic bile ducts.     

Magnetic resonance cholangiography for the diagnosis of biliary atresia

Purpose: The aim of this study was to evaluate the usefulness of magnetic resonance cholangiography (MRC) for the diagnosis of biliary atresia in infantile cholestatic jaundice. Methods: Forty-seven consecutive infants with cholestatic jaundice underwent single-shot MRC. The diagnosis of biliary atresia was made by MRC based on the nonvisualization of extrahepatic bile ducts and excluded on the basis of the complete visualization of extrahepatic bile ducts. The final diagnosis of biliary atresia (BA group, n = 23) or nonbiliary atresia (NBA group, n = 24) was established by operation or clinical follow-up until the jaundice resolved. Results: The extrahepatic bile ducts including the gallbladder, the cystic duct, the common bile duct, and the common hepatic duct were visualized in 23 of the 24 infants of the NBA group. The extrahepatic bile ducts, except the gallbladder, were not depicted in any infant of the BA group. MRC had an accuracy of 98%, sensitivity of 100% and specificity of 96%, for diagnosis of biliary atresia as the cause of infantile cholestatic jaundice. Conclusions: MRC is a very reliable noninvasive imaging modality for the diagnosis of biliary atresia. In infants with cholestatic jaundice and considered for exploratory laparotomy, MRC is recommended to avoid unnecessary surgery.     

腹腔镜胆囊切除术中肝外胆道解剖异常的防范

目的探讨腹腔镜胆囊切除术（1aparoscopic cholecystectomy，LC）中肝外胆道异常的诊断及处理。方法1999年10月～2008年6月1216例LC中，发现15例（1．2％）胆道解剖异常。3例胆囊管异常粗、短，开口在左右肝管汇合部；1例胆囊颈部结石嵌顿，胆总管较细，向上牵拉胆囊使胆总管走行移位；1例胆囊管与肝总管并行后低位开口，1例胆囊管在胆总管右侧回旋扭曲，开口于右肝管侧壁，2例胆囊壶腹部粘连严重，覆盖于胆总管及肝总管前方；3例在胆床附近见迷走胆管走行；3例在分离胆囊管时发现右后肝管开口于肝总管；1例Mirizzi综合征解剖不清。仔细分离，丝线结扎或上钛夹处理，解剖不清者中转开腹。结果13例顺利完成LC；2例（13．3％）中转开腹，其中1例副右肝管损伤，1例Mirizzi综合征。无腹腔内出血、腹腔感染、肠道损伤及死亡等严重并发症。15例随访3个月～4年，其中〉1年11例，无胆道狭窄及残余结石。结论LC术中精细解剖胆囊三角，确切辨认各管道关系，是预防胆道异常情况下肝外胆道损伤的关键。     

Eosinophilic cholangitis with obstructive jaundice mimicking bile duct carcinoma

A 55-year-old male who presented with obstructive jaundice and radiographically documented extrahepatic biliary tract obstruction is reported. Eosinophilic infiltration of the gallbladder, common bile duct, intrahepatic bile ducts, and bone marrow was observed. Eosinophilic cholangitis, a rare inflammatory condition that clinically resembles a biliary malignancy, should be taken into consideration in the differential diagnosis in the evaluation of presumed neoplasm of the bile ducts.     

Agenesis of the extrahepatic bile ducts: report of five cases

Five patients were encountered in whom agenesis either of the proximal extrahepatic biliary ducts (four patients) or total absence of the extrahepatic bile ducts and gall bladder (one patient) were evaluated. Jaundice was diagnosed from at birth to 3 weeks of age (average, 1.2 weeks of age) in these five patients. The patients' ages ranged from 2 to 8 weeks at the time of surgical exploration. Findings at surgery showed either absence of the entire extrahepatic biliary ducts or proximal bile duct remnants, no evidence of an inflammatory process, and no fibrous mass present at the portahepatis. Liver biopsy specimens showed histological evidence of cholestasis, minimal bile duct proliferation and fibrosis, and nearly complete absence of inflammation. In three patients in whom a portocholecystostomy was performed, no bile flow was obtained. Two patients underwent surgical exploration and liver biopsy only. One patient died as a result of severe congenital heart disease at 3 months of age. Four patients have undergone successful hepatic transplantation. These patients are now 10 months to 6 years of age. In our review of the literature, we were unable to find any reports of bile duct agenesis despite the fact that it appears to be a known phenomenon. We conclude that patients with biliary agenesis have early onset of jaundice when compared with patients with biliary atresia, absence of inflammation at the portahepatis at the time of surgical exploration, as well as on biopsy of the liver. Portoenterostomy or portocholecystostomy are likely to fail. We believe that liver transplantation is the treatment of choice for this rare entity.     

医源性胆道损伤52例报告

目的:总结医源性胆道损伤的经验教训。方法:对过去33年间5 2例医源性胆道损伤进行回顾性分析。结果:肝外胆道手术所致4 8例,胃大部切除术及肝脏手术所致各2例。损伤部位在肝总管与胆总管交界处34例,肝总管6例,胆总管6例,左右肝管汇合部4例,左、右肝管各1例。胆管完全性损伤30例,部分性损伤2 2例。结论:要警惕医源性胆道损伤的发生,及早诊断并修复胆道的连续性是提高疗效的关键     

Aberrant biliary ducts and cysto-hepatic ducts. A comprehensive study]

Though very different, aberrant bile ducts and cysto-hepatic ducts are often confused. Aberrant bile ducts are abnormal ducts which do not drain any segment or sector of the liver. They are filled of bile counter-flow and can be injured not only in the gallbladder bed, but also elsewhere on the surface of the liver. Cysto-hepatic ducts are normal ducts, draining segment or sector of the liver, but because of an embryologic sliding, their branching is on the gallbladder or on the cystic duct. All these ducts can be injured during cholecystectomy, and it would be of importance to recognize the true type of duct one has to deal with, by radiologic explorations. The management is different as aberrant bile ducts need only to be ligated, instead, the cysto-hepatic ducts may require a reimplantation in the common bile duct or Roux en y loop. We discuss all these problems on the basis of 1200 traditional cholecystectomies where 1 aberrant bile duct, 3 cysto-hepatic ducts, and 3 external biliary fistulas were encountered.     

Primary sclerosing cholangitis in which differential diagnosis from gallbladder carcinoma was difficult

We report a case of localized primary sclerosing cholangitis (PSC) which was difficult to distinguish from gallbladder carcinoma. A 75-year-old woman with elevated serum bilirubin was hospitalized and underwent endoscopic nasobiliary drainage (ENBD). There was no history of diseases such as gallbladder stone, pancreatitis, or ulcerative colitis. Cholangiography through the ENBD tube showed localized stenosis of the common bile duct; the gallbladder could not be seen. Angiography showed no encasement of the hepatic artery. Ultrasonography showed a tumor in the cystic duct, and the tumor had invaded the gallbladder and common bile duct. We diagnosed gallbladder carcinoma on radioimaging, and performed an S4aS5 subsegmentectomy of the liver and resection of the extrahepatic biliary tree. Pathologically, no malignant cells were detected, and fibrosis around bile ducts and infiltration of inflammatory cells into hepatic tissue were found. It is well known that PSC is sometimes difficult to differentially diagnose from cholangiocarcinoma. Our case is of high interest because ultrasonography showed findings suggestive of gallbladder carcinoma. It is therefore necessary to keep the possibility of PSC in mind for the diagnosis and treatment of such localized biliary stenosis.     

Absence of intestinal bile promotes bacterial translocation.

Previously, the authors documented that extrahepatic biliary obstruction promotes the systemic translocation of bacteria from the intestine to visceral tissues. The current experiments were performed to determine whether it was the absence of intestinal bile or the presence of biliary obstruction that promoted bacterial translocation. Four groups of rats were studied: 1) nonoperated controls (n = 20), sham common bile duct-ligated (n = 22), common bile duct-ligated (n = 25), and common bile duct-diverted (choledochovesical bypass) (n = 23). The sham-ligated group underwent laparotomy and manipulation of the portal region; whereas the ligated group had their common bile ducts ligated, while the choledochovesical group had a silastic tube placed from the common bile duct to the bladder. Seven days later, at death, the incidence of bacterial translocation was higher in the groups of rats subjected to common bile duct ligation (41%) or diversion (32%) than in the control (3%) or sham-ligated (5%) groups (P less than 0.05). Histologic sections of ileums of ligated and diverted animals both showed subepithelial edema. These findings suggest that it is primarily the absence of bile in the intestine that promotes mucosal injury and bacterial translocation and not biliary obstruction.     

豚鼠肝外胆道不同部位组织缺血-再灌注损伤的比较观察

目的比较肝外胆道不同部位组织缺血-再灌注损伤的特点及差异。方法制成豚鼠肝外胆道缺血-再灌注模型,观察不同部位组织缺血30min再灌注60min后,上皮细胞线粒体平均体积（     

Fluorescence cholangiography during laparoscopic cholecystectomy: a feasibility study on early biliary tract delineation

Background

Laparoscopic cholecystectomy (LC) is one of the most commonly performed laparoscopic procedures. Bile duct injury is a rare but serious complication during this procedure, mostly caused by misidentification of the extrahepatic bile duct anatomy. Intraoperative cholangiography may be helpful to reduce the risk of bile duct injury; however, this is not a common procedure worldwide. Near-infrared fluorescence cholangiography (NIRFC) using indocyanine green (ICG) is a promising alternative for the identification of the biliary tree. This prospective observational study was designed to assess the feasibility and image quality of intermittent NIRFC during LC, using a newly developed laparoscopic fluorescence system.

Methods

Consecutive patients undergoing elective LC were included and received a single intravenous injection of ICG directly after induction of anesthesia. During dissection of the base of the gallbladder and the cystic duct, the extrahepatic bile ducts were visualized by using a dedicated laparoscope, which offers both conventional state-of-the-art imaging and fluorescence imaging. Intraoperative recognition of the biliary structures was registered at set time points, as well as the establishment of the critical view of safety.

Results

Fifteen patients were included between December 2011 and May 2012. ICG was visible in the liver and bile ducts within 20 min after intravenous administration and remained for approximately 2 h, using the fluorescence mode of the laparoscope. The common bile duct and cystic duct could be clearly identified at an early stage of the operation and, more important, significantly earlier than with the conventional camera mode. No per- or postoperative complications occurred as a consequence of ICG use.

Conclusions

Intermittent fluorescence imaging using a newly developed laparoscope and preoperative administration of ICG seems a useful aid in accelerating visualization of the extrahepatic bile ducts during laparoscopic cholecystectomy.     

Clinical problems in the surgical treatment of pancreaticobiliary maljunction

The results of surgical treatment of pancreaticobiliary maljunction at our department are described. The 67 patients who underwent surgery for this disease were divided by age into an adult group (45 patients, aged 16 years and over) and a pediatric group (22 patients, aged less than 16 years). The incidence of concomitant carcinoma before surgery and the incidence and severity of postoperative cholangitis were compared between these two groups. In addition, the cell proliferating activity of the biliary tract epithelium in cancer-free patients was compared between the two groups, using the proliferating cell nuclear antigen labeling index (PCNA LI). Ten patients (all adults) were diagnosed with cancer (gallbladder carcinoma in 7 and bile duct carcinoma in 3) before surgery. The surgical techniques used for reconstruction in the cancer-free patients were: in the adult group, hepaticoduodenostomy in 9 patients, Roux-en-Y hepaticojejunostomy in 17, jejunal interposition in 8, and another technique in 1. In the pediatric group, hepatico-duodenostomy was performed in 17 patients. Roux-en-Y hepaticojejunostomy in 3, and jejunal interposition in 2. Postoperative cholangitis occurred in 6 adults (including 2 with severe form) and 1 child (mild case). The PCNA LI of the biliary tract epithelium was high compared to control findings in the biliary tract epithelium of 10 adult patients without pancreaticobiliary maljunction. In the adult group with dilated extrahepatic bile ducts (n=10 examined) this index was 11.4% for the bile duct epithelium (control, 1.5%) and 12.7% for the gallbladder epithelium (control, 1.4%). In the adult group with non-dilated extrahepatic bile ducts (n=5 examined) it was 5.9% for the bile duct epithelium and 13.1% for the gallbladder epithelium. In the pediatric group (n=10 with extrahepatic bile duct dilatation) it was 7.5% for the bile duct and 9.7% for the gallbladder epithelium. (Differences from control values were all significant.) These results suggest that surgery for this disease should be performed as early as possible and that extrahepatic bile duct excision and biliary reconstruction should be performed whether or not extrahepatic bile ducts are dilated.     

New clues for the developing human biliary system at the porta hepatis

The human biliary system is formed from the hepatic diverticulum, a structure which develops from the embryonic foregut in the fourth week of gestation. The cephalic portion of the hepatic diverticulum lies within the septum transversum, and gives rise to entodermal cells which become the primitive hepatocytes. The caudal part of the hepatic diverticulum is molded by mesenchyme to form the gallbladder, cystic duct, and extrahepatic bile duct. The gallbladder is initially tubular in shape, and undergoes morphological changes to become saccular during the 11th week of gestation. The extrahepatic bile duct elongates and widens as gestation progresses, and intramural mucus glands develop. There is no solid stage during the development of the extrahepatic bile duct. The extrahepatic bile duct is a well-defined tubular structure by the 6th week of gestation, whereas the intrahepatic biliary system during this period of gestation is represented by the primitive ductal plate. The ductal plate undergoes structural changes from the 11th week of gestation, beginning at the porta hepatis and progressing through gestation to the periphery of the liver. This remodeling process shapes the ductal plate from a flat sheath of biliary epithelium surrounding the portal vein branches into a network of interconnecting tubular structures. Mesenchyme plays an important role in ductal plate remodeling. The intrahepatic biliary system is in luminal continuity with the extrahepatic bile duct throughout gestation at the porta hepatis. The major bile ducts at the porta hepatis are fully formed by the 16th week of gestation. Received: September 30, 2000 / Accepted: January 10, 2001     

Gallbladder cancer spreading into the aberrant cystic duct: First literature report

Introduction and importanceAlthough variations from the standard anatomy of the extrahepatic bile ducts are common, duplication of the cystic duct draining a single gallbladder is an extremely rare variant. We herein describe the first report of gallbladder cancer spreading into the aberrant cystic duct.Case presentationA 60-year-old female presented with upper abdominal pain, and she was diagnosed with gallbladder cancer. Intraoperatively, she was found to have a duplicated cystic duct draining a single gallbladder, and her cancer had spread into the aberrant cystic duct entering the anterior right hepatic duct. Right hepatectomy with extrahepatic bile duct resection was performed to achieve R0 resection.Clinical discussionIn the English literature, 28 cases of duplicated cystic duct draining a single gallbladder have been reported. However, no cases of gallbladder cancer have been described in these previous reports.ConclusionWe report the first case of gallbladder cancer spreading into the aberrant cystic duct. To perform an oncologically adequate operation, exact assessment of the biliary tree is essential not only preoperatively but also intraoperatively.     

Human "pancreatic" bladder. Two case reports

The aim of this study is to report two similar cases with an "accessory biliary duct" confluent to the main pancreatic duct. There was pancreatic juice inside the "gallbladder". There was no connection between "accessory biliary duct" and intra or extrahepatic biliary ducts. This anomalous junction of the "cystic duct" and the main pancreatic duct may be explained by embryology. These two cases could be the first human "pancreatic" bladders reported.     

Successful surgical treatment of hepatocellular carcinoma invading into biliary tree.

A 41-year-old woman was admitted to hospital with obstructive jaundice. Computed tomography showed a large mass in the right hepatic lobe and marked dilatation of the biliary tree in the left lateral segment of the liver. Angiography showed evidence of neovascularity. Percutaneous transhepatic cholangiography revealed complete obstruction of the common bile duct just below the bifurcation. The serum level of alpha-fetoprotein on admission was 1,080,000 ng/ml. These findings suggested to us a primary hepatocellular carcinoma invading the intrahepatic bile duct. Extended right lobectomy and hepaticojejunostomy for bile drainage was carried out. The patient is doing well 3 years after surgery. Hepatocellular carcinoma (HCC) invading to the portal vein is not so rare, but invasion into the bile duct is much less common. In 1947, Mallory described a single case of HCC invading the gallbladder and obstructing extrahepatic bile ducts. In 1975, Lin termed this HCC "Icteric type hepatoma". The incidence of such HCC in Japan was reported to be 1.9-9%. Obstructive jaundice is a clinical manifestation of the terminal stage in HCC. We describe here our treatment of a woman with HCC invading the common bile duct. Right extended lobectomy and reconstruction of hepaticojejunostomy were effective.     

The surgery of "correctable" biliary atresia

Seven of 114 (6%) patients treated in Denver for biliary atresia had a variant of the disease in which only microscopic bile ducts or ductules were identified at the porta hepatis, hepatic lesions were present, even in the neonatal example, and intrahepatic biliary hypoplasia was uniformly present. The excised bile duct cysts consisted of fibrosis or scar tissue and with little or no epithelial lining. Six patients had corrective surgery. The first patient was treated by choledochoenterostomy and became totally obstructed. Definitive operation in this patient and all subsequent patients consisted of excision of all extrahepatic duct structures and Roux-en-Y portoenterostomy. Two patients died, the one who did not have surgical correction and a second who had correction at age 35 weeks. The other five patients have been followed for 18 to 158 months and are anicteric but have some degree of residual liver damage. We conclude that so-called correctable biliary atresia occurs in less than 10% of cases, that because of coincident biliary hypoplasia, "cure" is not a possible outcome, and that the treatment of choice is complete surgical excision of the extrahepatic bile ducts and Roux-en-Y portoenterostomy.     

Biliary atresia registry, 1976 to 1989

The Registry provides information about 904 children with biliary atresia from more than 100 institutions. There was a 1.4 to 1 female predominance; racial distribution was 62% caucasian, 20% black, 11% Hispanic, 4.2% asian, and 1.5% American Indian. Eight hundred sixteen (90%) underwent corrective surgery (median age at operation, 69 days). Intraoperatively, 70% had totally obliterated extrahepatic bile ducts, 22% had patency of the gallbladder and distal common duct, whereas only 8% had "correctable" biliary atresia (proximal duct patency). A variety of reconstructions were used, but the majority of patients had a Roux-en-Y portoenterostomy with or without exteriorization. Follow-up was available for 670 children (74%) with average length of follow-up of 5 years (range, 1 to 16 years). Five-year actuarial survival was 48% following Kasai's operation, but was less than 10% (at 3 years) if no operative correction was done. Survival was unaffected by sex, type of reconstruction, or cholangitis. Predictors of a bad outcome were (1) caucasian race; (2) operative age greater than 60 days; (3) presence of cirrhosis at initial biopsy; (4) totally nonpatent extrahepatic ducts; (5) absent ducts at the level of transection in the liver hilus; and (6) subsequent development of varices or ascites. Identification of factors predictive of the ultimate outcome provide a basis for either continued efforts with management of Kasai's operation or for early referral for liver transplantation.