MR cholangiography of congenital biliary malformations in infancy

We presented MR cholangiography (MRC) of congenital biliary malformations in infancy. MRC was obtained during induced sleeping. In two cases of congenital dilation of bile duct, MRC revealed cystic or spindle dilatation of intra- and extra hepatic bile ducts. In one biliary atresia, MRC revealed the serpentine gall bladder and cystic dilatation of the extrahepatic bile duct without connection to the dilated hilar bile duct. MR cholangiography, which can be obtained noninvasively, is useful for the diagnosis and the preoperative assessment of congenital biliary malformations in infancy.     

Crohn''s disease and sclerosing cholangitis: CT and PTC diagnosis, with MR findings

Computed tomography (CT), percutaneous transhepatic cholangiography (PTC) and magnetic resonance imaging (MRI) findings in a case of sclerosing cholangitis associated with Crohn's disease of the colon and terminal ileum are described. CT gives additional information on dilatation of peripheral bile ducts and confirms findings of PTC, i.e. a decreased arborization of the biliary tree, a nodular appearance of the common bile duct and multifocal bile duct strictures. CT findings could be recognized on MRI which provided no additional information.     

Biliary complications of Fasciola hepatica: the role of endoscopic retrograde cholangiography in management

The parasite Fasciola hepatica resides in the biliary tree but rarely causes significant clinical sequelae. In this report, we review our experience with four patients in whom F. hepatica infection resulted in biliary complications, especially severe biliary colic and jaundice. The diagnosis was achieved with endoscopic retrograde cholangiography which demonstrated the worms in the extrahepatic bile ducts. Endoscopic sphincterotomy was performed uneventfully in all patients allowing balloon extraction of the parasites and resolution of their symptoms.     

Laparoscopic intracorporeal ultrasound. An alternative to cholangiography?

The purpose of this study was to compare laparoscopic intracorporeal ultrasound (LICU) examination of the biliary duct system with cholangiography for delineation of duct anatomy and determination of presence or absence of ductal calculi. Thirty-one patients had LICU examination of the extrahepatic bile ducts after exposure of the gallbladder but prior to dissection of the cystic duct. After LICU examination, cystic duct dissection and cholangiography were done. Evaluation of duct anatomy and decision for duct exploration were based on findings of both tests. All patients had successful LICU examination and 30 had successful cholangiography. Duct size as determined by LICU corresponded precisely with cholangiography. LICU provided useful anatomical information in two patients with aberrant anatomy and detected cholangiogram. LICU aids in delineation of biliary duct anatomy and accurately determines presence or absence of duct calculi.     

Biliary malignancies

Biliary malignancies, including cancers of the intrahepatic and extrahepatic bile ducts, gallbladder and ampulla, should be considered in the differential diagnosis of patients with obstructive jaundice. Cancers of the intrahepatic bile ducts and ampulla are managed as liver and peri-ampullary tumours respectively. Extrahepatic bile duct cancers are diagnosed by cholangiography and evaluated for resectability by imaging and angiography. Vascular infiltration is the main contra-indication for resection, which may also involve the liver. Every attempt must be made to achieve curative resection, but local resection may be justified even if non-curative. Gallbladder cancers are usually advanced at the time of diagnosis and are unresectable--surgical palliation improves the quality of life by relieving biliary and gastric outlet obstruction. Long-term survival is possible after curative resection in early lesions that are usually diagnosed as an incidental finding after cholecystectomy for presumed gallstone disease. The role of adjuvant therapy in biliary malignancies needs further evaluation.     

A microcholangiographic study of liver disease models in rats

RATIONALE AND OBJECTIVES: Rats develop hepatobiliary injury due to small bowel bacterial overgrowth (SBBO) that, at specimen, resembles cholangiography sclerosing cholangitis. To better visualize the smaller bile ducts, we used microcholangiography to determine the spectrum of biliary lesions in this and five other models of liver disease. METHODS: The models studied were as follows: (1) Surgically created jejunal, self-filling blind loops induce SBBO. (2) Intraperitoneal injection of a bacterial cell wall polymer, peptidoglycan-polysaccharide (PG-PS), causes granulomatous hepatitis. (3) Intraperitoneal injection of endotoxin (lipopolysaccharide) causes sinusoidal congestion and shock. (4) Bile duct ligation induces bile duct proliferation. (5) Alpha-naphthyl-isothiocyanate (ANIT) induces bile duct proliferation. (6) Carbon tetrachloride (CCl4) causes fibrosis and cirrhosis. Warmed barium sulfate, gelatin, and saline were injected in the extrahepatic bile duct. Liver slices (2 mm) underwent microradiographic techniques, and images were correlated with histology. RESULTS: Rats with SBBO had irregular and dilated extrahepatic bile ducts with thickened walls. Rats treated with endotoxin and CCl4 had normal microcholangiograms. Bile duct proliferation was identified following ANIT and bile duct ligation. Rats given PG-PS demonstrated irregular intrahepatic bile ducts. Microcholangiograms following SBBO and PG-PS showed similarities including focal ductal dilatation, narrowing, proliferation, and destruction. CONCLUSION: Various models of liver injury induce characteristic cholangiographic appearances. Microcholangiography is useful in examining biliary tract lesions and complements histology.     

Routine intraoperative cholangiography and its contribution to the selective cholangiographer

Routine intraoperative cholangiography (IOC) during cholecystectomy is controversial. In order to address this debate, we asked the following questions: What intraoperative information is provided to the surgeon? What IOC criteria or standards are necessary to observe this information? Between 1990 and 1993, 624 laparoscopic cholangiography (LC) cases were performed at Virginia Mason Hospital, during which 86% (535) of the patients underwent successfully performed IOCs. Each of these cholangiograms was sought, and 420 (78%) were reviewed by a radiologist and a surgeon. Specific items involved the presence or absence of filling defects, bile duct diameter, contrast leaks, flow into the duodenum, benign or malignant stricture, contrast in a portion of the pancreatic duct, and anomalous ducts. "Relevant findings" were defined as filling defects, stricture, leaks, and the following anomalous ducts: a bile duct from the right side of the liver entering near or into the cystic duct. The entire biliary tree was visualized in 86%, and the bifurcation was seen in 95% of the cases. Considering these deficiencies, we found a 10% incidence of filling defects. Anomalies were common in the biliary tree (39%), and knowledge of the presence of some of them are important for safe dissection (at least 4%). Also, at least 68 relevant findings would have been missed in 420 LC cases without IOC. If the IOC had not visualized the biliary tree proximal to the cystic duct, 30 of 68 or 44% of these findings would not have been observed. If an IOC is performed on a routine or selective basis, the study should visualize the entire biliary tree.     

Value of intraoperative cholangiography in laparoscopic cholecystectomy

By introduction of laparoscopic cholecystectomy, an increase of accidental common bile duct injuries up to 1.2% has been reported. In the present study of 325 cholecystectomies we evaluated whether mandatory intraoperative cholangiography (IOC) can reduce the rate of accidental bile duct injuries or, at least, identify them early in order to make an adequate repair possible. In addition 163 patients underwent preoperative intravenous cholangiography (IVC). Both imaging techniques were compared with regard to their sensitivity in the detection of anatomic variations and stones of the extrahepatic bile duct system. Our results demonstrated a great advantage of the IOC. The IOC was feasible in 98.1% of the cases and presented a complete depiction of the extrahepatic bile duct system in 99.3%. IVCs showed the biliary system in 91.4% of the cases but without visualization of the cystic duct in 51.5% and the hepatic confluence in 16%. Anatomic variations of the bile duct system which consecutively influenced the operative management were found in additional 27.6% exclusively by IOC. 71.4% of bile duct stones were not detected by IVC. The intraoperative time consumption of IOC was unimportant. The x-ray-load was clearly lower by a factor of 3.5. There was no complication after IOC. In comparison, 6.1% of patients demonstrated an anaphylactic reaction by IVC. One common duct injury (0.3%) was detected intraoperatively by IOC and at the same operation treated without postoperative complications. In conclusion, we recommend an IOC in addition to a thorough preoperative ultrasound-examination. By this technique intraoperatively identified stones of the common bile duct can be sufficiently treated by postoperative endoscopic extraction and anatomic variations of the bile duct system will be visualized and therefore accidental injuries will be avoided.     

Cardiac transplantation in patients aged 60 years or older

The indications and best technique for peroperative cholangiography during laparoscopic cholecystectomy remain unclear, but the operation has been associated with an increased use of preoperative endoscopic retrograde cholangiography. Cystic duct cholangiography, particularly in the hands of the trainee, can be time consuming, and bile duct injury may be caused by attempts to cannulate the cystic duct. This study analyses 113 consecutive patients undergoing peroperative cholangiography through the gallbladder, or cholecystocholangiography. It was successful in 92 (81.4%) patients, the procedure adding less than 10 min to the operating time. There were no cholangiogram-related complications. Common anatomical variations included both short and particularly wide cystic ducts. This information helps to minimize the risk of damage to the common bile duct. This study demonstrates that cholecystocholangiography is a safe, simple, and effective alternative to cystic duct cholangiography with virtually no "learning curve." It provides a "road-map" of biliary anatomy and identifies common bile duct stones prior to the commencement of dissection. Unsuccessful cholecystocholangiography does not preclude the use of cystic duct cholangiography later in the operation. Difficult anatomy is demonstrated prior to dissection. When unsuspected bile duct calculi necessitate open exploration, further laparoscopic dissection is avoided.     

Persistent obstructive jaundice, cholangitis, and biliary cirrhosis due to common bile duct stenosis in chronic pancreatitis

Long strictures of the intrapancreatic portion of the common bile duct were found in 6 patients with chronic pancreatitis. These strictures were responsible for painless obstructive jaundice, recurrent cholangitis, secondary biliary cirrhosis, and chronic abdominal pain difficult to distinguish from that caused by pancreatitis. Endoscopic retrograde cholangiopancreatography and intraoperative cholangiography were invaluable in making the diagnosis and in planning surgical correction. Decompression of the biliary tree by anastomosis of the gallbladder or common duct to the small intestine completely relieved symptoms and allowed liver function to improve significantly. Common duct stricture as a complication of chronic pancreatitis should be considered in the differential diagnosis of extrahepatic biliary obstruction and whenever surgical treatment of chronic pancreatitis is contemplated.     

Psychopathology as adaptive development along distinctive pathways

The vast majority of post-operative bile duct strictures occur following cholecystectomy, these injuries having been seen at an increased frequency since the introduction of laparoscopic cholecystectomy. Bile duct injuries usually present early in the post-operative period, obstructive jaundice or evidence of a bile leak being the most common mode of presentation. In patients presenting with a post-operative bile duct stricture months to years after surgery, cholangitis is the most common symptom. The 'gold standard' for the diagnosis of bile duct strictures is cholangiography. Percutaneous transhepatic cholangiography is generally more valuable than endoscopic retrograde cholangiography in that it defines the anatomy of the proximal biliary tree that is to be used in surgical reconstruction. The most commonly employed surgical procedure with the best overall results for the treatment of bile duct stricture is a Roux-en-Y hepaticojejunostomy. The results of the surgical repair of bile duct strictures are excellent, long-term success rates being in excess of 80% in most series. Recent data have suggested that, at intermediate follow-up of approximately 3 years, an excellent outcome can be obtained following repair of bile duct injuries after laparoscopic cholecystectomy. Percutaneous and endoscopic techniques for the dilatation of bile duct strictures can be useful adjuncts to the management of bile duct strictures if the anatomical situation and clinical scenario favour this approach. In selected patients, the results of both endoscopic and percutaneous dilatation are comparable to those of surgical reconstruction.     

New Gaba-containing analogues of human growth hormone-releasing hormone (1-30)-amide: I. Synthesis and in vitro biological activity

BACKGROUND AND METHODS: Congenital cystic dilatation of the bile ducts represents an uncommon anomaly of the biliary system. We report on 6 patients suffering from cystic biliary duct dilatations which were treated in our hospital between 1980 and 1992. Clinical signs included upper abdominal pain, white or clay-colored stool, icterus and/or palpable tumor. According to the classification of Todani, 4 children had type Ia cysts, 1 child a type Va cyst and 1 child a type Ia cyst with extrahepatic biliary atresia. RESULTS: Among the diagnostic methods sonography is preeminent and permitted demonstration of intra- and extrahepatic biliary duct dilatations in all of our patients. In 2 patients small cystic dilatations could be distinguished from hepatic vessels by colour-coded Doppler sonography. CONCLUSION: The treatment of choice is the resection of the dilated extrahepatic biliary ducts followed by hepaticojejunostomy using the Roux-en-Y-technique.     

Acquired biliary atresia

Three infants are described in whom acquired biliary atresia developed during the perinatal period. In two cases this was related to a spontaneous perforation of the bile duct, and in the other it probably was related to previous surgery for duodenal and ileal atresias. Clinically, the symptoms in these patients differed from the congenital forms of biliary atresia; two of the infants had dilated intrahepatic ducts on ultrasonography, and all had restriction of disease to the extrahepatic bile ducts and an excellent response to surgery.     

The developing human biliary system at the porta hepatis level between 29 days and 8 weeks of gestation: a way to understanding biliary atresia. Part 1

The developing biliary system in normal human embryos from 29 days to 8 weeks post-fertilization was studied. The primitive extrahepatic bile duct that originates from the embryonic hepatic foregut diverticulum is in contact with the hepatic anlage from the start of organogenesis and remains so throughout the gestational ages examined. The primitive extrahepatic bile duct maintains continuity with the ductal plate from which intrahepatic bile ducts are eventually formed. Contrary to long-held concepts of biliary development, no 'solid stage' of entodermal occlusion of the common bile duct lumen was found at any stage of gestation in the material investigated. Therefore, biliary atresia is not caused by incomplete vacuolization of the 'solid stage'.     

Preoperative diagnosis of laparoscopic cholecystectomy. Is there an indication for routine intravenous cholangiography?

In a retrospective study including 163 patients we investigated the necessity of i.v. cholangiography in preoperative routine diagnostic workup prior to laparoscopic cholecystectomy. We evaluated the evidence of i.v. cholangiography concerning the anatomy of the biliary system, the evidence of common bile duct or cystic duct stones and the influence on the further therapeutic procedure. While the common bile duct could be demonstrated in 96.3%, the cystic duct could be visualized in only 54.6%. One out of two patients with a short cystic duct was identified. Stones in the gallbladder were recognized in 72.4% of cases, while only two out of three patients with common bile duct stones were diagnosed. In nine cases a deep junction of the cystic duct was found, but there was no influence on further operative procedure. Thus we found no improvement after routine use of i.v. cholangiography concerning the evidence of common bile duct stones or avoidance of intraoperative lesions of the common bile duct. The routine use of i.v. cholangiography prior to laparoscopic cholecystectomy is therefore not justified.     

Familial occurrence of congenital bile duct cysts

Congenital bile duct cysts are now a well-documented anomaly of the biliary tree, and have become more common in Japan. Familial occurrence of congenital bile duct cysts, however, is extremely rare, with only six reported cases in the literature. We report a familial pattern of congenital bile duct cysts in a mother and her daughter. A 33-year-old female was admitted for evaluation of right upper quadrant abdominal pain and fever 6 days after an uneventful delivery of her second child. A computed tomography (CT) and ultrasound scan (US) revealed an obstructed biliary tract. Percutaneous transhepatic biliary drainage was then performed, and a cholangiogram revealed a Scholtz type B choledochocele without an anomalous connection of the pancreaticobiliary ducts. Endoscopic US demonstrated that the choledochocele was associated with a stone in the cyst. A pylorus-preserving pancreatoduodenal resection was performed, and a histological study revealed that the choledochocele was lined by biliary mucosa without evidence of malignancy. The newborn infant had an abdominal tumour. An US and CT revealed a congenital bile duct cyst. An operation was performed and the intraoperative cholangiogram showed an Alonso-Lej type I congenital bile duct cyst with an anomalous connection of the pancreaticobiliary ducts. Whether congenital bile duct cysts are hereditary remains to be elucidated.     

Percutaneous transhepatic cholangiography utilizing the Chiba University needle

Percutaneous transhepatic cholangiography with the Chiba University needle has been performed in 42 patients with cholestasis. The examination was successful in 100% of patients with dilated intrahepatic bile ducts and in 89% of those with normal-size ducts. Complications were minimal. The results of this study suggest that PTC with the Chiba needle is superior to the current conventional methods. The patients with intrahepatic cholestasis, which often simulates extrahepatic biliary obstruction, were spared an unnecessary laparotomy.     

Anatomic anomalies in neonatal cholestatic jaundice

Disorders of the biliary tree are an important cause of cholestatic jaundice in infancy. For the most frequent diseases in this group, biliary atresia and choledochal cyst, prognosis is strongly dependent on timely diagnosis and treatment. In biliary atresia the bile flow is obstructed due to obliteration of the extrahepatic bile ducts. Construction of an hepatic portoenterostomy before 60 days of age will result in restoration of bile flow in the vast majority of patients. When failed, the disease is progressive and ultimately fatal, unless a liver transplantation is performed. For those patients in which restoration of the bile flow succeeds, the subsequent course is strongly dependent on the occurrence of cholangitis. For all patients fat-soluble vitamins should be supplemented and caloric intake should be carefully monitored. Presentation of a choledochal cyst can be either before or after the first year of life. It is mostly characterized by jaundice, with or without abdominal pain. Therapy consists of resection of the cyst, followed by a hepatico-jejunostomy. Paucity of bile ducts is an intrahepatic disorder, in which--almost--no bile ducts can be found in the portal tracts. This anomaly is frequently found in combination with a typical facies, a pulmonary stenosis and vertebral anomalies, a combination which is called Alagille syndrome. Prognosis is generally good.     

Apropos of a case of acute pancreatitis revealing cystic dilatation of the common bile duct

Dilatation of the common bile duct is rarely caused by cystic formations. Though the pathogenesis is uncertain, congenital disorders have been suggested. Most cases are observed in small children (80% female predominance) with only 20% of the cases reported in adults. Clinical signs vary. Recurrent acute pancreatitis has been reported but is rare. New imaging techniques using CT-scan cholangiography and sometimes MR cholangiography have greatly improved the diagnostic approach. MR of the bile ducts is a recent noninvasive technique enabling an analysis of the biliopancreatic ducts without contrast injection into the bile. To our knowledge, cystic dilatation of the common bile duct has not been previously reported in the literature. We report an interesting case in a 25-year-old woman who developed an episode of acute pancreatitis during the post partum period. We describe the clinical aspects and the different imaging findings, including magnetic resonance cholangiography results.     

Chronic pancreatitis: a cause of cholestasis

The bile ducts were visualised using endoscopic retrograde cholangiopancreatography (ERCP), percutaneous or intravenous cholangiography in 38 patients with non-gallstone chronic pancreatitis. Stenosis of the intrapancreatic portion of the distal common bile duct was demonstrated in 11 patients. Ten of the 11 developed transient cholestasis during exacerbations of their chronic pancreatitis. In six cholestasis eventually persisted requiring surgical relief. Secondary biliary cirrhosis was present in one patient. No evidence of pancreatic carcinoma was found in the patients explored surgically. Ten of the patients are alive more than one year after diagnosis. Chronic pancreatitis was of alcoholic aetiology in 10 of the patients with biliary stenosis. Cholestasis and biliary stricture are common but poorly recognised complications of non-gallstone chronic pancreatitis, especially when pancreatitis is severe and due to alcohol.