Three-dimensional magnetic resonance cholangiopancreatography for evaluation of obstructive jaundice

The purpose of this study was to assess the diagnostic value of magnetic resonance cholangiopancreatography (MRCP) in studying the anatomy, sites, and causes of obstructive jaundice. From September 1994 to May 1996 three-dimensional MRCP was performed on 31 patients with abdominal pain and obstructive jaundice with a fast spin-echo T2-weighted pulse sequence. The images were reconstructed using maximal intensity projection, AVERAGE and SURFACE algorithm processing techniques at a graphics workstation. All the reconstructed images were compared with those obtained using conventional cholangiographic techniques, such as endoscopic retrograde cholangiopancreatography, percutaneous transhepatic cholangiography and drainage, and intraoperative cholangiography. The patients' diagnoses included choledochal cyst (13), cholangiocarcinoma (five), choledocholithiasis (four), pancreatic head carcinoma (three), rhabdomyosarcoma (one), papillary Vater carcinoma (one), recurrent gastric carcinoma (one), ascaris (one), and biliary atresia (two). Extrahepatic biliary dilatation was present in all 13 patients with choledochal cyst; the pancreatic ducts and their entrance level to the common bile duct were observed in eight of these patients. The level of obstruction in patients with cholangiocarcinoma was well documented but the biliary tract of one patient with biliary atresia was not identified by MRCP. In one patient with biliary rhabdomyosarcoma, MRCP clearly delineated the extrabiliary extension of the tumor. In a patient with ascaris in the common bile duct an increase in signal intensity inside the digestive tract of the worm denoted fluid in its gut. Lithiasis was shown in all of the four patients with choledocholithiasis. Thus, MRCP is a useful tool in the assessment of biliary tract obstruction and its causes, and is a valuable addition to ultrasonography.     

Scanning electron microscopy studies of the human lens capsule after capsulorhexis

Choledochal cysts are malformations of the biliary ductal system, which rarely occur in infancy. In neonates and infants, choledochal cysts are congenital, may be associated with distal biliary atresia and typically presents with cholestatic jaundice and acholic stools characteristic of biliary obstruction. In older children and adults, it may be acquired in association with an anomalous pancreaticobiliary union and presents with any combination of intermittent jaundice, abdominal pain, and a palpable abdominal mass. Early detection of choledochal cysts is important in the prevention of the fatal complications of biliary obstruction. Here, we present two cases of congenital choledochal cyst in which the patients were 9 weeks old and 7 weeks old respectively. Both of them presented with jaundice, acholic stools, and hepatomegaly. Combined use of sonography, computed tomography and hepatobiliary scintigraphy demonstrated the presence of choledochal cysts. Thereafter, they underwent surgical excision of the cysts with Roux-en-Y reconstruction of the biliary tree; the results were successful.     

Reducing the addictiveness of cigarettes. Council on Scientific Affairs, American Medical Association

Biliary atresia is a disorder of infants in which there is obliteration or discontinuity of the extrahepatic biliary system, resulting in obstruction of bile flow. Untreated, the resulting cholestasis leads to progressive conjugated hyperbilirubinemia, cirrhosis, and hepatic failure. Biliary atresia has an incidence of approximately one in 10,000 live births worldwide. Evidence to date supports a number of pathogenic mechanisms for the development of biliary atresia. An infectious cause, such as by a virus, would seem most pausible in many cases. The clinical observation that biliary atresia is rarely encountered in premature infants would support an agent acting late in gestation. However, no infectious or toxic agent has been conclusively implicated in biliary atresia. Genetic mechanisms likely play important roles, even regarding susceptibility to other specific causes, but no gene whose altered function would result in obstruction or atresia of the biliary tree has been identified. The variety of clinical presentations support the notion that the proposed mechanisms are not mutually exclusive but may play roles individually or in combination in certain patients. Biliary atresia, when untreated, is fatal within 2 years, with a median survival of 8 months. The natural history of biliary atresia has been favorably altered by the Kasai portoenterostomy. Approximately 25 to 35% of patients who undergo a Kasai portoenterostomy will survive more than 10 years without liver transplantation. One third of the patients drain bile but develop complications of cirrhosis and require liver transplantation before age 10. For the remaining one third of patients, bile flow is inadequate following portoenterostomy and the children develop progressive fibrosis and cirrhosis. The portoenterostomy should be done before there is irreversible sclerosis of the intrahepatic bile ducts. Consequently, a prompt evaluation is indicated for any infant older than 14 days with jaundice to determine if conjugated hyperbilirubinemia is present. If infectious, metabolic, endocrine disorders are unlikely and if the child has findings consistent with biliary atresia, then exploratory laparotomy and intraoperative cholangiogram should be done expeditiously by a surgeon who has experience doing the Kasai portoenteostomy. Biliary atresia represents the most common indication for pediatric liver transplantation, representing more than 50% of cases in most series. Transplantation is indicated when symptoms of end stage liver disease occur, including recurrent cholangitis, progressive jaundice, portal hypertension complications, ascites, decreased synthetic function, and growth/nutritional failure.     

Prenatal diagnosis of biliary atresia associated with choledochal cyst

We report a case of biliary atresia promptly detected by its association with a choledochal cyst, prenatally diagnosed at 20 weeks' gestation. The baby was operated at 14 days of life. A choledochal cyst was found and fibrosis of the extrahepatic bile ducts was also noted. So, excision of the choledochal cyst and a Roux-en-Y porto-jejunostomy was done. Nine months later, despite an appropriate biliary drainage, echographic and histological changes compatible with liver cirrhosis have been detected. Including this one, six cases have been reported.     

Biliary atresia and biliary cysts

The authors present a review of the classification, aetiology, presentation, treatment and long-term outcome of children and adults with biliary atresia and choledochal cyst disease. Biliary atresia should be suspected in any infant with jaundice beyond the second week of life. Although the aetiology and pathogenesis remain unclear, early management with portoenterostomy has significantly improved the course of this disease. Recent advances in immunosuppression have made liver transplantation a valuable and necessary adjunct to biliary bypass. With choledochal cyst disease, adults, unlike children, often present with acute biliary tract symptoms or pancreatitis. The treatment of choice remains extrahepatic cyst excision and biliary bypass. This treatment has excellent long-term results that minimize the development of malignancy.     

Evaluation of mebrofenin hepatoscintigraphy in neonatal-onset jaundice

BACKGROUND: The prognosis of infants with prolonged neonatal jaundice is dependent on early diagnosis because of the need for prompt surgical management of biliary atresia. OBJECTIVE: To evaluate the usefulness of 99 mTcm-trimethylbromo-iminodiacetic acid (TBIDA, mebrofenin) in the investigation of infantile jaundice. MATERIALS AND METHODS: A retrospective study was undertaken of 58 patients with unexplained prolonged neonatal jaundice. Sixty-eight scans were reviewed. RESULTS: Mebrofenin scintigraphy confirmed the presence of a choledochal cyst in three of the four cases with that diagnosis. There were no false negative results in the nine patients with extrahepatic biliary atresia (EHBA). Three further infants had an incorrect histological diagnosis of EHBA. A gall bladder was identified by US in each case and in one of these, scintigraphy showed gut excretion. In the 16 patients with no gut excretion by 24 h, the final diagnoses were intrahepatic cholestasis (n = 7), Alagille's syndrome (n = 3), neonatal hepatitis (n = 3), alpha-1-antitrypsin deficiency (n = 2) and juvenile xanthogranuloma (n = 1). Seven infants had repeat scintigraphy after the administration of ursodeoxycholic acid (URSO). This changed five non-excretors with hepatitis into excretors. Two infants with hepatitis continued to show non-excretion after URSO, but a gallbladder was identified by US in both. CONCLUSIONS: Mebrofenin scintigraphy is accurate in confirming the presence of a choledochal cyst and in refuting the diagnosis of EHBA. While histology and scintigraphy are each 100 % sensitive for the diagnosis of EHBA, neither, individually, is accurate and the investigation of prolonged neonatal jaundice requires a multi-modality imaging strategy.     

Is it possible to differentiate between choledochal cyst and congenital biliary atresia (type I cyst) by antenatal ultrasonography?

Both choledochal cyst and congenital biliary atresia (type I cyst) may share the same ultrasonographic pattern. We report 2 cases which were shown to have cystic structures on the upper abdomen by antenatal ultrasonography. The size of the choledochal cyst found at 24 weeks' gestation increased steadily as gestational age advanced. But the size of the cyst in congenital biliary atresia found at 29 weeks' gestation remained unchanged throughout the remaining pregnancy. Cyst enlargement, therefore, may suggest the possibility of a choledochal cyst.     

Choledochal cyst: a review of 19 cases

Nineteen cases of choledochal cyst are reviewed. Two distinct groups of patients were identified. Patients under one year of age, initially diagnosed as having biliary atresia, had a higher mortality rate, a higher incidence of severe cirrhosis with portal hypertension, and associated atresia or stenosis in the biliary tree. The second group, presenting between 3 and 20 years of age with more classic symptoms, had mild cirrhosis without portal hypertension and had associated choledocholithiasis and pancreatitis. It is suggested that the younger patients had a congenital form of cystic bile duct dilatation and that the older patients had an acquired form, perhaps related to a common channel with reflux of pancreatic juice into the common bile duct. Postoperative follow-up supports the current view that choledochocyst-jejunostomy with choleystectomy has a lower rate of long-term complications than does choledochocyst-duodenostomy.     

Successful management of ruptured choledochal cyst by primary cyst excision and biliary reconstruction

One to two percent of patients who have choledochal cysts present with cyst rupture and bile peritonitis. Reported cases have been managed with external drainage of the cyst followed by a second procedure to excise the cyst and reconstruct the biliary tract. The authors report two cases of ruptured choledochal cyst treated with primary cyst excision and biliary drainage. The satisfactory outcome of these patients suggests that this is the preferred management.     

Minimally invasive interventions in treatment of mechanical jaundice

For the period from 1972 to 1997 676 transcutaneous transhepatic and 370 endoscopical interventions in 953 patients with mechanical jaundice were carried out. In 58.5% of cases jaundice was caused by malignancies, in 41.4%--by benign strictures of bile ducts. 40 external biliary bypasses, 427 external-internal biliary bypasses, 55 cholecystostomies and 154 operations of biliary endoprosthetic reconstructions were conducted under US control and TV imaging. In 33.5% of cases biliary bypass and endoscopical procedures were made as a preliminary stage of preparation of patients for operation or as a final method of treatment after conventional operation procedures, in 66.5% of cases it was final method of treatment. There were no deaths caused by biliary bypass procedures nor complications related to laparotomy. Thus low invasive interventions, undoubtedly, are the method of choice in mechanical jaundice for preparation of patients before radical operations as well as for relief of the course of disease in patients with inoperable cancer which causes obstruction of biliary ducts.     

Malignant change in the biliary tract after excision of choledochal cyst

BACKGROUND: Choledochal cyst is a rare congenital condition with a high risk of malignant change if untreated. The risk of malignancy after surgical excision of choledochal cyst is not known. METHODS: Forty-eight patients with choledochal cysts managed over a 21-year period were reviewed, to determine the risk of malignant change after cyst excision. Thirty-nine of 48 patients had no carcinoma at first admission; their mean(s.d.) age was 20(18) years. Thirty-seven of 39 patients underwent cyst excision and cholecystectomy followed by hepaticoenterostomy. RESULTS: Cyst excision was incomplete in 28 of the 37 patients because dilated portions of the biliary ducts remained proximally and/or distally. In these 37 patients, no carcinoma has developed in the remnant proximal hepatic duct or the terminal bile duct after mean(s.d.) follow-up of 9.1(6.4) years. In the remaining nine patients, biliary carcinoma was diagnosed at the first visit. Six patients died from recurrence with a mean(s.d.) survival time of 13(11) months, while three patients were alive and free from recurrence 2 months, 1 year and 7 years after operation. CONCLUSION: Malignant change has not been observed after total or subtotal excision of choledochal cysts in this series.     

Value of MR cholangiopancreatography in evaluating choledochal cysts

OBJECTIVE: The aim of this retrospective study was to clarify whether MR cholangiopancreatography (MRCP) is a suitable replacement for ERCP in evaluation of the choledochal cyst. MATERIALS AND METHODS: Sixteen patients (six adult and 10 pediatric) with choledochal cysts underwent MRCP using a half-Fourier acquisition single-shot turbo spin-echo sequence. Extent of the cyst, defects within the biliary tree, and presence or absence of the anomalous junction of the pancreaticobiliary duct were evaluated. Findings were compared with those of ERCP. RESULTS: MRCP better defined the proximal biliary tree than did ERCP in two patients. Defects within the biliary tree were diagnosed correctly on MRCP in eight patients; however, two defects within the distal common bile duct were missed in pediatric patients. The presence of the anomalous junction of the pancreaticobiliary duct was revealed accurately by MRCP in all adult patients but was revealed accurately in only four of the 10 pediatric patients. CONCLUSION: MRCP appears to offer diagnostic information that is equivalent to that of ERCP for assessment of choledochal cysts in adults. In pediatric patients, MRCP should not replace ERCP; however, MRCP can play an important role as a noninvasive examination and should be considered a first-choice imaging technique for evaluation of choledochal cysts.     

Bile duct cysts in adults

INTRODUCTION: Bile duct cysts are rare, congenital dilations of the intrahepatic and/or extrahepatic biliary tract. Most of them present during childhood. The classical triad right upper quadrant pain, jaundice and abdominal mass is present only in a few instances. We report here the bile duct cysts which were diagnosed at our institution from 1989 to 1996. METHODS: 3245 consecutive endoscopic retrograde cholangiopancreatograms (ERCP) were evaluated retrospectively. Diagnosis was made when localized cystic dilations of the intrahepatic and/or extrahepatic biliary tract were present. Diffuse dilations of the intrahepatic and extrahepatic biliary tract were excluded. RESULTS: Bile duct cysts were found in 20 patients (17 females, 3 males) among 3245 ERCPs. Their mean age was 56 +/- 20 (median 64, range 10 to 83) years. The cyst types (according to the Alonso-Lej classification with the Todani modification) were type I in 11 (55%), type II, III and IV in two instances each (10%), and type V (or Caroli's disease) in 3 patients (15%). Leading symptoms were cholestasis in 14 patients, 10 of whom had abdominal pain, jaundice in 4 patients, and single cases of pancreatitis, cholangitis, and abdominal mass. In 2 patients the diagnosis was made incidentally. 10 patients had bile duct stones. We performed endoscopic sphincterotomy in 15 patients with concretions or persistent symptoms, 3 patients had cyst resection. One of these, with a type I cyst, already had a disseminated cholangiocarcinoma. 10 of 17 patients without cyst resection are currently symptom-free after complete removal of all gallstones. One male patient with cholecystolithiasis, who is not operable due to advanced liver disease, has recurrent cholangitis, 4 patients have died from causes unrelated to the bile duct cysts, and 2 patients are lost to follow up. CONCLUSION: Bile duct cysts in adults are rare. There is a preponderance in the female gender, and the most common type is the extrahepatic (choledochal) cyst. The leading symptoms are cholestasis and right upper quadrant pain. There is an increased risk of cholangiocarcinoma. In young patients the cysts should be entirely removed to prevent malignancy. Older persons are usually symptomless after complete removal of gallstones.     

Magnetic resonance cholangiography for evaluation of cholestatic jaundice in neonates and infants

Distinguishing extrahepatic biliary atresia from other causes of cholestasis in neonates and infants is important because surgical intervention before 2 months of age allows for long-term survival. The purpose of this prospective study was to evaluate the usefulness of magnetic resonance (MR) cholangiography in differentiating biliary atresia from other causes of cholestatic jaundice in neonates and infants. Nine anicteric infants (control group) aged 10 to 224 days (mean +/- SD, 8 +/- 65 days) and 15 neonates and infants with cholestatic jaundice, aged 22 to 142 days (mean +/- SD, 71 +/- 37) underwent MR cholangiography. The final diagnosis of extrabiliary atresia (6 patients) was based on laparotomy findings (4 patients) or autopsy (2 patients), while neonatal hepatitis (9 patients) was diagnosed according to the liver biopsy findings and clinical recovery during follow-up. Percutaneous liver biopsies were performed in all 15 patients. Results showed that the gall bladder and common bile duct (CBD) could be visualized using MR cholangiography in all patients in the control group. Nonvisualization of the CBD (6/6 patients) and demonstration of a small gall bladder (6/6 patients) characterized MR cholangiography findings in patients with biliary atresia. MR cholangiography failed to depict the CBD in one infant with hepatitis. We conclude that demonstration of the CBD by MR cholangiography in neonates and infants with cholestasis can be used to exclude the diagnosis of biliary atresia. In patients with cholestatic jaundice considered for exploratory laparotomy, preoperative MR cholangiography is recommended to avoid unnecessary surgery.     

Magnetic resonance imaging of proximal aortopulmonary window

Of the 187 cases of infantile choledochal cyst treated at our hospitals, we encountered 13 with spontaneous perforation. All cases were under 4 years old. Eight cases were found to have biliary peritonitis and 5 had a sealed perforation. The shape of the extrahepatic bile duct was cystic in 8 and fusiform in 5. The cyst wall around the perforation was filmy and bile was found to be oozing through the thinned wall. Nine perforations were single while 4 cases had multiple perforations. Four of 17 perforations occurred in the posterior part of the cyst wall. Only 1 case of perforation was associated with protein plugs in a common channel, while 7 of the 10 cases of choledochal cyst requiring percutaneous biliary drainage due to signs of raised intrabiliary pressure were found to have protein plugs. We consider that spontaneous perforation of a choledochal cyst is not rare in infancy. The etiology of a perforation must be epithelial irritation of the biliary tract due to refluxed pancreatic juice caused by pancreatico-biliary malunion associated with mural immaturity due to infancy, rather than an abnormal rise in ductal pressure or congenital mural weakness at a certain point.     

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.     

Cancer arising in choledochal cyst and management

Biliary cancer develops in 20-30% of the patients with choledochal cyst and pancreatobiliary malunion. Some bile acid fractions and refluxed pancreatic enxymes into the bile duct is probably responsible for carcinogenesis. Cancer often develops in the extrahepatic bile duct and gallbladder, and rarely in the intrahepatic duct. In cystic dilatation, cancer often occurs in the common bile duct, while in diffuse or non-dilated type it occurs in the gallbladder. Cancer usually occurs in younger patients than does biliary cancer in general population, and the average age is in the 40s. The risk of malignancy in cysts with internal drainage is higher than that in primary cysts, and early removal of the retained cyst should be performed as quickly as possible. Although the prognosis of biliary cancer is usually dismal, aggressive procedures are recently gaining better results than that by conventional methods. The prevention of cancer is the procedure of choice by early excision. Removal of the whole extrahepatic bile duct is necessary, even in case of malunion with no biliary dilatation. Cancer rarely arises in the intrahepatic duct after excisional surgery, due to long standing biliary stricture. Wide anastomosis with ductoplasty should be essential. Cancer also occurs in the remnant duct. Excision of the distal duct in the pancreas is also necessary.     

Asymptomatic intrahepatic choledochal cyst associated with chronic active hepatitis C

A rare case of asymptomatic congenital choledochal cyst (Todani Type V) associated with chronic active hepatitis C is reported. A 45 year-old man was admitted for work-up of localized cystic dilatation of the intrahepatic bile ducts after effective interferon treatment for chronic active hepatitis C. Radiologic examination revealed localized cystic dilatation of the intrahepatic bile ducts in the atrophic left lobe. A left hepatic lobectomy was performed because malignancy had not been excluded. Macroscopic examination of the resected specimen revealed cystic dilatation of the intrahepatic bile ducts. Histologically, there was no evidence of malignancy. Hepatic lobectomy is the treatment of choice for Type V cysts if the disease is confined to a single lobe. Choledochal cysts should be excised to eliminate the potential for cholangitis, lithiasis, and carcinoma.     

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.     

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.