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.     

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.     

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.     

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.     

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.     

Analysis of c-Ki-ras oncogene and p53 immunocytochemistry in the gallbladder mucosa of an experimental dog model of anomalous arrangement of the pancreaticobiliary ducts

Anomalous arrangement of the pancreaticobiliary ducts is a congenital condition which predisposes the affected person to biliary tract carcinoma. We developed an experimental dog model of anomalous arrangement of the pancreaticobiliary ducts to investigate the mechanism of carcinogenesis in this condition. We used this model to analyze point mutations in the c-Ki-ras gene, and to assess the expression of mutant p53 protein in the gallbladder mucosa. The histopathological appearance of the gallbladder mucosa was also examined. Glandular structures were seen in four of seven (57%) gallbladders examined 14 months after the surgical creation of an anastomosis between the gallbladder and the pancreatic duct. Goblet cells were seen in two of seven gallbladders (29%). However, dot-blot hybridization and immunohistochemical study did not reveal any mutations in the c-Ki-ras gene, or any over-expression of the p53 protein in the specimens. These results show that the gallbladder mucosa is damaged by refluxing pancreatic juice in this dog model of anomalous arrangement of the pancreaticobiliary ducts, but that severe damage may be necessary to induce mutations in the c-Ki-ras proto-oncogene, or in the p53 gene.     

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.     

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.     

Hepatic peribiliary cysts: multiple tiny cysts within the larger portal tract, hepatic hilum, or both

PURPOSE: To describe characteristic imaging features of hepatic peribiliary cysts. MATERIALS AND METHODS: Four patients with hepatic cysts in which the radiologic (n = 3) or histologic (n = 1) findings were consistent with peribiliary cysts of the liver (multiple small cysts seen exclusively in the larger portal tract, hepatic hilum, or both at gross examination and dilatations of extramural peribiliary gland at histologic examination) underwent computed tomography (CT) and ultrasound (US). In three patients, CT was performed after drip infusion of cholangiographic contrast material. RESULTS: Contrast material-enhanced CT clearly depicted many tiny cysts along the larger portal veins up to the third- or fourth-order branch (n = 3). US depicted multiple cysts in the echogenic portal tract definitely (n = 2) or equivocally (n = 2). On cholangiographic contrast-enhanced CT scans, cystic areas were located adjacent to or surrounding the bile ducts, and the possibility of biliary dilatation, communication, or both was disproved. CONCLUSION: Hepatic peribiliary cysts can be diagnosed with US and enhanced CT, especially with CT performed after administration of cholangiographic contrast material.     

Cholangiopancreatography with magnetic resonance. Clinical use of a new "inversion-recovery" sequence

This study was aimed at correlating the yield of a three-dimensional (3D) inversion-recovery (IR) turbo spin-echo MR cholangiopancreatography (MRCP) sequence with that of ERCP and PTC in the imaging of the normal and abnormal biliopancreatic tract. Thirty patients with suspected biliary and pancreatic diseases were examined with MRCP first and then with ERCP or PTC; they were also submitted to US, CT and conventional MR studies and in 5 of them CT cholangiography was also performed. Five patients were normal and 25 had various obstructive abnormalities: 5 patients had gallbladder stones, 8 common bile duct stones, 5 a cholangiocarcinoma and 7 an adenocarcinoma of the pancreatic head or papilla. MRCP was performed with a superconductive magnet at 0.5 T, with volumetric images on coronal planes acquired using an IR turbo SE sequence (TR 2500, TE 1000, TF 89, 4 NEX) with respiratory triggering and vascular presaturation. Segmental intrahepatic bile ducts were correctly depicted in all the patients with benign or malignant obstruction of the common bile duct, but with some respiratory artifacts. Common bile duct stones were correctly depicted in 7 of 8 patients, but studying also the single coronal slices. With this method, the stones were clearly demonstrated in 22 examined gallbladders. Neoplastic obstruction and the obstruction level were correctly identified in all patients. Pancreatic ducts were shown in normal patients and in 8 of 13 patients with neoplastic or lithiasic obstruction of the common bile duct mainly on the pancreatic head. ERCP was carried out successfully in 5 patients with common bile duct stones and in 7 patients with neoplastic obstruction; in the other cancer patients, PTC was necessary. To conclude, respiratory-triggered 3D IR turbo spin-echo MRCP is a noninvasive technique to study mostly biliary conditions which yields similar information to ERCP and PTC in a large number of patients. Moreover, this sequence can be used with midfield MR units to study the obstruction of the biliary and pancreatic ducts not only when invasive techniques fail, but also routinely.     

Cystic dilatation of the common bile duct

The Authors report a case of cystic dilatation of the common bile duct and a review of the Literature. The lesion is a congenital malformation of infancy or childhood, being rare in adult life. The main symptoms are biliary colic with jaundice and fever. Modern techniques such as abdominal ultrasonography, CT, ERCP, PTC generally provide to achieve preoperative diagnosis. When radical surgery is possible the complete excision of the cyst is the management of choice. The case reported is classified as type 1 of Todani's classification. Surgical management consisted in a complete excision of the cyst. Bile flow was re-established by hepaticojejunostomy.     

Surgical significance of extrahepatic biliary tree anomalies

A patient with an anomalous insertion of the right hepatic duct into the cystic duct was noted during cholecystectomy and confirmed by operative cholangiography. This case and related anomalies of the bile ducts are of sufficient importance that, because of the technical difficulties and dangers incidental to their presence, no surgeon who operates on the gallbladder and bile ducts can afford to be unaware of their existence. Adequate exposure, careful dissection, and accurate knowledge of the regional anatomy plus a realization of the frequency and multiplicity of abnormalities of the extrahepatic biliary tree are requisites for safe biliary tract surgery. In addition, carefully performed operative cholangiography can be an indispensable aid in the clarification of anatomic variations. In case of recognized operative injury to the extrahepatic biliary tree, primary repair or biliary-intestinal anastomosis can usually be carried out with good results.     

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.     

Long-term survival of a patient with bile duct carcinoma by endoscopic biliary drainage

An 80-yr-old female presented with obstructive jaundice. Endoscopic retrograde cholangiopancreatography showed a carcinoma in the middle extrahepatic bile duct, and a biliary endoprosthesis was inserted. Exfoliative cytology of the bile and forceps biopsy of the tumor revealed a papillary adenocarcinoma. Surgical resection could not be done because of her cardiovascular complications, and neither chemotherapy nor radiotherapy was administered. Stents were exchanged and cleaned 21 times because of occlusion and cholangitis. Subsequent serial cholangiogram showed a slow growth of the papillary tumor, but local invasion to the adjacent organs or distant metastasis was not observed. The patient survived for 7 yr and 6 months after insertion of the biliary endoprosthesis.     

Pancreatic trauma in patients with pancreatobiliary anomaly

One patient with a choledochal cyst and anomalous pancreaticobiliary junction had pancreatic transection causing bile peritonitis. Intraoperative cholangiopancreatography revealed this anomaly. In another patient with pancreas divisum, cannulation of the minor papilla (ERCP) demonstrated focal stenosis of the dorsal pancreatic duct, corresponding to the site of the minor laceration. The possibility of a coexisting pancreatobiliary anomaly should be considered in the diagnosis of pancreatic trauma, particularly in terms of the interpretation of pancreatograms.     

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.     

Choledochal cyst and neoplasm: a comprehensive review of 106 cases and presentation of two original cases

Choledochal cysts are prone to complications: cholangitis, biliary cirrhosis, portal hypertension, lithiasis, rupture, pancreatitis, and carcinoma. The coincidence of choledochal cysts and neoplasia ranges from 2.5 to 26 per cent. One hundred six cases of choledochal cysts with neoplasms have been collected from the literature. We have tabulated the results of 68 cases found to have a neoplasm at the initial laparotomy and of 38 patients with pristine choledochal cysts treated electively who subsequently developed a neoplasm. Two original cases are presented. The primary site of neoplasia was not confined only to choledochal cysts. There appears to be a propensity for malignancy to develop anywhere in the biliary tract or gallbladder or pancreas in conjunction with the choledochal cyst. Accompanying choledochal cysts is a high incidence of an anomalous relation at the pancreaticobiliary junction with subsequent malignancy formation. A pathogenetic basis is postulated.     

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.     

Biliary bile acids in the gall-bladder and the common bile duct of patients with anomalous pancreaticobiliary ductal junction

The high incidence of biliary tract carcinoma in patients with anomalous pancreaticobiliary ductal junction (APBDJ) with or without choledochal cyst (CC) has been well documented. Twenty-two patients with APBDJ were divided into three groups: Group A, four patients not associated with CC and biliary tract carcinoma; Group B, 13 patients with CC but without biliary tract carcinoma; and Group C, five patients with biliary tract carcinoma (four with and one without CC). Profiles of bile acids in the gall-bladder and/or common bile duct were analysed in these patients and compared with those in the control patients with cholecystlithiasis to examine the hypothesis that the levels of deoxycholic acid (DCA) and lithocholic acid (LCA) are elevated in patients with APBDJ because these secondary bile acids are mutagenic. Bile acids were quantified by gas-liquid chromatography. Total bile acid concentration in the gall-bladder bile was significantly lower in any group with APBDJ than that of controls. In the gall-bladder, increased proportion of chenodeoxycholic acid (CDCA) in Group A and B, decreased proportion of DCA in Group B and increased proportion of cholic acid (CA) in Group C were found in bile. In the bile duct, total bile acid concentration and proportion of DCA were significantly low in bile from Group C and decreased proportion of DCA and increased proportion of CDCA were found in bile from Group B. In both the gall-bladder and hepatic bile, proportion of LCA was not significantly different between any intergroups.(ABSTRACT TRUNCATED AT 250 WORDS)     

Prospective study of the detection of anomalous connections of pancreatobiliary ducts during routine medical examinations

BACKGROUND/AIMS: It is important to achieve detection of of anomalous connections of the pancreatobiliary ducts (ACPBD), because this condition often leads to pancreatobiliary disease. The present prospective investigation focused on revealing the incidence of ACPBD in asymptomatic individuals undergoing medical checkups. MATERIAL AND METHODS: Extracorporeal ultrasonography (US) was performed on all asymptomatic individuals undergoing medical checkups. Identification by abdominal ultrasonography of 3 mm or more gallbladder wall thickening, or 10 mm or more dilatation of the bile duct were considered indicative of ACPBD and endoscopic ultrasonography (EUS) was carried out in selected cases. Endoscopic retrograde cholangiopancreatography (ERCP) was then performed to confirm the existence of ACPBD. RESULTS: The overall incidence of ACPBD was 0.03% (9/27,076 subjects). ACPBD was found in 23% of cases with bile duct dilatation, and in 2.9% with gallbladder wall thickening. CONCLUSION: ACPBD was not as rare a disease as expected. For early detection of ACPBD, identification by abdominal ultrasonography of gallbladder wall thickening or dilatation of the bile duct are indicative. Patients who are suspected of having this abnormality should then undergo EUS. The final diagnosis should then be made by ERCP.