Anomalous Arrangement Of The Pancreaticobiliary Duct Research Articles

Anatomic anomalies of pancreatic duct in children with chronic pancreatitis

Choledochal cyst (CC) is closely associated with anomalous arrangement of the pancreaticobiliary duct, which is considered a high-risk factor for biliary tract malignancy. Early diagnosis and early treatment for CC could lead to a good prognosis. This study investigated late complications and long-term outcomes after surgery for CC.Fifty-six patients with CC and over 10 years of postoperative follow-up were analyzed retrospectively. All patients had undergone total resection of the extrahepatic bile duct and hepaticojejunostomy.Six patients showed liver dysfunction manifested in the first 10 years after surgery, but all returned to normal thereafter. Dilatation of intrahepatic bile ducts persisted in 6 postoperatively, and in 3, this was still apparent more than 10 years after. Recurrent abdominal pain was encountered in 3, 1 had pancreas divisum with a pancreatic stone, and 1 had adhesive small bowel obstruction. Two patients developed biliary tract malignancy. A 14-year-old girl died of recurrent common bile duct cancer 2 years after the initial resection of CC with adenocarcinoma. A 26-year-old man with repeated cholangitis owing to multiple intrahepatic bile stones developed cholangiocarcinoma 26 years after the initial resection of CC. Event-free survival rate and overall survival rate were 89% (50/56) and 96% (54/56), respectively.Choledochal cyst generally has an excellent prognosis with early total resection and reconstruction. Long-term surveillance for the development of malignancy is still essential, especially if there is ongoing dilatation of the intrahepatic bile duct or biliary stones.

Diagnosis and treatment of pancreaticobiliary maljunction in children

Pancreaticobiliary maljunction (PBM), which may be called by synonymous terms such as "anomalous arrangement of the pancreaticobiliary duct" or "anomalous union of biliopancreatic ducts," is defined as an anatomical maljunction of the pancreatic duct and the biliary duct outside of the duodenal wall beyond the influence of the sphincter of Oddi. Pancreaticobiliary maljunction is classified into three groups according to the type of the pancreaticobiliary junction. Pancreaticobiliary maljunction is diagnosed mainly by endoscopic retrograde cholangiopancreatography; however, in pediatric patients, it may be diagnosed by intraoperative cholangiography, and more recently has been diagnosed by computed tomographic cholangiography or contrast-enhanced helical computed tomography. Children with PBM without choledochal cysts usually do not show any symptoms except for abdominal pain and hyperamylasemia. Pancreaticobiliary maljunction is considered to be a major risk factor for biliary tract malignancy. In patients with PBM, free reflux of pancreatic juice into the biliary tract might induce biliary tract damage and biliary carcinogenesis. Thus, total resection of the extrahepatic bile duct and hepaticojejunostomy are recommended in children diagnosed with PBM with choledochal cyst. Early diagnosis and early surgical treatment provide a good prognosis with few complications. In addition, successive follow-up is necessary for early detection of biliary tract malignancy, especially in patients demonstrating postoperative complications.

A Case of 1p36 Deletion Syndrome Accompanied With Anomalous Arrangement of the Pancreaticobiliary Duct

Kawashima, Hisashi MD, PhD1; Kinjo, Naoko MD1; Uejima, Hajime MD, PhD1; Ioi, Hiroaki MD, PhD1; Takekuma, Kouji MD, PhD1; Nagae, Itsurou MD, PhD2; Ishii, Kentarou MD3; Itoi, Takao MD, PhD3; Numabe, Hironao MD, PhD4 Author Information

Impact of Age at Diagnosis on Clinical Features in Children with Anomalous Arrangement of the Pancreaticobiliary Duct

In patients with an anomalous arrangement of the pancreaticobiliary duct (AAPBD), clinical presentations may differ between infants and older children. The optimal timing of surgery remains controversial, particularly in early infancy. The aim of this study was to evaluate the clinicopathological features and clinical outcomes using comparative methods between infants and older cases. MATERIALS AD METHODS: From 1983 to 2007, a total of 85 consecutive children with AAPBD were treated at our institute. They included 46 with the cystic type, 33 with the fusiform type, and 6 with the non-dilatation type. These patients were divided into 2 age groups: "infant" (n=9), <12 months old; and "older", >1 year old (n=76). A retrospective study was performed. Mean age was 5.2 months (range, 8 days-11 months) in the infant group and 5.2 years (range, 1.2-17.3 years) in the older group. Jaundice was significantly more frequent in the infant group ( P<0.05), whereas abdominal pain was more common in the older group ( P<0.001). Bleeding tendencies such as cranial hemorrhage or bloody stools were noted in only 3 infants. In terms of liver histology, liver cirrhosis was observed in 2 infants, one of whom was a 3-month-old girl with severe jaundice resulting in living-donor liver transplantation, despite bile drainage. A single postoperative death occurred due to an adenocarcinoma arising in a choledochal cyst in a 12-year-old girl. Problems characteristic of infantile AAPBD were a severe bleeding tendency and irreversible liver cirrhosis, which could develop as young as 3 months old. The surgical recommendation for infantile AAPBD is thus early surgery before the age of 3 months to prevent liver failure.

Long-term outcomes after hepaticojejunostomy for choledochal cyst: a 10- to 27-year follow-up

Introduction Choledochal cyst (CC) is closely associated with anomalous arrangement of the pancreaticobiliary duct, which is considered a high-risk factor for biliary tract malignancy. Early diagnosis and early treatment for CC could lead to a good prognosis. This study investigated late complications and long-term outcomes after surgery for CC. Patients and Methods Fifty-six patients with CC and over 10 years of postoperative follow-up were analyzed retrospectively. All patients had undergone total resection of the extrahepatic bile duct and hepaticojejunostomy. Results Six patients showed liver dysfunction manifested in the first 10 years after surgery, but all returned to normal thereafter. Dilatation of intrahepatic bile ducts persisted in 6 postoperatively, and in 3, this was still apparent more than 10 years after. Recurrent abdominal pain was encountered in 3, 1 had pancreas divisum with a pancreatic stone, and 1 had adhesive small bowel obstruction. Two patients developed biliary tract malignancy. A 14-year-old girl died of recurrent common bile duct cancer 2 years after the initial resection of CC with adenocarcinoma. A 26-year-old man with repeated cholangitis owing to multiple intrahepatic bile stones developed cholangiocarcinoma 26 years after the initial resection of CC. Event-free survival rate and overall survival rate were 89% (50/56) and 96% (54/56), respectively. Conclusions Choledochal cyst generally has an excellent prognosis with early total resection and reconstruction. Long-term surveillance for the development of malignancy is still essential, especially if there is ongoing dilatation of the intrahepatic bile duct or biliary stones.

Development of bile duct cancer in a 26-year-old man after resection of infantile choledochal cyst

Anomalous arrangement of the pancreaticobiliary duct is considered to be a high-risk factor for biliary tract malignancy. We report a case of intrahepatic cholangiocarcinoma in a 26-year-old man after total resection of choledochal cyst with anomalous arrangement of the pancreaticobiliary duct at the age of 5 months. He had been doing well after total resection of the choledochal cyst; however, he suddenly presented with a spiky fever and abdominal pain in the right upper quadrant at the age of 26 years. Computed tomographic scan and percutaneous transhepatic cholangioscope revealed multiple stones and stenosis of the hepatic duct and the left intrahepatic bile duct. Histologic examination of a biopsy specimen obtained from the stenotic site showed adenocarcinoma of the intrahepatic bile duct. Left lobectomy with re-reconstruction by right hepaticojejunostomy was performed, and his postoperative course was uneventful. One year after the operation, however, he died of carcinomatous peritonitis with recurrence of cholangiocarcinoma. This report warns us that bile stasis owing to stenosis of the intrahepatic bile duct and repeated cholangitis with multiple stones are high-risk factors for carcinogenesis of the intrahepatic bile duct even after total resection of the infantile choledochal cyst.

B-037 Clinical problems in the infants with anomalous arrangement of the pancreaticobiliary duct(Oral Presentation Hepatobiliary, pancreas,Better Life for Sick Children, Better Future for Pediatric Surgery,the 45th Annual Meeting of Japanese Society of Pediatric Surgeons)

B-037 Clinical problems in the infants with anomalous arrangement of the pancreaticobiliary duct(Oral Presentation Hepatobiliary, pancreas,Better Life for Sick Children, Better Future for Pediatric Surgery,the 45th Annual Meeting of Japanese Society of Pediatric Surgeons)

Clinical significance of the confluence of the cystic duct in patients with anomalous arrangement of the pancreaticobiliary duct

Background/Purpose In anomalous arrangement of the pancreaticobiliary duct (AAPBD), there is anatomical diversity of the cystic duct. In this study, we evaluated the influence of the level of insertion of the cystic duct into the extrahepatic bile duct on the pathophysiology of AAPBD. Methods Thirty-two children with AAPBD were examined using cholangiopancreatography. If the cystic duct entered the hepatic duct at the lower middle point of the extrahepatic bile duct, it was defined as low confluence; otherwise, it was considered as high confluence. Clinical details and radiological variables were compared between these 2 groups. Results Low confluence was noted in 8 of the 32 patients. Seven of the 8 had fusiform-type or nondilatation-type choledochus, and the cystic-type was significantly less frequent than in the high-confluence group. The diameter of the common bile duct was significantly smaller and the main pancreatic duct was significantly greater than in the high-confluence group. Pancreatitis was more common and biliopancreatic reflux on computed tomography combined with intravenous infusion cholangiography was more often seen in the low-confluence group than in the high-confluence group. Conclusions Bile juice could regurgitate into the pancreatic duct via the low confluence of the cystic duct, resulting in severe pancreatitis in patients with AAPBD.

Significance of the Anomalous Arrangement of the Pancreaticobiliary Duct in the Etiology of Biliary Atresia

The anomalous arrangement of the pancreaticobiliary duct (AAPBD) is one theory used to explain the etiology of biliary atresia. We investigated whether AAPBD could be involved and evaluated its significance for the etiology of biliary atresia. Of 43 patients with biliary atresia, the area between the common bile duct and the duodenum could be visualized by operative cholangiogram in 5 patients with an uncorrectable type of biliary atresia. Three of the 5 showed an anomalous arrangement of the pancreaticobiliary duct. In these 3 patients, the type of anomalous arrangement of the pancreaticobiliary duct and the length of the common channel were studied by operative cholangiogram. Histological findings of the gallbladder and the common bile duct were examined in addition to the measurement of the serum amylase levels. All 3 patients showed AAPBD with the P-C type of pancreaticobiliary junction. The length of the common channel ranged from 7 mm to 12 mm. Two of the 3 cases did not show an elevated serum amylase level. Epithelial hyperplasia of the gallbladder was observed in one patient, while the other two showed no hyperplasia. Inflammatory changes in the mucosa of the gallbladder and the common bile duct were not remarkable in these 3 patients. From these results it seems that AAPBD in biliary atresia might not be an etiological factor for atresia of the extrahepatic bile duct, but might be an associated anomaly in biliary atresia. Other factors should be examined to clarify the etiological factor leading to lumenal obstruction of the extrahepatic bile duct.

Surgical Treatment for Anomalous Arrangement of the Pancreaticobiliary Duct With Nondilatation of the Common Bile Duct

Surgical Treatment for Anomalous Arrangement of the Pancreaticobiliary Duct With Nondilatation of the Common Bile Duct

Estrogen receptor expression in anomalous arrangement of the pancreaticobiliary duct

Background/Purpose Anomalous arrangement of the pancreaticobiliary duct (AAPBD) is a malformation of the bile and pancreatic ducts which is commonly associated with congenital biliary dilatation and predominantly occurs in girls. Estrogen receptor (ER) is reported to modulate cholangiocyte proliferation and play a role in tumorigenesis of estrogen-dependent malignancies. The present study investigated the presence of ER in the gallbladder of patients with AAPBD to elucidate whether ER expression is correlated with sexual specificity and biliary histology. Methods Specimens comprised samples of 29 gallbladders from patients with AAPBD (21 girls and 8 boys; mean age at surgery, 5.3 years). Medical records were retrospectively reviewed, and sex, age, radiographic parameters, and laboratory data were collected for this study. Immunohistochemistry was performed using anti-ER and anti–MIB-1 antibodies, and MIB-1 score was calculated for evaluation of cell proliferation. Results ER expression was observed in both the nuclei and cytoplasm of gallbladder epithelium in 12 of 29 specimens. No difference in background characteristics were noted between ER-positive and ER-negative groups. Mucosal hyperplasia was found in 17 specimens. ER-positive cases were significantly more common in AAPBD with mucosal hyperplasia than without ( P <0.03). MIB-1 score did not differ significantly between ER-positive and ER-negative groups. Conclusions ER expression seems to be related to hyperplastic mucosa of the gallbladder in AAPBD. ER might play a role in the proliferation of gallbladder epithelium.

Surgical treatment for anomalous arrangement of the pancreaticobiliary duct with nondilatation of the common bile duct

Background/purpose For anomalous arrangement of the pancreaticobiliary duct (AAPBD) with nondilatation of the common bile duct (CBD), the optimal surgical procedure remains controversial. The authors investigated which procedure would be most effective for AAPBD with nondilatation of the CBD. Methods The authors encountered 60 children with AAPBD in our institution between 1979 and 2002. Six of the 60 were classified as the nondilated type (CBD diameter; less than 8 mm), whereas the other 54 were classified as the dilated type (CBD diameter; more than 9 mm). Amylase levels in serum, CBD, and gallbladder were examined. Cellular activity of the resected gallbladder was examined for the incidence of hyperplasia and Ki-67 labeling index (Ki-67 LI). Results The amylase level in the nondilated type was elevated as in the dilated type. Epithelial hyperplasia of the gallbladder was present in 4 of the 6 with the nondilated type (67%). 10 of the 20 with the dilated type (50%), and none of the 6 controls (0%). The Ki-67 LI of the dilated type was significantly higher than that of control. Conclusions A free reflux of pancreatic juice into the biliary system was found regardless of dilatation, and cellular proliferative activity of the gallbladder mucosa was increased in both the nondilated and dilated type. Therefore, excision of the extrahepatic bile duct including cholecystectomy is recommended for AAPBD with nondilatation of the CBD.

Cyclooxygenase-2 expression in the gallbladder of patients with anomalous arrangement of the pancreaticobiliary duct

Anomalous arrangement of the pancreaticobiliary duct (AAPBD) is frequently associated with gallbladder carcinoma. Cyclooxygenase (COX) is a key enzyme in the synthesis of prostaglandins, and 2 isoforms have been identified: COX-1 and COX-2. Overexpressed in several precancerous lesions, the COX-2 isoform is thought to be involved in chronic inflammation and carcinogenesis. To determine whether COX expression correlates with biliary histology in patients with AAPBD, the authors investigated the expression of COX-1 and COX-2 in the gallbladder of patients with AAPBD. Gallbladder specimens were obtained from 31 patients with AAPBD (mean age, 5.0 years), and from 7 patients with other hepatobiliary diseases as controls (mean age, 2.0 years). The authors quantified levels of COX-1 and COX-2 by the extent and intensity of staining after immunohistochemistry using anti-COX-1 and anti-COX-2 antibodies. Mucosal hyperplasia of the gallbladder was identified in 18 of 31 patients with AAPBD. The gallbladder epithelium of all specimens expressed COX-2. Marked expression of COX-2 was noted in specimens with mucosal hyperplasia, the mean immunoreactive score of which was significantly higher than that of specimens without mucosal hyperplasia (7.50 +/- 1.86 and 5.62 +/- 2.26, respectively; P =.021). In contrast, COX-1 expression was not detected in any specimen. Enhanced expression of COX-2 is related to mucosal hyperplasia of the gallbladder in patients with AAPBD. Thus, COX-2 may play a regulatory role in the proliferation of gallbladder epithelium, which may lead to carcinogenesis in patients with AAPBD.

New insight into bile flow dynamics in anomalous arrangement of the pancreaticobiliary duct.

In an anomalous arrangement of the pancreaticobiliary duct (AAPBD), two-way regurgitation, including pancreaticobiliary reflux and biliopancreatic reflux (BPR), might occur. Although pancreaticobiliary reflux has been shown by high levels of pancreatic enzymes in bile, there have been few reports of clinical evidence of BPR in AAPBD. Recently, spiral computed tomography combined with intravenous infusion cholangiography (IVC-SCT) has been reported to be useful for visualizing the biliary anatomy, which may allow a new insight into bile flow dynamics in AAPBD. Fifteen paediatric patients with AAPBD were examined before operation using both IVC-SCT and endoscopic retrograde cholangiopancreatography. Visualization of the main pancreatic duct on IVC-SCT defined the presence of BPR. Blood samples were also analysed when the initial symptom was strongly present. BPR was observed in six of 15 patients. Pancreatic enlargement and hyperamylasaemia were significantly more frequent in patients without BPR than in those with BPR. Levels of enzyme markers of cholestasis were also higher in the former group. The present study, using IVC-SCT, has identified clinical evidence of BPR in AAPBD. Further studies are necessary to determine the correlation between BPR and clinical manifestations.

Mucosal cell proliferation activity of the gallbladder in children with anomalous arrangement of the pancreaticobiliary duct.

Anomalous arrangement of the pancreaticobiliary duct (AAPBD) is an anatomical maljunction of the bile duct and the pancreatic duct that is frequently associated with gallbladder carcinoma. In patients with AAPBD, it has been postulated that pancreatic juice regurgitates into the biliary tree, and the mixture of refluxed pancreatic juice and stagnant bile juice acts as an irritant factor to the biliary tract epithelium, leading to chronic inflammation and metaplasia. Eventually these mucosal changes may progress to invasive carcinoma. We reviewed clinicopathologic studies on epithelial changes of the gallbladder in patients with AAPBD to clarify the implications relevant to carcinogenesis. Conventional histological studies have shown that the most characteristic change observed in the gallbladder of children with this anomaly was epithelial hyperplasia. Furthermore, the incidence of mucosal hyperplasia was significantly increased in the gallbladder of children in whom the pancreatic duct joined the common bile duct (P-C type) compared with the incidence in children in whom the common bile duct joined the pancreatic duct (C-P type). In addition, cell kinetic studies have demonstrated increased cellular proliferative activity of the gallbladder in children with AAPBD. Cell proliferative activity was significantly elevated in children with the P-C type of AAPBD compared with that in children with the C-P type of anomaly. In conclusion, AAPBD may yield increased cell proliferation in the gallbladder of patients with this anomaly in early childhood, resulting in epithelial hyperplasia. Although it remains unknown which agents are responsible for promoting the activation of cellular function, it seems that bile acids and refluxed pancreatic proteases are likely play a role in such promotion. Further investigations are needed to elucidate the mechanism of increased cellular function.

Cellular activity in the gallbladder of children with anomalous arrangement of the pancreaticobiliary duct

Background/Purpose: Anomalous arrangement of the pancreaticobiliary duct (AAPBD) is closely related to congenital biliary dilatation and frequently associated with biliary tract malignancy. To examine the mechanism of biliary tract carcinogenesis in patients with AAPBD, we investigated histologically the early changes in cell proliferative kinetics of the gallbladder mucosa of children with AAPBD. Methods: Twenty-three specimens of gallbladder were obtained from 23 children with AAPBD, and six control specimens were obtained from pediatric patients. All specimens were fixed routinely and paraffin embedded and examined histologically with H&E staining and immunohistochemically with monoclonal antibody Ki-67(MIB-1), which reacts with a human nuclear antigen associated with cell proliferation. Ki-67 labeling index (Ki-67 LI) was obtained by counting the numbers of Ki-67-positive cells per 1,000 gallbladder epithelial cells. Results: Significant differences in Ki-67 LI were noted between children with and without AAPBD. Furthermore, Ki-67 LI and the incidence of epithelial hyperplasia of gallbladder were significantly higher in children with AAPBD in whom the major pancreatic duct joined the common bile duct (P-C type) than in those in whom the common bile duct joined the major pancreatic duct (C-P type). Conclusions: Cellular proliferative activity was increased in children with AAPBD, especially those with the P-C-type anomaly. These results suggest that the early mucosal changes of the gallbladder occurred in early childhood of patients with AAPBD and might be associated with gallbladder cancer. Early diagnosis and early surgical division of the biliary tract and pancreatic duct is recommended for children with AAPBD.

A CASE OF PERFORATED CHOLEDOCHAL CYST

A case of perforated choledochal cyst is reported with a review of the literature. A healthy 2-year-old 11 month boy was admitted to the hospital because of acute onset of epigastralgia, vomiting and elevated plasma level of amylase with high-grade fever. Imaging studies revealed a cystic dilatation of the common bile duct, Auggesting a choledochal cyst with pancreatitis. The disease was well controlled conservatively 4 days following admission, when he abruptly developed a severe right upper quadrant abdominal pain. There were abdominal distension, rebound tenderness and muscular defense at the same region. Emargency laparotomy revealed darkly stained ascites and thickened wall of the choledochal cyst with two perforated holes on the anterior wall. Biliary peritonitis due to perforated choledochal cyst was suggested. Peritoneal drainage was performed and a T-tube was placed through the holes into the cyst. A postoperative T-tube cholangiogram confirmed the presence of an anomalous insertion of the pancreatic duct into the common duct. Three months after the initial surgery, the choledochal cyst was resected and the biliary tract was reconstructed by hepaticojejunostomy. An anomalous arrangement of the pancreaticobiliary duct seemed to facilitate reflux of pancreatic secretions into the biliary tree and contribute to cholangitis followed by spontaneous perforation of the choledochal cyst.

A case of adenomyomatosis of gallbladder associated with anomalous arrangement of the pancreaticobiliary duct

A case of adenomyomatosis of gallbladder associated with anomalous arrangement of the pancreaticobiliary duct

Early mucosal changes of the gallbladder in patients with anomalous arrangement of the pancreaticobiliary duct

BACKGROUND & AIMS: Anomalous arrangement of the pancreaticobiliary duct (AAPBD) is frequently associated with gallbladder cancer. The aim of this study was to characterize the early epithelial changes of the gallbladder that might be associated with carcinogenesis in patients with AAPBD. METHODS: Routinely fixed tissue specimens obtained from pediatric patients with AAPBD and control patients were examined histologically with H&E, periodic acid-Schiff, and argyrophilic nucleolar organizer region (AgNOR) staining. RESULTS: Epithelial hyperplasia was frequently observed in the gallbladder of patients with AAPBD but not in patients without this anomaly. The incidence of epithelial hyperplasia was significantly higher in the gallbladder of patients in whom the major pancreatic duct joined the common bile duct (P-C type) than in patients in whom the common bile duct joined the pancreatic duct (C-P type). The mean AgNOR number in the gallbladder mucosa was significantly higher in patients with AAPBD than in patients without AAPBD. Patients with C-P type AAPBD had a significantly higher mean AgNOR number of the gallbladder mucosa than patients with C-P type AAPBD. CONCLUSIONS: Patients with AAPBD, especially those with the P-C type anomaly, have increased cellular proliferative activity of the gallbladder mucosa in early childhood. (Gastroenterology 1996 May;110(5):1614-8)

A CASE OF EARLY GALLBLADDER CANCER ASSOSIATED WITH ANOMALOUS ARRANGEMENT OF THE PANCREATICOBILIARY DUCT WITHOUT DILATION OF THE COMMON BILE DUCT

A 50-year-old man was admitted to the hospital because of epigastric pain and hyperamylasemia. Abdominal CT and ultrasonography revealed few polyps in the gallbladder. ERCP film showed the common channel of 23mm in length and common bile duct of 8mm in width. A diagnosis of anomalous arrangement of the pancreaticobiliary duct without dilation of the common bile duct was made. The values of amylase of bile juce were 9, 700 IU/l in the gallbladder and 177, 700 IU/l in the common bile duct. So cholecystectomy with resection of the extrahepatic common bile duct and hepatico-jejunostomy were performed. By histopathologic study a papillaryadenocarcinoma (m, ly0, v0) was found in a part of the gallbladder well. In a review of the Japanese literaure, only 11 cases of early cancer of the gallbladder assosiated with anomalous arrangement of the pancreaticobiliary duct without diliation of the common-bile duct could be seen.