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Background: The aim of this study was to assess factors for delineating the pancreaticobiliary junction in the presence of pediatric congenital choledochal cysts (CCC) using Magnetic resonance cholangiopancreatography (MRCP)., Methods: Retrospective review of medical records for 48 patients with CCC was conducted, including demographics, biliary amylase and MRCP findings if available. With univariate and multivariate logistic regression, we measured significant factors affecting pancreaticobiliary maljunction(PBM) diagnoses by MRCP., Results: Of the subjects enrolled with CCC. Twenty-eight cases had PBM according to MRCP. Univariate analysis confirmed that age, cyst diameter > 30 mm and cysts that descended to the introitus pelvis affected junctional delineation and detection of PBM (P<0.05). Stepwise logistic regression analysis confirmed large cysts in the introitus pelvis predicted pancreaticobiliary junctional delineation in MRCP and these data agreed with the literature. A correlation between cyst diameter and the length of the common channel was found as was cyst diameter and biliary amylase although there were no significant differences between them., Conclusions: Age, cyst diameter >30 mm and descending cysts into the introitus pelvis affected junctional delineation of the pancreatic and bile duct in PBM with MRCP. Large cyst descension into the introitus pelvis was an independent factors affecting PBM detection.

Aim: To demonstrate the imaging findings of biliopancreatic and pancreatico-biliary reflux in patients with anomalous union of the pancreatico-biliary duct (AUPBD) on gadoxetic acid-enhanced functional magnetic resonance cholangiography (fMRC)., Methods: This study included six consecutive patients (two men and four women; mean age 47.5 years) with AUPBD. All subjects underwent endoscopic retrograde cholangiopancreatography (ERCP); one subject also underwent bile sampling of the common bile duct (CBD) to measure the amylase level because his gadoxetic acid-enhanced fMRC images showed evidence of pancreatico-biliary reflux of pancreatic secretions. Of the five patients with choledochal cysts, four underwent pylorus-preserving pancreaticoduodenectomy., Results: The five cases of choledochal cysts were classified as Todani classification I. In three of the six patients with AUPBD, injected contrast media reached the distal CBD and pancreatic duct on delay images, suggesting biliopancreatic reflux. In two of these six patients, a band-like filling defect was noted in the CBD on pre-fatty meal images, which decreased in size on delayed post-fatty meal images, suggesting pancreatico-biliary reflux of pancreatic secretions, and the bile sampled from the CBD in one patient had an amylase level of 113,000 IU/L. In one of the six patients with AUPBD, contrast media did not reach the distal CBD due to multiple CBD stones., Conclusion: Gadoxetic acid-enhanced fMRC successfully demonstrated biliopancreatic reflux of bile and pancreatico-biliary reflux of pancreatic secretions in patients with AUPBD with and without choledochal cysts.

Background/purpose: The aim of this study was to investigate the diagnostic potential of computed tomography cholangiography (CTC) and magnetic resonance cholangiopancreatography (MRCP) in children with pancreaticobiliary maljunction (PBM)., Methods: Fifty-three children with PBM were consecutively treated between 1997 and 2009. Among them, the patients who underwent CTC and/or MRCP preoperatively were enrolled in this study. Computed tomography cholangiography was examined after infusion of meglumine iodoxamate with subsequent 3-dimensional rendering. The visualization of the biliary and pancreatic duct systems was evaluated and compared with that visualized with MRCP. The findings of direct cholangiography were used as the standard of reference., Results: Of the 53 cases with PBM, 17 cases were examined by CTC, 10 cases by MRCP, and 17 with both. The extrahepatic bile tract was visualized in 32 (94.1%) of 34 patients in CTC and in all 27 patients in MRCP. The intrahepatic bile duct was more frequently demonstrated by MRCP than by CTC (96.3% vs 70.6%, P = .02). Pancreaticobiliary maljunction was noted in 13 (38.2%) of 34 with CTC and in 12 (44.4%) of 27 with MRCP. The minimum age for visualization of PBM was at 10 months in CTC and at 1 year and 11 months in MRCP, respectively. The main pancreatic duct was more frequently visualized by MRCP than by CTC (81.5% vs 8.8%, P < .001)., Conclusions: Magnetic resonance cholangiopancreatography provides superior visualization of the intrahepatic duct and the pancreatic system when compared with CTC. However, it is still challenging to perform a good-quality examination in young infant. The great advantage of CTC is its ability to produce high-quality images without respiratory artifacts and that it allows accurate assessment of the presence of PBM equivalent to MRCP., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Anomalous union between the pancreatic and biliary systems (APBDU) has been reported to produce choledochal cyst. The aim of this experiment was to evaluate the role of sphincteroplasty to adverse effect of APBDU in an animal model. Twelve mongrel puppies were randomly divided into a control group (n = 5) and an experimental group (n = 7). A well-established model of APBDU was produced in both groups. Transduodenal sphincteroplasty was performed only on the experimental group. For all animals, serial chemical analyses of serum were performed, and biliary tree sizes were measured by magnetic resonance cholangiography 2.5 months after the experimental surgery. At the time of animal sacrifice, 3 months after the experimental surgery, operative cholangiography was performed, and bile juice and tissues were obtained for chemical analysis and histologic examination. Dilatation of the bile duct and thickening of the wall of the bile duct were observed less frequently in the experimental group than in the control group. There were no significant differences found in pancreatic enzyme activity in the bile juice between the two groups. Denudation of the mucosa was the predominant mucosal change seen in the experimental group, while epithelial hyperplasia was the predominant mucosal change found in the control group. Our experiment shows that sphincteroplasty is not effective to prevent the pancreaticobiliary reflux, but may be effective to reduce the degree of both bile duct dilatation and mural thickening in the APBDU puppy model.

Aim: To clarify the pathogenesis of biliary dilatation associated with anomalous union of the pancreatic and biliary ducts (AUPBD)., Methods: Direct cholangiopancreatograms of 350 adult patients with or with suspicion of hepatobiliary or pancreatic disorders were reviewed. AUPBD was diagnosed cholangiopancreatographically, when the pancreaticobiliary ductal union was located above the narrow distal segment of the bile duct, which represents the action of the sphincter of Oddi. The narrow portion of the terminal choledochus was defined as symmetrical stricture of the common bile duct just above the pancreaticobiliary ductal union., Results: AUPBD was found in 36 patients. Among cholangiopancreatographic features, the narrow portion of the terminal choledochus was the most pathognomonic for AUPBD (accuracy, 98%); it was present in 29 (81%) patients with AUPBD, but was not found in any patients without AUPBD. Among patients with AUPBD, biliary dilatation (>10 mm) was more frequent in those with the narrow portion of the terminal choledochus (23/29) than in those without (2/7; P=0.018) AUPBD. Among the patients with both AUPBD and the narrow portion of the terminal choledochus, there was a strong negative correlation between the minimum diameter of the narrow portion and the maximum diameter of the choledochus (r=-0.78, P<0.001), suggesting that the degree of biliary narrowing at the narrow portion correlates with that of upstream biliary dilatation., Conclusion: The narrow portion of the terminal choledochus, a pathognomonic radiologic feature of AUPBD, may be a cause of biliary dilatation in patients with AUPBD.

Aim: To investigate the relationship between pancreatic amylase in bile duct and the clinico-pathological features in adult patients with choledochal cyst and anomalous pancreatico-biliary ductal union (APBDU)., Methods: From 39 patients who underwent surgery for choledochal cyst between March 1995 and March 2003, we selected 15 adult patients who had some symptoms and were radiologically diagnosed as APBDU, and their clinico-pathological features were subsequently evaluated retrospectively. However, we could not obtain biliary amylase in all the patients because of the surgeon's slip. Therefore, we measured the amylase level in gall bladder of 10 patients and in common bile duct of 11 patients., Results: Levels of amylase in common bile duct and gall bladder ranged from 11,500 to 212,000 IU/L, and the younger the patients, the higher the biliary amylase level (r = -0.982, P<0.01). Pathologically, significant correlation was found between the size of choledochal cyst and the grade of inflammation (r = 0.798, P<0.01). And, significant correlation was found between the level of amylase in gall bladder and the grade of hyperplasia. On the other hand, there was no correlation to the age of symptomatic onset or inflammatory grade (r = 0.743, P<0.05). Level of lipase was elevated from 6,000 to 159,000 IU/L in bile duct and from 14,400 to 117,000 IU/L in the gall bladder; however, there was no significant correlation with age or clinico-pathological features., Conclusion: The results support the notion that amylase has a particular role in the onset of symptoms, and suggest that a large amount of biliary amylase induces early onset of symptom, thereby making early diagnosis possible.

Objective: The purpose of this study was to determine whether MR cholangiopancreatography can accurately depict anomalous pancreaticobiliary ductal union in children with choledochal cysts., Subjects and Methods: Twenty children (age range, 1 month-13 years; mean age, 4.6 years; all girls) who were diagnosed with choledochal cyst by sonography underwent MR cholangiopancreatography with a single-shot fast spin-echo sequence. The type of choledochal cyst and anomalous pancreaticobiliary ductal union were characterized on the basis of MR cholangiopancreatographic findings and were compared with the finding of intraoperative cholangiography., Results: The type of choledochal cyst (type Ia, n = 4; type Ic, n = 7; type IVa, n = 7; type IVb, n = 2) determined on MR cholangiopancreatography correlated with that identified on intraoperative cholangiography in each patient. Anomalous pancreaticobiliary ductal union was detected by MR cholangiopancreatography and intraoperative cholangiography in 12 (60%) and 16 (80%) of 20 patients, respectively. The types of anomalous pancreaticobiliary ductal union as determined on MR cholangiopancreatography (type A, n = 2; type B, n = 7; type C, n = 3) were concordant with those of intraoperative cholangiography in 11 of 12 patients. In five of eight patients with choledochal cyst (type Ia, n = 1; type IVa, n = 5; type IVb, n = 2) in whom MR cholangiopancreatography could not depict anomalous pancreaticobiliary ductal union, anomalous pancreaticobiliary ductal union was documented on intraoperative cholangiography that was performed after choledochal cyst resection., Conclusion: MR cholangiopancreatography provides diagnostic information about anomalous pancreaticobiliary ductal union in children with choledochal cyst.

In this article the authors discuss whether or not diagnostic potential of MR cholangiopancreatography is strong enough to replace direct cholangiography in all cases. The pre-surgery analysis of a variety of pancreato-biliary disorders diagnosed using MRCP images is presented with the emphasising the importance of source images. Six cases of pancreato-biliary disorders are presented in which MRCP indicated the place of ductal stenosis as well as the morphologic variants or ductal uninspected shape which is critical for surgery or planned drainage. Coronal and axial MRCP source and MIP images were obtained with 0.5 T Gyroscan NT. Anomalies of the biliary or pancreatic ducts included two cases of choledochal cystic dilatation; two cases of aberrant biliary ducts, one case of gallbladder duct variant and a case of an additional pancreatic duct. In 3 out of 6 cases, the MRCP source images produced using the complementary method supplied more complete information concerning ductal junctions than the MIP images. Whereas in 3 out of 6 cases, both kinds of images were equally reliable. In 4 out of 6 cases, endoscopy was performed, and in 2 cases ERCP images were not diagnostic for ductal anatomy. However, full delineation of biliary and pancreatic ducts was complete in all MRCP images. MRCP within source images and maximum intensity projections show particular promise for the assessment of pancreato-biliary anomalies in order to reduce the number of higher-risk endoscopic interventions. The technique should be the method of choice in cases of suspected pancreato-biliary anomaly resulting from any imaging modality and is helpful for planning the optimal drainage method. In the long run this practice would reduce the number of ducts damaged during surgery.

Objective: Since choledochal cyst is frequently associated with the anomalous union of pancreaticobiliary duct (AUPBD), AUPBD has been regarded to be the etiologic factor of choledochal cyst. However, the clinical significance of AUPBD an patients with choledochal cyst has not been clearly defined. Therefore, to clarify the significance of AUPBD in choledochal cyst patients, we compared the clinical features of patients with choledochal cyst according to the presence or absence of AUPBD., Methods: Among 52 cases which were diagnosed as choledochal cyst out of 5,037 ERCP referrals between August 1990 and December 1996, we selected 44 cases, in which the pancreaticobiliary junction was clearly visualized on cholangio-pancreaticography. These cases were divided into AUPBD-present group (n = 28) and AUPBD-absent group (n = 16). Clinical features were compared between the two groups. Furthermore, in AUPBD-present group, clinical data were also analyzed according to Kimura's classification of AUPBD., Results: In our study, AUPBD was associated with choledochal cyst in 28 (64%) cases. AUPBD was found only in type I and IV according to Todani's classification of choledochal cyst. There were no significant differences between the AUPBD-present group and the AUPBD-absent group in the incidence of gallstone disease, while the incidence of acute inflammation was 93% (26/28) in the AUPBD-absent group (p < 0.01). Carcinoma developed only in the AUOBD-present group (9/28, 32%) (p < 0.05). Pancreatic disorders (i.e. pancreatic stone, pancreatitis or pancreatic cancer) occurred in 12 of 28 cases in the AUPBD-present group (43%), while only in 1 of 16 cases in the AUPBD-absent group (6%) (p < 0.05)., Conclusion: AUPBD associated with choledochal cyst may have implications not only as a possible etiologic factor but also as an important factor that may affect the clinical course, surgical planning and prognosis. In cases with choledochal cyst, we should make an effort to evaluate the presence of AUPBD.

Congenital variants of the pancreaticobiliary junction are rare anomalies that are usually diagnosed in childhood because of recurrent abdominal pain and jaundice. These lesions are associated with several pancreaticobiliary diseases including pancreatitis and malignancy. We observed a rare anomaly of the pancreaticobiliary tract with a combination of several ductal malformations, i.e., choledochal cyst, long common channel and incomplete pancreas divisum in a patient with pancreatic cancer.

Fusion variations of the pancreatic ducts were studied to elucidate the significance of such variations. We classified structural fusion anomalies of the main and accessory pancreatic ducts on endoscopic retrograde cholangio-pancreatography (ERCP) in 37 patients with anomalous arrangement of the pancreaticobiliary ductal system (AAPB). The fusion variations of the pancreatic ducts were classified into five types: common, ansa pancreatica, branch fusion, looped, and separated. These fusion variations, except for common type, were found in 68% of the 37 patients with AAPB on ERCP. Fusion variations of the pancreatic ducts were very frequent (93%) in the 30 patients with congenital cystic dilatation of the common bile duct (CCD). The branch confluence fashion, in which the terminal bile duct communicated with a pancreatic duct branch, was found only in patients with cystic dilatation cyst of the CCD, and it appeared that cystic dilatation cyst of CCD might differ from spindle or cylindrical cyst originating from embryonic formation of an anomalous confluence. It was also suggested that in patients with fusion variations of the pancreatic ducts, the flow of pancreatic juice might be disordered, leading to the development of acute pancreatitis or pancreatic dysfunction. Consequently, it appears to be necessary to carefully examine patients with AAPB for the presence or absence of any fusion variations of the pancreatic ducts and to observe such patients with long-term monitoring by ERCP, and computed temography, and with pancreatic function tests.

Nine patients with choledochal cyst have been reviewed. There was a 6:3 preponderance of females. Four patients presented in adulthood. Cholangiocarcinoma developed in three patients with an interval ranging from 2 to 35 years following surgery. The predominant complaint was of abdominal pain and symptoms had been present from weeks up to 28 years. Imaging by sonography may be all that is required in the paediatric age group, but in adults further confirmation of the choledochal cyst and the associated anomalous junction of the pancreatico-biliary duct (AJPB) by direct cholangiography is recommended.

"Narrow segments" (or stenotic portions) of the common bile duct (CBD) distal to cysts in two patients (a 50-yr-old woman and a 25-yr-old man) with choledochal cyst (CholCyst) were studied macroscopically and histologically. The narrow segments of the ducts were patent and connected with the main pancreatic duct extraduodenally, whereas the CBD in controls were neither dilated nor stenotic, and joined the main pancreatic duct within the duodenal wall. These narrow segments were 1 cm long. In case 1, a minute orifice was found in the narrow segment of the duct macroscopically and was identified microscopically as a small duct from a pancreatic lobule. In case 2, a small pancreatic duct also joined the narrow segment of the duct. These small pancreatic ducts were derived from the ventral pancreas, based on distribution of PP-islets. Therefore, the narrow duct distal to the cyst in patients with CholCyst (or congenital biliary dilatation) was considered to be a branch of the ventral pancreatic duct.

We studied relationship between an involvement of Oddi's sphincter in a choledochopancreatic junction and clinical appearances in 120 patients with anomalous arrangement of the pancreaticobiliary ductal system. The 120 composed of 83 with a sphincteric mechanism at the junction of the common bile and pancreatic duct and 37 without the sphincteric mechanism. We found that influences of sphincteric mechanism at the junction closely related to the length of the common duct between an orifice of the papilla of Vater and a junction of the common bile and pancreatic duct. And in all 83 with a sphincteric mechanism at the junction the length of the common duct showed less than 1.5 cm, and in all 37 without a sphincteric mechanism at the junction the common duct showed more than 1.5 cm in length. There was no correlation between a distribution of the sphincter muscle and clinical appearances, association with congenital choledochal cyst, cholelithiasis and bile duct cancer in the 120 patients.

The main pancreatic duct and the common bile duct open into the second part of the duodenum alone or after joining as a common channel. A common channel of greater than 15 mm (an anomalous pancreaticobiliary duct) is associated with congenital cystic dilatation of the common bile duct and carcinoma of the gall bladder. Even a long common channel (greater than or equal to 8 mm) is associated with a higher frequency of carcinoma of the gall bladder. Gall stones smaller than the common channel and a long common channel predispose to gall stone induced acute pancreatitis. Separate openings for the two ductal systems predisposes to development of gall stones and alcohol induced chronic pancreatitis. The role of ductal union has also been investigated in primary sclerosing cholangitis and biliary atresia.

The authors report the case of an 3 months old caucasian boy which had an extrahepatic biliary atresia associate with a congenital biliary cyst and an anomalous pancreaticobiliary junction. The patency between gallbladder and duodenum permits the operative opacification of this long common pancreaticobiliary channel. The congenital biliary cyst was located on the upper part of the common bile duct and was obstructed by biliary concretions. Unfortunately, no patency between intrahepatic bile ducts and this cyst was found. Because this long common pancreaticobiliary channel may cause biliary dilatation or biliary cancer, we performed an hepatic portoenterostomy rather an hepatic portocholecystostomy. A few weeks later, the bile flow was restored and the jaundice decreased. With a follow up of 18 months, this boy is currently anicteric, despite the hepatic fibrosis found on the operative biopsy. The association extrahepatic biliary atresia-long common pancreaticobiliary channel is uncommon and only 20 cases were reported. In our case, the pancreatic juice reflux into the biliary tract might have caused the dilatation of the common bile duct and the biliary atresia by chronic obstructive cholangiopathy.