[Double extrahepatic bile duct with pancreaticobiliary maljunction:a case report].

A 76-year-old woman with a suspected double extrahepatic bile duct was referred to our hospital. MRCP revealed that the left hepatic and posterior ducts combined to form the ventral bile duct and that the anterior duct formed the dorsal bile duct. ERCP demonstrated that the ventral bile duct was linked with the Wirsung duct. Amylase levels in the bile were unusually high. Based on these findings, we diagnosed a double extrahepatic bile duct with pancreaticobiliary maljunction and choledocholithiasis. Duplicate bile duct resection and bile duct jejunal anastomosis were performed considering the risk of biliary cancer due to pancreaticobiliary maljunction. The resected bile duct epithelium demonstrated no atypia or hyperplastic changes.

Risk of carcinogenesis in the biliary epithelium of children with congenital biliary dilatation through epigenetic and genetic regulation.

PURPOSES: Congenital biliary dilatation (CBD), defined as pancreaticobiliary maljunction (PBM) with biliary dilatation, is a high risk factor for biliary tract cancer (BTC). KRAS and p53 mutations reportedly affect this process, but the mechanisms are unclear, as is the likelihood of BTC later in life in children with CBD. We investigated potential carcinogenetic pathways in children with CBD compared with adults. METHODS: The subjects of this study were nine children with CBD and 13 adults with PBM (10 dilated, 3 non-dilated) without BTC who underwent extrahepatic bile duct resections, as well as four control patients who underwent pancreaticoduodenectomy for non-biliary cancer. We evaluated expressions of Ki-67, KRAS, p53, histone deacetylase (HDAC) and activation-induced cytidine deaminase (AID) in the biliary tract epithelium immunohistochemically. RESULTS: The Ki-67 labeling index (LI) and expressions of KRAS, p53, HDAC, and AID in the gallbladder epithelium were significantly higher or tended to be higher in both the children with CBD and the adults with PBM than in the controls. CONCLUSIONS: BTC may develop later in children with CBD and in adults with PBM, via HDAC and AID expression and through epigenetic and genetic regulation.

Pancreaticobiliary maljunction without biliary dilatation in pediatric patients.

PURPOSE: Pancreaticobiliary maljunction (PBM) without biliary dilatation is a condition in which dilatation of the bile duct is not seen in patients with PBM. Recently, the Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) published new diagnostic criteria for PBM. In these criteria, biliary dilatation is defined according to the standard diameter at each age. We reviewed cases of pediatric patients with PBM without biliary dilatation. METHODS: From 1992 to 2019, 134 patients with PBM were treated in our institution. Among these, 7 patients were retrospectively diagnosed with PBM without biliary dilatation. The clinical information was retrospectively assessed in these patients. RESULTS: Of the seven patients, six were female. All patients had symptoms similar to those of patients with congenital biliary dilatation. In all seven patients, the diagnosis of PBM was made before definitive surgery. Six patients had type B PBM, and one had type D PBM. All patients underwent extrahepatic bile duct resection and hepaticojejunostomy, and their symptoms resolved. One patient experienced postoperative complications of anastomotic leakage followed by anastomotic stricture. CONCLUSION: The present report revealed important clinical features of this entity. However, there are still some issues that need to be discussed, and further research is needed.

Preoperative one-stop magnetic resonance imaging evaluation of the pancreaticobiliary junction and hepatic arteries in children with pancreaticobiliary maljunction: a prospective cohort study.

PURPOSE: Pancreaticobiliary maljunction (PBM) is routinely assessed by intraoperative cholangiography (IOC), whereas accompanying abnormalities in the hepatic artery are assessed by preoperative contrast multi-slice computed tomography (MSCT). We evaluated the efficiency of performing one-stop preoperative magnetic resonance imaging (MRI) for delineating the anatomy of the pancreaticobiliary junction and the hepatic artery. METHODS: The subjects of this prospective analysis were children who underwent Roux-en-Y surgery for PBM in our institution during a recent 3-year period. Preoperative one-stop MRI was conducted using 3.0-T MRI. The efficiency of one-stop MRI was compared with that of IOC for assessing the bile duct, and with contrast MSCT for assessing the blood vessels. RESULTS: Sixty-five children underwent one-stop preoperative MRI, which had a 100% concordance rate, versus IOC for assessing the bile duct type. Protein plugs or cholelithiasis were identified by IOC in 8 children and by one-stop MRI in 45 children (P = 0.0233). Cholangitis was not identified by IOC in any children but it was identified by one-stop MRI in 29 children. MSCT was also performed in 46 children and revealed a variant hepatic artery in 9 and cholangitis in 21. One-stop MRI had a 100% concordance rate versus MSCT. CONCLUSION: Preoperative one-stop MRI accurately delineates the bile duct anatomy as well as the hepatic artery, cholangitis, and protein plugs in children with PBM.

Endoscopic retrograde cholangiopancreatography in children with symptomatic pancreaticobiliary maljunction: A retrospective multicenter study.

BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP) has been widely used in pediatric patients with cholangiopancreatic diseases. AIM: To evaluate the efficacy, safety, and long-term follow-up results of ERCP in symptomatic pancreaticobiliary maljunction (PBM). METHODS: A multicenter, retrospective study was conducted on 75 pediatric patients who were diagnosed with PBM and underwent therapeutic ERCP at three endoscopy centers between January 2008 and March 2019. They were divided into four PBM groups based on the fluoroscopy in ERCP. Their clinical characteristics, specific ERCP procedures, adverse events, and long-term follow-up results were retrospectively reviewed. RESULTS: Totally, 112 ERCPs were performed on the 75 children with symptomatic PBM. Clinical manifestations included abdominal pain (62/75, 82.7%), vomiting (35/75, 46.7%), acholic stool (4/75, 5.3%), fever (3/75, 4.0%), acute pancreatitis (47/75, 62.7%), hyperbilirubinemia (13/75, 17.3%), and elevated liver enzymes (22/75, 29.3%). ERCP interventions included endoscopic sphincterotomy, endoscopic retrograde biliary or pancreatic drainage, stone extraction, etc. Procedure-related complications were observed in 12 patients and included post-ERCP pancreatitis (9/75, 12.0%), gastrointestinal bleeding (1/75, 1.3%), and infection (2/75, 2.7%). During a mean follow-up period of 46 mo (range: 2 to 134 mo), ERCP therapy alleviated the biliary obstruction and reduced the incidence of pancreatitis. The overall effective rate of ERCP therapy was 82.4%; seven patients (9.3%) were lost to follow-up, eight (11.8%) re-experienced pancreatitis, and eleven (16.2%) underwent radical surgery, known as prophylactic excision of the extrahepatic bile duct and hepaticojejunostomy. CONCLUSION: ERCP is a safe and effective treatment option to relieve biliary or pancreatic obstruction in symptomatic PBM, with the characteristics of minor trauma, fewer complications, and repeatability.

Congenital or acquired? Obstructive jaundice in reoperated duodenal atresia.

A 55-day-old boy was transferred to our unit with intestinal obstruction and obstructive jaundice after two neonatal operations for duodenal atresia and intestinal malrotation. Abdominal ultrasound showed dilated intrahepatic and extrahepatic ducts with cut-off at the distal common bile duct (CBD). He underwent emergency laparotomy for adhesive intestinal obstruction with a contained abscess from mid-jejunal perforation. Biliary dissection was not attempted due to poor preoperative nutritional status. Tube cholecystostomy was created for biliary decompression. Postoperative magnetic resonance cholangiopancreatography showed dilated CBD with cut-off at the ampulla but did not demonstrate pancreaticobiliary maljunction (PBMJ). The diagnostic dilemma was whether our patient had congenital PBMJ or had developed biliary stricture from perioperative ischaemic scarring. He underwent definitive surgery at 7 months: excision of dilated CBD with Roux-en-Y hepaticojejeunal reconstruction, excisional tapering duodenoplasty and jejunostomy creation. Intraoperative finding was type I choledochal cyst and subsequently confirmed on histology. Postoperative recovery was uneventful and bilirubin levels normalised.

Increased prevalence of pancreaticobiliary maljunction in biliary malignancies.

AIM: The incidence of pancreaticobiliary maljunction is thought to approximate 1:100,000 within Western populations. We aimed to study the significance of pancreaticobiliary maljunction in biliary tract malignancies. METHODS: Medical records and magnetic resonance cholangiopancreatography images of 252 consecutive patients treated for biliary malignancies during 2005-2016 were reviewed. Patients with other known risk factors for biliary cancers (n = 27) were excluded. A common pancreaticobiliary channel measuring ⩾10 mm outside the duodenal wall was defined as pancreaticobiliary maljunction. MAIN RESULTS: Of the 225 patients, a reliably interpretable preoperative magnetic resonance cholangiopancreatography was available for 73 (32%). Sex (47% vs 57% females) and age at diagnosis (67 vs 66 years) were similar among patients with or without an magnetic resonance cholangiopancreatography (p = ns for both). In magnetic resonance cholangiopancreatography, a pancreaticobiliary maljunction with a median length of 20 mm (range 10-23 mm) was identified in four patients (5.5%, 95% confidence interval 1.6-14), while none had evident accompanying biliary tree dilatation. Pancreaticobiliary maljunction patients were significantly more often females (100% vs 43%, p = 0.043), less likely to have intrahepatic bile duct cancer (0% vs 65%, p = 0.019) while more likely to have gallbladder cancer (75% vs 22%, p = 0.044) compared to the others. Age at diagnosis (66 vs 67 years, p = 0.898), extrahepatic bile duct cancer incidence (25% vs 13%, p = 0.453), and survival status at last follow-up (50% vs 42% alive, p = 1.000) were comparable between the subgroups. CONCLUSION: The prevalence of pancreaticobiliary maljunction is substantially higher in adults with biliary malignancies than one would expect based on its incidence, reinforcing the etiologic role of pancreaticobiliary maljunction especially in females with gallbladder cancer.