[Gallbladder carcinosarcoma associated with pancreaticobiliary maljunction with biliary dilation:a case report].

A 68-year-old female patient was referred to our hospital with acute cholangitis. Computed tomography revealed common bile duct dilatation, gallbladder fundal tumor, and gallbladder wall thickening attached to the tumor. Cholangiography revealed pancreaticobiliary maljunction with biliary dilation. The patient was diagnosed with pancreaticobiliary maljunction with biliary dilation and gallbladder cancer and underwent liver S4b+5 and bile duct resection and reconstruction. Pathological results revealed that the gallbladder fundal tumor included sarcoma, and the gallbladder wall thickening had adenocarcinoma;thus, the patient was diagnosed with gallbladder carcinosarcoma.

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.

Imaging of Pancreaticobiliary Maljunction.

Pancreaticobiliary maljunction (PBM) is a congenital malformation in which the pancreatic and bile ducts join outside the duodenal wall, usually forming a long common channel. A major issue in patients with PBM is the risk of biliary cancer. Because the sphincter of Oddi does not regulate the pancreaticobiliary junction in PBM, pancreatic juice frequently refluxes into the biliary tract and can cause various complications, including biliary cancer. Most cancers arise in the gallbladder or dilated common bile duct, suggesting that bile stasis is related to carcinogenesis. Early diagnosis and prophylactic surgery to reduce the risk of cancer are beneficial. The diagnosis of PBM is made mainly on the basis of imaging findings. The development of diagnostic imaging modalities such as multidetector CT and MR cholangiopancreatography has provided radiologists with an important role in diagnosis of PBM and its complications. Radiologists should be aware of PBM despite the fact that it is rare in non-Asian populations. In this review, the authors present an overview of PBM with emphasis on diagnosis and management of PBM and its complications. For early diagnosis, the presence of extrahepatic bile duct dilatation or gallbladder wall thickening may provide a clue to PBM with or without biliary dilatation, respectively. The pancreaticobiliary anatomy should be closely examined if imaging reveals these findings. Radiologists should also carefully evaluate follow-up images in PBM patients even years after prophylactic surgery because residual bile ducts remain at risk for cancer.©RSNA, 2020.

Development of a CT-based radiomics-clinical model to diagnose acute pancreatitis on nonobvious findings on CT in children with pancreaticobiliary maljunction.

OBJECTIVES: Since neither abdominal pain nor pancreatic enzyme elevation is specific for acute pancreatitis (AP), the diagnosis of AP in patients with pancreaticobiliary maljunction (PBM) may be challenging when the pancreas appears normal or nonobvious on CT. This study aimed to develop a quantitative radiomics-based nomogram of pancreatic CT for identifying AP in children with PBM who have nonobvious findings on CT. METHODS: PBM patients with a diagnosis of AP evaluated at the Children's Hospital of Soochow University from June 2015 to October 2022 were retrospectively reviewed. The radiological features and clinical factors associated with AP were evaluated. Based on the selected variables, multivariate logistic regression was used to construct clinical, radiomics, and combined models. RESULTS: Two clinical parameters and 6 radiomics characteristics were chosen based on their significant association with AP, as demonstrated in the training (area under curve [AUC]: 0.767, 0.892) and validation (AUC: 0.757, 0.836) datasets. The radiomics-clinical nomogram demonstrated superior performance in both the training (AUC, 0.938) and validation (AUC, 0.864) datasets, exhibiting satisfactory calibration (P > .05). CONCLUSIONS: Our radiomics-based nomogram is an accurate, noninvasive diagnostic technique that can identify AP in children with PBM even when CT presentation is not obvious. ADVANCES IN KNOWLEDGE: This study extracted imaging features of nonobvious pancreatitis. Then it developed and evaluated a combined model with these features.

Magnetic resonance cholangiopancreatography identification of pancreaticobiliary maljunction in the colombian pediatric population. / Unión biliopancreática anómala por colangiorresonancia en población pediátrica colombiana.

OVERVIEW: Pancreaticobiliary maljunction (PBM) is a congenital malformation characterized by a long common pancreaticobiliary channel which causes sphincter of Oddi malfunction. In children, it is typically diagnosed using magnetic resonance cholangiopancreatography (MRCP). It is associated with congenital biliary dilatation, pancreatitis, and gallbladder and bile duct tumors at adulthood. Studies in the western population are rare. Given its morbidity rate, it should be searched for in the western pediatric population. The objective of this study was to look for and identify the presence of pancreaticobiliary maljunction through MRCP in pediatric patients with biliary or pancreatic disease, as well as to find out other associated factors. METHODS: MRCP was used to measure common channel length, pancreatic duct length, and bile duct diameter in 41 pediatric patients with biliary or pancreatic disease. RESULTS: The common channel could only be measured in 17.6% of cases, 50% of which were >8 mm long. All patients were female and had congenital biliary dilatation. No age-related differences were found in terms of bile duct length. CONCLUSIONS: PBM is present in the western pediatric population, but prevalence and morbidity are unknown. Larger studies are required to identify morbidity and mortality, as well as prevalence among patients.

OBJETIVO: La unión biliopancreática anómala (UBPA) es una malformación congénita caracterizada por un canal común pancreatobiliar largo que impide el adecuado funcionamiento del esfínter de Oddi. Su diagnóstico en niños se realiza comúnmente mediante colangiopancreatografía por resonancia magnética (CPRM). Se asocia a dilatación biliar congénita, pancreatitis y tumores de la vesícula y la vía biliar en la edad adulta. Los estudios en población occidental son escasos; debido a su morbilidad resulta de relevancia la búsqueda en población pediátrica occidental. Este estudio pretende buscar e identificar la presencia de unión biliopancreática anómala mediante CPRM de pacientes pediátricos con enfermedad de la vía biliar o pancreática, al igual que identificar otros factores asociados. METODOS: Se midió por CPRM la longitud del canal común, el conducto pancreático y el diámetro de la vía biliar de 41 pacientes pediátricos con patología biliar o pancreática. RESULTADOS: El canal común solo pudo ser medido en el 17,6% de los casos, de los cuales el 50% tuvo una longitud >8 mm, siendo todos ellos pacientes femeninos con dilatación biliar congénita; no se encontraron diferencias en la longitud de la vía biliar relacionado con la edad. CONCLUSIONES: La UBPA es una malformación que se encuentra presente en población pediátrica occidental con prevalencia y morbilidad desconocida; se requieren estudios a mayor escala para identificar morbimortalidad y prevalencia de pacientes con esta malformación.

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.

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.