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Background Pancreatic juice is constantly activated by contaminated bile in patients with pancreaticobiliary maljunction (PBM). Here, we report a case of laparoscopic distal pancreatectomy for a patient with PBM and sphincterotomized papilla, resulting in fatal pancreatic fistula. Case presentation A 79-year-old man was diagnosed with pancreatic intraductal papillary mucinous neoplasm and common bile duct stones. Endoscopic sphincterotomy was performed prior to surgery. The pancreatic duct was simultaneously visualized when the contrast agent was injected into the common bile duct. Sudden bleeding was observed from the abdominal drain on postoperative day (POD) 6. Emergent stent graft placement and coil embolization were performed for bleeding from the splenic artery. On POD 9, the drainage fluid changed to yellowish in color with bile contamination. For internal drainage of the digestive fluid, endoscopic retrograde biliary tube and pancreatic drainage tube were placed. On POD 24, second emergent coil embolization was performed for bleeding from the left gastric artery. On POD 25, open abdominal drainage was performed. On POD 32, third emergent coil embolization was performed for bleeding from the gastroduodenal artery. Subsequently, remnant pancreatic resection was performed. On POD 39, massive bleeding was again observed from the abdominal drain. Emergency arterial portography revealed bleeding in the right wall of the superior mesenteric vein. The patient died of hemorrhagic shock on the same day. Conclusions The extreme risk of severe pancreatic fistula after distal pancreatectomy should be considered in patients with PBM and sphincterotomized papilla. In this extraordinary situation, surgeons should promptly decide whether to resect the remnant pancreas to prevent losing the patient.

Background The genetic changes underlying carcinogenesis in patients with risk factors of gallbladder carcinoma (GBC) remains controversial, especially in patients with pancreaticobiliary maljunction (PBM). This study aimed to clarify the association between risk factors of GBC and genetic changes using next-generation sequencing (NGS). Methods We retrospectively analyzed resected tissues of 64 patients who were diagnosed with GBC (n = 26), PBM [with GBC (n = 8), without GBC (n = 20)], and chronic cholecystitis, used as a control group (n = 10). DNA was extracted from tumors and their surrounding tissues, which were precisely separated by laser-capture microdissection. Gene alterations of 50 cancer-related genes were detected by NGS and compared with clinical information, including PBM status. Results The most frequent gene alterations in GBC tissues occurred in TP53 (50%), followed by EGFR (20.6%), RB1 (17.6%), and ERBB2 (17.6%). Gene alterations that were targetable by molecular targeted drugs were detected in 20 cases (58.8%). Statistical analysis of gene alterations and risk factors revealed that TP53 alteration rate was higher in GBC patients with PBM than those without PBM (p = 0.038), and the TP53 mutation rates in the epithelium of control patients, epithelium of PBM patients without GBC, peritumoral mucosa of GBC patients with PBM, and tumor tissue of GBC patients with PBM were 10, 10, 38, and 75%, respectively (p Conclusions TP53 alteration more than KRAS mutation was revealed to underlie carcinogenesis in patients with PBM.

BACKGROUND: The indications for endoscopic retrograde cholangiopancreatography (ERCP) in infant patients (age

Background Spontaneous common bile duct (CBD) perforation is an extremely rare disease in adults. We report an adult case of CBD perforation due to choledocolithiasis accompanied with pancreaticobiliary maljunction, which is, to our knowledge, the first such case report based on a search using PubMed. Case presentation A 71-year-old woman with consciousness disorder was transported to the emergency department of another hospital. She was diagnosed as having severe peritonitis with septic shock and transferred to our hospital for emergency surgery. Enhanced computed tomography (CT) revealed supraduodenal CBD dilation similar to a diverticulum and a defect of bile duct wall continuity. Furthermore, CT showed a long common channel of the pancreaticobiliary duct, so she was diagnosed as having spontaneous CBD perforation with pancreaticobiliary maljunction. Emergency surgery was performed that revealed a necrotic diverticulum-like change on the supraduodenal part, and a 2.5 × 1 cm perforation was found on the anterolateral wall of the CBD. Peritoneal lavage was performed, and CBD perforation was resolved with a T-tube. The patient suffered refractory intra-abdominal and retroperitoneal abscess formation and bleeding from the abdominal wall, which required a long period of postoperative management. The T-tube was removed on day 136, and the patient was transferred on day 153. Conclusion The cause of CBD perforation is commonly considered to be increased intraductal pressure or weakness of the bile duct wall. In this case, pancreaticobiliary maljunction may have significantly influenced onset and the postoperative course. This case suggests that early surgical intervention and appropriate drainage are important to ensure survival.

Background Pancreaticobiliary maljunction (PBM) is a condition characterized by chronic inflammation due to refluxed pancreatic juice into the biliary tract that is associated with an elevated risk of biliary tract cancer. DNA double-strand breaks (DSBs) are considered the most serious form of DNA damage. DSBs are provoked by inflammatory cell damage and are recognized as an important oncogenic event in several cancers. This study used γ-H2AX, an established marker of DSB formation, to evaluate the impact of DNA damage on carcinogenesis in PBM. Methods We investigated γ-H2AX expression immunohistochemically in gallbladder epithelium samples obtained from 71 PBM cases and 19 control cases. Results Fourteen PBM cases with gallbladder adenocarcinoma were evaluated at non-neoplastic regions. A wide range of nuclear γ-H2AX staining was detected in all PBM and control specimens. γ-H2AX expression was significantly higher in PBM cases versus controls (median γ-H2AX-positive proportion: 14.4 % vs. 4.4 %, p = 0.001). Among the PBM cases, γ-H2AX expression was significantly higher in patients with carcinoma than in those without (median γ-H2AX-positive proportion: 21.4 % vs. 11.0 %, p = 0.031). Conclusions DSBs occurred significantly more abundantly in the PBM gallbladder mucosa, especially in the context of cancer, indicating an involvement in PBM-related carcinogenesis.

We used a novel diagnostic Fourier transform (FT) algorithm of the entire extrahepatic bile duct (EHBD) measured by magnetic resonance cholangiography (MRC) to evaluate subtle deformation of bile duct lumen, indicating the malignant potential of EHBD, in patients with pancreaticobiliary maljunction (PBMJ) and in a comparative group of controls without PBMJ. From the workstation, the EHBD lumen was traced automatically and a 2D diagram cross section was measured at 0.5 mm-longitudinal intervals. The FT-based integrated power spectral density function value (FTPSDI) of the diameter or area (mm2 or mm4/Hz) and the phase value distribution entropy (PVDE) were also measured. There were 16 patients with undilated PBMJ and 7 with dilated PBMJ. The control group comprised 10 patients with a normal bile duct, 20 with bile duct carcinoma (BDC), and 1 with primary sclerosing cholangitis. Both the diameter and area of the dilated bile ducts and the ducts with early- or advanced-stage BDC were significantly greater than those of the normal duct (p

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. 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. 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. 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 (PBM) is a malformation in which the pancreatic and bile ducts join outside the duodenal wall. It is associated with various biliary and pancreatic diseases. In addition, patients with PBM carry a substantial lifetime risk of developing biliary or gallbladder carcinoma. We aimed to present a multicenter case series of PBM from Turkey. This study was conducted in adult and pediatric PBM patients who were referred to three tertiary reference centers of Turkey for endoscopic retrograde cholangiopancreatography (ERCP) between July 2007 and May 2020. The clinical presentations, types of PBM, ERCP findings, surgical histories, and the postoperative courses, including the development of biliary malignancies, were retrospectively reviewed. The study group included 47 (31 adult and 16 children) patients. Type D PBM was more frequent (13/41: 27.7%) than that reported in Eastern studies. Type A PBM was more common in the adults (51.6% vs. 12.5%, p

Background Metachronous pancreatic and gallbladder cancer is a rare condition and has a dismal prognosis. Herein, we present a patient with triple metachronous primary pancreatic and gallbladder cancer associated with pancreaticobiliary maljunction who achieved long-term survival after undergoing repeat curative surgery. Case presentation A 65-year-old female patient with advanced gallbladder cancer associated with pancreaticobiliary maljunction underwent extended cholecystectomy with extrahepatic bile duct resection. The pathological diagnosis was T3N0M0 stage III A papillary adenocarcinoma with hepatic invasion. During a monthly follow-up, a diffuse hypovascular 2.0 × 1.5-cm mass was detected in the pancreatic head 6.2 years after the initial surgery. Hence, the patient underwent pancreaticoduodenectomy. Histological examination revealed T3N0M0 stage IIA well-differentiated adenocarcinoma without lymph node metastases. Marked inflammatory reaction was observed in the non-cancerous lesions of the proximal pancreatic head parenchyma containing bile pigment within ductular lumens. After 12.5 years from the initial surgery, total pancreatectomy for a 4.0 × 3.0-cm mass in the remnant pancreas was performed. Histological examination revealed T3N1M0 stage IIB moderately differentiated adenocarcinoma with lymph node metastases. Hence, surgical curative resection was achieved. Based on the pathological findings, a definitive diagnosis of triple metachronous pancreatic and gallbladder cancer was made. The pathology suggests no precursor lesions such as pancreatic intraepithelial neoplasia (PanIN) and atypical flat lesions, but marked inflammations in the non-cancerous lesions, strengthening our hypothesis that chronic inflammation induced by the pancreaticobiliary maljunction is related to carcinogenesis of the pancreas. Despite further adjuvant chemotherapy, the patient’s general condition worsened; however, she remained alive 15.2 years after the initial surgery while receiving the best supportive care. Conclusions Repeat curative surgery for triple metachronous cancer was associated with a favorable prognosis. Both the biliary tract and the pancreas should be closely monitored during follow-up among patients with pancreaticobiliary maljunction, which can be managed with curative surgery.

Treatments for hepatolithiasis include peroral endoscopy, percutaneous cholangioscopy, and surgery. Balloon-assisted endoscopic retrograde cholangiopancreatography (BAERCP) has been widely performed in recent years for patients with hepatolithiasis after biliary reconstruction. However, accidental bowel perforation caused by BAERCP may need emergency surgery. Here, we describe a 77-year-old Japanese woman diagnosed with acute cholangitis due to hepatolithiasis after biliary reconstruction (a biliary diversion operation for pancreaticobiliary maljunction). She underwent BAERCP for treatment of hepatolithiasis, however, a small-bowel perforation occurred. She underwent an emergency operation to suture the perforation and add a catheter jejunostomy. She had no postoperative complications after surgery and was discharged 11 days after surgery. One month later, she was readmitted and underwent percutaneous transjejunal cholangioscopy-guided lithotripsy with complete removal of the calculi. Although endoscopists should be careful to avoid small-bowel perforation during BAERCP, if perforation occurs, addition of a catheter jejunostomy during emergency surgery can be easily transitioned to subsequent treatment of the hepatolithiasis.

Background: The commonly used anatomical classification of congenital biliary dilatation by Todani is not directly related to types of pancreaticobiliary maljunction (PBM). This work is to investigate clinical presentation and surgical outcomes according to PBM classification proposed by the Japanese Study Group on Pancreaticobiliary Maljunction. Pancreaticobiliary junction angle, common channel length, and diameter of dilatation are studied as well. Materials and Methods: Patients of redo operation, without preoperative images, and lacking clearly documented outcomes were excluded. After exclusion, 79 patients who underwent Roux-en-Y hepaticojejunostomy between January 1994 and December 2019 were enrolled for this retrospective study. Medical records were reviewed, and perioperative parameters were collected. Todani's classification, PBM types, pancreaticobiliary junction angle, common channel length, and dilatation diameter were determined based on magnetic resonance cholangiopancreatography or computed tomography. Clinical presentation and outcome were compared between groups of different anatomical features. Results: PBM type A (stenotic), type B (nonstenotic), and type C (dilated channel) consisted of 48 (60.8%), 18 (22.8%), and 11 (13.9%) patients, respectively; and two patients (2.5%) had no PBM. Patients of PBM type A were younger and had more Todani's type Ia lesion. Patients of PBM type B and C had either Todani's type Ia or Ic lesion, but type IVa had only PBM type B. Longer common channel (1.27 vs. 0.81 cm, P < 0.001) and wider dilatation (4 vs. 2 cm, P < 0.001) were found in patients with right pancreaticobiliary angle (90°). Clinical outcome was similar in different Todani's types, PBM types, and pancreaticobiliary angle. Serum alkaline phosphatase level higher than 675 U/L was associated with major perioperative complications. Preoperative jaundice, mass, and dilatation wider than 5 cm were related to subsequent liver cirrhosis. Conclusion: PBM types and pancreaticobiliary junction groups are feasible for surgical planning, but not related directly to outcome. Palpable abdominal mass suggests higher risk of perioperative and late complications. For subsequent liver cirrhosis, laboratory data of disease onset and dilatation diameter wider than 5 cm are important risk factors.

Ultrasonography has shown that many T2 gallbladder carcinomas (GBCs) demonstrate a deep hypoechoic area, and many shallow T2 GBCs show a conically thickened, outermost hyperechoic layer. However, this report describes a case of a hyperplastic polyp, of the gallbladder, with similar sonographic findings. This case reviews and analyzes the ultrasonographic imaging, as well as the pathologic correlation. Endoscopic ultrasound (EUS) revealed a thickened, outermost hyperechoic layer beneath a protruding lesion in the gallbladder fundus. The lesion was coupled with a deep hypoechoic area and a pancreaticobiliary maljunction (PBM). Pre-contrast computed tomogram (CT) demonstrated a soft tissue density mass in the fundus. Magnetic resonance imaging (MRI) disclosed no decreased-diffusion lesion under the diffusion-weighted image. Without any finding suggestive of an obvious advanced GBC, a full-thickness laparoscopic cholecystectomy was performed. The histopathology report indicated a hyperplastic polyp, which consisted of hypertrophic muscle, increased fibrosis, and a conically thickened subserosal fatty layer underneath the polyp. A specific benign protruded lesion may, though rarely, demonstrate similar EUS findings to those of shallow T2 carcinoma.

Background and Aim Pancreaticobiliary anomalies are rare and often present with cryptic signs and symptoms, thus delaying appropriate treatment. Methods Endoscopic retrograde cholangiopancreatography (ERCP) was used to define pancreaticobiliary anomalies. A retrospective review was performed of 5522 ERCPs conducted at a tertiary care center from 1972 to 2015. Results There were 249 (4.5%) patients with pancreaticobiliary anomalies, including 179 patients with pancreas divisum (PD), 44 patients with choledochal cyst (CC) (Todani's classification Type I: extrahepatic cyst 31, Type III; choledochocele 9, Type V: Caroli's disease 4), 20 patients with anomalous pancreaticobiliary ductal union (APDU), and 6 patients with other abnormalities. Of 179 patients with pancreas divisum, 8 (4.5%) required minor sphincterotomies for multiple unexplained acute pancreatitis. Of the 31, 15 (48%) Type I CC patients underwent an operation. In patients with Type III CC (choledochocele), seven of the nine were treated by endoscopic sphincterotomy, and two patients were treated by surgery. Four patients with Type V CC (Caroli's disease) were managed nonoperatively. Of the 20 patients with APDU, 8 (40%) required operative intervention. Six patients were found to have other anomalies: two with pancreas bifidum, one with a duplication of the gallbladder, one with a cystic duct diverticulum, one with an annular pancreas, and one with an abnormal cystic duct origin. These patients were treated based on their etiology. Conclusion Pancreaticobiliary anomalies are rare and can be defined using ERCP. The appreciation of these abnormalities is important for the proper diagnosis and treatment of these rare biliary and pancreatic disorders., Pancreaticobiliary anomalies were analyzed using endoscopic retrograde cholangiopancreatography (5522 patients) in an urban medical center in the United States. A total of 249 (4.5%) patients were found to have a pancreaticobiliary anomaly. There were 179 patients with pancreas divisum, 44 patients with choledochal cyst, 20 patients with anomalous pancreaticobiliary ductal union, and 6 patients with other abnormalities.

Background The present study aimed to evaluate anatomical variations of the right hepatic artery (RHA) in patients with congenital biliary dilatation (CBD) and the appropriate approach in laparoscopic surgery for CBD. Methods The medical records of 36 patients who underwent laparoscopic or open surgery for CBD from 1996 to 2018 were retrospectively reviewed. Radiological evaluation of the origin and course of the RHA in these 36 patients were compared with 195 control patients without CBD. Results The incidence of the RHA crossing anterior to the common hepatic duct (CHD) was significantly higher in patients with CBD than in those without CBD (33% versus 10%, P = .0001). There was no intraoperative injury of the RHA, irrespective of the course of the RHA. The CHD was divided at the caudal side of the RHA in 11 of 12 patients (92%) with the anterior type of RHA, and in 13 of 24 patients (54%) with the posterior type of RHA (P = .03). Conclusions Patients with CBD had a higher incidence of the RHA crossing anterior to the CHD than patients without CBD. Preservation of the RHA in each situation is necessary during surgery for CBD in the era of laparoscopic surgery.

Pancreatitis is the most common complication of endoscopic retrograde cholangiopancreatography (ERCP). To assess the prevalence and factors associated with post-ERCP pancreatitis (PEP) in a Chinese pediatric population. Sixty-six children who underwent ERCP between March 2018 and March 2019 at Shanghai Children’s Medical Center were retrospectively recruited for the study. Clinical data, including demographics, indications, comorbidities, and procedural data, were reviewed to identify the prevalence and factors associated with PEP. Ninety-two ERCPs were performed on 66 pediatric patients aged from 8 months to 14 years. The indications for ERCP were chronic pancreatitis (49, 53.2%), pancreaticobiliary maljunction (19, 20.7%), pancreas divisum (19, 20.7%), and pancreatic pseudocyst (5, 5.4%). All ERCPs were performed for therapeutic purposes. PEP was identified in 19 (20.7%) patients; there were ten mild cases, eight moderate cases, and one severe case. The univariate analysis revealed that a history of chronic pancreatitis was negatively associated with PEP (P = 0.033), and sphincterotomy was positively associated with PEP (P = 0.01). The multivariate analysis showed that sphincterotomy was a risk factor for PEP (P = 0.017, OR 4.17; 95% CI, 1.29, 13.54). Our data revealed a high prevalence of PEP in a Chinese pediatric population. Chronic pancreatitis was a protective factor, and sphincterotomy was a risk factor for PEP development.

BACKGROUND Forme fruste choledochal cyst (FFCC), characterized by a minimally dilated common bile duct (CBD) and associated pancreaticobiliary maljunction (PBM), is difficult to diagnose in a timely manner in clinical practice. However, endoscopic retrograde cholangiopancreatography (ERCP) can clearly delineate CBD and PBM. Hence, we evaluated the value of ERCP in the diagnosis and management of FFCC. METHODS The medical records of patients diagnosed with FFCC were retrospectively reviewed. From January 2003 to December 2010, patients underwent surgery without ERCP (non-ERCP group); from January 2011, all patients underwent pre-operative ERCP (ERCP group). Demographics, laboratory results, perioperative data and post-operative complications were compared between the two groups. RESULTS There were 24 patients in the ERCP group and 11 in the non-ERCP group. Laboratory data improved to a greater extent after ERCP. ERCP was superior to both magnetic resonance cholangiopancreatography and ultrasonography in delineating CBD and PBM. In the ERCP group, 20 of the 24 patients were noted to have filling defects in the biliary tract and underwent pre-operative endoscopic sphincterotomy. Intra-operative blood loss was lower and transfusion less frequent in the ERCP group (P

Highlights • Lemmel syndrome is a rare condition that leads to cholangitis and/or pancreatitis due to intraduodenal diverticulum. • Surgery is considered for the treatment of severe or repeated symptoms in patients with this condition. • We herein report a successfully diagnosed and treated case of Lemmel syndrome with pancreaticobiliary maljunction., Background Lemmel syndrome is a rare condition that leads to cholangitis and/or pancreatitis due to intraduodenal diverticulum. Surgery is considered for the treatment of severe or repeated symptoms in patients with this condition. Case presentation An 81-year-old woman was admitted to our hospital, complaining of general fatigue, BT 38.8 degree, and right hypochondoralgia. Her hepatobiliary enzyme levels were elevated, and enhanced abdominal computed tomography revealed dilation of the common bile duct and intraduodenal diverticulum. After restarting oral intake, her symptoms were exacerbated. Endoscopic retrograde cholangio-pancreatography (ERCP) revealed pancreaticobiliary maljunction and parapapillary diverticulum. Under a diagnosis of Lemmel syndrome with pancreaticobiliary maljunction complicated by acute pancreatitis and cholangitis, we performed extrahepatic bile duct resection with cholecystectomy and papilloplasty. Her postoperative course was uneventful, and the patient was discharged 20 days after surgery. She remains well at 5 months after surgery. Conclusion We herein report a successfully diagnosed and treated case of Lemmel syndrome with pancreaticobiliary maljunction.

Highlights • The article represents an extremely rare case of the combination of choledochocele and extra-hepatic bile duct duplication. • The combination of choledochocele and bile duct duplication might escalate the pancreatitis and cholangitis progression. • The pathogenesis in relation to pancreaticobiliary maljunction is discussed., Background Combination of choledochocele and extra-hepatic duct duplication is an extremely rare congenital abnormality. Case presentation The patient was an 81-year-old Japanese man. He visited the emergency room for severe abdominal colic pain. He was diagnosed with severe pancreatitis with cholelithiasis and treated conservatively by percutaneous trans-hepatic gallbladder drainage (PTGBD) for 4 months. Thereafter, he was transferred to our institute and cholangiography was performed via the PTGBD tube, revealing cholecysto- and choledocho-lithiasis. The cystic-duct joined the right hepatic duct with extra-hepatic bile duct duplication and the terminal bile duct flowed into the cystic papilla of Vater. The main pancreatic duct also joined into the cystic papilla. These observations confirmed choledochocele with extra-hepatic bile duct duplication. Surgical exploration was performed, and hepatico-jejunostomy with hepatic-ductplasty and cholecystectomy with choledocholithotomy were carried out. He was discharged and his course was uneventful. Conclusion A very rare combined case of choledochocele with bile duct duplication, which would escalate the pancreatitis and cholangitis, was successfully treated. Their pathogeneses in relation to pancreaticobiliary maljunction is discussed.

Background: To investigate the effect of embedded hepaticojejunostomy in children with pancreaticobiliary maljunction (PBM) without biliary dilatation. Materials and Methods: The clinical data of 10 patients with nondilated PBM from February 2017 to July 2020 were retrospectively analyzed. Perioperative liver function indexes were compared. Results: All patients were diagnosed by magnetic resonance cholangiopancreatography (MRCP) combined with intraoperative cholangiography. There were 5 cases of Komi type I and 5 cases of type II; the diameter of the common bile duct was 4-9 mm (median: 6 mm); and the length of the common channel was 5-15 mm (median: 9.25 mm). The procedure for one patient with common duct stones was converted to open surgery. Laparoscopic cholecystectomy, common bile duct resection, and embedded hepaticojejunostomy were successfully performed in all 10 cases. The average operation time was 225 ± 96.64 min, and the intraoperative blood loss was 2-5 mL. The mean time to oral intake was 3.5 ± 1.65 days (range: 2-5 days), and the mean hospitalization duration was 6.2 ± 2.44 days (range: 5-8 days). The differences in liver function indexes in the perioperative period were statistically significant (P < .05). The patients were followed-up for 13 to 54 months (median: 40 months). All patients grew well and there was no bile duct dilatation, calculus, or cirrhosis on B-ultrasound examinations. Conclusions: The clinical manifestations of nondilated PBM are often concealed, and preoperative MRCP was important for obtaining a diagnosis. Laparoscopic cholecystectomy, common bile duct resection, and embedded hepaticojejunostomy are feasible for treating nondilated PBM.

Rationale: Mixed neuroendocrine non-neuroendocrine neoplasm (MiNEN) is a rare tumor. MiNEN of the gallbladder (GB) with pancreaticobiliary maljunction (PMJ) is extremely rare. The origin of MiNEN of the GB remains unknown; the biliary tract normally lacks neuroendocrine cells. MiNEN of the GB has a poor prognosis; because of its rarity, no treatment or management guidelines have been established yet. Patient concerns: A 47-year-old male presenting with right hypochondrial pain and malaise for 3 months was referred to our hospital for further management. Diagnosis: The neuron-specific enolase level was increased. Contrast-enhanced computed tomography revealed a mass of 70 mm in size with unclear boundaries in the liver. The GB was surrounded by this mass, narrowing the lumen of the GB. Many swollen lymph nodes were observed in the hepatoduodenal ligament. Endoscopic retrograde cholangiopancreatography revealed a PMJ with a non-dilated biliary duct. A percutaneous biopsy was performed on the liver mass, and the pathological findings were neuroendocrine carcinoma (NEC) (small cell type). We diagnosed a NEC of the GB, T3N1M0, stage IIIB (Union for International Cancer Control, 7th edition). Interventions: Because of advanced lymph node metastasis, we considered this tumor difficult to cure solely by surgical intervention. After initial chemotherapy consisting of cisplatin and irinotecan, a marked reduction in both tumor and lymph node sizes enabled conversion surgery. The pathological diagnosis of the resected tumor was MiNEN consisting of NEC and adenocarcinoma. The primary lesion was the adenocarcinoma occupying the luminal side of the GB. As a postsurgical treatment, the patient received additional irradiation therapy to the common hepatic duct and liver stump because of positive surgical margins. Outcomes: At 13 months postoperatively, computed tomography findings revealed the appearance of a hypervascular liver tumor, and laboratory data showed increased serum neuron-specific enolase levels. Chemotherapy was unsuccessful, leading to the death of the patient 36 months from the date of diagnosis. Lessons: There are several reports on the development of MiNEN of the GB. In our case, a PMJ-related adenocarcinoma of the GB transdifferentiated into NEC. Further accumulation of cases is necessary to establish a treatment strategy for MiNEN of the GB.

Introduction The right hepatic artery crossing the ventral side of the common hepatic duct is a relatively frequent abnormality. This aberrant right hepatic artery not only interferes with dissection of the common bile duct and hepaticojejunostomy for choledochal cyst but can also cause postoperative anastomotic stenosis. Case presentation A 14-year-old patient presented with upper abdominal pain and was diagnosed with a choledochal cyst (Type IVA in Todani Classification) and pancreaticobiliary maljunction. Abdominal enhanced computed tomography showed aberrant right hepatic artery located at the ventral side of the common hepatic duct. Laparoscopic choledochal cyst resection and hepaticojejunostomy were planned. Intraoperative findings also showed the aberrant right hepatic artery crossing the common hepatic duct ventrally as detected on preoperative computed tomography. Laparoscopic dorsal side repositioning of the aberrant right hepatic artery was performed because it appeared to compress the common hepatic duct and risked causing postoperative anastomotic stenosis. We performed laparoscopic hepaticojejunostomy by replacing the aberrant right hepatic artery dorsally to facilitate suturing and prevent postoperative anastomotic stenosis. The postoperative course was uneventful, with no findings suggestive of anastomotic stenosis. Discussion The abnormality of the right hepatic artery is reported to be a primary cause of anastomotic stenosis after hepaticojejunostomy. Once anastomotic stenosis or stricture develops, it is often difficult to treat. The prevention of the stenosis is important. Conclusions In choledochal cyst with aberrant right hepatic artery, dorsal repositioning is effective for preventing postoperative anastomotic stenosis and cholestasis., Highlights • The right hepatic artery crossing the anterior aspect of the common hepatic duct is a relatively frequent abnormality. • The aberrant right hepatic artery can cause the postoperative anastomotic stenosis. • We performed laparoscopic hepaticojejunostomy by replacing the aberrant right hepatic artery dorsally.

Background/Aims: To investigate the clinical profiles of children with pancreatitis caused by pancreaticobiliary malformation.Methods: We retrospectively analysed the clinical data of children diagnosed with pancreatitis at our institute from June 2017 to January 2021.Results: A total of 195 patients and 169 control subjects were included in this study. Twenty-six (13.3%) patients had pancreaticobiliary malformation-related pancreatitis. The average age of onset in the pancreaticobiliary malformation pancreatitis (PMP) group was lower than that in the non-PMP group, and the difference was statistically significant. The number of patients in the PMP group that had jaundice was significantly higher than that of the non-PMP group (P < 0.05). Logistic regression analysis showed that total bilirubin (TB) and γ-glutamyltransferase (GGT) (odds ratio = 1.096, P < 0.01) were independent predictors of pancreaticobiliary malformation-related pancreatitis in children. The positive detection rate of pancreaticobiliary malformation was 68% for abdominal ultrasound, 38.4% for abdominal enhanced computed tomography, and 91.3% for magnetic resonance cholangiopancreatography (MRCP). The recurrence rate (34.6%) in the PMP group was higher than that in the non-PMP group (15.4%, P < 0.05); surgical therapy had the lowest recurrence rate. Age at initial onset of pancreatitis was younger and the period to recurrence was shorter in the PMP group than in the non-PMP group (P < 0.05).Conclusion: Pancreaticobiliary malformation is one of the major causes of paediatric pancreatitis. Elevated TB and GGT in patients with pancreatitis may be suggestive for underlying pancreaticobiliary malformation not solely to pancreatitis. MRCP should be used when pancreatitis due to pancreaticobiliary malformation is suspected. Surgery or endoscopic retrograde cholangiopancreatography-guided intervention may be helpful but further study is needed.

Background Pancreaticobiliary maljunction (PBM) can be classified into two categories, PBM with congenital biliary dilatation (CBD) or PBM without biliary dilatation, and the management of PBM is often controversial. The treatment for PBM with CBD is prophylactic flow diversion surgery, and some authors have reported that the incidence of cancer after extrahepatic bile duct excision is less than 1%. A very rare case of intrahepatic cholangiocarcinoma 6 years after flow diversion surgery for PBM with CBD is reported. Case summary A 30-year-old man was diagnosed as having PBM with CBD, Todani classification type IVA, because of abnormal liver enzyme profiles. He underwent flow diversion surgery and cholecystectomy, and the specimen showed adenocarcinoma foci, pT1, pStage IA. Five and a half years passed without any recurrence of bile duct cancer. However, 6 years after his operation, computed tomography showed a gradually growing nodule in the bile duct. Fluorodeoxyglucose positron emission tomography showed high uptake, and magnetic resonance imaging showed restricted diffusion signals. On double balloon enteroscopy, the nodule at the posterior bile duct-jejunum anastomosis was directly visualized, and its biopsy specimen showed adenocarcinoma. The patient underwent right lobectomy and biliary reconstruction. The pathological diagnosis was intraductal papillary neoplasm with high-grade intraepithelial neoplasia, pTis, pN0, pStage 0. The patient's postoperative course was uneventful, and he has had no recurrence up to the present time. Conclusion This case suggests the necessity of careful observation after flow diversion surgery, especially when PBM with CBD is detected in adulthood.

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.

AIMS To investigate whether genetic or inflammatory pro-oncogenic factors are relevant to the increased risk of gallbladder cancers in patients with pancreaticobiliary maljunction (PBM). METHODS AND RESULTS Mutations in KRAS exon 2 were examined by a highly sensitive, droplet digital PCR platform using surgically resected specimens of PBM-associated (n = 31) and non-associated gallbladder cancers (n = 49). The tissue expression of IL-6 and IL-33, which are suspected to promote biliary carcinogenesis, was analysed by quantitative real-time PCR and in-situ hybridisation. The incidence of KRAS mutations was similarly low in PBM-associated (five of 32 cases; 16%) and non-associated cancers (four of 49 cases; 8%) (P = 0.272). The tissue expression of IL-33 mRNA, but not IL-6 mRNA, was significantly higher in PBM-associated gallbladder cancers than in gallbladder cancers without PBM (P = 0.004). A similar degree of IL-33 overexpression was also observed in the background non-cancerous mucosa in cases of PBM-associated gallbladder cancers, and was significantly greater than that in PBM cases with cholecystitis alone (P

BACKGROUND Pancreaticobiliary maljunction (PBM) is an uncommon congenital anomaly of the pancreatic and biliary ductal system, defined as a union of the pancreatic and biliary ducts located outside the duodenal wall. According to the Komi classification of PBM, the common bile duct (CBD) directly fuses with the ventral pancreatic duct in all types. Pancreas divisum (PD) occurs when the ventral and dorsal ducts of the embryonic pancreas fail to fuse during the second month of fetal development. The coexistence of PBM and PD is an infrequent condition. Here, we report an unusual variant of PBM associated with PD in a pediatric patient, in whom an anomalous communication existed between the CBD and dorsal pancreatic duct. CASE SUMMARY A boy aged 4 years and 2 mo was hospitalized for abdominal pain with nausea and jaundice for 5 d. Abdominal ultrasound showed cholecystitis with cholestasis in the gallbladder, dilated middle-upper CBD, and a strong echo in the lower CBD, indicating biliary stones. The diagnosis was extrahepatic biliary obstruction caused by biliary stones, which is an indication for endoscopic retrograde cholangiopancreatography (ERCP). ERCP was performed to remove biliary stones. During the ERCP, we found a rare communication between the CBD and dorsal pancreatic duct. After clearing the CBD with a balloon, an 8.5 Fr 4-cm pigtail plastic pancreatic stent was placed in the biliary duct through the major papilla. Six months later, his biliary stent was removed after he had no symptoms and normal laboratory tests. In the following 4-year period, the child grew up normally with no more attacks of abdominal pain. CONCLUSION We consider that ERCP is effective and safe in pediatric patients with PBM combined with PD, and can be the initial therapy to manage such cases, especially when it is combined with aberrant communication between the CBD and dorsal pancreatic duct.

Background In congenital biliary dilatation, the protein plug is likely impacted, especially in a dilated common channel. However, nobody has discussed whether this dilatation of common channel remains after radical operation. The aim of this study was to investigate the situation of the dilated common channel after radical operation. Methods The status of the common channel was investigated at radical operation and follow-up. We then evaluated whether dilation of the common channel remained postoperatively. Moreover, the association between status of the common channel and postoperative pancreatic complication was evaluated. Results Postoperative condition of the common channel was able to be evaluated ultrasonographically in 42 patients. The common channel was dilated at the time of operation in 18 patients and remained dilated postoperatively in 11 of these 18 patients. Dilatation of the common channel was not identified in any patients without dilatation at the time of operation. No patients in this series showed any postoperative pancreatic complications, even if a dilated common channel remained. Conclusions Dilatation of the common channel can remain postoperatively in congenital biliary dilatation, but does not appear to be associated with postoperative pancreatic complications.

Aim Pancreaticobiliary maljunction (PBM) with or without congenital biliary dilatation (CBD) is a risk factor for biliary tract cancer. We investigated long‐term outcomes after biliary diversion operation with special reference to types of CBD. Methods Subjects comprised 40 adult patients who underwent biliary diversion operation for PBM without biliary tract cancer. Group A comprised 20 patients with type Ia or Ic CBD, or non‐dilated bile ducts, while group B comprised 20 patients with type IV‐A CBD. The clinical findings and postoperative outcomes were compared between groups. Results Of 40 patients, nine patients suffered from repeated cholangitis and eight of these nine patients suffered from hepatolithiasis after biliary diversion operation. Biliary tract cancer or pancreatic cancer was detected in four patients at 3 years and 2 months to 24 years after the operation. In three of these four patients, the serum concentration of carbohydrate antigen 19‐9 increased before detection of carcinoma. One patient died of hepatic failure due to repeated cholangitis. The proportions of patients with repeated cholangitis, hepatolithiasis, and re‐operation, and patients who died of biliary tract cancer, pancreatic cancer, or hepatic failure, were significantly higher in group B than in group A. The survival rate was significantly worse in group B than in group A. Conclusions Careful long‐term follow‐up with measurement of serum tumor markers is necessary after biliary diversion operation for PBM, especially in patients with type IV‐A CBD or repeated cholangitis., This study showed that careful long‐term follow‐up with measurement of serum tumor markers is necessary after biliary diversion operation for PBM, especially in patients with type IV‐A CBD who were found to have significantly less favorable outcomes.

We report here two cases of Kasai type IIIa biliary atresia (BA), associated with pancreaticobiliary maljunction (PBM). Both cases were referred to our hospital because of hyperbilirubinemia and diagnosed with BA preoperatively by abdominal ultrasonography and biliary scintigraphy. They underwent Kasai portojejunostomy at 49 and 37 days old, respectively. Intraoperative cholangiographies performed from their gallbladders clarified Kasai type IIIa BA with PBM. Laboratory test of colorless fluid from the gallbladder of one patient showed high level of lipase (460 U/L). Reported cases of BA associated with PBM are rare and this is a second report showed elevated level of a pancreatic enzyme in bile. The findings in our cases indicate the pathogenesis of biliary anomaly in association with PBM. Keywords: Kasai type IIIa biliary atresia, Pancreaticobiliary maljunction, Infantile obstructive cholangiopathy

Background The present study was aimed to investigate the expression profile of long non-coding RNAs (lncRNAs) and its potential as biomarker for pancreaticobiliary maljunction (PBM). Methods The differential expression of lncRNA and messenger RNA (mRNA) from 15 pediatric patients with PBM and 15 control subjects were analyzed with microArray and validated with quantitative reverse-transcription polymerase chain reaction (qRT-PCR). Gene Ontology enrichment and Kyoto encyclopedia of genes and genomes (KEGG) pathway analysis were used to investigate the biological functions of these genes. The area under curve (AUC) of receiver operating characteristic (ROC) curve was used to predict the biomarker of lncRNA in PBM. Results There were 2915 mRNAs and 173 lncRNAs upregulated, 2121 mRNAs and 316 lncRNAs downregulated in PBM cases, respectively. The enriched GO associated with differentially expressed mRNA were extracellular matrix, extracellular region and kinetochore. The most enriched pathway of Protein digestion and absorption was associated with cancer and PI3K-Akt signaling. Cis- and Trans-target gene analysis indicated that mRNAs were predicted to be regulated by one lncRNA and one mRNA corresponded to several lncRNAs. The results showed that the expression trends of NR_110876, NR_132344, XR_946886 and XR_002956345 were consistent with the microarray results, and the difference was statistically significant NR_132344, XR_946886 and XR_002956345 (P

Introduction and importance Pancreaticobiliary maljunction (PBM) is a rare congenital anomaly that is frequently associated with carcinoma of the biliary tract. However, there is still no clear evidence that PBM is associated with pancreatic tumors. Here we describe a case of gallbladder cancer and intraductal papillary mucinous neoplasm (IPMN) that is associated with PBM. Case presentation A 72-year-old man underwent a cholecystectomy with hepatectomy (S4a + S5) and regional lymph node dissection for gallbladder adenocarcinoma invading the front lobe branch of the hepatic artery. A pylorus-preserving pancreaticodudenectomy was also performed for pancreatic IPMN. Clinical discussion Presence of mucin type 6 (MUC6) -positive pyloric gland metaplasia in both the dilated pancreatic duct and the gallbladder background mucosa suggests that pancreatic IPMN and gallbladder cancer may have a common phenotypic origin. Additionally, analysis of 41 reported cases of pancreatic cancer associated with PBM revealed that in all metachronous multiple cancer cases, biliary tract cancer preceded the pancreatic cancer with congenital biliary dilatation accompanied by PBM. The analysis also revealed an increased proportion of pancreatic cancer cases with PBM in patients who had not undergone a flow diversion procedure located in pancreatic head. Conclusion We show an interesting relationship between pancreatic/gallbladder cancer and PBM. More comprehensive evaluations of the whole pancreaticobiliary system in follow-up of patients with PBM is required to understand the full extent of this relationship., Highlights • Pyloric gland metaplasia seen both in the pancreatic duct and in gallbladder suggests that IPMN and gallbladder cancer may have a common origin. • The analysis of pancreatic cancer with PBM cases revealed that in all metachronous cases, biliary tract cancer preceded the pancreatic cancer. • This observation suggests that PBM patients should be monitored for synchronous or metachronous cancer of the whole pancreaticobiliary system.

The most important role of ultrasound (US) in the management of gallbladder (GB) lesions is to detect lesions earlier and differentiate them from GB carcinoma (GBC). To avoid overlooking lesions, postural changes and high-frequency transducers with magnified images should be employed. GB lesions are divided into polypoid lesions (GPLs) and wall thickening (GWT). For GPLs, classification into pedunculated and sessile types should be done first. This classification is useful not only for the differential diagnosis but also for the depth diagnosis, as pedunculated carcinomas are confined to the mucosa. Both rapid GB wall blood flow (GWBF) and the irregularity of color signal patterns on Doppler imaging, and heterogeneous enhancement in the venous phase on contrast-enhanced ultrasound (CEUS) suggest GBC. Since GWT occurs in various conditions, subdividing into diffuse and focal forms is important. Unlike diffuse GWT, focal GWT is specific for GB and has a higher incidence of GBC. The discontinuity and irregularity of the innermost hyperechoic layer and irregular or disrupted GB wall layer structure suggest GBC. Rapid GWBF is also useful for the diagnosis of wall-thickened type GBC and pancreaticobiliary maljunction. Detailed B-mode evaluation using high-frequency transducers, combined with Doppler imaging and CEUS, enables a more accurate diagnosis.

Resumen La anomalía de la unión biliopancreática (AUBP) es una malformación congénita en la que los conductos pancreáticos y biliares se unen anatómicamente fuera de la pared duodenal. Debido a la excesiva longitud del canal común, la acción del esfínter no afecta la unión pancreatobiliar, lo que permite el reflujo del jugo pancreático hacia la vía biliar. Se asocia con quistes del colédoco y sus manifestaciones incluyen cáncer de la vía biliar, pancreatitis, coledocolitiasis y colangitis. Es una rara patología, especialmente en países occidentales. La colangiopancreatografía por resonancia magnética (CPRM) se ha convertido en el método más utilizado por no ser invasivo, teniendo en cuenta que la colangiopancreatografía retrógrada endoscópica (CPRE) se encuentra contraindicada en casos de pancreatitis aguda y colangitis, además del riesgo de pancreatitis posterior al procedimiento. La distancia entre la unión biliopancreática y la pared duodenal debe ser superior a 9 mm de longitud para diagnosticar AUBP mediante CPRM. En caso de que mida entre 6 y 9 mm y la acción del esfínter afecte la unión pancreatobiliar, se lo denomina unión biliopancreática alta (UBPA) y se debe confirmar mediante CPRE. Describimos los hallazgos en imágenes de siete pacientes jóvenes con clínica abdominal y diagnóstico de AUBP o UBPA por CPRM, y además revisamos la literatura sobre el tema. Abstract Pancreaticobiliary maljunction (PBM) is a congenital malformation in which the pancreatic and biliary ducts are anatomically joined outside the duodenal wall. Due to the excessive length of the common channel, the action of the sphincter does not affect the pancreaticobiliary junction, allowing the reflux of pancreatic juice into the bile duct. It is associated with cysts of the common bile duct and its manifestations include cancer of the bile duct, pancreatitis, choledocholithiasis and cholangitis. It is a rare pathology, especially in Western countries. Magnetic resonance cholangiopancreatography (MRCP) has become the most used method because it is non-invasive, taking into account that endoscopic retrograde cholangiopancreatography (ERCP) is contraindicated in cases of acute pancreatitis and cholangitis, in addition to the risk of pancreatitis after the procedure. The distance between the biliopancreatic junction and the duodenal wall must be greater than 9 mm in length to diagnose PBM by MRCP. If it is between 6 to 9 mm and the action of the sphincter affects the pancreaticobiliary junction, it is called high confluence of pancreaticobiliary ducts (HCPBD) and should be confirmed by ERCP. We describe the imaging findings of 7 young patients with abdominal symptoms and diagnosis of PBM or HCPBD by MRCP, and also review the literature on the subject.

Synchronous malignancies involving the gallbladder and the bile duct are exceedingly rare. Moreover, their association with anomalous pancreaticobiliary duct junction (APBDJ) has been reported mostly from the Far Eastern countries. Over time, many studies have suggested the definite risk of malignancy attributable to the 'carcinogenic anatomical configuration' of the long common biliopancreatic channel, allowing reflux of pancreatic juices in the biliary tract. In this report, we present a case of an elderly man from South India who was initially diagnosed with synchronous gall bladder with bile duct malignancy; the patient turned out to have an APBDJ on further evaluation.

Introduction Pancreaticobiliary maljunction (PBM) is a congenital anomaly wherein the persistent reflux of the pancreatic juice into the biliary tract induces biliary tract cancer. The prediction criteria for gallbladder carcinogenesis have been reported previously through results obtained from examining carcinogenic and non-carcinogenic cases with the parameters that classified each confluent form in PBM. This study aimed to validate these previous study results and provide new recommendations for gallbladder carcinogenesis prevention. Methods Twenty-four patients with PBM underwent hepaticojejunostomy. The prediction criteria for gallbladder carcinogenesis consist of three elements. The confluent forms that satisfied none or one of the three criteria were defined as a low score group, and those that satisfied two or three were defined as the high score group. Immunohistology and pathological search were performed on the gallbladders' sections in both groups to evaluate chronic inflammation. Results The cases with dysplasia, positive Ki67 labeling index, and gallbladder cancer were more common in the high score group and tended to have more lymphocyte infiltration. These findings indicate that the degree of inflammation and cell proliferation might be more severe in the high score group than in the low score group. Conclusions There is a close relationship between the confluent form and the histopathological findings of the gallbladder in patients with PBM. The confluent forms observed in the high score group might have an additional correlation with increased proliferation activity and subsequent malignant transformation of the gallbladder epithelium., Highlights • Pancreaticobiliary maljunction (PBM) may induce biliary tract cancer. • This is the first report showing a correlation between the form in PBM and pathological findings of the gallbladder. • We were able to show that PBM has a group with confluent forms with a strong carcinogenic tendency.

A 69-year-old man was referred to our hospital because of appetite loss. Imaging showed a nodular tumor in the perihilar bile duct and a second flat lesion in the distal bile duct. Right hepatopancreaticoduodenectomy was performed, and the histopathological findings demonstrated that the perihilar and distal lesions were moderately and poorly differentiated adenocarcinoma, respectively, and anatomically separated. Furthermore, the resected specimens showed no pancreaticobiliary maljunction. Histological and TP53 gene analyses in a rare case of synchronous double bile duct cancers suggest that there are various genetic pathways through which bile duct cancer develops, highlighting the complexity of its pathogenesis.

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. 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. 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. Preoperative one-stop MRI accurately delineates the bile duct anatomy as well as the hepatic artery, cholangitis, and protein plugs in children with PBM.

Pancreaticobiliary maljunction (PBM) is the congenital junction of the bile and pancreatic ducts into a common channel located anatomically outside of the duodenal wall. The complex type (Type D) is the rarest type and occurs in conjunction with other pancreaticobiliary anomalies. We present a case of complex PBM with pancreas divisum presenting as acute pancreatitis and obstructive symptoms secondary to a pancreatic pseudocyst. Surgical management is discussed, as complex type PBM is most predisposed to post-operative complications.

Background: Perforation of a choledochal cyst (CC) is not rare, but the pathogenesis of spontaneous perforation has not been established. Pancreaticobiliary maljunction (PBM) is commonly seen in association with choledochal cyst. To explore the relationship between PBM and perforated CC, a retrospective study was conducted.Methods: We analyzed all the patients with CC who underwent surgery in our hospital from 2014.06.01 to 2018.12.31. All patients were divided into two groups: group 1 were patients with perforated CC, and group 2 were patients with non-perforated CC. We recalled all the patients records to identify types of PBM. PBM was divided into four types [(A) stenotic type, (B) non-stenotic type, (C) dilated channel type, and (D) complex type] according to the classification proposed by the Committee on Diagnostic Criteria of the Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) in 2015.Results: There were 186 patients with CC in all, and 116 patients showed PBM. Twenty patients in group 1 and 96 patients in group 2. There was an extremely higher percentage of type C PBM in group 1 than in group 2 (60 and 17.7%, respectively). More fusiform dilatation cases were found in group 1 (70%) than in group 2 (58.3%). Also there were more type C PBM in fusiform cases and type A PBM were frequently seen in cystic cases (P < 0.01).Conclusions: We found that Type C PBM and fusiform common bile duct maybe relate to the perforation of choledochal cyst. Patients with type C PBM and fusiform common bile duct should be treated more proactively, preferably before they perforate.

Purpose This study aimed to elucidate the characteristics of biliary tract carcinoma (BTC) in young patients. Methods This is a nationwide multicenter, retrospective cohort study supervised by the Japanese Society of Hepato‐Biliary‐Pancreatic Surgery (JSHBPS). Clinicopathological data of patients aged, Highlight Ariake and colleagues elucidated that the most frequent factor associated with biliary tract carcinoma in young patients was pancreaticobiliary maljunction. Further analysis revealed that the distribution of cancer‐associated factors differed dramatically depending on the site of biliary tract carcinoma, suggesting that each site has a different mechanism of cancer development.

OBJECTIVE: To identify the best mode for diagnosing and treating the patients with choledochal cysts. METHODS: A retrospective study was performed with medical records of patients diagnosed with choledochal cysts from January 1994 to December 2017. In all cases, the diagnosis was based on ultrasound examination. All the patients underwent cyst resection and were divided in two groups: bile enteric anastomosis in the high portion of the common hepatic duct or in the dilated lower portion. RESULTS: Eighty-one cases were studied. The age of presentation was 4 y 2 mo ± 4 y 1 mo, and the age for the surgical treatment was 5 y 5 mo ± 4 y 6 mo. In 61 cases, US was the only image examination performed. There were 67 cases of Todani type I (82.7%), 13 cases of type IV (16.0%) and one case of type III (1.2%). Nine patients (29.0%) in the first period and 2 patients (4.0%) in the second period presented with postoperative complications (p=0.016). CONCLUSION: In patients with choledochal cysts, US is the only necessary diagnostic imaging. Performing the bile enteric anastomosis in the lower portion of the common hepatic duct is safer and has a lower risk of complications.

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.

Pancreaticobiliary maljunction (PBM) is a congenital malformation in which the pancreatic and bile ducts join anatomically outside the duodenal wall. In PBM, the common channel is so long that sphincter action does not directly affect the pancreaticobiliary junction. Since the hydropressure in the pancreatic duct is usually greater than that in the bile duct, pancreatic juice frequently refluxes into the biliary tract (pancreatobiliary reflux) in PBM. Persistence of refluxed pancreatic juice injures the epithelium of the biliary tract and leads to hyperplastic change with increased cell proliferative activity in the gallbladder. Then, mutation of oncogenes and/or tumor suppressor genes occurs in the epithelia, leading to biliary tract carcinogenesis. In a nationwide survey in Japan, biliary cancer was detected in 21.6% of adult patients with congenital biliary dilatation and in 42.4% of PBM patients without biliary dilatation. Hyperplastic change of the gallbladder epithelium is radiologically detected as gallbladder wall thickening. To detect PBM without biliary dilatation early, magnetic resonance cholangiopancreatography (MRCP) is warranted in patients showing thickening of the gallbladder wall on screening ultrasound, which is suspicious of PBM.

Background: Incidence and long-term outcomes of choledochal malformations (CMs) in children remain unclear. Methods: Clinical characteristics, operative details, complications, and follow-up data were collected from eight pediatric surgical centers in Sweden, Norway, Denmark, and Finland, which also answered a questionnaire addressing management practices. Results: During 2000-2017, 126 pediatric CMs were diagnosed, corresponding an incidence of 1:37,400. Diagnostic, treatment, and follow-up practices varied markedly. Of patients with complete clinical data (n = 119), 85% and 11% had type I and IV CMs and were managed by open hepaticojejunostomy at median age of 2.5 (interquartile range 0.46- 5.8) years. Associated malformations were more common in fusiform and type IV (23%) than cystic CMs (8%, p = 0.043). Pancreaticobiliary maljunction was more frequently confirmed in patients presenting with pancreatitis (26% vs. 7%, p = 0.005) and with fusiform CMs (56% vs. 25%, p = 0.001). Cholangitis/pancreatitis episodes, occurring in 12% during postoperative follow-up of 4.0 (2.0-7.9) years, associated with longer surveillance (OR 1.32, 95% CI 1.13-1.54, p