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Background: Former studies showed that magnetic resonance cholangiopancreatography (MRCP) is useful in diagnosing the presence of santorinicele; however, few studies have evaluated MRCP in diagnosing wirsungocele and the association between pancreatitis and santorinicele or wirsungocele. The purpose of the study was to explore the performance of MRCP in diagnosing santorinicele and wirsungocele and investigate the potential association among pancreatitis, pancreas divisum, and santorinicele or wirsungocele. Method: Sixty-five patients (mean age, 55.68 years; range, 11-82 years) with santorinicele or wirsungocele were included and sorted into two groups: the santorinicele group (n = 48) and the wirsungocele group (n = 17). All patients underwent MRCP. The images were evaluated for the appearance and size of santorinicele or wirsungocele. The diagnostic sensitivity of MRCP was assessed. Additionally, whether two groups are correlated with pancreas divisum or pancreatitis were investigated. Result: The sensitivity of MRCP in detecting santorinicele and wirsungocele showed no difference (68.8% and 76.5%, respectively). The proportion of patients who developed pancreatitis in santorinicele and wirsungocele groups were 60.4% and 11.8%, respectively (p < 0.05). Pancreas divisum accounted for 77.1% and 11.8% of the patients in the santorinicele and wirsungocele groups, respectively (p < 0.05). Patients with santorinicele and pancreas divisum tended to be older when they acquired pancreatitis. Conclusion: MRCP could be an alternative imaging method to detect cystic dilation of the pancreatic duct. Pancreatitis is more common in patients with santorinicele than in those with wirsungocele. Moreover, santorinicele is more closely associated with pancreatitis than with pancreas divisum.

Introdução: Pancreas Divisum é uma anomalia anatômica do ducto pancreático em que a maioria do suco pancreático passa pela papila menor e a minoria pela papila maior. Relato de caso: Paciente com 54 anos com Pâncreas Divisumque obteve diagnóstico de pâncreas divisum ocasionalmente durante investigação para elucidação de enzimas hepáticas alteradas e dispepsia. Discussão: Pâncreas Divisum é uma entidade pouco abordada devido à falta de sintomatologia na maioria dos casos, mas há indícios mais recentes que apontam que essa entidade poderia estar relacionada com a maior incidência de outras patologias como pancreatites e tumor pancreático. Conclusão: Por ser uma doença rara e assintomática e, pela possibilidade de evoluir com complicações, deve-se pensar preemptivamente no intuito de prever diagnósticos mais precoces para que se possa intervir em casos de necessidade e evitar desfechos desfavoráveis.

Background The complication of duplication of alimentary tracts and pancreas divisum (PD) is a rare malformation and the development of pancreatic ductal adenocarcinoma (PDAC) in this malformation is also extremely rare. There have been some reports of complication of malignancy in a gastric duplication cyst (GDC) and PD. However, there have been no reports of complication of PDAC in cases with GDC and PD. Case presentation A 54-year-old woman was followed up at the previous hospital due to a history of ovarian endometrial adenocarcinoma. She also had a surgical history of partial excision for a GDC and pancreatic tail of PD in her childhood. A gynecological follow-up computed tomography (CT) examination revealed the pancreatic body tumor and the bifurcated main pancreatic duct dilatation. Furthermore, magnetic resonance cholangiopancreatography also revealed that the ventral main pancreatic duct communicated with the GDC. The initial levels of tumor markers were high, but we could not achieve preoperative histopathological diagnosis. The preoperative diagnosis was PDAC occurring in a case with PD and GDC. She received two courses of neoadjuvant chemotherapy with gemcitabine and nab-paclitaxel. A CT examination after neoadjuvant chemotherapy revealed the shrinkage of the tumor, and then we performed distal pancreatectomy with splenectomy and GDC resection. A histopathological examination revealed invasive PDAC and lymph node metastases; pathological staging was T1N1M0, stage III. Furthermore, PD and GDC were also histopathologically detected. The postoperative course was uneventful, and she was discharged on the postoperative day 25. She received S-1 monotherapy for 6 months, and no recurrence has been detected at 1 year after radical resection. Conclusions We herein presented an extremely rare combined case of PD, GDC and PDAC. We successfully treated it by neoadjuvant chemotherapy and distal pancreatectomy with GDC resection, and postoperative chemotherapy.

Background and study aims Although the majority of patients with pancreas divisum (PDiv) are asymptomatic, a subgroup present with recurrent pancreatitis or pain for which endoscopic therapy may be indicated. The aim of this study was to evaluate success rates and long-term outcomes of endoscopic treatment in patients with symptomatic PDiv. Patients and methods A multicenter, retrospective cohort study was performed. Patients with symptomatic PDiv presenting with recurrent acute pancreatitis (RAP), chronic pancreatitis (CP), or chronic abdominal pancreatic-type pain (CAP) who underwent endoscopic retrograde cholangiopancreatography (ERCP) between January 2000 and December 2019 were included. The primary outcome was clinical success, defined as either no recurrent episode of acute pancreatitis (AP) for RAP patients, no flares for CP patients, or absence of abdominal pain for patients with CAP after technically successful ERCP. Results In 60 of 81 patients (74.1 %) a technically successful papilla minor intervention was performed. Adverse events were reported in 30 patients (37 %), with post-ERCP pancreatitis in 18 patients. The clinical success rate for patients with at least 3 months of follow-up was 42.6 %, with higher rates of success among patients presenting with RAP (44.4 %) as compared to those with CP (33.3 %) or CAP (33.3 %). Long-term sustained response was present in 40.9 % of patients with a technically successful intervention. In patients with RAP who did not completely respond to treatment, the mean number of AP episodes after treatment decreased significantly from 3.5 to 1.1 per year, and subsequently the interval between AP episodes increased from 278 to 690 days (P = 0.0006). A potential predictive factor of failure of clinical success after technically successful ERCP, at univariate analysis, was male sex (OR = 0.25, P = 0.02). Conclusions Endoscopic therapy in patients with symptomatic PDiv is moderately effective, with its highest yield in patients presenting with RAP. Future studies are needed to assess factors predictive for success of endoscopic therapy and potential risk factors for relapse after ERCP.

Diagnostik und klinische Relevanz eines Pankreas divisum. Ultraschall (US), Magnetresonanz-Cholangiopankreatikographie (MRCP), Magnetresonanztomographie (MRT), Computertomographie (CT), endoskopische retrograde Cholangiopankreatikographie (ERCP) Das Pankreas divisum ist eine Anlagevariante, die bei ca. 10 % der Bevolkerung auftritt, davon werden ca. 5 % symptomatisch. In der Diagnostik ist die MRT mit MRCP inzwischen die Methode der Wahl. Die MRT mit MRCP ist in der Diagnostik des Pankreas divisum mit der ERCP gleichwertig, hat dabei aber den Vorteil, dass diese Modalitat nicht invasiv ist, gleichzeitig eine Umfelddiagnostik ermoglicht und es nahezu keine Kontraindikationen gibt. Es ist wichtig, die Anatomie des pankreatischen Gangsystems zu kennen und zu beachten, damit bei symptomatischen Patienten mogliche Interventionen rechtzeitig geplant werden konnen.

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

Around 10% to 30% patients with acute pancreatitis (AP) do not have a cause after the routine investigations, and are considered as having idiopathic acute pancreatitis (IAP). Establishing the etiology in such patients will prevent recurrences and evolution to chronic pancreatitis. Endoscopic ultrasonography (EUS) and magnetic resonance cholangiopancreatography (MRCP) characteristically are used to diagnose IAP when routine methods fail, but their exact role is not determined. This prospective study was undertaken in a tertiary care hospital, in which patients admitted initially with diagnosis of IAP were evaluated. These patients underwent MRCP and EUS at least 4 weeks after an attack of AP. The results of EUS and MRCP were compared and analyzed with various clinical variables using suitable statistical tests. A total of 31 patients with IAP were included. EUS and/or MRCP was able to establish at least one etiology in 17 patients (54.8%). The diagnoses revealed were gallbladder (GB) microlithiasis, GB sludge, choledocholithiasis, pancreatobiliary ductal anomalies, pancreatic adenocarcinoma, and intraductal papillary mucinous neoplasm. Comparing the diagnostic accuracy of both the modalities, EUS (14/31) was able to diagnose more cases than MRCP (8/31). The diagnostic capability of EUS was lower in patients who had a cholecystectomy (12.5% vs. 56.5%; p = 0.03). EUS and MRCP are useful modalities in the etiological diagnosis of IAP and should be used in conjunction. EUS is better for establishing a possible biliary etiology and MRCP for an anatomical alteration in pancreatobiliary ducts.

BACKGROUND AND AIMS: Many patients undergoing total pancreatectomy with islet autotransplant (TPIAT) for severe, refractory chronic pancreatitis or recurrent acute pancreatitis have a history of endoscopic retrograde cholangiopancreatography (ERCP). Using data from the multicenter POST (Prospective Observational Study of TPIAT) cohort, we aimed to determine clinical characteristics associated with ERCP and the effect of ERCP on islet yield. METHODS: Using data from 230 participants (11 centers), demographics, pancreatitis history, and imaging features were tested for association with ERCP procedures. Logistic and linear regression were used to assess association of islet yield measures with having any pre-operative ERCPs and with the number of ERCPs, adjusting for confounders. RESULTS: 175 (76%) underwent ERCPs [median number of ERCPs (IQR) 2 (1–4)]. ERCP was more common in those with obstructed pancreatic duct (p=0.0009), pancreas divisum (p=0.0009), prior pancreatic surgery (p=0.005), and longer disease duration (p=0.004). A greater number of ERCPs was associated with disease duration (p

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 and study aims The etiology of idiopathic acute pancreatitis (IAP) should always be defined. Our aim was to compare the diagnostic value of endoscopic ultrasound (EUS) versus secretin-enhanced magnetic resonance cholangiopancreatography (S-MRCP) in patients with IAP. Patients and Methods Patients admitted to a single tertiary care University hospital with IAP were invited to participate in the study. Enrolled patients underwent EUS and S-MRCP in a single-blinded comparative study. EUS and S-MRCP were performed no sooner than 4 weeks after discharge. The diagnostic yield of EUS and S-MRCP and demographic variables were included in the analysis. Additional follow-up, results of subsequent serology, radiographic exams, and relevant histological analysis were considered in determination of the final diagnosis. Results A total of 34 patients were enrolled; EUS was normal in six, cholelithiasis was defined in 15, choledocholithiasis in two, pancreas divisum in three, branch-type intraductal papillary mucinous tumor (IPMT) in three, and chronic pancreatitis in five. S-MRCP identified choledocholithiasis in one, divisum in four, branch-type IPMT in three, chronic pancreatitis in two; 24 subjects diagnosed as normal by S-MRCP. Diagnostic correlation between EUS and S-MRCP was slight (kappa = 0.236, 95 % confidence interval: 0.055–0.416). EUS provided a statistically significantly higher diagnostic yield than S-MRCP: 79.4 % (CI95 %: 65 %–94 %) vs 29.4 % (CI95 %: 13 %–46 %) (P = 0.0002). The sensitivity, specificity, and positive and negative predictive values of EUS and S-MRCP were 90 %, 80 %, 96 %, 57 % and 33 %, 100 %, 100 % and 16 %, respectively. Conclusion The diagnostic yield of EUS is higher than S-MRCP in patients with IAP.

Recurrent acute pancreatitis is characterized by frequent episodes of inflammation in the pancreas. The most common causes are alcohol abuse and gallstones but approximately 30% of cases are defined as idiopathic because initial evaluation fails to detect the etiology. In these patients, extensive laboratory and imaging evaluations usually lead to the uncovering of an occult biliary duct stone or sphincter of Oddi dysfunction as the main reason for frequent pancreatitis. We report a 42-year-old female with a longstanding history of acute recurrent pancreatitis in the setting of pancreas divisum due to its rarity. We focus on appropriate investigations by application of specialized laboratory and radiological modalities because determining the etiology is a crucial step in patient management.

Pancreas divisum (PD) as a cause of pancreatitis has been debated. In this study, we report the association of multiple gene polymorphisms on the risk of RAP in the presence of PD. We enrolled 687 individuals (167 IRAP, 276 ICP, and 244 unrelated healthy controls) from May 2015 to September 2016. Patients were divided into those with/without PD. Associations between the significantly prevalent SNPs and IRAP/ICP in the presence of PD were evaluated. Clinical data were analyzed using Mann–Whitney U/Chi-square test. Effect size of association of SNPs with IRAP/ICP was expressed as odds ratio (OR) (95% CI). Gene–gene interaction was assessed by transheterozygosity analyses. Bonferroni-corrected two-tailed “p” value of ≤ 0.01 was considered statistically significant. Thirty-three (19.8%) and 82 (29.7%) patients with IRAP and ICP, respectively, had PD. Among the patients with IRAP, duration of disease was significantly shorter in those with PD compared to those without (mean [95% CI] duration: 1.6 (1.3–1.9) vs 2.7 (2.3–3.1) years; p = 0.005). There were no differences in gender, race, and diabetes among patients with/without PD in IRAP/ICP groups. Mean (95% CI) pancreatic duct diameter (mm) was significantly higher in the presence of PD in patients with both IRAP [1.6 (1.4–1.9) v/s 1.29 (1.2–1.4); p = 0.03)] and ICP [5.2 (4.5–5.9) v/s 4.5 (3.9–5.1); p = 0.02]. CTSB (rs12338) polymorphisms were significantly associated with IRAP [OR (95% CI) 2.44 (1.41–4.22); p = 0.001] among patients with PD. No association was observed with ICP. Transheterozygosity analysis did not show any significant associations of combination of SNPs with IRAP in the presence of PD. Risk of RAP due to PD increases in patients with rs12338 polymorphism in the cathepsin B gene.

Background: Except for pancreas divisum (PD), the prevalence of anatomic variants of the main pancreatic duct (MPD) seems to be insufficiently investigated. To date, their role in the occurrence of pancreatic exocrine insufficiency (PEI) and morphological changes suggestive of chronic pancreatitis (CP) has remained unclear.Methods: A systematic review was performed, searching MEDLINE and Web of Science, limited to articles published between 1960 and 1 June 2019.Results: Our review included a total number of 3234 subjects. The most common variant of MPD was type 3, followed by type 1, indicating MPD drainage pattern into major papilla (MP) as the most frequent. A sub-variant of type 3, known as 'reverse pancreas divisum' had a prevalence of 2.2%. Type 4 variant- PD, was found in 6.4% of all cases. The most common sub-variant of PD was complete PD, followed by incomplete PD and variant with MPD as only pancreatic duct. Type 5 variant (including ansa pancreatica) was present in 2.9% of subjects. Apart from one study with a significantly higher frequency of morphological changes suggestive of CP in patients with ansa pancreatica, the studies stated no significant association between pancreatic disease and MPD variants. Furthermore, only one study examined the influence of MPD variants on exocrine pancreatic function. Although equivocal, this association is most likely found to be insignificant.Conclusion: To elucidate linkage between MPD variants and the occurrence of chronic pancreatitis and impairment of pancreatic exocrine function, further clinical investigations are warranted.

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.

Islet isolation is an essential process in every human islet transplantation protocol. Intraductal enzyme delivery followed by adequate distention of the pancreas is the most critical step in islet isolation. Anomalies of the pancreatic duct system can significantly affect this process. Thus, identification and characterization of ductal patency is of paramount importance to achieve optimal islet isolation. To investigate the frequency of duct obliteration in the human pancreas and explore donor/patient characteristics associated with specific ductal variations. We examined ductal patency of pancreata allocated for islet allotransplantation (n = 597) and autotransplantation (n = 21) after removal of the duodenum during islet isolation procedure. Donor/patient factors were reviewed from the batch files. Among 559 deceased donor pancreata without pancreas divisum (n = 38, 6.4%), both ducts were patent in 50.1%, only ventral duct was patent in 46.7%, and only dorsal duct was patent in 3.2%. Donor age was not associated with the frequency of obliterated dorsal duct. Black race tended to have the higher frequency of patent dorsal duct. As expected, pancreas divisum was more frequent in chronic pancreatitis cases (n = 6, 28.6%). Within 7 cases of chronic pancreatitis with unknown etiology, we encountered one case of ventral duct obliteration. The minor duodenal papilla and aging do not likely play an important role in the occurrence of dorsal duct obliteration. Although frequency of obliterated ventral duct was low in our population, physicians, including gastroenterologists and endoscopists, as well as islet transplantation researchers should be aware of this possibility.

Background/Aims There are conflicting opinions regarding the management of recurrent acute pancreatitis (RAP). While some physicians recommend endoscopic retrograde cholangiopancreatography (ERCP) in this setting, others consider it to be contraindicated in patients with RAP. The aim of this study was to assess the practice patterns and clinical features influencing the management of RAP in the US. Methods An anonymous 35-question survey instrument was developed and refined through multiple iterations, and its use was approved by our Institutional Review Board. The survey was distributed via email to 408 gastroenterologists to assess the practice patterns in the management of RAP in multiple clinical scenarios. Results The survey was completed by 65 participants representing 36 of the top academic/tertiary care centers across the country. Approximately 90.8% of the participants indicated that they might offer or recommend ERCP in the management of RAP. Multinomial logistic regression analysis revealed that ductal dilatation and presence of symptoms were the most predictive variables (p

Background: Endoscopic retrograde cholangiopancreatography (ERCP) has been increasingly performed in children with symptomatic pancreas divisum (PD).Aim: To investigate the safety and efficacy of ERCP in the treatment of children with symptomatic PD.Methods: We performed a retrospective analysis on children with PD who were treated with ERCP at Shanghai Children's Medical Center between June 2015 and May 2020. Pertinent patient, clinical and procedural data were collected to assess the therapeutic effects and identify the risk factors for post-ERCP pancreatitis (PEP).Results: Overall, 114 ERCPs were performed in 46 children with PD. With a median follow-up of 28.5 months (12–71 months), 40 (87.0%) children achieved clinical remission, the median number of acute pancreatitis episodes decreased from four times per year pre-operatively to once per year post-operatively (P < 0.001), and the nutritional score improved post-operatively (P = 0.004). The incidence of PEP was 7.9%, and female sex, stone extraction, and gene mutations were identified as possible risk factors for PEP on univariate analysis. However, there was no statistical significance on multivariate analysis (P > 0.05).Conclusion: Therapeutic ERCP is an effective and safe intervention for children with symptomatic PD.

Pancreas divisum (PD) is a common pancreatic malformation caused by the failure of fusion between ventral and dorsal pancreatic ducts. There is a small branch of communication between the two systems in incomplete PD, and this variation has an incidence of 15%. A 43-year-old female patient presented to our department with recurrent abdominal pain. Magnetic resonance cholangiopancreatography (MRCP) showed that the ventral pancreatic duct was curved, with a local pouchlike dilatation. Endoscopic ultrasonography supported the diagnosis of incomplete PD and showed a thin branch of communication between ventral and dorsal pancreatic ducts. Endoscopic retrograde cholangiopancreatography (ERCP) and papillotomy of the minor papilla with double plastic stent implantation were performed. One pancreatic plastic stent was inserted across the minor and major papilla over the guide wire, creating a U-shape. The other wire-guided plastic stent was inserted through the minor papilla into the dorsal pancreatic duct. The pancreatic fluid drained smoothly after stent placement. During the 6-month follow-up, the patient remained well, without recurrence of pancreatitis.

Endoscopic retrograde cholangiopancreatography (ERCP) is generally performed via the major papilla when evaluating patients with pancreatic disease. However, in patients with pancreas divisum (PD) or distortion of the main pancreatic duct, endoscopic retrograde pancreatography (ERP) should be performed via the minor papilla (MP). Our aim was to evaluate the efficacy and safety of endoscopic pancreatic juice cytology (PJC), performed via the MP, in patients with PD.Patients with PD who underwent diagnostic ERP via the MP, between January 2010 and February 2021, were identified retrospectively from our hospital's ERCP database. Twenty-two patients contributing to 24 ERCPs were included in the analysis.MP cannulation was successful in 23 of 24 ERCPs (96%). In one patient, successful cannulation was achieved on the second attempt and the procedure was performed twice in another. Serial pancreatic juice aspiration cytologic examination (SPACE) was performed in 17 patients, with a single aspiration of pancreatic juice performed in the other five. The sensitivity, specificity, and accuracy rates of ERCP diagnosis, overall, were 56%, 100%, and 80%, respectively. When diagnosis only based on SPACE was considered, the accuracy rate was even higher at 87%. Three patients (13%) developed mild pancreatitis as an adverse event.The diagnostic ability of endoscopic PJC, via the MP in patients with PD was technically feasible and relatively effective under experienced pancreatobiliary endoscopists, however, requiring careful attention to post-ERCP pancreatitis when performed.

Pancreas divisum is a congenital anatomical anomaly characterized by a lack of fusion of the ventral and dorsal parts of the pancreas, which sometimes causes pancreatitis. Most cases of pancreas divisum are difficult to treat, and no established guidelines are currently available. Here we describe the case of patient with acute recurrent pancreatitis caused by pancreas divisum who was successfully treated with cruciate sphincterotomy of the minor papilla. A 70-year-old female was admitted to our hospital due to an emergency of acute abdominal pain, which was subsequently diagnosed as acute pancreatitis caused by pancreas divisum. The patient, recalcitrant to conservative endoscopic management of minor papilla, underwent the cruciate incision of the minor papilla, which allowed drainage of the pancreatic duct. Following this intervention, the patients clinical condition and serum amylase level promptly improved. No recurrence was observed over a follow-up period of 48 months. Cruciate incision of the minor papilla is an effective and safe technique for minimally invasive treatment of pancreas divisum. [Med-Science 2020; 9(1.000): 278-80]

Introduction Morphological variants of the pancreatobiliary system can predispose to chronic pancreatitis. The goal of the present study is to assess the prevalence of pancreatic duct patterns in the Indian population, both by cadaveric dissection and by magnetic resonance cholangiopancreatography (MRCP). Materials and Methods A total of 15 adult pancreas specimens of unknown age and gender, and 5 fetal pancreas specimens of different gestational ages with the intact second part of duodenum, were dissected by the piecemeal method. For clinical relevance, MRCP images of 103 clinically-diagnosed chronic pancreatitis patients irrespective of their etiology were obtained retrospectively from the existing database and studied. The anatomical patterns were classified as five different types based on the course of the main pancreatic duct and the accessory pancreatic duct and their openings into the duodenal wall, including variants like pancreas divisum and ansa pancreatica. Results In the cadaveric study, the main pancreatic duct was single with a straight course in 46.67% of the adult specimens, and in the MRCP study, the main pancreatic duct showed a descending course in 77.66% of the cases. The most common pattern was type III in both the cadaveric (80%) and radiological (55.33%) studies, and the accessory duct was absent on the MRCP in all type-III cases, while it ended blindly in the cadaveric specimens. Ansa pancreatica (type V) was observed in 1 adult specimen (6.7%), but not in the radiological study. Pancreas divisum (type IV) was observed in the 8 cases (7.76%) cases in the radiological study. Conclusion Knowledge of the anatomical variants of the pancreatic ductal system may be helpful for the radiologists during diagnostic and therapeutic interventional procedures.

Pancreas divisum, the most common congenital malformation of the pancreas, occurs due to a failure of fusion of the ductal systems of the dorsal and ventral pancreatic buds in the seventh week of intra-uterine life. This leads to a dominant dorsal pancreatic duct draining though the minor papilla and a small ventral pancreatic duct draining through the major papilla. The prevalence in western populations is about 10% and more than 95% of these patients are without pancreatic symptoms, with the anomaly found incidentally on abdominal imaging for an unrelated indication. The etiological role and clinical significance of pancreas divisum in relation to pancreatic disease has not yet been clearly defined, but may predispose to pancreatic disease in co-existence with other factors. Secretin-enhanced Magnetic Resonance Cholangiopancreatography is the non-invasive imaging modality of choice to identify pancreas divisum. Patients may be offered minor papilla therapy when they present with recurrent acute pancreatitis, severe acute pancreatitis and can be considered for therapy in the setting of chronic pancreatitis and chronic abdominal pain of pancreatic origin. Minor papilla endotherapy (sphincterotomy and/or stenting) via Endoscopic Retrograde Cholangiopancreatography and minor papilla surgical therapy have comparable outcomes with endotherapy typically considered first-line due to a favorable adverse event profile. The response to therapy is variable with maximal benefit seen in patients with recurrent acute pancreatitis and least with chronic pancreatic-type abdominal pain. Data supporting either therapy are of low quality as they are predominantly retrospective with a sub-optimal follow up period. Surgical options including a pancreatojejunostomy (Puestow or Frey procedure) or a total pancreatectomy with auto-islet cell transplantation may be considered in a subset of patients.

The aim of this study was to evaluate the long-term outcomes of therapeutic endoscopic retrograde cholangiopancreatography (ERCP) for pediatric patients with pancreas divisum (PD) presenting with acute recurrent pancreatitis (ARP) or chronic pancreatitis (CP).Between May 2008 and August 2017, pediatric patients with PD who received endotherapy at Ruijin Hospital were identified and grouped according to clinical presentation, namely ARP and CP. Primary success was defined as patients' improvement in symptoms after index ERCPs, without further intervention or any analgesic.A total of 74 ERCPs were performed in 38 pediatric patients. The frequency of at least 1 genetic mutation identified in patients with ARP and CP was 44.4% and 68.4%, respectively. Patients with CP required more ERCPs than those with ARP (2.4 ± 1.7 vs. 1.1 ± 0.4, P = 0.005). The incidence of post-ERCP complications was 14.9%, including pancreatitis of 13.5% and hemorrhage of 1.4%. During a median follow-up duration of 41 months (range, 12-123 months), the frequency of pancreatitis episodes decreased significantly from 2.31 to 0.45 (P 0.0001). The 25% recurrence and reintervention rates were estimated at 25 and 48 months, respectively, without significant difference between patients with ARP or CP. There was a nonsignificant trend towards a higher rate of primary success in patients with ARP than those with CP (92.9% vs. 69.6%, P = 0.123). After further endotherapy, 91.3% patients with CP improved clinically.Therapeutic ERCP is an effective and safe intervention for pediatric patients with symptomatic PD. Patients presenting with CP seem to achieve improvement after additional ERCPs.

Context Pancreas divisum (PD), the most common congenital anatomic variation of the pancreatic ductal system, is still rare in clinical practice. Endoscopic retrograde cholangiopancreatography (ERCP) is the first-line treatment for patients with symptomatic PD. However, even by experienced endoscopists, the procedure of ERCP can fail in 3% to 10% of cases. Traditionally, if we fail in cannulation we have no choice but to rely on surgery. Case report The 2 case reports of PD in this study with cannulation were both unsuccessful, fortunately, the use of endoscopic ultrasonography-guided pancreatic duct puncture combined with ERCP was eventually successful in treatment. Conclusions Using endoscopic ultrasonography-guided pancreatic duct puncture combined with ERCP is safe and effective for the treatment of the patients of difficult minor papilla cannulation in patients of PD, and can be used as a remedy for minor papilla cannulation failure.

Background Pancreas divisum is a congenital embryological disease caused by a lack of fusion between the ventral and dorsal pancreatic ducts in the early stages of embryogenesis. Recurrent acute pancreatitis, chronic pancreatitis or chronic abdominal pain are the main clinical syndromes at presentation and occur in only 5% of the patients with pancreas divisum. This review aimed to discuss diagnosis and treatment strategies in patients with symptomatic pancreas divisum. Data sources We report a literature review from 1990 up to January 2018 to explore the various diagnostic modalities and surgical techniques and results reported in the surgical treatment of pancreas divisum. Results There are limited reports available on this topic in the literature. We analyzed and described the main indications in the treatment of pancreas divisum, focusing on surgical treatment and a discussion of the different approaches. Furthermore, we report the results from our experience in two cases of pancreas divisum treated by pancreatic head resection with segmental duodenectomy (the Nakao procedure). Conclusions Pancreas divisum is a common pancreatic malformation in which only a few patients develop a symptomatic disease. Surgical treatment is needed in case of endoscopic drainage failure and in cases complicated with chronic pancreatitis and local complications. Many techniques, of greater or lesser complexity, have been proposed.

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.

Total pancreatectomy with islet autotransplantation (TPIAT) is used to treat debilitating chronic pancreatitis (CP) and acute recurrent pancreatitis (ARP) that has failed medical and endoscopic therapy. We performed a retrospective review of TPIAT patients at a free-standing children’s hospital to evaluate perioperative outcomes. Twenty patients (median age 13, 65% female) underwent TPIAT (2015 through 2017). Of the 20 patients, 95% had CP and 1 patient (5%) had ARP alone. Seventy-five percent of the patients had a pancreatitis-associated genetic mutation; 40% had pancreas divisum. The median surgical time was 757 (IQR 657 to 835) minutes. Median islet equivalents per kg of body weight (IEQ/kg) were 6404 (IQR 5018 to 7554). At 90 days postoperatively vs preoperatively, significantly fewer patients were receiving parenteral nutrition (0% vs 25%, P = .006) and opioids (45% vs 75%, P = .01). Short Form 36-Item Health Survey (SF-36) physical health module scores and total scores improved (34.0 preoperatively vs 54.6 at 90 days, P = .008, and 47.1 vs 65.3, P = .007, respectively); SF-10 physical health scores also improved (13.4 vs 33.1, P = .02). Insulin requirement decreased from 0.5 unit/kg/day to 0.4 unit/kg/day between discharge and 90 days (P = .02). TPIAT is an effective option when debilitating disease persists despite maximal medical and endoscopic therapy. Opioid, parenteral nutrition, and exogenous insulin use can successfully be weaned within 90 days after TPIAT, with gains in health-related quality of life.

Appropriately selecting patients with chronic pancreatitis associated with pancreas divisum (PD) for endoscopic retrograde cholangiopancreatography (ERCP)–based therapy versus surgery remains difficult. The objective of this study was to identify factors that predict success or failure of ERCP for treatment of chronic pancreatitis in PD. Patients undergoing ERCP for a diagnosis of PD and pancreatitis between 2008 and 2016 were identified and grouped according to whether they required one or two ERCPs or three or more ERCPs. Groups were compared along demographic, diagnostic, laboratory, ERCP-related, and outcome variables. Patients requiring 1–2 ERCPs were less likely to have back pain on initial presentation (4 vs. 24%, p = 0.02) and less likely to have a dilated bile duct on imaging prior to their first ERCP (8 vs. 30%, p = 0.04) than those requiring 3+ ERCPs. Patients requiring 1–2 ERCPs were also less likely to eventually require operative intervention for treatment of their chronic pancreatitis than those requiring 3+ ERCPs (24 vs. 44%, p = 0.047). On multivariable analysis, a dilated bile duct (odds ratio (OR) = 6.0, 95% confidence interval (CI) = 1.01–36.0, p = 0.048) was independently associated with requiring 3+ ERCPs. Back pain (OR = 6.3, 95% CI = 0.73–54.2, p = 0.09) trended toward but did not reach statistical significance for being independently associated with requiring 3+ ERCPs. The success of endoscopic treatment of chronic pancreatitis in patients with PD is dependent on proper patient selection. Patients with a dilated bile duct and back pain upon presentation may not respond well to endoscopic treatment alone and are more likely to eventually require operative intervention. Consideration should be given to early operative intervention in these patients.

The advancement of pancreatic endotherapy has increased the availability of minimally invasive endoscopic pancreatic ductal drainage techniques. In this regard, familiarity with endoscopic ultrasound-guided pancreatic duct drainage (EUS-PDD) is critical for treatment of obstructed pancreatic ductal systems, especially in nonsurgical candidates and in patients desiring a minimally invasive approach. Two distinct forms of EUS-PDD exist, viz. rendezvous-assisted endoscopic retrograde pancreatography (rendezvous-assisted ERP) and anterograde EUS-PDD. Anterograde EUS-PDD refers to transmural anterograde passage of a pancreatic drainage catheter or stent directly into the main pancreatic duct, through either the gastric or enteral wall. Rendezvous-assisted ERP should be attempted after failed conventional ERP, and anterograde EUS-PDD should be considered if rendezvous-assisted ERP fails or is not technically feasible. Common clinical scenarios that fulfil these conditions are chronic pancreatitis with high-grade main pancreatic duct obstruction, surgically altered anatomy with ductal/anastomotic obstruction, pancreas divisum, and disconnected pancreatic duct syndrome. The focus of this review article is anterograde EUS-PDD and its indications, technique, and outcomes. It also provides a summary of our own experience with this procedure, and a video demonstration of the technique.

Chronic pancreatitis usually requires medical treatment and, in rare cases, surgical intervention. The most frequent cause of consultation is chronic pain that is difficult to manage, and therapeutic options such as endoscopy can resolve most problems without surgery. However, when these options fail, surgical management is necessary. The most common surgery is a lateral pancreatic bypass or Puestow surgery. We present the case of a 31-year-old patient with unspecific abdominal discomfort. Abdominal ultrasonography showed significant dilation of the pancreatic duct (18 mm). Abdominal magnetic resonance imaging showed an impacted stone in the head of the pancreas and pancreas divisum. Resolution with endoscopy was attempted, but the pancreatic duct could not be accessed due to impacted lithiasis. After discussion in a multidisciplinary committee, surgical treatment was recommended, and a fully laparoscopic Puestow surgery was performed.

Secretin-enhanced MRCP (S-MRCP) provides multiple advantages compared with standard MRCP for imaging the pancreaticobiliary tree. By using secretin to induce fluid production from the pancreas, and leveraging fluid-sensitive MRCP sequences, S-MRCP increases visualization of ductal anatomy and provides insight into pancreatic function, allowing radiologists to offer additional insight for a range of pancreatic-related conditions. This narrative review provides detailed information on the practical implementation of S-MRCP, including patient preparation, logistics of secretin administration, and dynamic secretin-enhanced MRCP acquisition. Considerations are given for radiologists’ interpretation and reporting of S-MRCP examinations, including assessment of dynamic compliance of the main pancreatic duct and of duodenal fluid volume. Established indications for S-MRCP are reviewed, including pancreas divisum, anomalous pancreaticobiliary junction, Santorinicele, Wirsungocele, chronic pancreatitis, main pancreatic duct stenosis, and assessment of complex postoperative anatomy. Equivocal or controversial indications are also presented, along with the authors’ approach to such indications; these include acute or recurrent acute pancreatitis, pancreatic exocrine function, sphincter of Oddi dysfunction, and pancreatic neoplasms.

Antecedentes La pancreatitis es una enfermedad rara. La obstruccion es comun y se puede corregir con endoscopia; si fracasa, necesitara cirugia. Caso clinico Reportamos un paciente con pancreatitis secundaria a pancreas divisum. Se realizo derivacion tipo DuVal laparoscopica. Varon de 12 anos, con multiples cuadros de pancreatitis y fallidos intentos de esfinteroplastias. La colangiorresonancia mostro pancreas divisum. Se realizo una derivacion tipo DuVal por laparoscopia. Seguimiento de 5 anos. No ha habido recurrencia del dolor y la lipasa se normalizo a los 6 meses. El conducto pancreatico no estaba dilatado. Ante la dificultad para hacer una anastomosis lateral se utilizo la tecnica laparoscopica propuesta por DuVal. Background Pancreatitis is rare. Obstruction is common. They can be corrected with endoscopy, if they fail they need surgery. Case report We reported a patient with pancreatitis secondary to pancreas divisum, laparoscopic DuVal shunt was performed. Male 12 years, multiple pancreatitis pictures and failed sphinteroplasty attempts. Colangio resonance showed pancreas divisum. A DuVal-type shunt was built for laparoscopy. Follow-up 5 years. There has been no recurrence of pain and lipase was normalized at 6 months. The pancreatic duct was not dilated, in the face of difficulty making a lateral anastomosis was used the technique proposed by DuVal by laparoscopic approach.

We aimed to assess factors impacting the endoscopic minor papilla sphincterotomy (EMPS) success rate, clinical efficacy, and safety in a large cohort of patients with symptomatic pancreas divisum (PD).Retrospective study including patients with PD referred to the Pancreas Institute of Verona from May 2009 to May 2020 to undergo EMPS. The whole population was analyzed to assess EMPS technical success, defined as the rate of deep cannulation of the dorsal duct. Patients treated for recurrent pancreatitis (RP) with a minimum follow-up of 1 year were included to evaluate the clinical efficacy, defined as resolution or significant reduction of acute pancreatitis (AP) episodes. Safety was defined as the rate of procedure-related adverse events (AEs) according to an international lexicon. The effects of the main determinants on study outcomes were evaluated.Overall, 106 patients were evaluated. Technical success was obtained in 87 (82.1%). The presence of pancreatic calcifications was associated with failure (p 0.0001). Clinical efficacy was evaluated in 59 patients. Resolution/reduction of AP episodes after EMPS was observed in 93% of patients over a median follow-up of 49 months (IQR 37-92). Smoking habit was associated with AP recurrence (p = 0.026). The overall AE rate was 14.9%, with post-ERCP pancreatitis as the most common complication (12.6%).In our study, performed at a tertiary center, EMPS showed satisfactory technical success and an acceptable safety profile. If confirmed by prospective multicenter studies, EMPS could become the standard of care for the treatment of RP in PD.

The retroperitoneum is the anatomical space in the abdomen located behind the peritoneum. Structures that lie between the parietal peritoneum and the abdominal wall that are not suspended by a mesentery are classified as retroperitoneal in location and include the pancreas, adrenal glands, kidneys, duodenum, portions of the colon, great vessels, lymph nodes, and lymphatics. In this chapter, normal development and anatomy of the pancreas, adrenal glands, and retroperitoneum are discussed, as well as anatomic variants, congenital abnormalities, inflammatory, traumatic and neoplastic disorders.

The pancreas is a digestive organ with both exocrine and endocrine functions that sits in the retroperitoneum at the level of the second lumbar vertebra. It has an extremely close spatial relationship with multiple organs (i.e., the duodenum, stomach, transverse colon, and bile duct) and multiple major blood vessels, including the celiac axis, superior mesenteric artery and vein, portal vein, and vena cava. Acknowledgment and understanding of these close proximities is vital when performing surgical procedures involving the pancreas. Knowledge of pancreatic embryology is also key for the identification of congenital anomalies and their relevance for patients. An in-depth understanding of overall pancreatic anatomy and normal variants is also critical for the proper diagnosis and treatment of any potential resulting pancreatic pathology. Anatomic anomalies and variations of the pancreas include pancreas divisum, annular pancreas, ectopic pancreas, pancreatic agenesis and hypoplasia, pancreatic cysts, and variations of the course and configuration of the duct.

Objectives Pancreas divisum (PD) is a risk factor in children for the development of acute pancreatitis. Endoscopic retrograde cholangiopancreatography (ERCP) with minor papilla endoscopic sphincterotomy (mPES) may be of clinical benefit, however, the clinical outcomes from endotherapy remain unclear. We sought to review the outcomes and safety of therapeutic ERCP in children with PD. Methods We performed a retrospective chart of children with PD who underwent an ERCP between February 2012 and December 2018. Pertinent patient, clinical and procedure information was collected including procedure-related adverse events. A follow-up questionnaire of the parent was conducted to determine the clinical impact from endotherapy. Results Fifty-eight ERCPs were performed in 27 patients (14 boys; mean age: 9.7 years, range 2-19) with PD. All patients underwent a successful mPES. A genetic variant was identified in 19/26 (73%) tested patients. Post-ERCP pancreatitis (PEP) was the only observed adverse event; 21% (12/58). Median follow-up interval from first ERCP intervention to questionnaire completion was 31.5 months (range: 4--72 months). Of the 20 questionnaire responders, 13 reported clinical improvement from endotherapy. Conclusions The majority of children from our PD cohort possessed at least 1 genetic variant. Most questionnaire responders had a favorable response to endotherapy. PEP rate was comparable with that of prior reports in adult patients.

Chronic pancreatitis (CP) is a rare disease in children. We describe the first case of a 3-year-old Caucasian patient with CP with the presence of a homozygous pathogenic variant c.194 + 2T > C in serine protease inhibitor, Kazal type 1 (SPINK1) and pancreas divisum.

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.

Introduction In this study, we describe for the first time a Neurofibromatosis type 1 patient with pancreas divisum, multiple periampullary tumors and germline pathogenic variants in NF1 and CFTR genes. Case report A 62-year-old female NF1 patient presented with weakness, choluria, nausea, and diffuse abdominal pain to an emergency room service. Magnetic resonance imaging revealed an abdominal mass involving the periampullary region and pancreas divisum. After surgical resection, three synchronous neoplasms were detected including two ampullary tumors (adenocarcinoma of the major ampulla and a neuroendocrine tumor of the minor ampulla) and a gastrointestinal stromal tumor (GIST). Germline multigene panel testing (MGPT) identified two pathogenic heterozygous germline variants: NF1 c.838del and CFTR c.1210-34TG[12]T[5]. Conclusion This is the first report of a Neurofibromatosis type 1 patient with pancreas divisum and multiple periampullary tumors harboring pathogenic germline variants in NF1 and CFTR genes. The identification of two germline variants and a developmental anomaly in this patient may explain the unusual and more severe findings and underscores the importance of comprehensive molecular analyses in patients with complex phenotypes.

Detailed knowledge of pancreatic ductal anatomy is indispensable in order to optimize the therapeutic approach in pancreatic diseases. Magnetic resonance cholangiopancreatography is a precious tool that can help in characterizing aspects of the pancreatic disease as well as determining the anatomy of the ducts and thus to plan treatment. This chapter focuses on the structural anatomy of the pancreas and its ducts, including the most frequent anatomic variations.