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Introduction: Pancreas divisum (PD) is a common congenital anomaly of the pancreas, but its genetic basis remains unknown. The purpose of this genome-wide association study was to identify genetic loci associated with PD., Methods: Using the Mass General Brigham Biobank, patients diagnosed with PD were identified. Quality control and imputation were performed using standard approaches. Single nucleotide polymorphisms (SNPs) with minor allele frequency (MAF) ≥ 5% were tested for association with PD using mixed linear model-based association analysis. The significance threshold was set at 5 × 10 -8 ., Results: A total of 13,940 subjects were included, of which 251 (1.8%) were diagnosed with PD. A genetic locus in chromosome 3q29 was found to be associated with PD (lead SNP rs3850646, MAF PD  = 34.6% vs. MAF controls  = 26.4%, beta = 0.0106, P = 1.47 × 10 -8 ). The identified locus is located in the phosphatidylinositol glycan anchor biosynthesis class Xand p21 activated kinase 2genes. The heritability of PD was estimated at 27.5%. (Expression quantitative trait loci) and chromatin interaction analysis found 12 genes whose expression may be regulated by SNPs in this genomic locus., Conclusions: The results of this study suggest that a genetic locus at 3q29 is associated with PD. This locus is in the phosphatidylinositol glycan anchor biosynthesis class X and p21 activated kinase 2 genes. Twelve candidate genes were identified whose expression may be regulated by this locus. These findings may help us understand both normal and aberrant pancreatic development and may aid in clinical evaluation and genetic counseling of patients with PD and associated diseases, such as acute pancreatitis., (Copyright © 2024 Elsevier Inc. All rights reserved.)

BACKGROUND A choledochal cyst (CC), or biliary cyst, is a congenital or acquired anomaly of the biliary tree. Pancreas divisum (PD) is a rare congenital anomaly due to incomplete fusion of pancreatic ducts, which can complicate the clinical course of choledochal cysts. This rare combination is a surgical management challenge. This report presents the diagnosis and management of a 23-year-old woman with a combined choledochal cyst and pancreas divisum treated with pancreaticoduodenectomy. CASE REPORT This article presents the case of a 23-year-old woman who presented with severe, stabbing abdominal pain radiating to the back and epigastric tenderness and was diagnosed with pancreatitis. Initial imaging revealed a choledochal cyst, prompting further investigation with ERCP that showed concomitant PD. She was treated via pancreaticoduodenectomy. During the following 9 years, she was hospitalized over 2 dozen times for recurrent pancreatitis. CONCLUSIONS This report presents a complex case of a combined choledochal cyst and pancreas divisum, which was surgically managed by pancreaticoduodenectomy. The association of CC with PD should be suspected in patients with recurrent acute pancreatitis and/or cholangitis with no identifiable cause. Surgical treatment of CC with PD depends on the classification of the CC, and complications can include recurrent pancreatitis, though prognosis is often favorable. The purpose of this manuscript is to emphasize that pancreaticoduodenectomy is unlikely to provide favorable outcomes for CC with PD, especially considering there is evidence that less extensive surgical interventions produce better outcomes.

A 50-year old male with acute necrotizing pancreatitis had an unfavorable evolution in the third week of disease, with development of large volume ascites and walled-off necrosis affecting the head and body of pancreas, suspected to be infected, with viable parenchyma in the tail of pancreas. Endoscopic ultrasound guided drainage of the collection was performed through placement of a lumen apposing metal stent. Selective cannulation of main pancreatic duct was only possible though the minor papilla and after contrast media injection a type 1 pancreas divisum and partial disruption of dorsal pancreatic duct were diagnosed.

A 56-year-old man was referred to our hospital for fever and back pain. Contrast-enhanced computed tomography, magnetic resonance cholangiopancreatography (MRCP) and endoscopic ultrasound revealed marked dilatation of the main pancreatic duct from head to tail and surrounding cysts with no evidence of pancreatic masses or obstructive causes. Endoscopic retrograde pancreatography (ERP) from the major duodenal papilla showed a short and narrow ventral pancreatic duct and ERP from the minor duodenal papilla revealed a dilated dorsal pancreatic duct with a narrow branch which connected to the ventral pancreatic duct. Intra-ductal ultrasound showed no nodules in the main pancreatic duct, and pancreatic cytology was negative. Peroral pancreatoscopy showed that the main pancreatic duct was covered with normal epithelium without tumors. Biopsy from the pancreatic duct showed no neoplastic changes. He was diagnosed with incomplete pancreas divisum accompanied by dilatation of dorsal pancreatic duct. Sphincterotomy of the minor duodenal papilla was performed. Seven months later, MRCP re-examination showed that the main pancreatic duct dilatation was relieved. Review of the literature identified 10 cases of pancreas divisum accompanied by dilatation of main pancreatic duct to date. To our knowledge, this is the first reported case of pancreas divisum with pancreatic duct dilatation without Santorinicele., (© 2022. Japanese Society of Gastroenterology.)

Pancreas divisum is an abnormal condition of pancreas duct that occurs from organogenesis. This abnormal condition defined as a failure in fusion between dorsal and ventral part of the pancreas. The incidence reported 4%-14% in general population. Majority patient with pancreas divisum will not present with any sign or symptom, but in some cases may present with signs of pancreatitis. We illustrate a case of 39 years old male with pancreas divisum presenting as acute recurrent pancreatitis. Diagnosis of pancreas divisum was determined through magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP). Patient treated by sphincterotomy and dilation using Soehendra's dilator catheter. This is the first pancreas divisum case successfully treated and reported in Indonesia.

Objectives: 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., Design: 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., Results: 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., Conclusion: Risk of RAP due to PD increases in patients with rs12338 polymorphism in the cathepsin B gene.

Aims: The studies on post-endoscopic retrograde cholangiopancreatography (ERCP) pancreatitis (PEP) in pancreas divisum (PD) patients without chronic pancreatitis (CP) are rare. In this study, we aimed to evaluate the incidence of PEP in PD patients without CP and the risk and protective factors for PEP. Methods: Consecutive patients with symptomatic PD that underwent ERCP from January 2005 to December 2017 were retrospectively analyzed. The patients were divided into PD without CP group and CP group. The basic information and medical records of patients were collected. The risk and protective factors for PEP in PD patients without CP were analyzed by univariate logistic analysis. Results: A total of 89 ERCP procedures were performed in 51 PD patients without CP, and 249 procedures in 136 patients with CP. The incidence of PEP was significantly higher in PD patients without CP than those with CP (15.7% vs. 5.6%, p  = .005). Female gender were independent risk factors for PEP, while dorsal duct stent placement was a protective factor. Conclusion: CP may be a protective factor against PEP in PD patients. Female was a risk factor for PEP in PD patients and dorsal duct stent placement was a preventive factor that reduced the incidence of PEP in PD patients without CP.

Developmental anomalies of the pancreas include more common entities such as pancreas divisum and annular pancreas, and unusual entities such as heterotopic pancreas. While these anomalies can present a diagnostic challenge to the radiologist, when the pancreatic tissue involved in these processes develops pancreatitis, the radiographic appearance can present a diagnostic dilemma. Awareness of these pancreatic anomalies and familiarity with their appearance when inflamed is necessary to distinguish pancreatitis in the developmentally anomalous pancreas from other inflammatory or neoplastic processes.

The biliary tree and the pancreatic ducts originate from different buds of the primitive foregut. A plethora of anatomical variants and congenital anomalies have been described for both the biliary and the pancreatic duct systems, although they are usually asymptomatic. However, in some cases, these abnormalities can lead to serious clinical scenarios, especially when involving the biliopancreatic junction, with consequent mixing and reflux of the two different juices. To the best of our knowledge, we present the first case of pancreas divisum ducts draining into a choledochal cyst, with the lower third of the choledochus configuring a common biliopancreatic duct, in a child suffering from cholelithiasis and acute pancreatitis.

Introduction: 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., Methods: 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., Results: 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., Conclusions: 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.

Introduction : Pancreas divisum is the most common congenital malformation of the pancreas with the majority asymptomatic. The etiological role, pathogenesis, clinical significance and management of pancreas divisum in pancreatic disease has not been clearly defined and our understanding is yet to be fully elucidated. Areas covered : This review describes the role of pancreas divisum in the development of pancreatic disease and the ambiguity related to it. In our attempt to offer clarity, a comprehensive search on PubMed, Ovid, Embase and Cochrane Library from inception to May 2019 was undertaken using key words "pancreas divisum", "idiopathic recurrent acute pancreatitis" and "chronic pancreatitis". Expert opinion : Current research fails to define a clear association between pancreas divisum and pancreatic disease. Though debatable, several studies do suggest a pathological role of pancreas divisum in pancreatic disease and a benefit of minor papilla therapy in the setting of acute recurrent pancreatitis. Surgical and endoscopic therapeutic modalities have not been directly compared. With the current data available, it would be imprudent to advise a definitive line of management for pancreatic disease associated with pancreas divisum and should involve a comprehensive discussion with the individual patient to define expectations before embarking on any medical and/or interventional therapy.

Objectives: 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)., Methods: 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., Results: 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., Conclusions: 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., (Copyright © 2019 IAP and EPC. Published by Elsevier B.V. All rights reserved.)

Background: The purpose of this study is to compare the performance of three-dimensional magnetic resonance cholangiopancreatography (3D-MRCP) with non-MRCP T2-weighted magnetic resonance imaging (MRI) sequences for diagnosis of pancreas divisum (PD)., Methods: This is a retrospective study of 342 consecutive patients with abdominal MRI including 3D-MRCP. 3D-MRCP was a coronal respiration-navigated T2-weighted sequence with 1.5 mm slice thickness. Non-MRCP T2-weighted sequences were (1) a coronal inversion recovery sequence (TIRM) with 6 mm slice thickness and (2) a transverse single shot turbo spin echo sequence (HASTE) with 4 mm slice thickness. For 3D-MRCP, TIRM, and HASTE, presence of PD and assessment of evaluability were determined in a randomized manner. A consensus read by two radiologists using 3D-MRCP, non-MRCP T2-weighted sequences, and other available imaging sequences served as reference standard for diagnosis of PD. Statistical analysis included performance analysis of 3D-MRCP, TIRM, and HASTE and testing for noninferiority of non-MRCP T2-weighted sequences compared with 3D-MRCP., Results: Thirty-three of 342 patients (9.7%) were diagnosed with PD using the reference standard. Sensitivity/specificity of 3D-MRCP for detecting PD were 81.2%/69.7% (p < 0.001). Sensitivity/specificity of TIRM and HASTE were 92.5%/93.9 and 98.1%/97.0%, respectively (p < 0.001 each). Grouped sensitivity/specificity of non-MRCP T2-weighted sequences were 99.8%/91.0%. Non-MRCP T2-weighted sequences were non-inferior to 3D-MRCP alone for diagnosis of PD. 20.2, 7.3%, and 2.3% of 3D-MRCP, TIRM, and HASTE, respectively, were not evaluable due to motion artifacts or insufficient duct depiction., Conclusions: Non-MRCP T2-weighted MRI sequences offer high performance for diagnosis of PD and are noninferior to 3D-MRCP alone., Trial Registration: Not applicable.

Background and aims: Pancreas divisum (PD) is the most common congenital variant of the pancreatic ductal system and a potential cause of acute recurrent pancreatitis (ARP). Endoscopic therapy is a therapeutic option for symptomatic PD, but there is limited data on long-term results. We aimed to assess the effect of minor papilla endoscopic sphincterotomy (MiES) in the setting of ARP in patients with PD. Methods: Consecutive patients treated by MiES were included. Clinical data, including gender, age, smoking and drinking habits, number of episodes of acute pancreatitis (AP) as well as technical data pertaining to the endoscopic therapy were reviewed. Patients available for follow-up were contacted to assess the long-term impact of MiES using the Patient's Global Impression of Change (PGIC) questionnaire. Results: A total of 138 patients with PD including 77 patients with ARP underwent MiES; 48 patients were available for long-term follow-up using the PGIC score, with a mean follow-up period of 9.7 years. Procedure-related adverse events developed in 10 cases (12.9%): 5 post-MiES delayed bleeding and 5 mild pancreatitis. MiES was clinically successful in 35 patients (72.9%) who did not experience any more episodes of AP. Improvement in quality of life (PGIC ≥6) occurred in 41/48 patients (85.4%). On multivariate analysis, stenosis of the MiES was the only predictive factor for increased risk of recurrent pancreatitis after initial therapy. Conclusion: MiES resulted an efficient treatment for ARP in patients with PD with clinical benefit, patient satisfaction and improved quality of life even at long-term follow-up.

Circumportal pancreas is a rare and previously not well-recognized anatomical variant. In contrast, pancreas divisum is the most frequent anatomical variant in the pancreas. We report a case in which circumportal pancreas was accompanied with pancreas divisum in a deceased donor for islet transplantation. A unique aspect of our case is that the connecting portion behind the portal vein between the pancreas head and body originated from the ventral pancreas. This is the first case report describing a ventral embryogenic origin of the connecting portion of a circumportal pancreas accompanied with pancreas divisum.

Background: Pancreas divisum is the most common anatomical variation of pancreatic ductal system affecting 5-10% of population. Therapy includes different endoscopic and surgical procedures. The aim of this article was to summarize actual evidence of different treatment., Methods: A Medline search was performed to identify all studies, investigating endoscopic or surgical therapy of Pancreas divisum. An individual data simulation model was applied to compare endoscopic and surgical studies., Result: 56 observational studies (31 endoscopic and 25 surgical studies) were included in analyses. Surgery was significantly superior to endoscopic treatment in terms of success rate (72% vs. 62.3), complication rate (23.8% vs. 31.3%) and re-intervention rate (14.4% vs. 28.3%)., Conclusion: Surgery may be superior to endoscopy in terms of treatment success and complications. There is no study comparing these two therapies. Consequently, a randomized trial is needed to clarify if endoscopy or surgery is superior in the therapy of pancreas divisum., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: To review our institutional experience in the surgical treatment of pediatric chronic pancreatitis (CP) and evaluate predictors of long-term pain relief., Methods: Outcomes of patients ≤21 years surgically treated for CP in a single institution from 1995 to 2014 were evaluated., Results: Twenty patients underwent surgery for CP at a median of 16.6 years (IQR 10.7-20.6 years). The most common etiology was pancreas divisum (n = 7; 35%). Therapeutic endoscopy was the first-line treatment in 17 cases (85%). Surgical procedures included: longitudinal pancreaticojejunostomy (n = 4, 20%), pancreatectomy (n = 9, 45%), total pancreatectomy with islet autotransplantation (n = 2; 10%), sphincteroplasty (n = 2, 10%) and pseudocyst drainage (n = 3, 15%). At a median follow-up of 5.3 years (IQR 4.2-5.3), twelve patients (63.2%) were pain free and five (26.3%) were insulin dependent. In univariate analysis, previous surgical procedure or >5 endoscopic treatments were associated with a lower likelihood of pain relief (OR 0.06; 95% CI 0.006-0.57; OR 0.07; 95%, CI 0.01-0.89). However, these associations were not present in multivariate analysis., Conclusion: In children with CP, the step-up practice including a limited trial of endoscopic interventions followed by surgery tailored to anatomical abnormalities and gene mutation status is effective in ensuring long-term pain relief and preserving pancreatic function.

Background/aims: To evaluate the efficacy and safety of endoscopic retrograde cholangiopancreatography (ERCP) for the treatment of symptomatic pancreas divisum (PD) and to discuss whether ERCP procedures and outcomes in younger patients differ from those of adults., Methods: Symptomatic patients with PD were included in the study and divided into underaged (age ≤17 years) and adult (age ≥18 years) group. The clinical information of each patient was reviewed, and then the patients were contacted by telephone or their medical records were reviewed to determine their long-term followup outcomes., Results: A total of 141 procedures were performed in 82 patients (17 underaged and 65 adult patients). The ERCP indications included abdominal pain (39.02%), pancreatitis (12.20%), recurrent pancreatitis (36.59%), and other discomfort (12.20%). The endoscopic interventions included endoscopic pancreatic sphincterotomy in 44.68% of the patients, bouginage in 26.95%, pancreatic ductal stone extraction in 19.15%, endoscopic nasopancreatic drainage in 21.99%, and endoscopic retrograde pancreatic drainage in 56.74%. After a median follow-up of 41 months, the overall response rate was 62.32%. Between the underaged group and the adult group, significant differences were not observed in the ERCP procedures, complications and longterm follow-up results., Conclusions: ERCP is a safe and effective treatment for symptomatic PD. Based on the details, complications, and follow-up results, the ERCP procedure did not present differences between the underaged and adult groups.

Endoscopy plays an important role in both the diagnosis and the initial management of recurrent acute pancreatitis, as well as the investigation of refractory disease, but it has known limitations and risks. Sound selective use of these therapies, complemented with other lines of investigation such as genetic testing, can dramatically improve frequency of attacks and associated quality of life. Whether endoscopic therapy can reduce progression to chronic pancreatitis, or reduce the risk of malignancy, is debatable, and remains to be proven., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Pancreas divisum is a frequent congenital anatomical anomaly characterized by the failure of fusion of the ducts of Santorini and Wirsung during fetal development. Although the condition usually remains asymptomatic, it has been reported to be a predisposing factor of chronic and recurrent idiopathic pancreatitis. We report a case of acute non-traumatic pancreatitis in a 54-year-old Caucasian male with pancreas divisum. Diagnosis was established based on the findings from magnetic resonance imaging and magnetic resonance cholangiopancreatography. The patient was managed conservatively and was discharged home having an uneventful clinical course after five days of hospitalization. Although the role of the pancreas in the induction of acute pancreatitis is still a matter of debate, physicians have to be aware about this prevalent pancreatic anatomic abnormality. Timely detection may help in the prevention of potential recurrent pancreatic reaction.

Aim: To determine the magnetic resonance cholangiopancreatography (MRCP) and magnetic resonance imaging (MRI) features of pancreatitis with pancreas divisum (PD) and the differences vs pancreatitis without divisum., Methods: Institutional review board approval was obtained and the informed consent requirement was waived for this HIPAA-compliant study. During one year period, 1439 consecutive patients underwent successful MRCP without injection of secretin and abdominal MRI studies for a variety of clinical indications using a 1.5 T magnetic resonance scanner. Two experienced radiologists retrospectively reviewed all the studies in consensus. Disputes were resolved via consultation with a third experienced radiologist. The assessment included presence and the imaging findings of PD, pancreatitis, and distribution of abnormalities. The pancreatitis with divisum constituted the study group while the pancreatitis without divisum served as the control group. MRCP and MRI findings were correlated with final diagnosis. Fisher exact tests and Pearson × 2 tests were performed., Results: Pancreatitis was demonstrated at MRCP and MRI in 173 cases (38 cases with and 135 cases without divisum) among the 1439 consecutive cases. The recurrent acute pancreatitis accounted for 55.26% (21 of 38) in pancreatitis patients associated with PD, which was higher than 6.67% (9 of 135) in the control group, whereas the chronic pancreatitis was a dominant type in the control group (85.19%, 115 of 135) when compared to the study group (42.11%, 16 of 38) (χ(2) = 40.494, P < 0.0001). In cases of pancreatitis with PD, the dorsal pancreatitis accounted for a much higher percentage than that in pancreatitis without PD (17 of 38, 44.74% vs 30 of 135, 22.22%) (χ(2) = 7.257, P < 0.05)., Conclusion: MRCP and MRI can depict the features of pancreatitis associated with divisum. Recurrent acute pancreatitis and isolated dorsal involvement are more common in patients with divisum.

Objective: This systematic review analyzes the anatomical variants in the pancreas and its ductal system to report on their association with pancreatic pathologies. Methods: We conducted a search of the MEDLINE, Scopus, Web of Science, Google Scholar, CINAHL, and LILACS databases from their inception to July 2023. The methodological quality was assessed with the Anatomical Quality Assessment (AQUA) tool. Finally, the pooled prevalence was estimated using a random effects model. Results: 55 studies were found that met the eligibility criteria. The overall prevalence of pancreas divisum (PD) was 18% (95% CI = 15–21%). The prevalence of PD associated with pancreatitis was 30% (95% CI = 1–61%). Conclusions: An anatomical variant of the pancreas such as PD may be the cause of bile duct obstruction, resulting in various clinical complications, such as pancreatitis. Hence, knowing this variant is extremely important for surgeons, especially for those who treat the gastroduodenal region. [ABSTRACT FROM AUTHOR]

ObjectivesIn patients with acute recurrent pancreatitis (ARP), pancreas divisum, and no other etiologic factors, endoscopic retrograde cholangiopancreatography (ERCP) with minor papilla endoscopic sphincterotomy (miES) is often performed to enlarge the minor papillary orifice, based on limited data. The aims of this study are to describe the rationale and methodology of a sham-controlled clinical trial designed to test the hypothesis that miES reduces the risk of acute pancreatitis.MethodsThe SpHincterotomy for Acute Recurrent Pancreatitis (SHARP) trial is a multicenter, international, sham-controlled, randomized trial comparing endoscopic ultrasound + ERCP with miES versus endoscopic ultrasound + sham for the management of ARP. A total of 234 consented patients having 2 or more discrete episodes of acute pancreatitis, pancreas divisum confirmed by magnetic resonance cholangiopancreatography, and no other clear etiology for acute pancreatitis will be randomized. Both cohorts will be followed for a minimum of 6 months and a maximum of 48 months.ResultsThe trial is powered to detect a 33% risk reduction of acute pancreatitis frequency.ConclusionsThe SHARP trial will determine whether ERCP with miES benefits patients with idiopathic ARP and pancreas divisum. Trial planning has informed the importance of blinded outcome assessors and long-term follow-up.

Purpose: Anatomical variations of the pancreas are relatively frequent and often understudied. The ductal system of the pancreas has multiple variations, which are not frequently reported in the literature. Materials and methods: The anatomy of the pancreas was studied through macroscopic anatomical dissection on 50 organ complexes (the pancreas, spleen, and duodenum) donated to the department of human anatomy, from patients, who died of causes not related to pancreatic diseases. Results: In type I, the main pancreatic duct (Wirsung's duct, MPD) and the accessory pancreatic duct (Santorini's duct, APD) were merged but most of the head was drained by the MPD (10% of cases). In type II, the MPD and APD were merged but most of the head was drained by the APD (4% of cases). In type III, the APD was absent and the head was drained by the MPD (14% of cases). In type IV, there was an inverted pancreas divisum where the ducts did not merge but each drained a part of the head (6% of cases). Classical pancreas divisum where the ducts did not merge but each drained a part of the head was considered as type V (4% of cases). In type VI, the MPD and APD merged and each drained a part of the head (48% of cases). In type VII, the MPD and APD merged but the upper part of the head was drained by the main pancreatic duct (4% of cases). In type VIII, the MPD and APD merged but the lower part of the head was drained by the main pancreatic duct (4% of cases). In the IX type, the MPD and APD merged but the head was drained by the branches of the MPD (6% of cases). Conclusions: There are several drainage patterns of the pancreas. In some cases, one of the ducts provides more drainage of the gland than the other. This is clinically relevant since blockage of the main source of drainage leads to pancreatic juice stasis. It also explains cases when partial or total blockage of the duct results in the pancreatitis of an isolated zone. [ABSTRACT FROM AUTHOR]

Background: Pancreaticobiliary maljunction is a congenital anatomical abnorma l junction of the pancreatic duct and bile duct into a common channel outside the duodenal wall. Pancreas divisum is also a congenital anatomical abnormality characterized by unfused pancreatic ducts. Intestinal malrotation is caused by the failure of bowel rotation and fixation. We reported an optimal surgical intervention for the rare case of pancreaticobiliary maljunction and pancreas divisum accompanied intestinal malrotation.Case Presentation: A 2-year-old female presented with fever and jaundice. Abdominal ultrasound showed dilated common bile duct and intrahepatic bile ducts; MRCP showed pancreaticobiliary maljunction, pancreas divisum, and dilated biliary system; Abdominal contrast-enhanced CT showed a reversed relationship between the superior mesenteric artery and the superior mesenteric vein. An operation of laparoscopic resection of the extrahepatic bile duct, Roux-en-Y hepaticojejunostomy, and Ladd's procedure was performed after the inflammation of the biliary system was treated. The post-operative follow-up period was uneventful.Conclusions: The management of pancreas divisum can be conservative. We present an optimal pattern of Roux-en-Y hepaticojejunostomy to deal with pancreaticobiliary maljunction associated with intestinal malrotation. [ABSTRACT FROM AUTHOR]

Introduction: An ampullary carcinoma is a cancer that arises in the ampulla of Vater. It is a tiny opening in the beginning segment of the small intestine called the duodenum. Pancreatic and bile duct fluids are pumped into the intestines via the ampulla of Vater. Jaundice is the most common symptom of ampullary cancer. This is because the tumor in the ampulla of Vater blocks the bile duct. Instead of flowing into the intestines, the bile enters the bloodstream and causes yellowing of the skin. Other symptoms of ampullary cancer include: loss of appetite and weight loss, abdominal pain and vomiting. This case of ampullary carcinoma is associated with pancreas divisum which is a congenital anomaly where dorsal and ventral buds fail to fuse during fetal development 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. Case Report: We report the case of a 67 years old female who presented with abdominal pain in the upper and lower abdomen, vomiting for two days and a history of weight loss (20 kilos in 5 months). The patient previously underwent appendectomy, laparoscopic cholecystectomy and varicose vein surgery on both limbs. The patient underwent investigations such as blood tests, histopathology, ultrasonography, computer tomography and ampullary mass biopsy, based on which a final diagnosis was made. Following the diagnosis, a pancreaticoduodenectomy was performed alongside hysterectomy with bilateral adnexectomy. Discussions : Considering that the patient has a congenital anomaly of the pancreas, we can raise the question whether this anatomical variant represents a risk factor for the development of ampullary cancer, or if in this situation the pancreas is more prone to other pathologies or not. The surgical approach chosen was the Whipple procedure, a complicated operation that can lead to complications such as fistula or leak in the pancreas, leakage of bile or haemorrhage occurring postoperatively, which requires blood transfusion or reopening. Conclusions: Ampullary carcinoma is a very unusual form of carcinoma but can be treated with Whipple procedure. Ampullary carcinoma has a better outcome than pancreatic cancer. It can be managed surgically and the survival rate has also improved. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Aim: To determine the "t sign" diagnostic criteria on MRCP-MIP imaging in patients with pancreas divisum (PD). Material and Methods: Between May 2013 and August 2019 a total of 1289 patients who underwent both ERCP and MRCP, were enrolled to the study. To select the patients with PD diagnosis on both MRCP and ERCP were planned. Patients were compared with control group. MRCP assessment included presence and type of PD, relationship of common bile duct on axial and coronal MIPMRCP images. Test characteristics were introduced for demonstrating diagnostic value of "t sign" on MRCP in patients with PD in the study group compared with those in the control group. Analysis was performed using the Breeze/STAT statistical calculation with computation of the 95% confidence intervals (CIs). Results: Twenty-eight patients with diagnosis of PD according to the MRCP reports were selected for the study. Of the 28 cases, five patients without PD biliary morphology excluded from the study group due to ERCP results and one patient with the diagnosis of "probable PD" by the ERCP report was also excluded. Twenty-two patients had typical PD (95.45 %) and incomplete PD was demonstrated in one case (4.55%). Sensitivity, specificity, positive predictive value, negative predictive value and accuracy value of MRCP-MIP imaging for demonstrating PD with using "t sign" criteria were 100%, 99%, 100%, 99% and 99% respectively. (95% CI).. Conclusion: We concluded that, dorsal main pancreatic duct (MPD) and common bile duct cross relation was identified in all complete PD patients as a "t sign" diagnostic criteria on coronal MIP-MRCP images. [ABSTRACT FROM AUTHOR]

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 theMRCP 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. [ABSTRACT FROM AUTHOR]

Dorsal main pancreatic duct drains to the minor duodenal papilla. a, b, c MRCP revealed chronic pancreatitis associated with pancreas divisum. d The image suggested a main duct intraductal papillary mucinous neoplasm located at the pancreatic head. Routine ultrasound showed main duct dilatation (6 mm), and magnetic resonance imaging (MRI) showed pancreas divisum with signs of chronic pancreatitis. [Extracted from the article]

Pancreas divisum is the most common congenital malformation of the pancreas. Sometimes it is considered an etiology when patients present with recurrent episodes of pancreatitis. Endoscopic retrograde pancreatography (ERP) with selective cannulation of the minor papilla with sphincterotomy and stent placement are performed in these patients. Proximal migration of pancreatic stents in pancreas divisum is rare and challenging to manage. We describe a case of proximal migration of a pancreatic stent in a patient with pancreas divisum and perform a review of literature. [ABSTRACT FROM AUTHOR]

Introduction:  A retrospective study was performed to evaluate the clinical features, diagnostic methods, and treatment alternatives in children with pancreas divisum (PD).Materials and Methods:  Patients who underwent treatment for PD between 1999 and 2014 at our department were evaluated for sex, age, presenting symptoms, physical examination findings, biochemical markers, diagnostic methods, treatment modalities, and results of treatment during follow-up.Results:  Seven patients who underwent treatment of symptomatic PD were included in the study. The median for follow-up period was 8 years (from 26 months to 16 years). Male-to-female ratio was 4:3 and the median age at presentation was 11 years (2-14 years). Presenting symptoms were recurrent episodic epigastric pain. Pancreatitis was documented by elevated amylase or lipase levels. Endoscopic retrograde cholangiopancreatography (ERCP) was the method of diagnosis of PD in all patients. Five patients had complete PD and two had incomplete variants. Three patients improved after ERCP papillotomy. In three patients, papillotomy was unsuccessful but they have only mild episodes of pancreatitis. One patient presented at the age of 4 years with recurrent pancreatitis. She was treated surgically by duodenum-preserving resection of the pancreatic head (DPRPH) because of severe recurrent pancreatitis occurring even after ERCP papillotomy. The patient is 26 months after operation without any reported problems.Conclusion:  Patients with symptomatic PD are indicated for ERCP papillotomy attempt. If there is not improvement after ERCP, then recurrent bouts of severe pancreatitis are considered as an indication for surgical procedure. DPRPH is a safe and feasible surgical alternative. [ABSTRACT FROM AUTHOR]

1 Pancreatogram images showing: a the initial appearance of the narrow Santorini duct; b the broad and shallow contrast passage in the Santorini duct owing to the presence of mucus; c a tumorous lesion in the Santorini duct. Because of the location of the neoplasm within the Santorini duct in a pancreas divisum, this was classified as a main duct IPMN (Video 1). [Extracted from the article]

Background The purpose of this study was to perform a meta-analysis assessing the efficacy and predictors of success of endoscopic therapy in the management of patients with pancreas divisum. Methods An electronic database search (PubMed and ScienceDirect) was performed for relevant studies. Studies were selected based on predefined criteria and data were extracted on patient population, follow up, endotherapy methods, success rates and complication rates. A randomeffect model was used to pool the effect size across studies. Heterogeneity testing and publication bias assessment were performed. Multivariate regression analysis was performed to identify predictors of successful endoscopic therapy. Results Of 381 articles reviewed, 23 studies with 874 patients met the inclusion criteria. All were case series with suboptimal quality. Endoscopic therapy included minor papilla sphincterotomy, minor papilla sphincteroplasty and dorsal duct stenting. Mean follow-up duration was 37 months. The rate of "improvement" as defined by authors after endoscopic therapy varied significantly across studies, ranging from 31-96%: 589/874 patients were reported to have improved, corresponding to a pooled efficacy rate of 67.5% (95% confidence interval [CI] 0.610-0.734; P=0.0001). The pooled rate of pancreatitis after endoscopic retrograde cholangiopancreatography was 10.1% (95%CI 0.084-0.124; 2-sided P=0.0001). On subgroup analysis, patients with recurrent acute pancreatitis had better endoscopic outcomes (pooled efficacy rate 76%, 95%CI 0.712-0.803, P=0.0001). Dorsal duct stenting and longer follow up were the only parameters predictive of successful endotherapy. Significant heterogeneity was observed within and across studies. Conclusions Endoscopic efficacy in pancreas divisum is estimated at 67.5%. Available studies are of poor quality with significant heterogeneity. Comparative studies with rigorous methodology are needed. [ABSTRACT FROM AUTHOR]

Abstract A rare pediatric case of pancreaticobiliary maljunction (PBM) with pancreas divisum is reported. The case showed extremely rare arrangement of the biliary tree and pancreatic duct; the common channel usually seen in PBM did not exist and the common bile duct was connected to the dorsal pancreatic duct via a dilated intermediary duct, in which a large protein plug had formed. The common bile duct was not dilated at all. All of these anomalies may have been caused by abnormal development of the ventral and dorsal pancreatic ducts at an early gestational age. We report a very rare pediatric case of pancreaticobiliary maljunction (PBM) complicated with pancreas divisum. In this case, the common bile duct showed a normal caliber and it was connected to the dorsal pancreatic duct through an intermediary dilated duct, which contained a protein plug that dammed up bile and pancreatic juice flow. This anomaly is very similar to type IIIc3 choledochal cyst, whose classification was addressed by Komi et al. (1), but it differed in the following point; in our case, the common bile duct did not connect to the duodenum through the ampulla of Vater (the common channel was absent) and bile was drained to the duodenum through Santorini duct. These quite rare anomalies might have been caused in the fetus by some kind of maldevelopment of the ventral and dorsal pancreatic ducts. [ABSTRACT FROM AUTHOR]