Endoscopic minor papilla sphincterotomy in patients with complete pancreas divisum and acute recurrent pancreatitis: a metanalysis.

BACKGROUND AND AIMS: Pancreas divisum (PD) is a congenital variant of the pancreatic ductal system and a potential cause of acute recurrent pancreatitis (ARP). Endoscopic minor papilla sphincterotomy (MiES) is the most common procedure performed in the management of PD-related ARP. The aim of this study is to perform a meta-analysis estimating the efficacy and the safety of MiES in the management of patients with PD-related ARP. METHODS: A research was performed in Pubmed, EMBASE and Web of science, the studies were reviewed and selected according to inclusion and exclusion criteria. Evaluation of Heterogeneity and publication bias was performed, and a random effect model was used to estimate the effect size of each study. RESULTS: One hundred and thirteen articles were selected and reviewed, 13 met the inclusion criteria. All the studies were retrospective with a mean follow-up duration of 45.9 months. A total of 323 patients with PD-related ARP treated with MiES were included in the meta-analysis. The overall clinical success rate of MiES (defined as no further episodes of ARP, reduction of episodes of ARP, or improvement in quality of life) was of 77% (95%CI: 72%-81%; p = 0.30). Evaluating only the studies with clinical success rate defined as "no further AP in the follow-up" the clinical success rate was of 69.8% (95%CI: 61.3%-77.2%; p = 0.57), while evaluating the studies with other definitions (reduction of episodes of ARP or improvement in quality of life) the clinical success rate was of 81.2% (95%CI: 75.2%-86.1%; p = 0.45). The common fixed effects model disclosed a 25.5% overall adverse events rate (95%CI: 19.3%-32.8%; p = 0.42): acute pancreatitis in 14.3% (95%CI: 9.7%-20.6%; p = 0.36), bleeding in 5.6% (95%CI: 2.9%-10.4%; p = 0.98), and other adverse events in 5.6% (95%CI: 2.9%-10.4%; p = 0.67). CONCLUSION: MiES is an effective and relatively safe treatment in the management of PD-related ARP. The retrospective nature of the studies selected is the main limitations of this metanalysis. Prospective trials are needed to confirm these data.

Design and execution of sham-controlled endoscopic trials in acute pancreatitis: Lessons learned from the SHARP trial.

Using the ongoing NIDDK-funded multicenter randomized clinical trial, Sphincterotomy for Acute Recurrent Pancreatitis (SHARP) as an example, this article discusses the rationale and key aspects of study design that need to be considered when conducting a clinical trial of endoscopic therapy in acute pancreatitis. SHARP, the first trial using a sham ERCP in the placebo group, is designed to address a decades long controversy in clinical pancreatology, i.e. whether minor papilla sphincterotomy benefits patients with idiopathic acute recurrent pancreatitis who also have pancreas divisum. Although the trial has already enrolled and randomized over 5 times the number of subjects enrolled in the only randomized trial in this area published in 1992 (107 vs. 19), recruitment has been challenging and we are at ∼46% of target recruitment. The review discusses the challenges in the execution of the trial and strategies the SHARP team has used to address these, which investigators planning or considering treatment trials in pancreatitis may find helpful. It will also inform the general gastroenterologists the importance of discussing and referring potentially eligible subjects to centers participating in clinical trials. Developing evidence-based treatment will provide a solid scientific basis for physicians to recommend evidence-based treatments for pancreatitis.

Safety and efficacy of minor papillotomy in children and adolescents with pancreas divisum.

INTRODUCTION: Pancreas Divisum (PD) is a common pancreatic ductal variant which is twice as common in pediatric patients with acute recurrent pancreatitis (ARP) relative to the general population (14% vs. 7%). Endoscopic retrograde cholangiopancreatography (ERCP) with minor papillotomy has been performed to facilitate drainage of pancreatic juice from the diminutive minor papilla to prevent pancreatitis and pancreatic damage. METHODS: We searched our prospectively-maintained endoscopy databases for patients 18 and younger who underwent ERCP with minor papillotomy between 2009 and 2019. Demographic data, indications, procedural interventions and findings, as well as available clinical outcomes data were analyzed. RESULTS: 54 ARP/PD patients underwent ERCP with minor papillotomy. Median age was 14 (range 7-18) years, and 26 (48.1%) patients were female. Post-ERCP pancreatitis developed in 10/54 patients (18.5%). 12-month post-ERCP clinical trajectory was available in 47/54 (87%) patients and most patients (38/47, 80.8%) improved clinically after minor papillotomy, with 9/47 (19.1%) experiencing resolution of pancreatitis episodes and none indicated worsening severity or frequency of pancreatitis episodes following ERCP. CONCLUSION: The majority of children and adolescents with PD and ARP who underwent ERCP with minor papillotomy experienced subjective improvement in their symptoms following the intervention. These data suggest that ERCP with minor papillotomy for pediatric patients with PD and ARP is beneficial and may be curative in a subset of patients-higher rates of improvement than have been previously reported in adults.

Periampullary tumors in a patient with pancreatic divisum and neurofibromatosis type 1: a case report.

INTRODUCTION: We present a case of a male patient with neurofibromatosis type 1 diagnosed with pancreatic divisum and several gastrointestinal tumors. A 55-year-old man was admitted to the hospital with recurrent chronic pancreatitis, indicating a large mass in the ampulla. In addition, genetic testing revealed two unique germline mutations in the neurofibromin (NF1) gene, and their potential interaction in promoting cancer was further investigated. CONCLUSION: The first similar case was reported in 2020. The current case was distinct from other cases since an additional two NF1 mutations were found in the patient. In conjunction with prior case reports, our findings imply that genetic testing in patients diagnosed with neurofibromatosis type 1 could be helpful in the development of effective treatments.

Pancreaticobiliary maljunction in Turkish patients: a multicenter case series.

BACKGROUND AND AIMS: 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. METHODS: 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. RESULTS: 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 < 0.05), whereas type C was more common in pediatric patients (31.3% vs. 13.2%, p < 0.05). Although fusiform anatomy was predominant in both of the groups, cystic dilatation was more common (25.8% vs. 12.5%) in adults and the common bile duct diameter was greater [22 mm (range 11-58) vs. 12 mm (range 5-33)] in adult patients compared to pediatric patients. Resective surgeries were more frequently done in pediatric patients (73.3% vs. 53.6%), whereas cholecystectomy was more frequently performed in adult patients (21.4% vs. 6.7%). CONCLUSION: Although our findings were compatible with Eastern studies, type D PBM (associated with pancreas divisum) was more frequent in our study population.

Pancreaticobiliary maljunction and pancreas divisum accompanied with intestinal malrotation: a case report.

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.

Pancreatic ductal anatomy: more than meets the eye.

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.

Reappraisal of factors impacting the cannulation rate and clinical efficacy of endoscopic minor papilla sphincterotomy.

BACKGROUND: 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). METHODS: 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. RESULTS: 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%). CONCLUSIONS: 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.

Pancreas Divisum Increases the Risk of Recurrent Acute Pancreatitis in Patients with rs12338 Polymorphism in the Cathepsin B Gene.

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.

Pancreas Divisum as a Rare Etiology of Recurrent Pancreatitis: A Rare Case Ever Documented and Reported In Indonesia.

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.

Incidence and risk factors for post-ERCP pancreatitis in pancreas divisum patients without chronic pancreatitis.

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.

Pancreas divisum inversus.

Pancreatic duct variations are usually diagnosed incidentally, in particular when using magnetic resonance cholangiopancreatography (MRCP), the most accurate imaging modality for depicting the pancreatic ductal system. However, the frequency and the embryologic development of pancreatic variants have not been well investigated. The purpose of this prospective study was to investigate the frequency of pancreatic ductal variants, providing potential explanations of their embryologic basis. The pancreatic ductal anatomies of 202 patients with mean ± standard deviation (SD) age of 54 ± 27 years, 56% females, who underwent MRCP for different indications between April 2018 and March 2019, were prospectively collected. Normal pancreatic ductal variants were identified in 196 cases (97%), and variants of pancreas divisum in six cases (3%). In the type C variant of the normal pancreatic anatomy, found in 3% of the cases, the dorsal duct was joined to the ventral duct while the accessory duct did not communicate with the dorsal duct. Unlike the classic type C variant, in our cases, the accessory pancreatic duct (APD) was long (mean ± SD of 58 ± 8.5 mm) and originated in the lower portion of the pancreatic head, caudally to the duct of Wirsung. This was a new subtype of the type C variant or a new variant, which could be called "pancreas divisum inversus"; the APD could be called the isolated duct of Santorini. Reporting this new variant could increase knowledge regarding the pancreatic anatomy in order to avoid misdiagnosis and to help in better understanding pancreatic diseases and their relative treatment. Clin. Anat., 33:646-652, 2020. © 2019 Wiley Periodicals, Inc.

Separate pancreatic ducts draining into a type IV choledochal cyst: simultaneous anatomical variants in a child suffering from cholelithiasis and acute pancreatitis.

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.

Long-term outcomes of therapeutic ERCP in pediatric patients with pancreas divisum presenting with acute recurrent or chronic pancreatitis.

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 ±â€¯1.7 vs. 1.1 ±â€¯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.

Pancreas divisum and recurrent pancreatitis: long-term results of minor papilla sphincterotomy.

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.

Is MRCP necessary to diagnose pancreas divisum?

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.

Diagnosis and treatment of pancreas divisum: A literature review.

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.

Circumportal pancreas accompanied with pancreas divisum in a deceased donor for islet transplantation.

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.

Efficacy of Endoscopic Minor Papilla Sphincterotomy for Symptomatic Santorinicele.

BACKGROUND & AIMS: Santorinicele, a rare focal cystic dilation of the distal portion of the dorsal pancreatic duct at the minor papilla, can be a cause of recurrent acute pancreatitis (RAP). Endoscopic minor papilla sphincterotomy (EMPS) has been evaluated as a treatment in case reports but never systematically investigated. METHODS: We performed a retrospective analysis of the efficacy of EMPS in reducing episodes of pancreatitis. We collected data on 30 patients with santorinicele and RAP who underwent EMPS from June 2009 through April 2015 at University Hospital of Verona in Italy. The mean follow-up period was 43.8 months. RESULTS: The average number of pancreatitis episodes per year before EMPS was 1.59 vs 0.18 episodes after EMPS; the average number of pancreatitis cases that occurred during a comparable time period before EMPS was 2.63 vs 0.67 cases after EMPS (P < .0001). Complete responses to EMPS (no recurrence of pancreatitis) were reported for 80% of patients. Six patients relapsed after a mean time of 16 months. Five patients were found to have a potential cause of RAP beyond santorinicele (2 patients had post-sphincterotomy stenosis, 1 patient was a chronic consumer of alcohol, 1 patient had a mutation in the CFTR gene, and 1 patient had a side-branch intraductal papillary mucinous neoplasm). CONCLUSIONS: EMPS is effective in reducing the incidence of pancreatitis in patients with santorinicele.

The impact of surgical strategies on outcomes for pediatric chronic pancreatitis.

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.