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Objective: Rare disease 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, although the prognosis is often favorable. The purpose of this report 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. [ABSTRACT FROM AUTHOR]

Groove pancreatitis (GP) is a rare and clinically distinct form of chronic pancreatitis affecting the pancreaticoduodenal groove comprising the head of the pancreas, duodenum, and the common bile duct. It is more prevalent in individuals in their 4–5th decade of life and disproportionately affects men compared with women. Excessive alcohol consumption, tobacco smoking, pancreatic ductal stones, pancreatic divisum, annular pancreas, ectopic pancreas, duodenal wall thickening, and peptic ulcers are significant risk factors implicated in the development of GP. The usual presenting symptoms include severe abdominal pain, nausea, vomiting, diarrhea, weight loss, and jaundice. Establishing a diagnosis of GP is often challenging due to significant clinical and radiological overlap with numerous benign and malignant conditions affecting the same anatomical location. This can lead to a delay in initiation of treatment leading to increasing morbidity, mortality, and complication rates. Promising research in artificial intelligence (AI) has garnered immense interest in recent years. Due to its widespread application in diagnostic imaging with a high degree of sensitivity and specificity, AI has the potential of becoming a vital tool in differentiating GP from pancreatic malignancies, thereby preventing a missed or delayed diagnosis. In this article, we provide a comprehensive review of GP, covering the etiology, pathogenesis, clinical presentation, radiological and endoscopic evaluation, management strategies, and future directions. This article also aims to increase awareness about this lesser known and often‐misdiagnosed clinical entity amongst clinicians to ultimately improve patient outcomes. [ABSTRACT FROM AUTHOR]

Pancreas divisum (PD) represents a prevalent congenital pancreatic variant, typically arising from the failure of fusion between the ventral and dorsal pancreatic ducts. This condition is frequently associated with recurrent pancreatitis. We herein present a case involving an incomplete PD diagnosis following the identification of a refractory postoperative pancreatic fistula (POPF) after laparoscopic distal pancreatectomy (DP) for pancreatic cancer. A 74-year-old female patient, who had undergone laparoscopic DP for pancreatic cancer, developed a POPF accompanied by intraabdominal bleeding, necessitating urgent intervention radiology to avert life-threatening complications. Following this, intraabdominal drainage was performed through an intraoperative drainage root. Subsequent fistulography and endoscopic retrograde pancreatography unveiled the presence of an incomplete PD for the first time. Consequently, a stent was placed in the Santorini duct. However, the volume of pancreatic juice from the intraabdominal drainage tube exhibited no reduction. Despite repeated attempts to access the pancreatic duct via a guidewire through the drainage tube, these endeavors proved futile. Paradoxically, the removal of the external drainage tube led to a recurrence of intraabdominal abscess formation. Consequently, reinsertion of the drainage tube became imperative. Consideration was given to draining the abscess under endoscopic ultrasonography and performing pancreatic duct drainage. However, due to the diminution of the abscess cavity through the external fistula drainage procedure, coupled with the absence of pancreatic duct dilation and its tortuous course, it was deemed a formidable challenge. the patient necessitated a lifestyle adaptation with a permanently placed percutaneous drainage tube. [ABSTRACT FROM AUTHOR]

Pancreatic divisum (PD) is the most common congenital abnormality of the pancreatic duct. It can be associated with complications such as pancreatitis or pancreatitis-like pain. This article provides cumulative data of 57 case reports of PD and highlights the statistical findings of the most common presentations, diagnostic methods, treatment options, and associated complications. The goal of the article was to provide a comprehensive picture of clinical and epidemiological methods of diagnosis and treatment of PD. Objectives: Pancreatic divisum (PD) is the second most common congenital abnormality of the pancreatic duct, which affects 2% to 3% of the population. Most of the population remains asymptomatic, but in people who present with symptoms, it can be a cause of anguish and should be recognized. The main goal of this article was to provide a comprehensive picture of clinical and epidemiological methods of diagnosis and treatment of PD. Methods: A total of 57 PD case reports were considered in this descriptive analysis with 51 case reports and case series published within the last 25 years. The search strategies include systemic searches using scholarly search engines such as Medscape, Scopus, Cochrane, and PubMed. Results: The 57 cases we studied have an average age of presentation of 42 years, with female sex (58%) predominance. Common presenting symptoms were abdominal pain (87.72%) and radiation to the back (21.6%). Eighty-one percent of the case studies reported pancreatitis, and 63.2% had recurrent pancreatitis. At presentation, laboratory values demonstrated increased amylase, lipase, and liver enzymes. PD was diagnosed using magnetic resonance cholangiopancreatography (28.1%), endoscopic retrograde cholangiopancreatography (57.9%), endoscopic ultrasound (7%), or computed tomography (5.3%) scan of the abdomen. Of significance, biliary duct dilation was found in 70.6% of patients diagnosed as having PD. Incidental masses were found in 66.7% of the patients. The most successful treatment was sphincterotomy with or without stents (47.6%), followed by pancreatoduodenectomy (19%) and pancreaticojejunostomy (10%). Conclusions: Physicians managing pancreatitis should add PD to their differential diagnoses because it will help improve patient outcomes and avoid unfavorable consequences. [ABSTRACT FROM AUTHOR]

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. 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. 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). 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. [ABSTRACT FROM AUTHOR]

Congenital pancreaticobiliary anomalies like pancreatic divisum (PD), choledochal cysts (CDC), anomalous pancreaticobiliary ductal union (APBDU) have been reported in 5.7% of patients undergoing magnetic resonance cholangiopancreatography. CDC’s are characterized by abnormal dilatations of the intrahepatic and/or extrahepatic portion of the biliary tree and can be complicated by cystolithiasis, cholangitis, pancreatitis and malignant transformation necessitating surgical managemnet. While CDCs are commonly associated with APBDU, combination of CDC with PD is rare and a potential surgical challenge. We report a case of recurrent cholangitis in a patient with CDC and coincidental classic PD and illustrate how preoperative identification, fastidious dissection technique to safeguard both the pancreatic ducts and simple intraoperative preemptive strategies may decrease consequences of distal stump blowout.

Abstract In clinical cases of pancreas divisum, endoscopic retrograde cholangiopancreatography often necessitates cannulation of the pancreatic duct through the minor papilla. Nevertheless, this procedure can be challenging because of the small size of the minor papilla and the difficulty in visualizing the ductal orifice. A new image‐enhanced endoscopy technique called texture and color enhancement imaging (TXI) has been developed, which enhances texture, brightness, and color compared with white‐light imaging, resulting in subtle differences in the surface mucosa. Herein, we describe the case of a 73‐year‐old man with pancreas divisum in whom TXI was useful in identifying the orifice of the minor papilla. He was referred to our hospital with repetitive acute exacerbation of chronic pancreatitis. Since contrast‐enhanced computed tomography revealed a pancreatic stone in the main pancreatic duct, endoscopic retrograde cholangoepancreatography was performed as a therapeutic intervention. Despite the initial difficulty in identifying the orifice of the minor papilla on white‐light imaging, TXI enhanced its visibility successfully, enabling dorsal pancreatic duct cannulation via the minor papilla. Subsequently, endoscopic pancreatic sphincterotomy was performed and a 6Fr plastic stent was placed. Post‐endoscopic therapy, the patient's abdominal pain was relieved. TXI was useful in identifying the minor papilla orifice and led to successful cannulation.

Pancreas divisum (PD) is the most common congenital variant of the pancreatic ductal system, in which only a few patients develop symptomatic disease. Overall, PD is an underrecognized cause of many cases of recurrent acute pancreatitis. The PD must be systematically suspected in case of multiple episodes of acute idiopathic pancreatitis when exhaustive etiological investigations are negative. We present a 37-year-old woman whom presented several previous pancreatic pains. She came to the emergency department for epigastric pain, accompanied by post-prandial dietary vomiting after a copious meal. Lipasemia was greater than 3 times normal (498 UI/L). An abdominal computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP) showed a PD. The patient improved after the initial management measures. An endoscopic cholangiopancreatography was planned after the resolution of the acute episode. Recurrent pancreatitis is defined as 2 or more episodes of distinct acute pancreatitis with more than 3 months between episodes. Patients with this condition are usually asymptomatic while 5% of patients develop acute pancreatitis or chronic pancreatitis. We can underline the interest of deepening the radiological and endoscopic investigations to make the diagnosis of PD and to propose an endoscopic or surgical treatment, in order to avoid recurrences.

This article discusses the management of multiple pancreatic ducts in a Whipple procedure, specifically focusing on a case of incomplete pancreatic divisum. The patient in the case study presented with obstructive jaundice and abdominal pain, and imaging revealed a mass in the head of the pancreas. During the procedure, two ducts were visualized at the transection mark, and it was determined that they were communicating. The smaller duct was ligated to preserve a larger channel for flow. The article highlights the challenges and considerations in managing multiple pancreatic ducts and suggests that surgical technique selection depends on the extent of resection and anatomical locations of the ducts. [Extracted from the article]

Abstract 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.

Abstract Background Pancreas divisum (PD), the most common pancreatic anomaly, is caused by the failure of pancreatic bud fusion in the embryo. Although most cases are asymptomatic, it can cause pancreatitis or epigastric pain. We report an unusual case of PD in an infant. Case presentation The patient was a 9-month-old girl with no pertinent medical history. She had suffered vomiting and diarrhea for 1 week before transfer to our hospital. Her general condition was poor, and abdominal distention was noted. Blood tests revealed microcytic anemia with normal chemical markers. The parents reported no episode of pancreatitis. Ultrasonography revealed massive ascites, which was later found to be bloody. Enhanced computed tomography and magnetic resonance imaging depicted a cystic lesion, approximately 2 cm in size, anterior to the second portion of the duodenum. During exploratory laparotomy, a pinhole was identified on the cyst wall, which was mistakenly identified as a duodenal perforation, and direct closure was performed. Postoperative levels of serum amylase and inflammation markers were elevated, and the amount of ascites increased, impairing oral feeding. The level of pancreatic enzymes in the ascites was high. Imaging studies were repeated, but the cause of pancreatic fistula was not identified. Conservative therapy, including administration of total parenteral nutrition, antibiotics, and octreotide, was initiated, but the situation did not improve. Three months after admission, endoscopic retrograde cholangiopancreatography showed a thick dorsal pancreatic duct communicating with a hypoplastic ventral duct, which was indicative of PD. Contrast medium leaking from the dorsal duct near the minor ampulla revealed the presence of a pseudocyst. Stenting via the minor papilla was impossible because the minor papilla was obstructed. Instead, a stent was inserted into the ventral pancreatic duct. Endoscopic transgastric drainage of the cyst was effective, and the patient was discharged, 7 months after admission. The patient is healthy, but the gastric stent needs to be replaced regularly. Conclusion In children, PD can manifest with pancreatic pseudocyst that causes pancreatic ascites, even in the absence of pancreatitis. This may be a previously unrecognized manifestation of PD in children, and clinicians need to be aware of it.

Embryological development of the pancreas is a complex phenomenon and, therefore, it can have multiple developmental aberrations. Fortunately, the majority of these pancreatic ductal anomalies are asymptomatic with no clinical relevance and are incidentally detected during diagnostic cross-sectional imaging or endoscopic retrograde cholangiopancreatography (ERCP) or autopsy. Occasionally, pancreatic duct anomalies can result in symptoms like abdominal pain or recurrent pancreatitis. Also, an accurate pre-operative diagnosis of ductal anomalies can prevent inadvertent duct injury during surgery. Conventionally, ERCP had been used for an accurate diagnosis of pancreatic duct anomalies. However, because it is invasive and associated with a risk of pancreatitis, it has been replaced with magnetic resonance cholangiopancreatography (MRCP). MRCP has demonstrated high sensitivity and specificity for the diagnosis of ductal anomalies, which can be further improved with the use of secretin-enhanced MRCP. Endoscopic ultrasound (EUS) is a new diagnostic and interventional tool in the armamentarium of endoscopists and has demonstrated promising results in the detection of pancreatic duct variations and anomalies. Along with the visualization of the course and configuration of the pancreatic duct, EUS can also visualize changes in the pancreatic parenchyma, thereby helping with an early diagnosis of any co-existent pancreatic disease. Absence of the stack sign and crossed duct sign are important EUS features to diagnose pancreas divisum. EUS can also help with the diagnosis of other congenital ductal anomalies like annular pancreas, ansa pancreatica, and anomalous pancreaticobiliary union, although the published experience is limited. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Environmental risk factors for pancreatic cancer include acute and chronic pancreatitis, obesity, and tobacco use. Differentiating a pancreatic neoplasm in a patient with pancreatitis can be challenging due to their similar presentations. A 57‐year‐old African American man with a history of congestive heart failure, pancreatitis, and incomplete pancreas divisum presented with an epigastric abdominal pain that radiated to his back. Imaging showed necrotizing pancreatitis, a developing splenic infarct, and a mass at the pancreas tail. The patient was discharged with pain medications and was recommended follow‐up imaging after resolution of his pancreatitis. He was readmitted to the emergency department 2 weeks later with recurrent acute abdominal pain. Computed tomography scan of abdomen and pelvis followed by magnetic resonance imaging and endoscopic ultrasound revealed an infiltrative pancreatic tail mass. Biopsy of the mass confirmed a locally advanced pancreatic tail adenocarcinoma. Chronic pancreatitis is associated with pancreatic cancer. Practitioners should be aware of the co‐existence of chronic pancreatitis and pancreatic cancer, and the initial steps to evaluate a malignancy in chronic pancreatitis. [ABSTRACT FROM AUTHOR]

The article discusses a case of a 47-year-old man with a rare neuroendocrine neoplasm in the minor papilla, diagnosed using endoscopic ultrasonography-guided fine-needle biopsy. The neoplasm was successfully resected through endoscopic papillectomy, with no complications and no recurrence within a year. The study highlights the effectiveness of endoscopic procedures for diagnosing and treating neoplasms in the minor papilla, emphasizing the importance of considering endoscopic papillectomy for smaller neoplasms without invasive features. [Extracted from the article]

The letter discusses the importance of considering various factors in assessing the durability of treatment effects for pancreatic duct stones in chronic pancreatitis. It highlights the need to exclude certain patient conditions, such as a history of pancreatic surgery or cancer, and adjust for the presence of pancreatic pseudocysts. Additionally, the study emphasizes the significance of recording the total number of ERCPs and ESWL sessions post-treatment to evaluate efficacy and safety accurately. Addressing these factors is crucial for drawing robust scientific conclusions in the treatment of pancreatic duct stones. [Extracted from the article]

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. [ABSTRACT FROM AUTHOR]

Background: Pancreas divisum (PD), the most common pancreatic anomaly, is caused by the failure of pancreatic bud fusion in the embryo. Although most cases are asymptomatic, it can cause pancreatitis or epigastric pain. We report an unusual case of PD in an infant. Case presentation: The patient was a 9-month-old girl with no pertinent medical history. She had suffered vomiting and diarrhea for 1 week before transfer to our hospital. Her general condition was poor, and abdominal distention was noted. Blood tests revealed microcytic anemia with normal chemical markers. The parents reported no episode of pancreatitis. Ultrasonography revealed massive ascites, which was later found to be bloody. Enhanced computed tomography and magnetic resonance imaging depicted a cystic lesion, approximately 2 cm in size, anterior to the second portion of the duodenum. During exploratory laparotomy, a pinhole was identified on the cyst wall, which was mistakenly identified as a duodenal perforation, and direct closure was performed. Postoperative levels of serum amylase and inflammation markers were elevated, and the amount of ascites increased, impairing oral feeding. The level of pancreatic enzymes in the ascites was high. Imaging studies were repeated, but the cause of pancreatic fistula was not identified. Conservative therapy, including administration of total parenteral nutrition, antibiotics, and octreotide, was initiated, but the situation did not improve. Three months after admission, endoscopic retrograde cholangiopancreatography showed a thick dorsal pancreatic duct communicating with a hypoplastic ventral duct, which was indicative of PD. Contrast medium leaking from the dorsal duct near the minor ampulla revealed the presence of a pseudocyst. Stenting via the minor papilla was impossible because the minor papilla was obstructed. Instead, a stent was inserted into the ventral pancreatic duct. Endoscopic transgastric drainage of the cyst was effective, and the patient was discharged, 7 months after admission. The patient is healthy, but the gastric stent needs to be replaced regularly. Conclusion: In children, PD can manifest with pancreatic pseudocyst that causes pancreatic ascites, even in the absence of pancreatitis. This may be a previously unrecognized manifestation of PD in children, and clinicians need to be aware of it. [ABSTRACT FROM AUTHOR]

Background: The use of pancreatic prostheses in children with acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) has evolved. The main established indication is the treatment of persistent abdominal pain. This study aimed to evaluate the efficacy of pancreatic stenting for refractory abdominal pain in pediatric patients with ARP and CP. Methods: We conducted a retrospective case series study. We included patients under 16 years of age diagnosed with ARP and CP in the study. Endoscopic retrograde cholangiopancreatography (ERCP) was performed with the insertion of one and later two pancreatic stents. We evaluated abdominal symptoms before and after treatment, number of changes, duration of treatment, and complications with follow-up at 24 months and after withdrawal. Results: Nine patients with ARP and CP were included in the study: six with undetermined etiology and three with pancreas divisum. The mean age was 12.4 years. Prosthesis placement relieved abdominal pain in 100% of cases, with 3.2 replacement sessions every 6.2 months for 27.4 months, and mild complications (15.7%). One patient experienced pain on removal of the prosthesis and required bypass surgery. Conclusion: Pancreatic stent placement in patients with refractory abdominal pain with ARP and CP proved to be effective and safe, providing medium-term symptom relief and minimal complications. [ABSTRACT FROM AUTHOR]

Objective: To determine the clinical spectrum and immediate outcome of pancreatic disorders in children attending National Institute of Child Health, Karachi, Pakistan. Study Design: Cross Sectional study. Setting: Inpatient and Outpatient Department of Pediatric Medicine, National Institute of Child Health, Karachi. Period: January 2022 to December 2022. Material & Methods: A total of 49 children of either gender aged between 2 to 15 years presenting with clinical features of pancreatic disorders were analyzed. At the time of enrollment, data about demographic and clinical characteristics were noted. Important laboratory investigations were sent to local institutional laboratory. Ultrasound and/or CT scan of abdomen of each child was performed and reported by consultant radiologist having 3 years of experience for confirmation of the diagnosis. Immediate outcome was noted after 15 days of treatment among all children. Outcomes were labeled in terms of improved, not improved or expired. Results: In a total of 49 children, 30 (61.2%) were male while the mean age was 8.46±3.40 years. Epigastric pain, vomiting and whole abdominal pain were the most frequent presenting complaints noted in 40 (81.6%), 35 (71.4%) and 32 (65.3%) children respectively. Atrophy, inflammation and edema, calcifications, pancreatic duct dilation and pancreatic divisum were the commonest radiological findings noted in 10 (20.4%), 21 (42.9%), 18 (36.7%), 13 (26.5%) and 2 (4.1%) children respectively. Chronic pancreatitis was found to be the most frequent pancreatic disorder in 29 (59.2%) patients and they were associated with low fecal elastase level while acute pancreatitis was noted in 20 (40.8%) cases. Improved outcomes were observed in 44 (89.8%) children while no mortality was reported. Conclusion: Majority of the children with pancreatic disorders were male and aged between 6 to 12 years. Chronic pancreatitis was the most common type of pancreatic disorder. Epigastric pain, vomiting and whole abdominal pain were the most frequent presenting complaints. Outcome was generally good and no mortality was noted. [ABSTRACT FROM AUTHOR]

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]

Key Clinical Message: Pancreas divisum (PD) can be one of the causes of unexplained chronic abdominal pain. In PD, the dominant duct drains the majority of the pancreas via the minor papilla, which can be conveyed in the imaging as crossing duct sign. We report a case a of 16‐year‐old man who presented with unexplained chronic abdominal pain. Contrast‐enhanced computed tomography and magnetic resonance cholangiopancreatography showed a bulky pancreas, but his pancreatic enzymes were normal. The crossing duct sign was prominent in imaging, which confirmed the diagnosis of PD. [ABSTRACT FROM AUTHOR]

Abstract Background Neuroendocrine tumors of the minor papilla are very rare, and only 20 cases have been reported in the literature. Neuroendocrine carcinoma of the minor papilla with pancreas divisum has not been reported previously, making this the first reported case. Neuroendocrine tumors of the minor papilla have been reported in association with pancreas divisum in about 50% of cases reported in the literature. We herein present our case of neuroendocrine carcinoma of the minor papilla with pancreas divisum in a 75-year-old male with a systematic literature review of the previous 20 reports of neuroendocrine tumors of the minor papilla. Case presentation A 75-year-old Asian man was referred to our hospital for evaluation of dilation of the main pancreatic duct noted on abdominal ultrasonography. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography showed a dilated dorsal pancreatic duct, which was not connected to the ventral pancreatic duct; however, it opened to the minor papilla, indicating pancreas divisum. The common bile duct had no communication with the pancreatic main duct and opened to the ampulla of Vater. A contrast-enhanced computed tomography scan showed a 12-mm hypervascular mass near the ampulla of Vater. Endoscopic ultrasonography showed a defined hypoechoic mass in the minor papilla with no invasion. The biopsies performed at the previous hospital found adenocarcinoma. The patient underwent a subtotal stomach-preserving pancreaticoduodenectomy. The pathological diagnosis was neuroendocrine carcinoma. At the 15-year follow-up visit, the patient was doing well with no evidence of tumor recurrence. Conclusion In our case, because the tumor was discovered during a medical check-up relatively early in the course of disease, the patient was doing well at the 15-year follow-up visit, with no evidence of tumor recurrence. Diagnosing a tumor of the minor papilla is very difficult because of the relatively small size and submucosal location. Carcinoids and endocrine cell micronests in the minor papilla occur more frequently than generally thought. It is very important to include neuroendocrine tumors of the minor papilla in the differential diagnosis of patients with recurrent pancreatitis or pancreatitis of unknown cause, especially for patients with pancreas divisum.

Chronic midgut malrotation is a rare condition found in the adult age that predisposes to severe complications. It derives from an incomplete rotation of the mesentery around the superior mesenteric artery during embryogenic development. This results in intestinal loops displacement and mesenteric malfixation. Nevertheless, other congenital abnormalities can be also associated, such as mesenteric cysts and biliopancreatic malformations. Imaging modalities employed in the evaluation of chronic midgut malrotation include contrast radiography, which permits to visualize the localization of the intestinal loops, and ultrasound, that can detect a twist of superior mesenteric vessels. Computed tomography is however considered the modality of choice, owing to its wide field of view and the rapid scan times. The role of magnetic resonance imaging in this field has been barely explored. In particular, magnetic resonance enterography has a consolidated role in the assessment of intestinal loops and allows detecting extra-intestinal findings as well. Moreover, the lack of radiation exposure makes this technique suitable for nonemergency cases, especially in young patients. This is the first description of simultaneous chronic midgut malrotation, mesenteric cyst and pancreas divisum discovered in a Crohn's disease patient. The performance of magnetic resonance enterography allowed to properly interpret this multifaceted clinical picture.

Key Clinical Message Pancreas divisum (PD) can be one of the causes of unexplained chronic abdominal pain. In PD, the dominant duct drains the majority of the pancreas via the minor papilla, which can be conveyed in the imaging as crossing duct sign. Abstract We report a case a of 16‐year‐old man who presented with unexplained chronic abdominal pain. Contrast‐enhanced computed tomography and magnetic resonance cholangiopancreatography showed a bulky pancreas, but his pancreatic enzymes were normal. The crossing duct sign was prominent in imaging, which confirmed the diagnosis of PD.

Teaching point: Wirsung duct duplication is a very rare condition that can lead to chronic pancreatitis.

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.

This document is a commentary on a study titled "Pancreatic Enzyme Use Reduces Pancreatitis Frequency in Children With Acute Recurrent or Chronic Pancreatitis: A Report From INSPPIRE." The authors of the commentary express interest in the study but suggest that certain points need further clarification. They propose that the study should include the usage of proton pump inhibitors (PPIs) as a risk factor analysis, consider the impact of endoscopic or surgical treatment on reducing additional acute pancreatitis episodes, analyze the duration of pancreatic enzyme therapy (PERT), and compare pain symptoms between the group receiving enzymes and the group not receiving enzymes. The authors disclose no conflicts of interest or competing interests. [Extracted from the article]

Key Clinical Message Invasive treatment might be taken into consideration at the time of initial acute pancreatitis in patients with pancreas divisum who do not complain of abdominal pain to prevent progression to chronic pancreatitis.

Background: Neuroendocrine tumors of the minor papilla are very rare, and only 20 cases have been reported in the literature. Neuroendocrine carcinoma of the minor papilla with pancreas divisum has not been reported previously, making this the first reported case. Neuroendocrine tumors of the minor papilla have been reported in association with pancreas divisum in about 50% of cases reported in the literature. We herein present our case of neuroendocrine carcinoma of the minor papilla with pancreas divisum in a 75-year-old male with a systematic literature review of the previous 20 reports of neuroendocrine tumors of the minor papilla. Case presentation: A 75-year-old Asian man was referred to our hospital for evaluation of dilation of the main pancreatic duct noted on abdominal ultrasonography. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography showed a dilated dorsal pancreatic duct, which was not connected to the ventral pancreatic duct; however, it opened to the minor papilla, indicating pancreas divisum. The common bile duct had no communication with the pancreatic main duct and opened to the ampulla of Vater. A contrast-enhanced computed tomography scan showed a 12-mm hypervascular mass near the ampulla of Vater. Endoscopic ultrasonography showed a defined hypoechoic mass in the minor papilla with no invasion. The biopsies performed at the previous hospital found adenocarcinoma. The patient underwent a subtotal stomach-preserving pancreaticoduodenectomy. The pathological diagnosis was neuroendocrine carcinoma. At the 15-year follow-up visit, the patient was doing well with no evidence of tumor recurrence. Conclusion: In our case, because the tumor was discovered during a medical check-up relatively early in the course of disease, the patient was doing well at the 15-year follow-up visit, with no evidence of tumor recurrence. Diagnosing a tumor of the minor papilla is very difficult because of the relatively small size and submucosal location. Carcinoids and endocrine cell micronests in the minor papilla occur more frequently than generally thought. It is very important to include neuroendocrine tumors of the minor papilla in the differential diagnosis of patients with recurrent pancreatitis or pancreatitis of unknown cause, especially for patients with pancreas divisum. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. 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. 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. 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. [ABSTRACT FROM AUTHOR]

When the etiology of pancreatitis cannot be determined despite sufficient investigation, recurrence and progression to chronic pancreatitis often involve genetic mutations. Herein, we describe a case of recurrent pancreatitis with the IVS3+2T>C mutation in the serine protease inhibitor Kazal type 1 (SPINK1) gene that progressed to chronic pancreatitis in only 3 years. A 35-year-old man was referred to our hospital, where he was diagnosed with mild pancreatitis and was treated conservatively. However, the patient experienced recurrent episodes of pancreatitis, which progressed to become chronic pancreatitis with a pancreatic calcification 1 year later. After 3 years, the patient developed pancreatic duct stenosis and required a pancreatic duct stent placement. Regarding the cause of chronic pancreatitis, alcohol abuse was ruled out based on history taking. Considering the course of treatment, autoimmune pancreatitis and obstructive pancreatitis, such as pancreatic divisum, were also ruled out. Finally, a germline genetic test was performed to determine the etiology of pancreatitis, which revealed the IVS3+2T>C mutation in SPINK1. This case shows the importance of genetic testing in patients with idiopathic pancreatitis to determine their etiology and is a rare incident that can report the progression of the disease from acute to chronic pancreatitis. [ABSTRACT FROM AUTHOR]

Embryological development of the pancreas is a complex phenomenon and, therefore, it can have multiple developmental aberrations. Fortunately, the majority of these pancreatic ductal anomalies are asymptomatic with no clinical relevance and are incidentally detected during diagnostic cross-sectional imaging or endoscopic retrograde cholangiopancreatography (ERCP) or autopsy. Occasionally, pancreatic duct anomalies can result in symptoms like abdominal pain or recurrent pancreatitis. Also, an accurate pre-operative diagnosis of ductal anomalies can prevent inadvertent duct injury during surgery. Conventionally, ERCP had been used for an accurate diagnosis of pancreatic duct anomalies. However, because it is invasive and associated with a risk of pancreatitis, it has been replaced with magnetic resonance cholangiopancreatography (MRCP). MRCP has demonstrated high sensitivity and specificity for the diagnosis of ductal anomalies, which can be further improved with the use of secretin-enhanced MRCP. Endoscopic ultrasound (EUS) is a new diagnostic and interventional tool in the armamentarium of endoscopists and has demonstrated promising results in the detection of pancreatic duct variations and anomalies. Along with the visualization of the course and configuration of the pancreatic duct, EUS can also visualize changes in the pancreatic parenchyma, thereby helping with an early diagnosis of any co-existent pancreatic disease. Absence of the stack sign and crossed duct sign are important EUS features to diagnose pancreas divisum. EUS can also help with the diagnosis of other congenital ductal anomalies like annular pancreas, ansa pancreatica, and anomalous pancreaticobiliary union, although the published experience is limited.

Abstract 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.

IntroductionStenting of the pancreas is a challenging task for the interventional gastroenterologist. The indications for pancreatic stent implantation are either prophylactic or therapeutic. We give an overview of currently available literature and techniques for the respective indications of pancreatic stent placement.MethodsA structured literature research was conducted (Pubmed.gov) primarily using the following key words: interventional endoscopy, pancreatic stenting, post-ERCP pancreatitis, pancreatic Q8 fistulae, pancreas divisum.ResultsProphylactic stent implantation aims to prevent PEP by using thin (3-5 Fr) and short (3-5 cm) designated pancreatic stents at least in high-risk patients. Therapeutic stent placement is intended to restore the proper flow of pancreatic secretion with stenoses, leaks, fistulas or anatomical malformation of the pancreatic duct. Depending on the etiology, plastic stents or SEMSs are used. Another field of pancreatic stenting represents EUS-guided puncture with stent implantation as an alternative access to the main pancreatic duct when transpapillary access is impossible. In addition to the implantation of plastic stents, which achieve good results, LAMS implantation can be discussed as an alternative access route.DiscussionThe field of pancreatic stenting is complex and belongs in the hands of experienced endoscopists in specialized institutions. This can ensure that the patient receives the optimal treatment with the best possible outcome.

Pancreatic duct stenting using endoscopy or surgery is widely used for the management of benign and malignant pancreatic diseases. Endoscopic pancreatic stents are mainly used to relieve pain caused by chronic pancreatitis and pancreas divisum, and to treat pancreatic duct disruption and stenotic pancreaticointestinal anastomosis after surgery. They are also used to prevent postendoscopic retrograde cholangiopancreatography pancreatitis and postoperative pancreatic fistula, treat pancreatic cancer, and locate radiolucent stones. Recent advances in endoscopic techniques, such as endoscopic ultrasonography and balloon enteroscopy, and newly designed stents have broadened the indications for pancreatic duct stenting. In this review we outlined the types, insertion procedures, efficacy, and complications of endoscopic pancreatic duct stent placement, and summarized the applications of pancreatic duct stents in surgery. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objectives: We aimed to describe the clinical characteristics of pediatric patients with pancreatitis caused by pancreaticobiliary malformation and to evaluate the efficacy and safety of magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP) in the diagnosis of the disease. Methods: Medical records of pediatric patients with pancreatitis related to pancreaticobiliary malformation diagnosed by ERCP and treated in our hospital between April 2008 and December 2020 were retrospectively reviewed. Clinical manifestations, laboratory indicators, genetic testing results and imaging findings including MRCP were collected. Results: Of the 148 patients with pancreaticobiliary malformation‐related pancreatitis, 90 (60.8%) had pancreaticobiliary maljunction (PBM), 52 (35.1%) had pancreatic divisum (PD), and six (4.1%) had annular pancreas (AnnP). Compared with the PD group, patients with PBM were younger (P < 0.001), and were more likely to have jaundice (P < 0.001) and fever (P = 0.034). Genetic mutation was found in 51.6% of patients with PD, 50.0% with AnnP, and 15.0% with PBM. Diagnostic rate of PBM, PD, and AnnP using MRCP was 46.7%, 15.4%, and 100%, respectively. In total, 87.8% of patients had symptomatic improvement after endoscopic treatment. ERCP‐related complications were observed in 28 out of the 260 procedures, including post‐ERCP pancreatitis (7.7%), infection (2.3%), and gastrointestinal bleeding (0.8%). Conclusions: PBM should be considered when jaundice and fever occur in pediatric patients. Genetic testing is recommended for those with PD and AnnP. The role of MRCP is limited in identifying pancreaticobiliary malformation in children. ERCP is effective and safe for the diagnosis and treatment for pediatric pancreatitis caused by pancreaticobiliary malformation. [ABSTRACT FROM AUTHOR]