The Epidemiology and Clinical Presentation of Pancreatic Divisum: A Case Series of 57 Case Reports.

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.

Anatomical pancreatic variants in intraductal papillary mucinous neoplasm patients: a cross-sectional study.

BACKGROUND: No previous studies have examined the possible relationship between intraductal papillary mucinous neoplasm (IPMN) and the developmental ductal variations of the pancreas, such as an ansa pancreatica and a meandering main pancreatic duct (MMPD). METHODS: This retrospective cross-sectional study enrolled 214 patients, 108 with IPMN disease and 106 subjects from a community at the tertiary care unit. The main pancreatic duct (MPD) was evaluated in the head of the pancreas by its course, which were non-MMPD: descending, vertical, and sigmoid, or MMPD including loop types, reverse-Z subtypes, and an N-shape, which was identified for the first time in this study. IPMN patients were also evaluated for worrisome features (WF) or high-risk stigmata (HRS), and the extent of IPMN cysts. RESULTS: Among IPMN patients, 18.4% had MMPD, which we observed in only 3.0% of the control group (P < 0.001). Patients with MMPD were more likely to belong to the IPMN group compared with non-MMPD patients [odds ratio (OR) 6.4, 95% confidence interval (CI) 2.2-24.9]. Compared with a descending shape MPD, IPMN patients with an N-shaped MPD were more likely to have a cystic mural nodule (OR 5.9, 95% CI 1.02-36.0). The presence of ansa pancreatica associated with more extent IPMN disease (OR 12.8, 95% CI 2.6-127.7). CONCLUSIONS: IPMN patients exhibited an MMPD more often than control patients. Ansa pancreatica associated with multiple cysts. Furthermore, an N-shape in IPMN patients associated with cystic mural nodules, suggesting that this shape serves as a risk factor for more severe IPMN.

Transabdominal Ultrasound Evaluation of Pancreaticobiliary Maljunction in Children.

ABSTRACT: The development of high-frequency ultrasound made the diagnosis of pancreaticobiliary maljunction (PBM) possible. However, no study has been performed to clarify the sensitivity and specificity of transabdominal ultrasound (TAUS) in the diagnosis of PBM. The purpose of this study was to evaluate the accuracy of TAUS in the diagnosis of pediatric PBM and to assess factors that may influence the accuracy of ultrasound. This was a prospective study and 43 patients with suspected PBM were enrolled. All of these patients underwent TAUS examination to detect the pancreaticobiliary ductal union. Final diagnoses were determined by endoscopic retrograde cholangiopancreatography or intraoperative cholangiography. Sensitivity and specificity were calculated. Fisher exact test was used to analyze the difference of sonographic features between false-negative group and true-positive group. Transabdominal ultrasound demonstrated 77.4% (95% confidence interval, 58.5%-89.7%) sensitivity and 100% (95% confidence interval, 69.9%-100%) specificity for PMB diagnosis. In the false-negative group, infant patients (71.4% vs 16.7%, P = 0.012), cystic dilatation of the common bile duct (CBD) (71.4% vs 16.7%, P = 0.012), and stenosis of the distal CBD (71.4% vs 16.7%, P = 0.012) were more frequently observed than in the true-positive group. On the other hand, the true-positive group showed a higher incidence of protein plugs than the false-negative group (62.5% vs 0%, P = 0.007). Transabdominal ultrasound may serve as a potential alternative detection modality for pediatric patients with suspected PBM. Nondetection of the anomaly may be attributed to factors, such as younger age, cystic dilatation of the CBD, and stenosis of the distal CBD.

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.

Relapsing pancreatitis caused by a duodenal duplication cyst communicating to an aberrant pancreatic duct in a girl.

Wang and colleagues report a rare anatomic anomaly consisting of an accessory pancreatic lobe which originated from the main pancreatic gland with duodenal duplication in a girl with relapsing pancreatitis. The anomaly was visualized on magnetic resonance cholangiopancreatography before being confirmed on endoscopic retrograde cholangiopancreatography and surgery.

Variaciones de la configuración ductal pancreática: factor predisponente de pancreatitis recurrente / Variations of pancreatic ductal configuration: predisposing factor of recurrent pancreatitis

El páncreas es un órgano de origen endodérmico, que se desarrolla de dos esbozos intestinales separados, uno dorsal y otro ventral,a partir de la cuarta o quinta semana de gestación. El páncreas se origina en el intestino anterior en la parte correspondiente a lafutura segunda porción duodenal. Allí se originan dos brotes: uno posterior o dorsal que aparece a principios de la cuarta semanay crece rápidamente en el mesenterio dorsal y el otro brote es anterior o ventral. Este último da origen a dos brotes, uno origina unaparte del páncreas y otro a la vía biliar e hígado. Existen diferentes variantes anatómicas, derivadas de este desarrollo embrionario;su conocimiento es de vital importancia en pacientes con persistencia de dolor abdominal y episodios de pancreatitis recurrente.El objetivo de este artículo es una revisión de las variantes anatómicas del conducto pancreático que pueden manifestarse como pancreatitis idiopática recurrente. (AU)

The pancreas is an organ of endodermal origin, which develops from two separate intestinal sketches, one dorsal and one ventral,from the fourth or fifth week of gestation. The pancreas originates in the anterior intestine in the part corresponding to the futuresecond duodenal portion. There two shoots originate: one posterior or dorsal that appears at the beginning of the fourth week andgrows rapidly in the dorsal mesentery and the other outbreak is anterior or ventral. The latter gives rise to two outbreaks, oneoriginates a part of the pancreas and another to the bile duct and liver. There are different anatomical variants, derived from thisembryonic development; their knowledge is of vital importance in patients with persistent abdominal pain and episodes of recurrentpancreatitis. The objective of this article is a review of the anatomical variants of the pancreatic duct that can manifest as recurrent idiopathic pancreatitis. (AU)

Abnormal Intestinal Anatomy in Late-stage Human Fetuses: Three Case Series.

OBJECTIVE: We reported three cases of fetuses with abnormal intestinal anatomy found during our recent study of the transverse mesocolon using 20 late-stage fetuses. CASES: The first case (CRL: 328 mm) appeared to have a duodenum and transverse colon trapped in Winslow's foramen (foramen epiploicum) and the duodenum superior portion elongated rightward. The second case (CRL: 264 mm) had a transverse colon inserted deeply into a space between the right kidney and duodenum. The third case (CRL: 276 mm) had a descending colon that ran inferiorly through a deep space between the left kidney and duodenum. Each case had a greater omentum that was shifted leftward, but this is usual. These 3 abnormalities were not evident in the anterior view during dissection of the liver, stomach, jejunum, and ileum. With underdeveloped pancreatic ducts due to unknown reason other than the internal hernia, the first case seemed to be fatal after birth. However, the second and third cases could have recovered after birth because there was no evidence of definite malrotation and because of loose attachments of the intestines to surrounding structures. CONCLUSIONS: The intestinal morphologies described here could cause some sort of symptoms, such as abdominal pain, whose cause might be difficult to determine.

Ansa pancreática: desafio no diagnóstico etiológico em paciente jovem com pancreatite grave / Ansa pancreatica: etiological diagnosis challenge in a young patient with severe pancreatitis

A ansa pancreática é uma variação anatômica rara dos ductos pancreáticos. Consiste numa comunicação entre o ducto pancreático principal (Wirsung) e o ducto pancreático acessório (Santorini). Recentemente, estudos têm demonstrado estar essa variação anatômica implicada como fator predisponente e significativamente associada a episódios recorrentes de pancreatite aguda. A pancreatite é uma entidade clínica pouco frequente na infância. Diferente dos adultos, as causas mais comuns incluem infecções virais, por ascaris, medicamentosas, traumas e anomalias estruturais. O objetivo deste estudo foi relatar um caso de pancreatite aguda grave não alcoólica e não biliar, em um paciente jovem de 15 anos, em cuja propedêutica imagenológica evidenciou-se alça, comunicando com os ductos pancreáticos ventral e dorsal, compatível com ansa pancreática.

Ansa pancreatica is a rare anatomical variation of the pancreatic ducts. It consists of communication between the main pancreatic duct (Wirsung) and the accessory pancreatic duct (Santorini). Recently, studies have shown that this anatomical variation is implicated as a predisposing factor and significantly associated with recurrent episodes of acute pancreatitis. Pancreatitis is a rare clinical entity in childhood. Different from that in the adults, the most common causes include viral and ascaris infections, drugs, traumas, and structural abnormalities. The objective of this study was to report a case of a severe non-alcoholic and non-biliary acute pancreatitis in a 15-year-old patient, whose propedeutic imaging showed a loop communicating with the ventral and dorsal pancreatic ducts, consistent with ansa pancreatica.

Bridging the Divisum: A Rare Case of Pancreaticopleural Fistula in the Setting of Complete Pancreatic Divisum Treated Endoscopically.

A pancreaticopleural fistula (PPF) is a rare complication of chronic pancreatitis that occurs either due to a pancreatic duct disruption or a pseudocyst extension. A pancreatic divisum, on the other hand, is a common anatomic variant of the pancreas that is rarely symptomatic. We describe a case of recurrent pleural effusion in a patient with a history of chronic pancreatitis. Investigations revealed the presence of a PPF and a concomitant complete pancreatic divisum. There was resolution of the pleural effusion on endoscopic therapy. This is the fourth reported case of a PPF in the setting of complete pancreatic divisum and the first reported case in a middle-aged female.

Magnetic resonance cholangiopancreatography identification of pancreaticobiliary maljunction in the colombian pediatric population. / Unión biliopancreática anómala por colangiorresonancia en población pediátrica colombiana.

OVERVIEW: Pancreaticobiliary maljunction (PBM) is a congenital malformation characterized by a long common pancreaticobiliary channel which causes sphincter of Oddi malfunction. In children, it is typically diagnosed using magnetic resonance cholangiopancreatography (MRCP). It is associated with congenital biliary dilatation, pancreatitis, and gallbladder and bile duct tumors at adulthood. Studies in the western population are rare. Given its morbidity rate, it should be searched for in the western pediatric population. The objective of this study was to look for and identify the presence of pancreaticobiliary maljunction through MRCP in pediatric patients with biliary or pancreatic disease, as well as to find out other associated factors. METHODS: MRCP was used to measure common channel length, pancreatic duct length, and bile duct diameter in 41 pediatric patients with biliary or pancreatic disease. RESULTS: The common channel could only be measured in 17.6% of cases, 50% of which were >8 mm long. All patients were female and had congenital biliary dilatation. No age-related differences were found in terms of bile duct length. CONCLUSIONS: PBM is present in the western pediatric population, but prevalence and morbidity are unknown. Larger studies are required to identify morbidity and mortality, as well as prevalence among patients.

OBJETIVO: La unión biliopancreática anómala (UBPA) es una malformación congénita caracterizada por un canal común pancreatobiliar largo que impide el adecuado funcionamiento del esfínter de Oddi. Su diagnóstico en niños se realiza comúnmente mediante colangiopancreatografía por resonancia magnética (CPRM). Se asocia a dilatación biliar congénita, pancreatitis y tumores de la vesícula y la vía biliar en la edad adulta. Los estudios en población occidental son escasos; debido a su morbilidad resulta de relevancia la búsqueda en población pediátrica occidental. Este estudio pretende buscar e identificar la presencia de unión biliopancreática anómala mediante CPRM de pacientes pediátricos con enfermedad de la vía biliar o pancreática, al igual que identificar otros factores asociados. METODOS: Se midió por CPRM la longitud del canal común, el conducto pancreático y el diámetro de la vía biliar de 41 pacientes pediátricos con patología biliar o pancreática. RESULTADOS: El canal común solo pudo ser medido en el 17,6% de los casos, de los cuales el 50% tuvo una longitud >8 mm, siendo todos ellos pacientes femeninos con dilatación biliar congénita; no se encontraron diferencias en la longitud de la vía biliar relacionado con la edad. CONCLUSIONES: La UBPA es una malformación que se encuentra presente en población pediátrica occidental con prevalencia y morbilidad desconocida; se requieren estudios a mayor escala para identificar morbimortalidad y prevalencia de pacientes con esta malformación.

Unión biliopancreática anómala por colangiorresonancia en población pediátrica colombiana / Magnetic resonance cholangiopancreatography identification of pancreaticobiliary maljunction in the colombian pediatric population

OBJETIVO: La unión biliopancreática anómala (UBPA) es una malformación congénita caracterizada por un canal común pancreatobiliar largo que impide el adecuado funcionamiento del esfínter de Oddi. Su diagnóstico en niños se realiza comúnmente mediante colangiopancreatografía por resonancia magnética (CPRM). Se asocia a dilatación biliar congénita, pancreatitis y tumores de la vesícula y la vía biliar en la edad adulta. Los estudios en población occidental son escasos; debido a su morbilidad, resulta de relevancia la búsqueda en población pediátrica occidental. Este estudio pretende buscar e identificar la presencia de unión biliopancreática anómala mediante CPRM de pacientes pediátricos con enfermedad de la vía biliar o pancreática, al igual que identificar otros factores asociados. MÉTODOS: Se midió por CPRM la longitud del canal común, el conducto pancreático y el diámetro de la vía biliar de 41 pacientes pediátricos con patología biliar o pancreática. RESULTADOS: El canal común solo pudo ser medido en el 17,6% de los casos, de los cuales el 50% tuvo una longitud >8 mm, siendo todos ellos pacientes femeninos con dilatación biliar congénita; no se encontraron diferencias en la longitud de la vía biliar relacionada con la edad. CONCLUSIONES: La UBPA es una malformación que se encuentra presente en población pediátrica occidental con prevalencia y morbilidad desconocida; se requieren estudios a mayor escala para identificar morbimortalidad y prevalencia de pacientes con esta malformación

OVERVIEW: Pancreaticobiliary maljunction (PBM) is a congenital malformation characterized by a long common pancreaticobiliary channel which causes sphincter of Oddi malfunction. In children, it is typically diagnosed using magnetic resonance cholangiopancreatography (MRCP). It is associated with congenital biliary dilatation, pancreatitis, and gallbladder and bile duct tumors at adulthood. Studies in the western population are rare. Given its morbidity rate, it should be searched for in the western pediatric population. The objective of this study was to look for and identify the presence of pancreaticobiliary maljunction through MRCP in pediatric patients with biliary or pancreatic disease, as well as to find out other associated factors. METHODS: MRCP was used to measure common channel length, pancreatic duct length, and bile duct diameter in 41 pediatric patients with biliary or pancreatic disease. RESULTS: The common channel could only be measured in 17.6% of cases, 50% of which were > 8 mm long. All patients were female and had congenital biliary dilatation. No age-related differences were found in terms of bile duct length. CONCLUSIONS: PBM is present in the western pediatric population, but prevalence and morbidity are unknown. Larger studies are required to identify morbidity and mortality, as well as prevalence among patients

Focal Parenchymal Atrophy and Fat Replacement Are Clues for Early Diagnosis of Pancreatic Cancer with Abnormalities of the Main Pancreatic Duct.

Pancreatic cancer is one of the most dangerous solid tumors, but its early diagnosis is difficult. The abnormality of the main pancreatic duct (MPD), such as a single localized stricture and upstream dilatation, might be useful in the early detection of pancreatic cancer. However, these findings are often observed in benign inflammatory cases. This study aimed to clarify whether early pancreatic cancer presenting MPD abnormalities has characteristic features different from those of benign cases. This is a single-center, retrospective study. We analyzed 20 patients who underwent pancreatectomy presenting with a single, localized MPD stricture without identifiable masses on imaging: 10 patients with pancreatic ductal adenocarcinoma (cancer group; 6 with stage 0 and 4 with stage I) and 10 patients with benign strictures (benign group; 8 with inflammation and 2 with low-grade pancreatic intraepithelial neoplasms). Pancreatectomy was performed in these benign cases because high-grade intraepithelial neoplasm was suspected. Although the proportion of patients with diabetes mellitus tended to be higher in the cancer group (6/10) than that in the benign group (1/10) (P = 0.058), other clinical characteristics were not different between the groups. Preoperative cytological malignancies were detected in four patients in the cancer group (4/10) but not in the benign group (P = 0.09). Focal parenchymal atrophy and fat replacement were more frequently detected on computed tomography in the cancer group (7/10) than in the benign group (1/10) (P = 0.02). In conclusion, focal parenchymal atrophy and fat replacement may provide clues for the early diagnosis of pancreatic cancer.

Clinical importance of main pancreatic duct variants and possible correlation with pancreatic diseases.

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

Trifidum anomaly of the main pancreatic duct.

BACKGROUND: Embryology of the human pancreas is very complex and any alteration in its development may lead to congenital biliopancreatic malformations and anomalies not thoroughly studied in literature. We here report a case of trifurcation of the Wirsung duct, avery unusual variant of the main pancreatic duct. METHOD: An 80- year-old woman underwent a magnetic resonance imaging (MRI) of the abdomen and a magnetic resonance cholangiopancreatography (MRCP) to characterize a hypoechoic lesion of the pancreas detected with ultrasonography. RESULTS: MRI and MRCP showed a 24-mm multicystic lesion communicating with a prominent main pancreatic duct, consistent with an intraductal papillary mucinous neoplasm, as well as an ansa pancreatica. Moreover a bifidity of the distal pancreatic duct and a further accessory duct of the body of the pancreas draining into the main pancreatic duct were identified. The pancreatic tail presented normal size and morphology on axial imaging. This anomaly, not reported yet in the literature, can be categorized as a number of a duplication anomaly, in which the main pancreatic duct is trifurcated along its length. CONCLUSION: Congenital anomalies of the pancreas and pancreatic duct are rare but not uncommonly detected on diagnostic imaging. MRI and MRCP are the non-invasive imaging modalities of choice for diagnosing congenital anomalies of the pancreas and the pancreatic duct.

Endoscopic retrograde cholangiopancreatography in children with symptomatic pancreaticobiliary maljunction: A retrospective multicenter study.

BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP) has been widely used in pediatric patients with cholangiopancreatic diseases. AIM: To evaluate the efficacy, safety, and long-term follow-up results of ERCP in symptomatic pancreaticobiliary maljunction (PBM). METHODS: A multicenter, retrospective study was conducted on 75 pediatric patients who were diagnosed with PBM and underwent therapeutic ERCP at three endoscopy centers between January 2008 and March 2019. They were divided into four PBM groups based on the fluoroscopy in ERCP. Their clinical characteristics, specific ERCP procedures, adverse events, and long-term follow-up results were retrospectively reviewed. RESULTS: Totally, 112 ERCPs were performed on the 75 children with symptomatic PBM. Clinical manifestations included abdominal pain (62/75, 82.7%), vomiting (35/75, 46.7%), acholic stool (4/75, 5.3%), fever (3/75, 4.0%), acute pancreatitis (47/75, 62.7%), hyperbilirubinemia (13/75, 17.3%), and elevated liver enzymes (22/75, 29.3%). ERCP interventions included endoscopic sphincterotomy, endoscopic retrograde biliary or pancreatic drainage, stone extraction, etc. Procedure-related complications were observed in 12 patients and included post-ERCP pancreatitis (9/75, 12.0%), gastrointestinal bleeding (1/75, 1.3%), and infection (2/75, 2.7%). During a mean follow-up period of 46 mo (range: 2 to 134 mo), ERCP therapy alleviated the biliary obstruction and reduced the incidence of pancreatitis. The overall effective rate of ERCP therapy was 82.4%; seven patients (9.3%) were lost to follow-up, eight (11.8%) re-experienced pancreatitis, and eleven (16.2%) underwent radical surgery, known as prophylactic excision of the extrahepatic bile duct and hepaticojejunostomy. CONCLUSION: ERCP is a safe and effective treatment option to relieve biliary or pancreatic obstruction in symptomatic PBM, with the characteristics of minor trauma, fewer complications, and repeatability.