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.

Gene expression profiling reveals upregulated FUT1 and MYBPC1 in children with pancreaticobiliary maljunction

Pancreaticobiliary maljunction (PBM) is associated with high risk of epithelial atypical growth and malignant transformation of the bile duct or gallbladder. However, overall changes in genetic expression have not been examined in children with PBM. Genome-wide expression was analyzed using peripheral blood samples from 10 children with PBM and 15 pediatric controls. Differentially expressed genes (DEGs) were identified using microarray. Bioinformatics analysis was conducted using Gene Ontology and KEGG analyses. The top 5 in the up-regulated genes in PBM were verified with qRT-PCR. Receiver operator characteristic curve analysis was conducted to evaluate the predictive accuracy of selected genes for PBM. The microarray experiments identified a total of 876 DEGs in PBM, among which 530 were up-regulated and the remaining 346 were down-regulated. Verification of the top 5 up-regulated genes (TYMS, MYBPC1, FUT1, XAGE2, and GREB1L) by qRT-PCR confirmed the up-regulation of MYBPC1 and FUT1. Receiver operating characteristic curve analysis suggested that FUT1 and MYBPC1 up-regulation could be used to predict PBM, with the area under the curve of 0.873 (95%CI=0.735−1.000) and 0.960 (95%CI=0.891−1.000), respectively. FUT1 and MYBPC1 were up-regulated in children with PBM, and could be used as potential biomarkers for PBM.

Recurrent acute pancreatitis caused by pancreatic biliary maljunction due to long common channel / Pancreatitis aguda recurrente causada por deficiente unión pancreático-biliar debido a un colédoco largo

The pancreatic biliary maljunction is a rare anomaly that affects mainly females, defined as an anatomical maljunction of the pancreatic duct and the biliary duct confluence, and may be a rare cause of recurrent acute pancreatitis. In order to early diagnosis and prompt treatment, ERCP has an important role in it

La Malformación de la unión biliopancreática es una afección rara y ocurre más en mujeres jóvenes. Es una causa de pancreatitis aguda de causa no conocida. CPRE es una herramienta eficiente para el diagnóstico y también para ser de la terapéutica

Congenital Variants and Anomalies of the Pancreas and Pancreatic Duct: Imaging by Magnetic Resonance Cholangiopancreaticography and Multidetector Computed Tomography

Though congenital anomalies of the pancreas and pancreatic duct are relatively uncommon and they are often discovered as an incidental finding in asymptomatic patients, some of these anomalies may lead to various clinical symptoms such as recurrent abdominal pain, nausea and vomiting. Recognition of these anomalies is important because these anomalies may be a surgically correctable cause of recurrent pancreatitis or the cause of gastric outlet obstruction. An awareness of these anomalies may help in surgical planning and prevent inadvertent ductal injury. The purpose of this article is to review normal pancreatic embryology, the appearance of ductal anatomic variants and developmental anomalies of the pancreas, with emphasis on magnetic resonance cholangiopancreaticography and multidetector computed tomography.

Endoscopic Treatment of a Pediatric Patient with Acute Pancreatitis Caused by Anomalous Union of Pancreaticobiliary Duct Combined with Incomplete Pancreatic Divisum / 대한소화기학회지

The most common causes of acute pancreatitis are microlithiasis and alcohol. In pediatrics, anomalies in pancreaticobiliary system should be considered as possible causes. Among many anomalies, pancreas divisum associated with anomalous pancreaticobiliary ductal union (APBDU) is very rare. APBDU is associated with acute pancreatitis, choledochal cyst, and gallbladder cancer. Pancreas divisum is also a well known cause of acute recurrent pancreatitis. In adult cases with such conditions, the role of endoscopic management including sphincterotomy or stenting through the Santorini duct is well documented. However, it is still controversial to perform endoscopic retrograde cholangiopancreatography in pediatrics. Herein, we experienced a case of 4 year 7 month old female patient suffered from recurrent attacks of acute pancreatitis, which were caused by APBDU and incomplete pancreas divisum. She was treated by endoscopic sphincteretomy of both openings to the Santorini's and Wirsung's ducts. Thus, we report this interesting case with literature review.

A Case of Intraductal Papillary Mucinous Neoplasm Arising from Santorini's Duct in a Patient with Complete Type of Pancreas Divisum / 대한소화기학회지

There have been an increasing number of reports of intraductal papillary mucinous neoplasms (IPMNs) of the pancreas since its first report by Ohhasi et al. in 1982. Most IPMNs arise from Wirsung's duct or its branches, whereas IPMNs arising from Santorini's duct are rare. Pancreas divisum is a common congenital anatomical anomaly characterized by the lack of fusion of the ventral and dorsal parts of the pancreas during the eighth week of fetal development. Although clinical significance of pancreas divisum has been the subject of debate for many years, there seems to be little doubt that in certain patients there is a causal relation between pancreas divisum and pancreatitis. Also, it is occasionally accompanied by a pancreatic tumor. Herein, we report a case of IPMN arising from Santorini's duct in patient with complete type of pancreas divisum.

Imaging Features of Adult Choledochal Cysts: a Pictorial Review

Choledochal cysts are rare congenital anomalies which are principally diagnosed by disproportional dilatation of the extrahepatic bile ducts. In addition, choledochal cysts are believed to arise from the anomalous union of the common bile duct and pancreatic duct outside the duodenal wall which is also proximal to the sphincter of the Oddi mechanism. The various types of choledochal cysts have been classified on the basis of these anomalous unions (Komi classification) and their anatomical locations (Todani classification). The multidetector computed tomography with reformatted imaging, magnetic resonance cholangiopancreatography, and an endoscopic retrograde cholangiography represent the important techniques providing the anatomical resolution and detail required to properly diagnose and classify choledochal cysts and their associated abnormal features of the biliary tree, as well as their pancreaticobile duct union. This study describes the various imaging features of a choledochal cyst in adults according to the various types of anomalous unions of the pancreaticobile duct according to Komi's classification and anatomic location according to Todani's classification. Lastly, we also review and discuss the associated abnormal findings developed in biliary systems.

Litiasis del conducto de Wirsung / Lithiasis of the Wirsung`s duct

La litiasis del conducto de Wirsung es una enfermedad poco frecuente generalmente asociada a pancreatitis crónica. Se reporta el caso de una paciente que ingresa con cuadro de pancreatitis aguda donde la ecografía permitió realizar el diagnóstico de litiasis pancreática corroborado por tomografía computada. El ultrasonido demostró ser un método eficaz, fácilmente accesible y no invasivo para la detección de esta patología.

Variantes anatômicas e anomalias congênitas das vias biliares e pancreáticas: análise através da colangiopancreatografia por ressonância magnética / Anatomic variants and congenital abnormalities of the biliary tree seen on magnetic resonance cholangiopancreatography

Com o objetivo de ilustrar, de maneira sistematizada, as principais variantes anatômicas e anomalias congênitas observadas em exames de colangiopancreatografia por ressonância magnética (CPRM), realizamos estudo retrospectivo em exames de CPRM com as mais diversas indicações, procurando por variantes anatômicas e anomalias congênitas das vias biliares e pancraáticas. Todos os exames foram realizados em equipamento de alto campo (1,0 T ou 1,5 T), obtendo-se imagens no plano axial ponderadas em T1 (FFE) e T2 (TSE), e no plano coronal, com técnica de CPRM. Apresentamos variantes anatômicas relacionadas à posição da vesícula biliar, à distribuição e posição das vias biliares intra-hepáticas, do ducto cístico, ducto colédoco e ductos pancreáticos. Mostramos também exemplos dos principais tipos de dilatação cística congênita das vias biliares. Reconhecer as principais variantes anatômicas e anomalias congênitas das vias biliares e pancreáticas permite otimizar a eficácia diagnóstica da CPRM.

In order to illustrate the role of magnetic resonance cholangiopancreatography (MRCP) in the diagnosis of anatomic variants and congenital abnormalities of the biliary tree, we onducted a retrospective study of MRCP scans performed due to different indications to identify anatomic variants and congenital abnormalities of the pancreas and biliary tree. High field MR machines (1.0 T and 1.5 T) and 3D TSE sequences were used to obtain images in the axial and coronal planes with MIP reconstructions in all studies. MRCP showed low cystic duct insertion, medial cystic duct insertion, and a parallel course of the cystic and hepatic ducts as well as bile duct bifurcation abnormalities and aberrant right and left hapatica duct. We also present examples of the main types of congenital cystic dilatation of the bile ducts. The recognition of the main anatomic variants of the biliary tree and pancreatic ductshelps to optimize the diagnostic accurancy of MR cholangiopancreatography.

Malformación del sistema pancreático-biliar como causa de perforación espontánea de la vía biliar extrahepática / A pancreatobiliary malformation causing spontaneous perforation of the extrahepatic bile duct

La ascitis biliar usualmente resulta de la perforación espontánea o traumática del conducto biliar principal, usualmente en la unión con el conducto cístico. La obstrucción ductal distal, el reflujo hacia la vía biliar común, la debilidad o una muy localizada malformación mural de la pared del conducto común han sido propuestas como causas probables. Presentamos el caso de una niña de 11 meses de edad quien sufrió ascitis biliar como consecuencia de la ruptura del colédoco. La perforación probablemente se debió a una malformación de la unión pancreático-biliar. Se le derivó la bilis hacia el exterior mediante una sonda.

Biliaryascitis usually results from a spontaneous or traumatic perforation of the common bile duct, usually at the junction with the cystic duct. Distal duct obstruction, pancreatic fluid reflux up the common bile duct, congenital weakness or a localized mural malformation of the wall of the common duct have been proposed as possible causes. We present an 11-months-old infant girl who developed a biliary ascitis as a consequence of biliary rupture. Perforation was due to malformation of the pancreatico-biliary junction. An uneventful external derivation by tube was performed.

Clinical Features of Neuroendocrine Tumor of the Pancreas: Single Center Study / 대한소화기학회지

BACKGROUND/AIMS: Pancreatic neuroendocrine tumors (PNET) are rare and manifest as functioning tumor (FT) or non-functioning tumor (NFT). Although malignant changes are observed in some cases, its prognosis is better than pancreatic cancer. We evaluated clinicoradiologic features and prognosis of FT and NFT. In addition, we tried to find the predictive factors for the recurrence of NFT after resection. METHODS: Between October 1994 and June 2004, we retrospectively evaluated the clinicopathologic features and prognosis of 12 cases of FT and 31 cases of NFT diagnosed by surgical pathology at single medical center in Korea. RESULTS: PNET included 6 insulinomas, 4 gastrinomas, 1 glucagonoma, 1 somatostatinoma and 31 NFT. The major clinical manifestations were neuroglycopenic symptoms (100%) in insulinoma, abdominal ulcer symptoms (75%) in gastrinoma, dermatitis (100%) in glucagonoma, steatorrhea (100%) in somatostatinoma, and abdominal discomfort or pain (45%) in NFT. NFT was located more proximally when compared to FT (p=0.023). NFT showed more malignant (64.5%) behavior compared to FT (41.7%) despite the lack of statistical significance. Curative resections were done without postoperative death in 38 cases. Six cases of NFT (21.4%) and 1 case of FT (10%) recurred with an average of 26.5 months. In the recurrent NFT, the findings of diabetes mellitus (p=0.010), abnormal pancreatic duct (p=0.026), Whipple's operation (p=0.013) and tumor emboli (p=0.03) were more common than in non-recurrent NFT. CONCLUSIONS: FT and NFT showed different clinicoradiologic features. In addition, NFT should be monitored more carefully because of frequent recurrence.

A Case of Intraductal Papillary Mucinous Neoplasm of the Pancreas Arising from Pancreas Divisum without Ventral Pancreatic Duct of Wirsung / 대한소화기학회지

Most reported cases of intraductal papillary mucinous neoplasms (IPMNs) originate from Wirsung's duct or their branches. IPMNs arising from Santorini's duct and its branches have rarely been reported. Eight cases of IPMN arising from Santorini's duct have been published worldwide. However, these cases are associated with incomplete type of pancreas divisum. Recently, one report of IPMN with complete absence of Wirsung's duct has been reported. This patient was a 57-year-old woman who was admitted to the hospital due to progressive jaundice. On endoscopic retrograde cholangiopancreatography, there was a severely bulging ampulla of Vater and patulous minor papilla draining mucinous material and a cystic lesion communicating with the dilated Santorini's duct without any communication with Wirsung's duct. A pancreaticoduodenectomy was performed and the pathologic examination of resected specimen showed no evidence of Wirsung's duct, but an IPMN arising from Santorini's duct with peripancreatic lymph node metastasis. Herein, we report a case of invasive IPMN arising from pancreatic head without ventral pancreatic duct with a review of the relevant literatures.

Pancreatico-biliary ductal union.

A long common channel distal to the pancreaticobiliary junction is the commonest anomalous arrangement of the pancreaticobiliary ductal system and is mostly observed in patients with congenital choledochal cysts. APBDU without choledochal cyst is a high-risk condition for the development of gallbladder carcinomas. Prophylactic excision of the extrahepatic biliary system and reconstruction of the biliary tract with hepatico-jejunostomy are recommended. APBDU should always be kept in mind when a patient with a long history of abdominal pain is found to have gall bladder wall thickness even without gallstones on imaging by a CT Scan or Ultra Sound. ERCP should be performed in these patients in order to detect APBDU. This may allow early detection of carcinoma of the biliary tract. Presence of common channel may be associated with a lower incidence of gallstones. However it requires corroboration by other studies. On the other hand carcinoma of the gall bladder appears to have a close association with abnormally long common channel.

El síndrome del conducto bilio-pancreático común en adultos / Common bilio pancreatic channel syndrome in Chilean adults

Background: The Common Channel Syndrome (CCS) is defined as the union of the biliary and pancreatic ducts outside of the duodenal wall with a common duct more than 15 mm long. It is classified morphologically in types 1 and 2, whether it is the biliary or the pancreatic duct the one that predominates. CCS is uncommon in Western countries. It is described particularly in children and has not yet been reported in Chile. Aim: To report the frequency of CCS among patients subjected to endoscopic retrograde cholangiopancreatography (ERCP). Patients and methods: Between 1992 and 1998, 1860 ERCPs were performed for the diagnosis of biliary or pancreatic diseases. Results : In six patients (0.35 percent) a CCS was diagnosed (3 type 1 and 3 type 2). All patients presented with pain and three with jaundice. Four patients had a previous cholecystectomy, three due to cholelithiasis and one due to gallbladder cancer. A common bile duct resection and bilioenteric anastomosis was done in three patients with a favorable outcome. Two patients with an advanced age and one with a gallbladder cancer, were not operated. Conclusions : CCS was found in 0.35 percent of patients subjected to an ERCP. It frequently presents in association with one or more bilio-pancreatic diseases: cholelithiasis, choledocolithiasis, cholangitis, pancreatitis and gallbladder cancer. Resection of the common bile duct and a bilio-enteric anastomosis is proposed as the surgical treatment

Choledochal cyst associated the with anomalous union of pancreaticobiliary duct (AUPBD) has a more grave clinical course than choledochal cyst alone

OBJECTIVE: Since choledochal cyst is frequently associated with the anomalous union of pancreaticobiliary duct (AUPBD), AUPBD has been regarded to be the etiologic factor of choledochal cyst. However, the clinical significance of AUPBD an patients with choledochal cyst has not been clearly defined. Therefore, to clarify the significance of AUPBD in choledochal cyst patients, we compared the clinical features of patients with choledochal cyst according to the presence or absence of AUPBD. METHODS: Among 52 cases which were diagnosed as choledochal cyst out of 5,037 ERCP referrals between August 1990 and December 1996, we selected 44 cases, in which the pancreaticobiliary junction was clearly visualized on cholangio-pancreaticography. These cases were divided into AUPBD-present group (n = 28) and AUPBD-absent group (n = 16). Clinical features were compared between the two groups. Furthermore, in AUPBD-present group, clinical data were also analyzed according to Kimura's classification of AUPBD. RESULTS: In our study, AUPBD was associated with choledochal cyst in 28 (64%) cases. AUPBD was found only in type I and IV according to Todani's classification of choledochal cyst. There were no significant differences between the AUPBD-present group and the AUPBD-absent group in the incidence of gallstone disease, while the incidence of acute inflammation was 93% (26/28) in the AUPBD-absent group (p < 0.01). Carcinoma developed only in the AUOBD-present group (9/28, 32%) (p < 0.05). Pancreatic disorders (i.e. pancreatic stone, pancreatitis or pancreatic cancer) occurred in 12 of 28 cases in the AUPBD-present group (43%), while only in 1 of 16 cases in the AUPBD-absent group (6%) (p < 0.05). CONCLUSION: AUPBD associated with choledochal cyst may have implications not only as a possible etiologic factor but also as an important factor that may affect the clinical course, surgical planning and prognosis. In cases with choledochal cyst, we should make an effort to evaluate the presence of AUPBD.

Carcinoma gall bladder; Local risk factors

Seccia and cavina have divided the risk factors for carcinoma gallbladder into three main groups: local, systemic and genetic. LOCAL RISK FACTORS FOR CA GALLBLADDER ARE AS FOLLOWS: 1. Cholelithiasis. 2. Polypoidal lesions of gallbladder. 3. Congenital anomalies. 4. Porcelain gallbladder. 5. Chronic typhoid infection

Bifid pancreas: a case report.

Bifid pancreas is a rare anatomical anomaly in which the main pancreatic duct is bifurcated along its length. Although the pancreatographic appearance has been described in the literature, there is no mention of the computerised tomographic appearance of this condition. We report a case of bifid pancreas with the ERCP and CT appearances, who also had alcohol induced changes of chronic pancreatitis.

Endoscopic stenting of minor papilla for symptomatic pancreas divisum.

Pancreatitis is one of the presentations of pancreas divisum. Endoscopic treatment by dorsal pancreatic duct stenting relieves symptoms in a majority of patients. We report a patient with pancreas divisum and calcific chronic pancreatitis in whom successful drainage of the dorsal duct with a stent through the minor papilla relieved the pain.