Anomalous Pancreaticobiliary Research Articles

An unusual variation of type IIIC anomalous pancreaticobiliary junction (APBJ)

An unusual variation of type IIIC anomalous pancreaticobiliary junction (APBJ)

Endoscopic Ultrasound in Pancreatic Duct Anomalies.

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.

Horizontal duodenal papilla is associated with a special spectrum of pancreaticobiliary diseases: a retrospective magnetic resonance cholangiopancreatography-based study.

Horizontal duodenal papilla (HDP) is not an uncommon ectopic major papilla. The impact of HDP on the occurrence of pancreaticobiliary diseases remains unclear. Here, we explored the associations in patients who underwent magnetic resonance cholangiopancreatography (MRCP). Consecutive patients who underwent MRCP at Xijing Hospital (Xi'an, China) between January 2020 and December 2021 were eligible. Patients were divided into HDP and regular papilla (RP) according to the position of the major papilla. The primary outcome was the proportion of congenital pancreaticobiliary diseases. A total of 2,194 patients were included, of whom 72 (3.3%) had HDP. Compared with the RP group (n = 2,122), the HDP group had a higher proportion of congenital pancreaticobiliary diseases, especially choledochal cyst (CC) or anomalous pancreaticobiliary junction (APBJ) (6.9% vs 1.4%, P = 0.001). More gallbladder cancer (6.9% vs 1.2%, P < 0.001) and pancreatic cysts (27.8% vs 16.3%, P = 0.01) were also identified in the HDP group. Morphologically, the HDP group had a longer extrahepatic bile duct (8.4 [7.6-9.3] cm vs 7.2 [6.5-8.1] cm, P < 0.001), and larger angles between the common bile duct-duodenum and pancreatic duct-duodenum. Multivariate analysis showed that the presence of HDP was an independent risk factor for gallbladder cancer. This study confirmed that HDP was not rare in patients underwent MRCP. A higher prevalence of congenital pancreaticobiliary malformations (especially CC or APBJ), gallbladder cancer and pancreatic cysts was observed in patients with HDP, as well as distinctive morphologic features.

An Unusual Variant of Anomalous Pancreaticobiliary Junction.

A 27-year-old previously healthy African American female presented to the Emergency Department with an acute onset of epigastric abdominal pain and nausea. Laboratory studies proved unremarkable. CT scan demonstrated intrahepatic and extrahepatic biliary ductal dilation with possible stones within the common bile duct. The patient was discharged with a surgery follow-up appointment. Laparoscopic cholecystectomy with intraoperative cholangiography was performed 3weeks later due to concern for choledocholithiasis. The intraoperative cholangiogram showed multiple abnormalities, concerning for an infectious or inflammatory process. Magnetic resonance cholangiopancreatography (MRCP) demonstrated a suspected anomalous pancreaticobiliary junction and cystic lesion near the pancreatic head. Endoscopic retrograde cholangiopancreatography (ERCP) for cholangioscopy showed normal-appearing pancreaticobiliary mucosa with 3 tributaries directly from the pancreas entering the bile duct and an ansa orientation to the pancreatic duct. Biopsies of the mucosa were benign. Annual MRCP and MRI to assess for findings concerning for neoplasm given the anomalous pancreaticobiliary junction were recommended.

Biliary Intraepithelial Neoplasia (BilIN) Diagnosed From Choledochal Cyst in 7-Year-Old Girl With Underlying Anomalous Pancreaticobiliary Ductal Union (APBDU)

Biliary Intraepithelial Neoplasia (BilIN) Diagnosed From Choledochal Cyst in 7-Year-Old Girl With Underlying Anomalous Pancreaticobiliary Ductal Union (APBDU)

S1907 A Rare Case of Type 1 Choledochal Cyst With Type C Anomalous Pancreaticobiliary Maljunction in a 52-Year-Old Patient

Introduction: Choledochal cysts are rare anomalies of the bile duct that generally present in childhood but can rarely be delayed until adulthood in 20% of cases. They are often diagnosed incidentally or present with acute pancreatitis or cholangitis. We report a case of a type 1 choledochal cyst (T1CC) and type C anomalous pancreaticobiliary maljunction (PBM), successfully managed by a multidisciplinary approach. Case Description/Methods: A 52-year-old woman presented with right upper quadrant pain, jaundice and vomiting. Labs revealed elevated lipase, bilirubin, AST, ALT and alkaline phosphatase. A CT of the abdomen showed no gallstones but a marked dilation of extrahepatic bile duct with a transition point in the distal intrapancreatic portion (Figure a). An endoscopic retrograde cholangiopancreaticogram (ERCP) showed fusiform dilation of the CBD consistent with T1CC (Figure b). Spyglass cholangioscopy revealed focal stenosis in the distal CBD joining above the ventral pancreatic duct opening within the long common channel, suggestive of type C anomalous PBM (Figure c). A plastic biliary stent was placed into the CBD. EUS guided fine needle biopsy of the stricture showed no evidence of malignancy. Her clinical course was complicated by multiple admissions due to stent occlusion. After multidisciplinary discussion, the patient underwent choledochal cyst resection with hepaticojejunostomy, cholecystectomy and biliary reconstruction. Intraoperative gallbladder specimen revealed poorly differentiated adenocarcinoma with plan for chemotherapy. Discussion: Choledochal cysts are a rare congenital anomaly characterized by enlargement of the CBD. There are five types of choledochal cysts (Table). Etiology remains unclear and while some believe these cysts are congenital in nature, others propose they arise from anomalous PBM. PBM is defined as congenital malformation in which the pancreatic and bile duct join outside the duodenal wall forming a long common channel. This leads to reflux of biliary secretions leading to chronic inflammation, increasing the risk of developing cholangiocarcinoma and gallbladder cancer, as seen in our patient. Placement of biliary stents can temporarily alleviate symptoms, but surgical excision is the definitive treatment. Our case highlights the importance of including type 1 choledochal cyst in the differential diagnosis even in adult patients with a significantly dilated CBD. A thorough endoscopic and radiological work up should be done to prevent progression to malignancy.Figure 1.: A. CT of the abdomen showing marked dilatation of extrahepatic bile duct with a transition point in the distal intrapancreatic portion. B. Cholangiogram showing Type 1 Choledochal Cyst and Type C Anomalous Pancreaticobiliary Maljunction. C. Spyglass cholangioscopy showing JagwireTM within the CBD (yellow arrow) revealing focal stenotic CBD joining the common channel just above the ventral pancreatic duct (blue arrow). Table 1. - Types of Choledochal Cysts Type Description Type I Fusiform dilation of the CBD Type II Bile duct diverticulum Type III Saccular dilation of the intraduodenal portion of the CBD Type IVa Multiple cysts at intra and extrahepatic ducts Type IVb Multiple cysts at extrahepatic ducts only Type V Fusiform or saccular cystic dilation of the intrahepatic bile duct

Diagnostic challenge: Mirizzi syndrome with anomalous pancreaticobiliary junction.

Diagnostic challenge: Mirizzi syndrome with anomalous pancreaticobiliary junction.

Gallbladder Carcinoma Complicated with Anomalous Pancreaticobiliary Ductal Union with Hyperkalemia

Gallbladder Carcinoma Complicated with Anomalous Pancreaticobiliary Ductal Union with Hyperkalemia

S1659 A Case of Gallbladder Agenesis and Anomalous Pancreaticobiliary Junction With Chronic Choledocholithiasis and Secondary Biliary Cirrhosis

S1659 A Case of Gallbladder Agenesis and Anomalous Pancreaticobiliary Junction With Chronic Choledocholithiasis and Secondary Biliary Cirrhosis

S1616 Primary Carcinosarcoma of the Gallbladder: An Unfortunate Fate From Anomalous Pancreaticobiliary Junction

S1616 Primary Carcinosarcoma of the Gallbladder: An Unfortunate Fate From Anomalous Pancreaticobiliary Junction

Nadir Bir Konjenital Safra Anomalisi: Pankreatikobiliyer Bilişim Anomalisi

20-year-old female patient was admitted with complaints of girdle-like abdominal pain, nausea and vomiting. A history of pancreatitis 2 years ago was found in the patient’s anamnesis. Physical examination showed tenderness in the epigastric region, other system examinations were normal. In laboratory tests, amylase was found as 1249 ıu/l, lipase was found as 1068 u/l, CRP (C-Reaktif Protein) was found as 49.83mg/l and they were found to be high. Liver function tests, kidney function tests and electrolytes were found to be normal. In the abdomen ultrasonography, pancreas was reported to have increased size and mild edema. The patient was hospitalized with these findings and intravenous fluid replacement was started. Magnetic resonance cholangiopancreatography (MRCP) was performed on the patient whose vital findings were stable. MRCP showed that common bile duct and pancreatic duct were joined at 17 mm proximal from the normal anatomic location. Anomalous pancreaticobiliary junction (APBJ) is a rare congenital anomaly and in recent years its diagnosis frequency has increased due to the widespread use of advanced radiological examinations such as MRCP. The purpose of this case presentation was to present the APBJ that we detected with MRCP in a patient with recurrent pancreatitis attacks.

Secretin-Enhanced MRCP: How and Why-AJR Expert Panel Narrative Review.

Secretin-enhanced MRCP (S-MRCP) has advantages over standard MRCP for imaging of the pancreaticobiliary tree. Through the use of secretin to induce fluid production from the pancreas and leveraging of fluid-sensitive MRCP sequences, S-MRCP facilitates visualization of ductal anatomy, and the findings provide insight into pancreatic function, allowing radiologists to provide additional insight into a range of pancreatic conditions. This narrative review provides detailed information on the practical implementation of S-MRCP, including patient preparation, logistics of secretin administration, and dynamic secretin-enhanced MRCP acquisition. Also discussed are radiologists' interpretation and reporting of S-MRCP examinations, including assessments of dynamic compliance of the main pancreatic duct and of duodenal fluid volume. Established indications for S-MRCP include pancreas divisum, anomalous pancreaticobiliary junction, Santorinicele, Wirsungocele, chronic pancreatitis, main pancreatic duct stenosis, and assessment of complex postoperative anatomy. Equivocal or controversial indications are also described along with an approach to such indications. These indications include acute and recurrent acute pancreatitis, pancreatic exocrine function, sphincter of Oddi dysfunction, and pancreatic neoplasms.

Causes and Countermeasures of Difficult Selective Biliary Cannulation: A Large Sample Size Retrospective Study.

Selective biliary cannulation is a difficult part of endoscopic retrograde cholangiopancreatography (ERCP). Therefore, endoscopists have focused on how to improve the cannulation success rate and reduce the postoperative complication rate. This study aims to provide practical advice on safe operation for endoscopists. Data from the ERCP database of our medical center from January 2008 to December 2017 were retrospectively reviewed. All patients underwent ERCP for the first time. Data pertaining to diseases, anatomic factors, difficult cannulation rates, cannulation success rates, and postoperative complication rates for various assistive cannulation techniques were analyzed by using the Pearson χ2 tests and Fisher exact tests. The difficult cannulation rate was 8.1%. The main influencing factors were benign and malignant biliary stricture, duodenal papillae or ampullary tumors, thick and long duodenal papillae, peridiverticular papillae, ectopic papillae, edematous papillae, ulcerative papillae, anomalous pancreaticobiliary junction, and Billroth II gastrectomy. The postoperative complication rates and overall cannulation success rates of 7 assistive techniques were not statistically different (P>0.05), but the overall complication rates were statistically different (P<0.05). Needle-knife precut sphincterotomy had the highest rate of complications. Benign and malignant biliary stricture, duodenal papillae or ampullary tumors, thick and long duodenal papillae, peridiverticular papillae, ectopic papillae, edematous papillae, ulcerative papillae, anomalous pancreaticobiliary junction, and Billroth II gastrectomy were the main factors of difficult selective biliary cannulation. The overall incidence of needle-knife precut sphincterotomy complications was higher than that of other techniques, but various assistive techniques were safe and effective in our single center.

S0117 Risk of Gallbladder Cancer With Increasing Gallstone Size: A Systematic Review and Meta-Analysis

INTRODUCTION: Gallbladder (GB) cancer (GBC) accounted for 3960 deaths in the US in 2019. With only 1 in 5 GBCs diagnosed at early stage, the overall 5-year survival rate is a meager 19%. Given the low 5-year survival, the best treatment is prevention. Risk factors for GBC include age, ethnicity, GB polyps >1 cm, primary sclerosing cholangitis, anomalous pancreaticobiliary junction, toxins, and GB stones. There remains controversy as to whether size of GB stones increases the risk of GBC. The aim of this systematic review and meta-analysis was to determine if risk of GBC increases with increasing size of GB stones. METHODS: A systematic search was conducted using the terms “gallbladder cancer,” “cholelithiasis,” “gallstones,” and “gallstone size” with all permutations of pubmed/medline, google scholar, embase, and cochrane. Papers were translated, if necessary. A recursive search of bibliographies of included articles was also performed. GB stone size was primarily determined post-cholecystectomy. The relevant data was inputted into RevMan 5.4 in order to generate odds ratios and Forest plots. RESULTS: The search yielded 357 total articles, of which 8 met our final inclusion criteria with GB stone measurement and prevalence of GBC in each study population. The data was then divided into 3 groups, based on size of GB stones, greater or lesser than 3 cm, 2 cm, or 1 cm. We found that the OR of developing GBC was 1.93 [1.29–2.90], 1.42 [1.06–1.90], and 1.62 [1.24–2.11] for stones greater than 3 cm, 2 cm, and 1 cm, respectively. CONCLUSION: Patients with GB stones >1 cm had increased odds of developing GBC, however we did not observe a linear relationship between GBC and increasing stone size. Our meta-analysis found an OR of 1.93 for stones >3 cm which is significantly less than the 9.1 previously reported. The association between GB stone size and GBC is likely related to the chronicity of GB stones rather than absolute size. Jain et al. proposed that chronic inflammation could subsequently lead to loss of heterozygosity of tumor suppressor genes. In summary, we found the risk of GBC is similar regardless of size of stones >1 cm. Therefore while we usually observe asymptomatic GB stones, clinicians should be aware that GB stones >1 cm may have increased risk of GBC in symptomatic individuals. Further studies are needed in asymptomatic individuals to define the optimal GB stone size cutoff to perform cholecystectomy to reduce GBC risk.Figure 1.: Odds of developing gallbladder cancer with increasing size of gallstones. (Events = # of cases of gallbladder cancer; Total = # of cases of patients with gallstones > or < the specified size).

1426 Type 1 Choledochal Cyst With Anomalous Pancreaticobiliary Malunion Diagnosed on Cholangioscopy

INTRODUCTION: Choledochal cysts are cystic dilatations found within the intra and/or extrahepatic bile ducts. Untreated choledochal cysts can lead to increased risk for strictures, pancreatitis, cholangitis, gallbladder cancer, cholangiocarcinoma and pancreatic cancer. Early and accurate identification of choledochal cysts is crucial to determine appropriate medical and surgical planning. We present a case of a patient with a type 1 choledochal cyst with anomalous pancreaticobiliary malunion. CASE DESCRIPTION/METHODS: A 42-year-old female with a PMH of a cholecystectomy and known biliary cyst presented with acute onset abdominal pain. MRCP demonstrated a 5.5 cm fusiform dilatation of the common bile duct (CBD). EUS confirmed a CBD dilatation (Figure 1). ERCP with spyglass cholangioscopy showed an abnormal pancreaticobiliary junction with a common channel about 1 cm long leading to two separate openings, one to the CBD/choledochal cyst and another to the pancreatic duct orifice (Figure 2). Fluoroscopy showed contrast filling a dilated cystic structure in the proximal CBD consistent with a choledochal cyst (Figure 3). A 7 French × 7 cm flex stent was deployed into the CBD. Findings were consistent with a type 1 choledochal cyst and an anomalous pancreaticobiliary malunion. Patient underwent ex-lap with resection of the choledochal cyst with roux-en-y hepaticojejunostomy creation, which she tolerated well. DISCUSSION: The Todani classification is based on cyst location and bile duct dilatation. There are 5 types with type 1 as the most common which comprises of 80-90% of all choledochal cysts. Type 1 choledochal cysts involve fusiform dilatation of the entire CBD without any intrahepatic dilatation. Cancer is more common in patients who are older and with type I and IV cysts. These are typically treated with complete cyst resection and Roux-en-Y hepaticojejunostomy. Additionally, 50-80% of patients with choledochal cysts have an anomalous pancreaticobiliary malunion. The KOMI classification is used to classify anomalous pancreaticobiliary malunions into 3 types, which is based on the CBD/pancreatic duct angle and presence of CBD dilation. This case was a KOMI type IIB anomalous pancreaticobiliary malunion given the common hepatic and pancreatic ducts join each other at an acute angle creating a single common channel.

Choledochal cysts: Similarities and differences between Asian and Western countries.

Choledochal cysts (CCs) are rare bile duct dilatations, intra-and/or extrahepatic, and have higher prevalence in the Asian population compared to Western populations. Most of the current literature on CC disease originates from Asia where these entities are most prevalent. They are thought to arise from an anomalous pancreaticobiliary junction, which are congenital anomalies between pancreatic and bile ducts. Some similarities in presentation between Eastern and Western patients exist such as female predominance, however, contemporary studies suggest that Asian patients may be more symptomatic on presentation. Even though CC disease presents with an increased malignant risk reported to be more than 10% after the second decade of life in Asian patients, this risk may be overstated in Western populations. Despite this difference in cancer risk, management guidelines for all patients with CC are based predominantly on observations reported from Asia where it is recommended that all CCs should be excised out of concern for the presence or development of biliary tract cancer.

Intracholecystic papillary-tubular neoplasm in a patient with choledochal cyst and anomalous pancreaticobiliary junction: case report

A 75-year-old female incidentally presented with an enhancing intraluminal gallbladder mass, main bile duct dilatation and anomalous pancreaticobiliary junction (APBJ) during the staging of gastric adenocarcinoma. Histopathological analysis confirmed the diagnosis of intracholecystic papillary-tubular neoplasm. Intracholecystic papillary-tubular neoplasms (ICPN) of the gallbladder are rare gallbladder neoplasms, defined as intramucosal, preinvasive, exophytic, mass forming lesions. An association between choledochal cysts and anomalous pancreaticobiliary junction with gallbladder neoplasms is well known, and this case potentially illustrates gallbladder carcinogenesis related to these biliary anomalies.

AB059. P-28. Is cholangiocarcinoma the only concern in western adult patients with choledochal cyst (CC) disease and anomalous pancreaticobiliary junction (APBJ)?

AB059. P-28. Is cholangiocarcinoma the only concern in western adult patients with choledochal cyst (CC) disease and anomalous pancreaticobiliary junction (APBJ)?

Endoscopic dissection of refractory pancreatic duct stricture via accessory pancreatic duct approach for concurrent treatment of anomalous pancreaticobiliary junction in aging patients.

BackgroundAlthough endoscopic management of pancreatic strictures by dilation and stenting is well established, some high-grade strictures are refractory to conventional methods. Here, we report a novel technique via accessory pancreatic duct (APD) approach to simultaneously release chronic pancreatitis-associated pancreatic stricture and correct anomalous pancreaticobiliary junction (APBJ). Due to APBJ and stricture of proximal main pancreatic duct, the APD turned out to be compensatory expansion. The stiff stenosis was dissected along the axial of APD using needle-knife electrocautery or holmium laser ablation, and then the supporting stent was placed into the pancreatic body duct. By doing so, the outflow channels of pancreatic and biliary ducts were exquisitely separated.Patients and methodsTwo patients aged 69 and 71 years underwent stricture dissection and stent insertion for fluent drainage of pancreatic juice. The postoperative course was marked by complete abdominal pain relief and normal blood amylase recovery. In the first patient, wire-guided needle-knife electrocautery under fluoroscopic control was applied to release refractory stricture. The second patient was treated by SpyGlass pancreatoscopy-guided holmium laser ablation to lift pancreatic stricture.ResultsPlastic stents in APD were removed at 3 months after surgery, and magnetic resonance imaging at 6 months showed strictly normal aspect of the pancreatic duct.ConclusionAlthough both cases were successful without severe complications, we recommend this approach only for selected patients with short refractory pancreatic strictures due to chronic pancreatitis. In order to prevent severe complications (bleeding, perforation or pancreatitis), direct-view endoscopy-guided electrotomy needs to be developed.

Choledochal cysts and anomalous pancreaticobiliary junction in a western population: a tertiary care, single center experience

Choledochal cysts and anomalous pancreaticobiliary junction in a western population: a tertiary care, single center experience