Anomalous Pancreatobiliary Junction Research Articles

CASE OF SYNCHRONOUS HEPATOBILIARY TUMOUR; DIAGNOSTIC DILEMMA

Synchronous biliary tract tumours are very rare. Synchronous double cancers of the extrahepatic bile duct with-out abnormal pancreatobiliary junction are extremely rare, and to date only 4 cases have been reported in the English literature [1-3]. Until now less than 10 cases have been reported all over the world. Usually, the coexistence is attributed to abnormal pancreatobiliary junction [4-6], local spread or metastasis or de novo. In cases apart from anomalous pancreatobiliary junction, p53 mutations have been associated with simultaneous cancers. Contrast enhanced tomography is done as the initial investigation and FDG-PET is increasingly being used in the staging work up. When there is a suspicion of periampullary growth on cross sectional imaging, side viewing scopy is done to evaluate the lesion and take biopsies. The case presented here is interesting as PET showed metabolically active lesions in the the gall bladder and periampullary region, the latter being subtle on endoscopy.

Diagnostic Accuracy of Pre-procedure MRI/MRCP in Patients Undergoing ERCP With Minor Papilla Cannulation in the Detection of Pancreas Divisum

Introduction: Pancreas Divisum (PD) is the most common congenital anatomic abnormality of the pancreas, affecting 5-10% of the US population. PD is often asymptomatic, however, pancreatitis is presumed to develop due to decreased flow across a stenotic minor papilla. Endoscopic retrograde cholangiopancreatography (ERCP) is the gold standard diagnostic modality for PD, however, it carries the risk for potential complications. The purpose of this study was to evaluate the diagnostic yield of magnetic resonance imaging/magnetic resonance cholangiopancreatography (MRI/MRCP) in detecting PD. Methods: This was a single center retrospective analysis of patients in our tertiary referral center who underwent ERCP with diagnostic confirmation of PD between April 2009 and November 2016. Patient demographics, pre-procedural MRCP, and clinical co-variates were examined, and their impact on diagnosis was evaluated with univariate analysis. Results: In this cohort, 82 patients with PD underwent 173 ERCPs. Complete data from the initial ERCP and pre-procedural MRCP was available for 79 (96%) patients. None of the MRCPs were secretinenhanced. MRCP identified PD in 50 patients (63%) and did not identify PD in 29 (37%). Females were more likely than males to have PD diagnosed on MRCP (OR 3.33, CI 1.26-8.78, p=0.013). Age, race, history of smoking, alcohol use, and opioid use were not significantly different between groups (p=0.85, p=0.08, p=0.44, p=0.11, and p=0.99, respectively). Anatomic factors including presence of a native papilla, complete versus partial PD, presence of an IPMN, Santorinicele, annular pancreas, or anomalous pancreatobiliary junction were also similar between the two groups (p=0.81, p=0.14, p=0.62, p=1.00, p=1.00, and p=0.53 respectively). Additionally, neither a history of prior pancreatitis nor a history of pancreatic stent exchange (p =0.07 and p=0.19) were correlated with absence of a diagnosis of PD on MRCP. Conclusion: Pancreas divisum may not be detected by pre-procedure non-secretin enhanced MRI/MRCP in over one third of patients undergoing ERCP with minor papilla cannulation. The presence of other pancreatic anatomic variants, as well as prior manipulation or inflammation of the pancreas, did not appear to impact the diagnostic yield.

Safety and Efficacy of Endoscopic Treatment in Pancreas Divisum in Patients With Recurrent Acute Pancreatitis

Introduction: Pancreas divisum (PD) is a common anatomic variant found in approximately 7% of the population. Management approaches in patients with divisum include medical therapy, endoscopic papillotomy with stent placement, and surgical therapy. This study evaluated a large single center experience in the endoscopic management of PD patients with recurrent acute pancreatitis. Methods: This retrospective study included patients with PD undergoing ERCP from period of April 2009 to November 2016. Various demographic and procedural parameters were collected. Data was abstracted from the medical record to follow the treatment course of patients with PD through the study time period. Results: A total of 174 ERCPs were performed on 82 patients during the study period. The average age of the patients was 45.6 years, 49.3 % were male, 58.5% were Caucasian. 64.6 % (n=53) patients had complete divisum. A total of 5 (5.8%) patients had IPMN, 3 (3.5%) patients had a santorinicele and none of the patient had a pancreatobiliary cancer. A single case of annular pancreas and another single case of anomalous pancreatobiliary junction was diagnosed. 35.3% (n=29) patients were active smokers, 29.2% (n=23) consumed alcohol and 37.8% (n=31) patients were on chronic opioids. 54 patients (31%) were admitted to the hospital post ERCP with an average length of stay of 4.2 days. Out of 82 patients, 36 underwent one ERCP with minor papillotomy. Cannulation was unsuccessful in 7 patients and surgical referral was made. 39 patients required multiple ERCPs (on average 3.2 ERCPs, range 2 to 8). Interventions such as minor papillotomy, balloon sweeps and pancreatic duct stent placement with gradual upsizing of stent were performed. At the end of follow up, 56 patients (68.2%) had clinical success, 19 patients (23.1%) had no response to endotherapy. 7 patients (8.5%) were lost follow up. 9 (5.2%) patients developed post ERCP pancreatitis, 3 patients had post papillotomy bleeding controlled with endoscopic intervention and 24 patients had non-pancreatitis post procedure abdominal pain. No deaths were observed. Conclusion: Endoscopic intervention with minor papillotomy and pancreatic duct stent placement with gradual stent upsizing appears to be safe and effective in a carefully selected cohort of patients with divisum presenting with recurrent acute pancreatitis. Prospective studies on the comparative efficacy, impact on quality of life, and healthcare costs are indicated.

Type III choledochal cyst and anomalous pancreatobiliary junction

Type III choledochal cyst and anomalous pancreatobiliary junction

Periampullary choledochal diverticula are not choledochal cysts

Choledochal cysts (CC) are rare, congenital anomalies of the biliary tree, associated with the development of biliary malignancies. Small periampullary choledochal diverticula (PCD) are a previously unreported type of biliary anomaly found primarily at ERCP. The aim of this study was to assess whether PCD are congenital or acquired lesions by comparing the clinical presentation, management, and risk of malignancy between patients with PCD and CC. Retrospective analysis of a medical center database. Academic tertiary referral center. Over the study period, data regarding 16 patients with PCD were identified and compared with that of 118 patients with CC. Retrospective review of ERCP, surgical pathology, billings, and a diagnostic imaging database from our institution from 1985 to 2009 was done. Clinical presentation, investigations, management strategies, complication rates, and long-term outcomes were compared in patients with classic CC and PCD over the same time period. Patients with PCD were less likely to be female (50% vs 81%), older aged (mean 68 vs 28 years), to complain of abdominal pain (88% vs 68%), and were less likely to present with jaundice (0% vs 32%) (P<.05 for all pairs). Patients with PCD also were noted to have lower frequency of anomalous pancreatobiliary junction (0% vs 83%) and biliary neoplasia (0% vs 5%) and more likely to have sphincter of Oddi dysfunction (63% vs 1%). Management of PCD was done with ERCP in 87% of cases and with surgery in 0% of cases, whereas management of CC was done with ERCP in 20% of cases and surgery in 80% of cases (P<.001). Long-term complications at a mean follow-up of 3.7 years after therapy were more common in CC (40% vs 6%, P=.02). Retrospective study. Lack of structured follow up. Small, periampullary, choledochal diverticula are a newly reported, likely acquired anomaly of the biliary tract that are frequently associated with sphincter of Oddi dysfunction and may be secondary to biliary hypertension. These acquired lesions should not be classified as CC.

Acute Pancreatitis Associated With Anomalous Pancreatobiliary Junction

Acute Pancreatitis Associated With Anomalous Pancreatobiliary Junction

Pancreatobiliary maljunction in Western adults

I read with interest the recent report by Bechtler et al. [1] about a choledochal cyst type I A with drainage through the ventral duct in a patient with pancreas divisum. It is true that most biliary cysts are secondary to anomalous pancreatobiliary junction, also called pancreatobiliary maljunction (PBMJ). This abnormality (an extramural junction of the pancreatic and biliary ducts in the duodenum, apparently beyond the scope of intramural sphincter function), has been extensively reported in Eastern people [2], but, as we have stated previously [3], PBMJ is not only an Eastern disease and this diagnosis is increasingly being made in other populations [4]. It is important, as Bechtler et al. [1] remark, to give attention to the distal part of the common bile duct as it can show side branches like a pancreatic duct ([Fig. 1], [2]).

Rôle diagnostique de l’écho-endoscopie dans la pancréatite aiguë

In approximately 20% of patients with acute pancreatitis, a cause is not established by history, physical examination, routine laboratory testing or abdominal imaging. Recent studies suggest that microlithiasis is causative in up to 60% of patients with an unexplained acute pancreatitis (UAP) and gallbladder in situ. In the others, many factors play an etiological role in idiopathic pancreatitis, including pancreatic tumors, early chronic pancreatitis, pancreas divisum or annular pancreas, choledochocele, intraductal papillary mucinous tumours (IPMT), an anomalous pancreatobiliary junction or sphincter of Oddi dysfunction (SOD). The close proximity of the endoscopic ultrasound probe (EUS) to the pancreas and biliary tract results in superior spatial resolution compared with CT scan and MRI.The diagnostic yield of EUS in unexplained acute pancreatitis is 80%. EUS appears to be diagnostic in the majority of patients with previously unexplained pancreatitis and offers an alternative to MRI and endoscopic retrograde cholangiopancreatography (ERCP) as the initial diagnostic test in patients with unexplained acute pancreatitis.

Laparoscopic Management of Choledochal Cysts: Technique and Outcomes—A Retrospective Study of 35 Patients from a Tertiary Center

Choledochal cysts are congenital dilations of the biliary tree. The accepted mode of treatment is total excision with hepaticojejunostomy. In this retrospective study, we present our technique and results of laparoscopic choledochal cyst excision. We retrospectively studied 35 patients who had undergone laparoscopic choledochal cyst excision in our institute from 1996 to 2008. Data about age, gender, type of choledochal cyst, symptoms, surgical technique, conversion rate, morbidity, and mortality were analyzed. There were Type 1B choledochal cysts in 27 patients (77%) and Type IVA in 8 patients (23%). An anomalous pancreatobiliary junction was found in 40%. Mean operative time was 295 minutes. Total cyst excision could be done in 26 patients. The conversion rate in our series was 8.5%. The overall morbidity rate was 14.3%, the reoperation rate was 8.5%, the mortality rate was 0%, and the incidence of carcinoma was 8.5%. To minimize the risk of malignancy, total excision of the cyst is ideal, but a small proximal cuff of cyst is retained for small-size ducts to aid in the hepaticojejunostomy anastomosis. A slit on one end of the small ducts will render the anastomosis in an oblique orientation, widening the lumen. Dissecting the posterior cyst wall from the underlying portal vein is the most crucial part of the procedure. Morbidity and mortality rates after laparoscopic management are comparable with published results of the open procedure. Laparoscopic surgery for choledochal cysts is feasible, safe, and even advantageous.

Cisto de colédoco em adulto: anomalia da junção do colédoco com o ducto pancreático submetido à ressecção do cisto e à derivação biliar e pancreática

Common bile duct cysts are rare congenital anomalies which have been diagnosed only in twenty per cent of adults. The etiology is uncertain, but many patients have an anomalous pancreatobiliary junction anatomy. We present a case of a young man with a type I Alonso-Lej/ Todani common bile duct cyst and an anomalous common bile duct-pancreatic junction anatomy. Because the common bile duct did not have a segment of normal caliber, to avoid compromising with the pancreatic channel after the excision of the cyst, we performed a Roux-en-Y anastomosis by anastomosing the biliary duct to the proximal excluded jejunal loop and the common duct-pancreatic junction to the same more distally loop.

Endoscopic ultrasonography for evaluating patients with recurrent pancreatitis

Acute recurrent pancreatitis (ARP) is still a complex diagnostic and therapeutic challenge in clinical practice. In up to 30% of cases of ARP, it is not possible to establish the etiology of the disease. In the other 70%, many factors play an etiological role in ARP: microlithiasis, sphincter of Oddi dysfunction (SOD), pancreas divisum, hereditary pancreatitis, cystic fibrosis, a choledochocele, annular pancreas, an anomalous pancreatobiliary junction, pancreatic tumors or chronic pancreatitis are diagnosed. EUS should be useful in ARP as it is sensitive for diagnosing bile duct stones, gallbladder sludge, pancreatic lesions, ductal abnormalities and chronic pancreatitis. Endoscopic ultrasound (EUS) appears to be diagnostic in the majority of patients with previously unexplained pancreatitis, and offers an alternative to endoscopic retrograde cholangiopancreatography (ERCP) as the initial diagnostic test in patients with ARP.

ERCP/Manometry Findings in 1,343 Idiopathic Pancreatitis Patients

Background: ERCP with sphincter of Oddi manometry can detect abnormalities in patients otherwise classified as idiopathic pancreatitis. Methods: All patients undergoing ERCP at Indiana University Medical Center have data prospectively entered into a database. 1,547 patients from 1994-2006 were classified initially as idiopathic pancreatitis. After review of medical history, non-invasive imaging, prior endoscopies, and blood studies, 204 patients were eliminated for known causes of pancreatitis. This study reviews the findings of ERCP with or without manometry of the remaining 1,343 patients in correlation with the pre-ERCP clinical diagnosis. Results: Clinical categories were based upon the pre-ERCP diagnosis: a single episode of acute pancreatitis in 281 (20.9%), acute recurrent pancreatitis (IARP) in 758 (56.4%), chronic pancreatitis (CP) in 304 (22.6%). Post-procedure diagnoses were: (See table). Biliary manometry findings were the following: elevated biliary sphincter pressures were found in 63 (41.7%) of the 151 attempted manometries in patients with 1 documented episode of pancreatitis, in 153 (32.9%) patients with acute recurrent pancreatitis of the 465 performed manometries, and in 30 (23.6%) of 127 manometries of CP. Elevated major pancreatic sphincter pressures were found in 93/154 (60.4%) with acute pancreatitis x1, in 266/488 (54.5%) with acute recurrent pancreatitis, and in 69/158 (43.7%) with chronic pancreatitis. Summary: In our large case series of idiopathic pancreatitis patients, ERCP and manometry frequently detects abnormalities that may be a cause of pancreatitis or lead to persistence of symptoms. Manometry abnormalities are the most common treatable cause of idiopathic pancreatitis. Long term outcomes of therapy studies are in progress. Conclusion: ERCP with sphincter of Oddi manometry is indicated for most patients with idiopathic pancreatitis. Tabled 1 ERCP findings Number of patients % of total Sphincter of Oddi dysfunction 539 40.1 Pancreas divisum 240 17.9 Periampullary diverticulum 82 6.1 Choledocholithiasis 61 4.6 Intraductal mucinous tumors 55 4.1 Cholelithiasis (alone) 26 1.9 Anomalous pancreatobiliary junction 7 0.5 Choledochocele 5 0.4 Papilla of Vater adenoma/cancer 4 0.3 Annular pancreas 4 0.3 Open table in a new tab

Laparoscopic Resection of Type I Choledochal Cyst in an Adult and Roux-en-Y Hepaticojejunostomy: A Case Report and Literature Review

Choledochal cysts are rare cystic dilatations of the extrahepatic biliary tree, the intrahepatic bile ducts, or both and carry a substantial risk of malignant transformation. Type I choledochal cysts, which involve the entire common hepatic and common bile ducts, represent 80% to 90% of these lesions. We report laparoscopic excision of symptomatic type I choledochal cyst in a 37-year-old woman, and review the literature. Laparoscopic excision of the extrahepatic biliary tree from the hepatic confluence to the anomalous pancreatobiliary junction with en bloc cholecystectomy and reconstruction with a Roux-en-Y hepaticojejunostomy was accomplished. Postoperative recovery was uneventful with a hospital stay of 3 days. She remains well and asymptomatic at 6 months of follow-up. Laparoscopic excision of choledochal cysts may be safely accomplished with a prompt recovery. Further experience with this approach in larger number of patients is justified and long-term follow-up data are needed.

Hepatobiliary and pancreatic: Anomalous pancreatobiliary junction with choledochal cyst

Hepatobiliary and pancreatic: Anomalous pancreatobiliary junction with choledochal cyst

Hepatobiliary and pancreatic: Anomalous pancreatobiliary ductal junction

A young man, aged 16 years, was referred for evaluation because of recurrent episodes of pancreatitis. At the age of 14 months, his liver function tests were found to be abnormal and he was treated by operative sphincteroplasty for stenosis of the lower bile duct. Since that time, he has had approximately two episodes of mild pancreatitis each year. Over the past 2 years, however, episodes of pancreatitis have increased in frequency and severity, and have often been accompanied by changes in liver function tests. At duodenal endoscopy, he had a satisfactory bile duct orifice and cholangiography revealed a bile duct at the upper limit of normal, air in the biliary system and an anomalous pancreatobiliary junction (Fig. 1). The main pancreatic duct and side-branches were near-normal. There was minor thickening of the gallbladder wall (3.8 mm) on an ultrasound study and exaggerated enhancement and thickening of the gallbladder wall on a computed tomography scan (Fig. 2). No gallbladder stones were seen. Because of relapsing pancreatitis, laparoscopic cholecystectomy was performed. Histological evaluation of the gallbladder revealed mild chronic cholecystitis and cholesterolosis. Anomalous pancreatobiliary ductal junction has been defined as a common channel of at least 15 mm. The disorder is associated with congenital anomalies of the biliary system (usually choledochal cysts) and with disorders such as relapsing pancreatitis, chronic pancreatitis, biliary strictures and gallbladder cancer. At least some patients with relapsing pancreatitis can be improved by endoscopic biliary sphincterotomy. However, in the patient described above, episodes of pancreatitis continued despite an adequate operative sphincteroplasty. As this patient had evidence of cholecystitis, a possible explanation for pancreatitis was biliary inflammation, perhaps accompanied by biliary debris. This appears to be caused by the excessive reflux of pancreatic juice into the biliary system. At least some of these patients are improved by cholecystectomy and, in addition, are protected from the longer-term risk of gallbladder cancer.

Anomalous choledocho-pancretic ductal junction in a choledochal cyst: A case report

Choledochal cysts are rare congenital anomalies, mostly detected in adults. Pathogenesis of these cysts seems to be in anomalous junction between pancreatic and common bile duct, above the papillary sphincter and outside of the duodenal wall. The absence of the sphincter above the junction is followed by reflux of the pancreatic juice into the bile duct leading to dilatation and fibrous changes of bile duct wall. A 38-year-old female is presented in whom a choledochal cyst was found 11 years earlier, during the operation performed for obstructive jaundice, when cystojejunostomy with Roux-en Y Jejunal limb was carried out. In February 1990, she was admitted to our Institution forjaundice and biliary colic. The patient was reoperated. Operative cholangiography showed an anomalous pancreatobiliary junction, choledochal cyst, dilated cystic duct and moderate dilatation of intrahepatic bile ducts. Cholecystectomy, desanastomosis with partial excision of choledochal cyst, and retrocolic choledochojejunostomy with the same Roux-en-Y jejunal limb were performed. Total excision of choledochal cyst was too risky due to chronic inflammatory changes in the hepatoduodenal ligament. Postoperative recovery was uneventful and the patient remained symptom-free so far.

Idiopathic acute recurrent pancreatitis.

Acute recurrent pancreatitis (ARP) results most commonly from alcohol abuse or gallstone disease. Initial evaluation fails to detect the cause of ARP in 10-30% of patients, and as a result the diagnosis of "idiopathic" ARP is given. In these patients, a more extensive evaluation including specialized labs, ERCP, endoscopic ultrasound, or magnetic resonance cholangiopancreatography typically leads to a diagnosis of microlithiasis, sphincter of Oddi dysfunction, or pancreas divisum. Less commonly, hereditary pancreatitis, cystic fibrosis, a choledochocele, annular pancreas, an anomalous pancreatobiliary junction, pancreatobiliary tumors, or chronic pancreatitis are diagnosed. Determining the etiology is important, as it helps to direct therapy, limits further unnecessary evaluation, and may improve a patient's long term prognosis.

Choledochal cyst due to anomalous pancreatobiliary junction in the adult: sonographic findings.

Adult cases of choledochal cyst due to anomalous pancreaticobiliary duct junction have been rarely reported. At present, sonography (US) is the first tool for diagnosing biliary disorders. The aim of this study was to reevaluate the US findings of choledochal cysts due to anomalous pancreaticobiliary duct junction in adults. We reviewed the clinical manifestations and US findings of 12 such adult cases confirmed by endoscopic retrograde cholangiopancreatic ductography (ERCP). Patients were assigned to three groups: (a) associated with biliary carcinoma (two cases), (b) associated with choledocholithiasis (one case), and (c) not associated with other abdominal diseases (nine cases). Patients in group c were asymptomatic, and the lesions were detected incidentally detected by US. In contrast, patients in group a sought medical care because of symptoms such as jaundice and those in group b sought medical attention because of abdominal pain. The diameter of a dilated bile duct on US was considerably less than that of ERCP (ERCP: 26-58 mm, mean = 37.6 mm; US: 13-32 mm, mean = 21.8 mm). Its diameter changed significantly under probe compression when the dilated bile duct took a purely cystic form but changed very little when it took a tubular form. Unlike cases in children, adult cases of choledochal cyst are generally asymptomatic. Careful US observation of the bile duct is thus expected to detect asymptomatic adult choledochal cysts cases. Cyst diameter can change significantly under probe compression, so it is important not to compress the bile duct during routine US examination.

Late follow-up following internal drainage of choledochal cysts reassessment of necessity for reoperation

To evaluate late results in children who had been treated with an internal drainage procedure for a choledochal cysts, we carried out follow-up examinations and performed endoscopic retrograde cholangiopancreatography (ERCP). Internal drainage procedures were performed on a total of 17 patients prior to 1972. The follow-up period ranged from 18 to 30 years. ERCP was performed on 8 of 10 patients followed up. All had dilatation of the common bile duct; 7 had an anomalous pancreatobiliary junction; and there were no carcinomas detected. The procedures consisted of choledochocystoduodenostomy (CCD) in 12 patients, choledochocystojejunostomy (Roux-Y) (CCJRY) in 4, and cholecystoduodenostomy (CYD) in 1. Of the 12 patients who were treated with CCD, 3 were lost to follow-up, 3 died of liver cirrhosis, and 6 survived. Five of the 6 surviving patients needed a reoperation for cholangitis; in 1 of these severe atypical abnormalities of the cyst were found on pathologic examination. Of the 4 patients who were treated with CCJRY, 1 died of liver cirrhosis but the 3 survivors had an asymptomatic postoperative course common bile duct and an anomalous pancreatobiliary junction noted during the current work-up. The patient who was treated with CYD needed another operation for choledocholithiasis. Our conclusions for following patients who were treated with an internal drainage procedure in childhood are twofold: (1) for patients who had CCD, cyst excision is recommended; and (2) for patients who had CCJRY performed at the age of 10 years or less, cautious observation is recommended as long as the patient remains asymptomatic.

The clinical spectrum of anomalous pancreatobiliary junction.

Anomalous junction of pancreatobiliary ducts (AJPB) is known to be associated with choledochal cyst and gallbladder cancer. This report describes our experience with eight patients with AJPB, six of whom were diagnosed at endoscopic retrograde cholangiography and two at transhepatic cholangiography. Four of the patients with AJPB had an associated congenital choledochal cyst, two had a normal biliary tree and one each had inspissated bile syndrome and cholangiocarcinoma. The last two associations have not been previously described. It is felt that the clinical spectrum of AJPB may unfold further with the widespread use of the cholangiographic techniques.