Anomalous Junction Research Articles

Comparative Study of the Diagnostic Ability of Magnetic Resonance Imaging and Multidetector Row Computed Tomography for Anomalous Pancreaticobiliary Ductal Junction

To compare the diagnostic performance of magnetic resonance (MR) imaging and multidetector row computed tomography (MDCT) for an anomalous pancreaticobiliary ductal junction (PBJ). This study included 26 patients who underwent MR imaging, MDCT, and endoscopic retrograde cholangiopancreatography. Seventeen of the patients were diagnosed by endoscopic retrograde cholangiopancreatography as having an anomalous PBJ. Three radiologists independently reviewed MR images alone, contrast-enhanced CT images alone, and MR images and CT images combined. Receiver operating characteristic curve analysis was used to compare diagnostic capabilities. The mean area under the receiver operating characteristic curve (Az [95% confidence interval]) was 0.75 (0.73-0.77) for MR imaging alone, 0.79 (0.70-0.89) for CT alone, and 0.84 (0.82-0.86) for the 2 modalities combined. Only between the combination and MR imaging alone was there significant difference in Az value (P < 0.05). The diagnostic performance of MR imaging and MDCT was equivalent for an anomalous PBJ. The addition of MDCT to MR imaging improved the diagnostic performance.

Strategies for diagnosis and treatment of anomalous junction of pancreaticobiliary duct complicated by acute pancreatitis and literature review

Objective To explore the strategies for diagnosis and treatment of anomalous junction of pancreaticobiliary duct (AJPBD) complicated by acute pancreatitis. Methods The clinical dataof 22 patients with abnormal pancreaticobiliary junction were analyzed retrospectively. Results The incidence of acute pancreatitis in this series was 31.8 % (7/22), thereinto, 5 cases(71.4%) in C-Ptype (the common bile duct joining the pancreatic duct) and 2(28.6%) in P-C type (the pancreatic duct joining the common bile duct). Seven patients underwent ERCP+ EST+ ENBD. Two patients with common bile duct stones were treated with stone basket and cholecystectomy was performed in two cases with gallstone. All patients were successfully treated. The follow-up for l year showed that there was no recurrence of pancreatitis. Conclusion Acute pancreatitis usually occurs in patients with AJPBD, especially in C-P type or with gallbladder stone or common bile duct stone. ERCP+EST+ENBD and prophylactic cholecystectomy are effective to prevent and treat acute pancreatitis. Key words: Pancreatitis; Anomalous junction of pancreaticobiliary duct; Endoscopic retrograde cholangiopancreatography; Cholecystectomy

Management of Type I Choledochal Cyst in Adult: Totally Laparoscopic Resection and Roux-en-Y Hepaticoenterostomy

Background/objectiveCholedochal 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 excisions. MethodsWe retrospectively studied 45 patients who had undergone laparoscopic choledochal cyst excision in our institutes from September 2006 to August 2009. Data including age, gender, type of cyst, symptoms, surgical technique, conversion rate, morbidity, and mortality were analyzed. ResultsThere were type Ic (cystic) choledochal cysts in 31 patients (68.9%) and type If (fusiform) in 14 patients (31.1%). An anomalous pancreaticobiliary duct junction union was found in 66.7%. Forty percent (18 out of 45) and 37.8% (17 out of 45) cases had stones within the cysts and gallbladders, respectively. The average size of the cysts was 40.3±16.9 cm2. The mean operative time was 307.7±58.0 min, the estimated operative blood loss was 252.3±162.5 ml, and the conversion rate was 8.9%. The mean hospital stay was 8.3±3.2 days. The overall morbidity rate was 17.1%, the reoperation rate was zero, and the mortality rate was also zero. ConclusionsTotally, laparoscopic management of type I choledochal cysts, although technically challenging, is safe and feasible in experienced hands.

Acute Pancreatitis Associated With Anomalous Pancreatobiliary Junction

Acute Pancreatitis Associated With Anomalous Pancreatobiliary Junction

T1569: Periampullary Choledochal Diverticula: Congenital or Acquired?

Bile duct diverticula historically have been classified as Type II choledochal cysts, an uncommon variety. Type II choledochal cysts are typically described as 1-3cm in diameter, arising from the common bile duct near the cystic duct and associated with an anomalous pancreatico-biliary duct junction. Management generally involves surgical resection because of their increased malignant potential. We have also observed, however, patients with small (<1cm) choledochal diverticula just proximal to the ampulla.

An Unusual Variant of Anomalous Pancreaticobiliary Junction in a Patient With Pancreas Divisum Diagnosed With Secretin-Magnetic Resonance Cholangiopancreatography

Anomalous pancreaticobiliary junction is an unusual variant of pancreaticobiliary anatomy of clinical importance because it is associated with increased risk of pancreatitis and, also, for the development of cholangiocarcinoma. We report an unusual variant of anomalous pancreaticobiliary junction, occurring in a patient with pancreas divisum, with an anomalous communication between the dorsal pancreatic and the common bile duct. The patient presented with a distal biliary stricture. This variant anatomy was occult on magnetic resonance cholangiopancreatography but was diagnosed on magnetic resonance cholangiopancreatography that was performed with intravenous secretin administration and further delineated by endoscopic retrograde cholangiopancreatography. The features that allowed the diagnosis to be made and the implications of this diagnosis are described in this report.

Endoscopic diagnosis and treatment of anomalous pancreaticobiliary ductal junction

Objective To evaluate application of endoscopic retrograde cholangiopancreatography （ERCP） in diagnosis and managements of anomalous pancreaticobiliary ductal junction （APBDJ）. Methods Data of APBDJ patients, including oecurence, sub-type characteristics, relationship with concurrent pancreaticobiliary diseases and endoscopic treatment, who underwent ERCP in the past 5 years, were retrospectively reviewed. Results APBDJ was diagnosed in 49 patients, which accounted for 0. 74% of all ERCP cases （6639） in the same period, among whom, there were 27 cases of P-B sub-type, 19 B-P type, and 3 complex type. Gallbladder carcinoma was accompanied in 16 patients, choledochal cyst in 19, chronic pan creatitis in 7, and pancreas divisum in 2, with morbidity rates significantly higher than those in non-APBDJ population （P 〈 0. 01 ）. The gallbladder carcinoma was most commonly seen in P-B patients （14/16）, and choledochal cyst most frequently occurred in B-P type （15/19）. The endoscopic managements applied in APBDJ patients included sphincterotomy in 10 cases, naso-biliary drainage in 16, plastic stenting in 15, metal stenting in 6, and pancreatic stent placement in 2. Conclusion ERCP is a direct and reliable method for the diagnosis of APBDJ, which is commonly complicated with gallbladder carcinoma, choledochal cyst, and chronic pancreatitis. Endoscopic therapy can be considered in accordance with complications. Key words: Cholangiopancreatography, endoscopic retrograde ; Diagnosis ; Therapy ; Anomalous pancreaticobiliary ductal junction

Identification of the transforming activity of Indian hedgehog by retroviral expression screening

To identify novel cancer-promoting genes in biliary tract cancer (BTC), we constructed a retroviral cDNA expression library from a clinical specimen of BTC with anomalous pancreaticobiliary duct junction (APBDJ), and used the library for a focus formation assay with 3T3 fibroblasts. One of the cDNAs rescued from transformed foci was found to encode Indian hedgehog homolog (IHH). The oncogenic potential of IHH was confirmed both in vitro with the focus formation assay and in vivo with a tumorigenicity assay in nude mice. The isolated IHH cDNA had no sequence alterations, suggesting that upregulation of IHH expression may contribute to malignant transformation. Quantitation of IHH mRNA among clinical specimens has revealed that the expression level of IHH in BTC with APBDJ is higher than that in BTC without APBDJ and than in non-cancerous biliary tissues. Our data thus implicate a direct role of IHH in the carcinogenesis of BTC with APBDJ.

Gallbladder cancer: a morphological and molecular update

Gallbladder cancer (GBC) shows a marked geographical variation in its incidence, with the highest figures being seen in India and Chile and relatively low levels in many Western countries. Risk factors for its development include the presence of gallstones, infection and the presence of an anomalous pancreatobiliary ductal junction. It can arise from either a pathway involving metaplasia or dysplasia or one in which there is a pre-existing adenoma. The former is the more common and, because it is often not associated with a macroscopically recognizable lesion, leads to the recommendation that all gallbladders need to be examined microscopically. Accurate staging of invasive cancers is essential to determine prognosis and treatment, and this requires extensive tumour sampling. A number of genetic alterations have been identified in the preinvasive and invasive stages of GBC and they support the morphological evidence of there being two pathways by which tumours develop. Some of these genetic changes are associated with particular risk factors. For example, cases with anomalous pancreatobiliary ductal junction show a higher frequency of K-ras mutations. Some changes are associated with differences in prognosis. For example, cancers without expression of p21 but with expression for p27 have a better prognosis, whereas those that express c-erb-B2 have a worse one. Work has also been done on identifying clinical, imaging and other factors that indicate that patients have a higher risk of having GBC. This is particularly important in high-incidence areas in which GBC is a significant public health problem.

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]).

Gallbladder Cancer: We Need to Do Better!

Gallbladder Cancer: We Need to Do Better!

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.

Anomalous triple junction surface pits in nanocrystalline zirconia thin films and their relationship to triple junction energy

Triple junctions (TJs) are the lines where three grains or grain boundaries meet and become increasingly important in nanocrystalline materials where they have a high areal number density and occupy a significant fraction of the total volume of the material. Surface pits are associated with TJs, just as surface grooves are associated with grain boundaries, and these pits may have particularly deleterious effects on the behaviors of thin films. We evaluate the surface topography associated with TJs in nanocrystalline ZrO 2 thin films using thickness mapping images produced by energy-filtered transmission electron microscopy (EFTEM), and compare our results with theoretical predictions. While many of the pits conform to the standard theoretical treatment, some of them exhibit considerably increased depth, possibly indicating that the junctions have line energy. No pits were observed with less than the theoretically predicted depth.

The ultrasonography study of non-biliary obstruction bile duct dilatation

Objective 62 cases with non-biliary obstruction bile duct dilatation were investigated with ultrasonography and compared with endoscopy in order to explore the etiology of this kind of focal bile ducet dil-atation. Methods The cases with non-biliary obstruction bile duct dilatation diadnosed by high resolution ultra-sound were analyzed retrospectively to reveal the road of cormnom bile duct as clear as possible. Results 45 of the 62 cases were corrected as bile papillitis and bile edema, 12 were choledochitis ,5 were anomalous junction of pancreatobiliary duct. Conclusion The improvement of the ultrasanography knowledge of non-biliary obstruc-tion bile duct dilatation would provide more valid image data for clinic. Key words: non-biliary obstruction; Bile duct dilatation; Bile papillitis disease

Hepatobiliary and pancreatic: Adenomyomatosis of the gallbladder with abscess formation

Adenomyomatosis is a disease of the gallbladder characterized by epithelial proliferation, hypertrophy of the muscularis externa and outpouching of the mucosa into the thickened muscle layer resulting in the formation of Rokitansky-Aschoff sinuses. The disorder may involve the whole of the gallbladder, a segment of gallbladder (often the mid-portion) or a localized area (usually the fundus). The disorder is more common in women than in men (ratio 3:1) and increases in prevalence with advancing age. At least 50% of patients have coexisting gallstones. In resected gallbladder specimens, the frequency of adenomyomatosis is debated but appears to range between 1% and 9%. This variation may reflect the application of different pathological criteria in different studies. The cause of adenomyomatosis remains unclear but may involve increases in intraluminal pressure because of an obstructing calculus or a narrow cystic duct. There are also reports of an association with an anomalous pancreaticobiliary ductal junction. Thus far, there is no persuasive evidence of an association between adenomyomatosis and gallbladder cancer. In the patient described below, adenomyomatosis appeared to be responsible for the development of a small abscess in the gallbladder wall. This may be the first report of this complication in the medical literature. The patient was a 78-year-old woman with primary biliary cirrhosis (Scheuer stage 4) who was admitted to hospital because of abnormalities on abdominal imaging. Changes on a contrast-enhanced computed tomography (CT) scan are shown in Figure 1. The left panel shows a spherical lesion in the fundus of the gallbladder (arrowhead) while the right panel shows a more heterogeneous lesion with some enhancement by contrast (arrow). With magnetic resonance imaging, cystic structures were shown within the gallbladder lesion and these structures had high signal intensity on T2-weighted images. Serum levels of inflammatory markers and tumor markers including carbohydrate antigen 19.9 were within the reference range. The patient was treated by cholecystectomy because of the possibility of a malignant polyp. A macroscopic examination of the resected specimen showed a round tumor with apparently normal mucosa and a central dimple. Histological evaluation revealed adenomyomatosis with abscess formation but no malignancy (Figure 2). An inflammatory cell infiltrate, largely composed of neutrophils, was observed in the thickened gallbladder wall and in the Rokitansky-Aschoff sinuses (right panel).

Prognostic Factors for Gallbladder Cancer in Japan

The objective of this study was to evaluate prognostic predictors for patients with gallbladder cancer (GBC) in a Japanese nationwide data base. GBC is the most common cancer of the biliary tract in Japan. Differences in the survival rates between Japan and other countries have been noted. The authors analyzed 4424 patients with GBC in Japan between 1988 and 1997. Staging was determined in accordance with American Joint Committee on Cancer stage. Survival is related closely to the surgical stage. Five-year survival rates for stage I, II, III, IVA, and IVB (5th edition) were 83%, 70%, 45%, 23%, and 9%, respectively. These differences were significant (P < 0.0001). The survival rate for patients aged <60 years was significantly better (P < 0.05). The survival rate for patients aged >69 years was significantly worse (P < 0.01). The cholecystectomy plus combined resection of bile duct and/or liver bed resection had an effect on prolonging the survival in stage II or III disease, but extended resection did not. The patients with anomalous pancreaticobiliary ductal junction had a survival advantage over those with cholelithiasis by univariate analysis. However, multivariate analyses indicated that only age, sex, stage, operative procedures were independent prognostic factors. Stage was the strongest covariate; patients diagnosed with stage II, III, IVA, or IVB disease were 2.2, 4.2, 8.1, and 13.6 times, respectively, were more likely to die. Staging is the strongest prognostic factor for GBC, but patient outcomes were also affected by age, sex, and operative procedures. The data do not support any advantage for extended resection. Neither gallstones nor anomalous pancreaticobiliary ductal junction influenced the GBC patient outcome.

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.

Unusual fusion between ventral and dorsal primordia causes anomalous pancreaticobiliary junction

Anomalous pancreaticobiliary junction (APBJ) is a congenital anomaly in which the pancreatic duct joins the common bile duct proximal to the sphincter of Oddi. Anatomical and immunohistochemical examination of the pancreas with APBJ has rarely been performed. A 72-year-old woman with gallbladder cancer and APBJ died of respiratory failure. Macroscopic features of the pancreas were examined in detail. Immunohistochemistry using anti-pancreatic polypeptide (anti-PP) antibody was done to discriminate ventral and dorsal pancreas. Macroscopically the inferior part of the head of the pancreas was smaller than normal. The posterior surface of the head was obliquely grooved. Part of the pancreatic head protruded into the posterior side of the pancreatic head. A PP-rich region was located in the superioposterior position of the pancreas head. Considering the relationship between the ventral and dorsal pancreas, it was inferred that the ventral primordium could obliquely fuse with the dorsal primordium during embryological development. As a result, APBJ occurs through an abnormal fusion between ventral and dorsal primordia.

Ball-valve gastric tumor associated with anomalous junction of the pancreatico-biliary ductal system and a right-sided round ligament: Report of a case

We report the case of a ball-valve gastric tumor associated with anomalous junction of the pancreatico-biliary ductal system (AJPBDS) and a right-sided round ligament, misdiagnosed preoperatively as advanced gastric cancer with pancreatic head invasion. A 72-year-old woman presented with chest pain, but laboratory data showed only anemia. Gastroscopy revealed a bleeding polypoid gastric tumor in the anterior wall of the stomach, herniating into the duodenum (ball-valve syndrome), and a Bormann type-2 tumor in the posterior wall. Ultrasonography showed gallbladder stones, dilatation of the intrahepatic bile duct and pancreatic duct, and a left-sided gallbladder (attributed to a right-sided round ligament with anomalous branches of the portal veins). Laparotomy revealed that the gastric tumors were not advanced cancer invading the pancreatic head. Intraoperative cholangiography showed an AJPBDS, causing dilatation of the intrahepatic bile duct and pancreatic duct. We performed distal gastrectomy and cholecystectomy without biliary diversion. Microscopy revealed that the polypoid tumor was a hyperplastic polyp.

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.