Clinical relevance of an unfused pancreatic duct system.

In man, the main pancreatic duct is normally derived from ventral and dorsal embryological buds of the pancreas. In a minority of people, failure of fusion of the two buds results in separate drainage of the dorsal and ventral pancreas, so that the accessory duct provides the main drainage for the gland. Patients with this anomaly demonstrated at endoscopic retrograde pancreatography (ERP) have been investigated to assess whether non-fusion of the main pancreatic duct predisposes to the development of pancreatitis. A failure of fusion of the pancreatic ducts was seen in 21 out of 449 (4.7%) successful pancreatograms; four of these 21 patients had definite clinical evidence of pancreatitis and two patients had possible pancreatic disease, but in the remainder the anomaly was not considered to be clinically relevant. An abnormal pancreatogram suggesting pancreatitis was present in 116 out of the 428 patients (27.1%) with a normally fused duct system. The anomaly was found as frequently in the whole series as it was seen in patients with pancreatitis. These findings suggest that embryological failure of pancreatic duct fusion does not predispose to the development of pancreatitis. However, the presence of this anomaly may lead to misinterpretation of ultrasonographic and CT scan findings.     

Anomalous pancreaticobiliary duct union associated with bile duct carcinoma

An abnormal high union of the common bile duct and the main pancreatic duct, without accompanying cystic dilatation of the bile ducts, is a rare occurrence. A case of obstructive jaundice due to bile duct carcinoma in connection with this anomaly is reported.     

A case of cholecystohepatic duct with atrophic common hepatic duct

BackgroundCholecystohepatic ducts are rare congenital variants of the biliary tree.Case outlineAn 81-year-old woman presented with biliary colic and elevated liver function tests. An ERCP demonstrated a common bile duct stone and stricture of the common hepatic duct. An operative cholangiogram demonstrated an atrophic common hepatic duct and retrograde filling of the gallbladder through a large cholecystoheptic duct. The patient had a cholecystectomy and reconstructive cholecystohepatic duct jejunostomy.DiscussionThis case demonstrates a rare congenital anomaly where the gallbladder fills retrograde during an intraoperative cholangiogram despite clipping of the cystic duct. The major path of biliary drainage was through a large cholecystoheptic duct similar to a gallbladder interposition; however, the common hepatic duct was still present but atrophic. This anomaly has not been described previously.     

Double common bile duct： A case report

Double common bile duct （DCBD） is a rare congenital anomaly in which two common bile ducts exist. One usually has normal drainage into the papilla duodeni major and the other usually named accessory common bile duct （ACBD） opens in different parts of upper gastrointestinal tract （stomach, duodenum, ductus pancreaticus or septum）. This anomaly is of great importance since it is often associated with biliary lithiasis, choledochal cyst, anomalous pancreaticobiliary junction （APBJ） and upper gastrointestinal tract malignancies. We recently recognized a rare case of DCBD associated with APB3 with lithiasis in better developed common bile duct. The opening site of ACBD was in the pancreatic duct. The anomaly was suspected by transabdominal ultrasonography and finally confirmed by endoscopic retrograde cholangiopancreatography （ERCP） followed by endoscopic sphincterotomy and stone extraction. According to the literature, the existence of DCBD with the opening of ACBD in the pancreatic duct is most frequently associated with APB3 and gallbladder carcinoma. In case of DCBD, the opening site of ACBD is of greatest clinical importance because of its close implications with concomitant pathology. The adequate diagnosis of this rare anomaly is significant since the operative complications may occur in cases with DCBD which is not recognized prior to surgical treatment.     

Cancer of the extrahepatic biliary tracts and abnormality of the biliopancreatic junction

This retrospective study was undertaken to evaluate the incidence of an abnormal pancreaticobiliary ductal union (long common duct) in biliary tract carcinoma. Of 86 patients presenting with such a cancer, 67 had a pre- or intra-operative radiologic examination. An abnormal pancreaticobiliary ductal union was found in only one 60 year old woman having stones associated with invasive gallbladder carcinoma. Operative cholangiogram revealed an early and marked Wirsung opacification with a 1.5 cm long common duct. This low incidence in our study contrasts deeply with Japanese data and raises several questions about the correct evaluation of pancreaticobiliary ductal union, the true consequences of such an anomaly and possible interracial variations in incidence.     

Familial Occurrence of Anomalous Pancreaticobiliary Duct Union Associated with Gallbladder Neoplasms

The authors present two cases of anomalous pancreaticobiliary duct union that occurred in a mother and her daughter; the former was associated with gallbladder adenocarcinoma, and the latter, gallbladder adenoma with high grade dysplasia. This disorder has been considered a developmental anomaly, although the true etiology has not been known. This is the first report of familial occurrence of this anomaly, and it may point to the presence of a genetic factor in its development. Another implication is the presence of histological features which suggest the possible sequence of anomalous pancreaticobiliary duct union to intestinal metaplasia to adenoma or dysplasia to cancer of the gallbladder.     

Anomalous pancreaticobiliary ductal junction without bile duct dilatation in gallbladder cancer

BACKGROUND/AIMS: Anomalous pancreaticobiliary junction is a rare anomaly but is a risk factor for primary carcinoma of the gallbladder. To define the relationship between anomalous pancreaticobiliary junction, especially if it is not associated with common bile duct dilatation, and gallbladder carcinoma, we retrospectively reviewed data of 126 patients with gallbladder carcinoma. METHODOLOGY: All these patients had undergone direct cholangiography either by endoscopic retrograde cholangiopancreaticography or percutaneous transhepatic cholangiography. RESULTS: Among 126 patients with gallbladder cancer, 23 patients (18.3%) exhibited anomalous pancreaticobiliary junction. Patients with anomalous pancreaticobiliary junction were younger (mean age: 54 +/- 9.1 years) than patients without anomalous pancreaticobiliary junction (mean age: 65 +/- 9.7 years). The incidence of gallstones in patients with anomalous pancreaticobiliary junction (17%) was significantly lower than in those without this anomaly (64%) (P < 0.01). Among the 23 patients with anomalous pancreaticobiliary junction, 12 patients (52%) had no bile duct dilatation and, 11 patients (48%) had bile duct dilatation in the form of fusiform or cylindrical dilatation. However, no cases with severe cystic dilatation were found. Patients of anomalous pancreaticobiliary junction without common bile duct dilatation had more advanced disease and poor prognosis than those with common bile duct dilatation. CONCLUSIONS: The present study revealed that gallbladder cancer in the patients of anomalous pancreaticobiliary junction without common bile duct dilatation was diagnosed at advanced stage and the prognosis was very poor. Therefore, if a minor abnormality is detected in the wall of acalculous gallbladder on ultrasonography, direct cholangiography should be done to exclude this anomaly.     

Obstructing Pseudocyst of the Duct of Santorini in Pancreas Divisum

Pancreas divisum is a pancreatic duct anomaly that occurs due to failure of fusion of the dorsal and ventral ducts. While recognition of this anomaly is increasing due to more aggressive endoscopic retrograde cholangiopancreatography, its significance remains unclear. A patient with chronic pancreatitis and a history of alcohol abuse was noted to have pancreas divisum. At surgical exploration, intraoperative pancreatography revealed an obstructing pseudocyst of the duct of Santorini. Extended sphincteroplasty and cystduodenostomy as well as Roux-en-Y pancreatojejunostomy were necessary to insure adequate accessory duct drainage. Surgical therapy of pancreas divisum in chronic pancreatitis should be designed to correct existing pancreatic duct obstruction.     

Preduodenal position of the common bile duct associated with annular pancreas: case report and literature review

A preduodenal position of the common bile duct (PDCBD) is a very rare embryological anomaly. Our patient, a 1-day-old boy, was diagnosed to have PDCBD, annular pancreas, duodenal stenosis, intestinal malrotation and a ventricular septal defect associated with 21 trisomy. Initially the fetal diagnosis was duodenal atresia. The diagnosis of duodenal stenosis was made on birth, and he underwent an emergency operation 24 hours later. The PDCBD was discovered during the duodenoduodenostomy procedure. While a transversal incision was being made for the ventral wall in the distended proximal duodenum, the common bile duct was accidentally cut. We found the common bile duct to run into the ventral side of the duodenal wall. Subsequently, the common bile duct was repaired with a stent using microscopic techniques. The case is being reported because of the rarity of this condition and also to stress the importance of accurately recognizing this anomaly when treating the concomitant diseases, particularly when surgical implications are involved.     

Trifidum anomaly of the main pancreatic duct

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

Anomalous connection of the right hepatic duct into the cystic duct: utility of magnetic resonance cholangiopancreatography

A 41-year-old female was admitted to our hospital for treatment of uterus carcinoma. Abdominal ultrasound showed gallbladder stones. Although magnetic resonance cholangiopancreatography revealed the right intrahepatic bile ducts, left hepatic duct and the common bile duct, the confluence of the right and left hepatic ducts was not visualized. At surgery, intra-operative cholangiography showed a biliary anomaly of the right hepatic duct entering the cystic duct. Subsequently cholecystectomy was accomplished without any injury to the bile duct. Our case may be the eighth such case of this rare biliary anomaly. When magnetic resonance cholangiopancreatography does not show the confluence of the right and left hepatic ducts, biliary anomaly of the right hepatic duct should be suspected and careful dissection should be performed from the Hartman's pouch, followed by intraoperative cholangiography, in order to avoid unnecessary injury to the bile duct.     

Gallbladder agenesis with a stone in the cystic duct bud

An 84-year-old woman was admitted to the hospital because of pyloric stenosis caused by gastric cancer. Abdominal computed tomography and magnetic resonance imaging failed to demonstrate the gallbladder, but showed a gallstone in a ductlike structure parallel to the common bile duct. When laparotomy was performed, the gallbladder and the fossa were not observed, and a blind-end duct, similar to a cystic duct, was found beside the common bile duct. Incisional exploration of the common bile duct was done after distal gastrectomy; the gallstone was not found in the common bile duct, but in the duct parallel to it. By observing the duct beneath the common bile duct with a cholangioscope, we considered it to be a hypoplastic cystic duct. After the gallstone was removed, a T-tube was placed into the common bile duct. Agenesis of the gallbladder is a rare congenital anomaly and is often asymptomatic. As far as we know, this is the first report of gallbladder agenesis with a hypoplastic cystic duct impacted with a stone. Careful intraoperative examination using a cholangioscope is useful to confirm the structure of the common bile duct.     

Cystic duct directly joining the main pancreatic duct

A unique anomaly of the direct union between the cystic duct and the main pancreatic duct is presented. A 19-year-old man with a history of repeated epigastralgia underwent endoscopic retrograde cholangiopancreaticography that showed a direct union between the cystic duct and the main pancreatic duct. No pancreaticobiliary maljunction was noticed. Cholecystectomy accompanied by resection of the long cystic duct was performed. The excised gallbladder showed cholesterolosis, chronic cholecystitis, and hyperplasia of the pseudopyloric glands microscopically. The patient has been well for 3 years since surgery.     

Ectopic opening of the common bile duct in the duodenal bulb: clinical implications

BACKGROUND: An ectopic opening of the common bile duct in the duodenal bulb is extremely rare, and the clinical significance of this anomaly has not been clarified. This study analyzed the clinical implications and cholangiographic findings of this anomaly. METHODS: A total of 18 patients (15 men, 3 women; median age, 51 years) with an ectopic opening of the common bile duct in the duodenal bulb were studied. Medical records, endoscopic findings, and cholangiographic and other radiographic findings were reviewed. RESULTS: All 18 patients had biliary pain; 7 had fever and chills. Fifteen (83%) had diffuse dilatation of the extrahepatic bile ducts with or without intrahepatic bile duct dilation. Associated bile duct stones were found in 10 (56%) patients. The papilla in the bulb had an orifice stained with bile at endoscopy, which was associated with duodenal ulcer disease found in 13 (72%) patients. The distal end of the common bile duct was tapered and narrowed and had a hook shape in all patients. CONCLUSION: An ectopic opening of the common bile duct in the duodenal bulb may be associated with clinical entities such as recurrent or intractable duodenal ulcer, choledocholithiasis, or acute cholangitis. Although these openings are rare, knowledge of the endoscopic and radiographic findings of an ectopic opening of the common bile duct in the duodenal bulb may help to clarify the cause of chronic recurrent duodenal ulcer disease in some patients and prevent damage to the bile duct during surgery.     

Mucosal cell proliferation activity of the gallbladder in children with anomalous arrangement of the pancreaticobiliary duct

Anomalous arrangement of the pancreaticobiliary duct (AAPBD) is an anatomical maljunction of the bile duct and the pancreatic duct that is frequently associated with gallbladder carcinoma. In patients with AAPBD, it has been postulated that pancreatic juice regurgitates into the biliary tree, and the mixture of refluxed pancreatic juice and stagnant bile juice acts as an irritant factor to the biliary tract epithelium, leading to chronic inflammation and metaplasia. Eventually these mucosal changes may progress to invasive carcinoma. We reviewed clinicopathologic studies on epithelial changes of the gallbladder in patients with AAPBD to clarify the implications relevant to carcinogenesis. Conventional histological studies have shown that the most characteristic change observed in the gallbladder of children with this anomaly was epithelial hyperplasia. Furthermore, the incidence of mucosal hyperplasia was significantly increased in the gallbladder of children in whom the pancreatic duct joined the common bile duct (P-C type) compared with the incidence in children in whom the common bile duct joined the pancreatic duct (C-P type). In addition, cell kinetic studies have demonstrated increased cellular proliferative activity of the gallbladder in children with AAPBD. Cell proliferative activity was significantly elevated in children with the P-C type of AAPBD compared with that in children with the C-P type of anomaly. In conclusion, AAPBD may yield increased cell proliferation in the gallbladder of patients with this anomaly in early childhood, resulting in epithelial hyperplasia. Although it remains unknown which agents are responsible for promoting the activation of cellular function, it seems that bile acids and refluxed pancreatic proteases are likely play a role in such promotion. Further investigations are needed to elucidate the mechanism of increased cellular function.     

A case of intrahepatic cholangiocarcinoma associated with Type IV choledochal cyst

Anomalous union of the pancreaticobiliary duct (AUPBD) is a congenital anomaly that is defined as a junction of the bile duct and pancreatic duct outside the duodenal wall. This anomaly results in a loss of normal sphincteric mechanisms at the pancreaticobiliary junction. As a result, regurgitation of pancreatic juice into the biliary system develops and causes choledochal cysts, choledocholithiasis, cholangitis, pancreatitis and malignancy of the biliary tract. Gallbladder cancer or common bile duct cancer associated with AUPBD and choledochal cysts have been frequently reported. But, intrahepatic cholangiocarcinoma associated with this condition has been only rarely reported. Here, we report a case of intrahepatic cholangiocarcinoma associated with AUPBD and choledochal cyst.     

Alder anomaly accompanied by a mutation of the myeloperoxidase structural gene

In this paper, a third form of Alder anomaly is described. In addition to the morphological changes which are characteristic of this anomaly, the peroxidase of the neutrophil polymorphonuclear leukocytes (NPNL) is abnormal in its properties. It is resistant to the fixation step in the May-Grünwald Giemsa procedure and is similar, therefore, to the eosinophil peroxidase. In the phenotype observed, 2 genetic changes are probably involved: one is a change in the regulation of NPNL granulation, the other is a mutation in the myeloperoxidase structural gene. In the light of these new findings, 3 forms of the anomaly are now known: the original Alder anomaly, in which the abnormal granulation, particularly in the granulocytes, is not linked with any hereditary disorder; the Alder-Reilly form, in which in addition to the abnormal granulation the leukocytes also contain inclusion bodies, the phenomenon sometimes being associated with mucopolysaccharidosis (gargoylism), and the third form of anomaly described here, where-apart from the abnormal granulation-the myeloperoxidase behaves differently, resembling that of eosinophils in its resistance to methanol fixation.     

Anomaly of the portal vein with an anomalous hepatic vein--the first case report

An anomaly of the portal vein associated with an anomalous hepatic vein is described as the first reported case. A 44-year-old woman was incidentally found to have a huge hemangioma by ultrasonography. Computed tomography revealed an anomalous portal system with a normally located gallbladder and round ligament. Arterial portography revealed anomalous branching of the portal vein, with absence of the left umbilical portion, the curved right portal branch mimicked the right-sided umbilical portion. Hepatic venography demonstrated patent umbilical veins communicating with the left hepatic vein. Intraoperatively the hepatic vein branch was found on the surface of the right hepatic lobe and the common bile duct was dorsal to the hepatic artery. A limited excision of the involved liver was performed safely with the help of the preoperative definition of the abnormal liver anatomy.     

Common Bile Duct Duplication: The more the murkier

Congenital duplication of the common bile duct is an extremely rare anomaly of the biliary tract, which putatively represents failure of regression of the embryological double biliary system. Depending on the morphology of the duplicated bile duct, the anomaly can be classified into five distinct subtypes as per the modified classification (proposed by Choi et al). Among the five subtypes of bile duct duplication, type V duplication is considered to be the least common with only two previous cases of type Va variant reported in medical literature prior to the current report.     

Double duct-to-mucosa pancreaticojejunostomy for bifid pancreatic duct

We report a pancreaticojejunostomy with double duct-to-mucosa anastomotic technique after pyloruspreserving pancreaticoduodenectomy for chronic pancreatitis with bifid pancreatic duct. A 49-year-old Japanese man was diagnosed preoperatively as having chronic pancreatitis with common bile duct stricture and pseudocyst of the pancreatic head. In a pancreaticoduodenectomy, the main pancreatic duct (7mm in diameter) and a secondary pancreatic duct (4mm in diameter) were identified intraoperatively at the transected surface. Pancreatography showed the main pancreatic duct as well as thesecondary pancreatic duct that drained the remaining dorsal pancreas, allowing us to diagnose bifid pancreatic duct. The pancreaticojejunostomy was performed in an end-to-side manner to create double duct-to-mucosa anastomoses and to approximate the pancreatic parenchyma and jejunal seromuscular layers. Although bifid pancreatic duct is a rare anatomical anomaly, it behooves every surgeon who performs pancreatic resections to be aware of this entity and the techniques for dealing with it.