Relationship between pancreaticobiliary maljunction and gallbladder carcinoma: meta-analysis.

BACKGROUND: Reports on the relationship between pancreaticobiliary maljunction (PBM) and gallbladder carcinoma (GBC) are conflicting. The frequency of PBM in GBC patients and the clinical features of GBC patients with PBM vary in different studies. DATA SOURCES: English-language articles describing the association between PBM and GBC were searched in the PubMed and Web of Science databases. Nine case-control studies fulfilled the inclusion criteria and addressed the relevant clinical questions of this analysis. Data were extracted independently by two reviewers using a predefined spreadsheet. RESULTS: The incidence of PBM was higher in GBC patients than in controls (10.60% vs 1.76%, OR: 7.41, 95% CI: 5.03 to 10.87, P<0.00001). The proportion of female patients with PBM was 1.96-fold higher than in GBC patients without PBM (80.5% vs 62.9%, OR: 1.96, 95% CI: 1.09 to 3.52, P=0.12). GBC patients with PBM were 10 years younger than those without PBM (SMD: -9.90, 95% CI: -11.70 to -8.10, P<0.00001). And a difference in the incidence of associated gallstone was found between GBC patients with and without PBM (10.8% vs 54.3%, OR: 0.09, 95% CI: 0.05 to 0.17, P<0.00001). Among the GBC patients with PBM, associated congenital dilatation of the common bile duct was present with a higher incidence ranging from 52.2% to 85.7%, and 70.0%-85.7% of them belonged to the P-C type of PBM (the main pancreatic duct enters the common bile duct). No substantial heterogeneity was found and no evidence of publication bias was observed. CONCLUSIONS: PBM is a high-risk factor for developing GBC, especially the P-C type of PBM without congenital dilatation of the common bile duct. To prevent GBC, laparoscopic cholecystectomy is highly recommended for PBM patients without congenital dilatation of the common bile duct, especially relatively young female patients without gallstones.

Congenital common bile duct web in association with hepatobiliary pancreatic ductal anomalies.

Congenital webs are extremely rare anomalies of the extrahepatic ductal system. As the vast majority of such cases are asymptomatic, detection is usually incidental during surgery for some other cause. In a young boy presenting with features of cholangitis, a congenital common bile duct web was discovered on T-tube cholangiogram. Further anomalies of the intrahepatic and pancreatic ductal systems were also detected. Since all known causes of acquired web formation were excluded, a congenital origin of the web was assumed and the patient is continuing to do well after a follow-up of 22 months.

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.

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 APBJ 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 APBJ 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.

Congenital biliary dilatation in dizygotic twins.

Congenital biliary dilatation (CBD) of different types was recently noted in dizygotic twins. Our cases suggest the possibility of hereditary involvement in CBD. On the other hand, CBD discordance in six sets of monozygotic twins has been reported, which would suggest that the occurrence of CBD is not compatible with single gene control, although environmental factors are plausible. To evaluate the genetic factors that may be implicated in CBD, it would be necessary to accumulate more familial cases and examine further studies on inheritance.

Congenital hepatic fibrosis: CT findings in 18 adults.

PURPOSE: To evaluate the computed tomographic (CT) findings in adult patients with pathologically proved congenital hepatic fibrosis. MATERIALS AND METHODS: This was a retrospective review of congenital hepatic fibrosis cases identified at two institutions over the course of 8 years. Eight men and 10 women with an age range of 22-72 years (mean age, 39 years) were included. Contrast material-enhanced and unenhanced CT scans were obtained through the liver in all patients. Two radiologists evaluated size of and morphologic findings (atrophy or hypertrophy localized according to hepatic segments) in the liver; increased diameter or number of hepatic arteries at the hilum; presence of hepatic nodules, varices, spontaneous splenorenal shunts, and splenomegaly; and association with other hepatic ductal plate malformations and renal abnormalities. RESULTS: Sixteen patients had morphologic abnormalities in the liver, 15 had splenomegaly (three underwent splenectomy for portal hypertension), and 14 had varices or spontaneous splenorenal shunts. An enlarged hepatic artery and a tangle of abnormally enlarged arterial vessels were identified in five and four patients, respectively, and four of these nine patients had large benign regenerative nodules. Ten patients had renal abnormalities and nine had an associated ductal plate malformation. CONCLUSION: This retrospective study shows that certain findings (ie, liver morphologic and associated ductal plate abnormalities, varices, splenomegaly, and renal abnormalities) are frequently observed in combination in patients with congenital hepatic fibrosis.

Studies on biliary tract carcinoma in the case with pancreaticobiliary maljunction.

BACKGROUND/AIMS: The purpose of this study was to clarify the clinicopathological features of pancreaticobiliary maljunction and to determine the appropriate surgical approach for biliary tract with pancreaticobiliary maljunction. METHODOLOGY: The data of 77 patients with pancreaticobiliary maljunction including 13, who had been treated for biliary tract cancer, were reviewed retrospectively. We assessed the clinical features, biological characteristics of the cancer, methods of surgical treatment, postoperative outcome and cell proliferating activity of the biliary epithelium, evaluated by the PCNALI (proliferating cell nuclear antigen-labeling index). RESULTS: The incidence of cancer development in the case with pancreaticobiliary maljunction was 13.4% in the bile duct dilatation group (n = 67) and 40.0% in the non-dilatation group (n = 10). Dissection of lymphadenectomy was performed in 10 (76.9%) of 13 patients, and curative resection was feasible in 9 of the 10 patients. Two (20.0%) of the 10 patients had lymph node involvement noted at surgery and died of recurrence. In the other eight patients without lymph node involvement at surgery, six patients underwent curative resection and are alive at 7 months to 11 years and 6 months after surgery. PCNALI of the biliary epithelium of the patients with pancreaticobiliary maljunction was significantly higher than that of the control group. CONCLUSIONS: For patients with pancreaticobiliary maljunction, it should be stressed that the extrahepatic bile duct be prophylactically removed, even when there are no neoplasmatic changes because of high prevalence of cancer development, presumably predicted by the increase of cell proliferative activity in the biliary epithelium. For patients with biliary cancer, early detection at the stage with no lymph node involvement is essential to secure for long-term survival.

Double cancer of gallbladder and bile duct associated with anomalous junction of the pancreaticobiliary ductal system.

We report a case of double cancer of the gallbladder and the common bile duct associated with anomalous junction of the pancreaticobiliary ductal system, and review the literature of similar case reports. A 66-year-old woman was admitted to an associated hospital complaining of upper abdominal pain, and was diagnosed as having pancreatitis. Abdominal imaging revealed an irregularly protruding mass at the body of the gallbladder and an intraluminal protrusion at the lower third of the common bile duct. Endoscopic retrograde cholangiopancreatography also revealed anomalous junction of the pancreaticobiliary ductal system with congenital biliary dilatation of 14 mm in the largest diameter. She underwent surgical resection of the gallbladder, the extrahepatic bile duct and the gallbladder bed of the liver with a dissection of the regional lymph nodes for double cancer of the gallbladder and the bile duct associated with anomalous junction of the pancreaticobiliary ductal system. She is still alive 33 months after surgery without any signs of recurrence. There were 12 patients (including our case) reported in the literature who had double cancer of the gallbladder and the extrahepatic bile duct associated with anomalous junction of the pancreaticobiliary ductal system. Only 33% of these 12 patients had jaundice. Tumors of the 12 patients were commonly early-stage cancer both in the gallbladder (36%) and in the extrahepatic bile duct (73%). Therefore, we concluded that precise preoperative imaging of the total biliary tract should be required in order to detect early-stage cancer in patients with anomalous junction of the pancreaticobiliary ductal system before planning surgical procedures, and consideration should be given to the possibility of multiple occurrences of biliary tract cancers.

Quiste coledociano. Descripción de tres casos / Coledochal cyst. Description of three cases

Los quistes coledocianos so dilataciones congénitas del conducto biliar común; su incidencia es muy baja; se han reportado más de 500 casos, de los cuales,, la tercera parte se han publicado en el Japón. La triada clásicadde ictericia, dolor y tumoración se evidencia en menos de un tercio de los niños, las complicaciones más frecuentes son colangitis, obstrucción duodenal y pancreatitis. El diagnóstico de los quistes se lo hace con ecografía, colangiografía intravenosa, tomografía axial computarizada o centellografía. El tratamiento es quirúrgico Exéresis del quiste y hepatiyeyunostomía en Y de Roux. En el presente trabajo se describen tres casos de quiste coledociano en pacientes pediátricos, con edades comprendidas entre tres y diez años, atendidos en el Servicio de Pediatría del "Hospital Santa Cruz", Caja Petrolera de Salud, en el lapso de meses, que acudieron a la consulta por presentar dolor intermitente en cuadrante abdominal superior derecho, sediagnosticaron los tres quistes por ecografía. El tratamiento realizado en los tres casos fue el de la quiatectomía y hepaticoyeyunostomía en Y de Roux. La evolución postoperatoria fue favorable, no evidenciandose ninguna complicación en los controles posteriores (seguimiento de dos años)

Current problems with intrahepatic bile duct stones in Japan--congenital biliary malformations as a cause.

BACKGROUND/AIMS: In patients with primary intrahepatic bile duct stones, strictures of the biliary duct are often present, but the relationship between these strictures and the formation of the stones remains controversial. Intrahepatic bile duct carcinoma in association with intrahepatic bile duct stones has recently been reported. The present study attempted to ascertain whether bile stasis induced by congenital biliary strictures is the basis for the formation of stones and occurrence of carcinoma. MATERIALS AND METHODS: We analyzed the location of strictures in 58 patients with strictures in the upper portion of the biliary tract including 38 patients with intrahepatic bile duct stones and 9 with intrahepatic bile duct carcinoma. The cell cycle of epithelial cells from the intrahepatic bile duct were analyzed with using proliferating cell nuclear antigen, which is a immunohistochemical staining method. RESULTS: Fifty six of 58 patients had congenital cystic dilatation of the common bile duct (two infant type and 54 adult type). Thirty eight patients had intrahepatic bile duct stones proximal to the strictures at the hepatic hilum. The location of the strictures were classified into four types. Nine patients had intrahepatic bile duct carcinoma and eight of the 9 carcinomas coexisted with intrahepatic bile duct stones. In the nine patients with intrahepatic bile duct carcinoma, the expression of proliferating cellular nuclear antigen (PCNA) in the carcinoma and the normal bile duct epithelium adjacent to the carcinoma was higher than that of patients with hepatocellular carcinoma without anomaly of the biliary duct. CONCLUSION: Considering the location of the strictures and clinical features, the strictures may have been formed congenitally. Furthermore, adult type cysts of the common bile duct with strictures in the upper portion of the biliary tract are thought to be the basis for the formation of primary intrahepatic bile duct stones. The most appropriate treatment for intrahepatic bile duct stones is thus suggested to be removal of the affected hepatic segment including the region of strictures, combined eventually with hepaticoenterostomy.

[Congenital cystic dilatation of the common bile duct. Radio-anatomical correlations in 14 patients]. / Dilatation kystique congénitale de la voie biliaire principale. Corrélations radio-anatomiques chez 14 malades.

OBJECTIVES: The aim of this study was to compare imaging and pathological results of congenital cystic enlargement of the biliary tract to determine the best preoperative management strategy. PATIENTS AND METHODS: Radiological findings of 14 cases treated by surgery were reviewed. Radiological examinations were reviewed: ultrasound (n = 20), computed tomography (n = 13), endoscopic ultrasound (n = 8), endoscopic retrograde cholangiopancreatography (n = 10), percutaneous transhepatic cholangiography (n = 3), peroperative cholangiography (n = 11). Imaging and surgical or pathological correlations were obtained with regard to topographical type using Todani's classification, pancreatobiliary junction, and associated diseases, especially biliary malignancies (cystic wall and gallbladder). RESULTS: Cystic enlargement of the biliary tract was type Ia in 2 patients, type Ib in 1, type Ic in 4, type IVa in 5, and type IVb in 2. The radio-pathological correlation was excellent for the topographical type, and quite good for intrahepatic extension. An abnormal pancreatobiliary junction was identified in 5 cases, and visualized before surgery in I case. This junction was not opacified pre- or pre-operatively in 7 cases. Gallbladder stones were present in 2 cases, choledocal stones, in 2 cases, and intrahepatic stones in one cases, always seen on ultrasound. Malignant degeneration was present in the cyst in one case in the pathological specimen, but was not visualized by imaging procedures or peroperatively; one intrahepatic degeneration was visualized on CT and histologically proven in the surgical specimen. CONCLUSION: Ultrasound and CT allow positive diagnosis of cystic enlargement of the biliary tract, and diagnosis of intrahepatic cyst and associated diseases. The bifurcation extension and the study of pancreatobiliary junction require peroperative or retrograde cholangiography.

[Treatment of congenital dilatation of the bile ducts. Apropos of 3 cases]. / Traitement des dilatations congénitales de la voie biliaire. A propos de trois cas.

Congenital dilatation of the bile duct is an uncommon lesion requiring surgical treatment. Complete excision of the diseased bile duct prevents development of carcinoma of the bile duct. We report three cases of congenital dilatation of the biliary three, each illustrating a particular aspect of the therapeutic strategy. One patient had an unusual anomaly of the pancreaticobiliary junction that required pancreaticoduodenectomy. Another patient developed carcinoma of the bile duct 15 years after an internal derivation of a choledochal cyst. The last patient had dilatations of the common and intrahepatic bile ducts associated with congenital hepatic fibrosis.

[Congenital dilatation of the common bile duct in children. Study of a series of 52 cases]. / Dilatation congénitale de la voie biliaire principale chez l'enfant. Etude d'une série de 52 cas.

OBJECTIVE: The aim of this study was to evaluate results at long-term follow-up of patients suffering from congenital dilatation of the bile duct according to the type of operative procedure. METHOD: A retrospective study of 52 patients who were operated on during a 25-year period was carried out. Of 52 patients, 43 (83%) were female and 9 (17%) were male, with a ratio of 4.8/1. In six cases, the diagnosis was made antenatally by ultrasonography. Transhepatic cholangiography showed an extrahepatic dilatation in 25 cases (48%) and a combined extra and intrahepatic dilatation in 27 cases (52%). A common bilio-pancreatic channel was found in 25 patients. Mean age at the time of operation was 4.2 years. Operative procedures included: cyst excision and hepaticojejunostomy in 47 cases, cystojejunostomy or cystoduodenostomy in 4 cases and cystostomy in one case. There were two postoperative deaths. RESULTS: Long-term follow-up was established in 43 of 50 survivors (80%). One patient was reoperated because of portal hypertension. Cholangitis developed in 11 patients: 2/2 patients who had undergone in internal drainage and 9/41 patients who had undergone a cyst excision with hepaticojejunostomy. In patients with cyst excision, cholangitis developed twice as frequently in those with associated intrahepatic dilatation. Two of 11 children with recurrent cholangitis died following sepsis, and three others were reoperated because of intrahepatic bile duct stones, one of these 15 years later. CONCLUSION: A total excision of the extrahepatic biliary tract, followed by hepaticojejunostomy is considered as a treatment of choice. The site of the biliary anastomosis should be adapted to the type of dilatation of intrahepatic bile duct in the case of a combined extra and intrahepatic dilatation.

[Congenital cystic dilatation of the choledochus: apropos of a case diagnosed in an adult Senegalese women]. / La dilatation kystique congénitale du cholédoque: à propos d'un cas diagnostiqué chez une adulte sénégalaise.

The authors report a case of congenital cystic dilatation of the choledochus diagnosed on a 37 years old senegalese woman. It is an uncommon affection in Africa. The clinical presentation with various signs is reviewed. Ultrasound or cholangiography confirms the diagnosis. Surgical excision of the cystic dilatation is the best treatment because of the high risk of cancerisation.

Congenital biliary tract disease.

Jaundice in the pediatric patient requires prompt and directed evaluation. This dictum is highlighted in infants with biliary atresia, in whom the progressive sclerosing process results in complete obliteration of patent but microscopic hilar biliary structures by 4 months of age. Kasai's operation, if done before that time, will re-establish bile drainage in 90% of infants. One fourth to one third of patients achieve long-term jaundice-free survival. Complications of cholangitis, portal hypertension, and fat malabsorption are experienced by many patients. In children with early or late operative failure, liver replacement now offers legitimate hope for extended survival. Choledochal cyst is a conglomerate of pancreaticobiliary anomalies consisting of a choledochal cyst, a common-channel-type pancreaticobiliary junction, intrahepatic cystic disease, and partial obstruction of the distal common bile duct. Many patients have one or more of these malformations. It is now widely accepted that the preferred treatment of choledochal cyst is total excision of the diseased biliary duct with reconstruction by Roux-en-Y choledochojejunostomy. "Internal" excision avoids injury to other structures in the hepatoduodenal ligament, particularly if pericystic inflammation is present. Congenital perforation of the common bile duct responds in most cases to simple peritoneal drainage of the perforation. Retention of the tube cholecystostomy is useful for subsequent cholangiographic follow-up. Tube cholecystostomy may also be useful for irrigation of the biliary tract in infants with inspissated bile syndrome.

Familial occurrence of congenital bile duct dilatation.

The occurrence of congenital bile duct dilatation (CBD) in both a mother and her daughter was recently experienced at Niigata University Hospital and Niigata Shimin Hospital. Bile duct dilatation with anomalous pancreaticobiliary ductal junction (AP-BDJ) was disclosed in both. Intrahepatic bile duct dilatation was only in the mother. Removal of dilated bile duct, cholecystectomy, and hepaticojejunostomy were performed in both. Fourteen cases of CBD from seven families were collected from literature and discussed.

[Carcinoma in situ in localized Caroli's disease]. / Carcinome in situ sur maladie de Caroli localisée.

A new case of Caroli's disease associated with cholangiocarcinoma is reported. It is the first in situ carcinoma description. Intrahepatic biliary cysts are a predisposing condition, even if mechanism remains hypothetical.

[Choledochal cyst]. / Un kyste du cholédoque.

The persistence of recurrent abdominal pains in a 6 year-old girl without medical history lead to an ultrasonography being performed that showed a liquid formation, assigned to a dilated biliary duct. An oral cholecystography showed that this formation corresponded to a voluminous choledochal cyst. Surgical treatment consisted of a cyst excision with an hepatico-jejunostomy as biliary drainage.

Antenatal diagnosis of choledochal cyst at 15 weeks' gestation: etiologic implications and management.

We present the fourth case of antenatally diagnosed choledochal cyst--which was found at 15 weeks' gestation--and its subsequent treatment. We believe that the etiology was distal obstruction.