胆源性胰腺炎和胰胆管合流异常的关系

目的 探讨胆源性胰腺炎(ABP)和胰胆管合流异常(anomalous pancreaticobiliary ductalunion,APBDU)的关系.方法 选择ABP患者131例,入院后抽取静脉血检查总胆红素(TB)、ALT、AST、ALP,γ-GT.先行非手术治疗,待病情稳定后复查上述指标,然后择期手术,并行术中胆道造影,观察胰胆管合流情况.结果 131例ABP患者中发现APBDU 27例,占20.6%.其中胆总管汇入胰管(Ⅰ型)8例,占29.6%,胰管汇入胆总管(Ⅱ型)16例,占59.3%,复杂型(Ⅲ型)3例,占11.1%.经非手术治疗后,TB、ALT、AST、ALP、γ-GT含量均较入院时明显降低(P＜0.05),其中APBDU患者的ALT为(71.81±23.19)U/L,AST为(47.85±27.87)U/L,γ-GT为(52.86±31.49)U/L,仍明显高于无APBDU患者的(51.96±15.40)U/L、(40.77±16.58)U/L和(34.86±26.47)U/L(P＜0.05).结论 胰胆管合流异常足导致ABP的重要原因之一.     

Association of gallbladder carcinoma and anomalous pancreaticobiliary ductal union

A total of 96 patients with gallbladder carcinoma in whom direct cholangiography clearly opacified the pancreaticobiliary ductal union and the common channel, and 65 patients with an anomalous union of these two duct systems at a distance greater than 15 mm from the papilla of Vater (normally less than 4.6 +/- 2.2 mm, mean +/- SD) were studied. It was found that this anomalous ductal union occurred in 16.7% of the patients with gallbladder carcinoma in comparison with an incidence of 2.8% among 641 consecutive patients with various hepatobiliary and pancreatic diseases studied by endoscopic retrograde cholangiopancreatography who did not have gallbladder carcinoma. It was also found that gallbladder carcinoma occurred in 24.6% of the 65 cases of anomalous ductal union in comparison with a 1.9% incidence of this cancer among 635 consecutive patients similarly studied and found to have normal ductal union (p less than 0.001). Thus, a close etiologic association was suggested between this anomaly in the terminal segment of the biliary tract and gallbladder carcinoma. Of the 65 patients with anomalous ductal union, 50 had the so-called congenital cystic dilatation of the common bile duct and 15 did not. Five of the 50 (10%) and 11 of the 15 (73.3%) had gallbladder carcinoma (p less than 0.01), and this carcinoma seems to be related to anomalous ductal union rather than to cystic dilatation of the common bile duct. As a tumorigenic factor in this anomaly, regurgitation of pancreatic juice has been stressed.     

Epithelial hyperplasia of the gallbladder in children with anomalous pancreaticobiliary ductal union

BACKGROUND/AIMS: Few data are available on the fate and incidence of epithelial hyperplasia throughout the life of anomalous pancreaticobiliary ductal union (APBD) patients. The pathological study in pediatric APBD patients is less recognized. METHODOLOGY: Ten resected gallbladders obtained from children with APBD and control patients without APBD were examined histologically, and immunohistochemically for the detection of Ki-67 (as a proliferative marker) and p53. K-ras mutations in codon 12 were also examined. Epithelial hyperplasia was classified into high-grade and low-grade hyperplasia. RESULTS: Six (60%) of 10 patients with APBD had epithelial hyperplasia of the gallbladder, whereas no patients without APBD exhibited this lesion. Diffuse epithelial hyperplasia was observed in 1 (50%) of 2 undilated-type APBD and 5 (63%) of 8 dilated-type. Two (33%) of 6 patients with epithelial hyperplasia exhibited high-grade hyperplasia. Ki-67 labeling index (LI) was significantly higher in hyperplastic mucosa than in control gallbladder mucosa. K-ras mutations and p53 overexpression were not detected in hyperplastic and normal mucosa. CONCLUSIONS: Epithelial hyperplasia of the gallbladder accompanied by increased proliferative activity exists at birth or is acquired in childhood with APBD patients and may be an important factor predisposing to the development of gallbladder carcinoma.     

Multiseptate gallbladder with anomalous pancreaticobiliary ductal union： A case report

Multiseptate gallbladder, characterized by the presence of multiple septa dividing the gallbladder lumen, is a very extremely rare congenital anomaly of the gallbladder. On the other hand, anomalous pancreaticobiliary ductal union is also one of the congenital anomalous biliary diseases and thought to be related with choledochal cyst or biliary tract malignancies. In this paper, we describe a unique and first patient of multiseptate gallbladder with anomalous pancreaticobiliary ductal union and a review of the literature. To clarify more characters of the multiseptate gallbladder,examination of a larger patient population will be needed and further studies will be required.     

胰胆管合流异常对胰腺损伤的实验研究

目的 探讨胰胆管合流异常(APBDU)对胰腺的损伤.方法 选择健康杂种猫10只,在胆管、胰管近十二指肠处纵行切开4～6 cm,然后间断吻合,以建立胰胆管合流异常的模型.手术造模时及术后6个月分别取胰腺组织行病理检查、电镜观察及胰腺组织丙二醛(MDA)水平检测.结果 7只猫生存6个月.术后6个月胰腺呈暗红色,充血、水肿,其中3只猫胰腺表面可见扩张的胰管.光镜下见3只猫胰腺有炎性细胞浸润,4只猫胰腺有间质血管增生、白细胞附壁.电镜下见胰腺腺泡细胞粗面内质网扩张,有的呈池状;线粒体数量增多、体积增大、崩解;高尔基复合体发达.手术前胰腺组织MDA水平为(1.23±0.7)nmol/mg prot,术后6个月升高达(2.90±1.9)nmol/mg prot,相差显著(F=4.80,P=0.0215).结论 胰胆管合流异常可导致胰腺损伤,抗氧化可能有助于减轻该损伤.     

Association between anomalous pancreaticobiliary ductal union and adenomyomatosis of the gall-bladder

A frequent association of biliary tract carcinoma and anomalous pancreaticobiliary ductal union (APBD) is well recognized, especially gall-bladder carcinoma in undilated type APBD. However, little is known about the presence and incidence of adenomyomatosis (AMT) of the gall-bladder, a presumed premalignant lesion, in patients with APBD. This retrospective study was conducted to elucidate the clinical features and incidence of AMT in APBD patients with relation to undilated type and dilated type APBD. We reviewed the clinicopathological records of 30 patients with APBD (28 women and two men) encountered during the past 10 years. Among them, 22 patients underwent cholecystectomy and the resected specimens were subjected to histopathological examinations. Eleven cases of APBD patients were undilated type and 11 cases were dilated type. Adenomyomatosis was found in six (55%) of 11 undilated type and one (9%) of 11 dilated type, and fundal type was predominantly observed in six (86%) of seven AMT. An overall incidence of AMT in APBD patients was 32%. An undilated type of APBD is frequently associated with AMT and we believe, therefore, that clinicians should be aware of a possible coexistence of APBD and AMT.     

Synchronous cancer of the biliary tract and pancreas associated with anomalous arrangement of the pancreaticobiliary ductal system.

A 58-year-old man on abdominal ultrasonography and CT had an irregularly elevated lesion at the neck of the gallbladder and a cyst of approximately 6.5 cm in diameter at the pancreatic tail. Percutaneous transhepatic cholangiography revealed a 2-cm shadow defect at the neck of the gallbladder and an irregular, translucent 30 x 12 mm lesion in the intrapancreatic bile duct. Total pancreatectomy and extended cholecystectomy with regional lymph node dissection was performed. An anomalous arrangement of the pancreaticobiliary ductal system (AAPBD) was demonstrated by postoperative contrast radiography of resected specimen. The lesions of the gallbladder and common bile duct were papillary adenocarcinoma. In addition, papillary adenocarcinoma was limited almost entirely to the mucosal layer of the main pancreatic duct and its branches, from the junction of the common bile duct and pancreatic duct to the pancreatic tail. The three tumors were not continuous. The cyst at the pancreatic tail was a pseudocyst. This case represents synchronous cancer of the gallbladder, common bile duct, and pancreas associated with AAPBD.     

Risk factor for extrahepatic bile duct cancer in patients with anomalous pancreaticobiliary ductal union

BACKGROUND/AIMS: Anomalous connection between the choledochus and pancreatic duct is considered to be a factor in the development of biliary tract diseases such as choledochal cyst, pancreatitis, cholangitis, gallbladder cancer, and bile duct cancer. Our purpose was analysis of combined disease, especially biliary neoplasm and evaluated microscopic changes of extrahepatic bile ducts. METHODOLOGY: To study the clinical characteristics of anomalous pancreaticobiliary ductal union (APBDU), we reviewed 14 APBDU cases from June 1994 to June 1998. We studied the associated disease, surgical treatment, and the histological findings of the extrahepatic bile ducts. RESULTS: Gallbladder cancer was identified in 5 out of 14 patients with APBDU. The incidences of metaplasia of gallbladder and bile duct with APBDU were higher than that of control gallbladder epithelium. The proliferating cell nuclear antigen-labeling index of the gallbladder in patients with APBDU was significantly higher than that in the control group. CONCLUSIONS: The patients with APBDU showed high incidence of gallbladder carcinoma and metaplasia in epithelium of gallbladder and bile duct. As this metaplasia in the gallbladder and bile duct is thought of as a precancerous condition, it is important to remove the place that causes bile stasis and to stop backflow of pancreatic juice into the bile duct in managing patients with this anomaly. In other words, prophylactic cholecystectomy and reconstruction of the biliary tract are both necessary.     

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.     

Analysis of pancreatic enzymes in the bile of congenital choledochal cyst with anomalous pancreaticobiliary ductal union

Pancreatic enzymes in the bile of congenital choledochal cyst with anomalous pancreaticobiliary ductal union were analyzed. Almost all pancreatic proteases were already activated in the bile aspirated immediately after insertion of PTCD tube. However, inactive proteases gradually increased after continuation of drainage by PTCD. Pancreatic protease activities appeared again after clamping the PTCD tube. In vitro study of trypsin activities in the bile containing no initial trypsin activity, active type of trypsin did not appear throughout the experimental period. It is suggested that continuous reflux of pancreatic juice into the bile and certain incubation time are necessary for activation of pancreatic protease in bile of congenital choledochal cyst.     

Alpha-fetoprotein producing carcinoma of the gallbladder associated with anomalous arrangement of the pancreaticobiliary ductal system—Early detection through an attack of acute pancreatitis

A 56-year-old female was admitted to our hospital with a diagnosis of acute pancreatitis. Ultrasonography revealed a hypoechoic tumor in the gallbladder. The serum α-fetoprotein (AFP) level was 971 ng/ml. After healing of the acute pancreatitis, the anomalous arrangement of the pancreaticobiliary ductal system (APDS) was demonstrated by ERCP with mild dilatation of the common bile duct. Within one month after admininon, AFP level reached 4390 ng/ml. On operation, a pedunculated tumor, 6.5 × 3 cm in size, was found in the gallbladder. Histological examination revealed a moderately differentiated adenocarcinoma and positive immunohistochemical staining of cancer cells for AFP. After absolutely curative (stage II) resection, normal serum AFP levels were recognized. This is the first report of an AFP-producing cancer of the gallbladder associated with APDS and it was detected as a result of an attack of acute pancreatitis.     

Alpha-fetoprotein producing carcinoma of the gallbladder associated with anomalous arrangement of the pancreaticobiliary ductal system--early detection through an attack of acute pancreatitis.

A 56-year-old female was admitted to our hospital with a diagnosis of acute pancreatitis. Ultrasonography revealed a hypoechoic tumor in the gallbladder. The serum alpha-fetoprotein (AFP) level was 971 ng/ml. After healing of the acute pancreatitis, the anomalous arrangement of the pancreaticobiliary ductal system (APDS) was demonstrated by ERCP with mild dilatation of the common bile duct. Within one month after admininon, AFP level reached 4390 ng/ml. On operation, a pedunculated tumor, 6.5 x 3 cm in size, was found in the gallbladder. Histological examination revealed a moderately differentiated adenocarcinoma and positive immunohistochemical staining of cancer cells for AFP. After absolutely curative (stage II) resection, normal serum AFP levels were recognized. This is the first report of an AFP-producing cancer of the gallbladder associated with APDS and it was detected as a result of an attack of acute pancreatitis.     

Clinicopathological studies of anomalous arrangement of the pancreaticobiliary ductal system with pancreas divisum

We examined the morphological aspects of the pancreaticobiliary ductal system in 13 patients with both anomalous arrangement of the pancreaticobiliary ductal system (AAPB) and associated pancreas divisum (PD), and compared their clinicopathological findings with those of patients with either AAPB or PD alone. PD is classified into three types, i.e., separate pancreas, nonfusion of the ventral and dorsal pancreatic ducts, and partial fusion of the ventral and dorsal pancreatic ducts. Of the 13 patients with AAPB and associated PD, 5 were male and 8 female; their mean age was 57 years. Nine of the 13 had clinical symptoms. Five of the 13 had gallbladder cancer, 3 had cholecystolithiasis, and 2 choledochal cyst. One patient showed nonfusion of both pancreatic ducts and the others showed partial fusion of the ducts. The length of the common duct from the orifice of the papilla of Vater to the junction of the common bile duct with the ventral pancreatic duct and the location of the union varied. The presence of both AAPB and PD made the arrangement of the pancreaticobiliary ducts very complicated. Clinical aspects were similar to those of AAPB and the patients were likely to be treated for a condition other than PD. However, since relapsing acute pancreatitis and intermittent epigastric pain are typical symptoms in patients with AAPB as well as in those with PD, we should carefully follow up those patients with both AAPB and PD.     

Thickened inner hypoechoic layer of the gallbladder wall in the diagnosis of anomalous pancreaticobiliary ductal union with endosonography

Background: An anomalous pancreaticobiliary ductal union (APBD) is a high-risk factor for biliary tract carcinoma, which often is not diagnosed before overt malignancy. The early detection of APBD is therefore clinically important. We evaluated the gallbladder wall in APBD patients with endoscopic ultrasonography. Methods: Clinicopathologic features and ultrasonographic findings of the gallbladder in 33 consecutive patients with APBD between 1986 and 1995 were studied in relation to two subtypes of APBD, that is, undilated (n = 17) and dilated (n = 16). The gallbladder wall was evaluated with conventional ultrasonography and/or endoscopic ultrasonography. Histologic examinations of 25 resected gallbladders were made. Results: Fourteen of the seventeen patients with undilated type APBD (82%) had diffuse thickened gallbladder wall of 4 mm or more, whereas 5 of the 16 with dilated type (31%) had this finding (p < 0.01). The thickened gallbladder wall consisted sonographically of two layers: diffuse thickened inner hypoechoic layer and outer hyperechoic layer. Mucosal hyperplasia was histologically found in 8 of 9 cases (89%) with thickened inner hypoechoic layer on endoscopic ultrasonography. Mucosal hyperplasia was observed in 10 of 11 undilated type APBD cases (91%) in which cholecystectomy was performed. In addition, the presence of anomalous union was shown by endoscopic ultrasonography in 9 of 11 patients with undilated type APBD (82%) and all 7 of those with dilated type. The characteristic ultrasonographic pattern of diffuse thickened inner hypoechoic layer was observed exclusively in patients with mucosal hyperplasia of the gallbladder associated with APBD among 2085 endoscopic ultrasonography examinations performed during the study period. Conclusions: Diffuse thickened inner hypoechoic layer of the gallbladder wall was frequently observed in APBD patients, especially those with the undilated type, on ultrasonography and/or endoscopic ultrasonography. This finding corresponded histologically to mucosal hyperplasia of the gallbladder mucosa. Thickened inner hypoechoic layer is a useful ultrasonographic sign that indicates mucosal hyperplasia of the gallbladder and, particularly, the possible coexistence of undilated type APBD before the appearance of overt malignancy. (Gastrointest Endosc 1997;46:520-6.)     

Sphincter of Oddi dysfunction in children with recurrent pancreatitis and anomalous pancreaticobiliary union: an etiologic concept.

BACKGROUND: The exact cause of recurrent pancreatitis among patients with anomalous pancreaticobiliary union is not known. Sphincter of Oddi dysfunction has been implicated as a mechanism. This study evaluated sphincter of Oddi function in children with anomalous pancreaticobiliary union and recurrent pancreatitis and assessed the results of endoscopic sphincterotomy in the management of this condition. METHODS: We retrospectively reviewed 128 endoscopic retrograde cholangiopancreatographic (ERCP) studies performed on children older than 1 year and adolescents with pancreaticobiliary disease. In 64 instances, ERCP was performed because of recurrent pancreatitis. Nine patients underwent sphincter of Oddi manometry followed by endoscopic sphincterotomy, and these patients were included in this study. A basal pressure greater than 35 mm Hg was considered diagnostic for sphincter of Oddi dysfunction. Follow-up data were obtained retrospectively from the patients' relatives and referring physicians. RESULTS: An anomalous pancreaticobiliary union was found in 18 of 64 (28%) patients with recurrent pancreatitis. The 9 patients who underwent sphincter manometry and endoscopic sphincterotomy were 5 girls and 4 boys 2.9 to 17 years of age (mean 7.8 years). A choledochal cyst was found in 7 of these 9 patients. Two patients had anomalous pancreaticobiliary union without common bile duct dilatation. All 9 patients had sphincter of Oddi dysfunction (mean basal pressure 96 +/- 37.8 mm Hg, range 48 to 156 mm Hg). The length of the common channel was 22.8 +/- 5.5 mm, and the length of the sphincter of Oddi segment was 12.1 +/- 1.9 mm (p < 0.001). In all patients the sphincter of Oddi segment was located within the duodenal wall. The mean follow-up period after endoscopic sphincterotomy was 26.4 months (range 18 to 38 months). Eight patients had excellent results defined as absence of symptoms and no subsequent episodes of acute pancreatitis. Treatment of 1 patient was considered moderately successful because the patient still had occasional pain without pancreatic enzyme elevation but no subsequent episodes of acute pancreatitis. One patient had mild postprocedural pancreatitis. CONCLUSIONS: Recurrent pancreatitis and anomalous pancreaticobiliary union are associated with sphincter of Oddi dysfunction in children and adolescents. Endoscopic sphincterotomy is beneficial to these patients.     

Anomalous arrangement of pancreaticobiliary ductal system associated with cholangiocellular carcinoma

A 66-year-old man with congenital cystic dilatation of the common bile duct (CDB; Alonso-Lej Type I) and anomalous arrangement of the pancreaticobiliary ductal system (AAPB) associated with intrahepatic bile duct cancer (cholangiocellular carcinoma; CCC) underwent an extended right hepatic lobectomy. In the resected specimen, the CCC was located in Couinaud’s segment V, with invasion to segment IV and the right hepatic duct and right portal vein. However, there was no cancer involvement of the dilated extrahepatic bile ducts, except for histologic findings of chronic inflammatory cellular infiltration and intestinal metaplasia. It is presumed that the probable mechanisms underlying carcinogenesis in CCC developing from the epithelium of intrahepatic bile ducts are the same mechanisms as those operating in carcinoma of the extrahepatic bile duct in patients with AAPB, although AAPB associated with CCC is uncommon. AAPB appeared to be related to the development of the CCC.     

Normal and anomalous pancreaticobiliary union in children and adolescents.

BACKGROUND: The normal length of the pancreaticobiliary union (common channel) in the pediatric population is not known, nor is the frequency of anomalous pancreaticobiliary union and the extent to which it is associated with pancreaticobiliary disease. METHODS: ERCP was performed on 136 patients younger than 1 year (group 1) and 128 older than 1 year (group 2). RESULTS: In group 1 the average length of the common channel was 1.8 +/- 0.61 mm with a maximal length of 3 mm (mean plus 2 standard deviations). In group 2 the average length and maximal length of the common channel increased with age. In the 1 to 3 year age range the average length was 2.2 +/- 0.47 mm with a maximal length of 2.7 mm, in the 4 to 6 year range it was 2.8 +/- 0.40 mm (3.6 mm maximal), in the 7 to 9 year range it was 3.2 +/- 0.43 mm (4.1 mm maximal), in the 10 to 12 year range it was 3.9 +/- 0.5 mm (4.4 mm maximal), and in the 13 to 15 year range it was 4.0 +/- 0.51 mm (5 mm maximal). The prevalence of the anomalous pancreaticobiliary union was 25% (66/264). In group 1 the anomaly was present in 4.4% (6 of 136) of patients, 1.3% (1/76) with neonatal hepatitis, 4.6% (3/44) with biliary atresia, and 100% (2/2) with choledochal cyst. In group 2 the anomaly was present in 46.9% (60/128) of patients, 100% (57/57) with choledochal cyst and 15.7% (3/19) with idiopathic recurrent pancreatitis without bile duct dilatation. CONCLUSIONS: The mean length of the common channel increases with age. The maximum normal length of the common channel in neonates and infants younger than 1 year is 3 mm. It increases with age to a maximum of 5 mm in children and adolescents between 13 and 15 years of age. Anomalous pancreaticobiliary union is relatively common among children and adolescents undergoing ERCP in our center, including those with idiopathic recurrent pancreatitis (15.7%). ERCP is valuable in the diagnosis of this anomaly.     

Endocrine cell carcinoma of the gallbladder with anomalous pancreaticobiliary ductal junction

We experienced a case of endocrine cell carcinoma of the gallbladder with anomalous pancreaticobiliary ductal junction. It is well known that anomalous pancreaticobiliary ductal junction has potentiality to cause gallbladder cancer and that most of the cases are papillary adenocarcinoma or well-differentiated adenocarcinoma. The direct relationship between endocrine cell carcinoma of the gallbladder and anomalous pancreaticobiliary ductal junction has not been elucidated.