胰胆管合流异常并发急性胰腺炎的诊治策略及文献复习

目的 探讨胰胆管合流异常并发急性胰腺炎的诊治策略.方法 回顾性分析22例胰胆管合流异常病人的临床资料.结果 22例中急性胰腺炎发生7例,占31.8%(7/22),其中C-P型5例(71.4%,5/7),P-C型2例(28.6%,2/7).7例均行ERCP+EST+ENBD,2例伴胆总管结石者行网篮取石,2例伴胆囊结石的病人在急性胰腺炎稳定后行胆囊切除术.所有病人均成功治愈,随访一年,未有胰腺炎复发.结论 胰胆管合流异常常并发急性胰腺炎的发生,C-P型及合并胆石症者更常见;ERCP+EST+ENBD+胆囊切除术是防治此类胰腺炎的有效方法.     

胰胆管合流异常并发急性胰腺炎的诊治策略及文献复习

目的 探讨胰胆管合流异常并发急性胰腺炎的诊治策略.方法 回顾性分析22例胰胆管合流异常病人的临床资料.结果 22例中急性胰腺炎发生7例,占31.8%(7/22),其中C-P型5例(71.4%,5/7),P-C型2例(28.6%,2/7).7例均行ERCP+EST+ENBD,2例伴胆总管结石者行网篮取石,2例伴胆囊结石的病人在急性胰腺炎稳定后行胆囊切除术.所有病人均成功治愈,随访一年,未有胰腺炎复发.结论 胰胆管合流异常常并发急性胰腺炎的发生,C-P型及合并胆石症者更常见;ERCP+EST+ENBD+胆囊切除术是防治此类胰腺炎的有效方法.     

Carcinoma in adenoma of the gallbladder in a patient with anomalous pancreaticobiliary ductal junction

We present a case of carcinoma in adenoma of the gallbladder with anomalous pancreaticobiliary ductal junction (APBDJ) in a 60-year-old Japanese woman. The patient had no abdominal symptoms. Serum levels of carcinoembryonic antigen, carbohydrate antigen 19-9, elastase 1, and α-fetoprotein were all within normal limits. Ultrasonography demonstrated two irregular hyperechoic lesions of the gallbladder. Computed tomography showed two soft-tissue density masses in the gallbladder. Endoscopic retrograde cholangiopancreatography showed APBDJ and two protruding lesions in the gallbladder. Angiography displayed a hypervascular lesion fed by the cystic artery. The patient underwent total cholecystectomy; the diagnosis of the intraoperative frozen section was well differentiated adenocarcinoma with possible mucosal invasion. Amylase level in the gallbladder bile was 90600 IU/l. Macroscopically, there were two polyps in the gallbladder. The larger one, 3.5 cm in diameter, was adenocarcinoma in adenoma, and the smaller one, an inflammatory polyp. We briefly discuss the relationship among adenoma, adenocarcinoma of the gallbladder, and APBDJ.     

Case report of an anomalous arrangement of the pancreaticobiliary ducts and nuclear DNA ploidy analysis

Two cases with an anomalous arrangement of the pancreaticobiliary ducts (APBD) are reported in order to investigate the mechanism of carcinogenesis of the extrahepatic biliary tract from the aspect of DNA ploidy analysis. Highly elevated biliary enzymes were found in both cases. Although inflammatory changes without any metaplasia or neoplasia were observed histologically, a cytofluorometric nuclear DNA ploidy analysis showed an aneuploid low ploidy pattern in a histogram of the gall bladder, an aneuploid high ploidy pattern in a histogram of the common bile duct in one patient, and a diploidy pattern in the gall bladder, an aneuploid low pattern in the common bile duct in another case. These results may show that APBD may play a role in the development in DNA ploidy abnormality with refluxed pancreatic juice which may induce repeated irritation and inflammation. Radical surgery including a complete excision of the extrahepatic biliary tract followed by reconstruction may achieve better results with regard to undesirable consequences with an abnormal nuclear DNA ploidy pattern and decrease the likelihood of developing carcinoma.     

Carcinoma of the pancreas associated with anomalous junction of pancreaticobiliary tracts: Report of two cases and review of the literature

We report two cases of carcinoma of the pancreas with anomalous junction of the pancreaticobiliary tracts. A 71-year-old Japanese woman had obstructive jaundice. Ultrasonography showed a hypoechoic mass in the pancreatic head and computed tomography demonstrated a low-density nodule in the pancreatic head. Endoscopic retrograde cholangiopancreatography displayed a double duct sign and an anomalous junction of the pancreaticobiliary tracts. The patient underwent a pancreatoduodenectomy. The histopathologic diagnosis of the resected specimen was adenocarcinoma of the pancreatic head. A 56-year-old Japanese man also developed obstructive jaundice. Ultrasonography and computed tomography showed a huge mass almost replacing the whole pancreas and involving the superior mesenteric artery, splenic artery, splenic vein, and portal vein. Multiple hepatic metastases and peritoneal dissemination were present. Endoscopic retrograde cholangiopancreatography demonstrated an anomalous junction of the pancreaticobiliary tracts. The patient died of hemorrhage from esophageal varices. We discuss the relationship between the anomalous junction of the pancreaticobiliary tracts and pancreatic carcinoma. Received for publication on May 30, 1997; accepted on Dec. 3, 1997     

ERCP在胆胰结合部疾病中的应用

随着十二指肠镜设备和器械发展,内镜逆行胰胆管造影（ERCP）在胆胰管结合部疾病治疗中占有非常重要地位。ERCP治疗的胆胰管结合部疾病主要包括结石、肿瘤、Oddi括约肌狭窄和胆胰管合流异常。胆胰管结合部结石嵌顿通过括约肌切开术（EST）或电针开窗可迅速缓解; 无法根治性切除的恶性肿瘤可行内镜胆管引流术（ERBD）或内镜胆管金属支架术（EMBE）建立胆汁内引流,严重黄疸术前利用鼻胆导管引流术（ENBD）可有效减黄;Oddi括约肌狭窄行EST优势明显,已经代替传统开腹括约肌成型术;治疗无胆总管扩张的胰胆管合流异常现处于探索阶段。     

Extracystic biliary carcinoma associated with anomalous pancreaticobiliary junction and cysts

Anomalous pancreaticobiliary junction with cystic dilatation of the biliary tract is usually associated with carcinoma arising from the cyst wall. We report an extracystic location of biliary carcinoma in the presence of anomalous pancreaticobiliary junction and cysts in a patient with obstruction of the origin of the left hepatic duct who underwent hepatobiliary resection. Cholangiocarcinoma was found to have arisen in a noncystic left hepatic duct, in conjunction with cystic dilatation involving both the cystic and common bile ducts. The present case supports a relationship between anomalous pancreaticobiliary junction and biliary carcinogenesis that may affect the extracystic biliary tree.     

Self-expandable metallic stents for malignant biliary obstruction with an anomalous pancreaticobiliary junction

Background Anomalous pancreaticobiliary junction (APBJ) is associated with pancreaticobiliary cancer. Limited data are available on endoscopic biliary drainage for unresectable malignant biliary obstruction with APBJ. This study evaluated the efficacy and safety of self-expandable metallic stents (EMSs) for the management of malignant biliary obstruction with APBJ. Methods Between 1993 and 2005, 324 patients with unresectable malignant biliary obstruction underwent insertion of an EMS. Six of these patients with concomitant APBJ constituted the subjects of this study. Early (≤30 days after EMS insertion) and late (>30 days after EMS insertion) stent-related complications and stent patency were evaluated in these six patients. Results The cause of biliary obstruction was gallbladder cancer in four patients and pancreatic cancer in two patients. Uncovered EMSs were inserted across the common channel without performance of a biliary sphincterotomy. The diameter of the uncovered EMS used was based on the diameter of the common channel. For all six patients, endoscopic biliary drainage was successful, and their jaundice subsided steadily. None of the six patients experienced early complications, including acute pancreatitis. The mean stent-related complication-free period was 163 days. Stent occlusion caused by tumor ingrowth occurred in two patients. Acute cholangitis and cholecystitis were observed in one patient each. Conclusions Uncovered EMSs are effective for palliation of unresectable malignant biliary obstruction in patients who have APBJ without increasing the risk of stent-related early complications.     

Double cancer of the gallbladder and common bile duct associated with an anomalous pancreaticobiliary ductal junction without a choledochal cyst: Report of a case

We report herein the case of a 37-year-old woman found to have double cancer of the gallbladder and common bile duct associated with an anomalous pancreaticobiliary ductal junction (APBDJ) without a choledochal cyst (CC). Abdominal ultrasonography showed an isoechoic mass in the gallbladder, and percutaneous transhepatic biliary drainage tubography revealed incomplete obstruction in the upper portion of the common bile duct and APBDJ. The patient underwent cholecystectomy, partial hepatic resection, pancreatoduodenectomy, and portal vein reconstruction. Pathological examination of the tumors from the gallbladder and bile duct revealed papillary carcinoma and poorly differentiated adenocarcinoma, respectively, and direct continuity was not observed between the tumors. A review of the literature on six cases of multiple primary carcinoma of the biliary tract associated with APBDJ without CC is presented following this case report. Double cancer of the biliary tract was found synchronously in five patients and metachronously in one. Gallbladder cancer showed subserosal invasion in four patients, while bile duct cancer invaded the pancreas in one patient and reached the serosa in two patients. Considering the potential for cancer to arise in the biliary tract and the difficulties associated with monitoring it, cholecystectomy and resection of the extrahepatic common bile duct may be the most appropriate treatment for patients with an APBDJ without a CC.     

Outcomes of ERCP: prospective series from a rural centre

BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP) is an important procedure for the investigation and management of pancreaticobiliary disease. There is a recognized potential for significant morbidity, and a number of studies have identified patient and operator risk factors for the development of complications, including small case volume. We look at the outcomes of ERCP from a single operator at a rural centre and compare these with published figures. METHOD: Findings from 700 consecutive ERCP were collected prospectively between August 1997 and May 2006. Patients were included on an intention to treat basis, and all predetermined morbidity criteria were recorded. RESULTS: Five hundred and forty-four therapeutic and 156 diagnostic ERCP were included in the study. There were a total of 40 complications (5.71%), with three cases of ERCP-specific mortality (0.4%). The success of therapeutic intervention was 94.3%. CONCLUSIONS: The importance of comparing personal audit to published prospective studies has been emphasized. This has provided quality assurance finding, confirming that comparable success rates, morbidity and mortality are possible in a low-volume rural centre with an operator who has been properly trained and has ongoing ties with a tertiary hepatobiliary unit, a dedicated and skilled local team and suitable patient selection.     

胰胆管合流异常合并胆道肿瘤的诊断与治疗

目的 阐述胰胆管合流异常这种解剖学异常的临床分型与其所导致胆道肿瘤的关系，探讨如何选择适当的、合理的手术治疗。方法 回顾性分析我院自1979年12月至2001年10月间所收治的64例胰胆管合流异常症病人的临床表现、影像学特点和分型与其合并胆道肿瘤的关系。结果 本组病人中：胰管型(P-C型)28例，合并胆道肿瘤11例；胆管型(C-P型)32例，合并胆道肿瘤8例；共同通道型4例，胆道肿瘤2例。结论 胰胆管合流异常同胆道肿瘤的发生关系密切。胰、胆管合流异常病人的临床分型不同，所导致的胆道肿瘤发生的可能性不同。     

急性胆源性胰腺炎和胰胆管合流异常的关系

目的 探讨急性胆源性胰腺炎(ABP)和胰胆管合流异常(APBDU)的关系.方法 选择ABP手术患者165例.发病时抽取静脉血栓测总胆红素(TB)、丙氨酸氡基转移酶(ALT)、天冬氨酸氨基转移酶(AST)、碱性磷酸酶(ALP),γ-谷氨酰转移酶(GGT),保守治疗后复查;术中胆道造影观察胰胆管合流情况.结果 165例胆道造影患者中,发现APBDU 41例.保守治疗后APBDU和NAPBDU组的TB、ALT、AST、ALP、GGT都明显降低,有统计学意义(P<0.05).治疗后TB、ALP都恢复正常.APBDU组仍高于NAPBDU组,但没有统计学意义(P>0.05);APBDU组的ALT、AST和GGT都高于NAPBDU组,有统计学意义(P<0.05).结论 APBDU是导致ABP的重要原因之一.     

Septum formation of the common hepatic duct associated with an anomalous junction of the pancreaticobiliary ductal system and gallbladder cancer: Report of a case

We herein describe a 48-year-old woman who developed a septum formation of the bile duct combined with an anomalous arrangement of the pancreaticobiliary ductal system in conjunction with gallbladder cancer. A preoperative endoscopic retrograde cholangiogram demonstrated a filling defect in the common hepatic duct which was misdiagnosed to be an elevated lesion. Further exploration revealed a septum formation of the bile duct along with gallbladder cancer. A resection of the dilated bile duct and gallbladder along with a dissection of the regional lymph nodes was performed. A histological evaluation showed the septum to consist of a normal bile duct wall while the tumor in the gallbladder was poorly differentiated adenocarcinoma. The patient made an uneventful recovery. The septum formation was presumed to be congenital in origin.     

Estrogen receptor expression in anomalous arrangement of the pancreaticobiliary duct

Background/Purpose

Anomalous arrangement of the pancreaticobiliary duct (AAPBD) is a malformation of the bile and pancreatic ducts which is commonly associated with congenital biliary dilatation and predominantly occurs in girls. Estrogen receptor (ER) is reported to modulate cholangiocyte proliferation and play a role in tumorigenesis of estrogen-dependent malignancies. The present study investigated the presence of ER in the gallbladder of patients with AAPBD to elucidate whether ER expression is correlated with sexual specificity and biliary histology.

Methods

Specimens comprised samples of 29 gallbladders from patients with AAPBD (21 girls and 8 boys; mean age at surgery, 5.3 years). Medical records were retrospectively reviewed, and sex, age, radiographic parameters, and laboratory data were collected for this study. Immunohistochemistry was performed using anti-ER and anti-MIB-1 antibodies, and MIB-1 score was calculated for evaluation of cell proliferation.

Results

ER expression was observed in both the nuclei and cytoplasm of gallbladder epithelium in 12 of 29 specimens. No difference in background characteristics were noted between ER-positive and ER-negative groups. Mucosal hyperplasia was found in 17 specimens. ER-positive cases were significantly more common in AAPBD with mucosal hyperplasia than without (P <0.03). MIB-1 score did not differ significantly between ER-positive and ER-negative groups.

Conclusions

ER expression seems to be related to hyperplastic mucosa of the gallbladder in AAPBD. ER might play a role in the proliferation of gallbladder epithelium.     

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先天性胆管扩张症(congenital biliary dilatation,CBD)高发于亚洲人群,普遍认为其与胆总管壁薄弱和远端胆总管梗阻有关。胆总管壁远端梗阻的成因尚不清楚,比较流行的为胰胆管合流异常学说。产前诊断的年龄越小,扩张越明显,肝损害越重,生后要越早进行手术矫治。目前CBD被简化分为两型:胆总管远端狭窄型和非狭窄型。无论患儿的年龄大小或者症状的轻重,囊肿彻底切除、肝管空肠Roux-en-Y吻合术是根治CBD的标准术式。术前及术中详细的了解患儿整个胰胆道系统的改变情况,同时处理肝内胆管和胰管及共同管的异常病变,是减少手术后并发症的关键。     

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先天性胆管扩张症(congenital biliary dilatation,CBD)[1]是临床上所见的先天性胆道部分呈囊性或梭形扩张,其可发生于肝内外胆管的任何部位,因好发于胆总管,亦称为先天性胆总管囊肿。1723年由Vater首次报告,本病具有癌变倾向。CBD以婴幼儿和年轻人多见,好发于东方国家,男女之比为1∶3～4。目前其病因未完全明了,可能有:(1)先天性胰胆管合流异常(pancreaticobiliary maljunction,PBM)[2];(2)先天性胆道发育异常;(3)遗传因素等。     

Clinical experience of biliary tract carcinoma associated with anomalous union of the pancreaticobiliary ductal system

Between 1978 and 1988, 15 patients with gallbladder cancer and 2 patients with bile duct cancer were seen among 49 patients with anomalous union of the pancreaticobiliary ductal system. Radiographic findings revealed two types of this anomalous condition: one in which the pancreatic duct entered the common bile duct(type 1) and one in which the common bile duct entered the pancreatic duct (type 2). In gallbladder cancer, the common bile duct presented no dilatation, or in some patients, mild dilatation, and type-1 anomalous union was frequently found among these patients. In contrast, the two patients with bile duct cancer had cystic dilatation of the common bile duct and type-2 anomalous union. The bile amylase level, which was determined in seven patients, was extremely high in all the patients. Histopathologically, the tumors in most patients showed papillary to papillo-tubular proliferation in the mucosal layer while atypical epithelial hyperplasia was noted in the vicinity of the tumor area. These findings suggest that this congenital anomaly in both ducts results in a loss of the normal sphincteric mechanism of the doudenal papilla, and that chronic relapsing cholecystitis or cholangitis, caused by the reflux of pancreatic juice into the biliary tract, can induced progressive changes to atypical epithelial hyperplasia which may develop into carcinoma.