MRCP诊断先天性胆管扩张症的临床评价

目的 评价MRCP诊断先天性胆管扩张症的应用价值。方法 26例先天性胆管扩张症患儿术前行MRCP检查,并均接受扩张胆管切除,肝总管空肠Roux-Y吻合手术,术中再进行胆道穿刺造影,将两者所显示的胆管扩张程度,形态和胆胰管合流异常的结果进行对比分析。结果 MRCP显示胆总管囊性扩张17例,其余9例为梭形扩张,肝内、外胆管显像与术中胆道造影一致,7例显示出胰胆管合流异常,显示率26.9%,术中造影24例显示胰胆管合流异常,显示率92.3%,结论 MRCP可作为诊断先天性胆管扩张症优先选择方法。     

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??Roles of ERCP in the treatment of disorders at pancreaticobiliary junction QIN Ming-fang. Department of Minimally Invasive Surgery??Tianjin Nankai Hospital??Tianjin 300100, China
Abstract With the development and evolution of duodenum endoscopy devices and instruments, endoscopic retrograde cholangiopancreatography (ERCP) has been playing more and more important roles in the treatment of disorders at pancreaticobiliary junction, which includes: benign bile stones, Oddi’s sphincter stenosis, anomalous pancreaticobiliary junction and malignant lesions. The topics are discussed in detail with hand-on experience of the author in the article. The impacted bile stones at the pancreaticobiliary junction can be evacuated by endoscopic sphincterotomy (EST). Surgically incurable malignant lesions can be intraluminally drained by endoscopic retrograde biliary drainage (ERBD) or expandable metallic biliary endoprothesis (EMBE). Severe jaundice can be relieved by endoscopic nasobiliary drainage (ENBD). Oddi’s sphincter stenosis is effectively managed by EST. In addition, the treatment of anomalous pancreaticobiliary junction without dilated common bile duct is still under investigation.     

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。     

先天性胆管囊状和柱状扩张的胆胰管合流异常

目的 探讨先天性胆管囊状和柱状扩张与胆胰管合流异常（ＡＰＢＤ）特征的关系。方法 对５１例先天性胆管扩张患者行胆道造影检查。结果 ４３例确定有ＡＰＢＤ。囊状组胆管汇入胰管１８例（７２％）,柱状组胰管汇入胆管１４例（７８％）;囊状组和柱状组对比胆管远端内径（Ｐ〈０．０１）、胰管最大径（Ｐ〈０．０５）及胆胰管交角（Ｐ〈０．０１）均有显著性差异。结论 不同的ＡＰＢＤ常类型常导致不同形式胆道病理变化,囊状     

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.     

Discordance for anomalous pancreaticobiliary ductal junction and congenital biliary dilatation in a set of monozygotic twins.

A pair of monozygotic twins, 2-year-old Japanese girls, discordant for both anomalous pancreaticobiliary ductal junction and congenital biliary dilatation, are reported. The pathogenesis of the disease is discussed in relation to the occurrence of discordant monozygotic twins.     

胆囊切除术中胆道造影的价值

目的 探讨临床诊断为单纯胆囊病变拟行单纯胆囊切除术中胆道造影的价值。方法 分析904例术前诊断为胆囊结石、胆囊息肉样病变的患者在胆囊切除术中胆道造影发现胆道病变、变异的情况。结果 在904例中通过术中胆道造影发现胆总管结石79例、胰胆管合流异常21例、胆囊管冗长11例、胆囊管异常开口17例、右侧副肝管4例。结论 术前临床诊断为单纯胆囊病变行单纯胆囊切除时术中胆道造影可以发现术前未能确诊的胆总管结石，避免胆道二次探查，并有利于胰胆管合流异常和胆道解剖变异的诊断。     

Fusion variations of pancreatic ducts in patients with anomalous arrangement of pancreaticobiliary ductal system

Fusion variations of the pancreatic ducts were studied to elucidate the significance of such variations. We classified structural fusion anomalies of the main and accessory pancreatic ducts on endoscopic retrograde cholangio-pancreatography (ERCP) in 37 patients with anomalous arrangement of the pancreaticobiliary ductal system (AAPB). The fusion variations of the pancreatic ducts were classified into five types: common, ansa pancreatica, branch fusion, looped, and separated. These fusion variations, except for common type, were found in 68% of the 37 patients with AAPB on ERCP. Fusion variations of the pancreatic ducts were very frequent (93%) in the 30 patients with congenital cystic dilatation of the common bile duct (CCD). The branch confluence fashion, in which the terminal bile duct communicated with a pancreatic duct branch, was found only in patients with cystic dilatation cyst of the CCD, and it appeared that cystic dilatation cyst of CCD might differ from spindle or cylindrical cyst originating from embryonic formation of an anomalous confluence. It was also suggested that in patients with fusion variations of the pancreatic ducts, the flow of pancreatic juice might be disordered, leading to the development of acute pancreatitis or pancreatic dysfunction. Consequently, it appears to be necessary to carefully examine patients with AAPB for the presence or absence of any fusion variations of the pancreatic ducts and to observe such patients with long-term monitoring by ERCP, and computed temography, and with pancreatic function tests. Received for publication on Jan. 7, 1998; accepted on April 28, 1998     

Endoscopic retrograde cholangiopancreatography in children

Endoscopic retrograde cholangiopancreatography (ERCP) was performed less in children than in adults. Twenty-seven children, aged from 56 days to 14 years (average 6 years), received ERCP from August 1989 to December 1990. The cannulation into the papilla of Vater was successful in 24 children (88.9%) and their pancreaticobiliary ductal system was visualized clearly. Congenital biliary dilatation (CBD) was diagnosed in 16 patients and other biliary disorders in 8 patients. The APBD was confirmed in almost all CBD patients, showing a close etiological relation. It was concluded that ERCP is helpful to diagnose and differentiate pediatric biliary diseases and to study the etiology and pathophysiology of CBD.     

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.     

108例先天性胆管囊肿的诊治经验

目的 总结先天性胆管囊肿的诊治经验。方法 对1980年－2000年间收治的108例先天性胆管囊肿患者的临床资料进行回顾性分析。结果 108例的临床表现为：儿童病例多出现典型的腹痛、黄疸及腹部肿块,成人症状多不典型,常导致误诊,57例（52．7％）合并胆胰系统其他疾病,18例合并癌变,癌变率为16．6％。108例患者中,94例行B型超声检查,46例行ERCP检查,20例行PTC检查,71例行腹部CT检查,均确诊。合并胰胆管合流异常39例。1985年前诊断及分型以B超和术中所见决定,手术以内引流术为主。1985年后,诊断以ERCP、CT为主,同时行B超检查,术式以囊肿切除,肝管空肠Roux-en-Y吻合术为主,1994年提出防止先天性胆管囊肿癌变的新术式。术后并发症以胆道系统逆行性感染为主,均抗生素治疗后痊愈,全组无手术死亡。结论 先天性胆管囊肿20年诊治的观念有很大的改变,ERCP和CT对明确本病的分型、指导手术有帮助。目前推荐对Ⅰ、Ⅳ型先天性胆管囊肿采用囊肿全切除,肝管空肠Roux-en-Y吻合术,对反复感染的复杂的中央型V型caroli病可考虑行肝移植术。     

胆囊管异常汇合的诊断及其在腹腔镜胆囊切除术中的意义

目的 分析逆行胰胆管造影术（ERCP）在诊断胆囊管异常汇合的价值及其在预防腹腔镜胆囊切除术（LC）胆管损伤等并发症中的作用。方法 我院从1992年7月至1999年6月共行LC4 500余例,其中对780例LC患者术前行ERCP检查。选择胆囊管、肝总管和胆总管三者解剖关系均清楚的772例ERCP片,观测其胆囊管异常汇合情况,测量其长度和直径,并与100例胰胆管无异常的ERCP片对比。结果 在780例ERCP检查者中,发现胆囊管异常汇合者125例（16.03%）。该780例行LC中,中转开腹胆囊切除术35例（4.49%）,发生各种并发症6例（0.77%）。但无胆管损伤等严重并发症发生。结论 ERCP能准确诊断胆囊管的各种异常汇合,在预防LC胆管损伤等胆道并发症的发生中起重要作用。     

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.     

Morbidity and treatment in the anomalous arrangement of the pancreaticobiliary ductal system

A nationwide survey of cases of congenital dilatation of the biliary duct was conducted to obtain the data on cases of congenital dilation of the biliary duct in Japan between 1972 and 1982. A total of 645 cases were surveyed. An anomalous arrangement of the pancreaticobiliary ductal system (AAPABIDS) was associated with congenital dilatation of the biliary duct in 90.2% of the cases examined extensively. These cases and 62 cases that were collected in our hospital during the period from 1963 to 1984 were chosen as the subject of this study. The results may be summarized as follows: Various factors which affect the sphincter of Oddi are needed for the clinical manifestations of AAPABIDS. The incidence of biliary carcinoma increases with age, in parallel with that of intestinal metaplasia in cases of congenital dilatation of the biliary duct. These findings suggest that intestinal metaplasia may be one of the important factors in the development of biliary carcinoma. From the view points of timing and indication of surgery, it is necessary to cut off reflux of pancreatic juice into the biliary duct and remove the portion in which bile is stagnant prior to the development of serious complications.     

Biliary tract duplication accompanied by choledocholithiasis: Report of a case

Duplication of the biliary tract is extremely rare. In fact, to the best of our knowledge, only four previous reports of pediatric patients with this disease have been documented in the Japanese literature. This anomaly was diagnosed by perioperative endoscopic retrograde cholangiopancreatography (ERCP) in three of these patients, and incidentally during surgery for congenital biliary dilation in the other one. We report herein a case of biliary tract duplication which was diagnosed by T-tube cholangiography, only in the oblique view, after cholecystectomy and choledochotomy with T-tube drainage. The patient was a 13-year-old girl who was initially diagnosed as having choledocholithiasis based on the results of preoperative and intraoperative cholangiography; however, a postoperative T-tube cholangiography revealed residual stones. At this point, duplication of the biliary tract was diagnosed only in the first oblique view. Reoperation was carried out 26 days after the first operation. The biliary tract was dissected completely from the pancreaticobiliary junction to the left and right hepatic ducts in the porta hepatis and found to be duplicated from the level of porta hepatis to the suprapancreatic area. We report our experience of this case, followed by a discussion on the relevant literature.     

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.     

Pancreaticobiliary maljunction and carcinogenesis to biliary and pancreatic malignancy

Background  It is widely accepted that congenital choledochal cyst is associated with pancreaticobiliary maljunction (PBM). But, PBM is an independent disease entity from choledochal cyst. PBM is synonymous with “abnormal junction of the pancreaticobiliary ductal system”, “anomalous arrangement of pancreaticobiliary ducts”, “anomalous union of bilio-pancreatic ducts”, etc. Cases with PBM not associated with biliary duct dilatation are often found, and these cases are frequently complicated gallbladder cancer. The Japanese Study Group of Pancreaticobiliary Maljunction was started in 1983, and defined diagnostic criteria and nationwide registration system of PBM cases was started. PBM is defined as a union of the pancreatic and biliary ducts which is located outside the duodenal wall. Bile and pancreatic juice reflux and regurgitate mutually. Biliary carcinogenesis  The most bothersome problem is biliary carcinogenesis. Gallbladder cancers arise in 14.8% and bile duct cancers arise in 4.9%. The incidence of the gallbladder carcinoma of PBM without bile duct dilatation is 36.1%. Many investigators have tried to clarify the carcinogenic process, from various aspects. The biliary epithelia are injured by harmful substances, and in the course of repair, multiple alterations of oncogenes and tumor suppressor genes are followed, and they lead to carcinoma through multistage interaction. In the biliary epithelia of PBM, incidence and degree of hyperplasia are characteristic. K-ras gene mutations are observed in the cancerous as well as noncancerous lesions of biliary tract of PBM patients. Mutations of p53 gene and overexpression of p53 protein are also found in the cancerous and noncancerous lesions. These changes are called “hyperplasia–carcinoma sequence”. Treatment  Total excision of the extrahepatic bile duct with gallbladder followed by hepaticojejunostomy, Roux-en-Y, or end-to-side hepaticoduodenostomy are treatment of choice, even for cases with not dilated bile duct, because the incidence of cancer in the nondilated bile duct is not negligible, and genetic changes are seen in a nondilated bile duct.     

Epithelial cell proliferation activity of the biliary ductal system with congenital biliary malformations

Congenital biliary malformations such as anomalous arrangement of the pancreaticobiliary ductal system (AAPB), congenital cystic dilatation of the common bile duct (CCDB), and congenital biliary strictures at the hepatic hilum (CBSH) are newly designated disease entities and are frequently found in adult patients with biliary malignancy such as gallbladder carcinoma, common bile duct carcinoma, and intrahepatic bile duct carcinoma. In the present study, the relationship of these malformations and biliary malignancy was investigated. We studied 61 gallbladders of patients with AAPB and 56 gallbladders of patients without AAPB; 16 common bile ducts of patients with CCDB (12 with AAPB and 4 without AAPB) and 11 gallbladders of patients without CCDB; and 17 intrahepatic bile ducts of patients with CBSH and 6 intrahepatic bile ducts of patients without CBSH. Tissue sections from the mucosa of the gallbladder, common bile duct, and intrahepatic bile duct were stained for proliferating cell nuclear antigen (PCNA). The PCNA labeling indexes of patients with these malformations were significantly higher than those of patients without these malformations (P < 0.05). Cell proliferation of the epithelia in the biliary ductal system in patients with these congenital biliary malformations was accelerated. Consequently, these congenital malformations appear to be an important risk factor for the occurrence of biliary malignancy. Received for publication on Feb. 15, 1999; accepted on March 29, 1999     

Congenital bile duct dilation —Possibly an hereditary condition—

A female patient underwent resection of a large choledochal cyst and hepaticojejunostomy when she was 11 years old at Tokyo Medical and Dental University Hospital. Her mother, 48 years old, also had congenital bile duct dilatation (CBD) with cancer. The mother had an anomalous pancreatico-biliary ductal junction. To investigate the possibility of a genetic link, family members of another three consecutive CBD cases were examined for bile duct dilatation and anomalous pancreatico-biliary junction. Endoscopic retrograde choledocho-pancreatography (ERCP) revealed an anomalous pancreatico-biliary ductal junction, without bile duct dilatation in the mother of a 17-year-old CBD patient. Two familial occurrences of CBD had been reported in the Japanese literature. Their combinations were; a father—a daughter and two sisters, respectively. Considering that the sex ratio of CBD is one male to 2.6 females, our results suggest that CBD or anomalous pancreatico-biliary ductal junction may be 1) an X-linked dominant trait or 2) autosomal dominant trait with relatively low penetrance in males. Since anomalous pancreatico-biliary ductal junction does not necessarily develop into bile duct dilatation, more familial investigations on pancreatico-biliary ductal junction and bile duct dilatation are essential to clarify the genetic contribution to CBD.     

Congenital bile duct dilatation--possibly an hereditary condition

A female patient underwent resection of a large choledochal cyst and hepaticojejunostomy when she was 11 years old at Tokyo Medical and Dental University Hospital. Her mother, 48 years old, also had congenital bile duct dilatation (CBD) with cancer. The mother had an anomalous pancreatico-biliary ductal junction. To investigate the possibility of a genetic link, family members of another three consecutive CBD cases were examined for bile duct dilatation and anomalous pancreatico-biliary junction. Endoscopic retrograde choledocho-pancreatography (ERCP) revealed an anomalous pancreatico-biliary ductal junction, without bile duct dilatation in the mother of a 17-year-old CBD patient. Two familial occurrences of CBD had been reported in the Japanese literature. Their combinations were; a father--a daughter and two sisters, respectively. Considering that the sex ratio of CBD is one male to 2.6 females, our results suggest that CBD or anomalous pancreatico-biliary ductal junction may be 1) an X-linked dominant trait or 2) autosomal dominant trait with relatively low penetrance in males. Since anomalous pancreatico-biliary ductal junction does not necessarily develop into bile duct dilatation, more familial investigations on pancreatico-biliary ductal junction and bile duct dilatation are essential to clarify the genetic contribution to CBD.