【作者】 李臻；

【导师】 韩新巍；

【作者基本信息】 郑州大学 ， 影像医学与核医学， 2007， 硕士

【摘要】 背景和目的胰胆管合流异常(pancreaticobiliary maljunction，PBM)为胚胎发育异常导致的胆管和胰管在十二指肠壁外高位汇合的一种先天性畸形，功能上失去Oddi括约肌的控制，使胆汁和胰液相互逆流，进而引发诸多相关胆胰疾病。1916年日本学者Kizumi首先提出PBM概念，上世纪70年代日本Komi N对PBM的病理改变及其与先天性胆管扩张症、胆道癌的关系等进行了较为系列的叙述，并倡导成立了日本PBM研究会，对PBM的发生、病理、诊断、治疗等做了较全面的研究，加深了临床对本病的认识。近年，PBM与相关胆胰疾病的密切关系已成为临床研究的热点，尤其是与胆系癌(指肝外胆管癌和胆囊癌)的病因、病理学联系。国内外学者大多认为PBM与胆系癌有相关性，并已经从临床、动物实验等方面、在基因、分子水平间探讨它们之间的联系。有关PBM临床诊断的文献报告多限于经内窥镜逆行胰胆管造影(endoscopic retrograde cholangiopancreatography，ERCP)和外科术中胆管造影等，此类检查创伤大、并发症多；近期也有磁共振胰胆管成像(magnetic resonance cholangio-pancreato graphy，MRCP)的报告，MRCP虽无创，却存在一定的漏诊和误诊情况；CT(compmed tomography，CT)诊断和放射性核素扫描虽有报道，但因直观性差而应用很少。临床因阻塞性黄疸(obstructive jaundice，OJ)而经经皮肝穿刺胆管造影术(percutaneous transhepatic cholangiography,PTC)行介入治疗的病例愈来愈多，PTC时有发现PBM、并能够详细显示其解剖情况，但迄今文献中尚没有PBM的PTC影像学诊断特性的详细报道。PTC是在肝内胆管即远高于胰胆管汇合部的区域穿刺、插管和注射对比剂，为顺行性胆树造影，不会干涉胰胆汇合部的生理功能和解剖结构，对PBM的诊断价值更大。此外，在经皮肝穿刺胆管引流术(percutan-eous transhepatic cholangiodrainage，PTCD)过程中行胆道钳夹病理活检(percutaneous transhepatic cholangiobiopsy，PTCB)，根据病理性胆管癌诊断结果从病理学角度探讨PBM与胆系癌的关系，使本研究更为准确可靠。而既往有关PBM与胆系癌的相关性研究多集中在基础医学方面，从基因、大分子蛋白质代谢等角度探讨二者关系。本文旨在通过回顾性分析35例因OJ入院而行PTCD时胰管显影患者的临床和影像学资料，探讨PTC诊断PBM的技术可行性、影像学特征；初步提出PTC诊断PBM的标准，并从胆道病理学角度讨论合并阻塞性黄疸的PBM与胆系癌发生的相关性。材料与方法收集1999年1月至2007年2月间连续282例因OJ经PTC行介入治疗的病人的临床及影像学资料，其中25例因未解除胆道梗阻段而行外引流术。确立病例入选标准与PBM诊断条件，从中筛选出资料完整、胰管显影的病例35例，诊断为PBM 31例，检出率12.062％(31／257)。其中男19例，女12例，男女比例1.583：1，年龄37～88岁，平均63.032±12.090岁。临床表现皮肤黏膜黄染31例，伴腹痛、腹胀或纳差26例，小便黄、白陶土样便者18例，伴胆系结石12例。所有病例均经病理学证实。在不伴有PBM的226例行PTCD的OJ病例中，随机地抽取资料翔实的89例病例作为对照组，分析PBM与胆系癌发病率的相关性。OJ病因确诊方法：①PTC下胆道钳夹病理活检，其阳性结果被认为真实反映病灶特性。②经外科手术病理证实组织学类型及分化程度。否则由第三种方法证实。③根据患者病史、临床表现、特异性检查结果及病程随访等对病因作出综合诊断。所有患者PTCD均采用标准化操作技术。PTC成功后，明确梗阻部位及胆树扩张形态；然后尝试以导丝打通梗阻段，常规行PTCB以取得病理学诊断。阳性结果被认为真实反映病变特性，而阴性结果不除外假阴性的可能性。经导管于胆总管末段造影，发现胰管、胆胰共同管及十二指肠显影后，于最佳投照位置显示胆胰管汇合关系及Odii括约肌收缩、舒张状态，适时摄片以观察其影像学表现，准确测量相关数据。PTC图像分析采取双盲法，由两位放射科医师共同阅片，着重观察胆道梗阻部位、胆胰管及共同管形态、乳头位置等，并利用两脚规和直尺测量胆胰共同管的长度和直径、胆胰管直径及汇合角度等。以术中造影导管直径作为校正标准，计算出实际测量数值。反复阅片并达成共识后详细记录观察及测量结果，对有争议的结果再由另一放射科医生观察、分析，以保证结果的准确、可靠。所有数据由SPSS13.0统计软件包完成统计学处理。结果①OJ病例中PBM的PTC检出率为12.062％。②汇合处胆总管直径、胰管直径、共同管直径分别为3.201±1.617mm、2.061±0.817mm、3.027±1.034mm，与正常值比较无统计学差异。③共同管长度为9.875±4.548mm，胆总管、胰管汇合角度为55.302°±22.513°，与正常值比较有显著性差异。④男女之间、不同年龄之间胆胰共同管直径、汇合角度大小无显著性差异，共同管长度差异存在显著性。⑤十二指肠乳头开口异位影响共同管长度大小。⑥31例PBM胆系癌变率为61.290％，OJ伴与不伴PBM之间胆系癌发生率有显著性差异；但PBM汇合类型及角度对伴存的胆系癌分化程度、并发胆胰疾病的类别无显著影响；共同管长度及汇合角度对胆系癌变率的影响无显著性。结论一．PTC诊断PBM技术方法可行、操作安全、结果可靠，兼具诊断、治疗作用。二．PTC诊断PBM的参考标准：①胆胰管在十二指肠壁外高位汇合，十二指肠降段切线位投照时共同管长度＞6mm；②胆总管与主胰管汇合角度异常(＞45°)；③胆汁内胰淀粉酶含量增高，超过1000U(温氏单位)／L；④胆总管与主胰管之间存在未退化的背侧胰管等异常交通，导致胆胰逆流。具备其中一条即可诊断PBM。三．PBM的PTC影像学特征：①胆胰管以较长的共同管与十二指肠壁“Y型”连接，共同管长度大于6mm；②十二指肠乳头多数向降部中段以远异位，异位越远，共同管越长；③共同管可呈“悬空征”，胆胰管高位汇合点距离肠壁大于6mm；④胆胰管汇合角度较大；胆汁胰淀粉酶含量增高支持胰液逆流。影像学分型：B-P型、P-B型、复杂型。B-P型PBM多出现胆总管末端环行狭窄。四．PBM与胆系癌变有显著相关性。更多还原

【Abstract】 Background and objective: Pancreaticobiliary maljunction(PBM) is a congenital embryonic development malformation defined as common bile duct and pancreatic duct union that is located outside the duodenal wall and beyond the regulation of the sphincter of Oddi. Mutual reflux of bile and pancreatic juice into the pancreatic and bile ducts leads to some correlated disease. Japanese scholar Kizumi firstly proposed the concept of PBM in 1916. In 1970s, Komi N carried out the detailed research about the pathological changing of PBM and the relationship among PBM, congenital cholangiectasis and cholangiocarcinoma. He also advocated The Japanese Study Group on PBM and the Committee, and made the complete investigation,including the etiology, pathology, diagnosis, therapy principle and so on,which enormously deepened the clinical cognition to the desease. Recently, the close relation between PBM and correlated pancreaticobiliary deseases has become a research focus, especially the etiology and the pathological association with biliary carcinoma. Many researchers consider that PBM is closely related to occurrence of biliary malignant neoplasms, and have investigated the correlation from the clinic and animal experiment aspects, gene and molecular level. The records concerned with diagnosis of PBM is parum, of the total methods imageology is the main diagnostic examination, consist of endoscopic retrograde cholangiopancreato graphy (ERCP), intraoperative cholangiography, magnetic resonance cholangiopancreatography(MRCP), CT and hepatobiliary scintigraphy. Various kinds of imaging examinations have deferent features. Currently, more and more patients with obstructive jaundice(OJ) are receiving percutaneous transhepatic cholangiodrainage (PTCD) procedure for biliary decompression, sometimes percutaneous transhepatic cholangiography (PTC) can detect the PBM and display the anatomy of confluence. Nevertheless, few detail reports about PTC imaging characteristics of PBM in literature hitherto. Because the puncture and opacification are performed outside confluence of pancreaticobiliary ducts, which has scarcely influence on the function and anatomy of pancreaticobiliary confluence, so PTC is considered to have great diagnostic value for PBM. In addition, percutaneous transhepatic cholangio-biopsy (PTCB) during PTCD provides the pathologic evidence for OJ. To discuss the relationship between PBM and biliary carcinoma from the pathology point of view is an innovation of this study. The previous correlation research mainly concentrated on preclinical medicine.This study aims at retrospectively analyzing the detail data of 31 patients with OJ who were diagnosed as PBM to discuss the PTC technique availability, imaging characteristics of PBM diagnosis. Clarify preliminarily the diagnostic reference criterion for PBM during PTC and the correlation with biliary carcinoma.Materials and methods: Collecting the clinical data of consecutive 282 patients with OJ receiving the PTCD therapy in our hospital from January 1999 to February 2007. Clinical findings and cholangiopancreatographic results were analyzed. But 25 cases received the biliary external drainage as the obstructive sites weren’t relieved Meanwhile the standard to be selected for cases and diagnostic conditions was established. Among them 35 cases, both biliary and pancreatic ducts were opacified. 31 cases was radiologically diagnosed as PBM and the detection rate was 12.062%(31/257). Male (n=19) to female(n=12) ratio was 1.583:1, the age ranged from 37y to 88y. the average age was 63.032±12.090 years. All of the cases presented obstructive jaundice, there were 26 patients presented with abdominal pain, abdominal distention or poor appetite, 18 cases presented with yellow urine and white bole sedes, 12 cases accompanied with biliary stone.Of the 226 OJ cases without PBM, 89 detail cases were drawn randomly to be defined as control group to evaluate the dependablity between PBM and biliary carcinoma. There are three methods to determine the etiological factor of OJ. Firstly, the histopathology type and differentiation degree are confirmed by surgery. Secondly, PTCB provides the histopathology evidence. If it is negative, the comprehensive diagnosis is made according to the case history, clinical aspects, specific laboratory examination and the follow-up.All of the patients underwent the standard PTCD procedure successfully. PTC revealed the obstruction site and the dilatation degree of the biliary tract, the guide wire was managed to pass throungh the occlusion, then the PTCB was performed as routine. The positive findings was considered to reflect the ture pathological changes. But the negative results cannot exclude the possibility of false negative. When transcatheter opacification visualized the pancreatic duct, pancreaticobiliary common channel and the duodenum, photographs was necessary to demonstrate the confluence of pancreaticobiliary ducts and the contraction, relaxation condition of Oddi sphincter in the optimal posture in order to measure the correlated data conveniently.The double blind method is applied to analyze the PTC findings. Two radiologists analyze the PTC findings respectively. The obstructive site, the shape of the pancreaticobiliary ducts and the common duct, duodenal papilla’s site are the major observation objections. The compasses and ruler are utilized to measure the length and diameter of pancreaticobiliary common duct, pancreatic duct and common bile duct. The confluence angle is also a measure objections. Refer to the diameter of catheter in PTCD and calculate the practical numerical value. The coincident results are recorded. All of the data are analyzed by SPSS13.0 statistical package.Results: As a biliary decompression procedure for OJ, PTCD was performed successfully for all patients. The overall prevalence of PBM in OJ cases was 12.062%. The diameter of common bile duct, pancreatic duct and common duct near the confluence are 3.201±1.617mm, 2.061±0.817mm, 3.027±1.034 mm, respectively. Compared with the normal value, the results did not have statistical difference. The length of common duct and the confluence angle of the pancreaticobiliary ducts are 9.875±4.548 mm, 55.302°±22.513°, respectively. The significant difference exists between them and normal value. With regard to the diameter of common duct and the confluence angle, the difference was not significant between male and female, different year groups. But the length of common duct had significant difference among them. Ectopic duodenal papilla had influence on the length of common duct. The occurrence rate of biliary carcinoma was 61.290% in 31 PBM cases. The biliary carcinoma incidence in cases with PBM was significant higher than one in cases without PBM. The PBM confluence types and angle had no significant influence on the accompanied biliary carcinoma differentiate degree as well as the categories of pancreaticobiliary deseases. Nor had the length of common duct and confluence angle influence on canceration rate.Conclussion:(1) PTC is an effective, reliable, safe and technically available imaging method to diagnose PBM. PTC and PTCD not only make diagnosis for PBM, but undertake palliative therapy on OJ.(2) The referred PTC diagnostic standard of PBM is as follow: The high confluence of pancreaticobiliary ducts is detected by the tangential photograph of descending duodenum, the length of common duct exceeds 6mm. The confluence angle of the pancreaticobiliary ducts is increasing (>45°) and pancreatic amylase level in bile exceeds 1000U/L. The anomalous communicating branch (eg:vestigial Santorini duct) existing between common bile duct and pancreatic duct also leads to the PBM. The diagnosis can be made by one of the items above.(3)PTC imaging characteristics of PBM: The direct sign is a longer pancreaticobiliary common duct(>6mm) presented, generally, it’s not dilated. The duodenal papilla is mainly located in the distal part to the middle of descending duodenum. The distaler is the location of duodenal papilla, the longer is the length of common duct. The indirect sign is "sign of suspending in midair " of the common duct, the distance between the confluence and duodenal wall exceeds 6mm, or the confluence angle is larger, the high level of pancreatic amylase in bile certifies the existing pancreatic juice reflux. The forms of pancreaticobiliary confluence are categori zed into three types: When common bile duct appeares to join the main pancreatic duct, it is denoted as B-P type. While the main pancreatic duct appeares to join the common bile duct, it is denoted as P-B type. Complex type is the PBM accompanied with visualizing accessory pancreatic duct. In addition, a stenosis of common bile duct end is usually observed in B-P type.(4)PBM is highly associated with occurrence of biliary malignant neoplasms.更多还原