胆管神经内分泌癌的诊断和治疗

目的 提高对胆管神经内分泌肿瘤的认识。方法 报道国内首例肝总管神经内分泌癌合并肝脏转移，结合国内已经报道的8例未发生转移病例，总结该病的诊断和治疗。结果 男性3例，女性6例，8例以梗阻性黄疸起病，2例合并类癌综合征，梗阻部位胆总管下段2例，肝总管6例，胆总管中段1例，手术前均未能确诊；采取探查性手术，5例行根治性切除胆管空肠吻合术，2例行Whipple手术，1例行姑息切除＋肝动脉插管化疗，1例外引流，手术后病理确诊。结论 胆管神经内分泌癌是一种少见的胆道恶性肿瘤，梗阻性黄疸是常见的症状，NSE、Cg-A、SYP等免疫组化染色是诊断的要点；手术切除率高，根治性切除是治疗的首选，如果肿瘤转移，姑息性切除＋肝动脉化疗是比较好的选择。     

Surgical anatomy of the vasculobiliary apparatus at the hepatic hilum as applied to liver transplantations and major liver resections

Introduction

To evaluate the hepatic arterial, bile duct and portal venous anatomy as applicable to major liver resections.

活体造影法肝内胆管的解剖学研究及临床意义

目的　通过经皮胆道直接造影,研究肝内胆管的解剖与变异的类型与分布。 方法　经皮胆道造影50例, 用20 ml注射器接体外引流管进行造影,胆管充分显影的状态下,旋转拍摄胆管,观察胆管解剖与变异。 结果　50例成像中胆管变异42.0％（21例）。12.0% (6例)显示右前叶肝管、右后叶肝管及肝左管呈三分叉状汇合形成肝总管。8.0% (4例) 肝左管先和右前叶肝管汇合,在肝门处与右后叶肝管汇合。20.0% (10例) 肝左管先和右后叶肝管汇合,在肝门处与右前叶肝管汇合。2.0% (1例)多个肝管在肝门处汇合。 结论　胆管解剖与变异的分析对术前手术方式的确定有一定的临床意义。     

正常人胆管壁组织构筑的研究

对5例正常人胆道系统的组织构筑及平滑肌、弹性纤维、胶原纤维的分布进行了面积计量研究.从胆总管胰后段至左右肝管汇合部,平滑肌的面积分数从13.56±0.65%降至3.34±0.32%,弹性纤维的含量以肝总管和左右肝管汇合部为高,与胆总管胰后段和十二指肠上段比较,差别有显著性.胶原纤维的含量在肝外胆管各段之间和肝内胆管各段之间无显著差异,但肝内胆管与肝外胆管之间的差异显著.肝内与肝外胆管壁的组织构筑存在一定的差异,说明它们的生理功能有所不同.     

Small cell carcinoma of the extrahepatic bile duct: case report and immunohistochemical analysis

A small cell carcinoma of the extrahepatic bile duct in a 75-year-old Japanese man is reported. The patient suffered from obstructive jaundice, and percutaneous transhepatic cholangiography-drainage (PTCD) revealed a massive lesion in the lower common bile duct. Because it was diagnosed as a malignant tumor, pancreaticoduodenectomy was performed. A nodular infiltrating tumor measuring 4.5 x 3.0 x 2.0 cm was located in the intrapancreatic portion of the extrahepatic bile duct. Histologically, the tumor was composed of a dense proliferation of small atypical cells with a little region of high-grade dysplasia in the adjacent epithelium of the common bile duct. Tumor cells were immunoreactive to neuroendocrine markers such as chromogranin A, synaptophysin, CD56, and Leu7. Although carcinoma cells invaded into pancreas and duodenum, there were no histological findings that indicated the carcinoma arose from the mucosa of either the pancreatic duct or duodenum. These results indicated that the tumor was a small cell carcinoma derived from the epithelium of the extrahepatic bile duct; a rare neoplasm with only a few cases reported. A few neuroendocrine cells were recognized in the adjacent epithelium of the extrahepatic bile duct, suggesting that the tumor cells might be derived from them. Using immunohistochemical examination, no p53 abnormality was found. Tumor cells showed positive nuclear staining for p16, while negative for cyclin D1, suggesting that functional retinoblastoma protein (pRB) might be lost in the p16/pRB pathway, as in small cell lung cancer.     

Differences in gallstone structure in primary common bile duct lithiasis and gallbladder lithiasis

Summary Some differences between gallbladder lithiasis and primary common bile duct lithiasis are described. Microbiological cultures and biochemical analyses were carried out on the bile of two groups of patients: 27 suffering from gallbladder and 5 from primary common duct lithiasis. The microstructure and composition of gallstones were also examined by polarized light microscopy and X-ray diffraction. Women predominated in gallbladder lithiasis but not in primary common duct lithiasis group (P<0.05) and body weight was higher in the former group (P<0.02). Primary common duct lithiasis patients had a higher, although not significant, incidence of duodenal diverticulosis (P=0.15), and a higher incidence ofE. coli-positive cultures in bile (P<0.001). No significant difference in the biochemical composition of the bile was found between the groups. Brown pigment stones predominated in primary common duct lithiasis, while cholesterol stones did in gallbladder and secondary common duct lithiasis (P<0.0001). Stones formed in the gallbladder generally show linear, radial growths of cholesterol crystals, while those from the common duct present a polystratified, concentric deposition of microgranules composed mainly of pigmentary salts.These differences should be taken into account as additional criteria in the differential diagnosis between primary and secondary common duct lithiasis, as the classical criteria for diagnosing of the former greatly underestimate its actual incidence. The distinction between primary and secondary common duct lithiasis is of practical significance, since each entity requires different treatment.Abbreviations CBD common bile duct - CBDL common bile duct lithiasis - ERCP endoscopic retrograde cholangiopancreatography - GBL gallbladder lithiasis - HDL high density lipoproteins - PCBDL primary common bile duct lithiasis - SCBDL secondary common bile duct lithiasis - SGOT serum glutamic-oxalacetic transaminase - SGPT serum glutamic-pyruvic transaminase     

利用微血管铸型技术研究肝外胆管动脉的分布特征

目的　统计分析肝外胆管的血供来源和分布以及在十二指肠上段胆管的吻合动脉链之间的多环形血管网,为胆管外科手术提供临床解剖学理论依据。 方法　以丙烯酸树脂为动脉填充剂灌注6例肝外胆管动脉制备铸型标本,使用photoshop测量法测量微小血管内径,观测和分析肝外胆管的血供来源和分布情况。 结果　肝固有动脉左、右支在肝总管上方存在弓型交通支,十二指肠上段胆管左右边缘的吻合动脉链间有丰富的横向动脉,吻合动脉链的供血约53%来自下方,来自上方的约占46%,1%来自中段肝固有动脉。通过对每条横向动脉中部的内径和两侧起始部的内径测量,发现肝总管中段和上段的横向动脉较粗,中部的平均内径分别为（0.26±0.02）mm和（0.24±0.04）mm,与两侧的内径比也比较大,上段比值最大,为1.09,中段其次,为1,下段中部的平均内径为（0.14±0.03）mm;与两侧的内径比值为0.74。 结论　肝外胆管上、下方的血供来源比例相对均衡;肝外胆管手术应尽可能于动脉吻合相对较少的胆总管十二指肠上段和血管相对较细小肝总管下部做纵行小切口,以降低肝外胆管血供的损伤;肝总管段的血供丰富,支持现行原位肝移植离断部位在胆囊管汇合处上方的主张。     

Effects of the administration of pentoxifylline and prednisolone on the evolution of portal fibrogenesis secondary to biliary obstruction in growing animals: immunohistochemical analysis of the expression of TGF- β and VEGF

OBJECTIVE:

During the neonatal and infancy periods, some chronic liver diseases may lead to progressive hepatic fibrosis, which is a condition that can ultimately result in the loss of organ function and severe portal hypertension necessitating hepatic transplantation. In a previous report, pharmacological interventions were demonstrated to modulate hepatic fibrosis induced by bile duct ligation in young rats. The administration of pentoxifylline or prednisolone, or the combination of both, resulted in reduced fibrogenesis in portal spaces. The objectives of the present study were to evaluate the expression of transforming growth factor β and vascular endothelial growth factor after bile duct ligation in young rats and to assess the effect of those same drugs on cytokine expression.

METHODS:

In this experimental study, 80 young rats (21 or 22 days old) were submitted either to laparotomy and common bile duct ligation or to sham surgery. The animals were allocated into four groups according to surgical procedure, and the following treatments were administered: (1) common bile duct ligation + distilled water, (2) sham surgery + distilled water, (3) common bile duct ligation + pentoxifylline, or (4) common bile duct ligation + prednisolone. After 30 days, a hepatic fragment was collected from each animal for immunohistochemical analysis using monoclonal antibodies against transforming growth factor β and vascular endothelial growth factor. Digital morphometric and statistical analyses were performed.

RESULTS:

The administration of pentoxifylline reduced the transforming growth factor β-marked area and the amount of transforming growth factor β expressed in liver tissue. This effect was not observed after the administration of prednisolone. There was a significant reduction in vascular endothelial growth factor expression after the administration of either drug compared with the non-treatment group.

CONCLUSIONS:

The administration of pentoxifylline to cholestatic young rats resulted in the diminished expression of transforming growth factor β and vascular endothelial growth factor in liver tissue. The administration of steroids resulted in the diminished expression of vascular endothelial growth factor only. These pathways may be involved in hepatic fibrogenesis in young rats submitted to bile duct ligation and exposed to pentoxifylline or prednisolone.     

胆道造影检查在腹腔镜胆囊切除术中的应用

目的探讨腹腔镜胆囊切除术中经胆囊管胆道造影的临床价值。方法通过对58例Lc术中经IOC的病人临床资料进行回顾性分析。结果本组病例成功55例,占94．83％,失败3例,占5．17％。50例胆总管未发现结石,占90．91％,发现胆总管小结石（0．4cm）5例,占9．09％。其中4例经中转开腹行胆道探查,1例经腹腔镜胆总管切开胆纤镜网篮取石。胆囊管汇入右肝管1例。全组病例无胆道损伤、胆总管结石残留、胆漏、腹腔感染及IOC相关并发症。结论LC术中行IOC操作简单易行,成功率高,显影效果好,能及时发现胆道解剖变异;对基层医院减少胆道阴性探查、术中胆道损伤、术后胆总管结石残留等具有重要的临床应用价值。     

Management strategy for congenital choledochal cyst with co-existing intrahepatic dilation and aberrant bile duct as well as other complicated biliary anomalies

The purpose of this study was to investigate and discuss imaging methods and management strategies for congenital choledochal cyst with co-existing intrahepatic dilation and aberrant bile duct as well as other complicated biliary anomalies. In this study we reviewed and analyzed 72 patients with congenital choledochal cyst, ranging in age from 15 days to 12 years old and who were seen at our hospital during the past 12 years, from January 1993 to October 2005. The image manifestation and clinical significance of patients with co- existing intrahepatic biliary dilation and aberrant bile duct were carefully examined during operation via MRCP, cholangiography and choledochoscope. Twenty-two cases (30.1%) presented with intrahepatic bile duct dilation and 12 of these were of the cystic type. That is, the orifice of the dilated intrahepatic tract that converged into the common hepatic duct showed membrane or septum-like stenosis. In 10 cases the dilation tapered off from the porta hepatis to the initiating terminals of the intra-hepatic bile ducts and was not accompanied by stenosis. An aberrant bile duct was observed in 2 of the cases. In 3 cases, the right and left hepatic ducts converged at the choledochal cyst. In conclusion, the imaging methods for intrahepatic bile duct dilation possess important clinical significance. Further, for hepatojejunostomy with radical excision of a choledochal cyst, additional operative procedures for intrahepatic stenosis, possible bile duct malformation and pancreaticobiliary common duct calculi can potentially reduce postoperative complications.     

Primary T-cell rich B-cell lymphoma of the common bile duct

A 34-year-old woman was hospitalized for the investigation of a one-month history of intestinal disorders, gastric heaviness and transitory icteric episodes. Extensive clinical investigations suggested the diagnosis of gall bladder carcinoma or sclerosing cholangitis. At laparotomy, the proximal part of common bile duct was markedly thickened by a white, firm, fish-flesh like tumour extending in to the cystic duct, gall bladder wall and to the liver. Histological study showed a diffuse lymphoid proliferation of the common bile duct mainly composed of small cells mixed with scattered large atypical cells. Immunohistochemistry revealed that most of the small cells expressed T-cell markers with predominant CD 4 and - T-cell receptors and without phenotypic gap, whereas large atypical cells showed monotypic B phenotype with co-expression of and heavy chains and light chain restriction. No evidence of primary nodal lymphoma was found during extensive clinical, radiological, sonographic or scanographic examinations. Sequential chemotherapy (MACOP-B) was instituted and the patient was still alive 4 years after diagnosis. Morphological and immunohistochemistry findings fulfilled criteria for a primary high grade B-cell lymphoma (centroblastic type, Kiel classification) from common bile duct concealed by numerous small reactive T-cells, so called T-cell rich B-cell lymphoma, not previously described in this location.     

Anomalous pancreatico-biliary ductal union with cystic dilatation of the bile duct

We report, in an adult, an asymptomatic association between cystic dilatation of the bile duct (type IV A in Todani’s classification) and anomalous pancreatico-biliary ductal union (APBD) with stones in a long common channel. In APBD, the connection between the common bile duct and the main pancreatic duct is located outside the duodenal wall and is therefore not under the influence of the sphincter of Boyden. An abnormally long common channel is in excess of 15 mm. Two types of convergence anomalies are defined according to whether the bile duct opens into the main pancreatic duct (BP) or the main pancreatic duct into the bile duct (PB). In APBD, there is probably a reverse pressure gradient between the bile and pancreatic ducts, with regurgitation of pancreatic juice into the bile duct, repeated attacks of cholangitis, stenosis and cystic dilatation. A long common channel is associated with a higher incidence of carcinoma of the gall bladder or the bile duct.     

Surgical anatomy of sub-hepatic biliary system

IntroductionThe anatomy of liver and extra-hepatic biliary apparatus has always been the focus of attention among anatomists and surgeons. It has gained greater significance in the recent years in view of technical refinements in the field of cholecystectomy, hepatic surgery and transplantation. Present study emphasizes the normal as well as variations of extra-hepatic biliary apparatus, as most of the published work on the surgical anatomy of the extra-hepatic biliary apparatus refers various ethnic groups variations. Literature on this topic in western U.P. subjects is scanty.MethodA study was conducted on 59 individuals undergoing hepatobiliary surgery after informed consent in the Department of Surgery and on 30 cadavers in the Department of Anatomy, LLRM Medical College Meerut, and Saraswathi Institute of Medical Sciences, Hapur during September 2004 to May 2011.ResultThe study revealed that in all cases, the union of hepatic ducts was extra-hepatic, of which 10% were angular low union and 5% parallel low union. In 95% cases cystic duct had angular union and 4% had parallel type of union with common hepatic duct and in one case cystic duct united with accessory hepatic duct. 16% cases had short cystic duct i.e. 1–2 cm in length. 94% cases show usual relation in hepatoduodenal ligament and in 6% common bile duct was to the left of hepatic artery.DiscussionThe obtained results presented variations regarding certain parameters when compared to previous studies and they represent the ethnic parameters of western UP.     

One duct,two orifices,no names! the anatomy of the biliary cystic duct

The biliary cystic duct (CD) connects the gallbladder to the extra‐hepatic bile duct, and the point at which it does this delineates the division between the common hepatic duct and the common bile duct. Its clinical relevance in disease, and importance during interventions relating to the gallbladder mean that its normal and variant anatomy has been described extensively in literature. However, an aspect not yet fully described includes naming of its two orifices on either end. This is highly relevant for surgical, endoscopic, and percutaneous procedures. We describe these as encountered in normal CD and biliary tree anatomy. We believe this is crucial for interventions relating to the gallbladder and the biliary tree, including prevention of iatrogenic injury. Clin. Anat. 31:422–423, 2018. © 2018 Wiley Periodicals, Inc.     

Congenital anatomical abnormalities of the extrahepatic biliary duct: a personal audit

Summary Awareness of variations in the anatomy of extrahepatic bile ducts may be important in preventing iatrogenic injury to the duct system during cholecystectomy. Their delineation, before or during surgery is therefore considered mandatory in this surgical unit, and a policy of always performing a per-operative cholangiogram has allowed us to evaluate the duct system in a retrospective review of 2080 cases. Twelve surgically significant anatomical variations were found, consisting of absent cystic duct (three cases), abnormal termination of cystic duct (two into the right hepatic duct, and one into the left hepatic duct), one case of double cystic duct, and five cases of significant accessory bile ducts.

---

Anomalies congénitales des voies biliaires extra-hépatiques : évaluation d'une expérience

Résumé La connaissance des variations anatomiques des voies biliaires extra-hépatiques est un élément important pour la prévention des plaies biliaires iatrogènes au cours de la cholecystectomie. Leur recherche systématique préopératoire ou peropératoire est donc exigée dans notre pratique chirurgicale et le recours systématique à une cholangiographie per-opératoire a permis d'étudier les variations des canaux biliaires dans une étude rétrospective de 2080 cas. Douze types de variations canalaires avec incidence chirurgicale ont été découverts : l'absence de conduit cystique (trois cas), la terminaison anormale du conduit cystique (deux fois dans le canal hépatique droit, une fois dans le canal hépatique gauche), un cas de dédoublement du conduit cystique et cinq cas de canaux biliaires accessoires.

     

Factors producing bile infarction and bile duct proliferation in biliary obstruction

To clarify the factors producing bile infarction and bile duct proliferation in obstructive jaundice, the incidence of the hepatic lesions and the serum levels of the bile constituents were examined in three rat models. (1) Ligation of the common bile duct induced bile infarction, bile duct proliferation, retention of bile in the liver, and elevation of the serum levels of total bilirubin and total bile acids. (2) The rats treated by choledochotomy had bile in the abdominal cavity, but there was no retention of bile in the liver. The degree of development of bile infarction was similar to that of the common bile duct ligation group, but bile duct proliferation was not found: the serum levels of total bilirubin and total bile acids were elevated. (3) In the rats subjected to partial bile duct ligation, bile infarction and bile duct proliferation were seen only in the lobes with ligation of the hepatic ducts: only slight or no elevation of the serum levels of total bilirubin and total bile acids was found. These data suggest that bile infarction is caused by the toxic action of bile constituents other than bilirubin and bile acids, absorbed into the blood from the obstructed biliary system, and that bile duct proliferation is due to mechanical factors following bile retention or direct actions of retained bile in the liver.     

胆总管探查在胆管结石中应用的临床分析

目的 探讨开腹胆总管探查的适应条件、必要性、并发症及胆总管探查的最新进展。方法 对两家医院在2014年1月~2017年6月行开腹胆囊切除胆总管探查术62例患者的临床资料进行回顾性分析,探讨术中胆总管探查的必要性。结果 62例均痊愈,术中证实胆总管扩张 54例,胆总管探查发现结石50例,术中胆道镜探查发现残余结石14例,术后有4例患者出现并发症,其中胆总管残余结石1例,胆道感染2例,胆汁性腹膜炎1例。结论 开腹进行胆总管探查仍具有不可替代的位置,对于胆管多发结石、胆总管明显扩张的患者,术中胆道镜探查是必要的。     

腹腔镜胆道探查在有上腹部手术史患者中的应用

目的探讨上腹部手术后腹腔镜胆道探查手术的安全性及可行性。方法有上腹部手术史行腹腔镜胆道探查的16例患者(甲组),其中男性7例,女性9例;年龄52～84岁,平均年龄65.4岁。3例合并肝内胆管结石,胆管结石直径大于1.5cm患者10例,结石>2枚者7例。胆总管结石合并胆囊结石患者5例。同期无上腹部手术史行腹腔镜胆道探查243例(乙组),其中男性115例,女性128例;年龄30～81岁,平均年龄56.8岁。甲、乙两组手术方式包括腹腔镜胆囊切除、胆总管探查(LCBDE)、胆总管一期缝合术;LCBDE取石T管引流术(LCTD)。结果甲组手术时间为55～235 min,平均手术时间128.1 min;术中出血量20～300 mL,平均出血量130.6 mL;2例中转开腹。乙组手术时间45～190 min,平均手术时间105.2 min;术中出血量15～120 mL,平均出血量45.2 mL;1例中转开腹。两组术中均无胆道损伤、胃肠道损伤、大出血、气体栓塞等严重并发症发生。结论有上腹部手术史的胆道结石患者行腹腔镜胆道探查,由操作熟练的腹腔镜医师进行手术是安全可行的。     

The developing human biliary system at the porta hepatis level between 29 days and 8 weeks of gestation: A way to understanding biliary atresia. Part 1

The developing biliary system in normal human embryos from 29 days to 8 weeks post-fertilization was studied. The primitive extrahepatic bile duct that originates from the embryonic hepatic foregut diverticulum is in contact with the hepatic anlage from the start of organogenesis and remains so throughout the gestational ages examined. The primitive extrahepatic bile duct maintains continuity with the ductal plate from which intrahepatic bile ducts are eventually formed. Contrary to long-held concepts of biliary development, no 'solid stage' of entodermal occlusion of the common bile duct lumen was found at any stage of gestation in the material investigated. Therefore, biliary atresia is not caused by incomplete vacuolization of the 'solid stage'.     

急性胆源性胰腺炎的个体化治疗分析

目的 探讨急性胆源性胰腺炎（ABP）临床个体化治疗方法及效果。方法 回顾性分析34 例ABP患者的临床资料。其中原发病为胆囊结石18例,胆总管结石7例,胆囊结石合并胆总管结石 5 例,未见胆囊及胆管结石但有胆总管扩张表现 4例;轻型急性胰腺炎11 例,重症急性胰腺炎23例。34例均在采用保守治疗的同时实施早期微创治疗,其中经皮肝穿刺胆管引流术（PTCD）15例;胆囊穿刺引流术10例;经内镜逆行胰胆管造影术（ERCP）4例;经皮穿刺胰周引流术＋胆囊穿刺1例,经皮穿刺胰周引流术＋PTCD 4例,其中3例（8.82%）经上述治疗后无缓解施行急诊手术（胆囊切除＋胆总管切开取石＋T管引流术＋胰腺坏死组织清除术＋胰周引流术1例,以及加腹腔减压术2例）。结果 3例急诊手术患者中,2例治愈出院后无ABP再次发作,1例死亡;26例（76.47%）择期手术（胆囊切除术22例、胆囊切除＋胆总管切开＋T管引流术 4例）,随访期间,无ABP再次发作;4例（ 11.76%）患者暂未行手术治疗,随访期间4例中均无ABP再次发作;1例患者微创介入治疗（PTCD）后,住院期间死亡未行手术治疗。全组并发心功能损害15 例（44.12%）,肾功能损害 11 例（32.35%）,肝损害 12例（35.29%）,急性肺部感染9例（26.47%）。治愈32例（94.12%）,死亡2例（5.88%）。结论 对于ABP的治疗应按不同病因和病期采取个体化治疗方案,早期ERCP、超声引导下经皮经肝胆管或胆囊穿刺引流或胰周引流能显著提高疗效。