Congenital choledochal cyst: Occurrence of primary intrahepatic stones and prevention of carcinogenesis

Intrahepatic biliary stones and carcinogenesis are two major complications seen after corrective surgery for congenital choledochal cyst. The incidence of these two complications was reviewed in patients seen at out-patient clinics at a mean follow-up time of 13 years and 6 months. Intrahepatic biliary stones developed in 5 of 56 patients who had undergone a standard excisional operation; in 4 of the 5 patients, the stones were found in the preexisting intrahepatic biliary dilatation (with or without a downstream stenosis), 7–15 years postoperatively. Biliary stricture with dilatation is a putative cause of intrahepatic bile duct stones, and this hypothesis was supported here, since stones developed in all 3 of the patients who had the peculiar configuration of intrahepatic biliary dilatation and stenosis. Biliary carcinoma was not observed in any of the 56 patients who had undergone standard excision of a choledochal cyst followed by hepatico-jejunostomy, but developed in 1 of 12 patients in whom complete excisional resection of the dilated extrahepatic bile duct had not been performed. The occurrence of carcinoma in choledochal cysts is reviewed.     

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Caroli病是一种先天性的肝内胆管囊性扩张症。外科治疗的目的包括清除病灶,防止胆管癌变;通畅胆汁引流,缓解症状。肝切除是目前最常用的外科治疗方式,对局限性的Caroli病可达到根治的效果,病人术后胆管炎等症状可长期缓解。而对于少部分弥漫型病人,也可选择性清除肝内主要的扩张病灶,并进行密切的随访,如病人进一步进展,肝移植常成为治疗的选择。对于多数弥漫型病人,肝移植是最终的治疗选择,可获得满意的长期效果。     

Dilatation of the intrahepatic bile duct associated with congenital anomalous junction of the cystic duct--a new disease entity

We describe 3 children with dilatation of the intrahepatic bile duct, who had anomalous junctions of the cystic duct, 2 high and 1 low, without pancreaticobiliary maljunction. They were all male, and underwent excision of the gallbladder and the extrahepatic bile duct followed by a Roux-en-Y hepaticojejunostomy as a definitive surgery. Postoperatively, the dilated intrahepatic bile duct became normal in size. From these 3 cases, we propose a new disease entity—dilatation of the intrahepatic bile duct because of congenital anomalous junction of the cystic duct.     

变异右肝管横跨与左肝管汇合的解剖学特点和临床意义

目的探讨变异右肝管横跨与左肝管汇合的解剖学特点和临床意义。方法回顾性分析经胆道影像学检出的52例变异右肝管横跨与左肝管汇合病例的临床经过和手术情况，并对其中部分特殊病例进行了随访。结果变异右肝管结石[60．52％（23／38）]和左肝管结石[86．84％（33／38）]的比例较高，伴随的胆管扩张和狭窄也较多见。变异导致的胆汁流体力学紊乱和血管鞘压迫可能是感染和结石高发的原因。变异右肝管的结石漏诊率为34．78％（8／23），结石残留比例高达86．95％（20／23）。1例在行左半肝切除时不慎将变异右肝管切断。结论变异右肝管横跨与左肝管汇合有易发生肝内胆管结石的倾向，其诊断治疗有其特殊性及困难性，应引起临床重视。     

肝移植术后胆道狭窄的临床特点及治疗效果(附60例报告)

目的 探讨肝移植术后并发胆道狭窄的临床特点及治疗效果.方法 回顾性分析60例肝移植术后胆道狭窄患者的临床资料.并对其临床特点和治疗效果进行了分析和讨论.结果 肝移植术后胆道狭窄的主要症状为胆道梗阻和胆管炎表现.其平均诊断时间为术后(195.5±146.5)d.胆道狭窄早期,肝功能损害主要为血清丙氨酸转氨酶、总胆红素、谷氨酰转肽酶及碱性磷酸酶水平升高,而白蛋白降低;胆道狭窄晚期时,丙氨酸转氨酶水平下降,出现"胆酶分离"现象.经内窥镜逆行胰胆管造影(ERCP)检查发现,胆道狭窄部位为单纯吻合口狭窄6例;肝门部和/或合并吻合口狭窄11例;肝内胆管弥漫性狭窄11例;吻合口合并肝内胆管弥漫性/节段性狭窄32例.其它并发症还包括胆管内有异物、狭窄近端扩张、胆管扭曲及十二指肠乳头旁憩室等.经ERCP介入治疗,取出胆管内异物、坏死组织、结石以及狭窄段球囊扩张等处理后,33例患者治愈,18例好转,9例治疗无效.结论 肝移植术后胆道狭窄的临床表现既有特征性,又有多样性,且合并症较多;采用ERCP介入治疗胆道狭窄效果理想.     

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目的：探讨腹腔镜胆总管球囊导管扩张术和扩张导管扩张术在处理胆管狭窄中的临床应用。方法：采用腹腔镜胆总管探查，根据扩张情况对炎性狭窄或膜状狭窄结石取净者，胆管切口即时缝合。段状狭窄结石取净者，塑料支架内引流。恶性狭窄晚期者，自膨式金属支架或塑料支架内引流。结石未取净者，T管外引流。结果：炎性狭窄或膜状狭窄扩张61例，良性段状狭窄扩张37例，恶性太狭窄扩张12例，胆漏6例，残石3例，中转开腹1例，死亡1例。结论：选择合适病人，经腹腔镜胆总管探查术中采用球囊导管或扩张导管扩张胆管狭窄有效、可行。     

消化道重建术后肝内外胆管结石经皮经肝胆道镜治疗的临床分析

目的探讨消化道重建术后肝内外胆管结石经皮经肝胆道镜（PTCS）治疗的临床价值。 方法回顾性分析2014年3月至2018年6月什邡市人民医院行PTCS治疗的26例消化道重建术后肝内外胆管结石患者临床资料,分析PTCS临床治疗效果。 结果3例术中扩张瘘管时误操作导致瘘管断裂后中转开腹,手术中转率为11.54%（3/26）。23例手术成功,成功率88.46%（23/26）,其中1次取净结石9例（39.13%）,2次6例（26.09%）,3次6例（26.09%）,4次2例（8.70%）。术后患者间接胆红素（IBIL）、直接胆红素（DBIL）和总胆红素（TBIL）均显著低于术前水平,差异有统计学意义（P<0.05）。随访8~41（19.03±4.06）个月,结石复发率为26.92%（7/26）,再次行PTCS取石治疗,随访期间未再次复发。围手术期无一例死亡,并发症发生率为11.54%（3/26）,其中1例术中胆道壁损伤后大量出血,2例术后发生胆管炎,给予对症治疗措施后好转。 结论PTCS是治疗消化道重建术后肝内外胆管结石的有效手段。     

肝外胆管梗阻性疾病的MRCP诊断价值

目的探讨磁共振胆胰管成像技术对肝外胆管梗阻性疾病的诊断价值。方法对52例经病理或随访证实的肝外胆管梗阻性疾病患者的MRCP资料进行回顾性分析,总结良恶性肝外胆管梗阻的不同MRCP表现。结果52例中,MRCP均能够准确测定胆管扩张程度和梗阻的水平,其中良性梗阻27例,MRCP主要表现为胆管均匀扩张及逐渐狭窄,肝外胆管较肝内胆管扩张明显,肝内胆管呈“枯树枝状”;恶性梗阻25例,MRCP主要表现为胆管截断,胆胰管扩张,出现“双管征”,肝内外胆管扩张一致。恶性梗阻患者的肝内外胆管扩张程度明显大于良性梗阻者。结论MRCP作为一种无创的影像检查方法,对肝外胆管梗阻性疾病的诊断具有较高准确性,     

CT对肝外胆管梗阻性质的鉴别诊断

目的：探讨良恶性肝外胆管梗阻病变的CT表现及其诊断意义。方法：回顾经手术病理证实的肝外胆管梗阻病变76例(良性34例，恶性42例)。结果：肝内胆管呈枯枝状轻中度扩张，肝内外胆管不一致扩张(内轻外重)，肝外胆管远段梗阻，梗阻部胆管呈削尖状狭窄，肝外胆管壁呈弥漫环形增厚，对良性梗阻的诊断有重要意义。肝内胆管呈软藤状重度扩张，肝外胆管中段梗阻，梗阻部胆管呈伴或不伴肿块的截断型或突然狭窄型，肝外胆管壁局限不规则增厚，高度揭示恶性梗阻。结论：良恶性肝外胆管梗阻病变均有特征性CT表现，通过分析胆管形态和临床资料基本能判断梗阻的性质。     

Congenital dilatation of the common bile duct and pancreaticobiliary maljunction—clinical implications

Clinical conditions and diagnosis  Congenital dilatation of the common bile duct is a disease in which the extrahepatic bile duct, or both the extra and intrahepatic bile ducts, is dilated in various ways. Pancreaticobiliary maljunction is a disease in which the pancreatic duct meets the bile duct outside of the duodenal wall beyond the sphincter Oddi. Recently, these diseases have been thought to be closely related to each other but to be different malformations. Biliary tract carcinoma, especially bile duct carcinoma, is found in about 30% of patients with congenital dilatation of the bile duct. The concomitance of bile and pancreatic juice and their stasis in the biliary tract induce cellular proliferation and reproduction and stimulate genetic alterations in biliary epithelium, which may play an important role in carcinogenesis of the bile duct. Therapeutic strategies  Endoscopic retrograde cholangiopancreatography is useful for examining pancreaticobiliary maljunction. The operation is dilated bile duct resection and hepaticojejunostomy, which ensure that pancreatic juice and bile do not mix in the bile duct. Gallbladder carcinoma develops in more than 90% of pancreaticobiliary maljunction without bile duct dilatation.     

Reoperation for congenital choledochal cyst.

A reoperation after excisional procedure was carried out in seven cases due to early or late postoperative complications. Of the 12 patients with early complications, four underwent relaparotomy due to anastomotic leakage and bleeding. Late complications were seen in nine patients with recurrent cholangitis caused by an anastomotic stricture, and three patients with intrahepatic involvement required a reoperation several years after the initial surgery. Recurrent cholangitis after biliary reconstruction mainly occurs due to an anastomotic stricture of the hepaticoenterostomy. There was no significant difference in the results between hepaticoduodenostomy and hepaticojejunostomy over a long follow-up period. A wide anastomotic stoma that permits free drainage of bile into the intestine is imperative to the prevention of cholangitis, and can be created by an incision extending along the lateral wall of both the hepatic ducts with a hepaticoenterostomy at the hilum. This procedure is obviously necessary in all patients with or without intrahepatic involvement. Carcinoma of the intrahepatic ducts and the retained distal choledochus have rarely developed in patients undergoing cyst excision followed by biliary reconstruction. Complete excision of the whole extrahepatic bile duct could prevent carcinoma arising in the distal choledochus, although it could not prevent carcinoma arising from the intrahepatic ducts. However, patients with carcinoma of the intrahepatic duct were reported to have had symptoms of biliary stricture for a long time since the cyst excision. Bile stagnation in the intrahepatic ducts is possibly responsible for the development of carcinoma. A wide anastomosis resulting in free drainage of bile appears to be essential to the prevention of carcinoma arising in the intrahepatic ducts after cyst excision.     

手术结合纤维胆道镜治疗肝内胆管狭窄及肝内胆管结石的疗效分析

目的探讨手术结合纤维胆道镜治疗肝内胆管狭窄及肝内结石的疗效。方法通过T管窦道胆道镜球囊扩张治疗肝内胆管狭窄、取净肝内结石。结果81例肝内胆管结石患者合并肝内胆管狭窄43例，占53．09％；利用胆道球囊反复扩张，解除狭窄的成功率88．37％；取净结石的成功率100％。术后所有病例无严重并发症。结论纤维胆道镜治疗肝内胆管狭窄克服了外科手术的盲区，具有创伤小，治愈率高，并发症少，疗效确切的优点。内镜与手术的结合使肝内胆管狭窄的治疗取得了十分满意的疗效。     

Postoperative choledochofiberscopic removal in intrahepatic stones

An endoscopic approach for the diagnosis and treatment of intrahepatic stones is presented based on the experience of 4 cases, in which complete removal of intrahepatic stones was done by postoperative choledochoscopy. Postoperative choledochoscopy for the diagnosis and treatment of gall-stone disease is not difficult to perform. The choledochofiberscope is inserted into the bile duct via choledochal fistula 3 weeks after the operation. Stones are crushed or grasped with forceps under direct vision. In some cases, the stricture in the intrahepatic biliary tree is dilated with the use of a high frequency waves cutting knife to facilitate stone removal. In fact, over 410 stones were successfully removed in one case by 28 postoperative choledochoscopies without any complications. The details of the choledochofiberscope developed by us with the close cooperation of Machida Company of Tokyo was presented. Postoperative choledochoscopy seems to be a safe and simple procedure and is useful in the treatment of intrahepatic stones.     

Intrahepatic cholangiocarcinoma arising 34 years after excision of a type IV-A congenital choledochal cyst: Report of a case

We report a rare case of intrahepatic cholangiocarcinoma (IHCC) arising many years after excision of a type IV-A congenital choledochal cyst. A 44-year-old man was transferred to our hospital with acute cholangitis more than 34 years after several operations for congenital biliary dilatation. Imaging showed a huge tumor in the left medial section of the liver, extending to the porta hepatis. Although he had no jaundice, the intrahepatic bile ducts showed cylinder-like dilatation with narrowing of the hilar bile duct. At surgery, the tumor was found to arise from the dilated intrahepatic bile duct just above the narrow portion. He underwent a left hepatic trisectionectomy with a vascular procedure. Microscopically, the tumor was confirmed to be moderate-to-well-differentiated tubular adenocarcinoma. Thus, when the narrow segment is left untouched, careful long-term follow-up is important to detect new lesions at an early stage.     

局部胆管内压大小及持续时间对犬胆管壁的影响

目的探讨局部胆管内压大小及持续时间对犬胆管壁的影响。方法设计并自制一内置入式胆管扩张器,体外测试扩张气囊内压力与注水容积及囊径的关系。将20只杂交犬随机分为五组,A组为假手术组仅在胆总管内放置胆管扩张器;B、C组分别向扩张气囊内注水0.1ml、0.2ml（球囊压力分别为0.24Kpa、0.58Kpa）扩张胆总管10min后抽空气囊并留置胆管扩张器作胆汁引流;D、E组分别向扩张气囊内注水0.1ml、0.2ml扩张胆总管后,不抽空气囊并留置作胆汁引流。各组分别术后7天再次剖腹。观察各组扩张前后血清总胆红素（TB）、丙氨酸转氨酶（ALT）、天冬氨酸转氨酶（AST）值及胆管直径（BD）变化、胆管壁及肝组织学改变。结果与A组比较,C组扩张后BD增宽（P〈0.05）。D组扩张后BD显著增宽（P〈0.01）,E组扩张后TB、ALT、AST均增高（P〈0.01）。A、B组胆管壁组织学无明显变化,C组胆管壁粘膜充血水肿,部分粘膜脱落,D组胆管直径增粗及胆管壁增厚,E组胆管壁部分坏死,与网膜或十二指肠粘连。结论局部胆管内压大小及持续时间对犬胆管壁的影响存在双向互变关系,正常胆管壁在一定的时间内可承受一定的局部胆管内压而不致发生胆管壁的病理变化,适宜的局部胆管内压可引起局部胆管及其上游胆管壁扩张,反之,胆管壁可出现破坏性改变。     

A case of primary intrahepatic cholesterol gallstones difficult to manage by percutaneous transhepatic cholangioscopy

A case of a 60-year-old man with primary intrahepatic cholesterol gallstone was reported. Several stones, each size 3mm in diameter, were packed in the diverticulum-like dilated part of the posterior-inferior-ventral bile duct branch of the right lobe and 3 stones, each size 3-4mm in diameter, were scattered separately in the lateral segment of the left lobe of which the bile duct branches were diffusely tortuous and with mildly irregular dilatation. Morphologic appearance of the biliary system except in these 2 areas was normal. All stones including a large gallbladder stone consisted of cholesterol. Intraluminal approach, not only ERC but also PTCS, can hardly detect intrahepatic stones of more peripheral areas, although combination of US and enhanced CT may be useful even though bile duct branches are not dilated. We failed in PTCS lithotomy in this case for the first time in our experience with more than seventy cases of intrahepatic stones. However, by PTCS it was possible to investigate the morphology of the biliary system in detail and consequently, to make the diagnosis of primary intrahepatic stones. This patient has cured by cholecystectomy and minimum hepatectomy.     

Congenital cystic dilatation of bile ducts

Congenital cystic dilatation of bile ducts is a rare condition. We report a retrospective study about 18 patients having congenital bile duct cysts. According to Todani's classification, 11 cases were type I and 7 were type V. Six patients from the first group had a pancreatobiliary maljunction. A total resection of the cyst was conducted in the type I cysts. Anatomopathologic examination showed an adenocarcinoma of a common bile duct cyst. In one case, a cancer of the gall bladder associated to a common bile duct cyst in another case. Three patients with segmental dilatation of intrahepatic bile ducts (type V) underwent liver resection. Four patients had a diffuse form, one of them was treated by percutaneous drainage, and in the other cases a hepatojejunostomy was performed. Postoperative course was complicated with acute cholangitis in these four cases. Percutaneous drainage and antibiotics allowed a positive outcome in most of the cases. In one case, secondary biliary cirrhosis occurred as a long-term complication. Congenital cystic dilatation of bile ducts is considered to be a precancer state. Enterocystic anastomosis is proscribed and the resection has to be as complete as possible.     

腹腔镜下胆道镜液电碎石术治疗肝内外胆管结石

目的:总结腹腔镜下胆道镜液电碎石术治疗肝内外胆管结石的手术方法、效果及安全性。方法:回顾分析2005年1月至2012年1月采用腹腔镜下胆道镜液电碎石术治疗23例难取性肝内外胆管结石患者的临床资料,观察结石取净率及并发症发生率。结果:23例术中均碎石成功,其中22例一次性取净结石,结石取净率95.7%;1例因结石数量较多,术后6周经T管窦道行胆道镜取石术。无胆道穿孔、大出血、胆漏、切口感染等并发症发生。术后3～4 d拔除腹腔引流管,5～7 d出院。术后4周常规行T管造影,夹管3 d后拔除T管。结论:腹腔镜下结合胆道镜液电碎石术可显著提高肝内外胆管结石的疗效,手术安全、可靠。     

肝内外胆管结石患者术后病变胆管的影像学转归

目的：通过观察肝内外胆管结石患者治疗前后受累胆管的影像学变化评估治疗效果。方法：收集2006—2009年间符合条件的62例肝内外胆管结石患者的临床资料并进行随访,复查MRI+磁共振胰胆管造影（MRCP）。根据手术方式分成A组（胆总管切开取石+T管引流+肝部分切除术,8例）,B组（胆总管切开取石+T管引流+肝部分切除术+术后经T管窦道胆道镜取石术,16例）,C组（胆总管切开取石+T管引流+术后经T管窦道胆道镜取石术,38例）。观察各组的影像学转归情况;将各特征因素进行分级赋值,累计每组手术前后的评分分值,判断疗效。结果：全组手术前后肝内胆管扩张率、肝外胆管扩张率、肝内胆管狭窄率分别为98.4% vs. 79.0%,90.3% vs. 67.7%,40.3% vs. 29.0%;术后结石复发率为9.7%。3组术后的胆总管最大径均较术前明显减小（均P<0.05）,A组手术前后肝内胆管最大径差异无统计学意义（P>0.05）,而B,C组均明显小于术前（均P<0.05）。3组术后评分均明显低于术前（均P<0.05）,而3组间两两比较结果显示,B组手术前后评分差值大于C组,差异有统计学意义（P<0.05）。结论：经手术及胆道镜取石后,受累肝内外胆管大多未能恢复至正常状态。肝部分切除是对肝内胆管结石治疗的理想术式。     

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先天性胆管扩张症亦称先天性胆管囊肿,其病因目前认为主要是胰胆管合流异常,是一种比较少见的临床疾病,典型临床表现为黄疸、腹痛、腹部包块,常伴有胆管炎、胰腺炎、癌变等并发症。超声诊断通常作为首选检查方法,磁共振胰胆管成像(MRCP)结合CT检查是目前最为常用的诊断和术前评估方法。囊肿彻底切除+肝管空肠Roux-en-Y吻合术是目前最为推崇的手术方式,但对于肝内胆管的囊肿,目前认为肝移植可能是最终有效的选择。