先天性胆总管囊肿内引流术后癌变

文章总结了８例先天性胆总管囊肿内引流术后癌变病例。指出为防止囊肿癌变及其并发症的发生，对先天性胆总管囊肿病人应尽可能作根治性囊肿切除术和行肝管空肠ＲｏｕｘｅｎＹ吻合术，以预防胆管的癌变，对确诊为癌变病人，主张行胰十二指肠切除术。     

先天性胆总管囊肿内引流术后癌变

总结我科1980～1995年所遇8例先天性胆总管囊肿内引流术后癌变病例，对本病的发病机制、临床特点及防治进行了探讨。临床资料 全组8例，男3例，女5例，年龄25～52岁，平均38，5岁。行内引流术后至出现癌变行再手术的时间为2～18年，中间期为8，2年。原手术方式为单纯囊     

先天性胆总管囊肿癌变的防治：介绍一种新术式

先天性胆总管囊肿（ｃｏｎｇｅｎｉｔａｌｃｈｏｌｅｄｏｃｈａｌｃｙｓｔ,ＣＣＣ）癌变的发生率较高,可以认为它是一种癌前期病变。业已证明胆胰管合流异常是ＣＣＣ的主要病因之一,也是ＣＣＣ容易发生癌变的原因［１］,ＣＣＣ的现代外科处理就是建立在这种认识的基础之上。简言之ＣＣＣ的处理原则是：切除囊肿并实施胰胆分流。本文总结我院１９７９年至１９９９年４月２０年来治疗的病例,以期建立一种确切有效、合乎生理而相对简单的手术方式。１　临床资料１－１　一般资料本组５９例,男９例,女５０例。年龄１５～６８岁,平均３０…     

先天性胆总管囊肿癌变1例

<正>患者女,59岁。主因"间断性腹痛半年、加重10 d"入院。患者无明显诱因出现腹部阵发性隐痛,偶伴腹胀﹑反酸﹑发热,体温最高可达38.1℃。门诊诊断为梗阻性黄疸。体检:急性病容,皮肤﹑巩膜黄染,一般状态欠佳。右上腹压痛阳性,反跳痛﹑肌紧张阴性,腹部叩诊为鼓音,未触及腹部包块。实验室检查:CA19-9 133.10 U/m L,余肿瘤系列未见异常;总胆红素31.4μmol/L,直接胆红素8.2μmol/L。MRCP示胆总管明显扩张,左、右肝     

先天性胆总管囊肿癌变的临床研究

目的探讨先天性胆总管囊肿癌变的临床特点、治疗方法及预后分析。方法回顾性分析我院手术治疗的11例先天性胆总管囊肿癌变的临床资料。结果先天性胆总管囊肿癌变9例,内引流术后癌变2例。年龄、黄疸、体重减轻、总胆红素（TB）、糖类抗原（CA）19—9对于胆总管囊肿是否癌变差异有统计学意义。手术方式、周围脏器侵犯、淋巴结转移、术后免疫治疗对于胆总管囊肿癌变病人预后有明显影响。结论年龄、胰胆管汇合异常是胆总管囊肿癌变发生的危险因素;而黄疸、体重减轻、TB升高、CA19—9升高等因素有助于早期诊断胆总管囊肿癌变。该病预后不佳,根治性手术结合辅助治疗有助于改善预后。     

先天性胆总囊肿内引流术后癌变

文章总结了８例先天性胆总管囊肿内引流术后癌变病例，指出防止囊肿变及其并发症的发生，对行天性胆总管囊肿病奶治性囊肿切除术和行肝管空肠Ｒｏｕｘ－ｅｎ－Ｙ吻合术，以预防胆管的癌变，对确诊为癌变病人，主张行胰十二指肠切除术。     

先天性胆总管囊肿的外科治疗

目的：探讨先天性胆总管囊肿的诊治方法。方法：分析我院近10年外科手术治疗的36例先天性胆总管囊肿的临床资料。结果：全组36例,行囊肿切除、肝管空肠吻合术32例,行囊肿切除、肝左叶切除、肝管空肠吻合2例,2例Caroli病行囊肿前壁切除及部分肝组织切除、囊肿空肠吻合术。全组病例1例发生胆瘘经保守治疗痊愈,2例成年病人发生恶变,全部痊愈。结论：针对先天性胆总管囊肿的分型,采用相应的手术方式是成功的关键。对Ⅱ型Caroli病人肝移植是最有效的方法。     

先天性胆总管囊肿癌变的诊治经验

车文报道先天性胆总管囊肿(简称CCC)癌变9例，均经手术及组织学确诊，为腺癌6例，鳞癌或腺鳞癌3侧。已有周围器官，淋巴结或骨转移者6例。既往有4例曾行CCC肠道内引流术。车组行根浩性切除术3例；姑息性切豫1例；姑息性手术4例；剖腹探查活检1例。术后院内死亡2例．出院后7侧获得3个月～6年的随访。本文讨论了CCC癌变率．文献报道2．5％～15％．本组18.6%。CCC癌变多见于CCC内引流术后及成年患者。在讨论诊断中指出影像学检查难以鉴别CCC之良恶性．确诊需依手术探查及组织学检查。治疗上强调为防CCC癌变对CCC应行切除术而不行内引流术。CCC癌变例应行根冶性切除包括周围受累器官之胰十二指肠切豫术。不适切除病侧．姑息手术可延长病人的生存时间。     

先天性胆总管囊肿癌变的防治经验

目的：总结先天性胆总管囊肿癌变的防治经验。方法；对20年间收治的16例先天性胆总管囊肿癌变患者的临床资料进行回顾性分析。结果：7例既往未做过手术，9例为内引流术后癌变。临床表现为非特异性，其中12例表现为化脓性胆管炎，4例为上腹部包块，16例均伴乏力，消瘦。7例既往无手术史的癌变患者中5例行ERCP检查，其中4例合并胆胰管合流异常。手术方式包括剖腹探查术4例，T管引流术4例，囊肿切除加胰十二指肠切除术3例。囊肿部分发除，肝左外叶切除2例，囊肿切除，肝管空肠Roux-en-Y吻合术3例。癌变主要位于囊壁者14例，术后病理结果腺癌占多数（13例）。术后生存期为4－31个月，平均12．7月。结论：先天性胆总管囊肿癌变的临床表现不典型，术前诊断困难，预后极差，行内引流术后更易癌变。应以预防为主，对先天性胆管囊肿患者推荐行囊肿完全切除术。术中冰切片检查有助确诊，对浸润至胰腺的病例应考虑行囊肿切除及胰十二指肠切除术。     

先天性胆总管囊肿癌变3例

1病例资料例1男,2 8岁。因反复上腹痛1个月,皮肤巩膜黄染半个月入院。伴恶心、纳差、乏力、体重减轻、精神不振等症状。体查:右上腹稍膨隆,轻压痛,未扪及肿块,肝肋下三横指,质偏硬。实验室检查:血清总胆红素1 4 6.7μmol/L,结合胆红素5 1.0μmol/L,碱性磷酸酶4 0 6U/L,谷丙转氨酶2 1 3U/L,谷氨酰转肽酶7 3 3U/L。甲胎蛋白<2 0μg/L,癌胚抗原3.9μg/L,乙肝表面抗原阴性,血淀粉酶1 5 6U/L,尿淀粉酶3 7 6U/L,尿胆原阳性,尿胆红素阳性。B超检查示胆总管囊肿。手术探查见胆总管扩张1 4 0mm×9 0mm×8 0mm,张力较大。打开囊肿见后壁肿块,质…     

小儿巨大胆总管囊肿11例手术体会

目的总结小儿先天性巨大胆总管囊肿的外科手术治疗经验。方法对11例先天性巨大胆总管囊肿患儿施行胆囊切除、胆总管囊肿切除术、肝管夺肠Roux—en—Y型吻合术。结果术后均痊愈出院,10例获随访3～12个月,未见胆道感染、黄疸、营养不良等并发症出现。结论常规术中胆道造影,完全地切除囊肿,合理地处理囊肿远近端胆管异常是巨大胆总管囊肿手术成功的关键。     

先天性胆总管囊性扩张症的外科治疗(附145例报告)

目的　总结我院 3 5年外科治疗先天性胆总管囊性扩张症的经验。方法　对 1964～ 1999年共收治的 15 2例先天性胆总管囊性扩张症中接受手术治疗的 14 5例进行回顾性分析和随访。结果　 15 2例中未手术 7例 ,死亡 1例。手术治疗 14 5例 ,死亡 6例。 1岁以内出现症状、发生肝硬变和死亡例数比 1岁以上组高 (P<0 .0 5 )。单纯囊肿内引流术3 9例 (囊肿十二指肠吻合 5例、囊肿空肠Roux Y吻合 3 3例、囊肿胆囊吻合 1例 ) ,囊肿切除胆肠Roux Y吻合重建胆道10 6例 (胆支空肠袢套叠瓣或矩形瓣成形分别为 3 7例和 2 1例 ,48例未作抗返流瓣 )。获访 77例 ,平均 4.68年。获访病例生长发育良好。结论　该病早期手术肝损伤可逆转 ,预后良好 ,只要患儿能耐受应尽早选择囊肿切除胆肠Roux Y吻合套叠瓣成形胆道重建术     

腹腔镜下胆总管囊肿切除、肝管空肠吻合、腹腔外空肠吻合术50例报告

目的探讨腹腔镜下胆总管囊肿根治切除、肝管空肠吻合、腹腔外空肠吻合术的技巧和中期疗效。方法对大的囊肿,于囊肿中部切开前壁,再用电钩横断囊肿后壁;对小的囊肿,围绕囊肿周围游离,完整切除扩张胆管。对反复发生胆管炎症的患儿,采用Lilly’s方法游离囊肿。脐部切口扩大至1.5 cm,将空肠提出腹壁外,行空肠Roux-Y吻合。腹腔镜监视下肝管空肠端侧吻合。结果16例患儿行囊肿全部切除,34例患儿行Lilly’s囊肿切除。1例早期手术患儿术前反复发作胆管炎症,囊肿周围紧密粘连,分离中渗血明显中转开腹完成手术。手术时间190-450 min,平均226 min。8例患儿需要输血。术后住院时间6-16 d,平均8 d。49例术后随访3-39个月,平均26个月。术后并发症4例：1例发生胆漏,经腹腔引流后自愈;急性胰腺炎1例,保守治疗治愈;肠粘连肠梗阻1例,保守治疗;肠坏死1例,开腹探查见空肠肝支肠管梗阻坏死,行肠切除和再次胆肠吻合手术。余45例无腹痛、发热、黄疸等症状。无胆管狭窄和反流性胃炎病例,无手术死亡。结论分离囊肿后壁,避免门静脉损伤是最关键的腹腔镜操作;胆肠吻合是腹腔镜最难的技术。腹腔镜下胆总管囊肿根治术疗效满意。     

Well-Differentiated Endocrine Carcinoma Originating From the Bile Duct in Association With a Congenital Choledochal Cyst

We encountered a rare case of a well-differentiated endocrine carcinoma originating from the bile duct in association with a congenital choledochal cyst (CCC). The patient is a 28-year-old woman referred to our clinic for pruritus. Laboratory data showed mild elevation of serum hepatobiliary enzymes. Computed tomography and magnetic resonance imaging demonstrated pancreatobiliary maljunction and a Todani type IV-A CCC from the inferior bile duct to the bilateral intrahepatic bile ducts. A solid tumor was detected in the middle portion of the common bile duct. Pancreatoduodenectomy and total extrahepatic bile duct resection was performed. Based on pathologic and immunohistochemical examinations, a diagnosis of well-differentiated endocrine carcinoma was made according to the World Health Organization criteria. To our knowledge, this is the third report of a neuroendocrine tumor originating from the bile duct in association with a CCC.     

介绍一种处理胰内段胆总管囊肿的新手术方法(附12例效果分析)

目的介绍一种对胰内段胆总管囊肿处理的新方法 ,并分析其疗效。方法 12例患者接受该手术方式。胆总管囊肿切除至胰内段,距囊肿远端开口1 cm处行内荷包缝合关闭,石炭酸烧灼残留囊肿粘膜,缝合关闭残留囊肿腔。结果术中出血量为40~160 ml,平均(80.7±55.4)ml,手术时间为60~160 min,平均为(110.3±40.2)min,住院天数5~12 d,平均为(8.3±3.4)d,无并发症。均获随访,平均25.6(2~45)个月,无癌变及感染病例。结论该手术方法针对解剖困难、手术风险大的胰内段胆总管囊肿而言是有效、安全、简单的处理方式。     

Choledochal cyst, pancreatobiliary malunion, and cancer

A review of the clinical features of biliary cancer with choledochal cyst and pancreatobiliary malunion is presented, together with a recent case report. Biliary cancer develops in about 25% of patients with choledochal cyst and pancreatobiliary malunion, and usually occurs in younger patients (usually those in their 40s) than does biliary carcinoma in the general population. The risk of malignancy in the retained cyst with internal drainage is higher than that in the primary cyst. Early excision of the retained cyst should be performed as quickly as possible, even if the patient is symptomless. Some bile acid fractions and refluxed pancreatic enzymes in bile are possibly responsible for carcinogenesis. Carcinoma generally develops in the extrahepatic bile duct and gallbladder, and rarely in the intrahepatic bile duct. In cystic dilatation, cancer usually occurs in the common bile duct, while in diffuse or non-dilated type, it develops in the gallbladder. Multicentric carcinomas develop in the bile duct either synchronously or metachronously. The prognosis of biliary cancer is usually dismal. However, aggressive procedures are now gaining better results than conventional approaches. The procedure of choice for choledochal cyst or malunion is to prevent the development of cancer by performing an early excision. Removal of the entire extrahepatic bile duct is necessary, even in patients with malunion and no biliary dilatation. Carcinoma rarely arises in the intrahepatic bile duct after excisional surgery, probably due to the long-standing stricture of the bile duct. Capacious anastomosis and/or ductoplasty is essential. Carcinoma may also develop in the remnant bile duct. Excision of the distal duct extending into the pancreas is also necessary.     

Pancreatitis After a Primary and Secondary Excision of Congenital Choledochal Cysts

Purpose Pancreatitis has been reported long after total choledochal cyst excision. The aim of this study was to determine if the disease process of postoperative pancreatitis differs between a primary and secondary cyst excision in a long-term follow-up. Methods Among 53 postoperative patients who underwent a total cyst excision and were followed up, 44 patients underwent a primary cyst excision (primary excision group), while 9 patients underwent a secondary cyst excision after a previous cyst-duodenostomy for internal drainage (secondary excision group). The long-term clinical course, including the pancreatographic findings after a total cyst excision, was compared. Results In the primary excision group, six patients had mild pancreatitis. Endoscopic retrograde pancreatography demonstrated ductal dilatation that was limited to the common channel in two patients, concurrent with the ventral duct in three, and extended the duct of Santorini in three. Conservative treatments were carried out in three patients, and endoscopic irrigation in one patient with protein plugs in the ventral duct. A resection of the choledochal remnant in the pancreas was performed in two patients with choledochal remnant-associated pancreatitis. From the secondary excision group, 5 of the 9 patients had chronic pancreatitis. Endoscopic retrograde pancreatography showed entire pancreatic ductal dilatation. Two of these patients underwent duodenal papilloplasty at the same time as secondary surgery; however, the disease progressively worsened. Conclusion In patients undergoing a secondary total excision after internal drainage, it is difficult to half the ongoing aggravating process in pancreatitis.     

Choledochal cyst disease: A review

The authors present a review of the classification, etiology, presentation, treatment and long-term outcome of children and adults with choledochal cyst disease. Unlike children, adults often present with acute biliary tract symptoms or pancreatitis. The treatment of choice remains extrahepatic cyst excision and biliary bypass. This treatment has excellent long-term results that minimize the development of malignancy.     

先天性胆总管囊肿76例疗效观察

目的:探讨先天性胆总管囊肿的诊治与疗效。方法:本组76例,分别采用囊肿切除肝总管空肠Roux-Y吻合加矩形瓣防返流术38例;囊肿切除短段空肠代胆道术38例。术后进行3个月-5年的随访,得出优、良、差三种不同结果。结果:治愈出院74例,死亡2例。随访62例,占84％。囊肿切除肝总管空肠Roux-Y吻合加矩形瓣防返流术随访30例:优11例,良13例,差6例,优良率占80％。囊肿切除短段空肠代胆道加矩形瓣防返流术随访32例:优18例,良14例,优良率100％。结论:囊肿切除肝总管空肠Roux-Y吻合加矩形瓣防返流术术后并发症多,包括吻合口狭窄,逆行感染,复发性胆管炎等,疗效不理想;囊肿切除短段空肠代胆道术虽手术操作复杂,但主要优点是恢复了胆汁的生理通道,手术恢复后疗效相当满意。     

Laparoscopic Resection Of Congenital Choledochal Cyst, Choledochojejunostomy, and extraabdominal Roux-en-Y anastomosis

Background The feasibility of laparoscopic resection of choledochal cyst and hepaticojejunostomy in children is still unclear. This report presents the author’s experience with a first series of patients. Methods Data from 11 consecutive children (median age 17.5 months, SD 22, range 2 to 70) with choledochal cyst scheduled for laparoscopy were collected prospectively. There were nine type I and 2 type V cysts according to Todani’s classification. All except one patient had intermittent jaundice or recurrent pancreatitis. The laparoscopic technique included excision of the cyst. A Roux-en-Y anastomosis was constructed after exteriorization of the small bowel via the infraumbilical trocar incision. After repositioning of the bowel an end-to-side hepaticojejunostomy was carried out laparoscopically. Results The procedures were carried out in nine children without intraoperative events and a median duration of 289 min (SD 62). In two patients, the operation was converted after 60 and 90 min due to a lack of overview at the dorsal margin with problems in separation of the portal vein. Oral food intake was started within 2 days and tolerated well in all except one patient, in whom biliar fluid from the drain led to laparoscopic reevaluation on day 1. A small leak was resutured and the patient was discharged on day 5. In one patient, recurrent cholangitis and a dilated Roux-en-Y loop led to correction of some kinking of the loop via laparotomy after 3 months. All other patients are well with bile-stained stools after a mean follow-up of 13 months. Conclusions Laparoscopic resection of congenital choledochal cyst and choledochojejunostomy in children is feasible. We feel that there is a considerable learning curve with the technique. Future studies will have to prove the feasibility of laparoscopic Roux-en-Y bowel anastomosis without the need for bowel exteriorization.