先天性胆管囊状扩张症的手术治疗

目的　探讨先天性胆管囊状扩张症的最佳手术方式。方法　回顾分析我院 1980～2 0 0 2年 76例经手术治疗的先天性胆管囊状扩张症的临床诊疗资料。结果　本组 76例中 ,13例行囊肿空肠Roux en Y吻合术 ;3例行囊肿十二指肠吻合术 ;7例行囊肿外引流术 ;1例行憩室型总胆管囊肿切除T管引流术 ;5 5例行囊肿切除、总肝管空肠Roux en Y吻合术 (占手术病例的 72 4 % ) ;2例Ⅳa型囊肿行肝左外叶切除加总胆管囊肿切除、总肝管空肠Roux en Y吻合术 ,1例Ⅴ型囊肿行肝左外叶切除、总胆管切除、肝门胆管空肠Roux en Y吻合术。 16例囊肿内引流术、7例囊肿外引流术和5 5例囊肿切除总肝管空肠Roux en Y吻合术的术后并发症发生率分别为 5 6 3%、2 8 6 %和 3 6 % ,经统计学分析差异有显著性 (P <0 0 1)。结论　手术是先天性胆管囊状扩张症惟一有效的治疗方法。外引流术可用于全身状态极差而不能耐受较复杂手术的病人 ,待全身状况改善后应及时行二次手术 ;对于较为复杂的Ⅳa型和Ⅴ型病变 ,应选择合适的病例行肝叶切除术或肝移植术 ;总胆管囊肿切除、肝管空肠Roux en Y吻合术是Ⅰ型先天性胆管囊状扩张症的一种较为合理的手术方式。     

16例I型先天性胆管扩张症诊治体会

目的总结I型先天性胆管扩张症的诊治体会。方法16例I型先天性胆管扩张症患者经术前彩超扫查和磁共振水成像（MRCP）检查确诊,均行扩张部胆管（囊肿）黏膜剥除和胆一肠Rouz—en—Y吻合术,对患者的临床资料进行回顾性分析。结果16例患者均顺利完成手术。术后并发胆漏1例,经腹腔引流2周后痊愈。其中12患者例获得5—10年随访,1例偶发反流性胆管炎,1,2次／年,其余患者均恢复良好。结论胆管扩张部黏膜剥除加胆肠Roux—en—Y吻合术是I型先天性胆管扩张症的主要治疗手段,疗效好。但在急性感染发作期,可先行抗感染治疗或扩张部胆管外引流术,待症状控制、全身情况改善后,再行根治性手术。     

先天性胆总管囊肿的外科治疗

目的 比较先天性胆总管囊肿不同术式的治疗效果。方法 对1985年1月-1998年12月57例先天性胆总管囊肿的术式及疗效进行分析。结果 以I型为主(44例)占77．2％。B超确诊率为84．2％。57例中,囊肿切除、肝总管空肠Roux—Y吻合37例,囊肿大部切除,胆管与空肠Roux—Y吻合12例。其中有14例行囊肿引流术后再次行囊肿切除术。囊肿切除术明显降低胆管炎的复发,术后随访治疗效果优良占89．1％。结论 囊肿切除、胆管与空肠Roux-y吻合术应作为胆总管囊肿的首选术式,囊肿切除困难时应尽量大部分切除,并行大口胆管肠吻合。     

先天性胆管囊肿内引流术后胆管癌:附25例报告

目的 探讨先天性胆管囊肿内引流术后癌变及其手术处理原则。方法 对1970～1996年间收治的先天性胆管囊肿行内引流术的82例患者进行随访，并对其中术后25例癌变患者的临床资料进行分析。结果 本组先天性胆管囊肿内引流术后癌变率为30．49％(25／82)，其中囊肿十二指肠吻合术后癌变率为35．29％(14／51)，囊肿空肠Roux—en—Y吻合术后癌变率为22．58％(1l／31)。25例中3例行胰十二指肠切除术，4例行肿瘤切除胆道重建术，4例行局部病灶切除外引流术，14例肿瘤广泛转移无法切除仅行外引流术。结论 囊肿内引流术后癌变率高，应该摒弃该术式。     

�������������Ե�������֢�����ؽ���ʽ̽��

先天性胆管扩张症亦称先天性胆管囊肿(congenital choledochal cyst),可发生于肝内外胆管的任何部位,其中以肝外胆管受累为常见。通常按胆管病变的部位、范围对其进行分型,分型方法较多,其中以Todani于1977年提出的临床分型最为常用。此分型将先天性胆管扩张症分为以下5型[1]:I型:胆总管单发梭状囊肿;Ⅱ型:胆总管憩室;Ⅲ型:胆总管末端囊肿;IVa型:肝内、肝外胆管囊性扩张;IVb     

手术治疗成人先天性胆管扩张症41例分析

目的总结我院普外科治疗成人先天性胆管扩张症的经验。方法回顾分析2005年4月至2013年9月安阳地区医院普外科收治的接受囊肿切除、肝总管空肠Roux-Y吻合的41例成人先天性胆管扩张症患者的临床资料。根据Todani分型:Ia型27例,Ic型12例,IVa型2例。结果术后发生胆漏4例,胰漏1例,急性胰腺炎1例。32例随访1~8年,5例表现轻度胆管炎,2例吻合口狭窄,1例发生癌变。结论囊肿切除、胆管空肠Roux-Y吻合是成人先天性胆管扩张症首选治疗,该病易早期手术,可逆转肝损害,预后良好。     

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。     

成人先天性胆总管囊肿的诊断与治疗体会

目的总结成人先天性胆总管囊肿的诊断和治疗方法。方法回顾分析我院2000年1月至2009年10月19例经手术治疗的成人先天性胆总管囊肿患者的临床资料,全组病例均行囊肿切除、肝总管空肠Roux．Y吻合术,其中有二次胆道手术史的1例合并肝内胆管扩张及肝内胆管结石加行左肝外叶切除术．另一例因肝总管狭窄加行高位胆管成型术。结果术后并发胆漏1例．经非手术治疗治疗效果欠佳,再次手术引流治愈,本组无死亡病例。结论囊肿切除加肝总管空肠Roux-Y吻合术是肝外胆管囊肿治疗的首选方法,局限性肝内胆管囊肿可行肝叶切除术。     

成人先天性胆管扩张症手术治疗

目的 探讨成人先天性胆管扩张症手术治疗的经验体会。方法 回顾分析1985年1月至2004年12月中国医科大学附属第一医院普外科收治的成人先天胆管扩张症68例手术治疗资料。依Flanigan分型：Ⅰ型59例，Ⅱ型3例，Ⅳ型6例。所有病例均行囊肿切除，结果 术后发生胆汁漏6例。胰漏3例，急性胰腺炎2例。死亡2例，56例经1-20年随访，随访率为82．3％。5例表现轻度胆管炎，2例吻合口狭窄。余下49例均痊愈，无胆管炎及胆管狭窄表现。结论 囊肿切除、胆管空肠Roux-en-Y吻合是成人先天性胆管扩张症的首选治疗。切除囊肿时，囊肿近端在尽量切净囊肿基础上行大口吻合，防止术后吻合口狭窄；囊肿远端在保证睫液引流通畅、不损伤胰管开口的基础上，尽量切净囊肿内膜。     

成人先天性胆管囊状扩张症的临床分析

目的:探讨成人先天性胆管囊状扩张症的诊断及治疗。方法:回顾性分析1996年1月—2012年5月收治的53例成人先天性胆管囊状扩张症患者的临床资料。结果:53例患者均行手术治疗,Ia,Ib,Ic型39例和II型4例行囊肿完整切除、肝总管空肠Roux-en-Y吻合术,另3例Ia型行囊肿大部分切除、肝总管空肠Roux-en-Y吻合术;IVa型1例行肝左外叶切除、囊肿完整切除、胆管成形、肝总管空肠Roux-en-Y吻合术,另1例行囊肿完整切除、肝总管空肠Roux-en-Y吻合术;V型1例行左肝内胆管囊肿切除术;2例癌变患者,其中1例行癌变囊肿切除、局部转移淋巴结清扫术,1例行囊肿切除、左肝内胆管肿瘤切除术。53例手术患者中获随访42例,随访时间为6个月至3年,良性患者情况良好;2例癌变患者,1例术后生存26个月,死于肿瘤复发,多系统器官功能衰竭,1例术后26个月肿瘤复发,肝转移,再次手术行左半肝切除、S5(第V段肝脏)切除、肝肠吻合术,术后2个月出现肿瘤进展,死于多系统器官功能衰竭。结论:成人先天性胆管囊状扩张症的手术方式选择与治疗效果密切相关,不同的临床分型应选择不同的手术方式。     

先天性胆总管囊肿再次手术的原因分析及治疗

目的 探讨先天性胆总管囊肿再次手术的原因及治疗方法.方法 对41例先天性胆总管囊肿再次手术患者的临床资料进行总结分析.结果 按先天性胆总管囊肿Todani分型,41例中Ⅰ型32例,Ⅱ型1例,Ⅳ型3例,Ⅴ型5例(其中1例呈弥漫性左右肝分布).既往有1次胆道手术史者30例,2次者8例,3次者3例.41例中合并有胆管结石40例,胆道感染33例,肝门部胆管狭窄7例,原胆肠吻合口狭窄6例,囊肿癌变5例.本次行切除肝外胆管囊肿、肝管空肠Boux-en-Y吻合术者32例,其中2例囊肿癌变者同时行肝十二指肠韧带骨骼化和区域淋巴结清扫术;行肝叶切除联合胆总管囊肿切除、肝管空肠Roux-en-Y吻合术者8例;行胆肠吻合口狭窄切开成形、胆肠内引流重建术1例.41例术后均获随访,随访时间3个月至10年,平均53.7±32.1个月,随访期内发生胆道感染4例,无吻合口狭窄或结石复发等并发症发生.结论 胆总管囊肿术式选择不当及诊断失误导致的多种并发症是导致再手术的主要原因,肝内外胆管囊肿切除、肝管空肠Roux-en-Y吻合术可取得较为满意的疗效.     

Cystic involvement of the roof of the main biliary convergence in adult patients with congenital bile duct cysts: a difficult surgical challenge

BACKGROUND: Complete cyst excision of the extrahepatic disease component with biliary reconstruction on proximal healthy bile ducts is considered to be the treatment of choice in patients with congenital bile duct cysts (BDC). Proximal cystic disease that extends to the roof of the main biliary convergence (MBC) might challenge this standard of surgical care. METHODS: A retrospective multicenter study was conducted in 4 European surgical centers concerning their experience with adult patients suffering from type I and IV BDC according to the Todani classification. Clinical presentation, operative management, and postoperative outcome were compared between patients with or without proximal extrahepatic cystic disease that involved at least the roof of the MBC (defined as being BDC with MBC involvement subgroup). RESULTS: From an overall series of 49 adult patients suffering from type I or IV BDC according to the Todani classification, 7 patients had BDC with MBC involvement (14%). Patient age, clinical presentation, duration of symptoms, associated major coexistent hepatobiliary and pancreatic diseases, and synchronous cancer were not significantly different in these patients compared with a control group of 42 adult patients with BDC without MBC involvement. Incomplete proximal cyst excision rate was 86% in the cases of BDC with MBC involvement. Early and late postoperative results were similar in BDC with MBC involvement and in the control group of adult patients, but the incidence of subsequent cancer was significantly higher in the BDC with MBC involvement group (29% vs 0%; P < .02). CONCLUSION: BDC that involves the roof of the MBC is a real surgical challenge to obtain complete proximal cystic disease excision. As suggested in this small study, primary incomplete excision of this particular form of BDC might expose the patient to the risk of subsequent cancer, a feature that must be confirmed in larger series.     

先天性胆管扩张症诊治过程中应该注意的几个问题

目的复习文献及结合本中心的诊治经验,探讨先天性胆管扩张症诊治过程中应该注意的问题。方法总结分析我院2000年3月至2012年5月共收治的147例先天性胆管扩张症患者的临床资料。结果 147例患者中,接受手术治疗126例,其中术式包括:①囊肿完整切除、肝总管空肠端侧吻合98例;②囊肿大部分切除、肝总管空肠端侧吻合14例;③胰十二指肠切除术3例(其中1例为胆总管下端囊肿癌变);④11例癌变患者1例行胰十二指肠切除术,7例行胆管癌根治术,3例行姑息性外引流术;⑤1例患者因合并胆总管囊肿破裂出血并胆汁性腹膜炎而行单纯外引流术。126例手术患者中获随访89例,随访率为70.6%,随访2个月-8年,平均37个月。结论先天性胆管扩张症的手术方式已较为规范,但是关于囊肿切除范围、胆道重建中抗反流袢的长度以及成人先天性胆管扩张症等治疗方面应该引起外科医师的重视。     

Clinicopathologic classification of congenital cystic dilatation of the common bile duct

From 1970 to 1975, 103 patients with a localized dilatation of the biliary passages were treated at the First Department of Surgery of Kyoto University Medical Center. Of the 103, 101 had congenital cystic dilatation of the common bile duct (Alonso-Lej type I). These dilatations were classified into two subtypes, “infant type” and “adult type,” based on the anatomic location and clinical features. Infant type cyst was typically large cyst of the choledochus and most common in infancy and childhood. Adult type cyst was fusiform dilatation of the biliary tract, and most common in adults. Gallstones were seen in most of the patients with this type cyst.     

Isolated cystic dilatation of the cystic duct

Presented was an unusual case of congenital cystic dilatation of the extrahepatic biliary tract in which the cystic changes were confined solely to the cystic duct. There was concomitant common bile duct obstruction secondary to inflammation and mass effect from the cyst. Management consisted of cholecystectomy, excision of the cyst, common bile duct exploration, examination of the ampullary region by duodenotomy, and choledochoduodenostomy.     

С�������Ե�������֢�о���չ

先天性胆管扩张症(congenital biliary dilatation,CBD)高发于亚洲人群,普遍认为其与胆总管壁薄弱和远端胆总管梗阻有关。胆总管壁远端梗阻的成因尚不清楚,比较流行的为胰胆管合流异常学说。产前诊断的年龄越小,扩张越明显,肝损害越重,生后要越早进行手术矫治。目前CBD被简化分为两型:胆总管远端狭窄型和非狭窄型。无论患儿的年龄大小或者症状的轻重,囊肿彻底切除、肝管空肠Roux-en-Y吻合术是根治CBD的标准术式。术前及术中详细的了解患儿整个胰胆道系统的改变情况,同时处理肝内胆管和胰管及共同管的异常病变,是减少手术后并发症的关键。     

Bile duct cysts in adults: a multi-institutional retrospective study. French Associations for Surgical Research.

OBJECTIVE: To review the features of adult patients undergoing surgery for bile duct cysts, focusing on the anatomy of the biliary tree as well as the long-term outcome. SUMMARY BACKGROUND DATA: Bile duct cysts (BDCs) are uncommon in Western countries, and the majority of reported cases originate from Asia. Japanese authors have emphasized the frequent association of extra- and intrahepatic bile duct dilatations, but grading of patients based on Todani's classification is often hindered by the absence of an accurate definition of types IC and IVA cysts. Moreover, despite the increasing use of extrahepatic cyst excision, little is known about the long-term outcome in patients with intrahepatic bile duct involvement. METHODS: Forty-two adult patients with BDC were treated between 1980 and 1992 in 17 institutions of the French Associations for Surgical Research. Clinical presentation, radiologic presurgical evaluation, and surgical procedures were analyzed. The long-term postsurgical outcome was derived from patient charts, attending physicians, or direct patient contact. RESULTS: Twelve patients (30%) had recurrent abdominal pain or jaundice from childhood. Seven (17%) had undergone prior cystenterostomy. Twenty-one (50%) had a Todani-type IVA cyst with extra- and intrahepatic bile duct involvement. Of these, nine had segmental, exclusively left-sided intrahepatic bile duct dilatation. Biliary carcinoma was encountered in five patients (12%). Extrahepatic cyst excision with a Roux-en-Y hepaticojejunostomy was performed in 34 patients with type I or IV cysts. The overall operative mortality rate was 2.4%. Long-term results were clearly correlated with cyst type: during a mean follow-up of 8.4 years, 11 of 12 patients (92%) treated by cyst excision for type I cyst remained free of symptoms, whereas 31% of patients who underwent surgery for type IV cyst had episodic or severe cholangitis with intrahepatic stones. CONCLUSIONS: In patients with BDC, particular attention must be given to the associated intrahepatic bile duct dilatations. We propose a modification of Todani's classification to distinguish cystic, segmental, and fusiform dilatations of the intrahepatic biliary tree in type IV cysts. In patients with segmental left intrahepatic cystic dilatations, combined left liver lobectomy and extrahepatic cyst excision is suggested to decrease late postsurgical biliary complications.     

Biliary carcinogenesis in pancreaticobiliary maljunction

The coexistence of bile duct carcinoma in choledochal cysts is well known. With large number of cases of congenital bile duct dilatation and pancreaticobiliary maljunction (PBM), it is now apparent that gallbladder carcinoma is more frequent in PBM without bile duct dilatation. The incidence of Gallbladder cancer in choledochal cyst and bile duct cancer in PBM, regardless of the presence of bile duct dilatation is significantly higher than that in control patients with biliary cancer but without PBM. A recent survey shows that the incidence of bile duct cancer in PBM without dilatation is equivalent to that of gallbladder cancer and bile duct cancer in patients with choledochal dilatation. Pathology in PBM is a result of reflux of pancreatic juice and stasis of the mixture in the biliary system. Carcinogenic factors thus present in the biliary contents induce epithelial changes in the biliary tract. In the biliary contents, activated pancreatic enzymes and secondary or deconjugated bile acids are markedly increased and they irritate the biliary mucosa. Mutagens are proven to form in the bile of PBM. In the epithelia of PBM, hyperplasia, metaplasia, and dysplasia are often found, and proliferative activity is increased; furthermore, K-ras gene mutation and overexpression of p53 protein are demonstrated. As for treatment of PBM, whether it is symptomatic or not, an operative procedure, is necessary, to prevent carcinogenic changes in the gallbladder and bile duct. Regardless of whether dilatation is present or not, total excision of the extrahepatic bile duct, along with gallbladder, is the treatment of choice, followed by hepaticojejunostomy or hepaticoduodenostomy.     

109例成人型胆管囊肿的诊治经验

目的 探讨成人型胆管囊肿的临床特点和手术方式,总结胆管囊肿的诊治经验.方法 回顾性分析1984-2007年北京协和医院收治的109例成人型胆管囊肿的病例,总结病例特点.109例中男女之比为1:3.8.平均年龄32.8岁.Ⅰ型78例,Ⅳa型26例,Ⅴ型5例,没有Ⅱ、Ⅲ型病例.结果 成人型胆管囊肿临床表现以右上腹不适或疼痛占大多数,共100例(91.7%),其次为寒战、发热等发作性胆管炎症状.30例(27.5%)有过胆道系统手术史,26例初次手术在2000年前施行.B超结合MRCP是确诊的主要手段.94例行囊肿切除、肝管空肠Roux-Y吻合重建术,4例因肝内病变局限于左肝加做左肝外侧段切除术.4例囊肿恶变.无手术死亡病例.手术近期并发症有胰瘘、膈下脓肿.后期并发症主要是发作性胆管炎、吻合口狭窄和肝内胆管结石.结论 成人型胆管囊肿的临床表现不典型,以右上腹痛或不适最为多见.B超是初查的主要手段,为明确诊断、了解胆管囊肿分型和累及范围可首选MRCP.胆管囊肿理想的手术方式是囊肿切除、肝管空肠Roux-Y吻合重建术.     

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.