先天性胆管囊肿的外科治疗（附131例分析）

本文报告先天性胆管囊肿１３１例，其中Ⅰ型８９例，Ⅱ型２例，Ⅲ型４例，ＩＶ．型２５例，Ｖ型１１例。１１９例施行手术治疗。作者认为：对先天性胆管囊肿的手术治疗，首次手术时应尽可能切除肝外胆管囊肿，有困难时可大部切除，行高位大口胆肠吻合。对局限于肝段或一侧肝叶的胆管囊肿，可行肝段或一侧肝叶切除。对左右肝叶均有胆管囊肿者，可切除病变较重的肝叶或部分肝组织，解除主要肝管的狭窄与扩张，行肝管空肠吻合术。     

Adult choledochal cysts: an audit of surgical management

BACKGROUND: Choledochal cysts are rare congenital cystic dilatations of the biliary tree. Surgical management has evolved with regards to timing and surgical procedure of choice. We conducted a retrospective review of clinical presentation and surgical management of adult choledochal cysts. METHODS: Thirty-two patients with choledochal cysts who underwent surgery between April 1991 and January 2005 were reviewed. There were 27 Todani Type I, 2 Type II, 2 Type IVA and 1 Type V cysts. Eighty-four per cent of patients underwent complete cystectomy and hepaticojejunostomy. Seven patients had revision surgery comprising completion cystectomy and hepaticojejunostomy. RESULTS: There were no perioperative mortalities. Perioperative morbidity rate was 44% and the commonest complication perioperatively was wound infection (19%). Malignancy was noted in one histological specimen. This patient was disease free for 1 year postoperatively and was subsequently lost to follow up. No further malignancy was found on median follow up of 3.9 years (range, 1-14 years) for the other 31 patients. CONCLUSION: Adult choledochal cysts are rare and are often non-specific in their clinical presentation. In managing patients with choledochal cysts, it is important to first treat complications such as sepsis and pancreatitis before imaging of the biliary tree with endoscopic retrograde cholangiopancreatography or magnetic resonance cholangiopancreatography to evaluate the full extent and type of choledochal cyst. Surgical management should be planned single-stage surgery comprising complete cyst resection, cholecystectomy and Roux-en-Y hepaticojejunostomy and should be carried out by hepatobiliary specialists. Excellent perioperative morbidity and mortality results are possible with this strategy. Malignancy is rare and was only noted in 3% but close follow up is warranted.     

儿童及成人先天性胆总管囊肿对比临床分析

目的　研究儿童及成人先天性胆总管囊肿的不同特点 ,提高对先天性胆总管囊肿的诊治水平。方法　回顾分析 3 2例先天性胆总管囊肿的诊治资料 ,将其分为少儿 ( <14岁 )和成人( >14岁 )发病组 ,分析两者不同的临床表现、诊断和治疗特点。结果　少儿组 17例 ,临床表现为持续性黄疸、肝肿大、腹部包块 ,术前一般情况差 ,保守治疗 6例 ,其中 2例因肝硬化腹水 ,肝功能衰竭死亡 ,1例未愈出院 ,3例延期手术 ;手术 14例 ,13例行肝管空肠Roux -Y吻合 ,1例行肝管十二指肠吻合术。成人组 15例 ,以间歇性右上腹疼痛、畏寒发热、黄疸首次就诊 ,分别行囊肿切除肝管空肠吻合术 ( 12例 )、肝管十二指肠吻合术 ( 1例 )、肝左叶并囊肿切除肝管空肠吻合术 ( 2例 )。结论　先天性胆总管囊肿发病患儿起病急 ,病情发展快 ,易合并肝脏损害 ,应加强围手术期支持治疗 ;成人囊肿切除胆道重建术后 ,应密切随访 ,防治胆管炎及胆道癌变     

Congenital choledochal dilatation: Classification, clinical features, and long-term results

Choledochal cyst is generally accompanied by pancreatobiliary malunion. Pancreatic juice usually refluxes into the bile duct via the malunion, and bile juice occasionally regurgitates into the pancreatic duct. As a result, various pathological conditions occur in the biliary tract, pancreas, and liver. We have revised our classification of choledochal cyst on the basis of malunion. Abdominal mass, pain, and jaundice are regarded as the triad of choledochal cysts, but symptoms are quite non-specific. In neonates and infants less than 12 months of age, choledochal cyst is usually huge and no cylindrical dilatation is observed. Vomiting and jaundice with acholic stool are often observed. No hyperamylasemia is seen, despite the presence of a malunion. Whereas children over 13 months of age tend to show diffuse dilatation, and an abdominal mass is rarely palpable. Epigastralgia with hyperamylasemia, fever, vomiting, and slight jaundice are often observed. Cyst excision is the treatment of choice to prevent the development of ascending cholangitis and cancer in the cyst. Late complications of excisional surgery include recurrent cholangitis, pancreatitis, and rarely, biliary malignancy. These complications usually develop 5 years or more after initial surgery and become the object of reoperation. Complete removal of the extrahepatic bile duct from the hepatic hilum to the intrapancreatic duct and a hilar hepaticoenterostomy with wide anastomosis after ductoplasty, including the coexisting primary stricture, are essential at the primary surgery. Provided a large anastomosis, favorable results can be obtained with either duodenostomy or Roux-Y jejunostomy. Surgical enlargement of the hilar ducts and careful lifelong follow-up should be routine in all patients who have undergone cyst excision. When ductal stricture with intrahepatic gallstones is confirmed, reoperation at an early stage is indicated to obtain a good quality of life with short hospitalization.     

Surgical treatment of type IV-A choledochal cyst in a single institution: Children vs. adults

Background

The treatment of type IV-A choledochal cyst is particularly difficult and remains a challenge because of the rareness and the various presentations of the disease involving not only the extrahepatic but also the intrahepatic biliary tract. The purpose of this study is to analyze our clinical experience for surgical treatment of type IV-A choledochal cyst, and compare between children and adults.

Methods

During a 10-year period of time (2000–2010), clinical data of 81 consecutive patients with type IV-A choledochal cyst were retrospectively analyzed. We divided these patients into two groups, the child group (age ≤ 18 years) and the adult group (age > 18 years). According to whether the patient received additional liver resection, patients were divided into a extrahepatic cystectomy (EHC) group and an additional liver resection (LR) group. The long-term outcomes after surgery were evaluated in two groups.

Results

Of all 81 patients, there were 17 children and 64 adults; 16 children and 35 adults belonged to EHC group, one child and 29 adults belonged to LR group. The morbidity of biliary stricture and/or lithiasis in the adults was significantly higher than that in the children (p = 0.041 < 0.05). In the EHC group, the reoperation rate of adults was significantly higher than that of children (p = 0.019 < 0.05). For adult patients, the morbidity of biliary stricture and/or lithiasis and the reoperation rate in EHC group was significantly higher than that in LR group (p = 0.037 < 0.05 and p = 0.026 < 0.05 respectively). Five adults were found to have cholangiocarcinoma within a follow-up period, while no child was found to. However, for adult patients, no significant discrepancy was observed between EHC group and LR group (p = 0.366 > 0.05).

Conclusions

The present study suggests that the children have better outcomes than adults for patients with type IV-A choledochal cyst after EHC, while LR brings better outcomes than EHC for adult patients.     

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.     

Bile duct cysts: A changing spectrum of presentation

Since the report of our early experience in 1984, the clinical management of 33 patients (23 adults, 10 children) with bile duct cysts has been reviewed. The mean age was 28 years, and female preponderance was observed. Complicated clinical presentations (namely, cholangitis) and coexistent hepato-biliary-pancreatic diseases (present in 45% of the patients) were encountered more frequently in adult patients and those who had had previous cyst enteric drainage operations. Pancreatitis occurred in 27% and cyst-associated malignancy in 9%, with 2 patients alive 29 and 125 months, respectively, postoperatively after curative resection. Cyst type included type I, 58%; type II, 3%; and type VIA, 33%. Anomalous biliary-pancreatic junction (as a long common channel in 18 patients) was observed in 19 patients in whom the anatomy was documented. Eighteen patients (group I) underwent primary extrahepatic cyst excision, and 15 patients (group II) with previous cyst-related operations (35 previous surgical procedures) underwent secondary cyst excision. Mortality was nil, but postoperative morbidity was significantly higher in group II. During a mean follow-up of 51 months, satisfactory results were observed in 94% of the patients. In conclusion, primary complete cyst excision of extrahepatic component is recommended as the treatment of choice, achieving satisfactory clinical results in the majority of patients. The clinical spectrum of the disease has changed in our experience because of the routine use of early cyst excision in patients with bile duct cysts.     

成人胆管囊性扩张症恶变12例报告

近１０年来我院手术治疗成人先天性胆管囊性扩张症５７例，有１２例合并癌变（２１％）。其中６例切除病变（５例同时做内引流，１例Ｔ管引流），单纯胆肠吻合加Ｕ管引流３例，仅行活检３例。手术后中位生存期１０个月。因为成人胆管囊性扩张症的主要危险之一是恶变且预后不良，因此应尽量切除扩张的胆管，如不能切除的部位应充分引流。对已经癌变的病例，最有效的治疗方法是切除恶变的胆管及充分引流未恶变的扩张胆管。     

Long-term outcomes after hepaticojejunostomy for choledochal cyst: a 10- to 27-year follow-up

Introduction

Choledochal cyst (CC) is closely associated with anomalous arrangement of the pancreaticobiliary duct, which is considered a high-risk factor for biliary tract malignancy. Early diagnosis and early treatment for CC could lead to a good prognosis. This study investigated late complications and long-term outcomes after surgery for CC.

Patients and Methods

Fifty-six patients with CC and over 10 years of postoperative follow-up were analyzed retrospectively. All patients had undergone total resection of the extrahepatic bile duct and hepaticojejunostomy.

Results

Six patients showed liver dysfunction manifested in the first 10 years after surgery, but all returned to normal thereafter. Dilatation of intrahepatic bile ducts persisted in 6 postoperatively, and in 3, this was still apparent more than 10 years after. Recurrent abdominal pain was encountered in 3, 1 had pancreas divisum with a pancreatic stone, and 1 had adhesive small bowel obstruction. Two patients developed biliary tract malignancy. A 14-year-old girl died of recurrent common bile duct cancer 2 years after the initial resection of CC with adenocarcinoma. A 26-year-old man with repeated cholangitis owing to multiple intrahepatic bile stones developed cholangiocarcinoma 26 years after the initial resection of CC. Event-free survival rate and overall survival rate were 89% (50/56) and 96% (54/56), respectively.

Conclusions

Choledochal cyst generally has an excellent prognosis with early total resection and reconstruction. Long-term surveillance for the development of malignancy is still essential, especially if there is ongoing dilatation of the intrahepatic bile duct or biliary stones.     

先天性胆总管囊肿多次手术原因分析

目的分析先天性胆总管囊肿(CCC)多次手术的原因。方法回顾性分析我院1995年1月至2004年12月因各种原因行再次及多次手术的CCC15例患者的临床资料。15例中,小儿7例,成人8例。首次手术行囊肿空肠吻合3例、囊肿十二指肠吻合3例(其中1例为妊娠期合并胆总管囊肿)。成人胆总管囊肿被误诊手术4例。囊肿自发破裂行外引流2例。胆漏2例。吻合口狭窄1例。结果13例行2次手术,2例行3次手术。本组1例术后发生胆漏患儿死亡,余均治愈。结论对CCC应尽可能地行囊肿切除,胆道重建,但在某些情况下可考虑先行外引流术以缓解症状,待病情稳定后再行囊肿切除,胆道重建。     

成人先天性胆管囊肿的诊断与治疗(附97例报告)

作者总结了近３０年来成人先天性胆管囊肿９７例的治疗经验。患者平均就诊年龄为３７岁。多数患者症状不典型，常致诊断延误。７４例（７６％）合并肝胆胰系统疾病，１６例合并癌变，癌变率１７％。发现胰胆管合流异常２８例。９４例患者共行胆道手术１６９例次，囊肿切除、肝管空肠Ｒｏｕｘ－Ｙ吻合术及囊肿内引流术为主要术式。术后长期随访表明内引流术后常因胆管炎复发和囊肿癌变而需再次手术，相反囊肿切除术明显降低了胆管炎的复发率及癌变的发生。作者认为囊肿切除、肝管空肠Ｒｏｕｘ－Ｙ吻合术应作为成人胆管囊肿的首选术式。     

Long-term outcomes after excision of choledochal cysts in a single institution: Operative procedures and late complications

Purpose

The purpose of this study was to evaluate long-term outcomes for a minimum of 3 years after cyst excision in children with choledochal cysts, focusing on the relationship between operative procedures and outcomes.

Methods

Between 1977 and 2008, 138 children underwent cyst excision. Follow-up results were obtained from 120 patients. Their mean age was 20.9 years (range 4–49). The mean interval between surgery and conducting the analysis was 16.6 years (range 3–34). These patients were divided into two groups based on their operative procedures: group A (1977–2000) comprising 76 patients who underwent cyst excision with hepaticojejunostomy below the hilum, and group B (2001–2008) comprising 44 patients who underwent excision of the extrahepatic bile duct from the confluence of the hepatic duct to near the level of the pancreatobiliary junction with wide hilar hepaticojejunostomy. When hepatic strictures were seen near the hilum, duct plasty was made. We evaluated the long-term outcomes in the two groups.

Results

Late complications were seen in a total of 18 patients (15.0%). In group A, 16 patients (21.1%) had late complications, which included cholangitis and/or hepatic stones in 9, stones in residual intrapancreatic cysts in 4, intestinal obstruction in 2, and pancreatitis in 1. Of these 16 patients, 12 patients (15.8%), including 7 with hepatic stones (6 IV-A and 1 Ic cysts), 4 with remnant intrapancreatic cysts, and 3 with intestinal obstruction underwent surgical intervention. In group B, none of the patients developed cholangitis, pancreatitis, or stone formation. However, 2 patients (4.5%) developed intestinal obstruction that required surgery.

Conclusions

Although a longer follow-up period is necessary, late complications were more frequent in group A than in group B patients and with type IV-A cysts. We believe that excision of the extrahepatic bile duct with wide hilar hepaticojejunostomy is essential for the prevention of postoperative complications.     

凋亡抑制蛋白基因在先天性胆总管囊肿和胆管癌中的表达及意义

目的 探讨凋亡抑制蛋白基因(survivin)在先天性胆总管囊肿和胆管癌组织中的表达及意义.为进一步研究胆总管囊肿癌变的机制奠定理论基础.方法 采用免疫组化SP法检测Survivin在37例先天性胆总管囊肿组织(其中儿童20例,成人17例)和10例胆管癌组织中的表达,10例正常胆管作为对照.运用SPSS14.0统计学软件处理数据.结果 Survivin在37例先天性胆总管囊肿组织中,11例为阴性,15例弱阳性表达,9例阳性表达,2例强阳性表达.而在10例胆管癌组织中,Survivin有5例阳性和5例强阳性表达,未见阴性表达.正常胆管组织中仅有1例弱阳性表达,两两相比差异有统计学意义(P＜0.01).Survivin在成人胆总管囊肿中的表达强于儿童组,两者间差异有统计学意义(P＜0.01).结论 Survivin参与了胆总管囊肿癌变过程,可能与癌变的发生发展有关,在先天性胆总管囊肿癌变过程中可能起到促进作用.     

Intrahepatic duct dilatation in type 4 choledochal malformation: pressure-related, postoperative resolution

Background

Type 4 choledochal malformations (CMs) may be defined as those with both intrahepatic and extrahepatic bile duct dilatation. The aims of this study were to investigate possible causes of intrahepatic duct (IHD) dilatation in CM and to define the effect of surgery over time.

Methods

This study was a single-center retrospective review of a database of all children with CM undergoing surgery (excision of extrahepatic bile duct dilatation and hepaticojejunostomy) and identified as type 4 (on imaging and at surgery). Data included intraoperative choledochal pressure measurements and biliary amylase content and were expressed as median (interquartile range [IQR]). All comparisons used nonparametric statistical tests. P ≤.05 was regarded as significant.

Results

Twenty children were identified as type 4 CM (age, 4.3 years; range, 2.7-10.4 years) with preoperative IHD dilatation (right duct: diameter [range], 8.5 [4.5-14] mm; left: 8 [4-14.5] mm). Median intraoperative choledochal pressure was 17 (8-27) mm Hg (normal, <5 mm Hg), and intraoperative bile amylase was 3647 (range, 500-58?000) IU/L (normal, <100 IU/L). Preoperative IHD diameter correlated with choledochal pressure (right: r_s=0.46, P = .03; left: r_s=0.34, P = .07) but not with biliary amylase (P = .28 and P = .39, respectively).At 1 year postsurgery, median (range) IHD diameter had decreased to 1 (1-2.5) mm for right duct (P = .0002) and 1.5 (1-3) mm for left duct (P = .0006) and remained stable for up to a 10-year follow-up.

Conclusion

Our data suggest that IHD dilatation is related to sustained increased intrabiliary pressure rather than any intrinsic intrahepatic CM. Effective surgery invariably reduces measured IHD toward normal values.     

109例成人型胆管囊肿的诊治经验

Objective To discuss the diagnosis and treatment of congenital choledochal cyst (CCC).Methods The clinical data of 109 adult patients with congenital choledochal cyst admitted in Peking Union Medical College Hospita between 1984 and 2007 were retrospectively analyzed.The male: female ratio was 1 : 3.8.The mean age was 32.8 years.Seventy-eight of the patients belonged to Todani type Ⅰ, while twenty-six to type Ⅳ and five to type Ⅴ.There was no case of type Ⅱ or Ⅲ.Results Right upper abdominal pain or discomfort and recurrent cholangitis were the predominant presentations.Thirty patients had undergone previous surgical procedures while 26 procedures were done before 2000.B-US and MRCP were the primary methods for diagnosis after 2000.Ninety-four patients underwent complete excision of the extrahepatic cyst and hepaticojejunostomy.Four patients required the left lateral hepateetomy in addition.Four patients were testified malignant change by pathological examination.There were four pancreatic fistulas and two Subphrenic abscesses.There was no postoperative death.Forty-six patients were available for follow-up.The late complications in-eluded recurrent cholangitis, hepaticojejunostomy anastomosis stricture and intrahepatic biliary calcu-la.Conclusions Right upper abdominal pain or discomfort and recurrent cholangitis were the most common presentations in adult congenital choledochal cyst patients.B-US and MRCP should be the prefered imaging methods for diagnosis.Complete excision of the extrahepatic cyst and hepaticojeju-nostomy were ideal treatment.     

109例成人型胆管囊肿的诊治经验

Objective To discuss the diagnosis and treatment of congenital choledochal cyst (CCC).Methods The clinical data of 109 adult patients with congenital choledochal cyst admitted in Peking Union Medical College Hospita between 1984 and 2007 were retrospectively analyzed.The male: female ratio was 1 : 3.8.The mean age was 32.8 years.Seventy-eight of the patients belonged to Todani type Ⅰ, while twenty-six to type Ⅳ and five to type Ⅴ.There was no case of type Ⅱ or Ⅲ.Results Right upper abdominal pain or discomfort and recurrent cholangitis were the predominant presentations.Thirty patients had undergone previous surgical procedures while 26 procedures were done before 2000.B-US and MRCP were the primary methods for diagnosis after 2000.Ninety-four patients underwent complete excision of the extrahepatic cyst and hepaticojejunostomy.Four patients required the left lateral hepateetomy in addition.Four patients were testified malignant change by pathological examination.There were four pancreatic fistulas and two Subphrenic abscesses.There was no postoperative death.Forty-six patients were available for follow-up.The late complications in-eluded recurrent cholangitis, hepaticojejunostomy anastomosis stricture and intrahepatic biliary calcu-la.Conclusions Right upper abdominal pain or discomfort and recurrent cholangitis were the most common presentations in adult congenital choledochal cyst patients.B-US and MRCP should be the prefered imaging methods for diagnosis.Complete excision of the extrahepatic cyst and hepaticojeju-nostomy were ideal treatment.     

109例成人型胆管囊肿的诊治经验

Objective To discuss the diagnosis and treatment of congenital choledochal cyst (CCC).Methods The clinical data of 109 adult patients with congenital choledochal cyst admitted in Peking Union Medical College Hospita between 1984 and 2007 were retrospectively analyzed.The male: female ratio was 1 : 3.8.The mean age was 32.8 years.Seventy-eight of the patients belonged to Todani type Ⅰ, while twenty-six to type Ⅳ and five to type Ⅴ.There was no case of type Ⅱ or Ⅲ.Results Right upper abdominal pain or discomfort and recurrent cholangitis were the predominant presentations.Thirty patients had undergone previous surgical procedures while 26 procedures were done before 2000.B-US and MRCP were the primary methods for diagnosis after 2000.Ninety-four patients underwent complete excision of the extrahepatic cyst and hepaticojejunostomy.Four patients required the left lateral hepateetomy in addition.Four patients were testified malignant change by pathological examination.There were four pancreatic fistulas and two Subphrenic abscesses.There was no postoperative death.Forty-six patients were available for follow-up.The late complications in-eluded recurrent cholangitis, hepaticojejunostomy anastomosis stricture and intrahepatic biliary calcu-la.Conclusions Right upper abdominal pain or discomfort and recurrent cholangitis were the most common presentations in adult congenital choledochal cyst patients.B-US and MRCP should be the prefered imaging methods for diagnosis.Complete excision of the extrahepatic cyst and hepaticojeju-nostomy were ideal treatment.     

109例成人型胆管囊肿的诊治经验

Objective To discuss the diagnosis and treatment of congenital choledochal cyst (CCC).Methods The clinical data of 109 adult patients with congenital choledochal cyst admitted in Peking Union Medical College Hospita between 1984 and 2007 were retrospectively analyzed.The male: female ratio was 1 : 3.8.The mean age was 32.8 years.Seventy-eight of the patients belonged to Todani type Ⅰ, while twenty-six to type Ⅳ and five to type Ⅴ.There was no case of type Ⅱ or Ⅲ.Results Right upper abdominal pain or discomfort and recurrent cholangitis were the predominant presentations.Thirty patients had undergone previous surgical procedures while 26 procedures were done before 2000.B-US and MRCP were the primary methods for diagnosis after 2000.Ninety-four patients underwent complete excision of the extrahepatic cyst and hepaticojejunostomy.Four patients required the left lateral hepateetomy in addition.Four patients were testified malignant change by pathological examination.There were four pancreatic fistulas and two Subphrenic abscesses.There was no postoperative death.Forty-six patients were available for follow-up.The late complications in-eluded recurrent cholangitis, hepaticojejunostomy anastomosis stricture and intrahepatic biliary calcu-la.Conclusions Right upper abdominal pain or discomfort and recurrent cholangitis were the most common presentations in adult congenital choledochal cyst patients.B-US and MRCP should be the prefered imaging methods for diagnosis.Complete excision of the extrahepatic cyst and hepaticojeju-nostomy were ideal treatment.     

109例成人型胆管囊肿的诊治经验

Objective To discuss the diagnosis and treatment of congenital choledochal cyst (CCC).Methods The clinical data of 109 adult patients with congenital choledochal cyst admitted in Peking Union Medical College Hospita between 1984 and 2007 were retrospectively analyzed.The male: female ratio was 1 : 3.8.The mean age was 32.8 years.Seventy-eight of the patients belonged to Todani type Ⅰ, while twenty-six to type Ⅳ and five to type Ⅴ.There was no case of type Ⅱ or Ⅲ.Results Right upper abdominal pain or discomfort and recurrent cholangitis were the predominant presentations.Thirty patients had undergone previous surgical procedures while 26 procedures were done before 2000.B-US and MRCP were the primary methods for diagnosis after 2000.Ninety-four patients underwent complete excision of the extrahepatic cyst and hepaticojejunostomy.Four patients required the left lateral hepateetomy in addition.Four patients were testified malignant change by pathological examination.There were four pancreatic fistulas and two Subphrenic abscesses.There was no postoperative death.Forty-six patients were available for follow-up.The late complications in-eluded recurrent cholangitis, hepaticojejunostomy anastomosis stricture and intrahepatic biliary calcu-la.Conclusions Right upper abdominal pain or discomfort and recurrent cholangitis were the most common presentations in adult congenital choledochal cyst patients.B-US and MRCP should be the prefered imaging methods for diagnosis.Complete excision of the extrahepatic cyst and hepaticojeju-nostomy were ideal treatment.