Some considerations for management of choledochal cysts

BACKGROUND: There are five types of choledochal cysts, which are anomalies that involve intrahepatic or extrahepatic bile ducts, or both. These lesions are found most frequently in patients who are Asian, female, infants but are recognized with increasing frequency in adults. METHODS: We have managed 16 patients with this anomaly. One patient was Asian, and 1 was a child. There were 3 males and 13 females. The mean age was 29 years. There were 9 type I, 1 type II, 1 type III, 4 type IV, and 1 type V cysts. Resection of cysts and hepatico Roux-en-Y jejunostomy were performed in 9 patients for type I cysts. Pancreaticoduodenectomy was performed for a type I and a type IV cyst. The extrahepatic portion of a type IV cyst along with a segment of liver was resected in 1 patient. Operation was terminated on 1 patient with a type IV cyst because of extensive involvement of the intrahepatic ducts. She will undergo liver transplantation. The type II cyst was resected. No surgery was performed on a type III and type V cyst. Four of these patients were previously treated unsuccessfully by internal drainage procedures. RESULTS: There was no mortality. Morbidity was limited to a patient who previously underwent incomplete resection of a cyst and a cyst Roux-Y jejununostomy. No cholangiocarcinoma has been encountered in our patients after a mean follow-up of 5.5 years from the time of initial discovery of the choledochal cyst. CONCLUSIONS: Management of choledochal cysts is successful after their complete removal. Partial cyst resection and internal drainage is less satisfactory because of occasional pancreatitis, cholangitis, and cholangiocarcinoma. Resection of the intrahepatic and intrapancreatic portions of the cysts reduces the risk of cancer even though this risk is low after incomplete cyst excision. Biliary continuity after cyst resection is best established by Roux-Y hepaticojejunostomy.     

成人胆管囊肿癌变11例报告

目的　探讨胆管囊肿癌变的诊断、治疗、预防。方法　总结两家医院 1986年至 1997年11例胆管囊肿癌变的临床病理资料 ,对比手术方式和生存期。结果　胆管囊肿切除 ,胆管空肠吻合术 4例 ,胆管探查 ,T管引流术 2例。肿瘤及胆管囊壁切除胆管成形、胆总管十二指肠吻合、活检、胰头十二指肠切除并肝门部胆管空肠吻合术、切除原胆肠吻合口各一例。 1例根治性切除者至今已存活 3 .6年 ,其余中位生存时间 13 .6个月。结论　用影像学等检查早期发现本病 ,及早根治性治疗是提高本病生存率及改善生活质量的关键 ,早期切除胆管囊肿可以减少胆管囊肿癌变的发生     

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.     

腹腔镜胆总管囊肿根治术的手术体会及技术改进

目的 总结腹腔镜胆总管囊肿切除、肝管空肠Roux-Y吻合术的临床经验,并探讨其手术技巧和技术改进.方法 2010年11月-2012年11月,共实施腹腔镜胆总管囊肿根治术31例.采用4孔法,先游离胆囊,行胆道造影,然后扩大脐部切口提出空肠,完成Roux-Y吻合,还纳肠管后再切除囊肿,行胆肠吻合术.结果 31例患者,2例中转开腹,29例完成腹腔镜下胆总管囊肿根治术,手术时间为200 ～ 460 min,平均260 min;术后早期并发症3例(9.7％),其中胆瘘1例;术后腹壁切口出血1例;Roux-Y胆袢扭转1例.术后患儿均获得随访,随访时间为3～ 27个月,全部患儿肝功能正常,超声检查无结石形成,未见胆管狭窄及扩张.结论 腹腔镜胆总管囊肿根治术安全可靠,效果满意.     

成人胆总管囊肿回盲段间置肝管十二指肠吻合术

为寻求一种良好抗返流的肝肠吻合的方式，在动物试验（狗）的基础上，对4例成人胆总管囊肿行连同囊肿在内的胆总管切除，并行带蒂回盲段间置、肝管十二指肠吻合术。随访5个月至4年，经上消化道钡餐检查无一例间置肠管内返流，而且病人症状全消。提出该术式提供了抗返流的生理性瓣膜，是胆肠吻合中有效的抗返流的较佳术式。     

腹腔镜手术治疗肾上腺囊肿

目的 总结腹腔镜手术治疗肾上腺囊肿的经验.方法 肾上腺囊肿患者32例.囊肿位于左侧17例,右侧14例,双侧1例.囊肿直径平均6.4(3.5～14.5)cm,其中直径＜4.0 cm者3例,4.0～8.0 cm者25例,＞8.0 cm 4例.均行腹腔镜下肾上腺囊肿切除术,其中经腹途径5例,经腹膜后途径27例.对手术适应证、手术时间、术中出血量、术后住院时间等进行比较分析.结果 32例手术均获成功.5例经腹途径者囊肿直径平均(9.5±3.1)cm,手术时间(65.0±12.1)min,出血量(37.2±10.1)ml,术后平均住院时间(4.4±0.7)d;27例经腹膜后途径者分别为(5.8±1.2)cm、(43.3±6.4)min、(23.1±7.6)ml、(3.3±0.6)d;2组以上参数比较差异均有统计学意义(P＜0.05).32例术后平均随访28(2～60)个月,均未复发.结论 经腹腔和腹膜后途径手术治疗肾上腺囊肿各有优势,对于囊肿体积偏小、无明显粘连者采取经腹膜后途径更安全,操作更便捷.     

Postnatal management for prenatally diagnosed choledochal cysts

Purpose

The aim of this study was to determine an appropriate postnatal management plan for prenatally diagnosed congenital biliary dilatation (CBD).

Methods

Between 1962 and 2002, 5 (5.9 %) of 85 patients had CBD diagnosed prenatally and were examined clinically. Of these 5 patients, 2 (group A) underwent delayed primary definitive surgery after percutaneous transhepatic cholangiodrainage (PTCD), 1 (group B) underwent early definitive surgery in the neonatal period, and 2 (group C) underwent delayed primary definitive surgery without PTCD in early infancy (within 6 months after birth). The clinical data, operative findings, intra- and postoperative complications, and follow-up were evaluated in these 3 groups.

Results

There were no postoperative complications, such as catheter-related complications, in group A. However, there was adhesion around the choledochal cyst, and the operation was therefore difficult in group A. The diameter of the anastomosis in the hepaticojejunostomy was small, and the cyst wall was thin in group B. Consequently, anastomotic leakage of the hepaticojejunostomy occurred in group B. Neither operative nor postoperative complications such as anastomotic leakage or stenosis occurred in group C. Slight fibrosis of Glisson’s sheath was seen in 2 patients of groups A and C. No liver cirrhosis was seen in any group.

Conclusions

The authors propose that asymptomatic patients should undergo elective definitive surgery by 6 months of age. For symptomatic patients, especially when a differential diagnosis of type I cystic biliary atresia is doubtful, early definitive surgery is needed before 2 months of age. PTCD appears to be indicated only under certain circumstances, and delayed primary definitive surgery should be performed as early as possible thereafter.     

Type IV-A choledochal cysts: a challenge

Background/Purpose Type IV-A choledochal cysts are characterized by congenital cystic dilatation of the biliary tree extending to involve the intrahepatic biliary channels also. A single-center experience of the management of type IV-A choledochal cysts is presented.Methods Thirty-five out of 105 (33%) patients with choledochal cysts, who underwent surgery at a tertiary care center in northern India from January 1989 to December 2002, were found to have a type IV-A (Todanis classification) cyst. The mean age of the patients was 24 years (range, 3 months to 60 years); 17 patients in the group were adults and 22 were females. Presenting features were abdominal pain, jaundice, cholangitis, and abdominal lump, in various combinations.Results Excision of the extrahepatic part of the cyst and a wide bilio-enteric anastomosis was achieved in 32 (91%) patients, while internal drainage of the cyst was necessitated in 3 patients, for technical reasons: collaterals due to portal hypertension (1 patient) and dense adhesions (2 patients). Six (17%) patients developed postoperative complications: 3 had bilio-enteric anastomosis leaks, with 2 requiring a percutaneous proximal biliary diversion; 2 had intraabdominal bleeds requiring re-exploration, and 1 had external pancreatic fistula that closed spontaneously. Follow-up information was available for 28 (80%) patients. Mean duration of follow up was 25 months (median, 12 months; range, 6 months to 9 years). Three patients required re-operation, for anastomotic stricture (n = 2) and hepatolithiasis and recurrent cholangitis (n = 1) during follow up.Conclusions Excision of the extrahepatic part of the cyst and drainage of the intrahepatic part by a wide hilar or subhilar anastomosis gave satisfactory results in the majority of patients with type IV-A choledochal cysts. Close long-term follow up of these patients is essential, because they are likely to present with complications related to the residual intrahepatic part of the disease.     

"两步评估法"对肝门部胆管癌手术治疗价值初探

目的探讨两步评估方法对肝门部胆管癌术前评估价值。方法根据术前影像学资料,肝门部胆管癌的术前评估可分两步进行:首先对67例肝门部胆管癌病人进行改良T分期,并分析T分期对肿瘤切除率、手术方法的指导作用及与预后相关性;再采用改良T分期结合Bismuth分型方法分层,分析两者结合后对可治愈性手术方式选择及切除率的指导价值。结果改良T分期与肿瘤切除率、切缘阴性率及预后相关。随着T分期的增高,切除率及切缘阴性率显著下降(P= 0.032),T3期手术切除机会很小并且不能行治愈性切除;随T分期的增加,1、3年生存率下降(P<0.001)。但不同T分期的可治愈性手术方式无差异(P>0.05)。再结合Bismuth-Corlette分型表明T1/Bismuth-Ⅰ型的手术切除率和根治性切除率均显著高于其他分期/分型(P<0.05),T1及T2期治愈性手术方式符合Bismuth-Corlette分型手术原则。多普勒彩超联合MRI和MRCP对肿瘤的T分期、Bismuth分型最有价值。结论"两步评估方法"能较好地评估肿瘤可切除性、根治切除率及预后情况,并能指导具体的治愈性手术方式的选择,是一种理想的胆管癌术前评估方法。     

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。     

The management of prenatally diagnosed choledochal cysts

BACKGROUND: Although congenital biliary tree abnormalities are uncommon in the United States, more are being diagnosed antenatally with improved imaging techniques. METHODS: To determine the prognosis of prenatally diagnosed biliary disease, the authors reviewed the treatment of 3 children who had biliary cystic lesions found during routine prenatal ultrasonography. RESULTS: All 3 children were born at term. They had elevated bilirubin levels, and postnatal ultrasound scans confirmed the presence of a biliary cystic mass. They underwent exploration within 2 weeks of life. At operation, 2 children were found to have biliary atresia with a cystic biliary lesion. They underwent Kasai procedures and are doing well at 5 and 9 months of age. The third child was found to have a type I choledochal cyst and malrotation at exploration. He underwent cystectomy with Roux-en-Y hepaticojejunostomy and a Ladd's procedure and is doing well at 3 years of age. CONCLUSIONS: Prenatally diagnosed biliary cysts represent a different spectrum of disease than those diagnosed later in life. The sequelae of biliary disease start before birth, and early operation may be necessary to achieve a good outcome. Because it is impossible to distinguish between choledochal cysts and biliary atresia on antenatal ultrasound scan or magnetic resonance imaging, children with presumed choledochal cysts should undergo early exploration to rule out potential biliary atresia. Excellent outcome is possible with early operation in the absence of severe associated anomalies.     

Recurrent bile duct stricture: causes and long-term results of surgical management

Background/Purpose The management of recurrent bile duct strictures is a challenge for surgeons. This study reports the experience of revision surgery in patients referred following the failure of primary repair, and compares the outcome with that in patients who underwent management of recurrent stricture following primary repair at our institution. Methods Over a period of 15 years, 300 patients with postcholecystectomy benign biliary stricture underwent surgical repair at our institution; 36 patients (12%) were referred after a failed primary repair. Results In 25 (69%) patients, the primary repairs were done at peripheral hospitals. Twelve (33%) had had an early repair, at the time of cholecystectomy while 24 (67%) had a delayed repair at a later date; 83% and 67% of the patients who had undergone early and delayed repair, respectively, had not had a preoperative cholangiogram. Primary repairs performed were a bilioenteric anastomosis (22; 61%) or an end-to-end bile duct repair (14; 39%). Twenty-seven (75%) patients presented within 90 days after the primary repair, and the median interval to recurrent symptoms was 45 days (range, 1 day to 6.1 years). The median delay in referral after the development of symptoms of restricture was 175 days (range, 30 days to 22 years). Twenty-three (64%) patients had high strictures (Bismuth types III-V). All patients underwent a Roux-en-Y hepaticojejunostomy. At a median follow-up of 37 months (range, 12–144 months), 33 of 35 evaluable patients (94%) with recurrent stricture had an excellent/good outcome compared to 223 of 242 evaluable patients (92%) who had had their primary repair at our institution. Ten (4%) patients had a poor result following primary repair at our center. There was a significant difference in the stricture repair-to-recurrence interval between those patients referred to us with recurrent strictures and those who failed after primary repair at our institution (median interval, 1.5 vs 20 months; P = 0.001) Conclusions Patients referred with recurrent strictures had had their primary repair at peripheral settings; the failures were technical, presenting early (median, 1.5 months) with recurrent symptoms, compared to findings in patients with recurrent strictures following primary repair at our center. The long-term outcome following the repair of the primary and the recurrent strictures was no different in our experience.     

Emergency management of choledochal cysts in adult patients

Congenital cystic dilation of the biliary tree is rarely considered as cause of cholangitis in the adult patient. Emergency operation in seven adults with unsuspected choledochal cysts resulted in reoperation in all seven. Each patient presented with right upper quadrant pain, a mass, and cholangitis or jaundice. Abdominal ultrasonography incorrectly identified the cyst as a dilated gallbladder in three of the patients. All initial emergency drainage procedures required subsequent modification to cyst excision and Roux-Y reconstruction. From review of the experience presented and the literature to date, we recommend that otherwise stable patients be managed nonsurgically and undergo endoscopic retrograde cholangiopancreatography or percutaneous transhepatic cholangiography to plan primary single-stage excisional operation. Patients requiring emergency decompression should have cholecystostomy or choledochostomy for good control without compromising subsequent operation. At the time of excision, a technical consideration not previously reported is the presence of small daughter cysts in Calot's triangle which must be distinguished from the hepatic bile ducts. The surgical literature has frequently addressed the problems of elective surgery for choledochal cysts; however, emergency complications requiring urgent operative intervention are seldom addressed. We believe emergency intervention should correct the urgent complication without compromising the definitive surgical treatment.     

先天性胆管囊肿外科治疗要点及"三类五型"分型系统的临床意义

先天性胆管囊肿这一胆管畸形病变会导致胆管系统长期处于炎症状态,因此患者并发胆管结石、囊肿癌变的风险较高。外科手术是治愈先天性胆管囊肿的唯一治疗手段,其治疗要点是尽可能彻底切除囊肿病灶以及彻底实现"胆胰分流"。非规范化的外科手术治疗会给患者带来胆肠吻合口狭窄、残余胆管囊肿甚至囊肿癌变等难以处理的术后并发症,严重影响患者生活质量,威胁患者生命。笔者团队基于东方肝胆外科医院的先天性胆管囊肿大样本回顾性研究结果,围绕先天性胆管囊肿的病理解剖学特点,提出一种新的分型系统,将胆管囊肿分为"三类五型",即肝外胆管囊肿肝门型、主干型、末端型;中央型胆管囊肿;肝内胆管囊肿局限型、弥漫型,以期能够更为精确地指导外科治疗方案的设计和实施,并降低术后远期并发症风险。     

Development of bile duct cancer in a 26-year-old man after resection of infantile choledochal cyst

Anomalous arrangement of the pancreaticobiliary duct is considered to be a high-risk factor for biliary tract malignancy. We report a case of intrahepatic cholangiocarcinoma in a 26-year-old man after total resection of choledochal cyst with anomalous arrangement of the pancreaticobiliary duct at the age of 5 months. He had been doing well after total resection of the choledochal cyst; however, he suddenly presented with a spiky fever and abdominal pain in the right upper quadrant at the age of 26 years. Computed tomographic scan and percutaneous transhepatic cholangioscope revealed multiple stones and stenosis of the hepatic duct and the left intrahepatic bile duct. Histologic examination of a biopsy specimen obtained from the stenotic site showed adenocarcinoma of the intrahepatic bile duct. Left lobectomy with re-reconstruction by right hepaticojejunostomy was performed, and his postoperative course was uneventful. One year after the operation, however, he died of carcinomatous peritonitis with recurrence of cholangiocarcinoma. This report warns us that bile stasis owing to stenosis of the intrahepatic bile duct and repeated cholangitis with multiple stones are high-risk factors for carcinogenesis of the intrahepatic bile duct even after total resection of the infantile choledochal cyst.     

Totally laparoscopic management of choledochal cysts using a four-hole method

Background Choledochal cyst is a rare benign disease of the biliary tract. However, once diagnosed, it must be excised with the gallbladder because of the risk for cancer developing in the biliary tree, including the gallbladder. This report introduces a new surgical technique for totally laparoscopic excision of choledochal cyst and hepaticojejunostomy using a four-hole method. Methods Between October 2003 and May 2005, the authors performed totally laparoscopic choledochal cyst excision for 12 patients. All the patients except one were women, and the mean age was 37.3 years (range, 17–62 years). According to the Todani classification, there were five type Ia cases, four type Ic cases, and three type IV cases. Choledochal cyst excision and Roux-en-Y hepaticojejunostomy were performed laparoscopically using the four-port technique. Results The mean operation time was 228 min (range, 150–330 min). No operative or postoperative transfusion was required. An oral diet was started on postoperative day 3. The average length of hospital stay was 5.8 days. There was no major complication associated with anastomosis leakage or obstruction. No patient had an adverse response, as determined by clinical or laboratory evaluation during a 2- to 19-month follow-up period. Conclusions Considering that choledochal cyst is common among young women, who are especially interested in cosmetic results in addition to complete resolution of medical problems, the laparoscopic management of choledochal cyst may be an attractive treatment option. Presented at the 2005 meeting of the Society of American Gastrointestinal and Endoscopic Surgeons (SAGES), 13–16 April 2005, Fort Lauderdale, Florida     

169例成人胆总管囊肿诊治分析

目的 总结国人成人胆总管囊肿的发病趋势,探讨降低胆肠吻合术后吻合口狭窄的合理术式.方法 回顾性分析1977年7月至2008年10月诊治的169例成人胆总管囊肿患者临床资料.依据影像学检查及手术记录的描述,按改良Todani分型,分为Ⅰ型110例(65.1％),Ⅱ型1例(0.6％),Ⅳ型56例(33.1％),2例因临床资料缺乏无法分型.在所有病例中,手术治疗163例,其中行囊肿切除术119例,内引流术33例,胆道探查并T管引流术5例,手术探查活检5例,内镜下括约肌切开取石术1例.结果 随着诊断方法的进步与普及,成人胆总管囊肿在80年代表现为发病率显著增加,90年代后期至今则趋于稳定.由于对该病认识的深入和治疗方法的改进,既往因误诊而行多次胆道手术的患者近年来逐渐降至25％左右.癌变率由1977年至1995年间的l6.5％降为1996年至2008年间的9.7％.胆总管囊肿行囊肿切除治疗的主要远期并发症为吻合口狭窄引起的肝内胆管结石及反复发作胆管炎.结论 近年,成人胆总管囊肿的发病率趋于稳定.囊肿切除并胆肠吻合术是治疗成人胆总管囊肿的主要方法,可降低癌变率.为防止吻合口狭窄,应该根据肝门部胆管的不同情况术中采取不同的手术方式.     

复合型胆总管囊肿的腹腔镜手术治疗

目的 总结腹腔镜手术治疗复合型(Ⅳ-A)胆总管囊肿的经验.方法 回顾性分析2002-2009年间腹腔镜手术治疗65例胆总管囊肿患儿的临床资料.其中16例为Ⅳ-A型,切除肝外囊肿及肝门部胆管成形后行肝管空肠扩大吻合术.结果 16例复合型胆总管囊肿均顺利完成腹腔镜手术.8例合并肝总管狭窄,予以狭窄段切开或切除后扩大肝管空肠吻合;4例左右肝管汇合处狭窄,于分叉水平向左右肝管切开行双管-空肠吻合;2例合并右肝管开口隔膜狭窄,经肝门胆管将其切开;2例合并左肝管囊肿下游狭窄,自肝门向左肝管切开扩大成形后行肝管-空肠斜形吻合.2例出现术后并发症,1例暂时性胆漏自愈,1例吻合口狭窄再手术后解除.随访观察肝内囊肿明显减小直至消失.结论 腹腔镜提供的视野放大效果有利于囊肿根治性切除及肝门胆管狭窄矫治.对于复合型胆总管囊肿,腹腔镜肝门部或肝内胆肠吻合安全有效.

Abstract：

Objective To summarize our experience of laparoscopic surgery for complex choledochal cysts (type Ⅳ-A). Methods The clinical data of 65 children of choledochal cyst undergoing laparoscopic choledochal cyst resection were retrospectively reviewed from 2002 to 2009 in our institute.Among those type Ⅳ-A cyst was found in 16 patients. Hepaticojejunostomy was performed using a Roux-en-Y jejunal loop after extrahepatic cyst excision and ductoplasty. Results Laparoscopic procedures were successfully performed in 16 patients with type Ⅳ-A cysts. The stenotic segment was splited or excised and a wide hepaticojejunostomy was completed at the porta hepatis in 8 patients with a stricture extending to the level of common hepatic duct. The constrictive confluence of the bilateral hepatic duct was incised and the bi-ductal cystojejunostomy was achieved at the bifurcation in 4 cases. A septum was found at the orifice of right hepatic duct and was excised through the hilar stoma in 2 cases. A downstream stricture of the left hepatic duct was incised from the hilum to the dilated segment along the lateral wall in 2 patients, so that a long intrahepatic cystojejunostomy was completed in an oblique course. Postoperative complications developed in 2 cases including temporary bile leakage in one case and anastomotic stricture in another. The intrahepatic cysts were remarkably reduced in size during the follow-up. Conclusions With the magnified laparoscopic view, the radical resection of extrahepatic cyst and correction of the intrahepatic bile ductal stenosis can be easily performed. Laparoscopic hepaticojejunostomy and/or intrahepatic cystojejunostomy is effective and safe for children with type Ⅳ-A choledochal cysts.