Adult choledochal cyst

OBJECTIVE: Choledochal cyst is a rare disease in adults. Excellent results have been achieved with proper management. However, malignancy complicating choledochal cyst still carries a dismal prognosis despite radical surgery. The aim of this study was to review the clinical course and operative results of the disease in adulthood, with emphasis on the occurrence of malignancy. METHODS: A retrospective review of adult patients who underwent surgery for choledochal cysts in a 12-year period was performed. RESULTS: Of the 25 adults, 80% were female and the median age was 30 years. A total of 32% of patients had previous drainage procedures for their choledochal cyst disease. Malignancy was noted in five patients (20%). There were significantly more males among the patients with malignancy than among those with benign disease (60% vs 10%), a greater number of older patients (median age, 39 vs 27 years) and more impaired liver function tests, but there was no difference in terms of incidence of previous drainage procedures between the two groups. There was no operative mortality; operative morbidity was 36%. No significant long-term complications were noted in the benign group but three of the five patients with malignancy died or developed recurrence within a few months after surgery. CONCLUSION: The prognosis for patients with malignancy complicating choledochal cyst remains poor despite aggressive radical surgery such as Whipple's operation or additional hepatectomy. Malignancy should be suspected in older and male patients who present with jaundice or impaired liver function tests. Total cyst excision and hepaticojejunostomy is an effective and safe treatment for patients with the common type I choledochal cyst in order to reduce the chance of subsequent development of malignancy.     

Outcomes of laparoscopic versus open resection of pediatric choledochal cyst

BackgroundUntreated pediatric choledochal cyst (CC) is associated with complications including cholangitis, pancreatitis, and risk of malignancy. Therefore, CC is typically treated by surgical excision with biliary reconstruction. Both open and laparoscopic (lap) surgical approaches are regularly used, but outcomes have not been compared on a national level.MethodsThe Nationwide Readmissions Database was used to identify pediatric patients (age 0–21 years, excluding newborns) with choledochal cyst from 2016 to 2018 based on ICD-10 codes. Patients were stratified by operative approach (open vs. lap). Demographics, operative management, and complications were compared using standard statistical tests. Results were weighted for national estimates.ResultsCholedochal cyst excision was performed in 577 children (75% female) via lap (28%) and open (72%) surgical approaches. Patients undergoing an open resection experienced longer index hospital length of stay (LOS), higher total cost, and more complications. Anastomotic technique differed by approach, with Roux-en-Y hepaticojejunostomy (RYHJ) more often utilized with open cases (86% vs. 29%) and hepaticoduodenostomy (HD) more common with laparoscopic procedures (71% vs. 15%), both p < 0.001. There was no significant difference in post-operative cholangitis or mortality.ConclusionsAlthough utilized less frequently than an open approach, laparoscopic choledochal cyst resection is safe in pediatric patients and is associated with shorter LOS, lower costs, and fewer complications. HD anastomosis is more commonly performed during laparoscopic procedures, whereas RYHJ more commonly used with the open approach. While HD is associated with more short-term gastrointestinal dysfunction than RYHJ, the latter is more commonly associated with sepsis, wound infection, and respiratory dysfunction.Level of evidenceLevel III: Retrospective Comparative Study.     

Hepaticoduodenostomy for Biliary Reconstruction After Surgical Resection of Choledochal Cyst: a 25-Year Experience

We reviewed our experience of hepaticoduodenostomy done for choledochal cyst from 1982 to 2007 at our center. The aim of the study was to evaluate the efficacy of hepaticoduodenostomy as a mode of biliary reconstruction after surgical resection of choledochal cyst, to look for any complications associated with hepaticoduodenostomy, with review of the literature. A total of 56 patients underwent hepaticoduodenostomy after surgical resection of choledochal cyst, of which 54 patients underwent primary cyst excision with hepaticoduodenostomy and 2 patients underwent redo surgery (hepaticoduodenostomy). Most of the patients had an uneventful postoperative recovery. Early complications were biliary leak in three patients which subsided on conservative treatment. Long-term complication was seen in only one patient who developed anastomotic stricture 18 years after primary resection of choledochal cyst and hepaticoduodenostomy. Hepaticoduodenostomy is a relatively safe procedure with very low complication rates. It requires a single anastomosis and is simple to perform.     

Complicated choledochal cysts

Choledochal cysts have a potential for causing a variety of complications. This article presents the incidence, types, and presentation of complications in patients with choledochal cysts and the results of surgery in these patients. Prospectively collected data of 81 patients with choledochal cysts treated at a tertiary care center were retrospectively analyzed. Sixteen (20% of surgical patients; 100% of study group) patients were found to have a complicated choledochal cyst that either manifested as an atypical presentation or necessitated a change in the treatment planning. Eight (50%) of these patients had complications of infective nature, and another 8 (50%) had noninfective complications. Fifteen (94%) patients with complications were adults and 11 (63%) patients had Todani type-IVa cysts; a cystic type of intrahepatic component in 10 patients. Five (31%) patients had undergone prior cystenteric drainage operations. Excision of the choledochal cyst was possible in 14 (88%) patients under both emergency and elective situations; cyst excision was performed in the second stage after an initial external drainage procedure in two of these patients. There was no mortality and no major postoperative complications. Complications of choledochal cysts are more likely in adult patients and in those with both intra- and extrahepatic cysts (Todani type-IVa). Despite the presence of complications, the choledochal cyst can be safely excised in a majority of patients.     

Choledochal Cysts: Differences Between Pediatric and Adult Patients

Choledochal cysts in children and adults are believed to be different, but direct comparison between them is lacking in the literature. This study was aimed to identify the clinicopathological differences between 42 children and 59 adults with choledochal cyst treated by same surgeons at the Cathay General Hospital. The mean follow-up period was 8.9 years. The result showed that the female-to-male ratios were 1.5:1 in pediatric patients and 4.9:1 in adult patients. Compared with adults with choledochal cyst, the pediatric patients presented more abdominal mass (52.4% vs 21.2%, P = 0.002) and less abdominal pain (76.2% vs. 98.0%, P = 0.002), are more frequently associated with anomalous pancreaticobiliary ductal union (85.7% vs. 59.6%, P = 0.005) and sudden severe stenosis of terminal choledochus (76.2% vs. 42.3%, P = 0.001), are less commonly associated with choledocholithiasis, are not associated with malignant transformation (0% vs 21.2%), and have fewer perioperative and long-term complications. Nevertheless, patients who received total excision had fewer surgical complications in both groups. This result shows that choledochal cysts in pediatric and adult patients are different in clinicopathological manifestations, prognosis, and the underlying abnormalities of the pancreaticobiliary system, suggesting that patients with choledochal cyst should be managed according to these differences.     

Choledochal cysts in adults

HYPOTHESIS: Choledochal cyst is rarely diagnosed in adulthood. When complicated by biliary tract malignancy, the disease has a distinct presentation and carries a dismal prognosis despite radical surgical resection. DESIGN: Retrospective study. SETTING: Tertiary referral center. PATIENTS: A retrospective study was performed on 30 adult patients who presented with choledochal cyst from January 1, 1989, to December 31, 2000. MAIN OUTCOME MEASURES: The clinical presentation, management, and outcome of patients with and without biliary tract malignancy. RESULTS: Nine patients (30%) had biliary tract malignancy complicating choledochal cyst (group A). Compared with 21 patients without malignancy (group B), group A patients had a significantly higher incidence of previous internal drainage operations for choledochal cyst (P =.049) and presentation with cholangitis (P =.03). Four patients in group A underwent pancreaticoduodenectomy and 3 received a palliative biliary drainage operation. The overall median survival of patients in group A was 12 months. Complete excision of choledochal cyst and Roux-en-Y hepaticojejunostomy were performed for all patients in group B, among whom 2 underwent concomitant hemihepatectomy. The operative morbidity and mortality were 14% and 0%, respectively, and there were no long-term complications with a median follow-up of 66 months. CONCLUSIONS: Biliary tract malignancy complicating choledochal cyst in adults should be suspected in patients with a history of internal drainage of choledochal cyst and presentation with cholangitis. Complete excision of choledochal cyst with Roux-en-Y hepaticojejunostomy is the treatment of choice for patients without malignancy and can be performed with low operative morbidity and absence of long-term complications in adult patients.     

成人I型胆总管囊肿的手术治疗

目的 总结成人I型胆总管囊肿手术治疗经验.方法 回顾性分析经我科囊肿切除+肝管空肠Roux-en-Y吻合术的21例I型成人胆总管囊肿的临床资料.结果 3例发生术后早期并发症包括胆漏2例,胰瘘1例,均经非手术处理治愈.无手术死亡,2例切除标本发现胆管癌.随访9个月～5年,平均36个月,1例术后3年发生胆管炎,其余病人无...     

完全腹腔镜手术治疗成人先天性胆总管囊肿的临床分析

目的:探讨完全腹腔镜胆总管囊肿切除并肝管空肠Roux-en-Y吻合术治疗成人先天性胆总管囊肿的可行性、安全性及疗效。方法:回顾性分析昆明医科大学第一附属医院2014年5月—2016年5月12例行腹腔镜下胆总管囊肿手术治疗患者的临床资料。结果:11例患者完全腹腔镜下完成胆总管囊肿切除+肝管空肠吻合术,1例为减少手术费用在腹腔外行手工肠肠吻合。手术时间240~310 min,平均280 min;术中出血20~150 m L,平均60 m L;术后住院时间5~7 d,平均6.5 d。术后随访2~12个月,未出现手术并发症,无死亡病例。结论:完全腹腔镜下胆总管囊肿切除+肝管空肠Roux-en-Y吻合术治疗成人先天性胆总管囊肿是安全可行的,且具有明显微创优势,可进一步推广。     

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.     

Management of choledochal cyst: laparotomy or endoscopic

The most generally accepted therapy of choledochal cyst is cystectomy and biliar derivation by laparotomy. Last years, endoscopic papilotomy by ERCP has been a valuable therapeutic alternative, no only a diagnostic method. In this study, we reviewed five pediatric patients operated in our Deparment in last five years for choledochal cyst. The initial therapy was laparotomy (n=4) and endoscopic papilotomy by ERCP (n=1) This one was made in other Hospital. Follow-up has been between one and five years. All patients are living. Four patients who were operated by laparotomy are asyntomatic. Patient who was treated by ERCP needed a new ERCP in first posoperative month. Five years ago, she had a seriuos acute pancreatitis and we decided laparotomy and biliar derivation. Since laparotomy, she had two new episodes of acute pancreatitis and she has needed a new endoscopic dilatation with ballon by ERCP. She has been asyntomatic for four months. In conclusion, we think laparotomy with biliar derivation is safer than ERCP in management of children with choledochal cyst. ERCP must be reserved to emergency situations before laparotomy or after postoperative complications, never as exclusive therapy.     

A New Operative Approach for Type I Choledochal Cysts

To present a novel modification of the surgical technique for open excision of type I choledochal cysts. The treatment of choice for choledochal cyst is surgical excision because such cysts are associated with an increased incidence of cancer. The commonly used operative technique provides poor access to the structures posterior to the cyst, principally the portal vein, and poor visibility of the junction of the cyst with the hepatic ducts and the pancreatic duct. The modification reduces these problems. The key operative step involves early transection of the choledochal cyst near its midpoint. This provides improved access to the back of the cyst. Slitting the sides of the cyst allows clear visibility from within the cyst of the union of the hepatic ducts with the cyst, and the entrance of the pancreatic duct, thus protecting them. Excision of type I choledochal cysts using the described method was completed in 11 adult patients between 2003 and 2012. One patient (9 %) was found to have a concurrent gallbladder cancer and underwent an R0 resection at the time of choledochal cyst excision. No patients experienced any intraoperative complications. One patient (9 %) developed a portal vein thrombosis postoperatively. The described technique is a safe and feasible method for the excision of extrahepatic choledochal cysts, and provides an advantage in allowing the surgeon to visualize the hepatic and pancreatic ducts from within the cyst and thus protect them during cyst excision.     

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 cysts in children: a review of 28 years of treatment in a Dutch children's hospital.

OBJECTIVE: To analyse the results of surgical treatment of choledochal cysts. DESIGN: Retrospective study. SETTING: Children's hospital, The Netherlands. PATIENTS: 14 children presenting with choledochal cysts. INTERVENTIONS: Choledochoduodenostomy, Roux-en-Y choledochojejunostomy or Roux-en-Y hepaticojejunostomy. MAIN OUTCOME MEASURES: Morbidity and mortality RESULTS: The mean age of the patients was 20 months (2 weeks to 7 years). 10 patients had a type I choledochal cyst; three a type IV, and one a type V. Mean follow-up period was 6 years (18 months to 16 years). One patient with a type I cyst died of Klebsiella pneumoniae that was resistant to treatment. One patient with a type I cyst treated by choledochojejunostomy had two episodes of cholangitis. Another patient with a type I cyst, treated by choledochoduodenostomy, had one episode of cholangitis. Both could be treated with antibiotics. The other patients had had no complications up to 1997. CONCLUSION: This rare anomaly may lead to severe complications when left untreated or after late treatment. It is easy to manage with low associated morbidity.     

Choledochal cyst cholangiocarcinoma arising from adenoma: case report and a review of the literature

A case of cholangiocarcinoma arising in an unresected choledochal cyst in adulthood is presented. Although typically diagnosed in pediatric population, as many as 20% to 30% of choledochal cysts can be discovered in adulthood. Unresected choledochal cyst is clearly associated with increased risk of cholangiocarcinoma. Proper surgical treatment includes cyst resection and bilioenteric anastomosis. Asymptomatic patients with choledochal cyst previously treated by biliary diversion without cyst resection present a challenging issue and should be considered for cyst resection. Association of choledochal cyst with pancreaticobiliary anomalies is reviewed.     

Diagnosis and management of hydatid liver disease in children: a report of 156 patients with hydatid disease

Background/PurposeThere are many published reviews on adult hydatid disease and a guideline published by World Health Organization Informal Working Group (WHO-IWGE) in 2010. However, there are very few reports on hydatid liver disease in children with limited numbers of patients, and no comments were offered on childhood hydatid liver disease in the WHO-IWGE 2010 guideline. The aim of this study is to present our 17-year experience with 156 pediatric patients with hydatid liver disease and provide a treatment algorithm for children.MethodsThe clinical records of 156 children with hydatid liver disease treated from January 1994 to January 2011 were retrospectively reviewed. Patient sex, age at diagnosis, symptoms, disease location, cyst numbers and sizes, treatment choices, medical treatment duration, surgical methods, and complications were recorded. Treatment of liver hydatidosis included 3 different schedules: (1) small (<5 cm) liver cysts treated with albendazole (ABZ) only, (2) cysts (>5 cm) located at the liver surface treated with surgery combined with ABZ, and (3) all (>5 cm) liver cysts embedded deep in the liver parenchyme treated with percutaneous drainage and ABZ. Albendazole was given (10 mg/kg twice a day) and continued for 6 months after initial therapy.ResultsThere were 92 boys and 64 girls with an average age of 9.2 years (range, 1.1-15 years). A total of 376 cysts were detected in 156 patients. The follow-up period ranged from 1 to 10 years (median, 6.5 years). Complications were classified according to the Dindo classification. After the first 6 months of therapy, grade I complications occurred in 12.1% of patients, grade II complications in 7.4%, and grade IIIb complications in 7.3%. There were no grade IIIa, IVa, or IVb complications. At 1 year, grade II complications were recorded in 9.6% of 15 patients, and grade IIIb complications, in 1.2% of patients. During the 17 years reviewed, there were no mortalities (0% grade V complications).ConclusionsBased on this experience, we believe that suitable treatment should be chosen based on factors such as cyst number, cyst location (on the surface or deep in the organ), proximity to vascular structures, whether the cyst is complicated, and additional organ involvement or not. In addition, although the results of our study mostly agree with the results in the WHO-IWGE 2010 report, there are some noticeable differences between these 2 studies. Hence, we believe that the WHO-IWGE 2010 recommendations should be updated by incorporating the childhood observations.     

BILE DUCT CYSTS: PRESENTATION IN ADULTS

Bile duct or choledochal cysts are rare congenital anomalies and presentation in adult is infrequent. Seven cases of type I/IV bile duct cysts presenting in adult life have been treated by the authors during the past 2 years. The diagnosis was made using a combination of ultrasound and cholangiography (endoscopic retrograde cholangiopancreatography/percutaneous transhepatic cholangiography). All cases underwent total cyst excision and Roux-en-Y hepaticojejunostomy. There were no operative complications and all patients were asymptomatic in the follow-up period, which ranged from 3 to 23 months.     

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.     

Pediatric hepatobiliary disease

The surgical aspects of pediatric hepatobiliary disease concern a unique set of diagnoses, disease processes, diagnostic issues, and treatment problems. In this context, this article discusses features of biliary atresia, choledochal cyst, gallbladder disease, and liver abscess in the infant, child, and adolescent.     

108例先天性胆管囊肿的诊治经验

目的 总结先天性胆管囊肿的诊治经验。方法 对1980年－2000年间收治的108例先天性胆管囊肿患者的临床资料进行回顾性分析。结果 108例的临床表现为：儿童病例多出现典型的腹痛、黄疸及腹部肿块,成人症状多不典型,常导致误诊,57例（52．7％）合并胆胰系统其他疾病,18例合并癌变,癌变率为16．6％。108例患者中,94例行B型超声检查,46例行ERCP检查,20例行PTC检查,71例行腹部CT检查,均确诊。合并胰胆管合流异常39例。1985年前诊断及分型以B超和术中所见决定,手术以内引流术为主。1985年后,诊断以ERCP、CT为主,同时行B超检查,术式以囊肿切除,肝管空肠Roux-en-Y吻合术为主,1994年提出防止先天性胆管囊肿癌变的新术式。术后并发症以胆道系统逆行性感染为主,均抗生素治疗后痊愈,全组无手术死亡。结论 先天性胆管囊肿20年诊治的观念有很大的改变,ERCP和CT对明确本病的分型、指导手术有帮助。目前推荐对Ⅰ、Ⅳ型先天性胆管囊肿采用囊肿全切除,肝管空肠Roux-en-Y吻合术,对反复感染的复杂的中央型V型caroli病可考虑行肝移植术。     

Laparoscopic resection of type 1 choledochal cysts in pediatric patients

Background Choledochal cyst resection and hepaticojejunostomy have historically been performed using an open technique. We describe here the largest single experience with this procedure using laparoscopic techniques in eight consecutive pediatric patients. Methods There were six girls and two boys, of ages ranging from 3 months to 13 years. All had type I choledochal cysts. Three were asymptomatic, having been noted on prenatal ultrasonography. Five ports were utilized: one 5-mm telescope port at the umbilicus, two 3-mm operating ports on both sides of the umbilicus, one 5-mm left subcostal port for liver retraction, and one LLQ 5-mm assistant port. Results The median operating time was 155 min (range 110–250 min), with one conversion to an open procedure due to a high transection of the cyst leading to partial retraction of the left hepatic duct into the liver substance. Mean hospital stay was 3 days. At a mean follow-up of 18.8 months, all patients were anicteric and asymptomatic. Conclusions Laparoscopic resection of choledochal cysts can be performed safely in pediatric patients with minimal morbidity and good long-term results.