Congenital bile duct cyst (BDC) is a more indolent disease in children compared to adults, except for Todani type IV-A BDC: results of the European multicenter study of the French Surgical Association.

AIM: To compare clinical presentation, operative management and short- and long-term outcomes of congenital bile duct cysts (BDC) in adults with children. METHODS: Retrospective multi-institutional Association Francaise de Chirurgie study of Todani types I+IVB and IVA BDC. RESULTS: During the 37-year period to 2011, 33 centers included 314 patients (98 children; 216 adults). The adult population included more high-risk patients, with more active, more frequent prior treatment (47.7% vs 11.2%; p < 0.0001), more complicated presentation (50.5% vs 35.7%; p = 0.015), more synchronous biliary cancer (11.6% vs 0%; p = 0.0118) and more major surgery (23.6% vs 2%; p < 0.0001), but this latter feature was only true for type I+IVB BDC. Compared to children, the postoperative morbidity (48.1% vs 20.4%; p < 0.0001), the need for repeat procedures and the status at follow-up were worse in adults (27% vs 8.8%; p = 0.0009). However, severe postoperative morbidity and fair or poor status at follow-up were not statistically different for type IVA BDC, irrespective of patients' age. Synchronous cancer, prior HBP surgery and Todani type IVA BDC were independent predictive factors of poor or fair long-term outcome. CONCLUSION: BDC is a more indolent disease in children compared to adults, except for Todani type IV-A BDC.

Presentation and Clinical Outcomes of Choledochal Cysts in Children and Adults: A Multi-institutional Analysis.

IMPORTANCE: Choledochal cysts (CCs) are rare, with risk of infection and cancer. OBJECTIVE: To characterize the natural history, management, and long-term implications of CC disease. DESIGN, SETTING, AND PARTICIPANTS: A total of 394 patients who underwent resection of a CC between January 1, 1972, and April 11, 2014, were identified from an international multi-institutional database. Patients were followed up through September 27, 2014. Clinicopathologic characteristics, operative details, and outcome data were analyzed from May 1, 2014, to October 14, 2014. INTERVENTION: Resection of CC. MAIN OUTCOMES AND MEASURES: Management, morbidity, and overall survival. RESULTS: Among 394 patients, there were 135 children (34.3%) and 318 women (80.7%). Adults were more likely to present with abdominal pain (71.8% vs 40.7%; P < .001) and children were more likely to have jaundice (31.9% vs 11.6%; P < .001). Preoperative interventions were more commonly performed in adults (64.5% vs 31.1%; P < .001), including endoscopic retrograde pancreatography (55.6% vs 27.4%; P < .001), percutaneous transhepatic cholangiography (17.4% vs 5.9%; P < .001), and endobiliary stenting (18.1% vs 4.4%; P < .001)). Type I CCs were more often seen in children vs adults (79.7% vs 64.9%; P = .003); type IV CCs predominated in the adult population (23.9% vs 12.0%; P = .006). Extrahepatic bile duct resection with hepaticoenterostomy was the most frequently performed procedure in both age groups (80.3%). Perioperative morbidity was higher in adults (35.1% vs 16.3%; P < .001). On pathologic examination, 10 patients (2.5%) had cholangiocarcinoma. After a median follow-up of 28 months, 5-year overall survival was 95.5%. On follow-up, 13 patients (3.3%), presented with biliary cancer. CONCLUSIONS AND RELEVANCE: Presentation of CC varied between children and adults, and resection was associated with a degree of morbidity. Although concomitant cancer was uncommon, it occurred in 3.0% of the patients. Long-term surveillance is indicated given the possibility of future development of biliary cancer after CC resection.

Clinical analysis of 221 cases of adult choledochal cysts.

Choledochal cyst, usually recognized as a problem in infants, is increasingly reported in adult patients. The aim of this study was to share our experience in treating adult patients with choledochal cysts. Two hundred twenty-one adult patients with choledochal cysts who were treated in our hospital from January 2002 to December 2010 were enrolled. The clinical data were retrospectively collected and analyzed. There were 168 Type I, three Type II, three Type III, 26 Type IV, and 21 Type V cysts. The presentations were nonspecific with 177 patients having abdominal pain and a few patients having other rare symptoms including back pain, cirrhosis, occupying lesions, or spontaneous rupture. The cysts were completely removed in 169 patients, and 36 patients required liver resection. There was one death resulting from abdominal bleeding. Morbidity was limited to 27 patients, 11 of whom underwent invasive interventions. Choledochal cysts remain an interesting clinical problem. In some adult cases, the diagnosis is very difficult. Treatment is type-dependent, complete removal of the cysts is widely accepted, and lifelong follow-up for these patients is recommended.

Choledochal cyst and associated malignant tumors in adults: a multicenter survey in South Korea.

OBJECTIVE: To determine the clinical features and clinical outcomes of Korean adults treated surgically for choledochal cyst. DESIGN: Retrospective nationwide multicenter study. SETTING: Fifteen university hospitals (tertiary care referral centers) located in all 7 Korean provinces. PATIENTS: A total of 808 patients aged 18 years or older who underwent surgery for choledochal cyst from January 1, 1990, through December 31, 2007. MAIN OUTCOME MEASURES: Demographic information, surgical data, associated biliary malignant tumors, and factors predicting malignant tumors. RESULTS: Type I was most common (499 [68.2%]) followed by type IVa (208 [28.4%]). Of 654 patients, anomalous pancreaticobiliary ductal union was identified in 467 patients (71.4%), 291 with the choledochal type (62.3%), 96 with the pancreatic type (20.6%), and 80 with the complex type (17.1%). Biliary tract malignant tumor was associated in 80 patients (9.9%); 40 had bile duct cancer (50.0%), 35 had gallbladder cancer (43.8%), 3 had periampullary cancer, and 2 had synchronous gallbladder and bile duct cancer. Twenty-two patients (26.3%) had a recurrence, with a median follow-up duration of 51.8 months. Factors predicting malignant tumor by univariate analysis were age more than 40 years, the absence of a gallstone, elevated carcinoembryonic antigen or cancer antigen 19-9 serum level, and the presence of anomalous pancreaticobiliary ductal union, and by multivariate analysis, an elevated cancer antigen 19-9 level. CONCLUSIONS: Associated biliary malignant tumor should always be considered in patients with choledochal cyst, especially in aged patients or patients with anomalous pancreaticobiliary ductal union or an elevated tumor marker level. Lifelong follow-up is needed even after complete cyst excision because of the risk of the development of a metachronous biliary malignant tumor.

Forty-year experience with flow-diversion surgery for patients with congenital choledochal cysts with pancreaticobiliary maljunction at a single institution.

BACKGROUND: Congenital choledochal cyst with pancreaticobiliary maljunction (PBM) is known as a high-risk factor for various complications such as cholangitis, pancreatitis, and carcinogenesis of the biliary system by mutual refluxes of bile and pancreatic juice. Furthermore, it is not rare to suffer from postoperative complications if the wrong operative procedure is chosen. Therefore, we sought to review the relationship between operative procedure for types I and IV-A (Todani's classification) congenital choledochal cyst with PBM, and long-term treatment outcome. SUBJECTS AND METHODS: A retrospective review was carried out of 144 patients who underwent flow diversion surgery in our institution during the 40-year period from 1968 to 2008 and who did not have a coexisting malignant tumor at the time of surgery. RESULTS: Of these 144 patients, 137 underwent complete cyst excision and 7 underwent pancreas head resection as flow diversion surgery. The follow-up periods ranged from 1 to 345 months and from 1 to 271 months (average, 100.2 and 94.1) in patients with type I and type IV-A cysts, respectively. Regarding surgical treatment outcome, postoperative progress was good in 130 (90.3%) of the 144 patients. Fourteen patients required hospitalization for long-term postoperative complications such as cholangitis, pancreatitis, intrahepatic calculi, pancreatic calculus, and carcinogenesis during postoperative follow-up. Of these, 2 patients who underwent surgery for type IV-A cysts died because of secondary biliary cirrhosis with liver failure and advanced intrahepatic cholangiocarcinoma, respectively. CONCLUSIONS: The present study shows that flow diversion surgery for congenital choledochal cysts with PBM significantly reduces the risk of subsequent development of malignancy in the biliary tract, and it is vital to choose the appropriate operative procedure to prevent occurrence of these postoperative complications.

Management of type I choledochal cyst in adult: totally laparoscopic resection and Roux-en-Y hepaticoenterostomy.

BACKGROUND/OBJECTIVE: Choledochal cysts are congenital dilations of the biliary tree. The accepted mode of treatment is total excision with hepaticojejunostomy. In this retrospective study, we present our technique and results of laparoscopic choledochal cyst excisions. METHODS: We retrospectively studied 45 patients who had undergone laparoscopic choledochal cyst excision in our institutes from September 2006 to August 2009. Data including age, gender, type of cyst, symptoms, surgical technique, conversion rate, morbidity, and mortality were analyzed. RESULTS: There were type Ic (cystic) choledochal cysts in 31 patients (68.9%) and type If (fusiform) in 14 patients (31.1%). An anomalous pancreaticobiliary duct junction union was found in 66.7%. Forty percent (18 out of 45) and 37.8% (17 out of 45) cases had stones within the cysts and gallbladders, respectively. The average size of the cysts was 40.3 +/- 16.9 cm(2). The mean operative time was 307.7 +/- 58.0 min, the estimated operative blood loss was 252.3 +/- 162.5 ml, and the conversion rate was 8.9%. The mean hospital stay was 8.3 +/- 3.2 days. The overall morbidity rate was 17.1%, the reoperation rate was zero, and the mortality rate was also zero. CONCLUSIONS: Totally, laparoscopic management of type I choledochal cysts, although technically challenging, is safe and feasible in experienced hands.

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 cyst: a 10-year experience.

A retrospective study of 11 cases of choledochal cyst over a period of 10 years is presented. There were 8 females and 3 males in a ratio of 2.67:1. The classical triad of jaundice, mass and abdominal pain was seen in none of our patients. Ultrasonography was diagnostic in 9 out of 11 (80%) patients. Surgical procedures performed were, complete excision of the cyst with Roux en Y hepaticojejunostomy (nine cases), internal drainage of the cyst (one case) and excision of the extrahepatic cyst with Roux en Y hepaticojejunostomy in one case of Type IV choledochal cyst. One patient died in the immediate postoperative period. Others have remained well upto 10 years follow-up. We recommend total cyst excision with hepaticojejunostomy as the treatment of choice.

Malignancy in choledochal cysts.

BACKGROUND/AIMS: Malignancy in choledochal cysts is a rare condition. This study presents our experience with this condition, with emphasis on the clinical presentation, management and outcome. METHODOLOGY: Subjects included 80 adults with choledochal cysts treated from January 1979 to December 1995. Of these patients, 8 were found to have malignancy in the cyst and formed the basis of this study. RESULTS: Four patients had synchronous and 4 had metachronous carcinoma lesions arising in the choledochal cyst. The clinical presentation was: biliary tract infection in 5 patients, gastric outlet obstruction in 2 and right upper quadrant pain and body weight loss in 1. Operations for bile duct malignancy included total excision in 2 patients, choledochotomy with T-tube drainage in 2 patients, gastrojejunostomy in 2 patients, percutaneous transhepatic biliary drainage and gastrojejunostomy in 1 patient and metastatic lymph node biopsy only in 1. One patient died due to septic shock within 30 days of the operation (operative mortality). Postoperative survival time ranged from 4-13 months with a mean of 6.2 months. CONCLUSIONS: The frequency of malignancy in the choledochal cysts was 10% in the present series. Malignancy in the choledochal cyst should be highly suspected in patients with cholangitis symptoms, body weight loss and anemia. Prognosis in this disease entity is poor.

The spectrum of biliary tract disorders in infants and children. Experience with 300 cases.

OBJECTIVES: To assess the results of surgical treatment of infants and children with biliary tract disease. DESIGN: The records of children with biliary tract disorders requiring surgical intervention were reviewed retrospectively. Diagnosis, age, sex, clinical presentation, treatment, and outcome were evaluated. SETTING: A large pediatric referral facility. PATIENTS: A total of 300 patients treated from 1972 through 1993 were evaluated, including 102 with biliary atresia, 29 with choledochal cyst, and 169 with cholelithiasis. Hepatic portoenterostomy was performed in 87 patients with biliary atresia, and biopsy alone was performed in 15. Twenty girls and nine boys, 50% of whom were younger than 3 years, had choledochal cyst. Operative management included cyst excision and hepatojejunostomy in 25 patients, cyst jejunostomy in two patients, cystduodenostomy in one patient, and choledochocele excision in one patient. Gallstones were observed in 106 girls and 63 boys; 28 were aged 0 to 5 years, 31 were aged 6 to 10 years, and 110 were aged 11 to 18 years. Open cholecystectomy was performed in 143 patients, and laparoscopic cholecystectomy was performed in 26 patients. MAIN OUTCOME MEASURES: Principal outcomes examined were surgical morbidity and mortality. RESULTS: Hepatic portoenterostomy was successful in 28 (32%) of 87 patients with biliary atresia (all younger than 3 months), and 23 showed improvement following hepatic portoenterostomy; hepatic portoenterostomy failed in 36 patients. Twenty-eight (82.3%) of 34 patients survived liver transplantation. Overall survival was 71.5%. Survival of patients with choledochal cyst was 96.4% (28/29). Cholecystectomy was successful in all 169 patients. There were eight complications and one death (0.59% mortality). CONCLUSIONS: Survival in patients with biliary atresia (71%) has improved with hepatic portoenterostomy complemented by liver transplantation. Hepatic portoenterostomy is the procedure of choice in infants younger than 3 months. An improved outlook for patients with choledochal cyst can be expected after cyst excision and hepatojejunostomy. Gallstones are relatively common in children. Both open and laparoscopic cholecystectomy are safe and effective procedures in children.

Surgical treatment of congenital choledochal cyst.

20 cases of congenital choledochal cyst were operated by modified Lilly's method from 1980 to November 1985 in our Hospital. There were 4 males and 16 females. Their ages ranged from 50 days to 15 years with a mean of 5.1 years. All patients had preoperative ultrasonographic examination, barium meal radiography of the gastrointestinal tract or percutaneous transhepatic cholangiography. All had choledochal cystectomy with retention of the outer layer of the posterior wall of the cyst. We modified Lilly's method by injecting normal saline between the outer and inner layer of the choledochal cyst so that the outer layer could be isolated. Reconstruction of the biliary tract was then performed. 2 patients had choledochoduodenostomy and 18 patients had hepaticojejunostomy (1 end to end and 17 end to side). 19 patients had also had cholecystectomy. The post-operative course of the operation was found to be smooth and safe. Patients were followed up for a period from 6 months to 7 years. 3 patients had cirrhosis of liver, 2 of whom died within 8 months. The mortality rate in our series was 10%. The operative treatment and the problems of biliary reconstruction are discussed.