Although laparoscopic surgery for hepatobiliary disease in children is possible, it is technically challenging. In an attempt to overcome these difficulties, the da Vinci Robotic Surgical System(R) was used to facilitate the minimally invasive treatment of choledochal cyst in six children. In early consecutive three cases, we experienced three complications; a case of laparotomy conversion, a case of late stenosis of the hepaticojejunostomy, and a case of leakage from a hepaticojejunostomy. However, in the last three cases the complete resection of the choledochal cyst and Roux-en-Y hepaticojejunostomy were performed using the robotic surgical system without complication.

We think robot-assisted choledochal cyst resection in children appears safe and feasible, and may increase the variety of complex procedures in pediatric surgical fields.

Choledochal cyst is a congenital dilatation of the bile duct. Intrahepatic bile duct dilatation of type IVa by Todani's classification at the time of diagnosis resolved spontaneously after cyst excision and hepaticojejunostomy in many cases. It should be distinguished from the true cystic dilatation of the intrahepatic ducts, which tends to persist, albeit after some regression. We therefore studied postoperative intrahepatic duct dilatation changes in choledochal cyst. A total of seventy-six choledochal cysts were managed at the Division of Pediatric Surgery, Department of Surgery, Samsung Medical Center from May 1995 to December 2005. The ratio of males to females was 1:2.8. Preoperative radiologic diagnosis by Todani's classification was Type I (n=52, 68.4 %), II (n=1, 1.3 %), IVa (n=23, 30.3 %). Among fifty-five patients with intrahepatic bile duct dilatation we were able to follow up forty-eight by ultrasonography. Twenty-two patients were type IVa, and twenty-six patients were type I and showed intrahepatic duct dilatation. Mean follow-up duration was 35.3 months (9~105 months). Complete regression of dilated intrahepatic duct was observed in fifteen patients of type IVa and twenty-four patients of type I. Incomplete regression of dilated intrahepatic duct was observed in six patients in type IVa and two patients in type I. Only one patient in type IVa showed no change in ductal dilatation during a follow-up period of 15 months. We conclude that true type IVa is much less frequent than what was diagnosed preoperatively by imaging study. Therefore in type IVa patients who are diagnosed preoperatively the decision to perform liver resection should be carefully considered. Postoperative long term follow up of choledochal cyst with intrahepatic bile duct dilation is needed.

Forme fruste choledochal cyst (FFCC) is one of the diverse types of choledochal cyst with little or no dilatation of the extrahepatic bile duct (EHBD). It is considered that FFCC has to do with the pancreatobiliary malunion (PBM). In children, 3 to 6 millimeters of EHBD is assumed to be normal. Even though there is no clear-cut definition, FFCC is likely to be associated with bile duct dilatation less than 10 millimeters. Almost all cases have PBMs and symptoms of the pancreatitis or cholangitis. We experienced a case of FFCC in a 4-year-old boy. His EHBD measures 10mm diameter. He had symptoms of pancreatitis and elevated hepatic transaminases. The pancreatobiliary common channel was 28 millimeters. He underwent EHBD resection and Roux-en-Y hepaticojejunostomy and was discharged with no specific complications.

A 10-year-old-girl who underwent Roux-en-Y cystojejunostomy under the diagnosis of choledochal cyst at another hospital at the age of 3 months was referred to our hospital due to abdominal pain. Abdominal ultrasonography (USG) and computed tomography (CT) showed the type I choledochal cyst and multiple gall bladder stones. Severe inflammation and adhesion made difficulty of radical resection and only partial resection of choledochal cyst with Roux-en-Y hepaticojejunostomy could be performed. She complained of intermittent abdominal pain, fever, nausea and vomiting 2 1/2 years after the second operation. Follow-up abdominal CT scan showed the polypoid nodular lesion in the remnant choledochal cyst and suspicious metastatic lesion in the segment 7 of the liver. The duodenum was obstructed by the mass arising from the remnant choledochal cyst. The USG-guided liver biopsy revealed the moderately differentiated adenocarcinoma. A secondary palliative gastrojejunostomy was performed to relieve the obstruction of duodenum. She died of hepatic insufficiency 4 months later of third operation.

Choledochal cysts have been associated with complications such as cholangitis, pancreatitis, and malignancy of the biliary tract. Recently, the incidence of choledochal cyst in neonate and young infant is increasing due to advances in diagnostic imaging. The aim of this study is to investigate the rationale of excision of choledochal cyst during the neonatal period. The clinical outcome and correlation between age at surgery and the degree of liver fibrosis were reviewed retrospectively. A total of 198 patients with choledochal cyst who were managed surgically between January 1985 and December 2000 at the Department of Surgery, Seoul National University Children's Hospital were included in this study. The overall outcome and the outcome of patients who were managed surgically during the neonatal period were compared. Correlation between age and the degree of liver fibrosis was evaluated by chi-square test and Pearson exact test. The mean age of the patients was 2 years 7 months (ranged from 5 days to 15 years). Mean postoperative follow-up period was 7 years 1 month (7 months to 20 years). The results are as follows. Twelve patients (6%) had postoperative complications, cholangitis (7), bleeding (4) and ileus (1). Eleven patients operated during the neonatal period had no postoperative complications. The positive correlation between age group and degree of liver fibrosis was statistically significant (chi-square: p=0.0165, Pearson exact test: p=0.019). The results support the rationale that excision of choledochal cyst can be performed safely without increasing morbidity in neonates.

Improvement in prenatal ultrasonography is leading to diagnose choledochal cyst before birth and before onset of classical symptom more frequently. But, there is a controversy about optimal timing for cyst excision of prenatally diagnosed asymptomatic choledochal cyst. To identify the most appropriate timing for surgery in prenatally diagnosed choledochal cysts, we analyzed 6 patients who had operation for choledochal cysts within 30days after birth at the division of Pediatric Surgery, Samsung Medical Center and Inha University School of Medicine, from June 1995 to June 2002. Males were four and females 2, the mean age at operation was 11.2 days, and the median age 8.0 days. The range of gestational ages of the antenatal diagnosis of bile duct dilatation was 24 weeks to 32 weeks, mean was 38.3 weeks, and mean birth weight was 3,298.3 g. After birth, abdominal ultrasonography, hepatobiliary scintigraphy, and magnetic resonance cholangiopancratography (MRCP) were performed. Mean age at operation was 11.2 days. All patients had the cyst excision and Roux- en-Y hepaticojejunostomy. Immediate postoperative complication was not found. During the median follow-up period of 41 months, one patient was admitted due to cholangitis, and the other due to variceal bleeding. Early operative treatment of asymptomatic newborn is safe and effective to prevent developing complications later in life.

Congenital dilatation of the common bile duct (choledochal cyst) is an uncommon disease. Although the etiology is unknown yet, various theories such as distal obstruction of the common bile duct, congenital weakness of the duct and anomalous union of the pancreaticobiliary duct have been offered to explain the occurrence of choledochal cyst. Thirty - six cases of choledochal cyst over 22 years were analyzed clinically and classified according to Todani's classification and Kimura's anomalous union of pancreaticobiliary duct type. Todani type I consisted with 22 cases which were subdivided into 19 cases of type Ia, 1 case of type Ib and 2 of type Ic. Type IVa consisted with 14 cases including one case of Caroli's disease. There were 25 type BP cases and 10 type PB cases and 1 normal pancreatobiliary junction. Serum alkaline phosphatase was increased significantly in almost all cases. Seven patients (19.4%) had associated congenital anomalies such as double gallbladder, left - sided gallbladder, common bile duct web, biliary atresia, accessory hepatic duct, heterotopic pancreas, cleft lip and 2 cases of intestinal malrotation. All patients underwent cyst excision and Roux - en - Y hepaticojejunostomy and cholecystectomy. There was one death due to methicillin resistant Staphylococcus aureus sepsis.

A nationwide survey on Choledochal cyst was undertaken among 39 members of the Korean Association of Pediatric Surgeons. The members were required to complete a questionnaire and the case registration form for each patient during the five-year period of 1997 to 2001. Three hundred and forty eight patients were registered from 32 institutions. The average number of patients per surgeon was one to two every year. The male to female ratio was 1: 3.4. The age of patients on diagnosis was 49.0±44.4 months. The geographic distribution was 34.8% in Seoul and Kyoungki-do, 33.3% in Kyoungsang-do, 17.9% in Cholla-do, and 8.5% in Choongchung-do, in order of frequency. The three common clinical presentations were abdominal pain (63.8%), vomiting (35.3%), and jaundice (29.1%). Only seven patients (2%) presented with classic triad, and 25 patients were diagnosed by antenatal ultrasonographic examination. According to the Todani classification, 238 patients (71.3%) were type I, 3 (0.9%) type II, and 93 (27.8%) type IV. At the time of the operation, three important associated conditions were choledocholithiasis in 45 patients (15.1%), liver fibrosis (Grade 1-4) in 35, and previous operative procedure for biliary diseases in 10. Associated anomalies were observed in 13 patients (3.8%). Three hundred thirty nine (98.8%) of 343 lesions were treated by cyst excision and Roux-Y hepaticojejunostomy. One hundred seventy-six patients had an anomalous arrangement of the pancreatobiliary ductal system: APBD was not in 92 patients, biliary duct joined to the pancreatic duct in 51, and pancreatic duct joined to the biliary duct in 26. There were 8.5% early, and 7.7% late phase operative complications. The major complications were bleeding, anastomotic leakage, and acute pancreatitis. The combination of acute abdomen and choledochal cyst may suggest spontaneous rupture. Because of the development of late intrahepatic bile duct stones, long-term follow-up after cyst excision and hepaticojejunostomy is required. The optimal time of surgical intervention should also be considered in the situation of routine use of antenatal ultrasonographic examination. This is the first review of the choledochal cyst in Korea and provides baseline data for future comparisons.

To study the role of anomalous pancreatico-biliary ductal union (APBDU) in the development of choledochal cyst, we reviewed 23 cases. APBDU is defined as a long common channel(>0.4 mm). The patients ages ranged from 1 week to 112 months and the mean age was 22.5 months. Right upper quadrant pain was the most prevalent symptom. The diagnosis was made by ultrasonography and operative cholangiography in most patient. The preoperative diagnosis was made in 100 % of the cases. Gallstones were found in 5 cases. Todani type I and type IV were prevalent. A long common channel was found in all cases. The operative treatment consisted of cyst excision and Reux-en-Y hepaticojejunostomy or choledochojejunosotmy. One patient had postoperative pancreatitis. There was no mortality. We conclude that detection of choledochal cyst is occurring at a younger age and APBDU seems to play an important role in the pathogenesis of type I and IV cysts. Cyst excision is the treatment of choice to eliminate repeated cholangitis and malignant transformation.

Choledochal cyst is rare in the western countries, but common in oriental countries. Complicatioins include ascending cholangitis, recurrent pancreatities, progressive biliary cirrhosis, portal hypertension, stone formation and later malignant transformation. Bile peritonitis secondary to rupture is one of the rarest complications, with an incidence of 1.8 % to 18 %. The anomalous arrangement of the pancreatobiliary ductal system with a long common channel may cause inflammation leading to perforation of the cyst. The authors found 4 cases (14.2 %) of bile peritonitis among 28 cases of choledochal cyst treated from Jan. 1983 to Jan. 1998. The patients ages ranged from 6 months to 3 years and three were female. The perforation sites were located on the common bile duct at its junction with the cystic duct in 2 cases, the distal cyst wall in 1 case and the left hepatic duct at its junction with cyst in 1 case. The types of choledochal cysts by Todani's classification were Type IVa in 3 cases and type I in 1 case. By the new Komi's classification utilizing operative cholangiogram there were 2 cases of Type Ia, 1 case of type IIb and 1 case of type III. One stage cyst excision and hepaticojejunostomy(Roux-en Y type) was done in 3 cases, and two staged operation in 1 case. All patients had an uneventful course postoperatively. The average day of discharge was 9.8th postoperatively. In conclusion, primary excision of the choledochal cyst and biliary reconstruction is a safe and effective treatment of ruptured choledochal cyst in infants.

The anomalous pancreaticobiliary duct union (APBDU) might cause the formation of choledochal cyst and malignancies of hepatopancreaticobiliary system. The purpose of this study is to make an experimental animal model of APBDU similar to that of human. One to two-month-old Mongrel dogs (n=12) were divided into two groups; the control group (n=2) had a sham operation performed, and in the experimental group (n= 10) the end of distal' common bile duct (CBD) was anastomosed to the side of the dorsal pancreatic duct making APBDD. Serum was obtained for chemical analysis on the 10th postoperative day. The dogs were sacrificed at the 5th week (n=3), the 6th week (n=3), the 7th week (n=2), the 8th week (n=2) and the 6th month (n=2) after the experimental surgery. With sacrifice, operative cholangiogram was taken, and bile juice was obtained for chemistry and bacterial culture. The en-bloc specimens of the hepatopancreaticobiliary system were removed for microscopic examination. Serum and bile juice amylase levels were elevated in the experimental group (n=10), but not in the control group (n=2). Operative cholangiograms of control group revealed no evidence of bile duct dilatation.. On the other hand, the bile duct in the experimental group was markedly dilated without any evidence of stenosis at the anastomosis site (n=10). Histologic examination of the hepatopancreaticobiliary system in the experimental group resembled the findings of choledochal cyst in human. The APBDU of this animal model can produce bile duct dilatation by pancreaticobiliary reflux. We think that this animal model can be potentially promising for the research about the APBDU associated hepatopancreaticobiliary diseases.