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Background: The option of either single- or two-staged cyst excision has been proposed for perforated choledochal cysts (CCs), but which of the two methods is more effective remains controversial. We examined the complications and short-term outcomes of single-stage excision of perforated and non-perforated CCs., Methods: The medical records of patients treated for CCs from 2003 to 2016 were retrospectively reviewed. Outcomes were compared between patients with perforated CCs (Group A) and non-perforated CCs (Group B). The operative time, intraoperative bleeding, length of stay, and postoperative complications were analyzed., Results: Group A comprised 6 patients (2 males, 4 females; mean age, 29months), and Group B comprised 26 patients (2 males, 24 females; mean age, 41months). All patients underwent single-stage complete excision with Roux-en-Y hepaticojejunostomy. There were no significant differences in the operative time, bleeding, and/or length of stay. There were no operative deaths or complications such as anastomosis leakage or postoperative cholangitis, but a pancreatic fistula developed in one patient in Group A and two in Group B., Conclusion: Single-stage excision for a perforated CC is feasible if the patient's condition is stable., Levels of Evidence: Treatment Study, LEVELIII., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: There is a predisposition to the development of malignancy in congenital choledochal malformation (CCM) although the degree of risk is unknown. We investigated the role of CA19-9 in bile and the MIB-1 (Ki-67) epithelial proliferation index as markers of an at risk choledochal epithelium at the time of definitive surgery., Methods: Bile collected at surgery was analyzed for levels of amylase (as a surrogate of pancreatic reflux) and CA19-9. Immunohistochemical staining for CA19-9 and MIB-1 index (expressed as %) was performed on resected specimens. Data are quoted as median (IQR) and differences assessed using non-parametric statistics. A P value of 0.05 was regarded as significant., Results: Our study group consisted of 78 children with CCM (Type 1 fusiform, n=34; Type 1 cystic, n=30 and Type 4, n=14). Median bile CA19-9 was 159,400 (6-1×10(6)) kU/L. There was no correlation with bile amylase (P=0.49) or biliary pressure (P=0.17) but modest correlation with bilirubin (rs=0.24; P=0.02). In contrast, bile amylase was correlated with plasma γ-glutamyl transpeptidase (P=0.02), alkaline phosphatase (P=0.05) and aspartate aminotransferase (P=0.02); and inversely correlated with biliary pressure (rs=-0.38; P<0.0008). Epithelial expression of CA19-9 and MIB-1 was assessed in 43 specimens. CA19-9 was diffusely expressed on all choledochal epithelium. MIB-1 expression was divided into: high expression (>40%) n=3; moderate (20-40%) n=5, low (6-20%) n=7 and very low (≤5%) n=28. There was no correlation with choledochal pressure (P=0.87), CA19-9 (P=0.51) or bile amylase (P=0.55)., Conclusion: Biliary CA19-9 levels were grossly (and unexpectedly) raised in choledochal malformation and appear to arise from biliary rather than pancreatic epithelium. MIB-1 confirms that a small proportion (19%) has marked epithelial proliferation but no clinical correlates could be identified., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background/purpose: The aim of this study is to clarify the clinical features and risk factors of pre- and postoperative pancreatic complications in pediatric choledochal cysts., Methods: A retrospective chart review was carried out on pediatric patients with choledochal cysts who underwent radical operation at our department., Results: Twenty-one, 24, and 24 patients were classified into the Todani Ia, Ic, and IV-A choledochal cyst, respectively. Preoperative acute pancreatitis and protein plugs were observed in 31 (43.7%) and 11 (15.5%) patients, respectively. Patients with preoperative pancreatitis were more likely to have fusiform dilatation of choledochal cysts (79.3% vs. 35.0%) and a dilated common channel (53.9% vs. 23.1%) compared to those without preoperative pancreatitis. Compared to patients without preoperative protein plugs, those with protein plugs were more likely to have fusiform dilatation (90.9% vs. 46.5%) and pancreatic divisum with communicating ducts and a dilated ductal system (60.0% vs. 2.5%). Postoperatively, three patients (4.2%) experienced acute pancreatitis. One of these and all 3 had protein plugs and preoperative pancreatitis, respectively., Conclusions: Fusiform-type choledochal cyst is a significant risk factor for preoperative pancreatic complications in choledochal cysts. While postoperative pancreatic complications were relatively rare, preoperative pancreatic complications might be risk factors for postoperative pancreatitis., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Spontaneous perforation of a choledochal cyst with ensuing pseudocyst formation is a very rare complication. We report the development of a pseudocyst adjacent to a choledochal cyst in a very low-birth-weight infant at 2 months of age. Elective excision of the choledochal cyst and biliary tract reconstruction were successfully performed 2 months later when the infant weighed 3 kg. Delayed primary repair may be a viable alternative treatment for low-birth-weight infants with choledochal cysts., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background/purpose: This study aims to establish the possible mechanisms of pathogenesis of congenital biliary dilatation and to classify the disease accordingly., Methods: Radiologic features of congenital biliary dilatation and pancreaticobiliary malunion in 107 affected children were examined and correlated with laboratory results. Relative lengths/diameters were calculated to provide comparison between children of different ages. Intraluminal pressures of common bile duct (CBD) were measured intraoperatively., Results: The minimal relative diameters of distal CBD negatively correlated with the maximal relative diameters/lengths of dilated CBD, the maximal relative diameters of common hepatic duct, and left/right hepatic ducts. The intraluminal pressure in patients with a stenotic distal CBD (stenotic group) was significantly higher than that in patients with a nonstenotic distal CBD (nonstenotic group). The narrower the distal CBD, the more deranged the liver function. Conversely, serum/bile amylase levels were more elevated in the nonstenotic group. Common channel protein plugs were only found in the nonstenotic group, whereas common hepatic duct strictures, intrahepatic duct dilatations, and calculi were detected more frequently in the stenotic group., Conclusion: We propose to categorize congenital biliary dilatation into 2 subgroups: (1) cystic type with stenotic distal CBD associated with deranged liver function and common hepatic duct stricture and (2) fusiform type with nonstenotic distal CBD associated with pancreatitis and common channel protein plugs. Different underlying pathologies of each group require different operative strategies., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background/purpose: The aim of this study was to investigate the diagnostic potential of computed tomography cholangiography (CTC) and magnetic resonance cholangiopancreatography (MRCP) in children with pancreaticobiliary maljunction (PBM)., Methods: Fifty-three children with PBM were consecutively treated between 1997 and 2009. Among them, the patients who underwent CTC and/or MRCP preoperatively were enrolled in this study. Computed tomography cholangiography was examined after infusion of meglumine iodoxamate with subsequent 3-dimensional rendering. The visualization of the biliary and pancreatic duct systems was evaluated and compared with that visualized with MRCP. The findings of direct cholangiography were used as the standard of reference., Results: Of the 53 cases with PBM, 17 cases were examined by CTC, 10 cases by MRCP, and 17 with both. The extrahepatic bile tract was visualized in 32 (94.1%) of 34 patients in CTC and in all 27 patients in MRCP. The intrahepatic bile duct was more frequently demonstrated by MRCP than by CTC (96.3% vs 70.6%, P = .02). Pancreaticobiliary maljunction was noted in 13 (38.2%) of 34 with CTC and in 12 (44.4%) of 27 with MRCP. The minimum age for visualization of PBM was at 10 months in CTC and at 1 year and 11 months in MRCP, respectively. The main pancreatic duct was more frequently visualized by MRCP than by CTC (81.5% vs 8.8%, P < .001)., Conclusions: Magnetic resonance cholangiopancreatography provides superior visualization of the intrahepatic duct and the pancreatic system when compared with CTC. However, it is still challenging to perform a good-quality examination in young infant. The great advantage of CTC is its ability to produce high-quality images without respiratory artifacts and that it allows accurate assessment of the presence of PBM equivalent to MRCP., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Ectopic ureters in children are associated with symptoms related to an abnormal localization or structure of the ureteric orifice including reflux or obstruction. Approximately 20% to 25% of ectopic ureters drain a nonduplicated renal system. Associated malformations are common in these cases. We treated 2 girls with bilateral single-system ectopic ureter associated with choledochal cysts. In both cases, ureteral reimplantation and resection of the choledochal cyst with hepaticojejunostomy were carried out. The coincidence of bilateral single-system ectopic ureter with choledochal cyst without any other severe malformation is not described in the literature., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Background: The purpose of the study was to assess the efficacy of laparoscopic-assisted removal of protein plugs from the common channel in choledochal cysts., Methods: Between 2001 and 2009, 34 patients with choledochal cysts (mean age, 4.98 years) with protein plugs in the common channel successfully underwent laparoscopic cyst excision and Roux-en-Y hepatojejunostomy. Under direct vision during laparoscopy, urethroscopes or catheters were inserted into the common channel to irrigate and remove the protein plugs. Complete clearance was confirmed by either direct urethroscopic visualization or cholangiogram that demonstrated (1) no filling defects in the common channel, (2) significant reduction of common channel diameter, (3) free passage of contrast agent into the duodenum, and (4) no pancreatic duct reflux. Operative time and blood loss, postoperative hospital stay, duration of drainage, postoperative complications, perioperative ultrasonographic findings, and laboratory results were reviewed., Results: Protein plugs were completely removed in all the patients. The mean operative time, postoperative hospital stay, and drainage duration were 3.51 hours, 7.08 days, and 3.41 days, respectively. The median follow-up period was 32 months. Neither mortality nor complications of pancreatic juice leak, pancreatitis, or stone formation were observed. Liver function parameters and serum amylase levels returned to normal postoperatively., Conclusions: Laparoscopic-assisted clearance of the protein plugs in the common channel in choledochal cyst is effective, with good medium-term results observed., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Background: The intrahepatic gallstone is known as one of the complications of choledochal cyst. Stenosis of the hepatic duct may contribute to the formation of the stone. This report describes concurrent congenital stenosis of the hepatic duct identified during the primary operation of choledochal cyst., Methods: Fourteen children were operated on for choledochal cyst. To identify the concurrent stenosis, cholangiography and inspection of the hepatic duct were performed during the operation. In those patients with identified membranous stenosis, the membrane was resected. In those with nonmembranous stenosis, the wide hilar hepaticojejunostomy was performed after hepatic duct plasty., Results: Eleven sites of hepatic duct stenosis were identified, 8 membranous stenoses and 3 relative stenoses without membrane. The membranous stenoses were classified into 4 subtypes as follows: a small central opening, a marginal opening, 2 openings, and a bridgelike structure. At the primary surgery on choledochal cyst, those stenoses were released, and the formation of the gallstone has not been observed in 4 to 11 years in all cases.

Purpose: Forme fruste choledochal cyst (FFCC) is a choledochal cyst that has minimal or no dilatation of the extrahepatic bile duct (EHBD) and is associated with pancreaticobiliary malunion (PBMU). The authors reviewed the long-term outcome of their patients with FFCC., Methods: Inpatient and outpatient records of 281 patients with choledochal cyst were reviewed. In this study, minimal dilatation of the EHBD was defined as its maximum diameter being less than 10 mm., Results: There were 17 cases of FFCC identified. In all 17 patients, PBMU was present. The mean age at EHBD excision was 2.9 years. Fourteen patients had hepatico-jejunostomy, and three had hepatico-duodenostomy. The histology of the excised EHBD showed mucosal ulceration/sloughing (in 35.3% patients), fibrosis (52.9%), and inflammatory cell infiltration (41.2%). Over a mean postoperative follow-up period of 9.8 years, there have been no episodes of cholangitis or anastomotic stricture formation., Conclusions: The treatment of choice for FFCC in children is EHBD excision and hepatico-jejunostomy. There is little surgical morbidity if performed carefully.

Background/purpose: Low level of Insulinlike growth factor-I (IGF-I) has been reported in children with chronic liver disease like biliary atresia (BA) awaiting liver transplantation. However, there has been no report on IGF-I in BA managed without liver transplantation., Methods: The authors measured IGF-I and growth hormone (GH) in 21 postoperative BA, and 17 choledochal cysts (CC) as a control with normal liver function. To avoid an influence of aging, IGF-I was analyzed after converting them into a newly defined index "IGF%." IGF% is proportional to the lower limit of the value of IGF-I in gender- and age-matched normal control previously reported in literature., Results: IGF% in BA was significantly lower than that in CC. IGF% tended to be lower in Kasai's type III (atresia at the porta hepatis) and higher in the jaundice-free group. IGF% in patients with esophageal varices was significantly lower. The correlation between choline esterase and IGF% was positive and that for TTT and IGF% was negative., Conclusions: Low level of IGF-I is a characteristic finding in BA, especially in patients without need of liver transplantation. And it may reflect the severity of pathologic changes (ie, hepatic fibrosis and reduced volume of normally functioning liver) in BA liver., (Copyright 2003, Elsevier Science (USA). All rights reserved.)

Background/purpose: This study investigates the relationship between the location of the papilla of Vater and the length of the common channel in patients with congenital biliary dilatation (CBD)., Methods: Cholangiograms from 121 CBD patients and 13 normal controls were the subjects for this study. A length index defined as the length of the common channel divided by the height of the second lumbar vertebra was used for standardization., Results: In the controls, the papilla of Vater was located in the middle of the descending or second part of the duodenum in all cases. In 39 (32.2%) of the 121 CBD patients papilla of Vater was located in the descending duodenum (group I), and in 82 (67.8%) it was distal to the descending duodenum (group II). The average length index of the common channel in group II was significantly longer than in group I (1.123 +/- 0.374 v 0.660 +/- 0.246; P <.001). Findings for the common bile duct were similar., Conclusions: There is a significantly higher incidence of ectopic distal location of the papilla of Vater in CBD patients than in controls. The more distal the location of the papilla of Vater, the longer the common bile duct and the common channel., (Copyright 2001 by W.B. Saunders Company.)

Purpose: The aim of this study was to investigate the possible role of Fas and Fas ligand system in biliary atresia., Methods: Immunohistochemical stains of Fas and Fas ligand (FasL) and in situ hybridization of Fas ligand messenger RNA (mRNA) were performed on paraffin-embedded liver specimens of 36 biliary atresia, 6 choledochal cysts, and 14 nontumorous parts of pediatric liver tumors. Apoptosis was detected by terminal deoxynucleotidyl transferase deoxy-UTP nick end labeling (TUNEL). The grade of liver fibrosis and results of bile drainage on the patients with biliary atresia were compared with the results of FasL expression., Results: Fas protein was positive on the hepatocytes and bile ductule epithelia of all the livers examined and also positive on some monocytes around the portal area in all the biliary atresia patients. FasL protein was positive on bile ductule epithelia in 10 biliary atresia patients and also positive on some monocytes in most of the biliary atresia patients. Positive signals of FasL mRNA were noted on hepatocytes in 4 biliary atresia, bile ductule epithelia in 19 biliary atresia patients, and some monocytes in most of the biliary atresia patients. Apoptotic nuclei were present among monocytes in all the biliary atresia livers but present among bile ductule epithelia only on the BA with positive FasL mRNA signals on ductule epithelium. The fibrosis grade was similar between biliary atresia with positive FasL mRNA signals and negative signals. The bile drainage was better in the biliary atresia without positive FasL mRNA signals., Conclusions: Fas ligand expression on bile ductule epithelia in biliary atresia may be induced to counterattack the infiltrating lymphocytes. Although the factors for post-Kasai bile drainage are multiple, the authors suggest Fas ligand expression on bile ductule epithelia may be a poor prognostic factor by playing a role in the continuous damage and obliteration of intrahepatic bile ducts after Kasai operation.

Purpose: The aim of this study was to identify the most appropriate timing for surgery in newborns with choledochal cysts., Methods: The clinical and histological data of 8 newborn cases of choledochal cysts (newborn group) were compared with 45 cases that were diagnosed later than the newborn period (late group)., Results: The mean and standard deviation of age at diagnosis was 0.4+/-0.4 months and 43.4+/-37.8 months in the newborn and late group, respectively. The age at operation was significantly earlier in the newborn group than in the late group (4.9+/-4.5 months v54.7+/-47.0 months). Although no significant difference in the time that elapsed between the operation and the onset of symptoms between the newborn and late groups (4.5+/-4.7 months v11.3+/-21.1 months), the serum bilirubin level (4.6+/-3.8 mg/dL v1.8+/-3.4 mg/dL) and the grade of liver fibrosis (2.0+/-0.8 v1.1+/-0.8) were significantly higher in the newborn group than in the late group., Conclusion: The newborn group should be considered as a special group of patients with different clinical course and pathology than those of the late group, which necessitate the early surgical intervention to prevent progression of liver fibrosis.

Degenerative changes of intrahepatic bile ducts with lymphocytic infiltration into portal areas have been recognized as the characteristic histopathologic features in the liver with biliary atresia (BA). Occasionally, lymphocytic infiltration into biliary ductal epithelium was noted and has been thought to be mostly the result of nonspecific chronic inflammation. However, this histological presentation looks quite similar to that of acute graft-versus-host disease (GVHD) after bone marrow transplantation (BMT) or acute cellular rejection (ACR) after orthotopic liver transplantation (OLTx). According to this histological similarity, we presumed that the degeneration of intrahepatic bile ducts (DIBD) might have developed with lymphocytic infiltration into biliary epithelial cells (LIBE) as some part of an immunologic reaction, and focused on the DIBD with LIBE in the patients with BA. Liver specimens obtained from 31 patients with BA at the time of primary Kasai operation and 3 patients at the time of reoperation were reviewed histologically, and investigated DIBD with LIBE. Nine patients with choledochal cyst (CBD) and 5 patients with neonatal hepatitis (NH) were added to this study as a control population. All patients with BA presented with DIBD with LIBE. On the other hand, only 2 patients with CBD presented with DIBD with LIBE, and no patients with NH presented with DIBD with LIBE. The DIBD with LIBE is not limited to patients with BA, but it seems to be much more serious in this group. These findings may suggest that some sort of immunologic factors contribute to the persistent DIBD in the patients with BA.(ABSTRACT TRUNCATED AT 250 WORDS)

Choledochocele is a rare abnormality of unknown etiology that consists of cystic or diverticular dilatations of the intramural part of the common bile duct. The authors present a case of choledochocele with a common channel in a 15-year-old boy who had a 5-year history of intermittent right upper abdominal pain. In a review of the literature, macroscopic variations were noted as well as different types of epithelial lining inside the choledochocele. Whereas in young children mostly duodenal mucosa is described, biliary tract epithelium and undifferentiated epithelium are predominant later on. Etiologically, congenital intraluminal duodenal diverticulum should be suggested in relation to the pathogenesis of choledochocele. Treatment is total excision and reinsertion of both ducts in cases of biliary or undifferentiated epithelium; endoscopic sphincterotomy or marsupialization is indicated only when duodenal mucosa covers both sides of the choledochocele.

Gallbladder anomalies are rare and normally affect its shape, size, and position. We report on a 3-year-old boy with a gallbladder divided transversally by a windsock-type diaphragm, isolating the fundus from the rest of the biliary tree. Bilobed and multiseptated gallbladder have been described before, but this is the first isolated case of a congenital hourglass gallbladder.