Safety and benefit of modified mesohepatectomy in the treatment of bilobar involvement congenital biliary dilatation.

BACKGROUND: The diseased bile duct in bilobar congenital biliary dilatation is extensive and often requires major hepatectomy or liver transplantation associated with a higher risk. We aimed to evaluate the safety and benefit of modified mesohepatectomy, in comparison with trisectionectomy, to treat bilobar congenital biliary dilatation. METHODS: This study included 28 patients with type IV and V bilobar congenital biliary dilatation. An innovative mesohepatectomy comprising the hepatectomy technique beyond the P/U point and bile duct shaping was applied to 14 patients to address the extensively diseased bile duct and difficulty in hepaticojejunostomy. Another 14 patients received trisectionectomy. The perioperative and long-term outcomes of these patients were compared. RESULTS: The ratio of residual liver volume to standard liver volume in the mesohepatectomy group was higher (78.68% vs. 40.90%, p = 0.005), while the resection rate of the liver parenchyma was lower (28.25% vs. 63.97%, p = 0.000), than that in trisectionectomy group. The mesohepatectomy group had a lower severe complication (>Clavein III, 0% vs. 57.70%, p = 0.019) and incidence of posthepatectomy liver failure (7.14% vs. 42.86%, p = 0.038). No significant difference was observed in blood loss and bile leakage (p > 0.05). All the patients in the mesohepatectomy group achieved optimal results in the long-term follow-up. CONCLUSIONS: mesohepatectomy provides an efficient treatment option for bilobar congenital biliary dilatation and can achieve radical resection, retain more liver parenchyma, and reduce the difficulty of hepaticojejunostomy, especially for patients that are not eligible for major hepatectomy and liver transplantation.

Early and late outcomes of congenital biliary dilatation in pediatric patients.

BACKGROUND: This study aimed to reveal the early and late postoperative complications and outcomes after surgery for congenital biliary dilatation (CBD) by reviewing cases over the past 40 years. METHODS: We retrospectively evaluated 59 patients with CBD who underwent radical surgery for complications and outcomes, based on medical records. Early complications were defined as those requiring treatment within 5 years of the initial operation. Late complications were defined as those treated more than 5 years later. RESULTS: The median age at the first surgery was 37 months. Regarding biliary reconstruction, 54 of the 59 patients (91.5%) underwent hepaticojejunostomy. Although three patients underwent cholecystoduodenostomy and one patient underwent hepaticoduodenostomy, all were converted to hepaticojejunostomy after a median of 12.5 years. One patient developed synchronous biliary carcinoma and underwent pancreaticoduodenectomy. Early complications occurred in seven patients with 10 events (surgical site infection, n = 3 bile leakage, n = 3; ileus, n = 3; bile duct obstruction, n = 1 and intussusception, n = 1). Late complications occurred in nine patients with 12 events (ileus, n = 3; anastomotic stricture, n = 3; hepatolithiasis, n = 3; asynchronous biliary carcinoma, n = 2; pancreatolithiasis, n = 1). Two of the three patients with hepatolithiasis underwent hepatectomy refractory to the endoscopic approach. Two patients developed asynchronous biliary carcinoma at 34 and 13 years after last operation; both ultimately died of the carcinoma. Only 35 patients (61.4%) underwent a follow-up examination. A total of 11 female patients (45.8%) eventually married, and all successfully gave birth. CONCLUSION: Although the long-term prognosis is excellent with complete cyst excision and hepaticojejunostomy, we emphasize the importance of long-term follow-up.

Roux-en-Y hepaticojejunostomy or hepaticoduodenostomy for biliary reconstruction after resection of congenital biliary dilatation: a systematic review and meta-analysis.

The ideal surgical management for reconstruction after excision of congenital biliary dilatation remains controversial. This updated meta-analysis compared the clinical outcomes of hepaticoduodenostomy (HD) and hepaticojejunostomy (HJ) after resection of congenital biliary dilatation. PubMed, Web of Science, Embase, Ovid, and the Cochrane Library were searched for studies published from November 1981 through July 2020. The primary outcomes were the operative time, enteral feeding time, hospital stay, and postoperative complications. The quality and risk of bias were assessed with the Newcastle-Ottawa Quality Assessment Scale. Odds ratios (ORs) and mean differences (MDs) with 95% confidence intervals (CIs) were pooled using random-effects models. Thirteen total studies included 518 (55.76%) HD cases and 411 (44.24%) HJ cases. Five studies were published post-2013; one was a randomized clinical trial. Patients undergoing HD had a shorter hospital stay (MD, 0.40; p = 0.02) and operative time (MD, 59.54; p < 0.00001) and a lower incidence of adhesive intestinal obstruction (OR, 0.20; p = 0.02) than HJ. HD was comparable to conventional HJ with regard to most postoperative outcomes; however, it was associated with a higher incidence of postoperative bilious gastritis (OR, 6.24; p = 0.002). HD is as safe and feasible as HJ with better outcomes in the short run, although reports with long-term follow-up are relatively few. Long-term follow-up will be necessary to monitor possible associated malignancies in the future.

Carcinogenic risk in the biliary epithelium of children with congenital biliary dilatation via the DNA damage repair pathway.

PURPOSES: Congenital biliary dilatation (CBD) is a high-risk factor for biliary tract cancer (BTC). We previously reported the potential for carcinogenesis in the biliary epithelium of patients with CBD. In this study, we investigated potential carcinogenetic pathways, focusing on the DNA damage repair response, in children with CBD and compared the findings with those in adults. METHODS: We enrolled 6 children with CBD and 10 adults with CBD without BTC who underwent extrahepatic bile duct resections, plus 4 control patients who underwent pancreaticoduodenectomy for non-biliary cancer. Levels of phosphorylated histone H2AX (γH2AX), MRE11, and Ku-70 in the biliary tract epithelium were evaluated by immunohistochemistry. RESULTS: The levels of γH2AX, MRE11, and Ku-70 were significantly higher in the gallbladder epithelium and bile duct epithelium of both children and adults than in controls. CONCLUSIONS: Children and adults with CBD might develop BTC via the DNA damage repair pathway, as evidenced by increased γH2AX, MRE11, and Ku-70 expression.

Factors predicting surgical difficulties in congenital biliary dilatation in pediatric patients.

BACKGROUND: The effects of disease classification and the patient's preoperative condition on the difficulty of performing a laparotomy for pediatric congenital biliary dilatation (CBD) have not been fully elucidated. METHODS: The present study retrospectively analyzed 46 pediatric CBD laparotomies performed at the study center between March 2010 and December 2021 and predictors of operative time. The patients were separated into a short operative time group (SOT) (≤ 360 min, n = 27) and a long operative time group (LOT) (> 360 min, n = 19). RESULTS: The preoperative AST and ALT values were higher, and the bile duct anastomosis diameter was larger, in the LOT. Correlation analysis demonstrated that the maximum cyst diameter, preoperative neutrophil-to-lymphocyte ratio, AST, ALT, AMY, and bile duct anastomosis diameter correlated positively with operative time. Multivariate analysis identified the maximal cyst diameter, preoperative AST, and bile duct anastomosis diameter as significant factors affecting surgical time. Postoperatively, intrapancreatic stones and paralytic ileus were observed in one patient each in the SOT, and mild bile leakage was observed in one patient in the LOT. CONCLUSIONS: The maximum cyst diameter, preoperative AST, and bile duct anastomosis diameter have the potential to predict the difficulty of performing a pediatric CBD laparotomy.

Robotic versus laparoscopic radical surgery for pediatric congenital biliary dilatation: a comparison of surgical outcomes of a single surgeon's initial experience.

PURPOSE: This study aimed to clarify the validity of robot-assisted surgery (RAS) for pediatric patients with congenital biliary dilatation (CBD). METHODS: We retrospectively compared RAS and laparoscopic surgery (LS) for pediatric CBD performed by the same certified surgeon between 2016 and 2022. RESULTS: We included 6 RAS and 12 LS cases in this study. One case of RAS with laparotomy was excluded from the analysis. The patients in the two groups had comparable ages and body weights. The median surgery duration, the suture time per stitch, and the time to drain removal were 385 min, 145 s, and 5 days in the RAS group and 370 min (p = 0.28), 177 s (p = 0.03), and 6 days (p = 0.03) in the LS group, respectively. The time to create the Roux-en-Y limb was significantly longer in the RAS group. Postoperative complications occurred in one RAS case and in four LS cases. CONCLUSIONS: Less anastomotic time per stitch and less time to drain removal suggest that RAS may contribute to accurate suturing and fine intra-pancreatic bile duct dissection. In addition, RAS requiring large movements of forceps in a large surgical field, such as Roux-en-Y creation, is inferior to LS.

Choledochal cyst.

Choledochal cyst (CC) or congenital biliary dilatation, has a skewed distribution with hereditary features that is far more common in East Asian females. CC is usually associated with pancreaticobiliary malunion (PBMU) forming a common channel. CC requires early definitive diagnosis, since there is a risk for malignancy occurring in the CC and/or intrahepatic bile ducts (IHBD). Complete CC excision and Roux-en-Y hepaticoenterostomy is required and can be performed by open or minimally invasive surgery with hepatojejunostomy the recommended procedure of choice. Principles of open surgical intervention form the basis of minimally invasive management with laparoscopy and robotic assistance. Current surgical management is associated with fewer early and late complications, such as hepaticoenterostomy anastomotic leakage, cholangitis, anastomosis stricture, and cholangiocarcinoma. Specific features of CC management at Juntendo include: intraoperative endoscopy of the common channel and IHBD for inspecting and clearing debris to significantly reduce post-operative pancreatitis or stone formation; near infra-red fluorescence with indocyanine green for visualizing tissue planes especially during minimally invasive surgery for CC; and a classification system for CC based on PBMU that overcomes inconsistencies between existing classification systems and clinical presentation.

Laparoscopic definitive surgery for congenital biliary dilatation with aggressive hilar bile ductoplasty and complete resection of the intrapancreatic bile duct in pediatric patients is safe and effective, comparable to open surgery.

BACKGROUND: After surgery for congenital biliary dilatation (CBD), hilar and hepatic bile duct stenosis and intrapancreatic bile duct remnants can cause many postoperative complications. We investigated the efficacy of laparoscopic surgery with aggressive bile ductoplasty and complete excision of the intrapancreatic bile duct in CBD patients compared to open surgery. METHODS: Pediatric patients who underwent surgery for CBD at our institution between 2006 and 2020 were divided into two groups: laparoscopic surgery (Lap group) and open surgery (Op group). Surgical outcomes were compared between the two groups. RESULTS: We enrolled 160 patients (85 in the Lap group and 75 in the Op group). Hilar bile ductoplasty was performed in 52 (61%) patients from the Lap group and 40 (53%) patients from the Op group. The operative time was significantly longer, the amount of blood loss was significantly lower, and the length of hospital stay was significantly shorter in the Lap group than in the Op group. Postoperative early and late complications occurred at a similarly low rate in both groups. When the surgical outcomes were compared between the group who underwent laparoscopic bile ductoplasty and the group that did not, postoperative early and late complications occurred similarly in both groups. CONCLUSIONS: Laparoscopic surgery for CBD with aggressive hilar bile ductoplasty and complete excision of the intrapancreatic bile duct is safe and effective, comparable to open surgery. However, it is necessary to verify whether long-term occurrence of hepatolithiasis similar to open definitive surgery can be obtained in the future.

Pancreaticobiliary maljunction in Turkish patients: a multicenter case series.

BACKGROUND AND AIMS: Pancreaticobiliary maljunction (PBM) is a malformation in which the pancreatic and bile ducts join outside the duodenal wall. It is associated with various biliary and pancreatic diseases. In addition, patients with PBM carry a substantial lifetime risk of developing biliary or gallbladder carcinoma. We aimed to present a multicenter case series of PBM from Turkey. METHODS: This study was conducted in adult and pediatric PBM patients who were referred to three tertiary reference centers of Turkey for endoscopic retrograde cholangiopancreatography (ERCP) between July 2007 and May 2020. The clinical presentations, types of PBM, ERCP findings, surgical histories, and the postoperative courses, including the development of biliary malignancies, were retrospectively reviewed. RESULTS: The study group included 47 (31 adult and 16 children) patients. Type D PBM was more frequent (13/41: 27.7%) than that reported in Eastern studies. Type A PBM was more common in the adults (51.6% vs. 12.5%, p < 0.05), whereas type C was more common in pediatric patients (31.3% vs. 13.2%, p < 0.05). Although fusiform anatomy was predominant in both of the groups, cystic dilatation was more common (25.8% vs. 12.5%) in adults and the common bile duct diameter was greater [22 mm (range 11-58) vs. 12 mm (range 5-33)] in adult patients compared to pediatric patients. Resective surgeries were more frequently done in pediatric patients (73.3% vs. 53.6%), whereas cholecystectomy was more frequently performed in adult patients (21.4% vs. 6.7%). CONCLUSION: Although our findings were compatible with Eastern studies, type D PBM (associated with pancreas divisum) was more frequent in our study population.

Utility of endoscopic retrograde cholangiopancreatography in management of pediatric pancreaticobiliary disease.

BACKGROUND: The purpose of this study was to evaluate the utility of endoscopic retrograde cholangiopancreatography (ERCP) in pediatric patients with pancreaticobiliary diseases. METHODS: A retrospective review was performed on patients who underwent ERCP for the treatment of biliary tract disease and detailed examination of pancreatitis at our institution from January 1999 to December 2020. RESULTS: ERCP was performed for congenital biliary dilatation (CBD) (n = 42), choledocholithiasis (n = 9), common bile duct stenosis (n = 1), and several types of pancreatitis (n = 13). The only severe complication of ERCP was common bile duct injury. Three (5.8%) of 52 biliary diseases failed to be treated by ERCP. All patients with pancreatic disease were correctly diagnosed and treated. CONCLUSIONS: Endoscopic biliary drainage with a temporary stent was adequate for symptomatic relief in CBD. Stenting of the pancreatic duct was useful for improving the angulation and drainage of the pancreatic duct. ERCP was useful for understanding the anatomy of the pancreatic duct and revealing potential treatments. Therefore, ERCP and transendoscopic therapy are sufficiently feasible in pediatric patients and should be actively introduced for the investigation and treatment of pancreaticobiliary diseases.

Current treatment strategies for postoperative intrahepatic bile duct stones in congenital biliary dilatation: a single center retrospective study.

BACKGROUND: Intrahepatic bile duct (IHBD) stones are one of the most common late complications of Roux-en-Y hepaticojejunostomy for congenital biliary dilatation (CBD). We report the current treatment strategies for IHBD stones and their outcomes in our institute. METHODS: Between 1983 and 2021, 117 patients with CBD were surgically treated in our institute. Our treatment strategies included oral ursodeoxycholic acid (UDCA), double-balloon endoscopic retrograde cholangiography (DB-ERC), percutaneous cholangio-drainage (PTCD), and open surgery. A retrospective study was conducted using medical charts. RESULTS: Postoperative IHBD stones were identified in 12 of 117 patients with CBD (10.2%). Five patients received UDCA, and small stones were successfully resolved in two cases. DB-ERC was performed eight times in five patients, but the endoscope could not reach the porta hepatis due to a long jejunal loop in two of five patients. One patient presented with severe acute pancreatitis induced by prolonged DB-ERC. PTCD was performed in three patients, two of whom finally underwent open surgery due to unsuccessful lithotomy. Open surgery was eventually performed in three patients. Lithotomy was performed in one patient; lithotomy with strictureplasty was performed in another patient. The other patient was diagnosed with intrahepatic cholelithiasis with adenocarcinoma. He underwent left lobectomy and died of carcinomatous peritonitis. CONCLUSIONS: Oral UDCA may be effective for small stones. Although DB-ERC should be considered as a first-line interventional therapy for lithotomy, it may not be feasible due to a long jejunal loop, and pancreatitis may occur. Long-term follow-up and early detection and treatment for IHBD stones may yield a good prognosis.

A proposed feasible classification of common bile duct duplications based on a newly described variant and review of existing literature.

BACKGROUND: Duplication of the common bile duct (CBD) is extremely rare among the anatomical variations in the biliary tract system, which presents a septum within the CBD or an accessory CBD. In our study, we report a rare case of duplication of the common bile duct combined congenital biliary dilatation.we present a rare case of a septum in the dilated biliary tract. CASE PRESENTATION: We reported a 5-year-old Asian girl who had history of repeated abdominal pain for 4 days and aggravated for 1 day. Magnetic resonance cholangiopancreatography (MRCP) examination revealed duplicated common bile duct (DCBD) malformation with congenital biliary dilatation and distal cholelithiasis. The patient underwent choledochal cyst resection and biliary tract reconstruction and abdominal cavity irrigation and drainage under general anesthesia. A septum was found within the common bile duct during the operation. The septum divided the extrahepatic bile duct into two parts connected to the left and right hepatic ducts respectively and the gallbladder is attached to the repeated right bile duct which was not previously reported in the literature. CONCLUSIONS: We complement and adjust the classification of common bile duct duplication by reviewing the literature.

Risk of carcinogenesis in the biliary epithelium of children with congenital biliary dilatation through epigenetic and genetic regulation.

PURPOSES: Congenital biliary dilatation (CBD), defined as pancreaticobiliary maljunction (PBM) with biliary dilatation, is a high risk factor for biliary tract cancer (BTC). KRAS and p53 mutations reportedly affect this process, but the mechanisms are unclear, as is the likelihood of BTC later in life in children with CBD. We investigated potential carcinogenetic pathways in children with CBD compared with adults. METHODS: The subjects of this study were nine children with CBD and 13 adults with PBM (10 dilated, 3 non-dilated) without BTC who underwent extrahepatic bile duct resections, as well as four control patients who underwent pancreaticoduodenectomy for non-biliary cancer. We evaluated expressions of Ki-67, KRAS, p53, histone deacetylase (HDAC) and activation-induced cytidine deaminase (AID) in the biliary tract epithelium immunohistochemically. RESULTS: The Ki-67 labeling index (LI) and expressions of KRAS, p53, HDAC, and AID in the gallbladder epithelium were significantly higher or tended to be higher in both the children with CBD and the adults with PBM than in the controls. CONCLUSIONS: BTC may develop later in children with CBD and in adults with PBM, via HDAC and AID expression and through epigenetic and genetic regulation.

Using indocyanine green fluorescence in laparoscopic surgery to identify and preserve rare branching of the right hepatic artery in pediatric congenital biliary dilatation.

We describe a laparoscopic surgical technique using indocyanine green (ICG) fluorescence to identify and preserve rare arterial branching associated with pediatric congenital biliary dilatation. Congenital biliary dilatation with pancreaticobiliary maljunction was diagnosed in a 9-year-old girl, who presented with upper abdominal pain. Abdominal enhanced computed tomography (CT) showed that the accessory right hepatic artery (aRHA) branched from the posterior superior pancreaticoduodenal artery (PSPDA) and flowed through the right aspect of the dilated common bile duct (CBD) directly into the right lobe of the liver. We performed laparoscopic dilated biliary duct resection and hepaticojejunostomy, administering ICG intravenously, at a dose of 0.6 mg/kg. The ICG fluorescence overlay mode showed an aRHA running along the right side of the dilated CBD. The aRHA was dissected from the CBD without injury. After finishing the anastomosis, the beating of the aRHA was preserved, confirming that blood flow had been maintained.

Pancreaticobiliary maljunction without biliary dilatation in pediatric patients.

PURPOSE: Pancreaticobiliary maljunction (PBM) without biliary dilatation is a condition in which dilatation of the bile duct is not seen in patients with PBM. Recently, the Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) published new diagnostic criteria for PBM. In these criteria, biliary dilatation is defined according to the standard diameter at each age. We reviewed cases of pediatric patients with PBM without biliary dilatation. METHODS: From 1992 to 2019, 134 patients with PBM were treated in our institution. Among these, 7 patients were retrospectively diagnosed with PBM without biliary dilatation. The clinical information was retrospectively assessed in these patients. RESULTS: Of the seven patients, six were female. All patients had symptoms similar to those of patients with congenital biliary dilatation. In all seven patients, the diagnosis of PBM was made before definitive surgery. Six patients had type B PBM, and one had type D PBM. All patients underwent extrahepatic bile duct resection and hepaticojejunostomy, and their symptoms resolved. One patient experienced postoperative complications of anastomotic leakage followed by anastomotic stricture. CONCLUSION: The present report revealed important clinical features of this entity. However, there are still some issues that need to be discussed, and further research is needed.

Laparoscopic and robot-assisted surgery for adult congenital biliary dilatation achieves favorable short-term outcomes without increasing the risk of late complications.

PURPOSE: Minimally invasive surgery (MIS) is the optimal treatment for congenital biliary dilatation (CBD), but few studies on adults have been reported. We compared the postoperative outcomes of MIS with those of open surgery (OS) in adult patients with CBD. METHODS: The subjects of this retrospective study were 36 adult patients who underwent surgery for CBD. We compared the postoperative outcomes of 20 patients who underwent laparoscopic (n = 15) or robotic (n = 5) surgery with those of 16 patients who underwent OS. RESULTS: MIS was associated with a significantly higher rate of type I (p < 0.001), significantly less blood loss (p < 0.001), a significantly lower rate of internal stents (p = 0.048), significantly longer operation times (p = 0.009), and a significantly shorter postoperative hospital stay (p = 0.007) than OS. The postoperative outcomes of MIS for type I CBD were similar to those of the whole cohort. There were no significant differences in late complications between the groups. The robotic procedure had a significantly shorter operative time than laparoscopic surgery for hepaticojejunostomy (HJ; p = 0.029). CONCLUSIONS: MIS achieved favorable short-term outcomes without compromising mid-term outcomes compared to OS and is appropriate for adult patients with CBD. Robotic HJ may be more ideal than its laparoscopic counterpart.

Baseline assessment of enhanced recovery after pediatric surgery in mainland China.

BACKGROUND: Enhanced recovery after surgery (ERAS) is a clinical pathway that optimizes perioperative management based on evidence-based medicine. ERAS has been gradually introduced to pediatric surgery in recent years. However, there are limited reports on its overall implementation. We aimed to determine the implementation of ERAS in patients who received pediatric surgery in mainland China. METHODS: We designed a questionnaire involving 17 key ERAS elements and sent the questionnaire to 66 chiefs of pediatric surgery distributed throughout 31 provinces in mainland China to obtain a baseline assessment of the assimilation of ERAS protocols in the care of congenital biliary dilatation (CBD). RESULTS: A total of 66 questionnaires were collected. The range of elements implemented at participating centers was 4-16, with a mean of 10.23. The least commonly practiced elements were administration of non-opioid preoperative analgesia (6 centers, 9.09%), prevention of postoperative nausea and vomiting [PONV] (9 centers, 13.64%), and postoperative pain management (26 centers, 39.39%). CONCLUSIONS: The implementation of elements differed from center to center. Measures relying primarily on anesthesiologists had lower execution. The adherence to ERAS elements was often inhibited by a lack of institutional support, poor knowledge of ERAS protocols, and difficulties in coordinating multidisciplinary care, as well intransigence in changing surgical practices out of fear of liability for poor outcomes.

Treatment and follow-up of late onset intra hepatic bile duct stones in congenital biliary dilatation.

PURPOSE: The postoperative course after surgery for congenital biliary dilatation (CBD) has some complications. Intrahepatic bile duct (IHBD) stones were known as a late complication. We report on the treatment and long-term follow-up of postoperative IHBD stones in our department. METHODS: Patients who underwent CBD surgery at age 15 years or younger in our department were identified. Those followed up for 5 years or more were enrolled. Annual blood chemistry tests and abdominal ultrasonography were performed. Each patient's surgical procedure, IHBD stone diagnosis, treatments, and outcomes were retrospectively assessed. RESULTS: Fifty-one patients were analyzed. The median age at the last visit was 24 years (range 7-45 years), and the median age at CBD surgery was 3 years. Eight patients (16%) developed late-onset IHBD stones. The median age at onset was 25 years, and the median duration after surgery was 20 years. The initial treatment was double-balloon enteroscopy (DBE) in 4 cases, which resulted in stone removal in 3 of the 4 patients (75%). CONCLUSION: Since CBD may cause late-onset IHBD stones, continuous follow-up is required even in adulthood. In this study, DBE was effective and minimally invasive, and it is recommended as the initial treatment.

Late postoperative complications of congenital biliary dilatation in pediatric patients: a single-center experience of managing complications for over 20 years.

PURPOSE: To investigate late complications after surgery for congenital biliary dilatation (CBD). METHODS: We retrospectively reviewed the patients treated for late postoperative complications of extrahepatic bile duct resection with bilioenteric anastomosis for CBD at our hospital between 1999 and 2019. RESULTS: Twenty-seven complications, including bile duct stenosis with (n = 19) or without (n = 3) hepatolithiasis, remnant intrapancreatic bile duct (n = 2), intestinal obstruction (n = 2), and refractory cholangitis (n = 1) were treated in 26 patients. The median age at radical surgery and the initial treatment of complications was 3 years, 2 months and 14 years, 5 months, respectively. The median period from radical surgery to initial treatment of complications was 7 years, 1 month. Before 2013, bile duct stenosis was initially treated with bile duct plasty (n = 11) or hepatectomy (n = 3), and 71.4% (n = 10) of patients needed further treatment; after 2013, double-balloon endoscopic retrograde cholangiography (DBERC) was used (n = 8), and 25% (n = 2) of patients needed further treatment. Patients with remnant intrapancreatic bile duct, intestinal obstruction, and refractory cholangitis required surgery. CONCLUSION: Long-term follow-up is necessary after surgery for congenital biliary dilatation. DBERC is thus considered to be useful for bile duct stenosis management.

Differences in post-operative complications after reconstruction for congenital biliary dilatation in a single institution-Roux-en-Y hepaticojejunostomy versus hepaticoduodenostomy.

BACKGROUND: The standard surgical procedure for congenital biliary dilatation (CBD) consists of excision of extrahepatic bile duct and Roux-en-Y hepaticojejunostomy (HJ). However, alternative reconstructive operations for CBD includes hepaticoduodenostomy (HD). We compared postoperative complications and therapeutic outcomes of these two operations at our institution. METHODS: From 1981 to 2009, there were 23 traceable patients who underwent operation for CBD, They were divided into an HJ Group (n = 15) and an HD Group (n = 8). Demographic and outcome data were compared. RESULTS: There were no significant differences in postoperative complications (cholangitis, pancreatitis, and anastomotic stenosis) and current blood test data (AST, ALT, Total Bilirubin, Direct Bilirubin, Amylase) between the two groups. Current abdominal pain and carcinogenesis were not observed in either group. Intrahepatic stones occurred in one patient in each group, both > 25 years post-operation. CONCLUSION: HD is considered to be an acceptable reconstruction method compared to HJ, based on our results. There has been a suggestion that inadequate diversion in HD might increase the risk of cholangiocarcinoma. To date, in this series, that has not happened.