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One of the main causes of stricture at hepaticojejunostomy site after surgery for congenital biliary dilatation is inflammation or infection associated with bile leak. The aim of this study was to determine the risk factors and outcomes of bile leak after laparoscopic surgery. We retrospectively reviewed the demographics and outcomes of patients who underwent laparoscopic surgery for congenital biliary dilatation between September 2013 and December 2019. Data from patients with bile leak were compared to data from patients without bile leak. Fourteen of 78 patients had bile leak. Hepatic duct diameter at anastomosis was the only risk factor of bile leak. Patients with the diameter ≤ 10 mm had higher incidence of bile leak than in patients with the diameter > 10 mm (P = 0.0023). Among them, bile leak occurred more frequently in patients operated on by non-qualified surgeons based on the Japan Society for Endoscopic Surgery endoscopic surgical skill qualification system than by qualified surgeons (P = 0.027). However, none of the patients with bile leak developed anastomotic stricture afterwards. Although good technical skill is necessary to avoid bile leak in narrow hepatic duct cases (≤ 10 mm), slight bile leak may not result in anastomotic stricture.

Background: The commonly used anatomical classification of congenital biliary dilatation by Todani is not directly related to types of pancreaticobiliary maljunction (PBM). This work is to investigate clinical presentation and surgical outcomes according to PBM classification proposed by the Japanese Study Group on Pancreaticobiliary Maljunction. Pancreaticobiliary junction angle, common channel length, and diameter of dilatation are studied as well. Materials and Methods: Patients of redo operation, without preoperative images, and lacking clearly documented outcomes were excluded. After exclusion, 79 patients who underwent Roux-en-Y hepaticojejunostomy between January 1994 and December 2019 were enrolled for this retrospective study. Medical records were reviewed, and perioperative parameters were collected. Todani's classification, PBM types, pancreaticobiliary junction angle, common channel length, and dilatation diameter were determined based on magnetic resonance cholangiopancreatography or computed tomography. Clinical presentation and outcome were compared between groups of different anatomical features. Results: PBM type A (stenotic), type B (nonstenotic), and type C (dilated channel) consisted of 48 (60.8%), 18 (22.8%), and 11 (13.9%) patients, respectively; and two patients (2.5%) had no PBM. Patients of PBM type A were younger and had more Todani's type Ia lesion. Patients of PBM type B and C had either Todani's type Ia or Ic lesion, but type IVa had only PBM type B. Longer common channel (1.27 vs. 0.81 cm, P < 0.001) and wider dilatation (4 vs. 2 cm, P < 0.001) were found in patients with right pancreaticobiliary angle (90°). Clinical outcome was similar in different Todani's types, PBM types, and pancreaticobiliary angle. Serum alkaline phosphatase level higher than 675 U/L was associated with major perioperative complications. Preoperative jaundice, mass, and dilatation wider than 5 cm were related to subsequent liver cirrhosis. Conclusion: PBM types and pancreaticobiliary junction groups are feasible for surgical planning, but not related directly to outcome. Palpable abdominal mass suggests higher risk of perioperative and late complications. For subsequent liver cirrhosis, laboratory data of disease onset and dilatation diameter wider than 5 cm are important risk factors.

Background The present study aimed to evaluate anatomical variations of the right hepatic artery (RHA) in patients with congenital biliary dilatation (CBD) and the appropriate approach in laparoscopic surgery for CBD. Methods The medical records of 36 patients who underwent laparoscopic or open surgery for CBD from 1996 to 2018 were retrospectively reviewed. Radiological evaluation of the origin and course of the RHA in these 36 patients were compared with 195 control patients without CBD. Results The incidence of the RHA crossing anterior to the common hepatic duct (CHD) was significantly higher in patients with CBD than in those without CBD (33% versus 10%, P = .0001). There was no intraoperative injury of the RHA, irrespective of the course of the RHA. The CHD was divided at the caudal side of the RHA in 11 of 12 patients (92%) with the anterior type of RHA, and in 13 of 24 patients (54%) with the posterior type of RHA (P = .03). Conclusions Patients with CBD had a higher incidence of the RHA crossing anterior to the CHD than patients without CBD. Preservation of the RHA in each situation is necessary during surgery for CBD in the era of laparoscopic surgery.

Background: To investigate the effect of embedded hepaticojejunostomy in children with pancreaticobiliary maljunction (PBM) without biliary dilatation. Materials and Methods: The clinical data of 10 patients with nondilated PBM from February 2017 to July 2020 were retrospectively analyzed. Perioperative liver function indexes were compared. Results: All patients were diagnosed by magnetic resonance cholangiopancreatography (MRCP) combined with intraoperative cholangiography. There were 5 cases of Komi type I and 5 cases of type II; the diameter of the common bile duct was 4-9 mm (median: 6 mm); and the length of the common channel was 5-15 mm (median: 9.25 mm). The procedure for one patient with common duct stones was converted to open surgery. Laparoscopic cholecystectomy, common bile duct resection, and embedded hepaticojejunostomy were successfully performed in all 10 cases. The average operation time was 225 ± 96.64 min, and the intraoperative blood loss was 2-5 mL. The mean time to oral intake was 3.5 ± 1.65 days (range: 2-5 days), and the mean hospitalization duration was 6.2 ± 2.44 days (range: 5-8 days). The differences in liver function indexes in the perioperative period were statistically significant (P < .05). The patients were followed-up for 13 to 54 months (median: 40 months). All patients grew well and there was no bile duct dilatation, calculus, or cirrhosis on B-ultrasound examinations. Conclusions: The clinical manifestations of nondilated PBM are often concealed, and preoperative MRCP was important for obtaining a diagnosis. Laparoscopic cholecystectomy, common bile duct resection, and embedded hepaticojejunostomy are feasible for treating nondilated PBM.

Purpose: Various studies have shown the benefit of three-dimensional (3D) systems over two-dimensional (2D) systems in endoscopic surgery. However, few studies have focused on pediatric en...

Background In congenital biliary dilatation, the protein plug is likely impacted, especially in a dilated common channel. However, nobody has discussed whether this dilatation of common channel remains after radical operation. The aim of this study was to investigate the situation of the dilated common channel after radical operation. Methods The status of the common channel was investigated at radical operation and follow-up. We then evaluated whether dilation of the common channel remained postoperatively. Moreover, the association between status of the common channel and postoperative pancreatic complication was evaluated. Results Postoperative condition of the common channel was able to be evaluated ultrasonographically in 42 patients. The common channel was dilated at the time of operation in 18 patients and remained dilated postoperatively in 11 of these 18 patients. Dilatation of the common channel was not identified in any patients without dilatation at the time of operation. No patients in this series showed any postoperative pancreatic complications, even if a dilated common channel remained. Conclusions Dilatation of the common channel can remain postoperatively in congenital biliary dilatation, but does not appear to be associated with postoperative pancreatic complications.

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.

Much about the aetiology, pathophysiology, natural course and optimal treatment of choledochal malformation remains under debate. Surgeons continuously strive to optimize their roles in the management of choledochal malformation. Nowadays the standard treatment is complete cyst excision followed by Rouxen-Y hepaticojejunostomy, be it via a laparotomy, laparoscopy or robot-assisted procedure. Whatever surgical endeavor is undertaken, it will be a major operation, with significant morbidity. It is important to realize that especially in asymptomatic cases, this is considered prophylactic surgery, aimed at preventing symptoms but even more important the development of malignancy later in life. A clear overview of long-term outcomes is therefore necessary. This paper aims to review the long-term outcomes after surgery for choledochal malformation. We will focus on biliary complications such as cholangitis, the development of malignancy and quality of life. We will try and identify factors related to a worse outcome. Finally, we make a plea for a large scale study into quality and course of life after resection of a choledochal malformation, to help patients, parents and their treating physicians to come to a well-balanced decision regarding the treatment of a choledochal malformation. (C) 2020 The Authors. Published by Elsevier Inc.

Congenital biliary dilatation (CBD) associated with intestinal malrotation (IM) has rarely been reported. We herein report three cases of pediatric CBD associated with IM that was incidentally found at surgery. The patients underwent total resection of the extrahepatic bile duct followed by hepaticoduodenostomy at the hepatic hilum or hepaticoduodenostomy as the definitive surgery for CBD. Ladd's procedure was simultaneously performed for asymptomatic IM. Postoperative adhesive intestinal obstruction occurred in all three patients, and conservative treatment was successful. Duodenogastric regurgitation was also observed as a biliary reconstruction-related complication, and conversion to hepaticojejunostomy at the hepatic hilum was performed in two of the three patients. We herein present the clinical course of these three patients and discuss the clinical significance of the association between CBD and IM in terms of the surgical strategy and in comparison with 12 previously reported cases. Keywords: Congenital biliary dilatation, Intestinal malrotation, Postoperative adhesive intestinal obstruction

Laparoscopic surgery as a treatment for congenital biliary dilatation is uncommon. We herein present a series of laparoscopic surgeries for congenital biliary dilatation performed in our institution and review our experience with this approach over a long period of time. Medical records of 36 consecutive patients who underwent laparoscopic surgery for congenital biliary dilatation from 1996 to 2015 were retrospectively reviewed. Data on patient demographics, operative time, blood loss, hospital stay, and complications were evaluated. A comparison between the former period (Group A, 1996–2005) and the latter period (Group B, 2006–2015) was performed. The patients comprised 23 females and 13 males with a median age of 34 years. The median operative time, blood loss, and hospital stay was 493 min, 154 g, and 11 days, respectively. Total early and late complications occurred in 7 (19%) and 2 (5%) patients, respectively. A comparison between Groups A and B revealed no significant difference in operative time or complications, but operative blood loss, open conversion, and hospital stay were significantly lower in Group B than in Group A (P

This study aimed to investigate the clinical features and risk factors of bile duct perforation in pediatric congenital biliary dilatation (CBD) patients. CBD patients, whose initial symptom was abdominal pain, were enrolled in this study and were divided into perforated and non-perforated groups. The clinical features of the perforated group were investigated. Moreover, the age at operation, sex, and morphologic features of the extrahepatic bile duct were compared between the groups. Fifteen cases of bile duct perforation (10.4%) were identified among the 144 CBD patients who had abdominal pain. Majority of bile duct perforation occurred in patients aged

Cholangiocarcinoma developing from the remnant bile ducts following cyst excision for congenital biliary dilatation (CBD) is an increasing problem. There are some reports demonstrating that cholangiocarcinoma develops in approximately 0.7–6.5% of patients who undergo cyst excision; the incidence is 121.5 times higher than that of the general population. The cumulative incidence of cholangiocarcinoma at 15, 20, and 25 years after cyst excision for CBD is reported to be 1.6%, 3.9%, and 11.3%, respectively. Repeated cholangitis, hepatolithiasis, or remnant dilated bile ducts due to inadequate cyst excision have been reported to be risk factors for cholangiocarcinoma following cyst excision. Biliary-enteric anastomosis itself is also reported to be a risk factor for cholangiocarcinoma. The outcomes of treatment for cholangiocarcinoma developing from the remnant bile ducts following cyst excision are unfavorable. The overall cumulative survival rates at 2 and 3 years after treatment were reported to be 32% and 16%, respectively. The median survival time was 15 months. Most patients were diagnosed at the late stage of cholangiocarcinoma. Longer follow-up is needed, even after complete cyst excision, because of the lifelong risk of subsequent cholangiocarcinoma. The regular checkups that include the evaluation of tumor markers (including CEA and CA19-9) and imaging studies, such as abdominal US and CT, are useful for the diagnosis of cholangiocarcinoma developing from the remnant bile ducts.

The treatment of choice for congenital biliary dilatation is complete excision with Roux-en-Y hepaticojejunostomy, a procedure that is now being performed laparoscopically. We describe our technique (laparoscopic dilated bile duct dissection, distal common bile duct ligation, intrahepatic bile duct and common channel protein plug clearance, and customizing the length of the Roux-en-Y loop) and discuss attendant issues from our wealth of experience of treating this condition using both open and minimally invasive surgery.

Laparoscopic surgery has become a common procedure for congenital biliary dilatation. The dilated choledochus should be removed completely just above the confluence of the common biliopancreatic channel at the distal end and approximately 5 mm from the confluence of the right and left hepatic ducts at the proximal end to avoid complications of the its remnant. The operation is feasible and safe. The rate of conversion to open surgery is low. The rate of complication of laparoscopic surgery performed by skilled surgeons is also low, even lower than that of open surgery. There is no difference between hepaticoduodenostomy and hepaticojejunostomy concerning the rate of cholangitis. Gastritis due to bilious reflux occurred with a low rate in hepaticoduodenostomy. Both techniques could be used for congenital biliary dilatation; however, hepaticoduodenostomy should be applied for congenital biliary dilatation without intrahepatic dilatation of biliary tract.

Pancreaticobiliary maljunction (PBM) describes both cases in which the extrahepatic bile duct is dilated (PBM with biliary dilatation, congenital biliary dilatation) and those in which it is not dilated (PBM without biliary dilatation). A recent detailed report of extrahepatic bile duct measurements using ultrasonography has largely defined a non-dilated bile duct. According to the report, the maximum inner diameter of an extrahepatic bile duct increases according to age. Once the diagnosis of PBM is established, immediate prophylactic surgical treatment is recommended before the onset of malignant changes, even in patients without biliary dilatation. Cholecystectomy and resection of the extrahepatic bile duct, so-called flow-diversion surgery, are established standard surgical methods for PBM with biliary dilatation. As a therapeutic strategy for PBM without biliary dilatation, prophylactic cholecystectomy is also strongly recommended as standard surgical treatment for prevention of gallbladder cancer. However, whether additional prophylactic resection of the extrahepatic bile duct, as in flow-diversion surgery, should be performed for PBM without biliary dilatation remains controversial. Further investigation and surveillance are needed to clarify the appropriate surgical strategy for PBM without biliary dilatation.

Until now, there have been no practical clinical guidelines for congenital biliary dilatation (CBD). In this review article, the Japanese Study Group on Congenital Biliary Dilatation (JSCBD) propose to establish clinical practice guidelines for CBD. Because the evidence-based literature is relatively small, we decided to create guidelines based on the consensus of experts, using the medical literature for reference. A total of 20 clinical questions (CQs) were considered by the members of the editorial committee responsible for the guidelines. The CQs included the distinct aspects of CBD: (1) Concepts and Pathology (three CQs); (2) Diagnosis (six CQs); (3) Pancreaticobiliary Complications (three CQs); Treatments and Prognosis (eight CQs). Each statements and comments for CQs were made by the guidelines committee members. CQs were finally approved after review by members of the editorial committee and the guidelines evaluation board of CBD. These guidelines were created to provide assistance in the clinical practice of CBD management; their contents focus on clinical utility, and they include general information on CBD to make this disease more widely recognized.

Pancreaticobiliary maljunction (PBM) is a congenital anomaly, which can be defined as a union of the pancreatic and biliary ducts located outside off the duodenal wall. We herein investigate clinical features of PBM including as the 2nd report of a Japanese nationwide survey.During a period of 18 years (from 1990 to 2007), 2,561 patients with PBM were registered at 141 medical institutions in Japan. Among them, eligible patients (n = 2,529) were divided into two groups: adult (n = 1,511) and pediatric patients (n = 1,018). Comparisons of clinical features including associated biliary cancers were performed according to the biliary dilatation (BD), age factor, and time era.Only one case in pediatric patients with BD combined with a bile duct cancer (0.1 %). In adult patients, the bile duct cancer and the gallbladder cancer was seen in 6.9 and 13.4 % patients with BD and in 3.1 and 37.4 % patients without BD, respectively. In adult patients with BD, the occurrence rates of biliary cancers were increased in latter period (00'-07') compared with former period (90'-99'). The ratio of biliary cancer localization was changed between former and latter period, and the bile duct cancer was increased in latter period (from 5.5 to 9.3 %).The largest series of PBM were evaluated to clarify the clinical features including the associated biliary cancer in this Japan-nationwide survey. This report could be widely used in the future as a reference data for diagnosis and treatment of PBM.

Purpose We examined the clinical significance of duodenogastric regurgitation (DGR) as a late complication in the long-term follow-up after hepaticoduodenostomy (HD) as a reconstruction surgery for congenital biliary dilatation (CBD). Methods Seventeen patients with CBD were retrospectively analyzed for late complications (mean follow-up, 16.8 years). All patients had undergone total resection of the extrahepatic bile duct followed by HD. DGR was identified using endoscopic examination, intraluminal bile monitoring, and liver scanning. Results DGR was found in all 17 patients by endoscopic examination and intraluminal bile monitoring. Fourteen of the 17 (82.4%) patients with DGR had experienced abdominal symptoms since a mean of 6.9 years postoperatively. Liver scanning also revealed apparent DGR in all 14 symptomatic patients. We converted 7 of the 14 patients to hepaticojejunostomy reconstruction at a mean of 13.0 years after the initial excisional surgery. Their symptoms were completely relieved postoperatively. Conclusions DGR is an important complication after HD. Examination of patients for the development of DGR is an essential part of long-term follow-up in patients with CBD who have undergone HD as a reconstruction surgery. Conversion surgery is recommended in patients with DGR accompanied by long-term abdominal symptoms. Levels of evidence Level IV.

Background and Aim Endoscopic ultrasonography (EUS)-guided choledochoduodenostomy (EUS-CDS) is increasingly used in the treatment of malignant distal biliary obstruction. Standardized use of this technique requires improvements in instruments, including more convenient and safer devices. The present study was designed to evaluate the resistance force to migration (RFM) of a newly designed laser-cut metal stent and the feasibility of EUS-CDS using this stent. Methods This experimental study used a porcine model of biliary dilatation involving five male pigs. The new stent is a fully covered laser-cut stent with anti-migration anchoring hooks. The RFM of the new stents was compared with those of three commercially available covered metal stents using a phantom model. In the animal study, after ligation of Vater's ampulla with endoscopic clips, the dilated common bile duct was punctured under EUS guidance, followed by EUS-CDS using the new stent. One week after the procedure, the stents were removed endoscopically and the fistulas were assessed after the pigs were killed. Technical feasibility and clinical outcomes were evaluated. Results Among the four stents, the new stent had the highest RFM. Metal stent placement was successful in all five pigs, with no procedure-related complications occurring during and 1 week after endoscopic intervention. All stents remained in place without migration and were removed easily using a snare. At necropsy, fistulas were created between the bile duct and duodenum in all pigs. Conclusion EUS-CDS using a newly designed metal stent was feasible and effective in this porcine model of biliary dilatation.

Background Biliary cysts are duct dilatation that can occur on all biliary ducts, 20–25% is diagnosed in adults. The classic triad for the clinical presentation consists on abdominal pain, jaundice and abdominal mass. The standard treatment is surgical resection and bilioenteric anastomosis. The objective of this study is to analyze the prevalence and characteristics of biliary cysts in our center. Methods This is an observational retrospective study, we included patients older than 16 years old with biliary cyst from march 1989 to February 2015. The demographic and clinical information was collected from the charts and electronic records available at our Hospital. Results Biliary cysts were reported on 52 patients, only 25 clinical charts were available. The main symptom was abdominal pain in 21 (84%). The diagnosis was performed with abdominal ultrasound in 16 (64%). The most frequent type was IA in 9 (36%). All patients were treated with surgery as a definitive management. Discussion Vague clinical presentation results on a delay of the diagnosis and treatment. Surgical resection is recommended for patients since they have an increased risk for malignant transformation. Postoperative complications in our patients were stenosis of bilioenteric anastomosis in 3 (12%) patients. Conclusions Biliary cysts require an accurate diagnosis and surgical treatment in order to decrease the risk of malignant transformation and progression of the disease. Precise surgical treatment is needed to achieve complete resection and a long term postoperative follow up is mandatory., Highlights • The objective is to analyze prevalence and characteristics of biliary cysts in our center. • Vague clinical presentation results on a delay of the diagnosis and treatment. • Surgery is recommended because of increased risk for malignant transformation. • Most common complication in our patients was stenosis of bilioenteric anastomosis.

Background The Diagnostic Criteria for Pancreaticobiliary Maljunction 2013 were published by the Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) in 2014. The committee of JSGPM for diagnostic criteria for pancreaticobiliary maljunction has established the standard diameter of the bile duct, and a definition of dilatation of the bile duct was proposed in 2014. Methods The committee of JSGPM prepared the diagnostic criteria for congenital biliary dilatation in 2014, and a final revised version was approved in 2015. Results Congenital biliary dilatation is defined as a congenital malformation involving both local dilatation of the extrahepatic bile duct, including the common bile duct, and pancreaticobiliary maljunction. However, cases associated with intrahepatic bile duct dilatation can also be included. Various kinds of pathological conditions can occur on hepatobiliary systems and pancreas by bile duct dilatation and pancreaticobiliary maljunction. For a diagnosis of congenital biliary dilatation, both abnormal dilatation of the bile duct and pancreaticobiliary maljunction must be evident by either imaging test or anatomical examination. Acquired or secondary dilatation of the bile duct by obstruction due to biliary stones or malignancy should be strictly excluded. Conclusion Diagnostic criteria for congenital biliary dilatation 2015 were established from Japan representing a world first.

A departmental survey of personal methodology in the measurement of bile duct diameter was carried out by means of a representative diagram of a typical bile duct image configuration. The results revealed considerable differences in measurement practice, and factors that may explain the patterns of interobserver variation, among 20 respondents in this task, were postulated. There was considerable scope for error, by a factor of three times between the smallest and largest diameter selected noted within current practice variation. This reflects similar disparity found in the literature. Within the setting of the development of current therapeutic strategies, there may be a need for confirmation and/or re-establishment of contemporary normal ranges for biliary tree diameter. A study of reasonable magnitude would be required to clarify the many and complex common and special causes of variation in ultrasonic evaluation outlined towards enhancing the utility of this useful and hitherto important ultrasonic sign.

はじめに : 先天性胆道拡張症は比較的まれな疾患である．難治性の胆管炎や悪性腫瘍の発生の観点から外科的治療の対象となりえるが，外科的治療後でさえ繰り返す胆管炎や胆汁うっ滞のために二次性の肝硬変に陥ることもある．先天性胆道拡張症に対する肝移植適応の指針はいまだ確立されていない．対象と方法 : 今回，1,101例の肝移植症例を対象とし，5例のCaroli病を含む11例の先天性胆道拡張症の詳細を検討した．結果 : 11例中9例は肝移植に先行する手術既往が認められたが，2例の術後経過良好なCaroli病症例は肝移植を初回手術として受けていた．全症例が肝硬変に起因する難治性の症状を伴っており，20歳以下の全例に成長障害が認められた．摘出肝の病理組織学的所見はF2以上の線維化を呈していた．ABO不適合の1症例は死亡の転帰となった．Caroli病の1症例は肝内胆管癌を伴っていたが，術後11.8年の現在も無再発生存中である．まとめ : 肝不全に伴う難治性の症状や合併症を呈する先天性胆道拡張症患者は肝移植の適応である．臨床経過，成長障害の有無，画像所見および肝線維化の程度などの示唆に富む因子を熟慮したうえでの施行となるが，肝移植は先天性胆道拡張症に対する有効な治療法の一つである．なお，本稿は日本消化器外科学会の規定に従うDigestive Surgeryからの転載である．

We report six patients with adult congenital biliary dilatation treated by surgery. Of the six cases, five were female and the patients' age at diagnosis ranged from 19 to 51 years old. By Todani's classification for bile duct dilatation, three were categorized as Ia, one as Ib, and two as IVa. All six cases had anomalous arrangement of the pancreatobiliary duct. Resection of the cystic portion and hepaticojejunostomy (Roux-Y) were performed in all. After surgery, one patient classified as Ia and one as IVa had complications of cholangitis and intrahepatic stones. We removed the stones by the percutaneous transhepatic route with dilatation of the stenotic anastomosis, but cholangitis recurred in the IVa patient. Although surgical resection of the cystic portion and reconstruction of biliary tract is considered to be a standard treatment for adult congenital biliary dilatation, this IVa case had complications after surgical treatment. Thus short-term follow-up is necessary to prevent or diagnose stenotic anastomosis following the operation.

Background: Congenital biliary dilatation is a rare disease. Although the possibility of refractory cholangitis and/or the frequency of malignant tumors legitimize hepatobiliary surgery, repeated cholangitis and biliary obstruction result in secondary liver cirrhosis even after polysurgery. There are no definitive guidelines on liver transplantation for congenital biliary dilatation. Patients: A total of 1,101 liver transplantation recipients were enrolled in this study. Eleven patients with congenital biliary dilatation including 5 patients with Caroli’s disease were retrospectively analyzed in detail. Results: Nine of 11 patients underwent initial operations before liver transplantation while 2 patients with Caroli’s disease received liver transplantation as initial surgery, with good outcomes. All patients had intractable symptoms caused by liver cirrhosis, and growth delay was considerable in patients aged Conclusions: Patients with congenital biliary dilatation with refractory symptoms and complications secondary to liver failure are appropriate candidates for liver transplantation. We suggest that liver transplantation is an effective therapeutic option for patients with congenital biliary dilatation with due consideration to many accompanying factors, such as clinical course, growth delay, image findings and histopathological analysis.

Im Kindes- und Jugendalter kommen bei einer Cholestase mit Erweiterung der Gallenwege differenzialdiagnostisch als wichtigste Ursachen insbesondere Gallensteine, eine Choledochuszyste oder ein Tumor in Betracht. Durch den vorliegenden Fall wird die Schwierigkeit der richtigen Diagnosefindung deutlich. Das Rhabdomyosarkom der Gallenwege ist ein sehr seltener Weichteiltumor. Um eine Fehldeutung im Sinne einer Choledochuszyste zu vermeiden, muss in der Bildgebung besonders auf mogliche Veranderungen im Bereich des pankreatikobiliaren Systems geachtet werden.

Periampullary diverticulum (PAD) often presents as an incidental CT finding. Its significance and its effect on biliary dilation are unclear. The aim of our study was to determine if the presence of a PAD is associated with abnormal dilation of the common bile duct (CBD).Patients with PAD were retrospectively identified from the radiology database from November 2011 to November 2012 and those with known pancreaticobiliary pathology were excluded, except patients with cholelithiasis and prior cholecystectomy. A total of 150 patients with PAD were selected as well as a control group of 150 patients with no PAD. Data with respect to demographics, PAD size and location, ductal diameter, previous cholecystectomy and liver function tests were collected. To compare the groups, the Student's t-test and χ(2) analysis were used where appropriate.The male : female ratio was 1 : 1.2 with a median average of 71 years in the PAD group. There was no statistical difference in the CBD measurement (at the pre-ampulla and pancreatic head, and distal to confluence) between the PAD and control groups (4.8, 6.9 and 6.8 mm for the PAD group; 4.7, 6.8 and 6.4 mm for the control group; p = 0.5, 0.7 and 0.3). Also, no difference was observed in the right and left intrahepatic biliary ducts (2.7, 2.7 mm for the PAD group; 2.5, 2.6 mm for the control group; p = 0.2, 0.6). There was a significantly higher incidence of cholecystectomy history (23% vs 8.7%, p 0.01) and cholelithiasis (22% vs 11%, p 0.01) in the PAD group, and no difference in the liver function tests. Subgroup analysis of small vs large PAD (20 mm, ≥20 mm) did not show a difference in the CBD and intrahepatic biliary duct measurements. When comparing cholecystectomy vs non-cholecystectomy groups, CBD measurements were significantly higher in the cholecystectomy group.Our study confirms that PAD on its own does not lead to abnormal CBD dilatation. However, increased incidence of cholelithiasis and cholecystectomy was noted in the presence of PAD.PAD on its own does not cause CBD dilatation.

Purpose The aim of this study was to evaluate the diagnostic value of serum pancreatic isoamylases for pancreaticobiliary maljunction (PBM) with mild biliary dilatation. Methods Serum and bile from 8 children with PBM and mild biliary dilatation (6 to 11 mm in diameter) and 4 young pigs with an anastomosis constructed between an isolated pancreas-duodenal segment and the gallbladder were studied for pancreatic isoamylases. Using an electrophoretic technique, the assay of pancreatic isoamylases was expressed by peak appearance rate (PAR). Serum from 20 healthy children served as normal controls. Results In the serum of the patients, preoperatively there were 5 pancreatic peaks with PAR as follows: P1, 100%; P2, 100%; P3, 100%; P4, 100%; and P5, 66.7%. These abnormal pancreatic isoamylases disappeared 2 weeks after operative treatment. In normal controls, there were only P1 (PAR, 40%) and P2 (PAR, 100%). Mild cylindrical dilatation (6 to 8 mm in diameter) of the common bile duct developed in the porcine PBM model. There were P1 (PAR, 100%) and P2 (PAR, 100%) in the porcine serum preoperatively. Thirty days and 60 days after establishing the model, there appeared in the serum 6 pancreatic peaks with PAR as follows: P1, 100%; P2, 100%; P3, 75%; P4, 100%; P5, 100%, and P6; 75%. The bile patterns of pancreatic isoenzymes in the patients and pigs were similar to those in serum. Conclusions Abnormal pancreatic isoamylases are characteristically present in the serum from both children and a porcine model of PBM and mild biliary dilatation. Assay for these abnormalities is promising to recognize this subset of patients in whom diagnosis remains a challenge.

先天性胆道拡張症(以下,本症)の手術術式は,嚢胞切除および胆道再建術が標準術式とされている.この術式は併存する膵・胆管合流異常に対する分流手術としても合目的であり,現在,最良の手術方法と考えられている.しかし術後長期経過例の増加に伴い,再手術例の報告が散見されるようになってきた.今回本症術後20年目に発生した肝内結石症の1例を経験した.患者は21歳,女性.昭和53年,生後3カ月時に本症の診断にて胆摘,総胆管切除,総肝管空腸吻合術(ρ型)を施行された.平成12年10月頃より発熱,腹痛が頻発し,精査の結果左肝内結石症と診断され,肝左葉切除, Roux-en-Y式右肝管空腸吻合術を施行した.肝内結石症の原因は,肝内胆管に明らかな狭窄がないことにより吻合部の相対的狭窄によるものと推測された.初回手術時にはこのことを念頭に入れた胆道再建術が必要と思われた.

先天性胆道拡張症分流術後26年を経過して発症した肝内胆管癌の1例を経験したので報告する. 症例は46歳の男性で, 1975年先天性胆道拡張症戸谷分類IVa にて肝管空腸Roux-Y吻合を施行された. 2001年3月心窩部痛, 背部痛にて近医受診し, 肝腫瘤と肝門の胆管狭小を認め当院紹介入院となった. CTでS4に30mm大の腫瘤と肝十二指腸間膜周囲リンパ節腫大を認めた. ERCPで膵胆管合流異常, 遺残膵内胆管内に多数の結石を認めた.閉塞性黄疸の進行でPTCD施行し, 胆管造影で尾状葉, 右前区域, 後区域胆管根部に不整な狭窄を認めた. 血管造影で左肝動脈, 門脈左枝の狭窄を認めた. 以上より肝内胆管癌で左肝管から尾状葉, 右前区域, 後区域胆管根部および左肝動脈, 左門脈へ浸潤と診断した.拡大左葉尾状葉切除で切除可能と判断し開腹したが, 肝転移, 肝十二指腸間膜へ浸潤高度で切除不能のため胃空腸吻合術とした. 術後肝不全の進行で約1か月後死亡した.

We report a successful endoscopic stone extraction using a short type double-balloon endoscope (DBE) (EI-530 B; FUJIFILM Co, Tokyo, Japan) combined with long-type ultra-slim endoscope (L-USE) (EC-530XP; FUJIFILM Co, Tokyo, Japan) without using contrast media for intrahepatic stones in patient allergic to iodine with post-operation. A 43 year-old man with a history of congenital biliary dilatation who had undergone choledocojejunostomy and Roux-en Y reconstruction 37 years previously (Fig. 1-A) and posterior lateral hepatic segmentectomy and caudate lobectomy for intrahepatic stone 10 years previously was admitted for acute cholangitis caused by recurrence of intrahepatic stone and stenosis in choledochojejunal anastomosis (Fig. 1-B). DBE-assisted ERC was planned. This article is protected by copyright. All rights reserved.

Major biliary complications that require surgical intervention after hepaticojejunostomy are rare and technically challenging. While the hepaticojejunostomy can be refashioned in most patients requiring surgical reexploration after anastomotic dehiscence, a selected few may require a portoenterostomy, which involves anastomosis of the jejunum to a decapsulated area of the liver to establish a conduit from the intrahepatic bile ducts to the intestine. Herein, we describe the technique where a portoenterostomy has been used to restore bilioenteric continuity in three patients where reconstruction with a hepaticojejunostomy was not feasible. All patients survived the procedure and two needed percutaneous transhepatic biliary dilatation after 5 years and 6 months, respectively. One patient died of unrelated causes 12 years after the initial procedure and the other two are alive with normal bilirubin and intrahepatic ducts at 14 and 4 years. In rare cases where hepaticojejunostomy is not feasible due to small, friable or inflamed hepatic ducts, portoenterostomy with transanastomotic stenting provides an effective way of saving life and restoring bilioenteric continuity. Although this is not a procedure to be recommended without due consideration of other options, we have shown it can be life-saving and provide good long-term results in combination with postoperative radiological intervention, when necessary.

Pancreatic heterotopia is generally asymptomatic and occurs in gut-related sites. An antenatally detected subhepatic cyst was finally diagnosed to be a congenital biliary cystic dilatation (without a caudal duodenal communication) with macroscopic Heinrich Type 2 pancreatic heterotopia. The embryogenesis and pathology of the malformation are discussed.

近年, 経静脈性胆道造影 (drip infusion cholangiography) 併用下のspiral computed tomography (以下, DIC-SCT) が, 胆道系疾患の新しい非侵襲検査として普及した. そこで今回, 先天性胆道拡張症術後症例におけるDIC-SCTの有用性について検討を行った.先天性胆道拡張症の診断で, 以前に胆嚢・拡張胆管切除, 肝管腸吻合術を施行した7例を対象にDIC-SCTを行った. 手術時の年齢は, 4か月～40歳2か月で, 術後の経過観察期間は, 1年6か月～10年7か月であった.全例において, 肝内胆管1次分枝, 吻合部, および吻合部以下の腸管が描出され, 吻合部の横径は4mm～25mm, 前後径は3mm～20mmと測定できた. IV-A型の1例では, 右枝に拡張を認め, 左枝は描出されなかった.DIC-SCTは, 先天性胆道拡張症術後症例における吻合部の形態や胆汁うっ滞の有無を簡便に知る方法として優れていた.

症例は40歳の女性で, 右上腹部痛を主訴に来院した. 画像診断でAlonso-Lej I型先天性胆管拡張症と診断され, 血管造影で門脈cavernous transformationを認めた. 1995年3月1日胆管切除, 胆嚢摘出, 胆管空腸吻合術を施行した. 発達した側副門脈のため手術操作に難渋し, 約11時間を要し出血量は5,615g であった. 門脈圧は開腹時15cmH2O, 胆管切除後16cmH2Oと変化しなかった. 術後血管造影では小網内, 後腹膜の海綿状血管像が温存されていた. 患者は3年10か月間健在である. 門脈cavernous transformationを伴う先天性胆管拡張症はまれな病態で, 本邦報告例はわずか3例にすぎず, 胆管切除例は自験例が最初である. この病態では, 本来良性疾患である先天性胆管拡張症の手術リスクが高く, 胆管切除により生体に有益な門脈側副血行路の一部が失われるという不利益があるため, 手術適応や術式選択を慎重に決定すべきである.

Twenty-three cases of congenital biliary dilatation in children experienced at the hospital in a recent two decade were didided into two groups by type of the common bile duct, namely, cystic type dilatation (C-type; 12 cases) and fusiform type dilatation (F-type; 11 cases). There were five male and 18 female patients. Averaged age at operation was 2.8, ranging from 2 months to 10 years old. Items investigated included the age of onset of the disease, symptoms at the onset, blood biochemical data (white blood cell count, CRP, and total bilirubin), histopathologic types.The onset age was younger in C-type patients who manifested the disease less than one year of age. Higher total bilirubin level in serum noted in C-type cases than F-type cases. Elevated amylase level in the common bile duct was noted in F-type cases compared to the others.In C-type cases there were more pancreatico-choledochus types, and in C-type cases there were more choledoco-pancreas types in a type of pancreatico-biliary maljunction. Pathological sections of the common bile duct revealed severe chronic inflammation such as wall thickness and fibrosis in C-type cases.It is suggested that the age of onset, the presence of obstructive mechanism at the common bile duct, and type of pancreatico-biliary maljunction might be contributing factors to the type of dilatation.

OBJECTIVES To evaluate changes in the common duct diameter on sonography over time in patients with and without cholecystectomy. METHODS We retrospectively evaluated the common duct diameter, central biliary dilatation, and interval change in 1079 patients who underwent sonography at least 2 years apart over a 6-year period. A board-certified radiologist, blinded to clinical and laboratory data, measured the duct diameter. A total of 893 patients (568 female and 325 male) were divided into 3 groups: group 1, remote cholecystectomy before sonography (mean, 9.7 years before sonography; n = 117); group 2, interval cholecystectomy between the first and second sonographic examinations (n = 56); and group 3, no cholecystectomy (n = 720). All groups were stratified by age, and group 3 was also stratified by the absence (n = 528) or presence (n=192) of gallstones. RESULTS Duct diameters at baseline and follow-up averaged 4.5 and 5.2, 3.6 and 4.9, and 3.5 and 3.9 mm in groups 1, 2, and 3, respectively. Group 1 ducts were larger at baseline than in the other groups (P < .001). At follow-up, group 2 ducts showed a greater interval diameter increase than the other groups (P < .001). In a subanalysis of each group based on age, there was a mild increase in duct size with increasing age, although not clinically significant and within normal limits. In group 3 patients who never had gallstones, there was a significant small increase in duct size over decades (P < .001). The baseline duct sizes for patients with gallstones were not significantly different from those who never had gallstones (P = .15). CONCLUSIONS Patients with remote cholecystectomy have larger common duct diameters than those with no or interval cholecystectomy. Most asymptomatic patients with or without cholecystectomy have a normal common duct diameter.

The role of endoscopic retrograde cholangiopancreatography (ERCP) in the preoperative assessment of anomalous pancreaticobiliary junction was retrospectively evaluated in 74 consecutive patients (19 males and 55 females; aged 0–80 years). Sixty-three patients had congenital biliary dilatation and 11 did not. Type classification of congenital biliary dilatation was possible by ERCP alone in 45 patients (71%). The main causes of classification failure were previous bilio-enteric anastomosis and restriction of postural changes during ERCP due to general anesthesia in pediatric patients. Classification of anomalous junction was possible in 69 patients (93%). Technical difficulty in ERCP caused classification failure in 5 patients. Neoplastic lesions were found in 12 patients (16%) and all but 1 were correctly diagnosed by ERCP. We conclude that ERCP plays an important role in the preoperative diagnosis and type classification of anomalous pancreaticobiliary junction and congenital biliary dilatation.

Clinical cases of congenital biliary dilatation were divided into two groups, namely, adult group comprising of 22 patients over 15 year old and juvenile group comprising of 20 patients under 15 years old. These two groups were compared for operative procedure and frequency of postoperative cholangitis by reconstruction procedures, and the possible mechanism of onset of severe post-operative cholangitis in jejunal interposition was studied. 1) The most common reconstruction surgery was Roux-en-Y method (45%) followed by jejunal interposition (36%) in the adult group, and choledochoduodenostomy (75%) in the juvenile group. 2) Frequency of postoperative cholangitis was 27.2% in the adult or 5% in the juvenile group, the higher frequency of occurrence and more severe cases being noted in the adult group. 3) Two severe adult cases of cholangitis were al !those undergoing jejunal interposition for intrahe-patic biliary dilatation (Totani IVA). It was thought that this procedure might be imconpatible to such adult cases. 4) Scintigraphy of the bile duct and gastrointestinal tract indicated a possible mechanism of the onset of severe cholangitis after indicated a posssible mechanism of the onset of severe cholangitis after jejunal interposition that both retention of intrahepatic ble juice and bile juice in the crus of jejunal interposition might be involved.

症例は38歳の女性.8歳時に先天性胆道拡張症を指摘され手術を勧められていた.平成2年9月, 右季肋部痛, 食欲不振を主訴として来院.逆行性膵管胆管造影検査で左肝内胆管から総胆管, および膵内胆管の拡張を認め, 肝左葉内に直径2.5cmの腫瘤陰影を認めた.さらに術中, 膵内胆管にIIa+IIc様の癌 (3.8×2.2cm) を認め, 肝左葉切除兼膵頭十二指腸切除, 総胆管嚢胞切除術を施行した.術後, 切除標本の検索で肝内腫瘤はいわゆるcarcinosarcoma (Stage II) であり肝臓に微小の浸潤を認めた (hinf1, ly0, V0).膵内胆管病変は中分化型腺癌 (Stage II) で深達度はss (panc1), ly1, v0, pn1であった.その後胆嚢頸部の粘膜にも深達度ssの高分化型腺癌 (Stage I) を認めた (ly0, v0).先天性胆道拡張症に併存した肝内胆管癌と膵内胆管癌の重複癌の報告例, さらに胆嚢癌を合併した胆道系3重複癌報告例も自験例が第1例目である.