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.

Dilatation of the common channel in pediatric congenital biliary dilatation remaining after radical operation.

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.

Clinical features and risk factors of bile duct perforation associated with pediatric congenital biliary dilatation.

PURPOSE: This study aimed to investigate the clinical features and risk factors of bile duct perforation in pediatric congenital biliary dilatation (CBD) patients. METHODS: 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. RESULTS: 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 < 4 years. The median duration from onset of abdominal pain to bile duct perforation was 6 (4-14) days. Age at onset [< 4 years old; P = 0.02, OR 13.9, (1.663, 115.3)], shape of extrahepatic bile duct [non-cystic type; P = 0.009, OR 8.36, (1.683, 41.5)], and dilatation of the common channel [P = 0.02, OR 13.6, (1.651, 111.5)] were risk factors of bile duct perforation. CONCLUSIONS: Emergent bile duct drainage might be planned to prevent bile duct perforation if CBD patients have the abovementioned risk factors and experience persistent abdominal pain lasting for a few days from onset.

Bile duct paucity in childhood-spectrum, profile, and outcome.

We studied the etiological spectrum, clinicolaboratory and histological profile, and outcome of infants and children under 18 years of age presenting between December 2010 and May 2016 with histological evidence of paucity of intralobular bile ducts (PILBD, bile ducts to portal tract ratio < 0.6) Post-transplant PILBD was excluded. Of 632 pediatric liver biopsies screened, 70 had PILBD-44 were infants. PILBD was classified histologically into destructive (n = 50) and non-destructive PILBD (n = 20). Presentations were jaundice (98%), organomegaly (94%), pale stools (50%), and pruritus (43%). Infants had more cholestasis but less fibrosis on histology. Overall, 29 required liver transplantation (LT) for portal hypertension (n = 26), decompensation (n = 25), growth failure (n = 20), intractable pruritus (n = 5), and recurrent cholangitis (n = 2). Destructive PILBD has an odds for poor outcome (decompensation or need for LT within 1 year) of 1.53 (95% CI = 1.15-2.04). On binary logistic regression analysis, poor outcome was related to advanced fibrosis on liver biopsy [Exp (B) = 5.46, 95% CI = 1.56-19.04]. CONCLUSION: PILBD was present in 11% of pediatric liver biopsies and has a varied etiological spectrum. Destructive PILBD has poor outcome. Need for LT is guided by the presence of advanced fibrosis. What is Known: • Natural history of syndromic ductal paucity (Alagille syndrome) is complex. • Duct loss is commonly seen with late presentation of biliary atresia. What is New: • The study classifies the etiological spectrum of ductal paucity histologically into destructive and non-destructive. • Destructive duct loss carries poor prognosis regardless of the etiology of liver disease with subsequent need for liver transplantation.

Duplication of the extrahepatic bile duct: A case report and review of the literatures.

RATIONALE: Duplication of the extrahepatic bile duct is an extremely rare congenital anomaly of the biliary system. PATIENT CONCERNS: A 44-year-old woman presented with a history of continuous upper abdominal pain and vomiting. DIAGNOSES: Magnetic resonance cholangiopancreatography (MRCP) disclosed diffuse dilatation of the intrahepatic and extrahepatic bile ducts. Endoscopic retrograde cholangiopancreatography (ERCP) showed the presence of two extrahepatic bile ducts with calculus at the distal end of the CBD. INTERVENTIONS: Laparoscopic cholecystectomy (LC) was performed after an ERCP. Choledochoscopy, performed during the operation, showed duplicated common bile duct and the cystic duct was seen opening at the right side of the extrahepatic duct. OUTCOMES: The patient was doing well after 6 months of follow-up. LESSONS: We reported a case of a double common duct with choledocholithiasis and gallstone. This rare anomaly may lead to cholangitis, common bile duct injury during surgery, malignancy occurrence, and should be treated with extreme care.

Symptomatic pancreatic duct stones in the disconnected bile duct: A case series.

BACKGROUND: Pancreaticobiliary maljunction (PBM) refers to the union of the pancreatic and biliary ducts outside of the duodenal wall. Patients are at increased risk of bile duct and gallbladder cancer, likely secondary to pancreatic juice refluxing into the biliary tree, and it is recommended that they undergo biliary diversion. METHODS: This is a case series of all patients in our institution with PBM and bilioenteric anastomosis who presented with symptomatic pancreatic duct stones in a disconnected bile duct. IRB approval was obtained prior to the initiation of the study. RESULTS: We describe eight cases of this finding. All patients underwent ERCP, with stones successfully removed from the disconnected bile duct in seven patients and from the pancreatic duct in one patient. CONCLUSION: This novel finding has not been described in the medical literature, and may become more prevalent as more patients with PBM undergo bilioenteric anastomosis.

Von Meyenburg complex and complete ductal plate malformation along with Klatskin tumour: a rare association.

Von Meyenburg complexes (VMCs), or bile duct microhamartomas, are among the constellation of defects of ductal plate malformation. These present as multiple small intrahepatic cysts and are diagnosed incidentally. Association of intrahepatic VMCs with a bile duct cancer has rarely been reported. We describe a case of a 53-year-old man presenting with obstructive jaundice. Biochemistry and radiology gave a provisional diagnosis of a resectable Klatskin tumour. The patient underwent right hepatectomy with common bile duct and caudate lobe excision. The histopathological examination demonstrated intrahepatic VMCs with complete ductal malformation and malignancy at the hilum.

Jagged1 heterozygosity in mice results in a congenital cholangiopathy which is reversed by concomitant deletion of one copy of Poglut1 (Rumi).

UNLABELLED: Haploinsufficiency for the Notch ligand JAG1 in humans results in an autosomal-dominant, multisystem disorder known as Alagille syndrome, which is characterized by a congenital cholangiopathy of variable severity. Here, we show that on a C57BL/6 background, jagged1 heterozygous mice (Jag1(+/-) ) exhibit impaired intrahepatic bile duct (IHBD) development, decreased SOX9 expression, and thinning of the periportal vascular smooth muscle cell (VSMC) layer, which are apparent at embryonic day 18 and the first postnatal week. In contrast, mice double heterozygous for Jag1 and the glycosyltransferase, Poglut1 (Rumi), start showing a significant improvement in IHBD development and VSMC differentiation during the first week. At P30, Jag1(+/-) mice show widespread ductular reactions and ductopenia in liver and a mild, but statistically, significant bilirubinemia. In contrast, P30 Jag1/Rumi double-heterozygous mice show well-developed portal triads around most portal veins, with no elevation of serum bilirubin. Conditional deletion of Rumi in VSMCs results in progressive arborization of the IHBD tree, whereas deletion of Rumi in hepatoblasts frequently results in an increase in the number of hepatic arteries without affecting bile duct formation. Nevertheless, removing one copy of Rumi from either VSMCs or hepatoblasts is sufficient to partially suppress the Jag1(+/-) bile duct defects. Finally, all Rumi target sites of the human JAG1 are efficiently glucosylated, and loss of Rumi in VSMCs results in increased levels of full-length JAG1 and a shorter fragment of JAG1 without affecting Jag1 messenger RNA levels. CONCLUSIONS: On a C57BL/6 background, Jag1 haploinsufficiency results in bile duct paucity in mice. Removing one copy of Rumi suppresses the Jag1(+/-) bile duct phenotype, indicating that Rumi opposes JAG1 function in the liver.

Usefulness and safety of endoscopic retrograde cholangiopancreatography in children with pancreaticobiliary maljunction.

BACKGROUND: To assess the diagnostic ability and safety of endoscopic retrograde cholangiopancreatography (ERCP) in in-depth preoperative examination of children patients with pancreaticobiliary maljunction (PBM). METHODS: In 63 patients with a definite diagnosis of PBM, the ability to visualize the bile and main pancreatic ducts was compared between ERCP, which was performed in 63 patients with a definite diagnosis of PBM, and magnetic resonance cholangiopancreatography (MRCP), which was performed before ERCP in 29 patients. For ERCP, its complications were also evaluated. RESULTS: The intrahepatic bile ducts could be visualized using ERCP in 44 patients (69.8%) and using MRCP in 18 (62.1%). The extrahepatic bile ducts could be visualized using ERCP in 59 patients (93.7%) and using MRCP in 29 (100%). The rates of the visualization of the main pancreatic duct and pancreaticobiliary ductal union were significantly higher in using ERCP than in using MRCP (96.8 vs. 41.4% and 90.5 vs. 37.9%, respectively; P<0.0001). As complications, hyperamylasemia developed in 12 patients (19%), but no other severe complications such as pancreatitis were observed. CONCLUSIONS: ERCP as part of an in-depth preoperative examination of children with PBM is useful and safe.

Controversies in choledochal malformation.

Choledochal malformations (some of which are choledochal cysts) may be characterised as an abnormal dilatation of the biliary tract in the absence of acute obstruction. Most appear to be of congenital origin, probably related to distal bile duct stenosis, and almost 15% can now be detected antenatally. Excision and biliary reconstruction using a Roux loop as an open operation is still the standard to compare against, although laparoscopic reconstruction is increasingly reported. This article discusses recent advances in the understanding of choledochal malformation aetiology and classification, together with the role of newer modalites of surgical treatment such as laparoscopic excision and biliary reconstruction. Although these are definitely feasible, care should be taken before dispensing with standard open techniques that have minimal complications and proven long-term benefit.

Meckel syndrome with Caroli disease and choledochal cysts.

Meckel syndrome is a lethal autosomal recessive disorder characterized by the triad of cystic renal dysplasia, occipital encephalocele, or other anomaly of the central nervous system and post-axial polydactyly. Malformation of the ductal plate is an integral component of Meckel syndrome. Ductal plate malformations include congenital hepatic fibrosis, biliary hamartoma, autosomal dominant polycystic liver disease, Caroli disease, and choledochal cyst. The occurrence of cystic hepatic disease, Caroli disease, and choledochal cyst have not been highlighted. This is a report of a 26-week fetus with features of Meckel syndrome, Caroli disease, and choledochal cyst.

Imaging features of ductal plate malformations in adults.

Ductal plate malformations, also known as fibrocystic liver diseases, are a group of congenital disorders resulting from abnormal embryogenesis of the biliary ductal system. The abnormalities include choledochal cyst, Caroli's disease and Caroli's syndrome, adult autosomal dominant polycystic liver disease, and biliary hamartoma. The hepatic lesions can be associated with renal anomalies such as autosomal recessive polycystic kidney disease (ARPKD), medullary sponge kidney, and nephronophthisis. A clear knowledge of the embryology and pathogenesis of the ductal plate is central to the understanding of the characteristic imaging appearances of these complex disorders. Accurate diagnosis of ductal plate malformations is important to direct appropriate clinical management and prevent misdiagnosis.

Congenital anomaly of low insertion of cystic duct: endoscopic retrograde cholangiopancreatography findings and clinical significance.

BACKGROUND/AIM: Low insertion of cystic duct (LICD) may be problematic during cholecystectomy. This study was performed retrospectively to assess the prevalence of LICD and identify the risk factors of stone recurrence between LICD and non-LICD (NLICD) after removal of stones. METHODS: Between January 1999 and November 2005, 3546 patients received endoscopic retrograde cholangiopancreatography examination for suspicion of biliary tract diseases. The age and sex-matched group with NLICD was enrolled to compare the clinical differences with LICD group. LICD was defined as "the orifice level of the cystic duct being below the low third of the extrahepatic duct." Recurrence was defined as "patients suffering from cholangitis or biliary stones 1 year later after the first intervention." RESULTS: Of the enrolled 3546 patients (male/female=1821/1725), 191 (5.4%) had LICD. Excluding cases of malignancy, nonbiliary stones, and incomplete data, 122 LICD patients were available. Periampullary diverticula and positive bacterial culture from bile were less common in the LICD group than the NLICD group (P=0.045; P<0.001, respectively). Lower recurrent rate of common bile duct (CBD) stones in the recurrent cases were found in the LICD group compared with the NLICD group (P=0.024; P=0.039, respectively). Univariate analysis revealed that LICD [odds ratio (OR)=0.284; P=0.032] and CBD stones (OR=4.496; P=0.006) were significantly correlated to stone recurrence. CONCLUSIONS: Our study clearly demonstrated the prevalence (5.4%) of LICD in cases with suspicion of biliary tract disease based on endoscopic retrograde cholangiopancreatography. Notably, the strongest predictors, NLICD and CBD stones, appeared to result in the higher stone recurrence.

Gene expression profiling reveals upregulated UCA1 and BMF in gallbladder epithelia of children with pancreaticobiliary maljunction.

BACKGROUND: Pancreaticobiliary maljunction is usually associated with choledochal cysts and often causes biliary carcinoma; however, the mechanism of carcinogenesis remains unknown. No study has analyzed overall changes in genetic expression beginning during childhood in gallbladder epithelia with pancreaticobiliary maljunction. PATIENTS AND METHODS: The genomewide expression of gallbladder epithelia was analyzed in 6 children with pancreaticobiliary maljunction and in 4 pediatric controls. Selected genes that were expressed differentially were further analyzed by the real-time reverse transcription-polymerase chain reaction (RT-PCR). The products of upregulated genes confirmed by real-time RT-PCR were immunohistochemically analyzed using gallbladders from 19 children with pancreaticobiliary maljunction, 5 pediatric controls, and 5 children with gallstones. RESULTS: Microarray analysis identified 188 upregulated and 160 downregulated genes. RT-PCR confirmed upregulation in 5 of 6 genes and downregulation in 1 of 5 genes, including UCA1, DUOX2, DUOXA2, ID1, BMF, and GP2. Immunohistochemistry showed a significantly higher expression of BMF in the pancreaticobiliary maljunction patients than in the controls and gallstone patients. CONCLUSIONS: This study identified several deregulated genes in the gallbladder of children with pancreaticobiliary maljunction, which may contribute to the pathophysiology. UCA1, a noncoding RNA, is an oncofetal gene, and its upregulation may be important for biliary carcinogenesis. The elevated expression of BMF may function as an apoptotic activator in proliferative gallbladder epithelia.

Congenital cholestatic syndromes: what happens when children grow up?

Although advances in the management of children with congenital cholestasis have enabled many to survive into adulthood with their native livers, even the most common of these conditions remains rare in adult hepatology practice. Among four congenital cholestatic syndromes (biliary atresia, Alagille syndrome, Caroli disease and congenital hepatic fibrosis, and progressive familial intrahepatic cholestasis), the published data on outcomes of the syndromes into adulthood suggest that a spectrum of severity of liver disease can be expected, from cirrhosis (almost universal in adults with biliary atresia who have not required liver transplantation) to mild and subclinical (eg, in the previously undiagnosed affected parent of an infant with Alagille syndrome). Complications associated with portal hypertension and nutritional deficiencies are common, and other associated features of the cholestatic syndrome may require appropriate attention, such as congenital heart disease in Alagille syndrome. Indications for liver transplantation include synthetic failure, progressive encephalopathy, intractable pruritus, recurrent biliary sepsis and recurrent complications of portal hypertension. Improved understanding of biliary physiology will hopefully translate into improved therapy for children and adults with cholestasis.