Improved Accuracy in the Diagnosis of Intrahepatic Bile Duct Ectasia in Caroli's Disease by Combination of Ultrasound and Endoscopic Retrograde Cholangiography

2003 ◽  
Vol 21 (05) ◽  
pp. 223-225 ◽  
Keyword(s):  
Bile Duct ◽  
Intrahepatic Bile Duct ◽  
Endoscopic Retrograde Cholangiography ◽  
Endoscopic Retrograde ◽  
Caroli's Disease ◽  
Caroli’S Disease ◽  
Improved Accuracy

scholarly journals Caroli's Disease: Current Knowledge of Its Biliary Pathogenesis Obtained from an Orthologous Rat Model

2012 ◽  
Vol 2012 ◽  
pp. 1-10 ◽  
Author(s):  
Yasunori Sato ◽  
Xiang Shan Ren ◽  
Yasuni Nakanuma
Keyword(s):  
Bile Duct ◽  
Primary Cilia ◽  
Current Knowledge ◽  
Intrahepatic Bile Duct ◽  
Fluid Secretion ◽  
Polycystic Kidney ◽  
Caroli's Disease ◽  
Caroli’S Disease ◽  
Cell Matrix ◽  
Cell Matrix Interactions

Caroli's disease belongs to a group of hepatic fibropolycystic diseases and is a hepatic manifestation of autosomal recessive polycystic kidney disease (ARPKD). It is a congenital disorder characterized by segmental saccular dilatations of the large intrahepatic bile duct and is frequently associated with congenital hepatic fibrosis (CHF). The most viable theory explaining its pathogenesis suggests that it is related to ductal plate malformation. The development of the polycystic kidney (PCK) rat, an orthologous rodent model of Caroli's disease with CHF as well as ARPKD, has allowed the molecular pathogenesis of the disease and the therapeutic options for its treatment to be examined. The relevance of the findings of studies using PCK rats and/or the cholangiocyte cell line derived from them to the pathogenesis of human Caroli's disease is currently being analyzed. Fibrocystin/polyductin, the gene product responsible for ARPKD, is normally localized to primary cilia, and defects in the fibrocystin from primary cilia are observed in PCK cholangiocytes. Ciliopathies involving PCK cholangiocytes (cholangiociliopathies) appear to be associated with decreased intracellular calcium levels and increased cAMP concentrations, causing cholangiocyte hyperproliferation, abnormal cell matrix interactions, and altered fluid secretion, which ultimately result in bile duct dilatation. This article reviews the current knowledge about the pathogenesis of Caroli's disease with CHF, particularly focusing on studies of the mechanism responsible for the biliary dysgenesis observed in PCK rats.

scholarly journals Double-balloon endoscopic retrograde cholangiography can make a reliable diagnosis and good prognosis for postoperative complications of congenital biliary dilatation

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Chiyoe Shirota ◽  
Hiroki Kawashima ◽  
Takahisa Tainaka ◽  
Wataru Sumida ◽  
Kazuki Yokota ◽  
...  
Keyword(s):  
Bile Duct ◽  
Postoperative Complications ◽  
Radical Surgery ◽  
Endoscopic Retrograde Cholangiography ◽  
Endoscopic Retrograde ◽  
Congenital Biliary Dilatation ◽  
Intrahepatic Stones ◽  
Biliary Dilatation ◽  
Double Balloon ◽  
Anastomotic Strictures

AbstractBile duct and anastomotic strictures and intrahepatic stones are common postoperative complications of congenital biliary dilatation (CBD). We performed double-balloon endoscopic retrograde cholangiography (DBERC) for diagnostic and therapeutic purposes after radical surgery. We focused on the effectiveness of DBERC for the treatment of postoperative complications of CBD patients. Bile duct and anastomotic strictures and intrahepatic stones are common postoperative complications of congenital biliary dilatation (CBD). We performed double-balloon endoscopic retrograde cholangiography (DBERC) for diagnostic and therapeutic purposes after radical surgery. We focused on the effectiveness of DBERC for the treatment of postoperative complications of CBD patients. This retrospective study included 28 patients who underwent DBERC (44 procedures) after radical surgery for CBD between January 2011 and December 2019. Strictures were diagnosed as “bile duct strictures” if endoscopy confirmed the presence of bile duct mucosa between the stenotic and anastomotic regions, and as “anastomotic strictures” if the mucosa was absent. The median patient age was 4 (range 0–67) years at the time of primary surgery for CBD and 27.5 (range 8–76) years at the time of DBERC. All anastomotic strictures could be treated with only by 1–2 courses of balloon dilatation of DBERC, while many bile duct strictures (41.2%) needed ≥ 3 treatments, especially those who underwent operative bile duct plasty as the first treatment (83.3%). Although the study was limited by the short follow-up period after DBERC treatment, DBERC is recommended as the first-line treatment for hepatolithiasis associated with biliary and anastomotic strictures in CBD patients, and it can be safely performed multiple times.

scholarly journals Three Cases of Difficult Bile Duct Stone Removal by EUS-guided Choledochoduodenostomy

2020 ◽  
Vol 25 (2) ◽  
pp. 128-134
Author(s):  
Yeong Joo Jeong ◽  
Man Ki Choi ◽  
Seung Goun Hong
Keyword(s):  
Bile Duct ◽  
Common Bile Duct ◽  
Hospital Stay ◽  
Bile Duct Stone ◽  
Intrahepatic Bile Duct ◽  
Safe Procedure ◽  
Bile Duct Stones ◽  
Metal Stent ◽  
Endoscopic Retrograde ◽  
Duration Of Hospital Stay

After failed removal of common bile duct or intrahepatic bile duct (IHD) stones by endoscopic retrograde cholangiopancreatography (ERCP), percutaneous lithotripsy is well-known as an effective procedure. However, it is time-consuming because multiple sessions of transhepatic tract dilatation are required. Endoscopic ultrasound (EUS)-guided choledochoduodenostomy (CDS) has been recently used to approach IHD to remove difficult bile duct stones. We recently experienced EUS-guided CDS performed with metal stent. Common bile duct or IHD stones were removed by retrieval accessories after initial failed or inadequate ERCP in three patients. Serious complications including bleeding, infection, and perforation were not noted. The duration of hospital stay from EUS-guided procedure to discharge ranged from 10 to 14 days. Although this result is interim and ongoing, it suggests that EUS-guided CDS might be an effective and safe procedure after failed ERCP to remove difficult bile duct stones through the tract.

Cystic Bile Duct Dilatations and Caroli’s Disease

2015 ◽  
pp. 111-127
Author(s):  
Felice Giuliante ◽  
Agostino Maria De Rose ◽  
Gennaro Nuzzo
Keyword(s):  
Bile Duct ◽  
Caroli's Disease ◽  
Caroli’S Disease

scholarly journals Role of Endoscopic Retrograde Cholangiography and Nasobiliary Drainage in the Management of Postoperative Biliary Leak

1997 ◽  
Vol 3 (4) ◽  
pp. 221-229 ◽  
Author(s):  
M. K. Goenka ◽  
R. Kochhar ◽  
D. Bhasin ◽  
B. Nagi ◽  
J. D. Wig ◽  
...  
Keyword(s):  
Bile Duct ◽  
Common Bile Duct ◽  
Endoscopic Retrograde Cholangiography ◽  
Biliary Leak ◽  
Endoscopic Retrograde ◽  
Large Defect ◽  
Endoscopic Nasobiliary Drainage ◽  
Associated Lesions ◽  
Nasobiliary Drainage

In order to assess the role of endoscopic retrograde cholangiography in evaluating the patients with post-operative biliary leak and of endoscopic nasobiliary drainage in its management, 36 patients with biliary leak seen over a period of 9 years were studied. Thirty-two had biliary leak following cholecystectomy, 3 following repair of liver trauma and 1 following choledochoduodenostomy. Patients presented at an interval of 4 days to 210 days (mean ± SEM, 32.4 ± 6.7 days) following laparotomy. Hyperbilirubinemia was noticed in only 13 patients (36.1%), while abdominal ultrasonogram showed ascites or biloma in 24 (66.7%). Endoscopic retrograde cholangiography showed the leak to involve the common bile duct in 55.6%, cystic duct in 33.3% and intrahepatic biliary radicles in 8.3%. Associated lesions included bile duct obstruction due to stricture or accidental ligature in 20%, bile duct stone in 20% and liver abscess in 2.8%.Endoscopic nasobiliary drainage using a 7 Fr pig-tail catheter was attempted in 14 patients and could be established in 12 of them. Bile duct leak sealed in all but one of these 12 patients after an interval of 3 days to 40 days (mean ± SEM, 12.2 ± 3.2 days). A single patient with large defect and a proximal bile duct stricture did not respond and required surgery. Common bile duct stones were removed by endoscopic sphincterotomy in 3 out of 4 patients. One patient with large stone required surgical choledocholithotomy. In conclusion, endoscopic retrograde cholangiography was safe and useful in confirming the presence of leak as well as its site, size and associated abnormalities. Endoscopic nasobiliary drainage proved an effective therapy in post-operative biliary leak and could avoid re-exploration in 71.4% patients.

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