A 2-year-old, spayed female, Korean domestic short-hair cat was presented with depression and vomiting. The patient had history of weight loss lasting seven months. Physical examination revealed icterus in the pinna, oral mucosa, and sclera. Based on ultrasonography and computed tomography, tentative diagnosis was extrahepatic biliary tract obstruction with acquired portosystemic shunt (PSS). Tumor or inflammation of hepatobiliary system was suspected as the cause of obstruction of the common bile duct. But it could not be determined without biopsy. The severely dilated cystic duct was considered to cause portal hypertension and secondary multiple PSS. The patient expired without histopathologic examination.
Animals ; Biliary Tract ; Biopsy ; Cats ; Child, Preschool ; Cholestasis, Extrahepatic ; Common Bile Duct ; Cystic Duct ; Depression ; Diagnosis ; Female ; Humans ; Hypertension, Portal ; Inflammation ; Jaundice ; Mouth Mucosa ; Physical Examination ; Portasystemic Shunt, Surgical ; Sclera ; Ultrasonography ; Vomiting ; Weight Loss

Inspissated bile syndrome (IBS) is a rare condition in which thick intraluminal bile, including bile plugs, sludge, or stones, blocks the extrahepatic bile ducts in an infant. A 5-week-old female infant was admitted for evaluation of jaundice and acholic stool. Diagnostic tests, including ultrasound sonography, magnetic resonance cholangiopancreatography, and a hepatobiliary scan, were not conclusive. Although the diagnosis was unclear, the clinical and laboratory findings improved gradually on administration of urodeoxycholic acid and lipid emulsion containing omega-3 polyunsaturated fatty acids (PUFAs) for 3 weeks. However, a liver biopsy was suggestive of biliary atresia. This finding forced us to perform intraoperative cholangiography, which revealed a patent common bile duct with impacted thick bile. We performed normal saline irrigation and the symptom was improved, the final diagnosis was IBS. Thus, we herein report that IBS can be treated with omega-3 PUFAs as an alternative to surgical intervention.
Bile Ducts, Extrahepatic ; Bile* ; Biliary Atresia ; Biopsy ; Cholangiography ; Cholangiopancreatography, Magnetic Resonance ; Cholestasis ; Common Bile Duct ; Diagnosis ; Diagnostic Tests, Routine ; Fatty Acids, Omega-3 ; Fatty Acids, Unsaturated* ; Female ; Humans ; Infant ; Jaundice ; Liver ; Sewage ; Ultrasonography

PURPOSE: There is still some debate on surgical procedures for hepatocellular carcinoma (HCC) patients with bile duct tumor thrombi (BDTT, Ueda type 3 or 4). What is adequate extent of liver resection for curative treatment? Is extrahepatic bile duct resection mandatory for cure? The aim of this study is to answer these questions. METHODS: Between February 1994 and December 2012, 877 consecutive HCC patients underwent hepatic resection at Ajou University Hospital. Thirty HCC patients (3.4%) with BDTT (Ueda type 3 or 4) were retrospective reviewed in this study. RESULTS: In total, 20 patients enrolled in this study were divided into 2 groups: patients who underwent hemihepatectomy with extrahepatic bile duct resection (group 1, n = 10) and with only removal of BDTT (group 2, n = 10). The 1-, 3- and 5-year overall survival rates were 75.0%, 50.0%, and 27.8%, respectively. The 1-, 3-, and 5-year survival rates of group 1 were 100.0%, 80.0%, and 45.7%, and those of group 2 were 50.0%, 20.0%, and 10.0%, respectively (P = 0.014). The 1-, 3-, and 5-year recurrences free survival rates of group 1 were 90.0%, 70.0%, and 42.0%, and those of group 2 were 36.0%, 36.0%, and 0%, respectively (P = 0.014). Thrombectomy and infiltrative growth type (Ig) were found as independent prognostic factors for recurrence free survival by multivariate analysis. Thrombectomy, Ig, and high indocyanine green retention rate at 15 minutes were found as independent prognostic factors for overall survival by multivariate analysis. CONCLUSION: We suggest that the appropriate surgical procedure for icteric HCC patients should be comprised of ipsilateral hemihepatectomy with caudate lobectomy and extrahepatic bile duct resection.
Bile Ducts* ; Bile Ducts, Extrahepatic ; Bile* ; Carcinoma, Hepatocellular* ; Cholestasis ; Hepatectomy ; Humans ; Indocyanine Green ; Liver ; Multivariate Analysis ; Recurrence ; Retrospective Studies ; Survival Rate ; Thrombectomy

BACKGROUND/AIMS: The covered self-expandable metal stent (CMS) was developed to prevent tumor ingrowth-induced stent occlusion during the treatment of malignant biliary obstruction. However, complications such as cholecystitis, pancreatitis, and stent migration can occur after the endoscopic insertion of CMSs. The aim of the present study was to assess the efficacy and safety of a double-layered CMS (DCMS) for the management of malignant bile duct obstruction. METHODS: DCMSs were endoscopically introduced into 59 patients with unresectable malignant extrahepatic biliary obstruction at four tertiary referral centers, and the patient medical records were retrospectively reviewed. RESULTS: Both the technical and functional success rates were 100%. Procedure-related complications including pancreatitis, cholangitis, stent migration, and liver abscess occurred in five patients (8.5%). The median follow-up period was 265 days (range, 31 to 752 days). Cumulative stent patency rates were 68.2% and 40.8% at 6 and 12 months, respectively. At the final follow-up, the rate of stent occlusion was 33.9% (20/59), and the median stent patency period was 276 days (range, 2 to 706 days). CONCLUSIONS: The clinical outcomes of DCMSs were comparable to the outcomes previously reported for CMSs with respect to stent patency period and complication rates.
Cholangitis ; Cholecystitis ; Cholestasis ; Cholestasis, Extrahepatic ; Common Bile Duct Neoplasms ; Feasibility Studies* ; Follow-Up Studies ; Humans ; Liver Abscess ; Medical Records ; Pancreatitis ; Retrospective Studies ; Stents* ; Tertiary Care Centers

OBJECTIVEThe aim of our study was to assess the complications of hepatic fibrosis associated with bile duct ligation and the potential curative role of sepia ink extract in hepatic damage induced by bile duct ligation.

METHODSRattus norvegicus rats were divided into 3 groups: Sham-operated group, model rats that underwent common bile duct ligation (BDL), and BDL rats treated orally with sepia ink extract (200 mg/kg body weight) for 7, 14, and 28 d after BDL.

RESULTSThere was a significant reduction in hepatic enzymes, ALP, GGT, bilirubin levels, and oxidative stress in the BDL group after treatment with sepia ink extract. Collagen deposition reduced after sepia ink extract treatment as compared to BDL groups, suggesting that the liver was repaired. Histopathological examination of liver treated with sepia ink extract showed moderate degeneration in the hepatic architecture and mild degeneration in hepatocytes as compared to BDL groups.

CONCLUSIONSepia ink extract provides a curative effect and an antioxidant capacity on BDL rats and could ameliorate the complications of liver cholestasis.

Animals ; Antioxidants ; pharmacology ; Bile Ducts ; surgery ; Biomarkers ; blood ; Cholestasis, Extrahepatic ; blood ; etiology ; prevention & control ; Collagen ; metabolism ; Ink ; Liver ; metabolism ; Liver Function Tests ; Male ; Oxidative Stress ; Rats ; Sepia ; chemistry

Jaundice is one of the poor prognostic factors in the patient with hepatocellular carcinoma (HCC). In HCC patients, the most common cause of jaundice is liver parenchymal dysfunction and jaundice due to biliary obstruction is relatively rare. However, it is clinically important because biliary obstruction can be treated effectively with biliary drainage procedure and by that quality of life and survival of the patient can be improved. It is important to identify the mechanism and location of the bile duct obstruction for an appropriate management of the biliary obstruction. Endoscopic retrograde biliary drainage (ERBD) has commonly been selected as the first-line treatment. However, percutaneous transhepatic biliary drainage or endoscopic ultrasound guided biliary drainage also can be used when the endoscopic approach is impossible or when ERBD fails. Between two types of stents - plastic or self-expandable metal, there is no definitive evidence about which one is superior. Stent type should be selected according to the characteristics of obstruction and expected survival of patient.
Carcinoma, Hepatocellular* ; Cholestasis ; Cholestasis, Extrahepatic ; Disease Management ; Drainage ; Humans ; Jaundice ; Jaundice, Obstructive* ; Liver ; Plastics ; Quality of Life ; Stents ; Ultrasonography

No abstract available.
*Cholangiopancreatography, Endoscopic Retrograde ; Cholestasis, Extrahepatic/*therapy ; Common Bile Duct/*surgery ; Duodenum/*surgery ; *Endosonography ; Female ; Humans ; Male ; Neoplasms/*complications ; Prosthesis Implantation/*methods ; *Self Expandable Metallic Stents

Extrahepatic cholangiocarcinoma is often clinically challenging to diagnose. Even multidisciplinary approaches which include computed tomography, magnetic resonance imaging, and endoscopic retrograde cholangiography are unsatisfactory in some cases, especially with biliary stricture. Percutaneous transhepatic cholangioscopy (PTCS) with its direct visualization for biopsy appears to be a promising technique for detecting cholangiocarcinoma at an early stage. We report a case of adenocarcinoma in situ of the distal common bile duct (CBD) that was confirmed by PTCS. This case suggests the useful role of PTCS in the differential diagnosis of a distal CBD obstruction, particularly when other diagnostic modalities do not provide definitive information.
Carcinoma in Situ/complications/*diagnosis/pathology/surgery ; Cholangiocarcinoma/complications/*diagnosis/pathology/surgery ; Cholangiopancreatography, Endoscopic Retrograde ; Cholestasis, Extrahepatic/diagnosis/etiology ; Common Bile Duct/*pathology/surgery ; Common Bile Duct Neoplasms/complications/*diagnosis/pathology/surgery ; Constriction, Pathologic ; *Endoscopy, Digestive System ; Humans ; Male ; Middle Aged ; Pancreaticoduodenectomy ; Predictive Value of Tests ; Tomography, X-Ray Computed ; Treatment Outcome

Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive inherited disorder characterized by multiple recurrent episodes of severe cholestatic jaundice without obstruction of extrahepatic bile duct. We present the case of a 7-year-old boy with BRIC confirmed by mutation analysis in the ATP8B1 gene and typical clinical manifestation. Despite inheritance of BRIC, we detected a mutation on only one allele. To our knowledge, this is the first report of BRIC with a confirmed single heterozygote novel mutation in the ATP8B1 gene in Korea.
Alleles ; Bile Ducts, Extrahepatic ; Child ; Cholestasis, Intrahepatic ; Heterozygote ; Humans ; Jaundice, Obstructive ; Korea ; Wills

Ultrasonography (US) is as an important tool for differentiation of obstructive and non-obstructive causes of jaundice in infants and children. Beyond two weeks of age, extrahepatic biliary atresia and neonatal hepatitis are the two most common causes of persistent neonatal jaundice; differentiation of extrahepatic biliary atresia, which requires early surgical intervention, is very important. Meticulous analysis should focus on size and configuration of the gallbladder and anatomical changes of the portahepatis. In order to narrow the differential diagnosis, combined approaches using hepatic scintigraphy, MR cholangiography, and, at times, percutaneous liver biopsy are necessary. US is useful for demonstrating choledochal cyst, bile plug syndrome, and spontaneous perforation of the extrahepatic bile duct.
Bile ; Bile Ducts, Extrahepatic ; Biliary Atresia ; Biopsy ; Child ; Cholangiography ; Choledochal Cyst ; Cholestasis ; Diagnosis, Differential ; Gallbladder ; Hepatitis ; Humans ; Infant ; Jaundice ; Liver