Cases reported "Cholestasis, Intrahepatic"

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1/21. Multidisciplinary approach to palliation of obstructive jaundice caused by a central hepatocellular carcinoma.

    BACKGROUND/AIMS: Obstructive jaundice due to intraductal tumour growth is a rare symptom in association with hepatocellular carcinoma (HCC). methods: We report a 65-year-old white male who was admitted to our department with a 2-week history of progressive jaundice. At laparotomy, the liver showed advanced cirrhosis due to long-standing biliary obstruction. cholangiography confirmed total obstruction of the main bifurcation of the hepatic duct by intraductal tumour growth. Combination treatment with surgical segment III drainage, transcatheter arterial embolization and radioembolization with yttrium-90 resin particles and endoscopic stenting was performed. This form of treatment has never been reported before. RESULTS: With these combined procedures, relief of jaundice and a survival time of 32 months could be achieved. CONCLUSION: The combination of palliative methods may relieve jaundice, ensure a good quality of life and possibly prolong survival in patients with mechanical tumour obstruction of the biliary tree by HCC.
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2/21. Left hepatectomy for the choledochal cyst (type IV-A) with intrahepatic stenosis: report of a case.

    The case of a 16-year-old male with expansion of the gallbladder and dilatation of the common bile duct is reported. ultrasonography and computed tomography imaging showed expansion of the gallbladder and eminent cystic dilatation in the common bile duct and the left intrahepatic bile duct. Endoscopic retrograde cholangiopancreatography indicated expansion-like beads of the bilateral hepatic ductus and the left intrahepatic bile duct, including anomalies of the pancreaticobiliary ductal junction. Because relative stenosis of the membranous diaphragm was revealed in the porta hepatis, we diagnosed this case as a type IV-A choledochal cyst, using Todani's classification. Intraoperative cholangiography and cholangiofiberscopy showed a pinhole stricture and re-expansion of the tip of the left intrahepatic bile duct. As the narrow segment could not be expanded though we put proper pressure there, left hepatectomy was performed as a preventive measure in addition to extended biliary tract excision and cholangiojejunostomy. hepatectomy seems to be an appropriate choice in a case of intrahepatic stenosis to help increase the patient's postoperative quality of life.
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3/21. Acute pancreatitis following choledochoscopic stone extraction for hepatolithiasis.

    BACKGROUND: Hepatolithiasis is prevalent in Southeast asia and presents a difficult treatment problem. The main purposes of treatment are clearance of the stones and elimination of bile stasis. Acute pancreatitis due to migratory gall bladder stone is a well documented phenomenon. To the best of our knowledge, there is no previous report of acute pancreatitis due to intrahepatic stone. We report an intriguing case of acute pancreatitis subsequent to postoperative choledochoscopy for residual intrahepatic stone. CASE REPORT: The patient, a 56-year-old male, suffered from intermittent epigastralgia for about 2 years. Endoscopic retrograde cholangiopancreatography (ERCP) performed after admission showed incomplete contrast filling of the right intrahepatic duct. The patient underwent surgical intervention with the diagnosis of right intrahepatic stones. Postoperative T-tube cholangiography demonstrated impacted right intrahepatic stones behind the stricture. Postoperative matured T-tube tract ductal dilatation (2-4) was begun about 4 weeks after the operation. Choledochoscopy was performed after dilatation. After the third postoperative choledocoscopy for residual stone extraction, the patient complained of abdominal pain the next day. Abdominal CT showed distal common bile duct stone and acute pancreatitis. After medical treatment, patient recovered CONCLUSIONS: Extrahepatic ductal migration with resultant acute pancreatitis is possible in patients with hepatolithiasis. The awareness of such a possibility enables clinicians to promptly recognize and appropriately manage this kind of acute pancreatitis.
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4/21. Endoscopic intervention for hepatolithiasis associated with sharp angulation of right intrahepatic ducts.

    BACKGROUND: Hepatolithiasis (intrahepatic stones) is common in Asian patients. Hepatolithiasis with intrahepatic strictures and sharp ductal angulation poses a particularly difficult management problem. methods: Cases of hepatolithiasis with sharp angulation of right intrahepatic ducts were retrospectively reviewed. OBSERVATIONS: Five patients with hepatolithiasis and right sharp intrahepatic ductal angulation were treated endoscopically via ERCP. Two patients died soon after the procedure. In the remaining 3 patients, treatment by dilation of the intrahepatic strictures and stent placement was only partially successful. Attempts to access the sharply angulated intrahepatic duct were unsuccessful. CONCLUSIONS: Endoscopic management of hepatolithiasis associated with sharp angulation of a right intrahepatic duct is difficult and is generally managed best with percutaneous treatment modalities or surgery, where possible.
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5/21. Intermittent jaundice by tumor emboli from intrahepatic cholangiocarcinoma.

    Free-floating tumor debris or mucobilia as a cause of intermittent obstruction has been described infrequently. A patient with intermittent jaundice caused by tumor emboli from an intrahepatic polypoid mucinous cholangiocarcinoma is presented. Symptoms of intermittent jaundice and midepigastric pain persisted over 5 years despite an initial cholecystectomy and common bile duct exploration before definitive diagnosis and treatment of an hepatic trisegmentectomy (segments II, III, and IV). Intraductal mucin was confirmed intraoperatively and pathologically as the cause of the obstructive jaundice. The patient remains asymptomatic and without evidence of disease more than 5 years postoperatively. This report of a predominantly mucin-producing intrahepatic cholangiocarcinoma details a rare protracted clinical course of intermittent biliary obstruction from mucus emboli and highlights the possibility of long-term survival after complete resection.
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6/21. Biliary obstruction from hepatic regeneration following extended right hepatectomy for tumor.

    Two patients, aged 2 and 6 months, underwent extended right hepatectomy for hepatoblastoma. Tumor resection was complete in both and postoperative chemotherapy was begun. One patient became markedly jaundiced 4 months postoperatively. Imaging evaluation showed marked nodular liver enlargement and intrahepatic ductal dilatation. At laparotomy an obstructed distal common duct was draped over a huge regenerating liver nodule. A high Roux-En-Y choledochojejunostomy was followed by a decrease in bilirubin to normal within 4 days. Chemotherapy was restarted and the patient remains anicteric and healthy. No evidence of tumor recurrence was found. A second patient became jaundiced 2 weeks after resection. Imaging evaluation suggested tumor recurrence. At laparotomy biopsy failed to confirm recurrent tumor but the area of the porta was not explored. jaundice persisted and the patient died of liver failure thought secondary to tumor recurrence 4 months postoperation. Postmortem examination showed a completely obstructed common duct draped over a liver nodule and no evidence of tumor. These two patients illustrate the heretofore unrecorded entity of biliary obstruction from regenerating liver following resection for tumor. The sudden appearance of jaundice following a successful liver resection for tumor should raise the suspicion of benign repairable biliary obstruction rather than unresectable tumor recurrence. The definitive evaluation is reexploration rather than various imaging modalities, which may not only be misleading, but may delay life-saving intervention.
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7/21. Intrahepatic cholestasis with gallstones in two siblings: a new hepatobiliary syndrome in association with anomalous arrangement of pancreaticobiliary ducts.

    Two siblings (a 2-year-old female and 11-month-old male) with similar onset of obstructive jaundice and clinical manifestations from early infancy are described. The jaundice fluctuated but never completely disappeared. Abnormal amounts of cholate, chenodeoxycholate and ursodeoxycholate were found in serum bile acid fractions. pruritus, hyperbilirubinemia of predominantly the conjugated fraction and bilirubinuria were increased by repeated respiratory infections. ultrasonography showed several highly echogenic shadows in the gallbladder in both cases, and gallstones were found at surgery. Operative cholangiography showed an anomalous arrangement of pancreaticobiliary ductal system in both cases. The pedigree showed five relatives (including the father) on the paternal side had liver disease, and an autosomal recessive inheritance was suggested. The association of familial intrahepatic cholestasis with a large amount of serum bile acids (which seem to be due to abnormal bile acid metabolism), cholelithiasis and anomalous arrangement of the pancreaticobiliary ductal system is proposed as a new hepatobiliary syndrome.
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8/21. Liver resection for intrahepatic stones.

    Intrahepatic stones are difficult to manage, especially when they are associated with bile duct stricture, cholangitis and destruction of liver parenchyma. Suggested modes of treatment include surgical bile duct exploration, endoscopic procedures, transhepatic cholangiolithotomy and liver resection. This paper reports 2 patients in whom liver resection was performed because of intrahepatic ductal stones, bile duct strictures and repeated episodes of cholangitis. Liver resection was uncomplicated and long-term results were satisfactory. Our results support the view that liver resection is indicated in rare instances of intrahepatic bile duct stones associated with bile duct strictures.
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9/21. Benign recurrent intrahepatic cholestasis in a Chinese girl.

    A 15 year old Chinese girl presented with features of benign recurrent intrahepatic cholestasis, confirmed by liver biopsies performed during attack and remission. Her only younger brother also has features of this syndrome. Serial biochemistry monitoring during a recent attack demonstrated that a rise in the serum bile acids preceded the clinical onset of symptoms and the rise in serum bilirubin and ductal enzymes by 8 weeks. Whether this earlier rise in serum bile acids is related to the pathogenetic mechanism of this syndrome remains to be elucidated.
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10/21. Segmental biliary obstruction: false-negative diagnosis with direct cholangiography without US guidance.

    Segmental intrahepatic ductal obstruction with calculi can be a significant cause of acute cholangitis that may remain entirely undetected on a routine, "blind" direct cholangiogram. ultrasonography (US) or computed tomography can demonstrate isolated intrahepatic ductal dilatation with or without calculi. US, in particular, can be used to direct the needle puncture for percutaneous transhepatic cholangiography (PTC) and thus enable the differential diagnosis and appropriate therapy to be determined. Four cases are reported in which US-guided PTC enabled confirmation of the diagnosis of acute cholangitis secondary to segmental biliary obstruction and intrahepatic calculi. In two the correct diagnosis could not be made with initial blind direct cholangiography, and in the other two, US and US-guided PTC were performed initially.
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