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PubMedDate not supplied

Case of bile duct carcinoma of the hepatic hilus with segmental obstructive cholangitis.

Cholangitis is a risk factor for posthepatectomy liver failure and therefore should be treated aggressively by urgent biliary drainage before definitive liver surgery for bile duct carcinoma. We present a case of bile duct carcinoma of the hepatic hilus with segmental cholangitis. The patient was a 38-year-old man. Percutaneous transhepatic biliary drainage (PTBD) was performed in the left anterior, right anterior, and right posterior segmental ducts to alleviate jaundice and to evaluate the biliary system. One month after PTBD, the patient developed high fever with leukocytosis, suggesting the onset of segmental cholangitis. An urgent repeat PTBD was carried out at the caudate and left medial segmental bile ducts. The bile juice was purulent, and the tube cholangiogram revealed miliary abscesses. After PTBD, cholangitis was subsided, and extended left hepatic lobectomy with caudate lobectomy was performed. The postoperative recovery was uneventful and the patient has been well for 13 months. We conclude that the onset of fever in patients with hilar bile duct cancer, PTBD catheters, and undrained biliary segments suggests the presence of segmental cholangitis.

Adult↗
PubMed1998

Ischemic cholangitis.

Ischemia-induced bile duct lesions have been collectively labeled as ischemic cholangitis. The biliary epithelium is dependent on arterial blood flow, unlike the hepatic parenchyma with its dual arterial and portal venous blood supply. As such, the biliary epithelium is susceptible to injury when arterial blood flow is compromised. This compromise can occur at the level of the major, named hepatic artery branches or at the microscopic, peribiliary capillary plexus level. Typically, ischemic cholangitis manifests as segmental strictures and cholangiectases with resultant mechanical impairment of bile flow and, occasionally, secondary infection of the biliary system. Ischemic cholangitis after liver transplantation is becoming an important problem and likely is attributable to numerous factors. Hepatic arterial infusion of chemotherapy and systemic vasculitis are other causes of ischemic cholangitis. The role of ischemia in other chronic biliary and ductopenic diseases remains speculative.

Antineoplastic Agents↗
PubMed1995

[Radioisomorphisms of sclerosing cholangitis].

For differentiation of Primary Sclerosing Cholangitis (PSC) from AIDS-associated cholangitis (AAC), 95 CT or Cholangiography studies of 37 patients were reviewed and signs of biliary disease analysed. Only two each of PSC and AIDS-subjects were free from biliary lesions compatible with some kind of sclerosing cholangitis. Subgroup analysis revealed wide overlapping of morphological findings in PSC and AAC. We conclude that radioisomorphism of etiologically different forms of sclerosing cholangitis may indicate pathophysiologically similar courses.

AIDS-Related Opportunistic Infections↗
PubMed1994

Surgical biliary drainage in primary sclerosing cholangitis. The role of the Hepp-Couinaud approach.

OBJECTIVE: To assess the value of bilioenteric bypass, particularly by the Hepp-Couinaud technique, in patients with primary sclerosing cholangitis and dominant strictures in the extrahepatic biliary tract. DESIGN: Prospective study and analysis of a personal series. SETTING: Academic hospital. PATIENTS: Twenty-four of 69 patients with primary sclerosing cholangitis were selected for operative intervention. In all patients both intrahepatic and extrahepatic ducts had multiple strictures. In 22 patients the major proximal extrahepatic site of stricturing was at the confluence of the right, left, and common hepatic ducts. All patients were jaundiced at some stage before operation and 19 had persistent jaundice. Five patients were cirrhotic at presentation. INTERVENTIONS: Twenty Hepp procedures were performed in 19 patients, involving a side-to-side anastomosis 2.5 to 3.5 cm wide between a Roux-en-Y loop and the right and left hepatic ducts at their confluence. No transanastomotic stents were used. MAIN OUTCOME MEASURES: Survival and relief of jaundice. RESULTS: Follow-up ranged from 20 months to 11 years; median, 6.5 years. At the time of this report, three of the five cirrhotic patients had died, and the two surviving patients had progressive liver disease. In the 16 noncirrhotic patients who underwent bypass, actuarial survival was 100% up to 8 years. Four of these patients were mildly icteric but stable 2.3 and 7 years after bypass. Eleven patients remained free of jaundice for 2.3 to 9 years after bypass. One patient died of hepatic failure 8.5 years after bypass. CONCLUSIONS: In noncirrhotic patients with primary sclerosing cholangitis and dominant extrahepatic biliary strictures, bilioenteric bypass with the Hepp technique and without transanastomotic stenting relieves jaundice durably, safely, and effectively and is associated with freedom from recurrent bouts of cholangitis. This may attenuate or halt the development of secondary biliary cirrhosis. Patients with cirrhosis derive minimal benefit from biliary bypass and should undergo liver transplantation.

Actuarial Analysis↗
PubMed2004

Glucocorticosteroids for primary sclerosing cholangitis.

BACKGROUND: Glucocorticosteroids have been suggested for primary sclerosing cholangitis, which is characterised by chronic inflammation and fibrosis in the intrahepatic and extrahepatic biliary tree. OBJECTIVES: To assess the beneficial and harmful effects of glucocorticosteroids for patients with primary sclerosing cholangitis. SEARCH STRATEGY: We searched The Cochrane Hepato-Biliary Group Controlled Trials Register, The Cochrane Central Register of Controlled Trials on The Cochrane Library, MEDLINE, EMBASE from their inception until March 2003, and reference lists. SELECTION CRITERIA: Randomised clinical trials comparing any dose or duration of glucocorticosteroids versus placebo, no intervention, or other immunosuppressive agents. We included trials irrespective of language, blinding, or publication status. DATA COLLECTION AND ANALYSIS: Both reviewers extracted data independently and assessed the methodological quality by the generation of the allocation sequence, allocation concealment, double blinding, and follow-up. The results of the meta-analyses were presented as relative risks or weighted mean difference (WMD), both with 95% confidence intervals (CI). The primary outcome measures were mortality and liver-related morbidity. MAIN RESULTS: Two randomised clinical trials were eligible for inclusion. One trial compared biliary lavage with hydrocortisone versus saline in 17 patients. A significant number of adverse events (pancreatitis; cholangitis with septicaemia; paranoid ideas; fluid retention) and no cholangiographic improvement led to termination of the trial. The other trial compared budesonide versus prednisone in 18 patients. Patients had significantly higher serum bilirubin concentration after treatment with prednisone compared with budesonide (WMD 10.4 micro mol/litre, 95% CI 1.16 to 19.64 micro mol/litre). No other significant effects on clinical or biochemical outcomes were identified for any of the evaluated interventions. REVIEWERS' CONCLUSIONS: There is no evidence to support or refute peroral glucocorticosteroids for patients with primary sclerosing cholangitis.

Anti-Inflammatory Agents↗
PubMed1987

Acute cholangitis in association with acute pancreatitis: incidence, clinical features and outcome in relation to ERCP and endoscopic sphincterotomy.

Although endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic sphincterotomy (ES) have been shown to be valuable in managing patients with acute cholangitis, their role in patients who have simultaneous acute cholangitis and acute pancreatitis is not known. We have reviewed 32 consecutive patients presenting with both conditions over ten years which represents 23.0 per cent of all cases of gallstone-related acute cholangitis and 14.4 per cent of all cases of biliary acute pancreatitis admitted during the same period. The majority of patients were elderly (median 76 years) and female (75 per cent). Five patients had previously undergone cholecystectomy. Eleven patients were clinically shocked (34 per cent) and blood cultures were positive in 9/14 cases (64 per cent). Twenty patients (63 per cent) had a predicted severe attack of acute pancreatitis (modified Glasgow criteria). Common bile duct (CBD) stones were identified in 15 of 23 patients with successful ERCP. Of these 23 patients, 9 were treated by endoscopic sphincterotomy (ES) alone, 5 by ES and surgery, 4 by surgery alone and 5 were treated conservatively. There was one death (4.3 per cent). Nine patients were managed without cholangiography; four had surgery and five were treated conservatively. There were three deaths (33 per cent; P = 0.10). Evidence of recent CBD stone passage was apparent in eight patients (25 per cent) compared with five out of eighty-seven patients (5.7 per cent) with acute cholangitis alone (P less than 0.005). The results indicate that ERCP and ES may have an important role in the management of these patients.

Acute Disease↗
PubMed1989

Primary sclerosing cholangitis: natural history, prognostic factors and survival analysis.

The natural history of primary sclerosing cholangitis was assessed in 174 patients; 37 were asymptomatic and 137 had symptoms related to underlying liver disease. At the time of diagnosis, the mean age was 39.9 years, 66% of the primary sclerosing cholangitis patients were male and 71% had associated inflammatory bowel disease, most commonly chronic ulcerative colitis. Long-term follow-up (mean: 6.0 years; range: 2.7 to 15.5 years) was available in all patients. During follow-up, 59 (34%) of the patients died: 55 in the symptomatic group and four in the asymptomatic group. Median survival from the time of diagnosis of primary sclerosing cholangitis at the Mayo Clinic was 11.9 years. Survival in the asymptomatic group was significantly decreased compared with that in a control population matched for age, race and sex. Multivariate analysis (Cox proportional hazards regression modeling) revealed that age, serum bilirubin concentration, blood hemoglobin concentration, presence or absence of inflammatory bowel disease and histologic stage on liver biopsy were independent predictors of high risk of dying. The development of a multivariate statistical survival model is a major step in identifying individual primary sclerosing cholangitis patients at low, moderate and high risk of dying. Such models will be useful for stratifying patients in therapeutic trials, in patient counseling and in patient selection and timing of liver transplantation.

Adult↗
PubMed1993

Induced expression of heat-shock protein on biliary epithelium in patients with primary sclerosing cholangitis and primary biliary cirrhosis.

In both primary sclerosing cholangitis and primary biliary cirrhosis it is supposed that immunological mechanisms are involved in the progressive destruction of the bile ducts. The aberrant expression of human leukocyte antigen-DR in the bile ducts of patients with these disorders enables the biliary epithelium to present putative antigens to the surrounding lymphocytes; however, no such antigen has been identified. Heat-shock proteins have been implicated in the pathogenesis of various immunological destructive disorders. Liver biopsy specimens from patients with primary biliary cirrhosis (n = 10) and primary sclerosing cholangitis (n = 13) were compared with those from patients with chronic hepatitis C infection (n = 5) and alcoholic cirrhosis (n = 4) and from normal controls (n = 6). Liver sections were investigated by means of immunohistochemical study using a mouse monoclonal antibody, ML30, directed against the 65-kD heat-shock protein of Mycobacterium, with monoclonal antibody against human leukocyte antigen-DR and with the monoclonal antibody Identi-Tr TCR delta 1, which recognizes a determinant on the delta-chain of the gamma/delta form of the human T-cell receptor. Human leukocyte antigen-DR expression was found on the biliary epithelium of all primary sclerosing cholangitis and primary biliary cirrhosis patients but not on bile ducts from patients with alcoholic cirrhosis or chronic hepatitis C infection or those from normal controls. The biliary epithelium reacted with ML30 in 9 of 10 primary biliary cirrhosis patients and in all primary sclerosing cholangitis patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1995

CA19-9 does not predict cholangiocarcinoma in patients with primary sclerosing cholangitis undergoing liver transplantation.

The results of liver transplantation in patients with cholangiocarcinoma have been poor. It has been suggested that elevated serum CA19-9 levels predict cholangiocarcinoma in patients with primary sclerosing cholangitis. We analyzed the predictive value of CA19-9 antigen as a marker of cholangiocarcinoma in patients with primary sclerosing cholangitis evaluated for liver transplantation. We reviewed the charts of 26 patients with primary sclerosing cholangitis (stage IV) in whom preoperative serum CA19-9 levels were determined; 22 of 26 underwent liver transplant. Explant specimens were serially sectioned and examined for tumor. In 3 of the 26 patients, cholangiocarcinoma was diagnosed during pretransplantation evaluation; exploratory laparotomy on the last patient showed no evidence of cholangiocarcinoma, and this patient is awaiting transplantation. Twelve of the 26 patients had CA19-9 levels more than double the laboratory reference range (0-37 U/mL) (mean 183.1 +/- 103 U/mL, range 77-415 U/mL). Two of the 12 patients with elevated CA19-9 levels had cholangiocarcinoma. Of the 14 patients with normal levels, two had cholangiocarcinoma. No correlation between elevated CA19-9 and bile duct dysplasia was noted. Sensitivity for serum CA19-9 levels more than twice the reference range is 50%, specificity is 54.5%, positive predictive value is 16.6%. An elevated serum CA19-9 level in a patient with stage IV primary sclerosing cholangitis does not reliably predict coexisting cholangiocarcinoma. Persistently high or rising serum CA19-9 levels do not indicate more urgent need for liver transplantation.

Adult↗
PubMed1978

Cholangiographic abnormalities in ulcerative colitis associated pericholangitis which resemble sclerosing cholangitis.

Cholangiographic abnormalities in asymptomatic patients with pericholangitis and long-standing ulcerative colitis, which resemble sclerosing cholangitis, have not been previously reported. Endoscopic retrograde cholangiography (ERC) performed in one such patient suggesting intrahepatic sclerosing cholangitis stimulated the study of seven additional patients with largely asymptomatic pericholangitis. In seven of these eight patients, ERC demonstrated abnormalities which resembled sclerosing cholangitis. These consisted of beading and strictures mainly of the intrahepatic biliary tree (IHB). In two of the eight, the common bile duct was involved. In one, this was associated with histologic progression to cirrhosis and frank cholangitic episodes even though the initial clinical presentation and hepatic histology 2 1/2 years earlier suggested only pericholangitis. We therefore conclude that bile duct abnormalities resembling sclerosing cholangitis may be demonstrated cholangiographically in patients with ulcerative colitis who present with the typical picture of pericholangitis.

Bile Ducts, Intrahepatic↗
PubMed1995

Autoimmune cholangitis: a variant of primary biliary cirrhosis. Clinicopathologic and serologic correlations in 200 cases.

The term "autoimmune cholangitis" is used for a disease with clinical and pathologic features of primary biliary cirrhosis (PBC) but with negative anti-mitochondrial antibody (AMA) and positive anti-nuclear antibody (ANA) tests. In order to characterize autoimmune cholangitis and to determine whether this truly differs from PBC, we reviewed 200 cases morphologically consistent with PBC in which data on AMA and ANA status were available to us. Of these, 64 (32%) had a negative AMA, 114 (57%) had a positive ANA, and 40 (20%) had negative AMA and positive ANA (autoimmune cholangitis). The AMA-negative group was slightly younger on average (50 vs 55 years) than AMA positives (P < 0.05). There were no significant differences in gender (15.5% male overall), hepatic histopathology, or other laboratory tests between the groups of patients with any of the 4 possible combinations of AMA and ANA. Since the only consistently distinguishing feature among these patients is the autoantibody (AMA and ANA) profile, and they otherwise have virtually identical clinical and histopathologic features, autoimmune cholangitis can be considered to be the same as AMA-negative PBC.

Adult↗
PubMed1997

Liver transplantation in patients with Caroli's disease and recurrent cholangitis.

Caroli's disease is an uncommon congenital disorder of the intrahepatic biliary tree. It is characterized by multiple and segmental dilatations of the bile ducts. The clinical course of Caroli's disease is often complicated by recurrent episodes of bacterial cholangitis that seriously impair the patient's quality of life. Despite wide spectrum antimicrobial agents, medical treatment of cholangitis is frequently unsuccessful in patients with Caroli's disease due to the persistence of bacteria in dilatated bile ducts. Other therapies, including internal or external biliary drainages and various surgical or endoscopic procedures, have been used in the treatment of Caroli's disease, with poor results. There are no previous reports in the literature of liver transplantation for recurrent cholangitis in patients with Caroli's disease. We present two such cases, in which cholangitis is resolved.

Adult↗
PubMed2000

Reappraisal of the role of the bilioenteric conduit in the pathogenesis of postoperative cholangitis.

The incidence of postoperative cholangitis has changed very little despite progressive improvement in the treatment of biliary atresia. The role of the bilioenteric conduit in its pathogenesis is still uncertain. A retrospective study of 39 patients undergoing either a conventional Kasai operation (group 1, n = 20) or with placement of an antireflux valve (group 2, n = 10) or lengthening (group 3, n = 9) of the jejunal conduit from 40 to 60 cm was performed to compare the incidence of cholangitis. Postoperative cholangitis developed in 18 of the 39 patients (46%). The incidence was 10/20 (50%) in group 1, 5/10 (50%) in group 2, and 3/9 (33%) in group 3 (P = 0.679). An animal experiment was conducted concomitantly to compare quantitative bacterial cultures of the bilioenteric anastomosis and the liver before and 1 week after Roux-en-Y hepaticojejunostomy (HPJ) in piglets without (group A, 25 cm) and with (group B, 50 cm) lengthening of the jejunal conduit in a porcine model of extrahepatic biliary obstruction. The growth of bacteria in both the bilioenteric anastomosis and the liver was not affected by lengthening the jejunal conduit from 25 to 50 cm (P = 0.612 and 0. 057, respectively), despite a geometric increase in bacterial concentrations in both groups after HPJ. It is concluded that neither bacterial growth in the liver nor cholangitis following bile-duct reconstruction was affected by valving or lengthening the bilioenteric conduit.

Age Factors↗
PubMed2003

Endoscopic sphincterotomy for cholangitis after recent coronary artery bypass graft surgery.

It is particularly attractive to perform endoscopic retrograde cholangiopancreatography (ERCP) with sphincterotomy for cholangitis due to common bile duct stone because of the increased morbidity and mortality of the alternative therapy of choledochal exploration. The safety of therapeutic ERCP after recent myocardial injury is unknown since there are only five previously reported cases. Three patients underwent therapeutic ERCP after recent coronary artery bypass graft surgery for indication of recent cholangitis due to choledochal stones. Initially, the cholangitis was managed medically in all patients. Endoscopic sphincterotomy (ES) was performed 11, 17, and 14 days after coronary artery bypass graft surgery. The calculi were successfully extracted by sweeping the choledochus with a balloon-tipped catheter or basket in all cases. During ERCP the vital signs remained stable; no cardiac arrhythmias, hemorrhage, or pulmonary complications occurred. Our study demonstrates that therapeutic ERCP is not absolutely contraindicated after recent myocardial injury and suggests that ES is preferable to surgery for cholangitis due to common bile duct stones.

Aged↗
PubMed2005

Cytomegalovirus cholangitis and pancreatitis in an immunocompetent patient.

Cholangitis and pancreatitis associated with cytomegalovirus (CMV) infection in an immunocompetent patient is reported. Endoscopic retrograde cholangiography performed on a 55-year-old man for evaluation of the cause of jaundice and liver dysfunction revealed a distal focal irregular narrowing of the common bile duct. Microscopic findings of the resected specimen showed chronic cholangitis and CMV pancreatitis. Immunohistochemistry disclosed that epithelial cells in the inflamed bile duct were positive for CMV antigen, which was compatible with CMV cholangitis. Inflammation of the biliary tract or pancreas by CMV has been commonly reported as a complication in immunocompromised patients. Our report appears to be a rare case, but suggests that CMV cholangitis or pancreatitis should be considered in the differential diagnoses of common bile duct stenosis or pancreatitis even in immunocompetent individuals.

Cholangitis↗
PubMed1992

Primary sclerosing cholangitis.

Primary sclerosing cholangitis is a condition of unknown cause. It is recognized by liver dysfunction and its characteristic radiologic appearance, which is related to portal tract inflammation, bile duct proliferation, and periductal fibroses involving small intrahepatic and large extrahepatic ducts. The disease lasts about 10 years from the time of diagnosis. Primary sclerosing cholangitis is recognized by abnormal results on routine liver function tests or by the development of clinical jaundice. An autoimmune cause has been suggested because of its strong association with inflammatory bowel disease, certain antigens, AIDS, and immunoregulatory abnormalities. Results of medical management of sclerosing cholangitis have been disappointing. Immunosuppressive drugs, copper chelating agents, and antibiotics have failed to alter progression of the disease. Colectomy in patients with inflammatory bowel disease also has no influence. The judicious use of dilations of strictures, bypass procedures, or resection can palliate jaundice in patients with primary sclerosing cholangitis, but liver transplantation is the definitive treatment. Because palliative operations increase the hazards of liver transplantation, percutaneous dilations and stentings are preferred initially. Cirrhosis and portal hypertension are indications for transplantation. In the future, transplantation may be indicated earlier in the course of the disease.

Cholangitis, Sclerosing↗
PubMed1987

Improved survival with primary sclerosing cholangitis. A review of clinicopathologic features and comparison of symptomatic and asymptomatic patients.

The clinicopathologic features and natural history of primary sclerosing cholangitis were reviewed in 53 patients followed at the Yale Liver Center during the past 30 yr. At presentation, the mean age of patients was 46 yr, and the male to female ratio was 1.4:1. Biliary sclerosis was limited to the intrahepatic ductal system in 21% of the patients. Fifty-three percent of the patients had mild disease without portal hypertension at presentation, and 25% had no symptoms attributable to their liver disease. Long-term follow-up was available for 42 patients and averaged 56 mo. Over this period, 16 patients remained mildly symptomatic, and 11 were asymptomatic. Survival was calculated by a Kaplan-Meier life-table analysis and demonstrated that 75% of the patients were alive 9 yr after the diagnosis of primary sclerosing cholangitis. A multivariate analysis of clinical features revealed that hepatomegaly and a serum bilirubin level greater than 1.5 mg/dl at the onset of disease were independent discriminators of a poor prognosis. Patients referred to this university medical center displayed different clinical characteristics than previously reported in primary sclerosing cholangitis. A higher percentage were older, female, and asymptomatic, and more had disease limited to the intrahepatic ductal system. Survival was also considerably improved in this group of patients and suggests that the long-term prognosis for patients with primary sclerosing cholangitis may be considerably better than previously believed.

Adolescent↗
PubMed1989

Papillary stenosis and sclerosing cholangitis in an immunodeficient child.

Sclerosing cholangitis, an inflammatory disease of the biliary tree that occurs infrequently in childhood, has been recognized in combination with papillary stenosis in adults with the acquired immunodeficiency syndrome. A 10-yr-old child with a familial immunodeficiency syndrome characterized by defective T-cell function and deficiencies of immunoglobulins A and G developed papillary stenosis and sclerosing cholangitis associated with cryptosporidium enteritis. The patient presented with fever, jaundice, right upper quadrant pain, and elevated serum concentrations of transaminases and alkaline phosphatase. The pain and jaundice resolved after endoscopic sphincterotomy, but the biochemical abnormalities persisted. This case demonstrates that the combination of papillary stenosis and sclerosing cholangitis can occur in children as well as adults and may be associated with immunodeficiency syndromes other than the acquired immunodeficiency syndrome. Endoscopic sphincterotomy can provide symptomatic treatment for papillary stenosis in children with this condition, although the effect of sphincterotomy on the natural history of the sclerosing cholangitis is uncertain.

Ampulla of Vater↗