The early symptoms of hilar cholangiocarcinoma are nonspecific, with abdominal pain, discomfort, anorexia, weight loss, and/or pruritus seen in about one-third of patients (7,16,35,36). Most patients come to attention because of jaundice or abnormal liver function tests. Although most patients eventually become jaundiced, this may not be present in cases of incomplete biliary obstruction (i.e., right or left hepatic duct), which may go unrecognized for months. These patients are often further evaluated and diagnosed because of an elevated alkaline phos-phatase or gamma glutamyltransferase. Pruritus may precede jaundice by some weeks, and this symptom should prompt an evaluation, especially if associated with abnormal liver function tests. Patients with papillary tumors of the hilus may give a history of intermittent jaundice. Small fragments of tumor may detach from a friable papillary tumor of the right or left hepatic duct and pass into the common hepatic duct. Physical exam findings are often nonspecific but may provide some useful information. Jaundice will usually be obvious. Patients with pruritus often have multiple excoriations of the skin. The liver may be enlarged and firm as a result of biliary tract obstruction. The gallbladder is usually decompressed and nonpalpable with hilar obstruction. Thus, a palpable gallbladder suggests a more distal obstruction or an alternative diagnosis. Rarely, patients with long-standing biliary obstruction and/or portal vein involvement may have findings consistent with portal hypertension.
In patients with cholangiocarcinoma and no previous biliary intervention, cholangitis is rare at initial presentation, despite a 30% incidence of bacterial contamination (37,38). Endoscopic or percutaneous instrumentation will significantly increase the incidence of bacterial contamination and the risk of infection. In fact, the incidence of bacterbilia is nearly 100% after endo-scopic biliary intubation, and cholangitis is more common (38). Bacterial contamination of the biliary tract in partial obstruction is not always clinically apparent. The presence of overt or subclinical infection at the time of surgery is a major source of postoperative morbidity and mortality. Escherichia coli, Klebsiella, and Enterococcal species are the most common pathogens identified. However, this spectrum of organisms may change after endoscopic or percutaneous intubation, both of which are associated with greater morbidity and mortality following surgical resection or palliative bypass for hilar cholangiocarcinoma. In an analysis of 71 patients who underwent either resection or palliative biliary bypass for proximal cholangiocarcinoma, all patients stented endoscopically and 62% of those stented percutaneously had bacterbilia. Postoperative infectious complications were doubly increased in patients stented before operation compared to nonstented patients, while noninfectious complications were equal in both groups (38). Enterococcus, Klebsiella, Streptococcus viridans, and Enterobacter aerogenes were the most common organisms, and this spectrum of bacteria must be considered when administrating perioperative antibiotics; it is imperative to take intraoperative bile specimens for culture in order to guide selection of postoperative antibiotic therapy.
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