Surgical treatment of extrahepatic bile duct carcinoma

OBJECTIVE: To evaluate the experience in the diagnosis and surgical treatment of the extrahepatic bile duct carcinomas. METHODS: 242 patients with extrahepatic bile duct carcinoma over the past 20 years was retrospectively studied. RESULTS: The origin points were carcinomas of the upper bile duct in 168, of the middle bile duct in 18, and of the lower bile duct in 56 patients. The preoperative diagnostic rates for the location and the nature of the lesion were respectively raised to 97.2% and 94.5% by combination of ultrasonography and CT. The curative resection rates for the tumors in the upper, middle, and lower bile duct over the recent five years reached to 50.0%, 50.0% and 71.4%. respectively. Follow-up of patients with curative resection showed a one year recurrent rate of 73.9% and a three year recurrent rate of 100.0% with a mean recurrent time of 9.6 months in patients with local metastasis, in contrast to 13.3%, 71.4% and 17.5 months in those without metastasis. Metastasis was mainly responsible for the recurrence. Liver or multiple organ failure, intra-abdominal infection and gastrointestinal hemorrhage were the common and serious complications. CONCLUSION: The case number of the bile duct carcinoma presented a remarkable increment tendency. Ultrasonography and CT were satisfactory enough for diagnosis. To reduce the recurrent rate, resection of the tumor together with the lymph, nervous, fatty and connective tissues in the hepatic hilus, even the right celiac ganglia, should be considered the necessary procedure. Monitoring and protecting the main organs to prevent the multiple organ failure, controlling the gastrointestinal hemorrhage and the intra-abdominal infection are important to decreasing the mortality.     

Prognostic determinants in extrahepatic bile duct cancer

BACKGROUND/AIMS: The understanding of histopathological prognostic factors is critical to improving surgical outcome. This study investigated the microscopic features of cancer of the extrahepatic bile duct in order to clarify the prognostic determinants affecting surgical outcome. METHODOLOGY: In 90 cancers of the extrahepatic bile duct, the correlation between several microscopic parameters and survival was investigated. Lymphatic, venous, and perineural invasion, and the surgical margin (tumor-free or tumor-positive) were examined with serial step-wise sectioned specimens. RESULTS: Seven pT1-tumors showed no venous or perineural invasion and no lymph node involvement and were associated with prolonged survival (5 year survival, 86%) compared with pT2,3 tumors (23%). In pT2,3 tumors, lymphatic, venous, and perineural invasion was found in 80%, 47%, and 88%, respectively, with no significant differences in occurrence of these parameters according to the origin of the primary tumor. As for survival with pT2,3 tumors, lymph node involvement (58%) and status of the surgical margin were significant parameters (p=.0330 and p=.0309, respectively). In addition, these latter parameters differed significantly according to the origin of the primary tumor. CONCLUSION: In cancer of the extrahepatic bile duct, lymph node involvement and status of the surgical margin were the most important microscopic parameters affecting prognosis.     

p53 protein expression in extrahepatic bile duct cancer

p53 mutations, a tumor suppressor gene located on chromosome 17p, are the most common genetic alterations found in human cancers. Although the p53 expression or mutation has been investigated in a variety of cancers there have been very few studies in extrahepatic bile duct cancers. In this study, we investigated the immunohistochemical expression of p53 in formalin fixed paraffin embedded archival specimens of 36 extrahepatic bile duct cancers in which p53 expression was found in eighteen (50%) cases. There was no significant difference in age, gender, size of tumor, histologic grade, extent of tumor involvement, lymph node metastasis and tumor resectability according to p53 immunoreactivity. Comparison of survival duration according to p53 expression showed no significant difference. In conclusion, we reported 50 percent of p53 expression in extrahepatic bile duct cancers by immunohistochemical staining and we found no prognostic significance of p53 expression in dinicopathologic parameters.     

Assessment of tumor extent in extrahepatic bile duct cancers--utility of intraductal ultrasonography

Intraductal ultrasonography (IDUS) were performed in patients with extrahepatic bile duct cancer and compared to other diagnostic modalities and to resected specimens. Endoscopic ultrasonography (EUS) is a non-invasive diagnostic method useful for screening patients with bile duct cancers and determining whether they are resectable or not. While, EUS was not useful for the differential diagnosis of advanced and early tumors, and less useful in case of bile duct tumors located at the hilus hepatitis. IDUS proved useful without blind spot even in case of bile duct cancers at the hilus hepatis. IDUS was especially useful for the differential diagnosis of advanced and early tumors. IDUS is the very accurate diagnostic modality which make up for EUS and essential to determine the appropriate operation plan.     

Cystic dilation of extrahepatic bile ducts in adulthood: diagnosis, surgical treatment and long-term results

To evaluate the long-term results of surgery for choledohal cyst in adulthood, a series of 13 patients over the age of 16 operated on for choledochal cyst during a period of six years and followed-up for a minimum of 3 years was analyzed. Patients with type I and IVa cysts underwent extrahepatic cyst resection and Roux-en-Y hepatico-jejunostomy. Choledochoceles (type III) were managed endoscopically. No operative mortality or morbidity occurred. Type I and III cysts showed almost ideal follow-up with no sign of stricture on HIDA scan. One type IVa cyst patients developed recurrent cholangitis due to anastomotic stricture, managed percutaneously. Whenever possible, complete cyst resection and Roux-en-Y reconstruction is the treatment of choice for all extrahepatic biliary cysts. Intra- and extrahepatic dilatations are adequately treated by extrahepatic resection and careful endoscopic or radiologic surveillance. Small choledochoceles can be safely managed by endoscopic sphincterotomy.     

Intraductal ultrasonography for the diagnosis of proximal invasion in extrahepatic bile duct cancer

Intraductal ultrasonography (IDUS) was performed on 22 patients with extrahepatic bile duct cancer, using the percutaneous transhepatic approach. Intraductal ultrasonography images of the proximal invasion of the bile duct cancer were defined. In addition, three patients were examined through the peroral approach, to try to diagnose whether or not the cancer invaded to the bifurcation of the hepatic duct. Intraductal ultrasonography images obtained through the percutaneous approach could be classified into three patterns, types 1, 2 and 3, according to the features of the interior surface of the bile duct and the thickness of the bile duct wall. Type 1 images, which did not show protrusions into the bile duct lumen and had a bile duct wall of even thickness, were not likely to show bile duct cancer. Type 2 images showed protrusions of the tumour into the bile duct lumen and the surfaces of the protrusions were irregular. Type 3 images showed single or multiple low echoic papillary masses in the bile duct. Using the peroral technique, we considered all three cases to be type 1 and could diagnose that cancer had not invaded to the bifurcation of the hepatic ducts. From the results of this study, we suggest that proximal invasion of extrahepatic bile duct cancer can be diagnosed using IDUS.     

Long-term survival of a patient with bile duct carcinoma by endoscopic biliary drainage

An 80-yr-old female presented with obstructive jaundice. Endoscopic retrograde cholangiopancreatography showed a carcinoma in the middle extrahepatic bile duct, and a biliary endoprosthesis was inserted. Exfoliative cytology of the bile and forceps biopsy of the tumor revealed a papillary adenocarcinoma. Surgical resection could not be done because of her cardiovascular complications, and neither chemotherapy nor radiotherapy was administered. Stents were exchanged and cleaned 21 times because of occlusion and cholangitis. Subsequent serial cholangiogram showed a slow growth of the papillary tumor, but local invasion to the adjacent organs or distant metastasis was not observed. The patient survived for 7 yr and 6 months after insertion of the biliary endoprosthesis.     

Treatment of extrahepatic biliary atresia with interferon-alpha in a murine infectious model

The etiology of extrahepatic biliary atresia (EHBA) in newborns remains unknown, although a first infectious animal model with complete obstruction of the common bile duct could be established. Intraperitoneal inoculation of newborn Balb/c mice with rhesus rotavirus induced cholestasis, leading, in most cases, to biliary atresia with lethal outcome, similar to EHBA in human newborns. The influence of interferon-alpha (IFN-alpha) on the hepatotropism of rotavirus infection was investigated in this animal model. Single-dose therapy with 10000 IU of IFN-alpha protected all rhesus rotavirus-infected pups from cholestatic disease. The same dose, injected 5 d after infection, had no protective effect. Starting with onset of cholestatic symptoms, the treatment with 10000 IU of IFN-alpha daily showed good results in 29 mice. Seventy-six percent of the mice recovered after 1 wk of therapy. Histologic investigation revealed normal findings in the hepatobiliary tract of clinically normal mice. Twenty-one percent of the descendants of infected and prophylactic IFN-alpha-treated mice showed cholestatic symptoms after infection with rhesus rotavirus (79% in an untreated control group) and a milder form of the illness. In conclusion, we found that prophylactic treatment with IFN-alpha prevented the hepatobiliary system of newborn Balb/c mice from severe damage by rhesus rotavirus in this artificially designed infectious model for EHBA. Infected and icteric mice, treated for 1 wk with IFN-alpha, had good prospects for recovery and prevention of complete and irreversible occlusion of the extrahepatic bile ducts. Infected and prophylactic IFN-alpha-treated dams gave good protection to their descendants. This means that EHBA in this model could probably be averted by maternal antibodies against rotavirus.     

Successful treatment of a patient with octreotide-resistant necrolytic migratory erythema

We report a patient with the glucagonoma syndrome and octreotide-resistant necrolytic migratory erythema (NME). The NME responded on two occasions to an intravenous infusion of essential fatty acids (EFA) and amino acids (AA). A deficit of serum EFA prior to treatment was corrected following the infusion, whilst plasma AA were low before and after treatment. These findings indicate that NME, in the glucagonoma syndrome, may respond to correction of the EFA deficit, and that NME may be a disease of EFA deficiency.     

Long-term follow-up of a prospective randomized study of endoscopic versus surgical treatment of bile duct calculi in patients with gallbladder in situ

Eighty-three patients with bile duct calculi were entered in a prospective randomized study of endoscopic sphincterotomy (ES) and stone removal (group 1) versus surgery alone (group 2), and were followed for more than 5 years. In group 1 endoscopic stone clearance was successful in 35 of 39 patients. Thirteen patients subsequently had cholecystectomy with (n = 7) or without (n = 6) biliary symptoms and one had a cholecystostomy for acute cholecystitis. Two patients have had mild biliary colic or pancreatitis. Two patients died from gallbladder carcinoma after 9 days and 18 months. In group 2 bile duct stones were cleared surgically in 37 of 41 patients. Late complications occurred in two patients (incisional hernia and recurrent stone). One patient with gallbladder carcinoma was cured and another died after 16 months. Early major and minor complications occurred in three and four respectively of 39 patients in group 1, and in three and six respectively of 41 patients in group 2. There were no deaths. During follow-up the total morbidity rate reached 28 percent (11 of 39) and 5 percent (two of 41) (P = 0.005) and the non-biliary related mortality rate was 31 percent (12 of 39) and 10 percent (four of 41) (P = 0.02) in groups 1 and 2 respectively. Nine patients in group 1 and two in group 2 died from heart disease (P = 0.02). Total hospital stay was 2-42 (median 13) days and 6-36 (median 16) days in groups 1 and 2 respectively (P not significant). Endoscopic and surgical treatment of bile duct calculi in middle-aged and elderly patients with gallbladder in situ are equally effective in the long term. However, the significantly increased mortality rate from heart disease in patients treated endoscopically compared with those treated surgically might speak in favour of operation.     

Lithiasis of the extrahepatic bile ducts--its diagnosis and treatment

This article details the activities of the Chinese Medical Centres at Bath and Manchester, UK, both of which were set up by Sino-European Clinics Ltd (SEC) to promote traditional Chinese medicine (TCM) as a rational body of knowledge in the West. Both Centres are staffed by highly trained Chinese consultants providing complementary treatments such as acupuncture, herbs, moxibustion, and massage. In the last 5 years about 5,000 patients have been treated, many successfully. This article also discusses the growing professional interest in TCM and some significant recent developments in this field. It is the aim of this article and SEC to promote the integration of TCM and Western biomedicine in the West.     

Villous bile duct adenoma with mucinous bile

HISTORY AND ADMISSION FINDINGS: A 75-year-old woman was admitted because of colicky upper abdominal pain. Physical examination was unremarkable. INVESTIGATIONS: Alkaline phosphatase and gamma-glutamyl transpeptidase activities as well erythrocyte sedimentation rate were increased. Sonography revealed mild enlargement of the hepatocholedochal duct to 1.1 cm. Retrograde endoscopy showed a band-like immobile structure in the cystic duct. TREATMENT AND COURSE: At operation the hepatocholedochal duct was found to contain a greenish mucinous mass: no cause was discovered. Three years later a tumor, 1 x 0.5 cm, was found in the common hepatic duct at sonography, with clinical signs of recurrent chronic cholitis and early liver damage. Because of hepatic dysfunction and hypersplenism no surgical intervention was undertaken. When cholestasis progressed further, a partial full-thickness resection of the hepatic duct with removal of the tumor and the mucinous bile had to be performed two years later. Histological examination revealed a villous adenoma with slight epithelial dysplasia and mucus formation. CONCLUSIONS: Villous bile duct adenoma is a very rare cause of cholestasis. Mucous production can lead to mucinous bile, with resulting chronic cholecystitis and secondary biliary cirrhosis. The adenoma should be completely excised because of the danger of malignant transformation and risk of local recurrence.     

Successful surgical removal of a large arteriovenous malformation in a patient with hemophilia: case report

OBJECTIVE AND IMPORTANCE: This is the first reported case of the successful surgical removal of a large arteriovenous malformation (AVM) in a patient with hemophilia A. CLINICAL PRESENTATION: A 19-year-old male patient was admitted to our department with intracranial hemorrhage. He had previously been diagnosed with hemophilia A and a cerebral AVM. Carotid angiography revealed a large AVM in the right temporal and parietal lobes. The neurological and neuroradiological findings, especially those of single photon emission computed tomography, identified an area of devitalization around the lesion, which was thought to reduce the risk of new deficits resulting from surgical manipulation. INTERVENTION: We resected the AVM in conjunction with supplemental infusions of Factor VIII before, during, and after the operation. A slight cerebral hemorrhage on the 7th postoperative day was observed despite control with Factor VIII, but the patient was discharged without any new deficits. CONCLUSION: We evaluated and managed all problems of a patient with multiple complications and achieved a medical cure.     

Prognosis and treatment of bile duct carcinoma

Bile duct carcinomas present a therapeutic challenge because of different histologies, tumor locations, and resectabilities. The goal of our study was to identify prognostic factors to better delineate therapeutic options. Forty patients (30 males and 10 females) diagnosed with bile duct cancer, treated between 1985 and 1996, at Kaiser Permanente Medical Center, Los Angeles were retrospectively reviewed. Three prognostically significant variables were identified: tumor histology, tumor location, and resection. Papillary histology was the most significant determinant of long-term survival. Of six patients with papillary adenocarcinoma, four patients (67%) underwent resection, with all four achieving long-term survival. Lower-third lesions also demonstrated a survival advantage. Four out of 12 (33%) lower-third tumors were resected, with a median survival of 36 months. Irrespective of tumor histology or tumor location, tumor resection always afforded longer survival times than did palliative treatments. A prognostic classification system based on weighted values of significant variables is presented that accurately predicted long-term survival. In conclusion, bile duct tumors in general are incurable, except perhaps for a small subset of patients with papillary adenocarcinoma. Papillary histology is the most significant determinant of ultimate survival and cure. A multifunctional prognostic classification system can be helpful for this perplexing disease.     

Histologic study of biliary fibrous remnants in 48 cases of extrahepatic biliary atresia: correlation with postoperative bile flow restoration

The authors studied the transformation of infraslow oscillations of brain potentials in 15 patients with neuroses during 50 sessions of hypnosis. The results of such studies permitted to distinguish some important traits in the changes of infraslow oscillations of brain potentials in different stages of hypnosis. It is concluded that a study of these changes during hypnosis may establish some correlations between the physiological state of the brain and the unconscious mental processes.     

Intrahepatic bile duct injury and nodular regenerative hyperplasia of the liver in a patient with polyarteritis nodosa

Although involvement of the hepatic vasculature in patients with polyarteritis nodosa is not unusual, biliary manifestations are very rare. We describe a patient with polyarteritis nodosa presenting with a febrile cholestatic anicteric syndrome. Histological examination of the liver revealed necrotizing arteritis of small hepatic arteries associated with significant lesions of intrahepatic bile ducts of the sclerosing cholangitis type, i.e. fibrous collar around the ducts, periductal inflammation and ductal proliferation. Concomitant nodular regenerative hyperplasia was found, a condition which has rarely been described in association with polyarteritis nodosa. We think that hepatic arteritis compromising arterial blood flow to the liver was responsible for the most likely ischemic nature of the bile duct injury and the nodular regenerative hyperplasia seen in our patient.     

Successful treatment of cytomegalovirus encephalitis in a patient with Hodgkin's disease in remission

Cytomegalovirus encephalitis is a rare but life-threatening infection in non-AIDS patients. To our knowledge, no case that followed conventional treatment for Hodgkin's lymphoma has been reported. We present a patient with Hodgkin's disease in complete remission after combined modality treatment who was succesfully treated with a combination of ganciclovir and foscarnet.     

Night blindness secondary to vitamin A deficiency in a patient with bile duct strictures after liver transplantation

We have studied the incidence of gastro-oesophageal reflux associated with the laryngeal mask airway (LMA) in 82 paralysed patients undergoing ventilation for elective orthopaedic surgery. Anaesthesia was managed by skilled LMA users. A pH-sensitive probe was passed nasally into the oesophagus before induction and recordings made during five phases of anaesthesia. Anaesthesia was induced with propofol and fentanyl and maintained with 0.5-1.5% isoflurane and nitrous oxide in oxygen. Neuromuscular block was produced with vecuronium and the train-of-four count maintained at < or = 1. Towards the end of surgery, neuromuscular function was allowed to recover spontaneously. All LMAs were inserted at the first attempt and ventilation was successful in all patients. There were no adverse airway events. Mean oesophageal pH values during each phase of anaesthesia were: before insertion 5.88 (SD 0.77), placement 5.85 (0.74), maintenance 5.89 (0.73), emergence 5.71 (0.78) and removal 5.82 (0.75). There were no reflux events (pH < 4.0) during any phase of anaesthesia. We conclude that the incidence of gastro-oesophageal reflux is low in paralysed patients undergoing ventilation for elective orthopaedic surgery when antagonism of neuromuscular block is avoided. The validity of these findings for unskilled LMA users is unknown.