Peritoneal dialysis underscores its merits in portal hypertensionrelated and nephrogenic ascites.

In the Western world, peritoneal dialysis (PD) is less frequently applied as substitute therapy in end-stage renal disease (ESRD). In the Netherlands the use of PD has decreased from 30.3 to 13.5% due to several factors, but not due to lower PD-related outcome. The lower penetrance of PD diminishes experience with and exposure of young professionals to this treatment modality. This does not enhance a free and motivated choice among renal replacement therapies for patients who cannot be transplanted pre-emptively. To rejuvenate interest in PD and to underscore its merits, we would like to share the use of PD on two extraordinary occasions, where PD was the only way out. Ascites due to portal hypertension with profound gastrointestinal haemorrhage and nephrogenic ascites poses major management challenges in ESRD patients. In conclusion, PD came to the rescue and tremendously increased quality of life in the patients presented. To be readily available, a certain penetrance of and expertise in PD as renal replacement therapy is warranted.

Hepatopulmonary syndrome and venous emboli causing intracerebral hemorrhages after liver transplantation: a case report.

Increasing experience has fostered the acceptance of liver transplantation as a treatment for patients with hepatopulmonary syndrome. Morbidity and mortality is most commonly attributed to progressive arterial hypoxemia postoperatively. A cerebral hemorrhage has been reported in one patient with hepatopulmonary syndrome after transplantation. However, a postmortem examination of the brain was not performed and the pathogenesis or type of cerebral hemorrhage was undefined. We report on a patient with severe hepatopulmonary syndrome who developed multiple intracranial hemorrhages after transplantation. The intracerebral hemorrhages were most consistent with an embolic etiology on postmortem examination. We postulate that venous embolization, caused by the manipulation of a Swan Ganz catheter in a thrombosed central vein, resulted in pulmonary emboli that passed through dilated intrapulmonary vessels into the cerebral microcirculation. Special attention to central venous catheters and avoidance of manipulation may be warranted in subjects with severe hepatopulmonary syndrome after liver transplantation.

Results of pancreaticoduodenectomy for ampullary carcinoma and analysis of prognostic factors for survival.

BACKGROUND: Results of pancreaticoduodenectomy for ampullary carcinoma were evaluated, and prognostic factors for survival were analyzed. METHODS: During the period from 1984 to 1992 67 patients underwent subtotal or total pancreaticoduodenectomy for ampullary carcinoma. All clinicopathologic data and their influence on survival were studied. RESULTS: Subtotal pancreaticoduodenectomy was performed in 62 of 67 patients with a mortality of 6% and a morbidity of 65%; the remaining five patients underwent total pancreaticoduodenectomy. Intraabdominal infection was the most important complication. Resection margins were tumor free in 75% of 67 patients. The overall 5-year survival was 50%. Survival was significantly influenced by the involvement of resection margins. After resection with involved margins 5-year survival was 15% and 60% after resection with free margins (p < 0.001). Tumor size, lymph node involvement, and differentiation grade had limited and not significant influence on survival. CONCLUSIONS: Subtotal pancreaticoduodenectomy is the type of resection of first choice for ampullary carcinoma. Involvement of resection margins was the strongest prognostic factor for survival. Patients with a tumor size larger than 2 cm, with lymph node involvement, or with a poorly differentiated tumor still had a 5-year survival rate greater than 40%. Patients with involved margins might be candidates for studies on adjuvant therapy.

Benign fibrosing disease at the hepatic confluence mimicking Klatskin tumors.

BACKGROUND: Hilar obstructions remain a challenge with regard to diagnosis and treatment. METHODS: In the period from 1984 to 1990, 82 patients underwent resective surgery under the presumptive diagnosis of hilar cholangiocarcinoma (Klatskin tumor). The diagnosis was based on the combined appearances on direct cholangiography and ultrasonography in all cases, with the use of various other imaging modalities in some cases. RESULTS: The perioperative findings from an experienced surgical team were usually thought to be compatible with bile duct carcinoma. However, histologic examination of the resected specimens revealed benign fibrosing or localized sclerosing lesions in 11 patients (13.4%). CONCLUSIONS: The current state of diagnostic imaging fails as yet to discriminate reliably between benign and malignant hilar lesions. Whereas the immediate therapeutic consequences may be equal (resection followed by hepaticojejunostomy), the late consequences differ in a major way because benign disease has a much better prognosis. In the presence of suspicious hilar obstruction, operable lesions should not be treated by "palliative" intubational techniques and radiation therapy without a firm diagnosis of malignancy. However, overtreatment (extended liver resection, vascular reconstruction, and liver transplantation) should be avoided as well when a benign lesion has not been ruled out.

Risk factors for acquisition of hepatitis C virus infection: a case series and potential implications for disease surveillance.

BACKGROUND: Transmission of hepatitis C virus (HCV) is strongly associated with use of contaminated blood products and injection drugs. Other "non-parental" modes of transmission including sexual activity have been increasingly recognized. We examined risk factors for acquiring HCV in patients who were referred to two tertiary care centers and enrolled in an antiviral therapy protocol. METHODS: Interviews of 148 patients were conducted apart from their physician evaluation using a structured questionnaire covering demographics and risk factors for HCV acquisition. RESULTS: Risk factors (blood products, injection/intranasal drugs, razor blades/ toothbrushes, body/ear piercing, occupational exposure, sexual activity) were identified in 141 (95.3%) of participants; 23 (15.5%) had one (most frequently blood or drug exposure), 41 (27.7%) had two, and 84 (53.4%) had more than two risk factors. No patient reported sexual activity as a sole risk factor. Body piercing accounted for a high number of exposures in women. Men were more likely to have exposure to street drugs but less exposure to blood products than women. Blood product exposure was less common in younger than older HCV patients. CONCLUSION: One and often multiple risk factors could be identified in nearly all HCV-infected patients seen in a referral practice. None named sexual transmission as the sole risk factor. The development of a more complete profile of factors contributing to transmission of HCV infection may assist in clinical and preventive efforts. The recognition of the potential presence of multiple risk factors may have important implications in the approach to HCV surveillance, and particularly the use of hierarchical algorithms in the study of risk factors.

Early postoperative complications after (sub)total pancreatoduodenectomy: the AMC experience.

The postoperative course is described in 70 patients who underwent pancreatic resection for a tumor of the head of the pancreas and peri-ampullary region: 64 patients had malignant disease. Subtotal pancreatoduodenectomy was carried out in 52 patients and total pancreatoduodenectomy in 18 patients. The overall 30-day mortality was 4.3% (3 patients died: one after subtotal, 2 after total pancreatoduodenectomy). Major complications required surgical reintervention in 15 patients. Another 24 patients developed minor complications, and responded well to conservative treatment. Infective complications were the main cause of post-operative morbidity, occurring in 29 patients. Dehiscence of the pancreatico-jejunostomy required surgical reintervention in one patient. This patient died. Leakage of the pancreatico-jejunostomy was radiologically demonstrated in 10 other patients. Four of these 10 patients presented with clinical symptoms: one needed surgical intervention and 3 responded to conservative management. The results of this study confirm the present trend of decreasing mortality after pancreatoduodenectomy. Postoperative morbidity remains high. Usually, leakage of the pancreatic anastomosis was not associated with serious postoperative complications and subsided without the need of treatment in the majority of cases.

Does additive radiotherapy after hilar resection improve survival of cholangiocarcinoma? An analysis in sixty-four patients.

Between September 1983 and January 1990, 64 patients with Klatskin tumors were treated with resective therapy in the Academic Medical Centre Amsterdam. Twenty-nine patients received postoperative adjuvant radiotherapy, 22 patients were treated with resective therapy only and there were 13 postoperative deaths. The longterm results in the patients treated with or without adjuvant radiotherapy were retrospectively assessed. Three patients had a curative resection. Comparison of both therapeutic modalities demonstrated a statistically significant difference in survival (P less than 0.001) in favour of the patients who were treated with additive radiotherapy. The radiotherapy was generally well tolerated.

Hepatocellular carcinoma in the Amsterdam area. A retrospective analysis in 61 patients.

In a retrospective study clinical features of hepatocellular carcinoma (HCC) in the Amsterdam area (1984-89) were assessed in 61 cases. The data obtained were used to discuss some of the current concepts on aetiology, diagnosis, and treatment of HCC. Presenting symptoms and findings usually reflected advanced (incurable) disease. Of the patients 25% had a normal alpha-1-foetoprotein (AFP), 55% had elevated levels, and in 20% data were absent. Platelet counts greater than 500 x 10(9)/l were found in 8% and hypercalcaemia in 10% of the patients. Treatment modalities were none (70%), surgery (16%), chemotherapy (8%), radiotherapy (3%), and endoprosthesis (2%). Sixty to 70% had died after 3 months and more than 90% after 1 year. Long survivors included 2 patients with the fibrolamellar type of HCC. The low incidence of HCC in The Netherlands probably precludes cost-effective screening programs to identify resectable small HCC. Unidentified masses are malignant until proven otherwise and should be resected if no firm diagnosis of benign disease can be made. Awareness of HCC and its risk factors may lead to earlier diagnosis and more selective use of diagnostic tests.

Assessment of portal hypertension: understanding will improve treatment.

To many people it may be disappointing that many uncertainties remain with respect to the assessment of PHT. Only a few findings such as increased WHVPG and varices prove PHT. However, we have gained considerable knowledge. PHT is the consequence of a number of changes which involve the intrahepatic and extrahepatic circulation. Alcohol, viruses and drugs may disturb parenchymal architecture and cause cellular swelling, collagen and fibrin deposition and invasion with inflammatory cells. These processes finally may evolve into cirrhosis. Although in initial stages the parenchymal disturbance per se may account for PHT, increasingly impaired liver function results in metabolic changes which cause altered haemodynamics. Advanced PHT in parenchymal liver disease is the result of the complex interaction between local and systemic changes. The current techniques for the assessment of PHT are helpful for the qualitative aspects: increase in pressure can be assessed directly or indirectly; the portal venous system can be visualized even without arteriography. Gastroscopy remains a standard procedure for diagnosing PHT. Ultrasound-endoscopy is particularly helpful to confirm fundic varices and to assess changes after sclerotherapy. Increasingly, non-invasive methods to quantify PHT have become available such as the Duplex scanner. However, limitations and pitfalls need to be realized. The quantitative assessment remains (as yet?) a technique for research centres. It is obvious that the clinician in general practice can do without most of the more sophisticated techniques which have been discussed here. For the time being, PHT, and particularly variceal bleeding, is most often treated with endoscopic sclerotherapy. For that reason, only in a minority of the cases very detailed studies are required. However, the increasing knowledge opens new perspectives for the treatment and prevention of PHT on various levels. This may be a rather specific treatment of parenchymal liver disease (antivirals, d-penicillamine for Wilson's disease or venesections for haemochromatosis), drugs which may reduce local tissue damage via more general pathways (colchicine, steroids) and drugs which influence flow. Undoubtedly one of these years a more selective blocker of portal venous pressure will become available. Optimal assessment for that type of therapy makes it mandatory to master at least a few more advanced techniques. With respect to noncirrhotic PHT with causes which may vary from congenital or acquired clotting abnormalities to anatomical malformations (oesophageal web) and 'natural healthy herb tea', measures can be taken. It is clear that before treating any of the more rare causes, a proper diagnostic work-up is required.(ABSTRACT TRUNCATED AT 400 WORDS)

The imager replacing the pathologist in the diagnosis of hepatobiliary and pancreatic disease.

Advances in imaging technology, specifically cross-sectional imaging techniques (ultrasonography, computer tomography, magnetic resonance imaging), are dynamic and rapid. They have dramatically changed the management of hepatobiliary and pancreatic diseases. Although imaging is not identical to the traditional gold standard of a tissue diagnosis, it often obviates its need and provides a much better insight into clinically relevant pathology compared with a biopsy. However, this requires a thorough insight into the clinical and pathologic aspects of the disease, knowledge of limitations of imaging techniques, and insight in management implications. The clear identification of characteristic disease findings on imaging, such as a cirrhotic configuration of the liver or gallstones that match clinical findings, are most helpful. Imaging and tissue investigation often have a complementary role in patient management. Their yield is highest if they are part of a critical integration of clinical findings by a multidisciplinary team. The latter should help as much in identifying specific opportunities for treatment as preventing futile and potential harmful interventions. The contribution of imagers and pathologists to the management of patients will continue to be redefined in the new century. Noninvasive and virtual imaging will develop further. A specific and challenging role for the pathologists and clinical imagers in close cooperation with many other disciplines will be to identify sensitive molecular targets that can be used to provide noninvasive images that not only accurately provide a diagnosis, but also resolution of disease and response to specific therapy. Ann Diagn Pathol 5:57-66, 2001.

Primary sclerosing cholangitis and cholangiocarcinoma as a diagnostic and therapeutic dilemma.

Differentiating primary sclerosing cholangitis (PSC) from cholangiocarcinoma (CC) can be a diagnostic challenge with major therapeutic implications. In case of advanced or symptomatic PSC, liver transplantation (OLTx) can be life saving with excellent long-term outcome. However, the outcome of CC diagnosed prior or during OLTx is dismal. PSC is a premalignant condition associated with a risk of developing cholangio- or hepatocellular carcinoma in > 15% of patients. Imaging diagnoses should be integrated into the further clinical data. It is the sudden, rapid and irreversible deterioration of the patient's condition, and the rapid progression of cholangiographic abnormalities, which may strongly point towards a malignancy or a malignant evolution in case of PSC. Brush cytology, (guided) biopsy, and tumor markers such as Ca 19.9 and CEA levels can be of some help, but confirmation of malignancy is often associated with a poor outcome and exclusion from liver transplantation. Clinical deterioration of the PSC patient and signs indicating advanced liver damage are a justification to evaluate patients for liver transplantation. Early transplantation should be considered in appropriate patients.

Liver biopsy today. The Royal Free Hospital experience.

Experience of needle liver biopsy at the Royal Free Hospital London between 1960 and 1983 has been reviewed with regard to selection of patients, techniques, complications and indications.