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Adrenocortical carcinoma with tumor thrombus in the right hepatic vein.

Adrenocortical carcinoma is an unusual neoplasm with very poor prognosis. Patients present with an abdominal mass often exceeding 5 cm or as a functional tumor. Computed tomography is effective to demonstrate the neoplasm as an inhomogeneous adrenal lesion with irregular margins, and magnetic resonance imaging is helpful to visualize invasion into large vessels as well. Reported herein is a case of large adrenocortical carcinoma with tumor thrombus extending into the right hepatic vein.

Adrenal Cortex Neoplasms↗

Primary portal venopathy in the golden hamster treated with low doses of dimethylnitrosamine.

Ingestion of dimethylnitrosamine in a low dose and for a limited period by hamsters resulted in primary intrahepatic pylephlebitis. The process began at 24 h and progressed within 2-4 weeks to narrowing and, eventually, to partial obstruction of the portal bed. The lesions were characterized by endothelial necrosis followed by infiltrations of lymphoid cells extending into the portal tracts. Superficial lesions of terminal hepatic veins and parenchymal changes occurred only after a lapse of, respectively, 2 and 4 weeks when restrictions of portal blood had been assumed. The parenchymal changes consisted of regional regenerative hyperplasia accompanied by subendothelial prolapse of hepatocytes into the wall of terminal veins. The lesions of the portal veins persisted for a prolonged period of time after the exposure to dimethylnitrosamine had ceased. These findings, associated with characteristic histological aspects, suggested secondary immune reaction following the initial toxic pylephlebitis. The possible relevance of the experimental results to understanding the nature of endemic portal venopathy in man is discussed with regard to pathogenesis and etiology.

Animals↗

Sonography of Budd-Chiari syndrome.

OBJECTIVE: The purpose of this pictorial essay is to review the color Doppler sonographic features of Budd-Chari syndrome. CONCLUSION: Combining color and spectral data, sonography provides hemodynamic and anatomic information about vessel patency and collateral vessel formation. The diagnosis of Budd-Chari syndrome is based on the involvement of hepatic veins although intrahepatic collateral circulation and dilated caudate veins are also important and frequent signs. Half of the patients will develop regenerative nodules that can simulate hepatocellular carcinoma.

Adult↗

[A case of surgical correction of the inferior vena cava obstruction with Budd-Chiari syndrome].

A 37-year-old man is presented with Budd-Chiari syndrome associated with a membranous obstruction of the inferior vena cava. Clinical symptoms were ascites, leg edema and dilatation of the superficial abdominal veins. Aggressive surgery was carried out for this case. Midsternal incision and right subcostal oblique incision were made, and hepatic segment of the inferior vena cava was reconstructed using pericardial patch after endovenectomy and partial cardiopulmonary bypass was used without temporary interruption of liver circulation. Blood flow from the right hepatic vein was restored. Postoperative course was not eventful and this patient remains free of any evidence of ascites and liver dysfunction after operation. This procedure may be useful particularly for protecting the liver function which is damaged by hemostasis.

Adult↗

Primary living-donor liver transplantation at the University of Chicago: technical aspects of the first 104 recipients.

OBJECTIVE: To evaluate the impact of technical modifications on living-donor liver transplants in children since their introduction in 1989. SUMMARY BACKGROUND DATA: Although more than 4,000 liver transplants are performed every year in the United States, only approximately 500 are performed in children. Living-donor liver transplantation has helped to alleviate the organ shortage for small children in need of liver transplantation. Few centers have amassed a sufficient number of cases to evaluate the impact of the different techniques used in pediatric living-donor liver transplantation. METHODS: From 1989 through 1997, 104 primary living-donor liver transplants were performed at the University of Chicago. Three phases of the living-donor liver transplant program can be defined based on the techniques of vascular reconstruction: phase 1, November 1989 to November 1994 (n = 78); phase 2, November 1994 to January 1996 (n = 6); and January 1996 to present (n = 20). The patients' charts were reviewed retrospectively. The incidence and type of vascular complications and patient and graft survival rates were analyzed. RESULTS: Although the demographics of the patients have not changed during the three phases of the living-donor liver transplant program, the outcomes have improved. Without the use of conduits, the incidence of portal vein complications has significantly decreased from 44% to 8%. The incidence of hepatic artery thrombosis has decreased from 22% to 0% with the use of microvascular techniques. The combined use of both techniques has led to a significant increase in graft survival, from 74% to 94%. CONCLUSIONS: The living-donor liver transplant recipient operation has undergone significant technical changes since its introduction in 1989. These changes have decreased the vascular complications associated with this type of graft. Avoiding the use of vascular conduits and performing microvascular hepatic artery anastomoses are the critical steps in improving graft survival.

Anastomosis, Surgical↗

Preoperative visceral angiography alters intraoperative strategy during the Whipple procedure.

To determine the importance of preoperative visceral angiography prior to pancreaticoduodenectomy, all Whipple procedures performed between 1985 and 1991 at the Virginia Mason Medical Center were retrospectively reviewed. During this period, 77 pancreaticoduodenectomies were performed for both neoplastic disease (n = 54, 70%) and chronic pancreatitis (n = 23, 30%). Sixty-four preoperative angiograms were obtained, of which 39 (61%) were abnormal findings. Thirty percent (19 of 64) of the angiograms revealed a significant vascular abnormality that required specific preoperative or intraoperative measures that might not have been performed without knowledge of these findings. Examples include celiac axis revascularization for celiac occlusion, hepatic artery preservation for replaced vessels, preoperative embolization for pseudoaneurysm or arteriovenous fistula, and splenectomy for splenic vein thrombosis. Because of the high percentage of significant findings requiring an intraoperative or preoperative technical change, we recommend the use of angiography in order to diminish morbidity in all patients preparing to undergo pancreaticoduodenectomy.

Angiography↗

Correction of extrahepatic portal vein thrombosis by the mesenteric to left portal vein bypass.

OBJECTIVE: The goal of this study was to determine the effectiveness of mesenteric vein to left portal vein bypass operation (MLPVB) in correcting extrahepatic portal vein thrombosis (EHPVT) in children. The treatment of idiopathic EHPVT has been primarily palliative, whereas MLPVB restores hepatic portal flow in patients with EHPVT. METHODS: Thirty-four children with symptomatic EHPVT underwent surgery with intent to perform MLPVB and were followed for up to 7 years. MLPVB was successful in 31 patients (91%), all of whom maintain patent vein grafts and have symptomatic relief of EHPVT in follow-up. All patients had complete relief from gastrointestinal bleeding. Patients with hypersplenism had significant increases in platelet and leukocyte counts and reduction in spleen size. Superior mesenteric vein flow increased from 119 +/- 66 mL/min before bypass to 447 +/- 225 mL/min (P < 0.0001) after surgery. Postoperative blood flow in the bypass graft expressed as a fraction of calculated ideal portal flow for size correlated inversely with age (P < 0.001). Left-portal vein diameter increased from 2.6 +/- 1.6 mm to 7.3 +/- 2.4 mm 2 years after surgery (P < 0.002). Liver volume increased from 703 +/- 349 cm3 to 799 +/- 351 cm3 1 week after surgery (P < 0.001). Prothrombin time improved to normal in all patients 1 year after surgery. CONCLUSIONS: MLPVB provides excellent relief of symptoms in children with idiopathic EHPVT and results in liver growth and normalization of coagulation parameters. This surgery is corrective and should be done at as early an age as possible.

Blood Pressure↗

[Diagnostic error of thrombosis of the inferior vena cava with contrast-CT in tricuspid valve insufficiency].

Sonographic evidence of a cystic adrenal tumour was found in a 73-year-old female patient with a 6-month history of chest pain and dyspnoea. A computed tomogram of the abdomen revealed, in addition to the 5-cm lesion, a contrast medium-free area in the vena cava inferior, leading to an initial diagnosis of recurrent pulmonary emboli due to a thrombosis of the vena cava inferior. Further diagnostic procedures excluded emboli and indicated a dilating cardiomyopathy with mitral and tricuspid regurgitation. The contrast medium-free area in the vena cava inferior was caused by the influx and regurgitation of contrast medium-free blood from the renal and hepatic veins in addition to blood from the right atrium due to the tricuspid valve regurgitation. A thrombosis was excluded by means of a color-duplex investigation. The patient declined any further diagnostic procedures concerning the lesion.

Aged↗

Prognostic features and survival of hepatocellular carcinoma in Italy: impact of stage of disease.

The aim of this study was to evaluate the prognostic factors at presentation and survival in Italian patients with hepatocellular carcinoma (HCC). Clinical and demographic data of 176 patients consecutively observed from 1993 to 1997 were evaluated by univariate and multivariate analyses. Overall median survival was 18 months. At univariate analysis, low albumin, high bilirubin, high alkaline phosphatase, high alpha-fetoprotein (AFP); high platelet count, hepatitis B surface antigen (HBsAg)-positivity, the presence of ascites, of encephalopathy, of portal vein thrombosis (PVT), male sex, no treatment, poor differentiation, untreatable tumours and incidental diagnosis were each associated with shorter survival. HBsAg-positive subjects more often presented with untreatable lesions or diffuse tumours (P=0.001 and P=0.007, respectively) and had significantly worse survival (P=0.0057). By multiple regression analysis, low albumin, high bilirubin, abnormal AFP, presence of PVT and of untreatable lesions were independent risk factors for worse survival. Thus, the most important factors influencing survival are the degree of functional impairment of the liver, the presence of hepatitis B viral (HBV) infection, the type of diagnosis and the aggressiveness of the tumour.

Adult↗

[Magnetic resonance angiography in the study of abdominal neoplasms. Preliminary experience].

This study was designed to evaluate MRA imaging as a potential non-invasive method to study vascular infiltration in patients with abdominal neoplasms. Forty-three patients with abdominal tumors proven by CT and/or MRI were examined with MRA and subsequently with angiography. Of 15 cases of liver tumor, MRA allowed poor intratumoral vascularization to be demonstrated in 1 patient only, but it always provided a characteristic sign--i.e., the complete absence of flow in the hepatic segments infiltrated by the tumor and the evidence of pericapsular neovascularization. In 8 cases portal vein involvement was observed (4 cases of compression/dislocation, 2 cases of infiltration and 2 of thrombosis). In all cases MRA allowed the relationship between tumor and venous structures to be evaluated. It also demonstrated collateral vessel formations in 8 patients with cancrocirrhosis. Two cases of thrombosis and 3 infiltrations of the vena cava were demonstrated. Neither hepatic artery nor intrahepatic arterial vascularization could be correctly evaluated. Of 10 cases of renal tumors and in 3 of adrenal tumors, renal vein infiltration was seen in 4 cases and compression in 3, with only 1 false-positive finding. In these patients and in those with retroperitoneal masses, MRA provided valuable information on the relationship between tumor and vascular structures and was useful in detecting collateral vessels. In our experience, MRA is to be considered as a complementary technique to be performed after CT or MRI when additional information is needed as to the relationship between tumor and vascular structures. In the preoperative evaluation of abdominal neoplasm, angiography remains the gold standard, in spite of its invasiveness.

Abdominal Neoplasms↗

[Off label use in long-term anticoagulation after liver transplantation due to phenprocoumon-induced hepatic failure].

BACKGROUND: Drug therapy should be individualised according to criteria of efficacy, adverse effects, and treatment adherence. This is particularly important at the interface of inpatient and ambulatory care. Aspects of drug approval (labelling) and individual refunding by health care insurances should also be taken into account. CASE REPORT: A patient (male, 61, painter) showed elevated transaminases after treatment with phenprocoumon because of a deep vein thrombosis in 1999. Transaminases normalized completely after discontinuation of excluded. After a recurrent thrombosis in 2003 phenprocoumon was prescribed again followed by recurrent elevation of transaminases and subsequent cholestatic hepatitis progressing to fulminant hepatic failure that required liver transplantation. After transplantation the patient's general state of health was good and liver function nearly normal. Anticoagulation was indicated beyond the postoperative phase because of recurrent deep vein thrombosis and atrial fibrillation. A low molecular weight heparin was chosen for long-term treatment. CONCLUSION: A low molecular weight heparin appears to be the most appropriate way to maintain effective and safe anticoagulation in this patient. Coumarins carry a residual risk of an extrahepatic, immunologically mediated cross sensitization. Long-term use of ximelagatran (which has been withdrawn meanwhile) may also cause liver damage. For heparinoids, hirudines, and other drugs affecting coagulation like platelet aggregation inhibitors, therapeutic evidence is not sufficient. Though subcutaneous application of heparin is a disadvantage for the patient, therapeutic alternatives do not have better documented efficacy or less hepatotoxic potential. The low molecular weight heparin fulfils the criteria for refunding set by federal jurisdiction.

Anticoagulants↗

Venous thromboses in particular organs.

Although venous thrombosis most often occurs in the return circulation of the legs and pelvis, it may also occur in the veins of several organs and compromise venous return. Thus, the clinician, in any field will regularly be confronted with manifestations of venous thrombosis in particular organs. This review summarizes the pathogenesis and the main clinical features of venous thrombosis in the central retinal vein, the cerebral veins and sinuses of the skull, the renal and the portal veins and the hepatic and mesenteric veins as well as priapism. The principles of treatment are outlined.

Budd-Chiari Syndrome↗

Ultrasonic characteristics of periportal collateral circulation in hepatocellular carcinoma with portal vein invasion.

Real-time ultrasound (US) was used to analyze the morphological characteristics of periportal collateral circulation (PPCC) and the hepatic artery in hepatocellular carcinoma (HCC) patients with portal vein invasion (PVI). During a 5-year interval, a total of 17 HCC patients with main portal vein thrombosis and detectable periportal vessels were collected: 14 men and 3 women, aged 27 to 76 years old. We examined these patients' periportal vessels by real-time US, then differentiated PPCC from hepatic artery by duplex Doppler US. We analyzed the morphological appearances of real-time US imaging of PPCC and the hepatic artery. Our results showed that the PPCC was always torturously worm-like in appearance on real-time US, and the hepatic artery usually had a linear channel appearance on real-time US. When these two kinds of vessels were seen simultaneously along the pathway of a thrombosed portal vein, the inner vessel was always the hepatic artery with linear channel structure, and the outer vessel was always PPCC with a torturously worm-like structure. In conclusion, real-time US is a useful and reliable modality in detecting periportal vessels and differentiating PPCC from the hepatic artery.

Adult↗