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PubMed2003

The transjugular intrahepatic portosystemic stent-shunt (TIPS) as rescue therapy for complete Budd-Chiari syndrome and portal vein thrombosis.

We present a 40-year-old female patient with epigastric pain, ascites, and progressive liver failure, caused by Budd-Chiari syndrome (BCS) with thrombotic occlusion of the right and middle hepatic veins. As underlying diseases, essential thrombocythemia and resistance to activated protein C (APC) due to heterozygote factor V Leiden were found. Initial therapy with heparin caused thrombocytopenia (HIT) type II culminating in thrombosis of the last patent left hepatic vein and further deterioration of liver function. The decision against a surgical shunt and liver transplantation by our surgeons on the basis of the risks involved, prompted us to insert a transjugular intrahepatic portosystemic stent-shunt (TIPS). There was no measurable flow signal in the doppler sonography of the portal vein presumably due to thrombosis. A further evaluation with magnetic resonance tomography and angiography was impossible due to movement artefacts. TIPS initially served as a diagnostic tool allowing direct angiography-diagnosed thrombosis of the portal vein, the superior mesenteric and the splenic vein respectively. However, insertion of the TIPS shunt and subsequent fragmentation led to an effective hepatic decompression and full recanalisation of the portal vein. In the present case TIPS simultaneously allowed the diagnosis of portal vein thrombosis and served as rescue therapy of complicated Budd-Chiari syndrome. The potential development of HIT type II should be kept in mind when heparin is given, especially to patients with thrombophilia.

Activated Protein C Resistance↗
PubMed2001

[A patient with hepatocellular carcinoma and portal vein thrombosis in 1st branch who was treated by transcatheter arterial embolization].

We report a patient with hepatocellular carcinoma (HCC) with portal vein thrombosis in the 1st branch who was treated by transcatheter arterial embolization (TAE) and survived more than 3 years. A 58-year old male was diagnosed as having unresectable massive type HCC in the area of S8 with portal vein thrombosis from the P8 branch to the right portal branch. He was treated by TAE via the anterior branch of right hepatic artery. One week later, localized hepatic infarction in the anterior segment was recognized. Five months later, the portal vein thrombosis had disappeared and become necrotic. After 3 years and 4 months, he died of a relapse of a gastric varix, but with no portal thrombosis and a well controlled intra-hepatic recurrence that was treated by repeated TAE. This case suggests that TAE might be effective for cases of HCC with portal vein thrombosis in the 1st branch, if the liver function and portal flow are suitable.

Carcinoma, Hepatocellular↗
PubMed2006

Clinical features, biochemical parameters, and virological profiles of patients with hepatocellular carcinoma in Hong Kong.

BACKGROUND: Clinical features of hepatocellular carcinoma patients are changing because of screening. AIM: To examine the clinical features of hepatocellular carcinoma patients in Hong Kong and validity of different staging systems. METHODS: A total of 223 Chinese patients with hepatocellular carcinoma were studied. RESULTS: Seventy-eight percent of hepatocellular carcinoma patients had chronic hepatitis B (43% diagnosed by screening). Hepatitis B positivity, weight loss, jaundice, encephalopathy, alpha-fetoprotein level, portal vein thrombosis, extrahepatic metastasis, and treatment were shown to be independent factors affecting survival. Of chronic hepatitis B patients, hepatitis B virus DNA levels (P = 0.001) and portal vein thrombosis (P = 0.008) were independent factors affecting survival. Seventy-six percent of chronic hepatitis B patients with hepatocellular carcinoma were hepatitis B e antigen negative. Screening patients had hepatocellular carcinoma detected at an earlier stage and better survival (median survival: 21 vs. 4 months, P < 0.0001). All staging systems had good stratification of survival. Prognosis and median survival generated were different when compared with the US data. CONCLUSIONS: Chronic hepatitis B was the most common cause of hepatocellular carcinoma in Hong Kong. High-risk chronic hepatitis B patients should be followed irrespective of the hepatitis B e antigen status. Hepatitis B virus DNA levels at the time of diagnosis are an important survival predictor. Screening detected hepatocellular carcinoma at an earlier stage and prolonged survival. Staging systems should be validated in different populations.

Adult↗
PubMed1991

[Gazeous necrosis of the liver due to hepatic artery thrombosis after liver transplantation].

An emergency liver transplantation was performed in a 22 year-old female for fulminant hepatitis. The donor had had splenectomy with portal vein thrombosis which was diagnosed and removed during portoscopy. Nineteen days later, abdominal pain with shock and hepatic failure occurred. X-rays showed pneumoperitoneum and aeric images in the liver area. Laparotomy disclosed massive liver necrosis with gaz under the Glisson's capsula. The hepatic artery was thrombosed. In spite of emergency retransplantation, the patient died 8 days later, due to systemic aspergillosis. Thrombosis of hepatic artery was particular by the importance of gaz-forming infection, and emphasizes the role of rejection. The discovery of portal thrombosis allows to outline the precautions necessary in case of splenectomized donors. The severeness of aspergillosis is underscored.

Adult↗
PubMed1991

Transient porphyrinemia in a liver transplant recipient.

We report a case of acquired porphyrinemia in a liver transplant recipient. Thrombosis of the portal vein and hepatic artery led to massive necrosis of the patient's first donor liver, necessitating its removal. After an 18-hour anhepatic period, a second donor liver was transplanted. During this interval, the patient was warmed with an infrared heat lamp that emitted bright visible light. Following the anhepatic period, a photodistributed cutaneous erythema was noted. The level of total serum porphyrins during the anhepatic period was significantly elevated at 1.548 mumol/L (normal, less than 0.018 mumol/L). Fractionation showed that the levels of the hydrophobic porphyrins, coproporphyrin and protoporphyrin, were elevated, while the levels of the hydrophilic porphyrins remained normal. Following the successful second liver transplantation, the patient's porphyrin levels declined. This is the first reported case of acquired porphyrinemia in a liver transplant recipient.

Child↗
PubMed2005

Clinicopathological significance of expression of paxillin, syndecan-1 and EMMPRIN in hepatocellular carcinoma.

AIM: To evaluate the relationship of expression of paxillin, syndecan-1 and EMMPRIN proteins with clinicopathological features in hepatocellular carcinoma (HCC). METHODS: Fifty-one patients who underwent HCC resection were recruited in the study. Paxillin, syndecan-1 and EMMPRIN proteins in HCC tissues were detected with immunohistochemical staining. RESULTS: Of 51 cases of HCC, 23 (45%) exhibited paxillin protein positive expression. Of 42 cases of adjacent non-tumor liver tissues, 24 (57%) exhibited positive expression. Positive paxillin protein expression was associated with low differentiation (r = 0.406, P = 0.004), with the presence of portal vein thrombosis (r = 0.325, P = 0.021), with extra-hepatic metastasis (r = 0.346, P = 0.014). Of 51 cases of HCC, 28 (55%) exhibited syndecan-1 protein positive expression. Of 42 cases of adjacent non-tumor liver tissues, 23 (55%) exhibited positive expression. Positive snydecan-1 protein expression was associated with well differentiation (r = 0.491, P = 0.001), with no extra-hepatic metastasis (r = 0.346, P = 0.014). Of 51 cases of HCC, 28 (55%) exhibited EMMPRIN protein positive expression. Of 42 cases of adjacent non-tumor liver tissues, 21 (50%) exhibited positive expression. Expression of EMMPRIN protein was not associated with serum AFP level, HBsAg status, presence of microsatellite nodule, tumor size, presence of cirrhosis and necrosis, differentiation, presence of portal vein thrombosis, extra-hepatic metastasis, disease-free survival and overall survival (P>0.05). Expression of paxillin protein was correlated conversely with the expression of syndecan-1 protein in HCC (r = -0.366, P = 0.010). CONCLUSION: Expression of paxillin and syndecan-1 proteins in HCC may affect its invasive and metastatic ability of the tumor. There may be a converse correlation between the expression of paxillin and syndecan-1 protein in HCC. Expression of EMMPRIN protein may be detected in HCC, but it may play little role in the invasion and metastasis of HCC.

Adult↗
PubMed1995

Auxiliary liver transplantation for fulminant and subfulminant hepatic failure.

We report the first series of 9 auxiliary liver transplantations performed as a bridge to recovery in 8 patients with fulminant and subfulminant hepatic failure. Hepatic failure was due to hepatitis A virus (n = 3), hepatitis B virus (n = 1), hepatotoxic drugs (n = 2), autoimmune disease (n = 1), or it was of unknown origin (n = 1). The donor liver was reduced to a left lobe (n = 2), a left liver (n = 4), or a right liver (n = 3), and was implanted in an orthotopic position beside the native liver after it was resected by a left or a right hepatectomy. Conventional immunosuppression was used to prevent rejection. Six patients regained normal consciousness within 2 weeks, without any sequelae. Two patients had persisting encephalopathy due to graft initial dysfunction, one of whom showed portal vein thrombosis, which was successfully cleared. The other one showed hepatic vein stenosis and was retransplanted at day 15. Five of eight patients had to be reoperated because of a surgical complication. Five patients showed rapid regeneration of their native liver, but one died at day 45 from severe herpes virus broncholitis. The auxiliary grafts were removed (n = 3) or left to atrophy by tapering immunosuppression (n = 1). One patient developed cirrhosis of the native liver and died of infectious complications at day 42. The native livers of the two remaining patients are still atrophic, one at 4 months and one at 1 month posttransplant. Finally, 6 of 8 patients are alive with a follow-up of 1 to 17 months. Four of them have permanently stopped their immunosuppressive therapy. Our experience demonstrates that auxiliary orthotopic liver transplantation (1) is feasible in children and adults, using either a left or a right liver graft, (2) is efficient in providing adequate liver function, and (3) gives a real chance to the native liver to regenerate, offering these patients a future free of immunosuppression.

Adolescent↗
PubMedDate not supplied

[Portal thrombosis and liver cirrhosis. Value of ultrasound-guided puncture-biopsy of the thrombus].

OBJECTIVES AND METHODS: Portal vein thrombosis which occurs in the course of cirrhosis, associated or not with hepatocellular carcinoma, can be either cruoric or neoplastic. The aim of this study was to assess the feasibility and the results of ultrasound guided biopsy of portal vein thrombosis in 21 patients with cirrhosis (20 men and 1 woman; mean age 66 +/- 9 years), who were hospitalized between May 1989 and November 1993. Ultrasound guided biopsies of the cirrhotic liver, of the portal vein thrombosis and, when present, of a hepatic nodular lesion were performed. The diagnosis of hepatocellular carcinoma was made if the histological examination of the hepatic and portal biopsy samples were positive or if serum concentrations of alpha-fetoprotein were higher than 500 IU/mL. RESULTS: All portal vein thrombosis biopsies were successfully performed. No side-effects were observed. Before ultrasound guided biopsy of the portal thrombosis, diagnosis of hepatocellular carcinoma was made in 15 cases, and diagnosis of cirrhosis in 6 cases. After histological examination of the portal sample, hepatocellular carcinoma was diagnosed in 19 cases and cirrhosis in 2 cases. Thus, in 19% of cases, ultrasound guided biopsy of the portal thrombus provided a definitive diagnosis. CONCLUSION: Ultrasound guided biopsy of the portal vein thrombosis must be performed before liver transplantation for cirrhosis or hepatocellular carcinoma to exclude the presence of a vascular neoplastic extension.

Adult↗
PubMed2003

Laparoscopic radiofrequency ablation of the liver in proximity to major vasculature: effect of the Pringle maneuver.

BACKGROUND AND OBJECTIVES: The Pringle maneuver has been shown to increase ablation size during radiofrequency ablation (RFA). Efficacy of laparoscopic Pringle in proximity to major vasculature has not been well described. Laparoscopic RFA was performed in proximity to major hepatic vessels to examine effects of the Pringle on ablation size and vascular damage. METHODS: Laparoscopic RFA was performed in 10 pigs. Each underwent ablation of a peripheral site, and sites adjacent to the portal and hepatic veins. Ultrasound was used to position the RFA adjacent to vascular structures. US flow characteristics verified occlusion of blood flow. Five pigs underwent laparoscopic RFA with Pringle and five underwent laparoscopic RFA alone. Animals were then sacrificed for gross and microscopic evaluation. RESULTS: Peripheral, hepatic, and portal vein ablations showed no significant differences in volume between non-Pringle and Pringle lesions, though the median ablation volume for the peripheral site in the Pringle group was approximately twice that of the non-Pringle group. Pringle group overall median time to target temperature was significantly shorter (P = 0.047). Histologic examination revealed no evidence of endothelial damage or thermal-induced intravascular thrombosis of the hepatic or portal veins. CONCLUSIONS: Laparoscopic RFA with Pringle in proximity to major vascular structures does not significantly increase ablation size, or cause acute vascular damage. Further studies are necessary to determine the utility of the Pringle in proximity to major intrahepatic blood vessels.

Animals↗
PubMed2005

Portal hypertension secondary to myelofibrosis: a study of three cases.

BACKGROUND: In patients with idiopathic myelofibrosis (IM), portal hypertension (PHT) without thrombosis of the hepatic or splenoportal veins is infrequent. OBJECTIVE: To ascertain the mechanisms responsible for the development of PHT in IM and their therapeutic implications. PATIENTS AND METHODS: Color Doppler ultrasound with portal flow quantification, hepatic hemodynamic studies, and histological examinations of the liver biopsies were performed in three IM patients with PHT in whom hepatic and splenoportal thrombosis were ruled out. RESULTS: Two patients showed sinusoidal PHT (increased hepatic venous pressure gradient), normal portal flow, and massive myeloid metaplasia of the liver. Transjugular intrahepatic portosystemic shunt (TIPS) was indicated for variceal bleeding and ascites unresponsive to medical therapy, and resulted in an adequate control of these PHT complications. At the time of TIPS placement, direct portal pressure measurement showed a marked presinusoidal component in the PHT. A third patient died as a consequence of the IM before treatment of PHT could be instituted. This patient showed an extremely increased portal flow and lesser hepatic infiltration. CONCLUSIONS: IM patients with PHT might have a marked presinusoidal component contributing to PHT that is underestimated by hepatic vein catheterization. Treatment of the complications of PHT might not differ from that of patients with cirrhosis.

Aged↗
PubMed1985

Budd-Chiari syndrome: US evaluation.

Twelve patients with proved Budd-Chiari syndrome (eight acute and four chronic cases) were examined, using real-time ultrasonography (US). In all acute cases, US study showed at least one hepatic vein with findings suggestive of the syndrome, such as stenosis, dilatation, thick wall echoes, thrombosis, abnormal course, or extrahepatic anastomosis. In chronic cases, hepatic veins were usually not visible. Modifications of liver morphology were present in all patients except those with recent onset of the disease. Caudate lobe hypertrophy was present in only six cases. US study is therefore the procedure of choice for initial diagnosis of acute Budd-Chiari syndrome. Pitfalls were the failure to detect two caval thromboses and one hepatic vein web. Cavography should still be performed systematically, but hepatic phlebography is useful in selected cases only.

Adult↗
PubMed2004

Management of vascular complications after pediatric liver transplantation.

Sixty-five children underwent liver transplantation (LTx) from March 1995 to December 2002. Cirrhosis due to biliary atresia was the main indication, and hepatic artery thrombosis (HAT) the most common vascular complication (n = 5). Other vascular problems were portal vein thrombosis and stenosis. Another patient developed hepatomegaly and ascites due to a late stenosis of the left hepatic vein anastomosis. The two cases of venous stenosis were successfully treated by percutaneous angioplasty. One graft with HAT was saved, but four children died awaiting retransplant.

Adolescent↗
PubMedDate not supplied

Dynamic helical CT as a novel technique for diagnosing hepatic perfusion disorders.

BACKGROUND/AIMS: Perfusion disorders of the liver have seldom been studied by computed tomography (CT). Recent new-generation helical CT by speeding up the scanning time proves it is possible to evaluate these disorders. The purpose of this study is to demonstrate the various patterns of hemodynamic change of the liver in both normal and diseased status by dynamic helical CT technique. METHODOLOGY: In a period of 1 year, about 1,000 patients received dynamic helical CT examination of the liver due to either clinical suspicion of liver lesions or liver lesions of unknown nature. The examination was performed with a Picker PQ 2000 CT scanner. In total, 100 cc of iodinated contrast agent was injected at a rate of 3.5 cc per second. Two sets of images were acquired at 22 seconds and 75 seconds after the initiation of the contrast injection. Different patterns of hemodynamic change were found and the etiologies and mechanisms were investigated. RESULTS: Sixty-two cases were found to have perfusion disorder of the liver. Thirty cases were associated with tumors such as hepatoma (17), hemangioma (4) and hepatic metastasis (3). The other 32 cases were non-tumor associated. The perfusion disorders appeared due to liver cirrhosis, anatomic variant, iatrogenic injury, liver abscess, etc. The mechanisms for these perfusion disorders were classified as portal vein compression or thrombosis, arterioportal shunting, hepatic artery encasement, local hyperremic change, aberrant blood supply, steal effect, hepatic venous outflow obstruction, etc. These disorders presented as subcapsular, focal nodular, wedge-shaped, segmental, lobar, or even diffuse in shape and distribution. CONCLUSIONS: Dynamic helical CT opens a new window for demonstrating and understanding various hepatic perfusion disorders which reflect the hemodynamic change of the liver in both normal and diseased conditions.

Adult↗
PubMed1995

Vascular complications in living related liver transplantation detected with intraoperative and postoperative Doppler US.

BACKGROUND/AIMS: The purpose of this study was to clarify changes in the graft hemodynamics induced by vascular complications in living related liver transplantation. METHODS: This study included 46 pediatric recipients who underwent partial liver transplantation from living related donors. The blood flow was evaluated in the portal system, the hepatic artery and the hepatic vein with serial intra- and post-operative Doppler ultrasound (US). RESULTS: In 12 patients, intraoperative Doppler US showed a decrease in portal venous inflow (< 9 ml.min-1.kg-1) toward the liver graft and could act as a guide for ligation of collaterals in seven patients, portal re-construction in two, thrombectomy in one and relief of hepatic venous outflow obstruction in two for increasing the portal venous inflow. In five patients, intraoperative Doppler US showed poor arterial inflow, i.e. dampened arterial waveforms which involved both low pulsatility index (< 0.90) and low peak-systolic velocity (< 31 cm/s). In three of them, the waveform was more pulsatile after re-anastomosis or relief from stretching of the hepatic artery. The remaining two patients developed hepatic artery thrombosis. Most of the hepatic venous outflow obstruction (four of five patients) had flat waveforms, low flow velocity (< 10 cm/s) of the hepatic vein, and poor portal inflow (flow velocity < 14 cm/s). Postoperative Doppler US showed hepatic venous outflow obstruction in three patients, hepatic artery thrombosis in three (twice in one patient), portal vein stenosis in two and portal vein thrombosis in one. These complications were successfully managed with surgical procedures in three patients, transhepatic angioplasty in three and conservative treatments in four. Six patients died of non-vascular complications. CONCLUSIONS: Serial intra- and post-operative Doppler US was a useful technique for making an early diagnosis of abnormal hemodynamics of the graft circulation. Furthermore, intraoperative Doppler US could assess reconstructed vessels objectively and would reduce the incidence of vascular complications following transplantation.

Adolescent↗
PubMed2001

Feasibility and potential benefit of maintenance endoscopic variceal ligation in patients with unresectable hepatocellular carcinoma and acute esophageal variceal hemorrhage: a controlled trial.

BACKGROUND: Patients with unresectable hepatoma and acute esophageal variceal bleeding have extremely high rates of recurrent bleeding and mortality. This controlled study evaluates the feasibility and potential benefit of maintenance endoscopic variceal ligation in these patients. METHODS: Patients with unresectable hepatoma and acute esophageal variceal bleeding underwent emergent endoscopic variceal ligation. After hemostasis, patients were randomized to undergo maintenance or esophageal variceal ligation (EVL) as necessary (demand ligation). RESULTS: Fifty-four patients underwent maintenance EVL and 55 demanded EVL. One or more subsequent EVL session could be performed in only 30 patients (55.6%) in the maintenance group (actual maintenance ligation). Logistic regression analysis found that hepatic function determines the feasibility of maintenance ligation (Child-Pugh's A+B vs. C, OR 23.00: 95% CI [5.26, 100.66]). The survival and recurrent bleeding rates were similar in both groups. A subgroup analysis of patients with Child-Pugh's A and B hepatic reserve in both the maintenance EVL group (n = 24) and demand EVL group (n = 25) was performed to assess the potential benefit of maintenance ligation. Maintenance ligation reduced the rate of recurrent bleeding compared with demand ligation (p = 0.043). Cox regression showed that portal vein thrombosis and tumors in both hepatic lobes were also factors together with EVL that determined recurrence of bleeding. Survival was similar in both groups. CONCLUSIONS: Maintenance ligation is feasible in patients with unresectable hepatoma and variceal hemorrhage if they have a good hepatic reserve. Maintenance ligation might lower the rate of recurrent bleeding in this subgroup of patients.

Acute Disease↗
PubMed1997

Fatal hepatic veno-occlusive disease in a newborn infant.

We describe a newborn infant with veno-occlusive disease (VOD) of the liver. Prior to discharge from the hospital, the newborn, who had been treated for suspected neonatal infection, suddenly developed sepsis-like symptoms. The size of the liver as well as serum activity of hepatic enzymes increased progressively. Initial Doppler-flow studies demonstrated an absent flow in the vena portae, a finding that was compatible with vena portae thrombosis or occlusion of other hepatic veins. A therapy with recombinant tissue plasminogen activator (rt-PA) was initiated; due to extensive bleedings from various sides, the fibrinolytic therapy had to be withdrawn 12 hours later, when Doppler-flow examination revealed a reverse flow in hepatofugal direction. Despite supportive therapy, the general condition of the patient deteriorated continuously, finally resulting in liver and renal failure. Our patient died 19 days after birth. The autopsy demonstrated obliterative lesions of the centrilobular and sublobular hepatic veins, the classical signs of VOD of the liver. Despite extensive diagnostics and examinations, the etiology of VOD could not been elucidated in this newborn.

Alanine Transaminase↗