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Liver transplantation in small babies.

Pediatric liver transplantation is an effective treatment for end-stage liver disease with 1- and 5-year survivals approaching 90% and 70%, respectively. Survival is influenced by the recipient's age, weight, primary disease, vascular malformations, and nutritional status. Younger patients weighing less than 13 kg are considered to be a high-risk group. The aim of this article is to evaluate the impact of this group of patients on the overall results of our pediatric liver transplant program. From January 1986 through January 1992 we performed 76 liver transplants in 59 pediatric patients. Sixteen received a second graft and a third was required in one. Fourteen patients weighed less than 13 kg (mean, 11 kg; range, 6 to 13 kg). Their mean age was 12 months, with a range of 8 to 36 months. Indications for transplantation were: biliary atresia (9), Byler's disease (1), tyrosinemia (3), and alpha 1-antitrypsin deficiency (1). The incidence of rejection in this group (52%) was not significantly different from that in other patients (61%). Ten episodes of acute rejection required only steroids: in one monoclonal antibodies were added. Five patients had a new graft implanted, four for hepatic artery thrombosis and one for primary liver nonfunction. Nine patients are alive (64%) with the follow-up time ranging from 2 to 56 months (mean, 31). Five patients died of multiorgan failure (3), portal vein thrombosis (1), and primary liver nonfunction (1). Four-year graft and patient survival rates were 47% and 64%, respectively. Small babies are a high-risk group in a pediatric liver transplant program.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Value of plasma P-selectin for vascular complications in liver transplantation.

Recent data suggest that plasma P-selectin, an adhesion molecule, may be a clinically useful marker for thrombosis. Hepatic vessel thrombosis is one of the most serious complications following liver transplantation. To assess the contribution of the soluble P-selectin to this complication, we measured plasma P-selectin levels in 32 orthotopic liver transplantations, pre-, intra-, and post-operatively. We found that levels of circulating P-selectin were not different between cirrhotic patients and healthy subjects. Of the 32 patients, 8 had vascular complications. We found a significant increase in the plasma P-selectin concentration in the thrombotic group in early postoperative period compared with the non-thrombotic group (p < 0.002). It measurement may facilitate the diagnosis of thrombosis in the early postoperative period after liver transplantation, and therefore the management of these patients.

Adult↗

[Clinical experience in a case of orthotopic liver transplantation by use of an atypical caval vein anastomosis (author's transl)].

The two main indications for liver grafting are primary malignancy and parenchymatous liver disease. In both cases orthotopic transplantation remains the preferred technique. Since graft rejection was considered not to be the main problem in clinical liver transplantation, recent improved survival rates were reported to be due to a more aggressive diagnostic management and consequent treatment of postoperative complications nonrelated to graft rejection. Because of the limited number of organs available for organ grafting sometimes technical modifications may become necessary. This was the case in a 40-year old male patient suffering from primary malignant hepatoma. The donor was a child, and during operation a great difference between organ size and length and diameter of the hepatic vessels became evident. Orthotopic transplantation was performed using an unusual method of caval vein anastomosis. Initially the patient did very well, but later on liver function deteriorated and the patient died in the eighth postoperative week because of hepatic artery thrombosis. The autopsy showed that all other vascular anastomosis were patent and no signs of portal hypertension were evident. The surgical technique used in this case is described in detail and some interesting aspects are discussed.

Adult↗

Hepatic vascular diseases: CT and MRI.

Continued development of CT and MRI techniques has allowed these modalities to detect most hepatic vascular diseases, often obviating more invasive tests. In this article, the techniques for hepatic vascular diagnosis are reviewed, with emphasis on spiral CT and two-dimensional MRI techniques, and then the major vascular diseases on which these two modalities have a significant diagnosing impact are considered.

Budd-Chiari Syndrome↗

Direct bypassing of extrahepatic portal venous obstruction in children: a new technique for combined hepatic portal revascularization and treatment of extrahepatic portal hypertension.

BACKGROUND: Decompression of extrahepatic portal hypertension by directly bypassing the thrombosed portal vein has never been reported in cases of children with idiopathic (or neonatal) portal vein obstruction and cavernoma. METHODS: Seven children (15 years or younger) with portal vein obstruction requiring surgical decompression (urgently in two cases), and in whom preoperative Doppler had shown that the intrahepatic portal branches were hypoplastic but free of thrombus, were included in a pilot study. The cavernoma was bypassed by interposing a venous jugular autograft between the superior mesenteric vein and the distal portion of the left portal vein. Patients received follow-up using routine clinical parameters, upper gastrointestinal endoscopy, and Doppler ultrasound. RESULTS: The mesenterico-portal bypass restored a direct (physiological) hepatopetal portal flow. The operation resulted in effective portal decompression as demonstrated by decrease of the pressure gradient, rapid regression of clinical signs of portal hypertension, and definitive control of bleeding. CONCLUSIONS: This study shows that direct bypassing of portal cavernoma is possible and results in effective portal decompression. Restoration of the hepatic portal flow is a major advantage compared with conventional surgical shunting procedures. This new technique is potentially applicable to two thirds of children with portal vein thrombosis and should be considered when shunting procedures are indicated.

Adolescent↗

Radiochemotherapy and transplantation allow long-term survival for nonresectable hilar cholangiocarcinoma.

Results of liver transplantation in the treatment of cholangiocarcinoma have been poor as a result of the high incidence of locoregional dissemination and tumor recurrence. This study evaluates the effect of neoadjuvant chemoradiation therapy combined with orthotopic liver transplantation in a carefully selected group of patients with hilar cholangiocarcinoma. Seventeen patients were included in the study. The neoadjuvant protocol included 6,000 cgy biliary brachy-therapy delivered through percutaneous transhepatic catheters and intravenous infusion of 5-fluorouracil (300mg/m2/day) until transplantation. Five of the 17 patients demonstrated tumor progression precluding transplantation. One patient died of sepsis on the waiting list. Eleven patients underwent liver transplantation, a median of 3.4 months (range = 1-26 months) after diagnosis. Five of the 11 (45%) are alive without evidence of tumor recurrence with a median follow up of 7.5 years (range = 2.8-14.5 years). Six deaths occurred in the transplanted patients. Tumor recurrence was responsible for two deaths at 10 and 18months, respectively, after transplantation. Three mortalities resulted from bacterial or fungal peritonitis and sepsis. One patient underwent re-transplantation for chronic rejection and died from graft failure resulting from hepatic artery thrombosis 16 months after diagnosis without evidence of tumor recurrence. Complications of transhepatic catheter placement included bile duct perforation (n = 4) and biliary-portal vein fistula (n = 1). All these patients died of tumor recurrence or sepsis. Cholangiocarcinoma should not be considered an absolute exclusion criteria for orthotopic liver transplantation. Long-term, tumor-free survival was achieved in 45% of the transplanted patients. Complications of biliary catheter placement for brachytherapy were associated with poor outcome.

Adult↗

[Partial Budd-Chiari syndrome associated with a hypereosinophilic syndrome].

We report a rare case of hypereosinophilic syndrome associated with a partial obstruction of the hepatic veins (Budd-Chiari syndrome). The case was assessed by ultrasonic examination, computed tomography and magnetic resonance tomography. We postulate an association because endothelium damage and thrombosis is reported in hypereosinophilic syndromes. Elevated liver enzymes in a patient with hypereosinophilic syndrome could be associated with a Budd-Chiari syndrome.

Aged↗

Transjugular intrahepatic portosystemic shunt in the management of Budd Chiari syndrome.

Budd Chiari syndrome presents with a wide range of severity and duration of symptoms. Transjugular intrahepatic portosystemic shunt has been used to treat selected Budd Chiari syndrome patients for several years. The technique of transjugular intrahepatic portosystemic shunt may be more challenging than in cirrhosis because of hepatic vein occlusion. Covered transjugular intrahepatic portosystemic shunt stents have reduced the requirement for follow-up interventions. Transjugular intrahepatic portosystemic shunt has been a successful bridge to liver transplant for Budd Chiari syndrome but is the definitive treatment in many cases. Patient selection is important to determine who will benefit from transjugular intrahepatic portosystemic shunt or other treatments such as hepatic vein recanalization or liver transplant.

Budd-Chiari Syndrome↗

Venocclusive disease of the liver: prospective study of US evaluation.

Twenty-one bone marrow transplantation (BMT) patients were studied prospectively to determine the prevalence of sonographic hepatobiliary abnormalities and to determine if these abnormalities were associated with hepatic venocclusive disease (VOD). Baseline US was performed in all patients prior to chemoradiation therapy, with follow-up ultrasound (US) examinations at the time of BMT and 14 days and 28 days after BMT. Sonograms were reviewed for the presence of ascites, gallbladder wall thickening, hepatosplenomegaly, hepatofugal flow, hepatic vein compression, increased periportal echogenicity, and increased hepatic echotexture. The baseline scans showed 13 of 21 patients (62%) with abnormalities prior to BMT. Within 2 weeks after BMT, serial US showed interval development of hepatomegaly in five patients (three with VOD and two without), gallbladder wall thickening in one (with VOD), hepatic vein compression in two (both with VOD), hepatofugal flow in one (without VOD), and ascites in one (with VOD). US scans obtained 4 weeks after BMT in 15 of the 21 patients showed even fewer new abnormalities. No sonographic finding was strongly associated with VOD.

Adult↗

Index of suspicion.

Explore the source record for details and available documents.

Chelating Agents↗

Vascular diseases and MR angiography of the liver.

Delineation of the portal vein and its major attributes is essential for assessing portal hypertension and its complications. MR imaging appears to be well-suited for such an evaluation. This article provides an overview of the MR imaging techniques used to assess the portal system as well as highlights of the more common etiologies associated with altered portal venous flow.

Budd-Chiari Syndrome↗