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PubMedDate not supplied

Membranous obstruction of the inferior vena cava with Budd-Chiari syndrome in children: a report of nine cases.

Membranous obstruction of the inferior vena cava (IVC) with Budd-Chiari syndrome is a rare condition in children. Nine children with this lesion are described, eight of them from the Ovambo tribe in Namibia. Clinical features included hepatomegaly, splenomegaly, ascites, and dilated superficial abdominal veins draining cephalad below the umbilicus. Liver enzymes and serum proteins were slightly abnormal in most cases, and the ascitic fluid, when present, had a high protein content. Liver histology showed a wide range of changes, from almost normal to signs of severe chronic congestion. Angiographic appearance varied from a thin membrane between the IVC and right atrium to nonvisualization of the IVC except for dilated collaterals. Simultaneous injection of contrast medium into catheters positioned in the upper IVC and right atrium, through both the femoral and antecubital routes, is the most accurate way of demonstrating the extent of the obstruction and the patency of the hepatic veins.

Ascitic Fluid↗
PubMed1997

Portal vein complications after liver transplantation for biliary atresia.

The objective of this report is to review portal complications (PC) after pediatric liver transplantation (LT) for biliary atresia (BA) in the Bicêtre surgical series. From January 1, 1988, to February 28, 1995, 96 children with BA underwent 115 LTs Portal anastomosis was done on either the recipient portal vein (n = 85) or superior mesenteric vein (n = 11). No antiaggregative agents were administered postoperatively. Median follow-up was 50 months (range, 12 to 97). Nineteen PC (16.5%) occurred in 17 recipients: 16 portal thrombosis (PT) and 3 portal stenosis (PS). Fifteen instances of early PT occurred between days 0 and 17 (median, day 2). Emergency thrombectomy was performed in 9 cases (successful in 5). Three children underwent a secondary portosystemic shunt (successful in 2). Three PS were cured by either surgery or balloon dilatation. Four children died, 3 are alive with portal hypertension (PHT), and 10 are alive without PHT. Three-year patient actuarial survival is 82.4% in PC cases and 82% in others (NS). Significant risk factors of PC are young age and weight at the time of LT, small portal vein, and emergency LT. Analysis of our own results and review of the literature suggest that prevention of PC depends primarily on appropriate surgical technique. Reduction of postoperative hypercoagulability may also play an important role: a meta-analysis of 1,257 published pediatric LT show an overall risk of PT of 2.2% in teams using aspirin with or without dipyridamole compared with 7.8% when no antiaggregative agents are given (P = .0001).

Adolescent↗
PubMed1992

[A device for hepatic arterial catheterization using saphenous vein graft].

We performed regional arterial infusion chemotherapy for hepatocellular carcinoma by retaining a hepatic arterial reservoir. The catheters were placed via gastroduodenal artery and positioned at the junction with common hepatic artery during operation. But we encountered many complications and did not perform regional arterial infusion chemotherapy. This time we cannulated through the saphenous vein graft by which hepatic perfusion was maintained by the superior mesenteric artery; we did not cannulate through the common femoral artery using a subcutaneously implanted reservoir. Venous arterial anastomosis is performed with interrupted (7-0 Prolene) vascular suture. Postoperative angiography by the reservoir showed total hepatic perfusion. There have been no instances of hemorrhage, thrombosis, or catheter dislodgement with this technique. Duration of follow-up varied from 2 to 7 months.

Carcinoma, Hepatocellular↗
PubMedDate not supplied

Nontumorous attenuation changes on CT arterial portography.

OBJECTIVE: We prospectively studied the frequency and the distribution of nontumorous attenuation changes during CT arterial portography. MATERIALS AND METHODS: Computed tomography arterial portograms were obtained in 67 consecutive patients. The frequency and distribution of nontumorous attenuation changes (pseudolesions, areas of hypo- or hyperperfusion) were analyzed and related to the size of the tumor, the vascular status assessed by Doppler examination, and the presence of chronic liver disease. RESULTS: Pseudolesions were detected in 19 patients (28%). They were typical in 13 cases and atypical (round shaped or located in the caudate or right lobe) in 6. Segmental, lobar, or diffuse perfusion defects were seen in 36 patients (54%). In 56% of the cases, they were adjacent to large tumors and related to compression or thrombosis of intrahepatic portal branches as demonstrated by sonographic and Doppler findings. Areas of hyperperfusion were detected in 14 patients (21%). Of these cases, eight (57%) were peritumoral hyperperfusion. In all cases, the mean diameter of the tumors was large and there was compression of adjacent hepatic vein as demonstrated by sonographic and Doppler findings. CONCLUSION: Nontumorous attenuation changes are common in CT arterial portography. Factors leading to these artifacts include size of the tumor and vascular consequences of the tumor. Sonographic and Doppler studies may play a role in predicting these factors.

Adolescent↗
PubMed1994

Portal vein thrombosis: imaging findings.

Portal vein thrombosis (PVT) is usually a complication of pre-existing cirrhosis, abdominal malignancy (e.g., pancreatic or hepatocellular carcinoma), or abdominal inflammation (e.g., appendicitis, diverticulitis, pancreatitis). Less frequently, PVT can be associated with myeloproliferative or connective tissue disorders or inflammatory bowel disease [1]. PVT can cause or exacerbate portal hypertension; variceal bleeding or hypersplenism may then develop acutely or several years later. PVT also complicates portosystemic shunt surgery or hepatic transplantation. Unfortunately, the signs and symptoms of PVT can be subtle or nonspecific and can be overshadowed by the underlying illness. The radiologist may be the only physician to suggest the preoperative or premortem diagnosis of PVT. Familiarity with the imaging findings of PVT, therefore, is imperative.

Adult↗
PubMedDate not supplied

Low-molecular-weight heparin and calcium heparin in thrombosis prophylaxis in patients with percutaneous arterial and venous ports for colorectal liver metastases.

STUDY OBJECTIVE: The evaluation of low-molecular-weight heparin use to prevent arterial and venous thrombosis in patients with indwelling arterial Port-a-Cath implants. METHODS: From 1996 to March 2003 we placed 370 indwelling hepatic arterial catheters with a minimally invasive approach. The left distal subclavian artery was approached from beneath the left clavicle, then an angiographic study of the tumoral vascular district was performed and the gastroduodenal artery was occluded by an embolus. A polyurethane catheter was introduced distally into the hepatic artery and connected to a reservoir through a 3-4 cm long subcutaneous tunnel. In 90 patients a venous Port-a-Cath was placed for concurrent systemic chemotherapy. All 370 patients received regional chemotherapy and were treated with calcium heparin at a dose of 5000 IU twice a day and with low-molecular-weight heparin at prophylactic doses (dalteparin 2500 IU or nadroparin 3000 IU) during catheter permanence to prevent hepatic artery thrombosis. Intra-arterial trans-port radionuclide scans using technetium-99m-labeled micro-aggregated albumin were performed monthly to check the infusion distribution and hepatic artery patency. In the presence of anomalous patterns, thrombosis, pulmonary embolism or other complications, angiography and/or other diagnostic studies were performed to determine the cause of the vascular event and the local or systemic symptoms. The mean arterial and venous Port-a-Cath permanence times were 6 and 8 months, respectively. RESULTS: We observed episodes of hepatic artery thrombosis in 4.3% of patients. Three of these 17 patients were successfully treated by intra-arterial thrombolysis using urokinase. No venous thrombosis occurred as a consequence of regional and/or systemic chemotherapy, no episodes of arterial thrombosis were registered during arterial catheter permanence, nor did any hemorrhagic complications related to anti-coagulant therapy occur. Five patients treated with low-molecular-weight heparin required treatment suspension due to a platelet count of < 40,000/dL. CONCLUSION: Our experience suggests that low-molecular-weight heparin and/or calcium heparin at prophylactic doses could be useful in the prevention of arterial and venous thrombosis in patients with indwelling arterial catheters or venous Port-a-Cath treated with regional or systemic chemotherapy for hepatic metastases from colorectal cancer. The homogeneity of the patient group and the use of analogous chemotherapeutic drugs (fluoropyrimidines) avoided statistical contamination related to differences between kinds of cancer and between the chemotherapeutic agents used.

Antineoplastic Agents↗
PubMedDate not supplied

Septic thrombosis of the portal vein: CT and clinical observations.

The CT and clinical findings were reviewed in seven patients with septic thrombosis of the portal vein (STPV). Of the seven patients, five had associated pyogenic liver abscesses. Five of seven patients presented de novo with STPV without a clinically obvious extrahepatic source of intraabdominal infection. All seven patients were successfully managed nonsurgically with intravenous antibiotics and in two patients percutaneous drainage of hepatic abscesses. Serial follow-up examinations in five patients demonstrated complete resolution of portal venous thrombus in three patients and progression to cavernous transformation in two. When diagnosed early by CT or sonography, STVP may have a more benign clinical course following appropriate antibiotic therapy.

Humans↗
PubMed1979

Interposition mesocaval shunt for chronic primary occlusion of the hepatic veins.

Five patients with primitive chronic Budd-Chiari syndrome were treated by Dacron interposed mesocaval shunts for medically uncontrollable ascites. In two instances, hepatomegaly and ascites disappeared for four and four and one-half years. In one patient with severe stenosis of the inferior vena cava, moderate ascites required tapping once a month one year later, despite proved prosthesis patency. In two patients, death occurred ten and 30 days after shunting due to thrombosis of the graft. Inferior vena cava stenosis appears to be the major factor for decision of opportunity and type of portacaval shunt. From our material, we can describe three types of stenosis: type I, due to caudate lobe hypertrophy, and type II, due to right lobe hypertrophy, are suitable for side-by-side portacaval or mesocaval shunts. Type III, regular and extended narrowing of inferior vena cava, observed in long term evolutive forms, is presumably due to fibrosis and is not a good indication for conventional infrahepatic shunting procedures. Since this study was completed, another patient had a side-to-side portacaval anastomosis for chronic Budd-Chiari syndrome without caval stenosis. The patient has been observed for seven months, and ascites did not reappear. This underlines the importance of a complete radiologic and hemodynamic preoperative study of inferior vena cava outflow impairment.

Adolescent↗
PubMed2005

[Atypical presentation of distant metastases from hepatocarcinoma].

Hepatocarcinoma (HCC) is the most frequent primary malignant hepatic tumour. These tumours usually develop in cirrhotic liver; for this reason, periodic screening using alphafetoprotein determination and abdominal ultrasonography is considered in cirrhotic patients with preserved hepatocellular function. This strategy allows early detection of HCC, increasing the proportion of curable tumours. The most frequent metastasic dissemination is portal vein neoplasic thrombosis, being unusual the occurrence of spread metastases in other organs. We present 3 cases of atypical HCC metastasis with non specific clinical manifestations which initial diagnosis was wrong. Because of a longer survival of these patients in recent years, spread metastasis might be considered in patients with known HCC and non specific symptoms.

Aged↗
PubMed1990

Liver transplantation for the Budd-Chiari syndrome.

A retrospective study was performed that analyzed 23 patients who had an orthotopic liver transplantation for the Budd-Chiari syndrome with end-stage liver disease. Patient follow-up was as long as 14 years. The technical considerations relevant to the Budd-Chiari syndrome were discussed. There have been no serious complications of postoperative anticoagulation. Three patients, all of whom died, had recurrence of the Budd-Chiari syndrome. No other patient has had evidence of recurrent Budd-Chiari syndrome on postoperative liver biopsies. One-, 3-, and 5-year actuarial survival was 68.8%, 44.7%, and 44.7%, respectively. It was concluded that orthotopic liver transplantation is the most effective treatment for patients with the Budd-Chiari syndrome and end-stage liver disease.

Adolescent↗
PubMedDate not supplied

Essential fatty acid status in infants and children with chronic liver disease.

The relationship between essential fatty acid (EFA) status and degree of hyperbilirubinaemia and oxidant stress in infants and children with chronic liver diseases was evaluated. Thirty patients with chronic cholestasis and 30 with liver cirrhosis were examined; 30 healthy subjects served as controls. Patient groups had significant decreases in EFAs and significant elevation of total bilirubin. Levels of thiobarbituric acid reactive substances were significantly raised and were significantly inversely correlated to decreased EFA levels. There were also significant decreases in retinol, alpha-tocopherol and alpha-tocopherol/total lipids ratio, which had significant positive correlations with decreased EFA levels. Infants and children with chronic liver diseases have a high risk of EFA deficiency correlated with progressive elevation of serum bilirubin and progressive deterioration of oxidant status.

Adolescent↗
PubMed1985

[Sequential angio-urography (SAU) with photographic image subtraction].

Urography was performed in 250 patients: the technique of bolus injection of a large quantity of contrast medium was used in every case. By means of rapid seriography the early vascular phase was shown and reproduced, when necessary, with image subtraction. The abdominal aorta and the renal arteries were demonstrated in 97.6% of cases. Altogether, in 34.4% of cases the existence of pathological conditions non demonstrable by conventional urography was ascertained; in 12.4% of cases extrarenal pathology was diagnosed. This technique is suggested to be used sistematically, as an alternative to DSA, where digital equipments are not available.

Adolescent↗