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PubMedDate not supplied

Surgical complications and long-term outcome in pediatric liver transplantation.

BACKGROUND/AIMS: Liver transplantation has been widely accepted for the treatment of children with end-stage liver disease over the last 10 years particularly with the advent of reduced-size liver transplant technique. This study reviewed the perioperative and long-term results in the pediatric program of the Queensland Liver Transplant Service, Brisbane, Australia. METHODOLOGY: Retrospective analysis was performed in 153 children who received 176 liver grafts between 1985 and 1995, including 109 (62%) reduced-size and 67 (38%) whole liver grafts. Median follow-up period was 5.3 years. RESULTS: One-, 5-, and 10-year patient and graft survival rates were 82% and 74%, 75% and 63%, and 70% and 60%, respectively. Normal physical and intellectual development was observed in 98% of survivors. There were no significant differences in patient or graft survival rates between transplants using reduced-size and whole liver grafts. Portal vein thrombosis was the most common vascular complication, occurring in 8%. Hepatic artery thrombosis occurred in 7%, including 11% of children less than 1 year old and 8% of those under 10 kg. Biliary complication was found in 16% and posttransplant gastrointestinal perforation in 19%. CONCLUSIONS: Liver transplantation has the potential to cure and allow development in children with end-stage liver disease.

Adolescent↗

PubMed1997

Nashville experience with liver transplantation in Veterans Administration patients.

In this study, we compared results of 28 liver transplants performed in 25 patients referred through the Veterans Administration with 82 transplants performed in 75 nonveteran patients. The evaluation and follow-up care was provided by the same team of physicians and nurses and the transplant procedure performed in the same hospital for both patient groups. There was a significantly greater incidence of hepatitis C and/or previous alcohol abuse in veteran compared with non-VA patients [23/25 (92%) vs 29/75 (39%); P < 0.05] and a greater incidence of native portal vein thrombosis [6/25 (24%) vs 2/75 (2.6%); P < 0.01], but no difference in Child's-Pugh score (10.8 vs 9.9) or UNOS listing status (mean status 2.7 vs 2.8). The increased incidence of native portal vein thrombosis did not appear to be solely related to previous alcohol abuse or hepatitis C, since only 1 of 29 (3.4%) non-VA patients with these etiologies had this finding. There was no difference in patient or graft survival between the VA and non-VA groups with overall actuarial 6-, 12-, and 18-month patient survival of 86, 84, and 83% and graft survival of 80, 78, and 77%. There was no difference in major complication rates but there was a significantly longer average hospital stay (27 +/- 31 vs 18 +/- 12 days; P < 0.05) in the VA compared with non-VA group. One patient with native portal vein thrombosis in the non-VA group developed portal vein thrombosis in the postoperative period. There was no documented recidivism in any patient with a history of prior substance abuse in either group. This study confirms that veteran patients have a higher incidence of hepatitis C and previous alcohol abuse as causes of liver disease, have a higher incidence of native portal vein thrombosis, and have longer mean hospital stays, but experience the same survival in the first 18 months compared with nonveteran patients.

Adolescent↗

PubMed2006

Fatal thrombosis of antithrombin-deficient mice is rescued differently in the heart and liver by intercrossing with low tissue factor mice.

BACKGROUND: We previously reported that the targeted disruption of murine antithrombin (AT) gene resulted in embryonic lethality before 16.5 gestational days (gd) because of severe cardiac and hepatic thrombosis. OBJECTIVE AND METHODS: To investigate the influences of lowered tissue factor (TF) activity upon hypercoagulation of AT-/- embryos, we crossed AT+/- with low TF (mTF-/- hTF+) mice to yield homozygous AT-deficient mice with the extremely low TF activity, that is expressed from the inserted human TF mini gene. RESULTS: AT-/- embryos either with 50% TF (AT-/- mTF+/- hTF+) or with low (approximately 1% TF, AT-/- mTF-/- hTF+) were not born, although the survival was prolonged until 18.5 gd. In both genotypes, histological examination showed disseminated thrombosis in hepatic sinusoidal space or in the portal veins, suggesting that the thrombogenesis caused loss of hepatic blood flow. As in original AT-/-, AT-/- mTF+/- hTF+ showed subcutaneous (s.c.) bleeding and also suffered from the myocardial degeneration apparently because of coronary thrombus formation. However, AT-/- mTF-/- hTF+ had no skin hemorrhage and the thrombosis and degeneration were completely abolished in the heart. Myocardium of adult low TF mice had exhibited fibrosis secondary to hemorrhage; however, it was significantly decreased in low TF mice with AT+/-. CONCLUSIONS: Our current model suggests that, in the heart, TF plays an important role in the thrombogenesis and it counterbalances AT-dependent anticoagulation. AT may be a potent anticoagulant during mice development and the activation and subsequent regulation of TF-procoagulant activity take place differently between the liver and the heart. These differences appear to point to local regulatory mechanisms in murine hemostasis.

Animals↗

PubMed2001

Thrombosis of intracardiac or extracardiac conduits after modified Fontan operation in patients with azygous continuation of the inferior vena cava.

BACKGROUND: The Fontan procedure in patients with azygous continuation of the inferior vena cava, requires a cavo-pulmonary anastomosis, and deviation of the hepatic venous drainage to the pulmonary arteries using an intra- or extracardiac conduit. METHODS: We report thrombosis of two pericardial conduits and one Gore-Tex (W. L. Gore & Assoc, Flagstaff, AZ) graft used for deviation of hepatic venous blood to the pulmonary arterial tree in 3 patients aged 11, 24, and 28 years. Two of the conduits (pericardial) were intraatrial. The Gore-Tex graft was placed in an extracardiac position. The two pericardial conduits obstructed completely. RESULTS: One patient died at reoperation. In the 2nd patient, the conduit was excised and the hepatic veins were allowed to drain into the atrium. In the 3rd patient, partial thrombosis of the Gore-Tex conduit was noted 30 months after operation. The thrombus resolved with oral anticoagulation. CONCLUSIONS: Conduits carrying only hepatic venous blood flow may have a higher risk of thrombosis. Anticoagulation or alternative methods of directing hepatic blood flow to the pulmonary circulation must be considered in these patients.

Adult↗

PubMed2004

Combined liver vein and spleen pulp pressure measurements in patients with portal or splenic vein thrombosis.

BACKGROUND: Patients with thrombosis of the portal or splenic vein may develop portal hypertension with bleeding from oesophageal or gastric varices. The relevant portal pressure cannot be measured by liver vein catheterization or transhepatic puncture of the portal vein because the obstruction is peripheral to the accessible part of the portal system. METHODS: Liver vein catheterization was combined with percutaneous splenic pressure measurement in 10 patients with portal or splenic vein thrombosis and no cirrhosis, and 10 cirrhotic patients without thrombosis. The splenic pressure was measured by percutaneous puncture below the curvature of the ribs with an angle of the needle to skin of 30 degrees in order to minimize the risk of cutting the spleen if the patient took a deep breath. RESULTS: None of the patients in whom the described procedure was followed had complications. Pressure measurements in the spleen pulp and splenic vein were concordant. The pressure gradient across the portal venous system (splenic-to-wedged hepatic vein pressure) was -1.3 to 8.5 mmHg (median, 2.8 mmHg) in cirrhosis patients and 0-44 mmHg (median, 18 mmHg) in thrombosis patients, the variation reflecting various degrees of obstruction to flow in the portal venous system. Peripheral portal pressure (splenic-to-free liver vein pressure gradient) was 1.1-28 mmHg (median, 17 mmHg) in cirrhotic patients and 11-52 mmHg (median, 23 mmHg) in thrombosis patients. CONCLUSIONS: Liver vein catheterization combined with percutaneous splenic pressure measurement is feasible in quantifying pressure gradient across a thrombosis of the portal/splenic vein and in quantifying portal pressure peripheral to this kind of thrombosis.

Adult↗

PubMed2006

Vascular complications after pediatric liver transplantation from the living donors.

Early arterial or portal vein thrombosis is a complications that can lead to graft loss and patient death or need of immediate retransplantation. The aim of the study was to assess the incidence, causes, treatment, and outcome of vascular thrombosis after living related donor liver transplantation (LRdLTx). Between 1999 and 2004 71 LRdLTx were performed in children aged from 6 months to 10 years. Vascular thrombosis was found in 12 recipients. Hepatic artery thrombosis (HAT) occurred in 4 (5.6%), portal vein thrombosis (PVT) in 8 (11.2%) cases. HAT occurred 5 to 8 days, PVT 1 to 22 days after LTx. Diagnosis of vascular thrombosis was confirmed by routine Doppler ultrasound examination. Thrombectomy was successful in one patient with HAT and in three patients with PVT. Venous conduit was performed in one patient with PVT after second thrombosis. Two children developed biliary strictures as a late complication of HAT and required additional surgical interventions. Two children with PVT developed portal hypertension with esophageal bleeding, which required surgical intervention; one another underwent endoscopic variceal ligation for grade III varices. Follow-up ranged from 7 to 60 months. One patient died as a result of HAT after retransplantation due to multiple intrahepatic abscesses 2 months after first transplant. Any risk factors of vascular thrombosis that can be controlled should be avoided after transplantation. Routine posttransplant Doppler examination should be performed at least twice a day within 7 to 14 posttransplant days. Immediate thrombectomy should be always carried out to avoid late complications and even mortality.

Child↗

PubMed2005

Budd-Chiari syndrome: current options in interventional radiology treatment exemplified by three selected cases.

BACKGROUND: Budd-Chiari syndrome (BCS) is rare clinical state characterized by stenosis or complete obstruction of hepatic veins. Currently, interventional radiology techniques are more frequently used as a single method of treatment or as a bridge to liver transplantation. CASE REPORT: This study presents current interventional radiology techniques used in BCS treatment. Depending on the etiology of BCS, two main techniques are used: the transjugular intrahepatic portocaval shunt (TIPS) or percutaneous angioplasty (PTA) of the stenosed hepatic veins. Our first case was treated by PTA of the stenosed ostium of the hepatic vein. In the second, BCS was complicated by portal vein thrombosis and a TIPS was placed along with portal vein fibrynolysis. In the third case the TIPS was used as a single interventional radiological treatment. CONCLUSIONS: TIPS placement or angioplasty of hepatic vein ostium stenosis allow the successful treatment of BCS or an extension of the period of waiting for a liver transplantation.

Adult↗

PubMed1977

Portal vein anomaly and hepatic encephalopathy in a horse.

Periodic episodes of diffuse central nervous system disease occurred in a yearling Thoroughbred gelding that had a history of frequent respiratory tract disease and stunted growth. Hepatic encephalopathy was diagnosed on the basis of history, clinical signs, prolonged bromsulphalein clearance, and increased blood ammonia content. Because of the poor prognosis and recurrent clinical signs the horse was euthanatized. Necropsy revealed an arteriovenous anomaly and thrombosis of the portal vein. Histologically, there was diffuse primary astrocytosis of the brain.

Animals↗

PubMed1996

[The clinical value of laminin determination in advanced liver cirrhosis].

OBJECTIVE: To test prospectively whether serum laminin levels, which is taken to indicate portal hypertension, can predict the occurrence of severe complications in advanced cirrhosis of the liver. PATIENTS AND METHODS: In 38 patients (21 men, 17 women; mean age 55.6 +/- 13.4 years) with liver fibrosis (n = 4) or liver cirrhosis (n = 34) serum laminin was measured by a commercially available radioimmunoassay (Behring, Marburg). The severity of liver cirrhosis was graded according to the Child-Pugh-Christensen criteria. Portal hypertension was assessed by standard endoscopic methods and portal-vein duplex sonography. Within a mean observation period of 12.5 +/- 3.5 months, the following were used as signs of severe clinical complications of liver cirrhosis: stages III and IV of hepatic coma, treatment-refractory ascites, portal vein thrombosis and death due to multi-organ failure. Acute bleeding from oesophageal varices was confirmed by emergency endoscopy. RESULTS: At laminin concentrations of 3.25 +/- 0.20 U/ml there was a highly significant correlation (P < 0.001) with complications of liver cirrhosis. Using 2.6 U/ml as the critical level, the occurrence of severe complications had a positive predictive value of 0.80 with a sensitivity and specificity of 0.71 and 0.86 respectively. This means that a patient who, at the beginning of the study period, had a raised laminin concentration, had a relative risk of 2.65 (1.41-4.97) for later severe complications. CONCLUSION: Serum laminin concentration has a diagnostic efficiency of 0.79 as a prognostic indicator and can thus serve as a valuable addition to the Child-Pugh-Christensen classification of liver cirrhosis.

Adult↗

PubMed2005

Resolution of Budd-Chiari syndrome due to activation of endogenous fibrinolysis that may be induced by weight reduction.

We describe a 45-year-old female with polycythemia vera and Leiden factor V mutation, who suffered the subacute form of Budd-Chiari syndrome and was treated with anticoagulants and diuretics. Surprisingly, after 3 months clinical signs of Budd-Chiari syndrome resolved; venography disclosed the resolution of thrombosis in the vena cava inferior and hepatic veins. This was associated with considerable increase of endogenous fibrinolytic activity, documented by a substantial change in the euglobulin clot lysis time, and a decrease of plasminogen activator inhibitor antigen and activity. During the disease the patient followed a diet and significantly reduced her body weight. Putting all data together it could be speculated that weight reduction (along with anticoagulants) considerably activated endogenous fibrinolysis, resulting in spontaneous resolution of Budd-Chiari syndrome. The validity of this explanation should be explored in a larger clinical study.

Anticoagulants↗

PubMedDate not supplied

US of liver transplants: normal and abnormal.

Whole-liver transplantation is an accepted and successful method of treating end-stage liver disease. As a result of the shortage of cadaveric livers, split-liver transplantation and living donor liver transplantation are becoming more commonplace. Ultrasonography (US) is the initial imaging modality of choice for detection and follow-up of early and delayed complications from all types of liver transplantation. Vascular complications include thrombosis and stenosis of the hepatic artery, portal vein, or inferior vena cava, as well as hepatic artery pseudoaneurysms and celiac artery stenosis. Biliary complications include leaks, strictures, stones or sludge, dysfunction of the sphincter of Oddi, and recurrent disease. Neoplastic disease in the transplanted liver may represent recurrent neoplasia or posttransplantation lymphoproliferative disorder. Parenchymal disease may take the form of a focal mass or a diffuse parenchymal abnormality. Perihepatic fluid collections and ascites are common after liver transplantation. Knowledge of the surgical technique of liver transplantation and awareness of the normal US appearance of the transplanted liver permit early detection of complications and prevent misdiagnosis.

Adult↗

PubMed1994

IVOX in ARDS: respiratory effects and serious complications.

We describe the beneficial effects of an intravenous oxygenator (IVOX) on ventilatory pressure requirement, and also several severe complications related to its use in a young trauma victim developing severe acute respiratory distress syndrome (ARDS). In this patient, adequate systemic oxygenation could not be maintained despite the use of fractional inspired oxygen of 1.0, high level positive end-expiratory pressure (PEEP), and nitric oxide inhalation (30 ppm). The introduction of an intravenous oxygenator improved arterial oxygenation and CO2 elimination, allowing a decrease in minute ventilation and airway pressure. However, hepatic cytolysis, acute renal dysfunction and iliac vein thrombosis developed concomitantly, in spite of full anticoagulation with heparin. These complications resolved rapidly after removal of the IVOX device and the patient made a good recovery.

Adult↗

PubMed1988

[Hypertrophic cardiomyopathy mimicking clinical picture of Budd-Chiari syndrome].

A case of a patient with clinical picture of hepatosplenomegaly, portal hypertension, dilatation of hepatic veins and inferior vena cava, without venous thrombosis or other causes of obstruction of right-sided heart, is described. This picture is compatible with the Budd-Chiari syndrome. Echocardiography has shown a hypertrophic cardiomyopathy causing relevant dilatation of both atria and it has allowed us to exclude the presence of a constrictive pericarditis. The hypertrophic cardiomyopathy is first considered as a cardiac cause of cirrhosis mimicking the Budd-Chiari syndrome.

Aged↗

PubMed1994

The Budd-Chiari syndrome: clinical presentation and diagnostic findings in 45 patients treated by surgery.

We report on the clinical presentation and diagnostic findings in 45 patients with Budd-Chiari syndrome. The diagnosis was confirmed by histology at the time of liver transplantation (n = 37) or shunt surgery (n = 8). An underlying disorder could be established only in half of the patients, oral contraceptives as predisposing factor were known in 18 cases. Clinically, abdominal pain and distension as well as hepatomegaly and ascites were most frequent findings, whereas changes in laboratory data were more or less unspecific. By use of repeated ultrasound, a definite diagnosis of a Budd-Chiari syndrome could be confirmed in all cases by obligatory demonstration of obstruction or thrombosis of at least one major liver vein. Hepatic venography revealed only one false-negative result. Celio-mesenteric arteriography plus portography, cavography and preoperative liver biopsy did not present additional diagnostic information. These techniques may contribute to treatment planning of portosystemic shunt surgery or hepatic transplantation. In conclusion, the presence of hepatosplenomegaly, ascites, abdominal pain and distension, especially in combination with a known hypercoagulable state, should alert to the possibility of a Budd-Chiari syndrome. Ultrasound is the diagnostic tool of choice. Hepatic venography should only be performed if even repeated ultrasound is not conclusive.

Adolescent↗

PubMed1991

Liver transplantation in patients with portal vein thrombosis and central portacaval shunts.

The authors have analyzed the impact of pre-existing portal vein pathology on the outcome of orthotopic liver transplantation. The incidence was high in patients suffering from chronic active hepatitis, hypercoagulable states, trauma or previous dissection of the porta hepatis, and splenectomy. The existence of portal vein thrombosis (23 patients) or surgical central portosystemic shunt (10 patients) was documented by preoperative Doppler sonogram or angiography (26/33), or operative findings of occluded vein (7/33). Successful thrombectomy and dismantling of portacaval shunts were achieved in most cases (24/33). Only nine patients required the placement of an interposition vein graft to the superior mesenteric vein. The intraoperative course was characterized by increased blood loss and coagulopathy, significantly higher than in patients with a patent portal vein. When compared with all liver transplants, the immediate postoperative complication rate was higher for primary nonfunction (33% versus 8%), re-exploration for intraperitoneal bleeding and hematomas, and morbid infections. Rethrombosis rate of thrombectomized veins or vein graft was low (2/33). The mortality rate was 35% in the presence of portal vein thrombosis (PVT) and 30% for portacaval shuct (PCS), both significantly higher than the 12% for other orthotopic liver transplant (OLT) patients. These results are expected to improve with better patient selection, surgical experience, and anticipation of the complex postoperative course. The authors conclude that PVT or the presence of PCS are not contraindications to orthotopic liver transplantation.

Blood Loss, Surgical↗

PubMed1995

Orthotopic liver transplantation for hepatic-based metabolic disorders.

Between January 1989 and June 1993, a total of 470 liver transplantations were performed at King's College Hospital. Thirty-seven transplantations were performed in 34 patients with liver-based metabolic disease. There were 16 females and 18 males with a median age of 19 years (range 1 month to 62 years). There were 14 patients under 16 years of age. The indications for liver transplantation were Wilson's disease (n = 16), alpha 1-antitrypsin deficiency (n = 10), tyrosinaemia (n = 2), primary hyperoxaluria type 1 (PH1; n = 2), congenital haemochromatosis (n = 1), familial amyloidotic polyneuropathy (FAP; n = 1, familial hypercholesterolaemia) (n = 1) and Crigler-Najjar syndrome type I (CNS1; n = 1). These included two patients who received combined heart-liver grafts for familial hypercholesterolaemia and FAP, respectively. Two patients received combined liver-kidney transplants for PH1. There were four deaths: from sepsis (n = 2), acute hepatic vein obstruction in a left lateral segment graft (n = 1) and portal vein thrombosis with liver necrosis (n = 1). Three patients were retransplanted, one for chronic rejection and two for hepatic artery thrombosis, giving an overall graft survival of 81% and patient survival of 88% (30/34), at a mean follow-up of 34 months (range 10-64 months).

Acute Disease↗

PubMed1998

Paediatric liver transplantation: Queen Mary Hospital experience.

OBJECTIVE: To assess the results of paediatric liver transplantation in our institution. METHODS: From September 1993 to November 1996, 10 living-related liver transplants (LRLT) and 3 reduced-size liver transplants (RSLT) were performed on 12 children at our hospital. The medical records of the patients were reviewed. All patients suffered from end-stage liver disease resulting from biliary atresia with failed Kasai's operations. Their ages at initial transplantation ranged from 8 months to 11 years. Excluding the 2 older children aged 7.5 and 11 years, the remaining patients were aged 10.5 months on the average and weighed 6 to 9.5 kg (mean: 6.8 kg) at the time of initial transplantation. RESULTS: All living donors were discharged on postoperative day 4 to 8 and resumed their previous normal activities. All recipients were alive with normal liver function and growing after a follow-up period of 3-40 months (mean: 21 months). The patient survival rate was 100%. One patient with RSLT had hepatitis of undetermined aetiology and underwent retransplant with a graft from her mother. The graft survival rate was 92%. Postoperative complications included: postoperative bleeding (n = 3), hepatic vein stenosis (n = I), biliary-enteric anastomotic stenosis (n = 3), intestinal perforation (n = I) and portal vein thrombosis (n = I). They were all treated promptly. In all patients, the hepatic artery (diameter ranged from 1.5 to 2.5 mm) anastomosis was achieved by microvascular technique. There was no hepatic artery thrombosis in our patients. CONCLUSION: With technical refinements, early detection and prompt treatment of complications, and advances in immunotherapy, excellent results can be achieved in paediatric liver transplantation.

Biliary Atresia↗

PubMed2005

Multislice spiral CT angiography in evaluation of liver transplantation candidates.

BACKGROUND: Orthotopic liver transplantation has become the treatment of choice for patients with end-stage liver disease. This study was designed to study whether multislice spiral CT angiography (MSCTA) could be used for preoperative evaluation for orthotopic liver transplantation candidates. METHODS: Eighty consecutive potential candidates for liver transplantation were evaluated with dual-phase three-dimensional CT angiography (3DCTA). The arterial-phase was used to create vascular maps of the celiac axis (including the origin of the hepatic common artery, left gastric artery and splenic artery) and origin of the superior mesenteric artery. The portal venous-phase was used to analyze portal vein thrombosis and collateral vascularization of the portal vein. Statistical analyses were made using the chi-square test for differences between hepatic arterial anatomy of 80 patients and Michel's type I anatomy of 200 patients. Appearance of MSCTA and operative results of 16 patients were analyzed. RESULTS: Sixty-two patients (77.5%) showed conventional and 18 (22.5%) nonconventional hepatic arterial anatomy. A significant difference was found between the two groups in anatomy of the hepatic artery (P<0.05). Celiac axis stenosis was observed in 6 patients, SA aneurysm in 2, small-caliber hepatic arterial vessels in 2, and portal vein thrombosis in 15. Vascular structures of 16 operative patients were well defined. CONCLUSION: As a noninvasive examination, MSCTA can provide a comprehensive preoperative vascular evaluation for liver transplantation candidates.

Adult↗