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Preoperative vascular imaging in pediatric liver transplantation.

BACKGROUND/PURPOSE: There is a considerable variation in the use of vascular imaging techniques in the preoperative assessment of children scheduled for liver transplantation. Duplex Doppler ultrasound scan (US), magnetic resonance angiography (MRA), and conventional angiography are used to varying extents. The authors compared the results of preoperative vascular imaging studies with operative findings to determine their accuracy and usefulness. METHODS: Results of preoperative vascular imaging in 37 consecutive children undergoing cadaveric liver transplantation were compared with operative findings. Those undergoing relatively elective transplantations were investigated by US and MRA (group 1), whereas those requiring urgent transplants were assessed only by US (group 2). RESULTS: The median age of the cohort (15 boys; 22 girls) was 4 years (19 days to 16 years) and the median weight was 17 kg (2.9 to 82 kg). In group 1 (n = 26), 20 children had a normal-caliber, patent portal vein at transplant and 6 had a narrow but patent portal vein requiring venous reconstruction in 4. The sensitivity and specificity of MRA in the detection of an abnormally narrow portal vein were 100% (6/6) and 95% (19/20), respectively. If reversed or absent flow in the portal vein on US was taken as an indication of a potentially abnormal vein, the sensitivity and specificity of Doppler US were 83% (5/6) and 95% (19/20), respectively. Magnetic resonance angiography revealed arterial anomalies in 4 children but failed to detect small accessory hepatic arteries in 5. The single patient with an aberrant vena cava was identified by MRA. In group 2 (n = 11), venous findings at operation and on US were concordant in 10 (91%) cases; one infant with reversed flow in the portal vein on US had a thrombosed vein at surgery. Magnetic resonance angiography was useful in 2 patient groups: those with reversed flow on Doppler US or suspected portal vein thrombosis in whom an abnormal portal vein was present in 86% (6/7) and infants with the biliary atresia splenic malformation syndrome who had multiple venous and arterial anomalies. CONCLUSIONS: A detailed Doppler examination of the hepatic vasculature by an experienced sonographer/radiologist provides sufficient vascular imaging for most children scheduled for cadaveric liver transplantation. Routine MRA is recommended in children with the biliary atresia splenic malformation syndrome and in those with abnormal duplex Doppler US findings. Although there are limited data in this study, MRA is also valuable in children with Budd-Chiari syndrome, liver tumors, or a previous portosystemic shunt.

Adolescent↗

[Treatment of vascular and biliary complications following living related-donor liver transplantation].

Surgery for living related-donor liver transplantation(LRDLT) is difficult compared with orthotopic whole-liver transplantation. It is necessary to find methods both to prevent and treat vascular and biliary complications. Interventional radiological procedures have been increasingly used in recent years for the diagnosis and nonsurgical treatment of these complications, with encouraging results. Vascular complications include technical device of stricture, obstruction, and thrombosis. Hepatic arterial reconstruction requires great skill in LRDLT, although in the author's experience a surgical loupe may suffice for reconstruction in adult LRDLT if the surgeon has sufficient experience in microvascular surgery. Early postoperative life-threatening vascular complications must be treated urgently, including further surgery. Recently duct-to-duct (D-D) biliary reconstruction has been performed in addition to hepatico-jejunostomy in LRDLT. D-D anastomosis has the advantages of simple operative technique shorter duration of surgery, simple postoperative management, normal physiologic route, and elimination of reflux cholangitis.

Bile Duct Diseases↗

Pharmacological thrombolysis in Budd Chiari syndrome: a single centre experience and review of the literature.

BACKGROUND/AIMS: To review our experience of thrombolytic therapy in patients with acute Budd Chiari syndrome (BCS). METHODS: Records of 10 patients with BCS, treated by thrombolysis over a 12-year period were retrospectively analysed for demographics, clinical presentation/duration, primary disease, thrombolytic regimen, and follow-up. The same characteristics were also studied in previously reported patients. The agent used was recombinant tissue plasminogen activator (tPA) in all patients. RESULTS: Thrombolysis was used 12 times in 10 patients. Infusion was made systemically in three patients, into the hepatic artery in one patient, locally into a hepatic vein and/or IVC in four patients and locally within TIPS/portal vein in two patients. Only one infusion made systemically was partially successful. Adjunctive balloon angioplasty and/or stent insertion was undertaken for all eight procedures (in six patients) where local infusion was into the hepatic vein or TIPS. Six of these were ultimately successful (in five patients) and two were unsuccessful. Thrombolysis was more likely to be successful in the presence of a short history of thrombosis, when the thrombolytic agent was locally infused and when it was combined with a successful radiological procedure. Mean follow-up was 4.5 years (range 1-10 years). No serious bleeding complication occurred. CONCLUSIONS: We observed no benefit from thrombolysis when delivered systemically or arterially except in one case. Thrombolysis was useful in adjunctive management of BCS when the drug was infused locally into recently thrombosed veins that had appreciable flow following partial recanalisation. Thrombolysis was clearly of benefit in the repermeation of occluded/partially occluded hepatic veins/TIPS when early detection of new thrombus followed interventional procedures such as balloon angioplasty or stenting of hepatic veins.

Adolescent↗

Cavoportal transposition for portal vein thrombosis in a pediatric living-related liver transplantation.

We report a case of a living-related liver transplantation in a child in whom diffuse thrombosis of the portal venous system required the use of recipient vena cava to perfuse the donor portal vein (cavoportal transposition). An 8 (1/2)-month-old infant with biliary atresia received the left lateral segment of his father's liver. The child's portal vein was irretrievably thrombosed, as were the splenic and superior mesenteric veins. A cavoportal anastomosis provided excellent flow to the liver, and there was no engorgement of the graft, which had good immediate function. The main postoperative problem was loss of 3 to 3.5 liters of ascitic fluid through the drain for 2 weeks. The ascites eventually resolved over a period of 4 weeks. Twenty-four months after transplantation, the child is thriving on a normal diet and has no ascites. A Doppler examination showed good flow in the cavoportal anastomosis.

Anastomosis, Surgical↗

Portal vein thrombosis associated with cirrhosis: clinical importance.

A group of 86 cirrhotics undergoing therapeutic variceal decompressive procedures were studied. Patients with portal vein thrombosis (PVT) comprised 21% of the group and more frequently had uncontrollable hemorrhage at an earlier stage of liver disease. Mortality in the 18 cirrhotics with PVT was higher (56%), mostly as a result of rebleeding. Despite adjustments for stage of liver disease and type of operation, in no cirrhotic with PVT did postshunt encephalopathy develop, compared with 32% incidence in patients without PVT (P less than .05). It is concluded that patients with cirrhosis and PVT represent a different subpopulation of cirrhotics. Once adequate variceal decompression has been achieved, their prognosis should be superior to cirrhotics without PVT because their hepatic hemodynamics are unaffected by total shunting, hence precluding further impairment of liver function as a result of acute reduction of hepatic blood flow.

Adolescent↗

Treatment outcomes for hepatocellular carcinoma using chemoembolization in combination with other therapies.

BACKGROUND: Although transarterial chemoembolization (TACE) improves survival in patients with hepatocellular carcinoma (HCC), it is not known if TACE combined with other treatments is beneficial. AIM: To evaluate the evidence for improved outcomes in HCC with a multimodal treatment approach involving TACE. METHOD: PubMed search for all cohort and randomized trials (n=84) evaluating TACE combined with other therapies; meta-analysis performed where appropriate. RESULTS: A meta-analysis involving 4 RCTs showed a significant decrease in mortality favouring combination treatment (TACE plus percutaneous ablation) compared to monotherapy in patients with either small (<3cm) or large HCC nodules (>3cm) (OR, 0.534; 95% CI, 0.288-0.990; p=0.046). TACE combined with local radiotherapy improved survival in patients with tumour thrombosis of the portal vein in 7 non-randomized studies. Two RCTs and 13 non-randomized studies showed that TACE prior to hepatic resection does not improve survival nor tumour recurrence. Conversely, 2 RCTs and 5 comparative studies showed that transarterial injection of chemotherapeutic drugs mixed with lipiodol (TOCE) following hepatectomy confers survival benefit and less tumour recurrence. TACE before liver transplantation is safe and reduces drop-out rate from the waiting list, but there is no current evidence of improvement in subsequent survival or recurrence rate. CONCLUSIONS: A combined approach involving TACE and percutaneous ablation improves survival. Adjuvant TOCE improves outcome after hepatectomy. TACE is useful to control tumours burden while on the waiting list for OLT. Multimodal treatment seems to be the best way to optimize TACE outcomes in HCC.

Antineoplastic Combined Chemotherapy Protocols↗

Complicated appendiceal inflammatory disease in children: pylephlebitis and liver abscess.

Five children with complicated appendiceal inflammatory disease are reported. They presented with nonspecific signs and symptoms, but three had liver abscess and two had inflammation of the portal vein. The inflamed portal vein may act as a conduit to the liver for bacteria, or it may become thrombosed and cause portal hypertension and hypersplenism. In one child, symptomatic portal hypertension developed 10 years after the initial disease. In children, an ultrasonic finding of a focal liver mass of low-to-mixed echogenicity or the presence of low-attenuation areas on computed tomographic scans should suggest the possibility of a hepatic abscess, and the radiologist has a major role in suggesting complicated inflammatory disease of the appendix as the cause. Similarly, when portal vein thrombosis or portal hypertension are found, the radiologist should consider complicated inflammatory disease of the appendix as the cause.

Adolescent↗

[Portal and pulmonary hypertension in a patient with MCTD].

A 42-year-old woman with mixed connective tissue disease (MCTD) died due to the rupture of esophageal varices. The autopsy revealed fresh thrombi in the main trunk of the portal vein. Microscopic examination disclosed wide-spread periportal fibrosis and stenosis of peripheral portal veins without remodeling of hepatic lobular architecture, which was compatible to idiopathic portal hypertension (IPH). Anti-phospholipid antibody was negative. Accordingly it is likely that portal vein thrombosis developed secondary to IPH. In the literature 6 (37.5%) of 16 collagen vascular disease patients with IPH died, and three of them were due to rupture of esophageal varices. Therefore IPH should be considered to be one of the most important complications affecting its grave prognosis in patients with collagen vascular disease. The patient also had had pulmonary hypertension (PH) when the diagnosis of portal hypertension was made. In the literature we found 5 collagen vascular disease patients with both PH and IPH like this case. The most outstanding common clinical feature among these 6 patients was Raynaud's phenomenon associated with positive anti-RNP antibody. Moreover 5 of 6 cases including this case simultaneously developed both PH and IPH. The clinical course of these patients suggests there may be a common pathogenetic factor for these two lesions. A possible candidate involved in the pathogenesis of PH and IPH may be endothelin, one of the vasoactive substances, since its receptor is said to be expressed abundantly in pulmonary and portal vasculatures. However, further investigation is necessary to elucidate the mechanism of PH and IPH in collagen vascular diseases.

Adult↗

Extrafascial hyper-reduction of the hepatic graft.

The technique and limits of graft reduction in orthotopic liver transplantation (OLT) are not well-defined, especially for small recipients. To reduce the hepatic graft to the left lateral segment (segments II and III), the authors have used an extrafascial hepatectomy without any dissection of the hepatic hilum. Among 165 pediatric OLT, 22 procedures using such hyper-reduced hepatic grafts were performed upon 21 children of median age 2.6 years between 1988 and 1992. These 22 hyper-reduced hepatic grafts were transplanted orthotopically with conservation of the inferior vena cava of the recipient. The three-year patient survival rate was 66 percent (80 and 50 percent for those patients undergoing elective and emergent procedures, respectively). The main postoperative complications were: primary graft nonfunction (n = 1), arterial complications (n = 2), portal vein thrombosis (n = 1), intra-abdominal bleeding (n = 2) and biliary complications (n = 2). This technique is useful in instances of a large size discrepancy between the donor and recipient. However, it does not increase per se the absolute number of hepatic grafts available for transplantation.

Adolescent↗

Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism.

Congenital anomalies of the inferior vena cava such as absence or atresia are uncommon vascular defects and result from aberrant development during embryogenesis. We report a case of a young female patient affected by proximal deep venous thrombosis (DVT) complicated by liver and pulmonary embolism; subsequent extensive evaluation revealed the congenital absence of infrarenal inferior vena cava, with emboli probably occurring through collateral veins. Accordingly, in young patients with idiopathic DVT of the lower extremities and pelvic veins, the presence of inferior vena cava abnormalities should always be considered and investigated, together with classic coagulation factors, as a factor predisposing to thromboembolic complications.

Adult↗

Predictors and outcome of early- versus late-onset major bacterial infections in liver transplant recipients receiving tacrolimus (FK506) as primary immunosuppression.

Major bacterial infections and the predictors of early (within 100 days of transplantation) versus late onset (after 100 days post-transplant) bacterial infections were prospectively assessed in 130 consecutive liver transplant recipients receiving tacrolimus (FK506) as primary immunosuppression. The median follow-up period was 38 months. Overall, 35% (45/130) of the patients developed 67 episodes of major bacterial infections (0.52 episodes/patient). Sixty-three percent of the major bacterial infections occurred early, and 37% occurred in the late post-transplant period. Eighty-four percent of the abdominal infections occurred early, whereas 38% of the cases of pneumonia, 60% of the cases of primary bacteremia, and 50% of the biliary infections occurred late. By logistic regression analysis, portal vein thrombosis was the most significant independent risk factor for early-onset major bacterial infection (odds ratio 4.1; 95% CI 1.4-12.2), and recurrent hepatitis C was the most significant independent predictor of late-onset major bacterial infections (odds ratio 6.21; 95% CI 1.9-20.2). Thus, sources and risk factors differ for early versus late-onset bacterial infections after liver transplantation. Knowledge of the differences in the potential sources, the pathogens, and the predictors of early versus late-onset bacterial infections can be valuable in the evaluation and empiric treatment of liver transplant recipients with bacterial infections.

Adult↗

Vascular complications after liver transplantation: evaluation with Doppler US.

PURPOSE: To demonstrate the spectral and color Doppler ultrasonography (US) findings that would indicate vascular complications after liver transplantation and to report our single center results of vascular complications detected in liver transplant recipients. MATERIALS AND METHODS: Our study was consisted of 326 patients who underwent liver transplantation procedures between November 1997 and May 2004. The records of all patients were reviewed retrospectively for the details of each patient's post-transplant Doppler US examinations, visceral angiographic examinations, and/or surgical procedures. Doppler US findings were correlated with angiographic results or surgery. Sensitivity and specificity of Doppler US parameters for the diagnosis of vascular complications of the hepatic artery, portal vein, and hepatic veins were calculated. RESULTS: Vascular complications occurred in 47 patients (14%). Eight instances of vascular complications were detected intraoperatively by Doppler US at the time of transplantation. For hepatic artery complications, use of a Doppler US criteria resulted in a sensitivity and a specificity of 92% and 97%, respectively. Doppler US parameters also resulted in a sensitivity and a specificity of 100% in detecting portal vein complications, and resulted in a sensitivity of 99% and a specificity of 100% in detecting hepatic vein complications. CONCLUSION: Although it is clear that Doppler US evaluation is an effective choice for diagnosing vascular complications after liver transplantation, we also observed that Doppler US examination plays an important role in detecting vascular complications intraoperatively and improving the patient's chance for a successful outcome.

Adolescent↗

Early postoperative complications following liver transplantation.

Liver transplantation is a highly successful treatment for patients with end-stage liver disease and acute liver failure. However, serious postoperative complications can significantly compromise patient survival. Complications can be technical, medical, or immunological in nature. The risk of developing early postoperative complications is associated with the patient's preoperative condition, the quality of the donor liver, the quality of the donor and recipient procedure, initial graft function, and perioperative anaesthesiological and intensive care management. The patient's preoperative condition can include gastrointestinal bleeding, acute renal failure, a requirement for cathecholamines or mechanical ventilation, and prolonged encephalopathy for the most detrimental risk factors for developing early postoperative complications. The necessity for prolonged mechanical ventilation or the requirement for reintubation after transplantation can significantly increase the risk of developing pneumonia, sepsis, and multiple organ dysfunction. A decrease in infectious and other complications can be achieved by early postoperative enteral nutition, including the application of probiotics.

Constriction, Pathologic↗

Living donor liver transplantation: early single-center experience.

Adult living donor liver transplantation (ALDLT) is an accepted procedure to overcome the organ shortage. The advantages of ALDLT must be balanced against the first concern of donor safety. We analyzed the results of our early experience among a series of eight ALDLT performed between April 2001 and October 2003. All patients were listed as United Network for Organ Sharing UNOS status 2b and 3. Transplant recipients consisted of four men and four women. The living donors included four sons, three daughters, and one son-in-law (ages 20 to 45 years). One donor was anti-HBc-positive and negative for hepatitis B virus-DNA by polymerase chain reaction analysis in serum and in liver tissue. GR/WR >0.8 and fatty liver <10% were considered suitable for the hepatectomy. Residual left lobe volume was at least 33%. No exogenous blood and blood products were transfused into the donors and a cell-saver device was used in all donors (blood loss 490 +/- 160 mL). All procedures were right lobe hepatectomy; in one case the middle hepatic vein was withdrawn with the right graft. The mean ischemia time was 1.5 +/- 0.5 hours. All donors survived the procedure. Median hospital stay was 8.5 +/- 2.1 days in all donors but one who had a long stay because of drug-related hepatitis. One graft was lost and one donor aborted because of preoperative overestimated volumetry. Complications were experienced by two donors (25%). Five recipients (62.5%) experienced major complications; one patient underwent retransplantation because of donor graft loss. Two biliary and two vascular complications (33.3%) occurred in three patients. No perioperative death occurred. Two patients died at 9 and 10 months after transplant because of heart and respiratory failure in the first case and tumor recurrence in the second. One-year actuarial survival is 75%. ALDLT using right lobe has gained acceptance to overcome the organ shortage. Donor selection criteria must be stringent with respect to residual donor hepatic volume, steatosis, and liver function.

Constriction, Pathologic↗