Survival for pediatric HCC patients is significantly inferior to that for children with hepatoblastoma. Complete tumor excision remains the only realistic chance of cure, although it is often prevented by advanced disease. The presence of metastases is the most potent predictor of poor prognosis. A prospective worldwide cooperation in the field of pediatric HCC should be encouraged to look for novel therapeutic concepts.

The incidence of biliary complications (BCs) after living donor liver transplantation (LDLT) can reach 40%. Published data on the pediatric population are limited, and treatment protocols vary. Our aim was to describe the clinical scenario for BCs and treatment approaches after LDLT. Between October 1995 and December 2012, 489 pediatric LDLT procedures were performed. BCs developed in 71 patients (14.5%). Biliary strictures (BSs) developed in 45 (9.2%) patients, and bile leaks (BLs) developed in 33 patients (6.7%). The BL diagnosis was clinical in all cases, and 69.7% of the patients underwent surgery. Nearly half of the BS cases had clinical features or suggestive ultrasound findings. Liver biopsy findings suggested BSs in 51.7%. Percutaneous transhepatic cholangiography was performed in 95.6% of the BS patients. The success rate was 77% [mean number of percutaneous biliary interventions (PBIs) 5 3.9 6 1.98, median drainage time 5 8 months]. In conclusion, BL patients can be managed with conservative therapy, even though most of these patients will ultimately be treated with surgery. Diagnosing a BS requires a high degree of clinical suspicion because the available resources for its identification can fail in up to 50% of cases. A higher number of PBIs and the use of a drainage catheter for a longer time may be required to achieve better results with this technique. Liver Transpl 20:882-892,

Liver transplant is considered to be the last-resort treatment approach for pediatric patients with end-stage liver disease. Despite the remarkable advance in survival rates, liver transplant remains an intricate surgery with significant morbidity and mortality. Early diagnosis of complications is crucial for patient survival but is challenging given the lack of specificity in clinical presentation. Knowledge of the liver and vascular anatomy of the donor and the recipient or recipients before surgery is also important to avoid complications. In this framework, radiologists play a pivotal role on the multidisciplinary team in both pre- and postoperative scenarios by providing a road map to guide the surgery and by assisting in diagnosis of complications. The most common complications after liver transplant are (a) vascular, including the hepatic artery, portal vein, hepatic veins, and inferior vena cava; (b) biliary; (c) parenchymal; (d) perihepatic; and (e) neoplastic. The authors review surgical techniques, the role of each imaging modality, normal posttransplant imaging features, types of complications after liver transplant, and information required in the radiology report that is critical to patient care. They present an algorithm for an imaging approach for pediatric patients after liver transplant and describe key points that should be included in radiologic reports in the pre- and postoperative settings. Online supplemental material is available for this article. RSNA, 2017.

The availability of living donors allows transplant teams to indicate living donor liver transplantation (LDLT) early in the course of liver disease before the occurrence of life-threatening complications. Late referral to transplant centers is still a problem and can compromise the success of the procedure. The aim of this study was to examine the perioperative factors associated with patient and graft survival for 430 consecutive pediatric LDLT procedures at Sirio-Libanes Hospital/A. C. Camargo Hospital (Sã o Paulo, Brazil) between October 1995 and April 2011. The studied pretransplant variables included the following: recipient age and body weight, Pediatric End-Stage Liver Disease score, z score for height/age, bilirubin, albumin, international normalized ratio, hemoglobin, sodium, presence of ascites, and previous surgery. The analyzed technical aspects included the graft-to-recipient weight ratio and the use of vascular grafts for portal vein reconstruction. In addition, the occurrence of hepatic artery thrombosis (HAT), portal vein thrombosis (PVT), and biliary complications was also analyzed. The liver grafts included 348 left lateral segments, 5 monosegments, 51 left lobes, and 9 right lobes. In a univariate analysis, an age < 12 months, a low body weight ( 10 kg), malnutrition, hyperbilirubinemia, and HAT were associated with decreased patient and graft survival after LDLT. In a multivariate analysis, a body weight 10 kg and HAT were significantly associated with decreased patient and graft survival. The use of vascular grafts significantly increased the occurrence of PVT. In conclusion, a low body weight ( 10 kg) and the occurrence of HAT independently determined worse patient and graft survival in this large cohort of pediatric LDLT patients.

Ascites is the most common complication of cirrhosis and in adults it is associated with 50% mortality at 5 years if patients do not receive a liver transplant. The occurrence of hyponatremia in these patients has been associated with increased mortality on the waiting list. The importance of serum sodium levels and the presence of ascites in the pediatric setting remain to be clarified. A retrospective analysis of pediatric patients with cirrhosis on the transplant list was carried out between October 2000 and February 2012. The primary objective of this study was to evaluate the association of pretransplant variables with mortality within 90 days following the inclusion of patients on the waiting list. In all, 522 patients were included in the study; 345 (66%) patients were under 1 year of age; 208 (40%) of the children presented ascites. A multivariate Cox proportional hazards analysis was conducted and total bilirubin (P < 0.001, hazard ratio [HR] 5 2.09, 95% confidence interval [CI] 5 1.35-3.21), international normalized ratio (INR) (P < 0.001, HR 5 9.83, 95% CI 5 4.51-21.45), serum sodium levels (P 5 0.03, HR 5 0.96, 95% CI 5 0.92-0.99), ascites (P 5 0.001, HR 5 2.59, 95% CI 5 1.44-4.64), and categorized age (0-1 versus 1 year old) (P 5 0.025, HR 5 2.33, 95% CI 5 1.11-4.86) were independently associated with risk of death in 90 days. Malnutrition (Z score height/age, weight/age) and serum albumin (pediatric endstage liver disease [PELD] formula) were not included in the final model. Conclusion: The presence of ascites and serum sodium levels are important variables associated with decreased patient survival while candidates wait for a liver graft. Multicenter studies are necessary to validate these findings in order to improve current allocation policies based on the PELD score. (HEPATOLOGY

The technique of vascular reconstruction plays a major role in the outcome of living donor liver transplantation (LDLT). An increased use of vascular grafts (VGs) as replacements for sclerotic portal veins has become a standard technique for our group. The aim of this study was to analyze the factors associated with portal vein thrombosis (PVT) in pediatric LDLT. We performed a retrospective analysis of 486 primary pediatric LDLT procedures performed between October 1995 and May 2013. VGs used for portal reconstruction included living donor inferior mesenteric veins, living donor ovarian veins, recipient internal jugular veins, deceased donor iliac arteries, and deceased donor iliac veins. Thirty-four patients (7.0%) developed PVT. The incidence of PVT dropped from 10.1% to 2%; the overall utilization of VGs increased from 3.5% to 37.1%. In a multivariate analysis, only the use of VGs remained an independent risk factor for the occurrence of PVT (hazard ratio 5 7.2, 95% confidence interval 5 2.8-18.7, P < 0.001). There was no difference in survival rates between patients with PVT and patients without PVT. No patient with PVT underwent retransplantation. In conclusion, the use of VGs was independently associated with the development of PVT. Over time, there was a reduction in the incidence of early PVT in this cohort, and there was a trend toward a reduction in total PVT. The occurrence of isolated PVT in this study was not associated with decreased patient or graft survival.

Infants with end-stage liver disease represent a treatment challenge. Living donor liver transplantation (LDLT) is the only option for timely liver transplantation in many areas of the world, adding to the technical difficulties of the procedure. Factors that affect morbidity and mortality can now be determined, which opens a new era for improvement. We have accumulated an 11-year experience with LDLT for children weighing Ͻ10 kg. From October 1995 to October 2006, a total of 222 LDLT in patients Ͻ18 years of age were performed; 129 primary LDLT and 7 retransplants (4 LDLT and 3 deceased donor grafts) were performed in 129 infants weighing Ͻ10 kg. Forty-seven patients received grafts with graft-to-recipient weight ratio (GRWR) of Ͼ4%. Two patients received monosegmental grafts, and 2 patients underwent delayed abdominal wall closure. Portal vein thrombosis occurred in 5.4% of the patients, hepatic artery thrombosis in 3.1%, and both in 1.5%. Among several variables studied, only the bilirubin level at the time of transplantation was associated with increased risk of death (P ϭ 0.009). Grafts with GRWR Ͼ4% had no negative effect on patient survival. There were 7 retransplants, and 4 patients received a second parental LDLT. Patient survival rates at 1, 3, and 10 years after transplantation were 88.8%, 84.7%, and 82% for all children, and 87.5%, 84.9%, and 84.9% for infants weighing Ͻ10 kg. LDLT has results comparable to other modalities of liver transplantation in infants. Monosegment grafts were rarely required in this series, although they may be necessary in patients with lower body weight.

Biliary atresia (BA) is the main diagnosis leading to liver transplantation (LT) in children. When diagnosed early in life, a Kasai portoenterostomy (Kasai‐PE) can prevent or postpone LT. Instances of previous operations can result in difficulties during the LT. We hypothesized that a previous Kasai‐PE could affect LT outcomes. A retrospective cohort study of 347 BA patients submitted to LT between 1995 and 2013 at Hospital Sírio‐Libanês and A. C. Camargo Cancer Center was conducted. Patients were divided into those with a previous Kasai portoenterostomy early failure (K‐EF), Kasai portoenterostomy late failure (K‐LF), and those with no Kasai portoenterostomy (No‐K). Primary outcomes were patient and graft survival. A total of 94 (27.1%) patients had a K‐EF, 115 (33.1%) had a K‐LF, and 138 (39.8%) had No‐K before LT. Children in the K‐LF group were older and had lower Pediatric End‐Stage Liver Disease (PELD) scores. Patients in both K‐EF and K‐LF groups had more post‐LT biliary complications. After Cox‐multivariate analysis adjusting for confounding factors to determine the influence of Kasai‐PE on patient and graft survival, the K‐LF group had an 84% less probability of dying and a 55% less chance to undergo retransplantation. The K‐LF group had a protective effect on posttransplant patient and graft survival. When properly performed, the Kasai procedure can postpone LT and positively affect outcomes. Having a K‐EF and having not performed a Kasai‐PE had the same effect in patient and graft survival; however, a previous Kasai‐PE can increase post‐LT complications as biliary complications and bowel perforations. Liver Transpl 21:922‐927, 2015. © 2015 AASLD.