Eduardo Carone

Liver transplantation for arteriohepatic dysplasia (Alagille's syndrome).

Abstract. Thirteen out of 268 children (18 years old) underwent hepatic transplantation (OLT) for end‐stage liver disease (ESLD) associated with arteriohepatic dysplasia (AHD). Seven children are alive and well with normal liver function. Six children died, four within 11 days of the operation and the other two at 4 and 10 months after the OLT. Vascular complications with associated septicemia were responsible for the deaths of three children. Two died of heart failure and circulatory collapse, secondary to pulmonary hypertension and congenital heart disease. The remaining patient died of overwhelming sepsis not associated with technical complications. Seven patients had a portoenterostomy or portocholecystostomy early in life; five of these died after the OLT. Severe cardiovascular abnormalities in some of our patients suggest that complete hemodynamic monitoring with invasive studies should be performed in all patients with AHD, especially in cases of documented hypertrophy of the right ventricl. The improved quality of life in our surviving patients confirms the validity of OLT as a treatment of choice in cases of ESLD due to AHD.

Four hundred thirty consecutive pediatric living donor liver transplants: Variables associated with posttransplant patient and graft survival

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 (São 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. Liver Transpl, 2012.

Modified Pediatric End-Stage Liver Disease Scoring System and Pediatric Liver Transplantation in Brazil

The Pediatric End‐Stage Liver Disease (PELD) scoring system is a formula developed to provide a continuous numerical assessment of the risk of death in order to allocate livers to children in need of transplantation. The PELD scoring system was introduced in Brazil in July 2006. An important change was made in the system: the final number for listing patients less than 12 years old for transplantation was the calculated PELD score multiplied by 3. The consequences of this allocation policy were analyzed in 2 ways in this research: nationally and in the state of São Paulo (SP State). In the analysis of the national data, a comparison of the pre‐PELD era (July 2003 to July 2006) and the post‐PELD era (August 2006 to April 2009) showed that the total number of pediatric transplants for children under 12 years of age decreased 7%. Regionally, in SP State, there was a 62% increase in the number of deceased donor liver transplantation procedures for the pediatric population after the introduction of the modified PELD system. There was also a 6.1‐fold increase in split liver transplantation as well as a statistically significant decrease in the time on the waiting list (P < 0.001). In conclusion, changing the allocation policy in Brazil in order to benefit pediatric patients on the waiting list had different results according to analyses of national and regional data. A significant increase in deceased donor liver transplantation/split liver transplantation and a shorter time on the waiting list were observed in SP State. The modified PELD scoring system is simple and optimizes the utilization of deceased donor liver grafts in centers performing pediatric transplants. Liver Transpl, 2010.

Living donor liver transplantation for children in Brazil weighing less than 10 kilograms

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 <10 kg. From October 1995 to October 2006, a total of 222 LDLT in patients <18 years of age were performed; 129 primary LDLT and 7 retransplants (4 LDLT and 3 deceased donor grafts) were performed in 129 infants weighing <10 kg. Forty‐seven patients received grafts with graft‐to‐recipient weight ratio (GRWR) of >4%. 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 = 0.009). Grafts with GRWR >4% 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 <10 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. Liver Transpl 13:1153–1158, 2007.

Schistosoma mansoni infection in the liver graft: The impact on donor and recipient outcomes after transplantation.

The increasing number of transplants performed each year has led to the identification of unusual diseases in liver grafts from asymptomatic donors that were unrecognized before liver transplantation. Here we report our experience with patients who received liver grafts infected with schistosomiasis. From September 1991 to August 2010, 482 pediatric liver transplants were performed at A. C. Camargo Hospital/Sírio‐Libanês Hospital (São Paulo, Brazil). For the identification of Schistosoma mansoni infections, pathology slides for the recipients were reviewed; these included postreperfusion and follow‐up liver biopsy samples. We were able to identify 6 cases of schistosomiasis transmitted through infected grafts (5 of these grafts were from living donors). All living donors were confirmed to have normal liver chemistries, negative fecal tests for parasitic diseases, and normal abdominal ultrasound findings. Liver biopsy was not performed before transplantation. In all cases, features of schistosomiasis were absent in the liver explants. The living donors were treated with praziquantel and were taught to avoid risk factors for reinfection. No specific treatment for schistosomiasis was given to the recipients. There were no perioperative deaths, but 2 recipients died after living donor liver transplantation (LDLT) because of Kaposis sarcoma and non‐Hodgkins lymphoma. In conclusion, using liver grafts infected with S. mansoni eggs did not compromise the results of LDLT in this pediatric cohort. Because of the parasites life cycle and the therapeutic target of praziquantel, only donors should be treated for the infection. Three years of follow‐up showed an uneventful recovery for the living donors. Liver Transpl 17:1299–1303, 2011.

Left Lateral Segmentectomy for Pediatric Live-Donor Liver Transplantation : Special Attention to Segment IV Complications

Background. During left lateral segmentectomy for live-donor liver transplant, the vascular inflow to segment IV can be compromised. An area of ischemia can be seen intraoperatively and further segment IV resection may be needed to prevent necrosis and abscess formation. Methods. From July 1995 to February 2007, 324 consecutive living donor liver transplantations were performed at Hospital A. C. Camargo and Hospital Sirio-Libanes, Sao Paulo, Brazil. Two hundred eleven left lateral segments were transplanted in this period. Data on 204 left lateral segments donors were available for this analysis. Results. There were 108 female and 96 male donors. Median age was 29 years (range, 16–48 years). Median follow-up time was 2.2 years (range, 2 months–11.8 years). Median intensive care unit stay was 1 day (range, 1–3 days), and median hospital stay was 5 days (range, 4–47 days). Postoperative complications were encountered in 39 donors (19.1%). Partial segment IV resection on the course of the primary surgery due to parenchyma discoloration was required in 107 cases (52.5%). Ten patients (4.9%) developed segment IV necrosis or abscesses, although four of them had had segment IVB resection intraoperatively. Segment IV necrosis or abscess significantly increased hospital stay and the number of readmissions, from 5.5±3.5 days to 8.4±3.7 days (P=0.012) and from 6 of 194 (3%) to 5 of 10 (50%) (P=0.001), respectively. Conclusions. Middle hepatic segment abscess or necrosis was the most frequent complication after left lateral segmentectomy (4.9%). Objective intraoperative strategies need to be developed to evaluate middle hepatic segment ischemia to identify and treat patients at higher risk.

Portal vein obstruction after liver transplantation in children treated by simultaneous minilaparotomy and transhepatic approaches: Initial experience

Carnevale FC, Santos ACB, Seda‐Neto J, Zurstrassen CE, Moreira AM, Carone E, Marcelino ASZ, Porta G, Pugliese R, Miura I, Baggio VD, Guimarães T, Cerri GG, Chapchap P. Portal vein obstruction after liver transplantation in children treated by simultaneous minilaparotomy and transhepatic approaches: Initial experience.
Pediatr Transplantation 2011: 15: 47–52.

Liver transplantation from living related donors

OBJECTIVES: To present the experience with the first 12 living related liver transplants performed at Hospital Sírio-Libanês in São Paulo. METHODS: The donors were the fathers (6) and the mothers (6) with age ranging from 30 to 48 years. All candidates for donation were submitted to a full informed consent form, clinical and radiological evaluation and had blood withdrawn for autotransfusion. Recipient age ranged from 7 months to 10 years whereas recipient weight varied from 6.3 to 34 kg. Six patients were considered as high risk due to complications of advanced liver disease and were submitted to urgent transplantation. RESULTS: Mean donor hospital stay was 10 days with no mortality. Technical complications were observed in 4 recipients. Seven patients presented at least one episode of bacterial, viral or fungal infection. One or more biopsy proven rejection episodes were disclosed in 7 patients. Overall recipient survival was 67%, being 83% for elective cases and 50% for urgent cases. Long term follow up ranged from 8 to 25 months. Seven out of 8 survivors present excellent quality of life and normal liver function. The other patient is currently under reduced immunosuppression due to Epstein-Barr virus infection.CONCLUSIONS: These results demonstrate the safety and viability of living related liver transplantation which, in face of the current donor scarcity, should be considered as a valid option for the treatment of children with end stage liver disease.