Domino transplantation for pediatric liver recipients: Obstacles, challenges, and successes (original) (raw)
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Transplantation, 2018
Data describing the technical aspects of living donor domino liver transplantation (LD-DLT) in maple syrup urine disease (MSUD) are limited. The largest published series includes only 3 cases. One great challenge of this procedure is to ensure adequate vascular stumps for the living donor, the MSUD patient, and the recipient of the domino graft. Here, we describe our experience in 11 cases of LD-DLT in MSUD, highlighting the technical aspects of LD-DLT. From September 2012 to September 2017, 11 patients with MSUD underwent LDLT at our institution, and MSUD livers were used as domino grafts in 11 children. 1- MSUD PATIENTS: Ten patients received a left lateral segment. The donor's left hepatic vein was anastomosed to the confluence of the recipient's 3 hepatic veins (HV). No venous grafts (VG) were required for portal vein (PV) anastomosis. Single arterial anastomosis was performed with microsurgery in 10 out of 11 patients. 2- MSUD GRAFT RECIPIENTS: In 8 cases, HV reconstruc...
Current Concepts in Pediatric Liver Transplantation
Mount Sinai Journal of Medicine: A Journal of Translational and Personalized Medicine, 2012
Liver transplantation is the definitive treatment for end‐stage liver disease in both children and adults. Advances over the last 2 decades have resulted in excellent patient and graft survival rates in what were previously cases of fatal disorders. These developments have been due to innovations in surgical technique, increased surgical experience, refinements in immunosuppressive regimens, quality improvements in intraoperative anesthetic management, better understanding of the pathophysiology of the liver diseases, and better preoperative and postoperative care. Remarkably, the use of split‐liver and living‐related liver transplantation surgical techniques has helped mitigate the well‐recognized national organ shortage. This review will discuss the major aspects of pediatric liver transplantation as it pertains to indication for transplantation, recipient selection and listing for orthotopic liver transplantation, pre‐orthotopic liver transplantation care of children, optimal tim...
Successful domino liver transplantation in maple syrup urine disease using a related living donor
Brazilian Journal of Medical and Biological Research, 2014
Maple syrup urine disease (MSUD) is an autosomal recessive disease associated with high levels of branched-chain amino acids. Children with MSUD can present severe neurological damage, but liver transplantation (LT) allows the patient to resume a normal diet and avoid further neurological damage. The use of living related donors has been controversial because parents are obligatory heterozygotes. We report a case of a 2-year-old child with MSUD who underwent a living donor LT. The donor was the patient's mother, and his liver was then used as a domino graft. The postoperative course was uneventful in all three subjects. DNA analysis performed after the transplantation (sequencing of the coding regions of BCKDHA, BCKDHB, and DBT genes) showed that the MSUD patient was heterozygous for a pathogenic mutation in the BCKDHB gene. This mutation was not found in his mother, who is an obligatory carrier for MSUD according to the family history and, as expected, presented both normal clinical phenotype and levels of branched-chain amino acids. In conclusion, our data suggest that the use of a related donor in LT for MSUD was effective, and the liver of the MSUD patient was successfully used in domino transplantation. Routine donor genotyping may not be feasible, because the test is not widely available, and, most importantly, the disease is associated with both the presence of allelic and locus heterogeneity. Further studies with this population of patients are required to expand the use of related donors in MSUD.
Pediatric Liver Transplantation: A Surgical Perspective and New Concepts
Current Transplantation Reports, 2014
The first successful liver transplantation was carried out in 1967, and the recipient was a pediatric patient. Since then, many challenges have been overcome and, nowadays, 1-year patient survival after liver transplantation is about 90 % in pediatric patient populations. Standardization of surgical technique, development of better immunosuppressive medications, improved understanding of caring for patients with end-stage liver disease, and improvements in anesthesia and intensive care have had a tremendous impact on graft and patient survival. Despite all improvements, challenging issues in pediatric liver transplantation remain. In this article, we address some of these problems. and living donor liver transplantation (LDLT); improvement of intensive care treatment; development of new immunosuppressive medications; improved understanding of the balance between immunosuppression and opportunistic infection; and the adoption of a multidisciplinary team approach to patient care, which includes collaboration between transplant surgery, pediatric hepatology, pediatric intensive care, nephrology, pulmonary, cardiology, infectious disease, psychiatry, social work, pharmacy, and nutrition.
Pediatric Liver Transplantation for Inherited Metabolic Liver Disease: A Single-Center Experience
Transplantation Proceedings, 2011
Objective. We performed single-center outcome comparison of pediatric recipients who underwent liver transplantation for either genetic or metabolic disease including the clinical impact of using heterozygote parents as living donors. Materials and Methods. Pediatric liver transplant recipients from September 2007 to December 2010 were included. Patients were separated into 2 categories by etiology of liver disease: (1) genetic or metabolic liver disease (G/M) and (2) nongenetic or metabolic liver disease (non-G/M), which included all other remaining etiologies combined. Patient demographics, recipient and donor characteristics, graft type, operative data, recipient complications, allograft and patient survival were analyzed. Results. Forty liver transplants were performed on 40 patients; 18 were transplanted for G/M; mean waiting time was 101 days for G/M group and 57 days for non-G/M group; 9 patients were listed as status 1, 5 were granted PELD/MELD exceptions; the overall mean PELD/MELD score was 21. Four G/M patients had hepatocellular carcinoma in the explant without microvascular invasion. Overall complications requiring either surgery or interventional radiology occurred in 14 patients-G/M (n ϭ 5); CMV viremia was seen in 11 patients (G/M, n ϭ 1); detectable EBV DNA was detectable in 8 patients (G/M, n ϭ 4), acute cellular rejection was seen in 10 (G/M, n ϭ 5), postransplant lymphoproliferative disease occurred in 2 G/M patients; and 1 G/M patient showed significantly improved posttransplant neurologic motor function. Children with G/M who received a living donor liver transplant from heterozygote parents did well without any signs of expressing underlying metabolic disease. Posttransplant graft and patient overall survival at 12 months for G/M and non-G/M was 100%, and at 36 months, 83% and 100%, respectively. Conclusion. The majority of children transplanted for either genetic or metabolic disease were status 1 or awarded UNOS exception points. Cadaveric split livers and live donors including obligate heterozygotes resulted in excellent allograft and patient survival outcomes. In metabolic and genetic liver diseases, close follow-up and timely transplantation can preclude malignant spread and prevent disease progression and consequences, as well as reverse neurologic sequelae.
Living Donor Liver Transplantation for Pediatric Patients with Inheritable Metabolic Disorders
American Journal of Transplantation, 2005
Forty-six pediatric patients who underwent living donor liver transplantation (LDLT) using parental liver grafts for inheritable metabolic disorders (IMD) were evaluated to determine the outcomes of the surgery, decisive factors for post-transplant patient survival and the impact of using donors who were heterozygous for the particular disorder. Disorders included Wilson disease (WD, n = 21), ornithine transcarbamylase deficiency (OTCD, n = 6), tyrosinemia type I (TTI, n = 6), glycogen storage disease (GSD, n = 4), propionic acidemia (PPA, n = 3), methylmalonic acidemia (MMA, n = 2), Crigler-Najjar syndrome type I (CNSI, n = 2), bile acid synthetic defect (BASD, n = 1) and erythropoietic protoporphyria (EPP, n = 1). The post-transplant cumulative patient survival rates were 86.8 and 81.2% at 1 and 5 years, respectively. Posttransplant patient survival and recovery of the growth retardation were significantly better in the liveroriented diseases (WD, OTCD, TTI, CNSI and BASD) than in the non-liver-oriented diseases (GSD, PPA, MMA and EPP) and pre-transplant growth retardation disadvantageously affected post-transplant outcomes. Although 40 of 46 donors were considered heterozygous for each disorder, neither mortality nor morbidity related to the heterozygosis has been observed. LDLT using parental donors can be recommended as an effective treatment for pediatric patients with IMD. In the non-liver-oriented diseases, however, satisfactory outcomes were not obtained by hepatic replacement alone.
Liver transplantation for pediatric metabolic disease
Molecular Genetics and Metabolism, 2014
a b s t r a c t a r t i c l e i n f o Available online xxxx Keywords: Inborn errors of metabolism Liver transplant Hepatocyte transplant Liver failure Organic acidemia Amino aciduria
Twenty Years of Experience in Pediatric Living Donor Liver Transplantation
Transplantation, 2016
Background. Hepatic artery thrombosis (HAT) increases morbidity and mortality after liver transplantation (LT). The identification of risk factors for HAT may aid transplant teams in the development of strategies aimed at reducing HAT. This article describes the risk factors for HAT and outcomes after LT. Methods. This report describes a retrospective study (1995 to 2015) of primary pediatric living donor LT (LDLT). Pretransplant and technical variables were included in the study. Binary logistic regression was used for data analysis. Results. This study included 656 primary LDLT. The median age, body weight, and pediatric end-stage liver disease score at the time of transplant were 13 months, 8.4 kg and 15, respectively. Twenty-one (3.2%) patients developed HAT. Intraoperative HAT (odds ratio, 62.63; 95% confidence interval, 12.64-310.19; P < 0.001) and the use of liver grafts with a graft-to-recipient weight ratio less than 1.1% (odds ratio, 24.46; 95% confidence interval, 4.55-131.56; P < 0.001) retained statistical significance in the multivariate model. Patient and graft survivals were significantly worse in cases with HAT. The overtime trend analysis revealed a decrease in the incidence of HAT (P = 0.008) and an increase in the use of 2-arterial anastomosis (P < 0.001). Conclusions. A graft-to-recipient weight ratio of 1.1% or less and intraoperative HAT were independently associated with HAT. Trend analysis further revealed a significant reduction in the incidence of HAT over time, as well as the increased use of 2 hepatic arteries for anastomosis during graft implantation. The double artery anastomosis may represent an extra protection to pediatric recipients undergoing LDLT.
Pediatric liver transplantation for metabolic liver disease: experience at King's College Hospital
Transplantation, 2009
The aims of this article were to report a single-center experience of pediatric liver transplantation for liver-based metabolic disorders and to compare the outcome of cirrhotic versus noncirrhotic metabolic liver disease. The medical records of 96 patients younger than 18 years undergoing transplantation for liver-based metabolic disorders from 1989 to 2005 were reviewed. Hundred twelve transplants were performed in 96 patients at a median age of 59.7 months (range, 0-208 months). The cumulative 1-, 5-, and 10-year graft and patient survival rates were 83%, 77%, and 62% and 91%, 86%, and 82%, respectively. Acute liver failure at first presentation (hazard ratio [HR] 3.0; 95% confidence interval [CI] 1.1-8.1), age less than 1 year at time of transplantation (HR 4.6; 95% CI 1.7-12.4) and hospitalization (HR 3.2; 95% CI 1.1-9.3) were significant predictors of worse patient survival. For noncirrhotic disorders, the long-term patient (100% vs. 100%, 90% vs. 100%, and 90% vs. 75%, P=0.87...
2014
Forty-six pediatric patients who underwent living donor liver transplantation (LDLT) using parental liver grafts for inheritable metabolic disorders (IMD) were evaluated to determine the outcomes of the surgery, decisive factors for post-transplant patient survival and the impact of using donors who were heterozygous for the particular disorder. Disorders included Wilson disease (WD, n = 21), ornithine transcarbamylase deficiency (OTCD, n = 6), tyrosinemia type I (TTI, n = 6), glycogen storage disease (GSD, n = 4), propionic acidemia (PPA, n = 3), methylmalonic acidemia (MMA, n = 2), Crigler-Najjar syndrome type I (CNSI, n = 2), bile acid synthetic defect (BASD, n = 1) and erythropoietic protoporphyria (EPP, n = 1). The post-transplant cumulative patient survival rates were 86.8 and 81.2% at 1 and 5 years, respectively. Posttransplant patient survival and recovery of the growth retardation were significantly better in the liveroriented diseases (WD, OTCD, TTI, CNSI and BASD) than in the non-liver-oriented diseases (GSD, PPA, MMA and EPP) and pre-transplant growth retardation disadvantageously affected post-transplant outcomes. Although 40 of 46 donors were considered heterozygous for each disorder, neither mortality nor morbidity related to the heterozygosis has been observed. LDLT using parental donors can be recommended as an effective treatment for pediatric patients with IMD. In the non-liver-oriented diseases, however, satisfactory outcomes were not obtained by hepatic replacement alone.